Kealy's Journey with Linear Scleroderma from Mom's Perspective

Januay 10, 2012 visit

2012-01-16T15:04:00.000-06:00

It's 2012 already!

We left about 6:30am headed to the doctors appointment in Dallas. We arrived about 20 minutes before the appointment. Only stopping 1 time and that was in Skiatook for breakfast. Appointment was at 11:30. Not even ONE potty break - I think it is a RECORD!

Kealy's face looks good he said. All incision areas are as expected and looking good. As expected some re-absorption has taken place. He said we will need to come back in 2 months and at that time they will set the next surgery.

Regarding the next surgery, it will be a fat transfer only, but unlike the last time he intends on taking her own stem cells from the hip and using them. I am not sure what changed from this time and the last one - but he and even the lady that was scheduling the appointment (because she thought we were scheduling surgery and not just a check up) said that there would be no problem getting it approved this time. We will have more information on this from the next appointment.

Information on stem cells:
http://en.wikipedia.org/wiki/Mother_cell

She was released to be able to sleep however she pleases ( she is happy to not have to stay off that side of her face anymore ). And she can play softball if she chooses, but no contact sports such as basketball, football, etc.

After the appointment we headed to get gas, then Red Lobster for lunch (which also gave us dinner because of the leftovers). We did some walking around at the outlet mall, it was rainy and dreary so we didn't do to much. Stopped and got a treat at the Chocolate Factory then headed to our hotel. Thank you Traci again for the friends discount. :)

We stayed at the Courtyard in Allen, TX. It was a really nice hotel. We stayed there the whole evening, Kealy messing on the lobby computers, I sat by the fireplace. Then back to our rooms to watch a movie, eat our left overs and bed. Up at 9 and we were out by 10:30. Stopped at the coffee shop across the street for breakfast and then hit the road. We did end up having a few more stops on the way home. Just makes it easier on the body when you don't have to be in the car for 5 straight hours. Thought I would share some photo's of the hotel, as a thank you and to let you know how nice it is in case anyone ever wants to stay there:

2 queen size beds, nice comfy mattresses and loved the pillows.

Long nice fireplace in the sitting area of the lobby, there were several sitting areas.

Kealy on facebook (lol)

Lights above the fireplace, going up 2 stories.

View at night out our window.

Our room

Another view of our room.

View out our window in the morning before heading out.

Just a request for prayers for Kiara in Australia (we ment over the internet) she has very sever PRS (Parry Romberg Syndrome) and will be going in for surgery in the next few days to start fat transfers, then they will decide if they will do bone grafts or whatever the next steps will be. She is a teenager and has dealt with a lot through out her life. Pray they can do a perfect job throughout the process for her. It will be a long journey for her I am sure, much longer then Kealy or myself could ever imagine.
Thanks!

To Texas Tomorrow

2012-01-09T08:35:00.000-06:00

We are on the road again to Texas tomorrow morning bright and early. Kealy's stitches are still healing on her head. I think they would be in much better shape if she would take some time to care for them a little more than she does, but she doesn't want to be bothered by such thing. Ugh, drives me crazy. All the stitches in her mouth have come out I think she said. I thought they were all out previously, but she mentioned just a few days ago another one came out.

Around Christmas I guess (she didn't mention it to me, but to her sister) that she had started feeling the indention again in her forehead. And around the 1st of January it was visible once again. :( This I think was expected by the doctor, will find out for sure tomorrow. That is probably why he said 2 follow up surgeries. But we were hopeful that would not be the case, so when it started showing again it was a little upsetting.

Kealy is dying to dye her hair blond. I should of had this on video when she said it - she said because she wanted to look like me. That took me by surprise! Most the time I feel like her arch enemy. But she is going to have to wait for awhile until her head is healed and it's driving her crazy. In turn she drives me crazy over it.

Thanks to all who are helping fund our trips, I can't tell you how much that is needed and appreciated. And thanks to a friend from High School Jennifer Shaw & family who select a family each Christmas to send a gift too - and selected Kealy this Christmas. With the money she sent Kealy got a pair of tennis shoes (Reebok zig something or other and socks). THANK YOU.

I'm starting to teach Art Lessons to my neice today. This is my first time to do this so I hope it all goes well, she learns and enjoys it. I am also reaching the end of my unemployment and figured I would have had a job a long time ago - I keep trusting God is directing my path. I have had several interviews I thought went really well and felt I 'could' get the job - but it hasn't been so yet. Keep us in your prayers in that area too.

Thanks and I'll update later in the week on what the doctor has to say tomorrow.

The picture vaguely shows the indention (which is not too deep). And her eyelashes are real! Wish I had eyelashes that long :)

First Post Op Visit & What is LS ECDS

2011-12-12T11:19:00.000-06:00

On 12/6/2011 we made the trip back to Texas for her first post-op visit, this time just Kealy and myself.

Kealy a few days before at her sisters house.

We head out at 6:30am, get some breakfast on our way. Make one stop at the our favorite chocolate store at the Allen Town Outlet Mall - this to pick up some goodies for Kealy's doctor. She thought it would be a nice Thank You gift. We made it to the office with 5 minutes to spare!

We go back, the doctor comes in to see her along with his assistant, he said it looks good and he will have his assistant remove the taping and then he will be back. She put something on it to soften it up, and most of it came off easily except the 2 pieces closest to the incision at the top of her head. It hurt, I could tell, but she did well. Dr. Genecov came back it, took the anchor stitch out of her forehead for the scar tissue that was placed there from the top of her head. Looked her over and said it was doing very well. (we thought so too) He again said the surgery went very well. All the stitches are dissolveable, even the ones in her mouth. They said they would come loose and she would probably just spit them out. (yuck). The incision/stitches at the top of her forehead are kind of gathered a little bit, but he said as time goes that will even out/smooth out. She was concerned, the area that shows the most bruising on the side of her nose has a big lump in it - he said it was fat and normal for it to be like that and it too will smooth out.

Dr. Genecov and Kealy

She was given the go ahead since she was healing nicely and doing good physically to go back to school on Thursday. But, no bending, stooping, running, horse play etc. And still no sleeping or laying on that side of her face. And she was told that we didn't have to clean the incisions anymore with the peroxide/saline solution and did not have to put the Bacitracin on it anymore. I was so glad and so was she, as it makes her hair a greasy mess. It is taking forever to get it all washed out. Kealy is to go back to Dallas on January 10th for another post op visit. He said it will be determined then if further surgery is needed and when possibly. As about half of the fat injected is reabsorbed by the body, so it is hard to tell until the process is done. But for now she looks great and we are hoping she comes out on the end were just maybe she won't need anything further. We will see, in the back of my mind it is saying possibly one more. As in our initial consultation he said it would be 3 surgeries, so we are wondering if this will still be the case.

After Kealy gave the doctor his goodies, she asked if he liked chocolate - he said yes but had to confess that he had a problem with dark/milk chocolates. :( But then opened the box up and saw there was white chocolate on a lot of the things she picked out and was happy about that because he could eat that. :)

After leaving the doctors office, we stopped to eat at Taco Bell. Then on to see Destany once again. She was to have a spinal tab on the 8th and we haven't heard. She is hoping sometime soon that she will have a white count high enough (and also to be in remission) that she could go home for a least a weekend. So a shout out to DESTANY ~ we are praying for you girl!

Once we left there we headed to our hotel. The Courtyard Inn, a little girl (ha ha) that I use to babysit years ago, now all grown up works for a hotel chain contacted me before going this time and got us a discounted rate. Pretty awesome, Thank you Traci. It was a really nice hotel and had easy access to the area of things we needed, food, Wal-Mart, doctors office and highway 75. We got checked in, decided to find a Wal-Mart to do some walking around, spotted a gas station on the way back to the hotel so we filled up there. And stopped at a Waffle House for dinner, Kealy had never ate there and said she liked it. We stopped a CVS that was right next to the hotel and got a few snacks. She showered, we watched some TV, of course she found the hotels computers and did some facebooking, testing and bed.

In the morning (we stay overnight because 10 hours on the road for me driving/riding is just too much), Kealy goes to see what is for breakfast at the hotel. Somehow or another she managed to get the lady that was in charge of the breakfast (as it wasn't a free buffet) to give us a discount. Still don't have the story on that one. But Kealy got hers for 1/2 off and mine was almost a half off so we ate at the hotel. Good food. We eat, pack and head off once again for the home front. We thought about stopping to do some looking around again at the outlet, but we were both ready to just get home. We stop at Arby's in Okmulgee for lunch and then home.

12/11/2011 Kealy in her Christmas outfit

So now we are waiting to see what Dr. Genecov has to say on the January visit. I still feel we are on unknown ground, as some doctors will never suggest that she have surgery fearing it will make things worse and others disagree. You can tell what group we choose to go with. Just keep her in your prayers for continued healing and that she makes good decisions as this goes on in the care of her face regarding the things she should and should not do. And lastly Thank you to another donor to the cause, Frank Clark - - we appreciate it so much.

Some of you might ask, that are not familiar with it - what is Linear Scleroderma en coup de sabre (in Mom's words):

It is an autoimmune disease, where the body attacks itself and in the case of Linear Scleroderma (LS) it causes hardening of the skin, (causing scar tissue to build up), can also affect the muscles and bones. For those affected in the leg, it can cause it to be shorter and smaller (and I might add can be painful), same for the arm and can affect anywhere on the body in the same manner. Kealy's is a rarer form; en coup de sabre which mean cut of the sword, because it often looks like a cut down the face/forehead. Sometimes it does not show on the forehead but in other parts of the head, Kealy has 2 places in her hair with the indention's which also cause alopecia (or hair loss) because it kills the follicles because of the hardening. In her type it can affect the skin, skull and into the brain. This is why she has to have MRI's and why she was having headaches all the time before we knew what was going on. Because it can affect the brain, seizures are sometimes associated with it. Fatigue seems to be associated with it also, this is my observation from all the people I have been in contact with and just with my own observation with Kealy. The ECDS can also affect the eye/eye area, Kealy sees an opthomologist on a regular basis. When first getting diagnosed the protocol of doctors is a bit length, dermatologist, rheumatologist, neurologist, MRI, CT Scan, opthomologist, and finally a Specialist if you can find one. We also had Kealy's teeth/mouth checked by a dentist. [Don't be surprised most doctors don't even know what LS ECDS is.]

Doctor's do NOT know what causes this disease, nor is there a for sure treatment for it. Methotrexate (a low dose chemotherapy treatment), along with Prednisone (as steroid treatment) seems to be the standard treatment for this. Kealy was on the MTX for over 2 years, and was on Prednisone for months. She gained around 50 pounds because of the Prednisone and also had a hard time with her moods, it exasperated everything. There is some new treatments out there that not all doctors offer, which is an antibiotic treatment. Kealy has been on this for about a year now (Minocycline is what she is taking, I know of others that take a different antibiotic).

The disease is said to burn it self out, but then again that is not true as it can flair up at various times in peoples lives. So I believe it just goes into a remission state. In women sometimes upon getting pregnant it is said to possibly reactivate.

Parry Romberg Syndrome (PRS) is sometimes associated with it, which is very similar and is said to cause wasting away of the underlying tissue (instead of hardening). Affects more of the face, but usually only one side of the face also. And can have underlying conditions associated with it also, seizures, fatigue, strokes, eye & mouth problems - just a number of things.

The day after, going home.

2011-12-03T18:58:00.000-06:00

December 01, 2011

We go down for breakfast; Kealy is a bit upset she was looking forward to having biscuits and gravy. Which they did have the day before at the hotel. Well on this day – no biscuits and gravy. L She doesn’t seem to care if people see her all taped up and swollen and a little bruised. After that we pack up, check out and then stop at TJ Maxx (she had a little money and wants to spend it and just get out and move about). Jerry Don sits in the car while she shops a bit, getting herself another pair of loose fitting pajama bottoms, underwear and bought her dad a 82% cocoa bar for waiting patiently for her to get some moving & grooving out of her system for the long car ride home. By the way she is walking around in her PJ’s not caring what people think. She’s a character.

Heading home she sleeps most the time, we stop at Braums in Henryetta to have lunch – then on to the house. It was good to get home.

Kealy has done really well with pain, only having prescription pain killers only a few times. Most the time Advil works for her. Dr. Genecov said that her face will look about like it should once healed up right after surgery – then it will continue to bruise and swell for a few days. He said her eye may swell shut (but has not yet). But today she does have more bruising showing up and swelling seems to be up a bit. It is 3rd day post op as I write this blog.

She took a shower Friday which was a little tough for her. She can’t let the water beat on her head or face. But, she can wash her hair and face gently. We moved the shower chair into the shower as she was getting light headed. I have to clean the head incision 3 times a day with half saline/half hydrogen peroxide and then put Bacitricin on it.

We are getting ready to go to my mom’s for Thanksgiving; Kealy comes in with a stocking hat on wanting to know if she can wear it…..I was like GET THAT THING OFF! Man, my stomach jumped – I had to explain to her again why she can’t do stuff like that. Also wondering if she will be able to go back to school after the follow up or if she will have to continue to work from home – I totally understand why she needs to be home. One wrong move or accident can really mess things up. I feel like a vulture watching over her sometimes. She looks wonderful though, it is so nice just not to see the alopecia area and to know the damage from the LS won’t be staring her in the face every day!

One Surgery Down!

2011-12-03T18:16:00.000-06:00

Day of Surgery 11-30-2011

Kealy before checking in at
the butterfly tree.

Boo with her dad.

Boo and mom.

First room, waiting Valuim has kicked in.

The Bee Hive @ Medical City Childrens Hospital - Dallas, TX

Resting before surgery.

We can’t thank everyone enough that has helped us with this trip and future trips, besides those mentioned earlier: Ann Williams, The Delarosa’s, Bobby & Lori Johnson, Carol Ward, Jackie Ringler, Rocky & Jeannie Underwood, Jerry Don’s parents, the Skiatook Fraternal Order of Police and the Skiatook Church of Christ. It’s been truly amazing and overwhelming. And just all the PRAYERS – I know they have been heard and answered on Kealy’s behalf. And thanks to my Mom and Dad for watching Kealy’s puppy Molly Mae while we were gone! [If I missed someone - I will remember and mention later!]

Her companions, Chocolate Ruby & Macy. In Jail.

We get to the hospital at a little before 7:30am. Check in at the Bee Hive and wait. We go back to the room to ‘wait’, they give Kealy a Valium to help relax and also a pill to help with nausea. It helped, as she didn’t complain about being hungry or having to wait so much, because most the time she was napping. She was then wheeled to the surgery floor.

The nurse came in to get the IV started – fail, she said she would have to have the anesthesiologist do it, seems it was hard to find a vein and when she did it kept moving around on her. Later Dr. Sykes comes in the anesthesiologist (which I liked very much, as he called us the night before to go over everything and see if we had any questions or recommendations concerning Kealy) – he tried for a while too in her hand then up toward her wrist and finally had to put in the fold of her arm. This took all in all an hour, so instead of going in around 9, she was delayed until almost 10am. Glad she was pretty much out of it. The nurse had brought a small DVD player and movies to distract her, she picked Nacho Libra out to watch – but I don’t think she really watched one minute of it while it played.

While in the waiting room, they have a board for parents to watch, it shows a little scalpel when they start surgery, then little stitches when they are closing, and finally a little band aide when she is sent to recovery. Her code was BUL….D,K. Her surgery took 1 hour 30 minutes.

Dr. Genecov came out to talk with us, said everything went great and that she looked good and did well. He took fat from her stomach (going through her belly button so there are no visible scars) to use on her face and then in the coup de sabre line that was the deepest he actually used the scar tissue that was in her area of alopecia (which he had not planned on, but once he got in there said it was a good source and worked very well – keeping from having to use cadaver parts) So we were pleased that she only has all of her own tissue being used. I was a little concerned that it was affected by the Linear Scleroderma, that it would cause future problems – but he assured me it would not. That at this time it is basically just scar tissue. I didn’t get a count on the stitches she has. There are at least 15 on her head, some on her forehead, some on her check and some inside her mouth. Fat was put around her forehead, down the bridge of her nose, under her eye and in her check area along with the area that no longer had alopecia.

We finally get the call to go back and see her. She is just waking up and looks really good, much better than what I expected. I take a few pictures of her and then the nurse says we can go ahead and wake her up if we want so I gently tap her and she starts coming too. Crying and wanting to see her doctor, insisting that she sees her doctor – that she needs to thank him. After this going on for a while, the nurse finally tells her she can’t see him he is with another client. This was about to make me cry, which I was about to anyway, it was a pretty emotional thing, just to see her and then her to be so concerned with wanting to thank him. It told me how much this all really meant to her!

She had her Chocolate Ruby (dinosaur) and her new Macy (gorilla) with her throughout the ordeal to keep her company along with her polka dot blanky. J Kealy was given some pain meds as she said her head was hurting. She finally got the okay to head back up to the recovery room. Were it wasn’t long she was longing to eat. She first got some lemon lime soda to drink, then some ice cream, then some cinnamon teddy grahams and finally she ate some crackers I think it was. I was afraid she was going to fast with the food and it would all be coming back to visit her, so the nurse pull out a pale for her (just in case). She never did get sick. She got the go ahead to leave, but needed to use the restroom first, there she noticed her belly button oozing a bit, the nurse checked it and said all was fine. Kealy got dressed and we went over the discharge paperwork and instructions. Then not long we were headed home – she was pushed out the door in a wheel chair and loaded into the car.

On our way back to the hotel, we first had to stop and pick up some more meds. We made another stop at Dougherty’s Pharmacy to get Advil and Bacitracin. Then she was complaining she was still hungry. So we stopped at Jack in the Box (not recommended). She got herself 2 large curly fries and a pop. Isn’t this the preferred after surgery food! She ate almost all of those. Never got sick. Wow. We get back to the hotel, she rests a bit. The one thing is she can’t lie on the side of her face, nor is she suppose to really touch it much (only a very light rubbing) this until we go back to see him, then we will see how much longer she has to do this. We order in dinner that night about 6:00 pm from Humperdink’s which is right across from the hotel. Kealy eats’ a steak and mashed potatoes. And pretty much eats every bit of it. She amazes me. Jerry Don goes for a wrap and I have a salad. Then she pretty much just talks on the phone to a few people, texts some, at one point she got her dad to walk her around and located the hotels computers so she did some face booking, she even worked on some of her literature homework (I read one of the stories to her that was a little long as her eyes had been draining and had some sort of substance in them from the surgery) and she sleeps.

First night, I don’t get much sleep – constantly checking that she sleeps slightly propped up and that she doesn’t turn and lay on her face or that she messes with her face in her sleep. She did really well. It’s been the same since coming home, but I do get more sleep now and usually only check on her 2 to 3 times in the night and she said she usually will wake herself us when she feels herself touch her face.

Day before Surgery

2011-12-03T17:16:00.000-06:00

11-29-2011

We head out at 4:30am to get to the doctor’s appointment that morning on time. Stopping in Skiatook to fill up (gas was $2.89/gal), Kealy ran across the street to get her some food at Daylight Donuts - then off we went. Down highway 20, to 75, then 69/75 all the way - it really is an easy trip.....just long. 5 hours at the most unless something causes traffic back up. We stop in Dennison and get some breakfast at 8:26am (Braums), then on to Dallas. When getting to Dallas after talking to Kealy about what she packed to wear, we decided she needed to get some loose fitting button up shirt PJ's. So a quick trip to Target was made for those. {Glad we did, she wore those for 2 days}

Doctor’s pre-op appointment goes well, goes over the surgery and after care. Set up the post op appointment, which is Dec. 6th. While at the appointment, Kealy asks if she can go paintballing in December – the surgeon got up in her face and was like NO! It was pretty funny really. She asked a question at the last visit something about are you going to cut on my head, and he said something like – I am a surgeon this is what I do, I don’t put lotions and potions on you. (lol) He does really well with her.

Onto the Children’s Hospital, at Medical City; up to the Bee Hive they call it. There they did the pre-op blood work, which went smoothly, and she got to get a Justin Beiber sticker lol, and a baby gorilla she named Macy. It's a really cool place, you walk in and see a ton of butterflies hanging from the high ceilings and a huge tree, and then you have to walk up a flight of stairs to the Bee Hive, which is the outpatient clinic for kids. Once done at the Bee Hive, we had to go to building B, this hospital is quite complicated (people always trying to find out which way to go). In building B was the Craniofacial Center, there they took photos of Kealy's head in every direction for the doctor to review and use for surgery. It was after 1 when we finished with all the appointments.

Time for lunch, we had Taco Bell which was located between the Doctors office and the hospital. After lunch we took her to the other Children’s Hospital, which was located by her previous specialist (Dr. Jacobe) - she got to visit her friend Destany who is suffering with Leukemia. She took her a wig, made them matching t-shirts and also a card that she had people at school sign for her. Jerry Don & I waited in some chairs out in the hall while they visited for a good hour. Keep her in your prayers. I don't believe in coincidences, so praying that Kealy gets the forthwith to share Christ with her. Who knows if this is not why she was placed there in Dallas at the same time for such a time as this. There was a very cool mini train village set up at this hospital; we enjoyed walking around and viewing.

After leaving the hospital Destany was in we had to get gas, so instead of getting on the expressway we traveled on the street roads trying to find something. Not like around here, where it seems there is a gas station on every block. Finally at a light I had to stop beside a police car, I waved at them until they rolled down the window and asked them where I could find a gas station. [Then I said I’m from Oklahoma and don’t know anything] – which I meant to say I don’t know this area – Jerry Don said you could have done without that last comment LOL But it helped straight ahead a ways was a place to stop to get gas. Jerry Don had to wrestle with the new fangled pump they had – but we managed and were on the road again. Just after 5pm now.

Next was stopping to pick up the medications she would need after surgery. Pain killers and antibiotics. We were now all set for surgery and Kealy was ready to get it over with.

We get to the Hampton Hotel {BIG THANK YOU TO THE WEAST FAMILY}, check in, settle in a bit and then call my cousin Lecia who said she wanted to take us out for dinner. She lives somewhere in Wylie, TX. Kealy’s choice for the night, she picks Golden Corral. It was a good time and nice visit {THANK YOU SO MUCH LECIA}. I am telling you our life has been filled with blessings lately.

Back to the hotel we settle in for the night. As we need to be at the hospital at 7:30am.

Got the Go Ahead / Before shots

2011-11-26T19:33:00.000-06:00

Today Kealy gave me the go ahead to share the before photos and shots taken through out her journey with surgery. This is a huge thing for her, and I understand that. But I know how just seeing photos of other individuals has helped us to understand and direct us in the ways we go with her treatment etc. [These are the photos sent to Dr. Genecov for review before we went for consultation.]

Front view eyes closed

Front view eyes open

Head leaned back, I think this view shows the damage the disease does the most.

Profile left side

Profile right side

Main indention that leads down into the forehead, this is the one that will be corrected during surgery.

Second indention and area of alopecia, just to the right of the main indention. Possibly addressed at a later time, but since the main one is so large trying to address both is not possible.

Just imagine how hard it is going through the teenage years, now add the thought of something on your body, especially a girl and it being your face that starts to disfigure change, loose hair. It's pretty devastating. Yes I know it's not cancer or totally life threatening, but in some cases yes it actually is. Kealy is tired a lot, we have come to realize it is not depression but part of this disease. Not every one agrees but we know our daughter. And thank God, she has yet to experience seizures (and pray it never happens). She has been very tolerant of the changes her body has suffered because of this - gaining 50 pounds and mood swings due to prednisone. 7th and 8th grades were very hard for her due to this. It has been hard on her and us - but looking back I am very proud of her for the most part she has handled everything very well. And she is very brave and open just to allow me to share photos of her journey.

Can I say it again - I love this girl! Thanks for taking the journey with us. Keep her in your prayers.

Overwhelmed!

2011-11-26T10:02:00.000-06:00

WOW! How can we say thank you, I don't know how many times I have been in tears accepting or receiving donations for Kealy's trips to the Big D. Man the first one is coming fast!

We have 3 major trips, each 3 days in Dallas, then 3 follow up trips which will be one overnight stay each that I didn't even think of before because I was focused on the surgery trips. But, we are so blessed to have such loving people around us; from the Fraternal Order of Police that just came up and handed my husband a check, to our church family and immediate family doing the same, to wonderful friends that have also just came up and handed us money, mailed us checks with letters and cards of encouragement for Kealy - people that bought the items I was selling on facebook (some included a healthy overpayment - thank you Carol!). And the Weast's booking us two nights at the hotel with their Reward Points - how cool is that! It's just very emotional for me to see peoples generosity. With that said we have been handed $1,690 and 2 free nights at the hotel in less than 2 weeks. THANK YOU!

Her surgery is covered under her insurances, huge blessing there also.

I also got to get rid of my 'boot' from foot surgery as I can tolerate it he said, starting with 1/2 a day - so by the time we leave I should not have to drag that thing around with me. Yeah!

After emailing Dr. Genecov with a few additional questions, I find out that I misunderstood him when he said he was doing stem cell injections also - he said he would like to do this but it is not allowed at this time at the hospitals he works through. But in the future hopes it becomes an optional treatment.

He also stated (because I forgot to ask on the visit) that regarding the area on her stomach: As far as recovery, her abdomen will be sore for about 3-5 days... She will wear a garment to help decrease the swelling to he abdomen as well.

He will do the fat transfer to the forehead, check and face and remove the area of alopecia on the scalp with direct closure (leaving the second area alone at this time). The days are going by quickly, 2 days until we head that way. So far she is doing great, but I know the nerves are there and the fear of the unknown. I appreciate the teachers and friends at school that have helped her through those moments when she had been thinking about the surgery and become overwhelmed. Thank you.

Since seeing Dr. Genecov, she has seen her primary doctor (Dr. Yvan Thomas) for a check up and got the okay, she also saw Dr. Adelson who is so supportive and available if we need anything when she comes home. He still feels like the Minocycline is doing a good job, and it does appear to remain in remission. He also thought we made a good decision going with Dr. Genecov. It's such a hard disease to judge, because there is not a true and accurate way to measure it's progression, regression, etc.

My prayers have been going out to Kelsey who had surgery right before Thanksgiving with Dr. Sebiert in Wisconsin and remains in the hospital recovering. Kelsey and Kealy were affected in much the same way, I would have to say she is the closest one to the way Kealy looks with out the hair loss/scalp indention's Kealy has experienced. Her surgery was 8 hours (I believe) and a weeks stay in the hospital and a longer followup stay in the area. Just knowing Kealy I am glad we are taking the path to Dr. Genecov, I think she will do much better this route and he states the outcome is the same. We will see in the end.

My heart and support also go out to Lauren, who's son suffers. He is just a little guy. Praying she finds the answers she needs for her son and the direction they need to go for his treatment. We totally understand not wanting a child on Methotrexate :( I love that we have the Internet and can find each other and offer support and information to help us each along. Marilyn and the Romberg Group have been so helpful and supportive too. Good information gets disseminated through that group, I am glad I am part of it.

A little distraction, is that Kealy has a friend in Dallas at the Children's hospital who is fighting Leukemia. Kealy has made a card and had all the friends at school sign it, and also is making them matching shirts (with a little help from Lacie) and if we can find one today we will be taking her a wig (that is something she requested if Kealy could get it for her). We will see. But Kealy is excited to be able to visit her when she goes down for surgery.

Please continue to keep Kealy in your prayers (along with her friends, Kelsey and Destany as they recover).

Hope everyone had a Happy Thanksgiving, we got our tree and decorations put up for Christmas yesterday. Looking forward to more good family times.

Through everything I hope Kealy remembers:

Charm is deceptive, and beauty is fleeting;

but a woman who fears the Lord is to be praised.

~Proverbs 31:30

Surgery Consultation

2011-11-15T21:45:00.000-06:00

It's been a busy last week. Just getting doctor referral's, insurance okays - all takes time. And I so appreciate Kealy's insurance case manager at Community Care. She is so very helpful. So with everything getting approved for the doctor (since he is out of network) we prepared for our trip.

I think I do most the preparing. Getting the questions I emailed him printed out, since he wanted to answer them in person. The paperwork we had to take with us. Notifying the school of the visit and future possibilities. And seeing Kealy was mentally ready. Which she is.

We traveled down yesterday, Monday Nov. 14, 2011. A good day - my Grandma Lena's birthday (RIP) and also Matthew's (my son-in-law) birthday. Five hours is a long drive. We stop and eat at Chili's....yummy, in Allen. Then head onto the doctor's appointment. We arrive 1 hour early, and decide to go ahead and go to the doctors office. While we were downstairs getting directions to his office, I get a call on my phone which is from the doctors office. Good thing we were there early, as they just got the call from the insurance approving a CT scan and wanted it done right then. We go up to get the paperwork to take to the CT office, Kealy is a little upset because she just got her lip pierced (she paid for it herself) and we thought she would have to take it out. So after a big ta-do, the lady at the CT office said she didn't need to take it out. That was another blessing. 2 in a row now you see them. For me anyway little things mean a lot.

CT went well. Took the disc with the images back to the doctors office and waited. Finally getting to see Dr. Genecov after a bit of a wait. Lot's of people in and out.

He questioned about her history with LS. He explained how doctors look at LS and PRS. He said that he didn't think she was in the spectrum of PRS at this time, though it could progress to that. He was very observant of her face noticing that one side is slightly different, nose smaller, hallowing under left eye slightly etc. These aren't really noticeable as the indention down her forehead nor the alopecia. Asked if she had experienced seizures, we said no only the 2 times that she passed out, and didn't really know if those were considered seizures. As that can become a part of the LS as he said it can affect the bone structure and also the brain as it progresses due to scar tissue. He noted that she didn't really have skin changes that are usually noted with PRS patients.

Onto discussing what he plans to do after examining her. Main procedure (scheduled for November 30, 2011) will cut out the affected area on her head where the alopecia is (just in one place) and pull the hair together and stitch it up, then will do fat transfer and stem cell (her own) in to the area because it is so indented. The 2nd area of alopecia he will not address at this time because the main one is so large. Onto the forehead, he will not cut anything out of this area but do the fat transfer/stem cell transfer into the area. He said she still had good soft tissue coverage so it should go well. The total procedures should take 2 hours.

Kealy was worried she would have to be conscious during the procedure - he assured her she would be out!

He noted that stem cell therapy might be helpful in the future to help stop any reactivation of the disease, but at this time not enough is known. But it hold promise.

She will have 2 more procedures, at 6 week intervals.

Dr. Genecov said it was okay for her to continue with the Minocyline after the procedure. It would not hurt and could be helpful.

We will have to stay 2 nights in Dallas. This because she has to do pre-op on Nov. 29th, the day before surgery. And then she has to stay 1 night after surgery and be checked the next day before coming home. Then back in 10 days for the post op check up.

I was worried about insurance coverage, and he stated that it is NOT cosmetic, but RECONSTRUCTIVE surgery. SO TRUE. LS [Linear scleroderma (en coup de sabre) and Parry Romberg Syndrome) reek havoc on people and should not be looked at as cosmetic!!

He mentioned to her that it could reactivate when she has children, which I have mentioned to her before. But there is no for sures.

He checked her teeth and her bite.

Stated after surgery there would be swelling and her left eye would probably be swollen shut. But the eye should only be that way for 2 to 3 days. Also that the color change that she does have down the forehead in the LS line will probably stay that way, but could possibly address it in the future with another type of treatment.

I asked about if he would fill the area beside her eye and down her nose, as on some of the corrections I have seen they have not addressed this area. He stated he would. And that is why there are 2 - 3 follow up visits.

Kealy should miss 2 to 3 weeks of school. I fret about this, but also realize that this could really make a difference for her and how she feels about and views herself (even though I think she is beautiful just the way she is).

~~~~ so on to preparing for the trip down for the surgery~~~

I have been out of work since March, (currently waiting to hear back on an interview I went to last week) so money around here is a little tight. But things are coming together. GOD is SO Good! I finished a portrait this week, so there is a little money for the fund, posted some items on Facebook and sold a few items...so there's a little more money - - - then had some wonderful friends volunteer to use their Reward Points to book our hotel room for 2 nights and other friends stepping up in other ways. I JUST CAN'T THANK EVERYONE ENOUGH. I love you all. :)

We are looking at this same thing in 6 weeks, then in another 6 weeks. [3 trips total].

BUT IT IS ALL SO WORTH IT. K E A L Y O N A is so Worth it! I love her. :)
Hopefully soon she will let me post pictures, possibly a before and after. Will keep you posted.

~ Thanks to everyone that is helping her out on her journey. We appreciate it so much.

Rely on the LORD!

Be strong and confident!

Rely on the LORD!

~ Psalm 27:14

Next Step

2011-11-09T08:58:00.000-06:00

Next Step

We are looking at what the next step is for Kealy. She said last month that she was really ready now to look into surgery to remove and correct what this disease has done to her face and head.

Through a Facebook link that was posted on the PRS group site, I contacted Dr. Genecov's office:

http://www.craniofacial.net/syndromes-parry-romberg

Kealy has an appointment Monday 11/14 so we will see what he has to say. We had also been looking at Dr. Sebiert in NY, but since Dr. Genecov is within a reasonable driving distance, accepts one of Kealy's insurances and the other insurance has okayed the visit (& stated they have covered other surgeries by the doctor in the past). And he does good notable work - we feel good about going here. EVEN though Kealy did want to travel & visit NY.

I had to submit photos for review first. I will have to get Kealy's permission to post them to the blog. So those will wait until another day.

This blog has also been added as a reference to the Romber Connection site. Check that site out for good information & support! Thanks Marilyn and all those that help to make it a success.

http://therombergsconnection.com/

Happy 16th Birthday Baby Girl!

Tid Bits

2011-10-12T11:24:00.000-05:00

Additions to yesterdays blog......

The EEG results came back normal. The doctors office just said to call if something else happened. Beleive me we will.

Also, on a previous post I had commented on how we were contacting another specialist. Dr. Wigley. Well we did and were told that he doesn't treat/see pediatric patients. They said they would pass my email on to the doctors that do see kids, but apparently they don't do anything other than the MTX treatment either, because I never heard back. Pooohhhyyy

Lastly below is a pic from July 2011 with her cousin Tara, who graciously just donated her hair for the second or third time to Locks of Love. :)

Frustrated with this disease

2011-10-11T11:14:00.000-05:00

Do you ever feel like you are going in circles or waisting time. And it is frustrating when there is no clear right answers.

For 2 years Kealy was on Methotrexate, tolerating it and who knows what else - just hoping it would stop this disease. Did it, NO. Even while on it, it was progressing. Doctors like to think not - but truly all you have to do is go back and look at where she started and where she ended. We ALL hope and pray that things work. It wasn't. It progressed. When I look at it in black and while (figure of speech) - you can see that the indention progressed, the hair loss continued the second indention progressed. But when you hope sometimes things get fogged. And just maybe it did keep it from getting much much worse, who knows. But I do know when she came off of it, she had a growing spurt.

Now on to the Minocycline. Is it helping. I truly don't know. I think what the doctor felt as being better, now I think as not being as deep, but spreading out and getting wider. It definitely is further down on her face.

Sometimes it is just hard to keep up.

Do we want to do the filler, yes and no. I would really like to see the disease cut out and corrected for her so there is no progression in the future. As it will be removed. And if she proceeds with the filler this may cause problems with the removal of the diseased areas. Who knows.

I need to get her to the opthomologist, into the dentist and looking at surgeons. Then the thought of insurance, will it cover any of this. Will they look at it as cosmetic as it is not, cutting out a disease and then having to reconstruct is not cosmetic. I would not look at someone having a breast removed due to cancer and having it reconstructed as cosmetic. 'Would you?' We were just referred to a surgeon through the PRS facebook page today, that is much closer for us and his work looks good. I have submitted my information for them to contact me to see what they would have to say in Kealy's case.

A few weeks ago she fainted/passed out/had a seizure - who knows. All the tests so far have come back normal (waiting on the EEG results, they are delayed for some reason - hopefully they weren't lost!). This concerns me because I know from reading that seizures can be associated with both PRS and LS en coup de sabre. So far no problems since that day, only she said the fear that it might happen again.

She still suffers frustrations at school. Kid's I would never think of have made fun of her. I truly don't think they realize how it hurts her. I don't think she does either as she wants to sluff it off, but I know otherwise. She deals with this on a daily basis, it may just be one kid does the 'Kealy cut' as she said they call it - but just that one time is all it take to remind her she is different. And I know there are some kids that don't notice and if they do they don't care because they have better sense. I thank God for those kids and pray for awakenings for the others.

I just want her well and to feel whole and okay.

Thanks for listening to my frustrations this morning.

She was 12 when all this started..........she is fixing to turn 16! Wow time flies.

Anxious

2011-08-22T12:47:00.000-05:00

It's been a few months. Life has thrown us some curve balls, but we have delt with them and are going on with the game.

Kealy had been loosing more hair, we thought initially that it was the LS becoming active again. But, we all are now thinking it was because of the stress she was under. She has moved on to a better place mentally, and should continue progressing in a positive way. That is our hope and prayers for her.

She saw Dr. Adelson on Friday, 8/19 - he was very pleased with the improvement in the depth of her indentions (the ones up in her hair that are not visible, but were quite prominent upon feeling them). They have smoothed out some - I would have to give the credit to the Minocycline 100mg/day that she is on. She should remain on that for another 2 years he said. I was hopeful and sceptical about this treatment, but it seems to be working - the long term will tell. Thanks to the friends I have made by putting this blog out there that have lead us to question all treatments and explore new ones and thankful for a doctor that listens to his patients and thier parents and is willing to also explore new avenues not familar with him. [namely, Stephen B., Terri G., Kelsie R. and Dr. Adelson]

The doctor felt it is safe to go ahead with the filler that was discussed in my last blog. So he was going to contact the doctor that said she would do it (free) and that we should hear something from her in 2 weeks or give him a call back. We are excited and anxious about this. Really not knowing what to expect and a little concerned since it is such a large area to cover with a filler. Questions: what if she gets hit in the head, what if it moves around, does it interact with the LS, can it irritate the LS into responding negatively, reactions, what if she becomes uncomfortable with it, is it removeable, does it just absorb into her system over time, can that cause her any harm, how long will it last..... Depending on how this goes and how long it lasts will depend on if we look at an actual surgery correction in the future.

So far she has not suffered any seizures, her headaches have long passed except an occassional one and no other parts of her face or body have yet to be affected. It has been at least a year since an MRI was done on her brain/head, but I am pretty sure no lesions have developed.

I received this in an email, and just think it is worth sharing once again:
To get something you never had, you have to do something you never did.' When God takes something from your grasp, He's not punishing you, but merely opening your hands to receive something better. Concentrate on this sentence... 'The will of God will never take you where the Grace of God will not protect you.'

What to say

2011-03-24T15:52:00.000-05:00

To let those know that have been waiting to hear what the doctors said on March 8th, 2011.....

Kealy and I made it to the 'doctor's review', but just barely - let's just say that she is having a difficult time and a few of the items in my car went sailing out the window. But once there she was fine, and charmed the doctor's.

There were 12 to 15 doctors that reviewed her LS. They would file in the room at random times and look her over, talk to us a bit and move on. After that they all met together and discussed her. We heard back at the end of the week.

One doctor, whom I will mention at a later date told Dr. Adelson she would do the filler for free. [How awesome is that!!] BUT, before we get to that stage she is to go in with Dr. Adelson and have saline injected into the indention area to see how it will look and work...and let her know ahead of time what to expect in regards to how it will proceed and look. He said the saline would only last up to an hour and would disipate with no problems. She was to do the saline treatment this week....BUT, she is occupied at a location elsewhere and we are not sure when she will be available to proceed. Dr. Adelson did say just to let him know when we are ready to go.

She continues on with the minocyline 100mg once a day.

Continue to keep Kealy in your prayers, she has been dealt a lot of things to deal with in her short life time.
Thanks.

Hooray! For now....

2011-03-01T11:36:00.000-06:00

Let's see ~ Kealy had a doctors appointment last evening. She can go off her Methotrexate! Yeah big celebration (for me anyway)! :) We celebrated by going out to eat at Red Lobster....yummy! I am hoping this will help her, as she seems to be tired a lot. The kid sleeps all the time, so hoping that her energy improves.

Kealy's dad took her to the circus Sunday, she loves the circus!

Dr. Adelson is going to put her on (I believe it is) Minocycline, I haven't picked up the actual perscription yet to see or get the amount of dosage or how often. I am kind of excited to try this as I know it is not a standard treatment for LS, but I have read a few cases that have taken it for the systemic form of scleroderma and it was stated that it does make a difference in their skin. So we are praying for the best. Of course Kealy isn't - she doesn't want to take another pill ~ and who can blame the kid!

Just heard from a dad, whose daughter is younger than Kealy - she also is starting on antibiotic treatment this week, the drug prescribed for her is doxicycline.

This is the link to a website that discusses antibiotic treatment: http://www.roadback.org/

Dr. Adelson will also be meeting with several doctors, it sounded like this goes one once or twice a month and will be discussing Kealy's case with them and getting suggestions for a doctor who could do the best job on filling her indention in. As she wants to take this route instead of surgery at this time.

This is a link to a website that discusses the different type of fillers used and what to expect: http://www.plasticsurgery.com/facial-fillers/info.aspx

Sorry to say he did not get to make the conferences, or discuss optional treatments with doctors from out of state due to Oklahoma's blizzard we had at the time. By the time they said the plane's could go, the conference was over. :(

I was privileged to talk with Kelsie in CA, she is just starting treatment for her LS. I am sorry I couldn't give good reviews on prednisone, but there is truly no way to go back in Kealy's case and tell if it did any good or not (things can't be undone). But if we had it to do over with in her case we would not have done that medication protocol. Kelsie mentioned that she knows a lady with systemic scleroderma that is taking the antibiotic treatment and is doing well on it.

We just ask for continued prayers that she does okay not being on MTX, that the LS does not spread. Pray it never reaches into her brain and causes lesions or seizures, etc. Pray the antibiotic treatment improves it. And just pray for Kealy in general. Some of the kid's at school have been calling her crack head and she wants to make light of it, that it doesn't bother her - but words hurt.

I love my Boo!

Methotrexate

2011-02-07T16:57:00.000-06:00

Since Kealy has been on MTX for over 2 years now, we feel it is time she come off of the medication.

METHOTREXATE

Knowing that her LS may not stay in remission, we are looking at alternative treatments and/or surgery again. We have discussed this with Kealy so she is keenly aware of what may take place. Of course as a parent, we don't know all the answer's, we just try to decide what we think is best for our daughter. Is it the right thing - is it the wrong thing to do...we don't know. We just pray and go on each day with the decisions we make, trusting that they are what's best for that time in life.
We will probably not be going back to Dallas to see the specialist there, but will continue to see Dr. Adelson in Tulsa as her physician for her LS treatment. He is possibly going to be investigating alternative treatments to see what he thinks about trying them with Kealy. One of those being an antibiotic treatment. She goes back at the end of this month so we will see.

We had her tested for Lymes Disease, but I have found out there are several different types of test for this and the lab ran the wrong ones ~ not the ones that the doctor requested. So this may be repeated in the future. Mostly doing this out of curiosity, as some think there may be a link - just to many unknown factors with this disease.

I want to give a shout out to Kelsie in California who found our blog and we now are facebook friends....we are so looking forward to hopefully meeting up with her in April!

Pray we are taking the right direction in regards to what we do for Kealy.

Thanks and hugs to you all :)

August visit to Dallas

2010-08-30T15:31:00.000-05:00

We made another trek to Dallas to see Dr. Jacobe. We left early (5am) Friday morning, appointment was at 11am. Filled out additional paperwork for the study she is in. Saw the regular crew, along with a doctor from Florida and Lawrence (a doctor from Austria). Kealy really enjoyed visiting with him. Favorite American foods are: BBQ, pizza, ranch dressing and rice cripsy treats! :)

He stooped down for the picture with Kealy, as he is 6'8".

As far as the LS is concerned, it appears to be stable once again ~ therefore she is going to drop 1 pill (2.5mg) every 2 months for a while and see how it goes [was currently taking 20mg]. I beleive that we will probably go ahead and work her off the medications. And from there, not sure. There doesn't seem to be any other treatment, so possibly looking at surgery as next step if Kealy is ever up to it.

She gave 3 vials of blood for her regular check ups and then 7 more for the study she is in on morphea. That was alot! And she has come a long way, compared to the days we had to have 2 or 3 people on top of her and a couple on the sides!

After the appointment, lunch and blood sucking....we headed to the Dallas Zoo. Fort Worth Zoo was recommended, but do to time constraints we settled for the Dallas one. Didn't get to see the gorilla's, they were hiding down in the moat due to the heat. :( But over all it was nice. The landscaping was great. Tulsa seems to have a lot more animals though.

After that we swam in the hotel pool (it was really cold water) for a bit. Ordered in pizza, watched a movie and hit the sack. Kealy went to sleep, I on the other hand stayed up till midnight watching something like His and Hers Survival (not sure of the title) about a husband & wife that go on these survival trips and teach you what to do. A 9 volt battery & steel wool start an easy fire!

Saturday checked out about 11 and headed home (stopping off at the Allen Town Outlet Mall).

She takes her Methotrexate now on Saturdays, which we should have done a long time ago - but she was insistant it was Mondays (and we try to pick our battles [which doesn't seem to matter anymore]). She slept pretty much the whole way home from Dallas, over a 5 hour trip.

Keep her and us in your prayers ~ life lately has really been a struggle. :(

Day to catch up my blogs...

2010-07-10T11:54:00.006-05:00

Her new hair cut as of today 7/10/2010

You can see how it affects her head in these pictures when her hair isn't covering it up. We no longer take montly pictures and haven't for a long time as she doesn't want to do it and I am not going to press the issue.

It's been a little crazy with all of Kealy's medical/emotional issues lately.

Lets say that she has had a couple of stays in an inpatient care unit to help her sort out/deal with issues. That has not been easy. The last place she stayed was for 3 weeks. But God provided us with a good therapist, who not only helped her but us as her parents. I highly recommend the Love & Logic book series by Foster Cline & Jim Fay. There were some other books he recommended, but not for the general public, more for parents that deal with children that have anger issues that stem from abuse (as in Kealy's case, she has anger she doesn't understand and it all goes back to when she was 4 weeks old and younger - when she was severely mistreated by her biological mother). It overwhelms me that something at such a tender age can have such a lasting impact. Of course there is genetics involved too. etc.

We are looking at her just seeing Dr. Adelson in Tulsa for her LS on a regular basis again. Possibly just seeing Dr. Jacobe in Dallas maybe on a yearly basis. She is to see her in August again. It appears that nothing is going to change, she continues on the Methotrexate 20mg once a week along with Folic Acid.

She had to find a new pediatrician. And we were blessed enough to get her back with Dr. Mickle in Owasso. She had her as a doctor when she was tiny. It was good to see her again. She was up to date on all her shots except the Meningitis, she had to check to see if she could get it and it was determined because of the medication & her immune system that she not get it until after she has completed treatment which could be another 2 years. My heart goes out to the families in a neighboring town [Oolagah] that lost children and their children will suffer life long because of meningitis. It is kind of scary now after seeing what that disease can do - that my daughter can't get the shot for now.

Kealy also wanted to find a new therapist and psychiatrist, so we are in process of doing that. We still very much like her previous one and maybe going back to her. But for now Kealy wants to try someone else.

As for surgery - she hasn't brought that up again. And if everything stays as is, I don't think she will ever want to go through with it. That may change with age and/or if it starts progress. We will see. That's up to her at this point.

Lastly the therapist at her inpatient care thought she should be evaluated by a neuro-psychologist, he has done the initial intake, and will start her testing at the end of the month. They want to make sure all her brain is functioning as it should and also see that the LS is not affecting it in anyway.

April 2010 visit to Dallas

2010-04-26T13:52:00.004-05:00

Doesn't she look good!

The doctors visit went well. Kealy is to remain at 20mg Methotrexate for the time being. And it seems to have settled down once again since she went back up on the MTX. It is no longer reddish and has stopped itching. So we get to extend out duration between visits to 4 months.

We talked about surgery again. Dr. Jacobe still beleives that it would not be the best thing to do. Kealy on the other hand I think is getting closer to wanting something done. She has mentioned getting the photos done soon, so maybe tonight. At least I want to hear what the surgeon has to say.

Kealy has a rough time with kids at school sometimes....but for the most part I think most the kids leave her alone when it comes to her LS. We have had some dealings with other issues, but she is making improvements in those areas.

No longer in remission

2010-03-04T09:25:00.009-06:00

Well she had a few months of no changes.

She had her check up with Dr. Jacobe on Friday 2/26. Missed going in January due to my knee replacement which was 12/16 (wasn't cleared to drive that distance yet), then when we were scheduled to see her on 2/12 that's when Dallas had the freak snow storm (12")! But we finally made it to see her.

Upon examination, the linear part in her hair line has turned a little reddish, has started itching again and she also has a new indention inside the old indention.

So instead of going down on the Methotrexate again as we hoped, she had to go back up to the 20mg once again. And continue to go back every 2 months, when we hoped to move it to every 6 months.

Of course with all of this I am holding back tears, and what was so great about it (if that is possible) Kealy comforted me and said it's okay mom life goes on. She really is a trooper!

On our visit she also had to have blood work done. They also did a DNA strand test, not sure what that's for, but will find out next time.

We discussed with her that Kealy is now wanting to have surgery done. Dr. Jacobe did not recommend it, as she stated the patients she has seen that have had surgery before it is in complete remission (does that ever happen?) - that it will start to progress from the surgery area. So in Kealy's case would mean (according to Dr. Jacobe) that it could possibly spread from her nose down the rest of her face or from the crown of her head towards the back of her head.

BUT on a positive note - she did want us to bring her all the information I could on the surgeon and the surgery. So we will do that. Because I reiterated that he (Dr. Sebiert) has had great success even though it is not in remission when he operates. I have seen some awesome photos of his work and the change it has made in peoples lives. Little Hank that our church has been praying for is one of them. I met (via Internet) his mother, she found my blog not long after I started it. Her son had just been diagnoses with PRS (Parry Romberg Syndrome). You can link to his page via the links I have posted. So with all that said, we will take all our information (I am going to try and email it all to her research assistant Stephanie before had so she has time to review it), take into consideration what she has to say. Then we will probably go ahead and schedule an appointment with Dr. Sebiert let him examine Kealy and see what he has to say.

If she proceeds with surgery, we don't believe our insurance will cover it. That is another thing that will have to be checked into. So we maybe looking at fundraisers to take care of our baby. As her Dad said 'what ever it takes'.

I am going to have to set her down and get some updated photos to send with my initial email to Dr. Sebiert.

Raina went with us on this last trip. I was enjoyable other than the obvious. Hotel stay was brief, we all did work out, watch a movie and get to sleep about midnight. Up early for breakfast, doctors appointment at 9:15am. Lunch at the Olive Garden - yummy I love their soup and salad with bread sticks (chicken gnocchi). Then off for some shopping at the Allen Outlet Mall. I found some extra wide Nike's for my wide feet - that was a blessing in itself!

We each got one treat from the Sweet Factory (can't remember exact name) and took one home for Jerry Don. After shopping we headed back, Raina and Kealy both napped in the car. It's a little car lol. Get's good gas mileage though. Stopped in Okmulgee for dinner at Taco Bell (yummy, still one of my favorite places to eat). Then home. It was a good time with my girls! :)

We have really been on a roller coaster with Kealy lately, not because of the LS - but I am sure it plays a part in her emotions. Because of age, mental health diagnoses don't like to be made in children I have learned. So for the last several years she has been diagnosed with ADHD and mood disorder - which is just a general term that something else is going on. Because we have been on a daily struggle of late with her [we are in this with her, not against her], at one point looking into inpatient care, her psychologist called us in Monday without Kealy being present to explain that she believes she has Borderline Personality Disorder, technically I have read a diagnosis can't be made until age 18. After researching this the last several days, it does fit her (but she is not a cutter and that is mentioned a lot) - Good thing is it is treatable and there is evidence that her brain can be retrained to have appropriate emotional responses to things/situations. Now that we can understand we can better help her too.

She is my sunshine!

Just a note

2009-11-05T14:56:00.012-06:00

....for reference mostly. Kealy has a little depression going on, things affect her differently than most people - with her being the only one at home now, Raina getting married, my up coming knee replacement, and just dealing with the LS. So the doctor has changed some of her medicaitons. She is no longer taking Concerta (or Ritalin which she just took this last month during school days to see if that would help). The doctor has her on Vyvanse 40mg 1xd and Zoloft 25mg 1xd along with her Ability which is 7.5mg 1xd. These medications are for her ADHD / Mood Disorder. Please pray they help her in the ways she need....and just keep her in your prayers for everything God knows what she needs.

She remains on the Methotrexate 15mg on Mondays. Along with folic acid 1mg everyday. The MTX is for the LS and the folic acid is because she is taking MTX.

She turned 14 today!!! Wow how time flies. I sure hope these medications start helping her feel more herself and under control. It's been rough for her and us lately....so looking for some improvement. I woke her up singing Happy Birthday - for some reason she didn't want an oncore, then dad stopped at daylight donuts as a treat this morning so she could get breakfast there (she loves that place). I hope her school day went well =] Dinner tomorrow to celebrate and her bubba is coming too (Wade). That makes her happy. Not sure where she will choose to go. Steak somewhere I am sure. We paid for, and I took her to a Miley Cyrus concert along with one friend of her choosing, this was back in October (just a little early BD present) - she loved it.

October 2009 Checkup

2009-10-26T13:29:00.016-05:00

12 hours of driving in one day is tooooo much!

Kealy and I left at 4am Friday to travel to Dallas for her appointment and returned home the same day. I had a few questions:

1. Does she get to go down on the Methatrexate as previously discussed?

2. Can you now see the optomologist every year, instead of every 6 months?

3. She seems to be slumping more, can the medications cause this?

4. Her face has been getting red and hot again - is this something to be concerned with?

....there was something else, but I misplaced my notes and can't think of it. If I remember I will post later.

Dr. Jacobe's replies:

1. Yes - she is now taking (3) 2.5 mg pills in the morning and (3) 2.5 mg pills in the evening. So she is down from 20mg to 15mg one time a week on Mondays.

2. Yes - once a year is fine. =]

3. Have her pediatrician check her spine, the medication does not cause this. [my thoughts just lazy bad posture, but we will have it checked on her yearly checkup].

4. Kealy's morphea is still active, the medication is just keeping it in remission. She needs to watch it closely and pay attention to any new sensations (such as feeling like bugs are crawling on her head - this is a sensation she had when the indention's first started) - if this happens to call and let her know right away.

As stated we left at 4am, arrived at the doctors 9:30am - since we were there early, we spent most of that time filling out papers for the research study she is in....it has been 1 year since this all started and she signed up for the research. Appointment was at 10:30 and we still weren't done with the papers! We are also participating in an online study group for children with different types of scleroderma, it is both for the children and parents.

She had to have blood drawn again - 6 large vials, 2 for the doctor and 4 for the research. I am glad that she did not have to do another plug biopsy!

If you remember from previous, Kealy won't let me take photo's of her for reference at this time, so I was able to snap photos of the doctors photos...round a bout way of getting references for myself.

She doesn't go back again until January 2010.

Another interesting note, her brother met a lady in her 60's at college (she worked there) - he noticed an indention on her forehead that looked like Kealy's. Of course he calls to tell us, and we insist he go back and talk to her the next day. He did! Yeah brother. Well this lady had went to the doctor around Kealy's age - they told her that they thought it was due to puberty and/or possibly hitting her head (she went to a few doctors trying to find out and got no good information) - so it progressed. She showed Wade the extent of it, which was a little worse than Kealy's I think he said. But Wade was able to tell her what it was. It was encouraging that hers did not progress in a worse manner and she told Wade she did not have any seizes. We hope to meet her one day! =]

We got back and ended up going to the pediatrician on Saturday - she had a bladder infection. Pray that the antibiotics take care of it in just one round of medication. For a toe infection it took 2 rounds of antibiotics. She is feeling good so you wouldn't know anything is wrong.

No news is good news

2009-09-28T16:29:00.007-05:00

You know the old saying: 'No news must mean good news", well of late that is the case. Kealy is completely off the prednisone and has taken off some of the weight she gained on it. The LS appears to be in remission, meaning the indention seems to be staying the same in all locations, hair loss has remained the same. There was a possibility once she came off the prednisone it may 'start' back up. That has not been the case. And if it continues to remain the same, on her next visit to Dallas she will hopefully be able to come down off the Methotrexate to possibly 15 or 12.5 mg once a week (she currently is on 20 mg once per week). Then watch to see if it remains with no changes. I always ask about light therapy - and get the same answer 'no it doesn't work on en coup de sabre'...

We have not taken any photos in the last 3 to 4 months - I asked the other day if she was ready to take one again...and the answer was no. So we will give her some more time. It's been nice to step back and feel kinda normal again. Of course life with Kealy is never normal, she keeps us all on our toes. She has complained a little bit about a few kids at school calling her a 'butt head' - I didn't even think of the obvious - I was thinking she was being a pain in the butt to them (which can happen lol) but she said NO mom - they said my indention on my forehead looked like a butt. Aren't we so nice to each other - I think we like to cut each other to the heart sometimes! [referring to people in general]

The school year has gone back and forth good to bad for her - I think mostly due to her adhd and mood disorder.

She had her ophthmalogist appointment today - all was clear and went well. We are hoping now to only see him once a year. We have to clear this with Dr. Jacobe before we know for sure.

Who knows....

2009-07-13T10:18:00.008-05:00

God is our refuge and strength, an ever-present help in trouble.
Psalm 46:1

Who knows what the plans are for Kealy?

For a day she was able to experience the joy of having the visible indention on her forehead gone! It returned that night. Why it disappeared during that one day, only to return - I do not know. How, why. I have to say it was tears of joy throughout that whole day for me - just to think it was gone, to have the hope that it reversed, that no more worries of headaches, possible seizers, thinking it may spread to her brain and cause lesions, no more hair loss, but hope that possible hair regrowth among other things - but the hope that it was healing and would be faded somewhat upon seeing her. To see that it had returned. For her it was heartbreaking - and for me. I cried like a baby at church when Russell began to talk about it in class, sometimes it just becomes a bit overwhelming [I think maybe I have some of the premenopause stuff going on - emotional/hot junk :( ].

I feel I am a weak person - because it's not like she has some life threatening disease! It felt like the day I first found Linear Scleraderma En Coup de Sabre on the internet - I cried on and off for a couple of days then. I am still in that stage right now. Like I need to get past it and keep moving on. It's not about me, but about helping Kealy along the journey called life. But, it's Like a rug has been pulled out from under us.

Daniel Chapter 3: 16 Shadrach, Meshach and Abednego replied to the king, "O Nebuchadnezzar, we do not need to defend ourselves before you in this matter. 17 If we are thrown into the blazing furnace, the God we serve is able to save us from it, and he will rescue us from your hand, O king. 18 But even if he does not, we want you to know, O king, that we will not serve your gods or worship the image of gold you have set up."

So today, I am back to 'take care' stage and will be emailing the doctor for a refill on her Methotrexate and Celebrate the fact that this is her last week of Prednisone!

Kealy's weight has started to come off and that in itself makes her happy too.

She is courageous and so outgoing and loving - [also I might add, sometimes a true pain in the butt]..... she has a heart for others. And someday I know she will be blessed by all of this and be a blessing to others.

I am not saying this because I am in need, for I have learned to be content whatever the circumstances. -- Philippians 4:11

Today's update from Brother & Sister's visit

2009-07-09T14:05:00.003-05:00

Raina and Wade both drove out the camp last night to see Kealy and the change. They are only about 30-45 minutes away from her camp. Raina said:

I told Kealy come here I have to feel your head [if you remember she is not fond of people feeling her head]. Raina said it was a marked difference. Still there, but great improvement!

I am still amazed. I requested Wade take a picture with her head leaned back - this is the best way to judge the scalp area. As you can see from the photo, it is not gone - but better! This is hope, for when all the doctor's tell you that it can't be reversed, that if she wants to be rid of the indentions she will have to go through surgery. Please continue to pray for her - we greatly appreciate it! I look forward to seeing her when she comes home Saturday.

Okay maybe a Miracle in progress....

2009-07-08T09:19:00.004-05:00

Kealy called me this morning, and said 'mom the dent on my forehead is gone'....being the sceptic I am - I asked did anyone else notice. She said yes Russell (our minister did) and she was calling on his phone. Are you sure - she said yes mom, she would see if someone could take a picture and send me. Okay I am holding my breath. I get to work and can't stand it, I call the minister back - and hang up after 2 rings (they are at camp, & I think he may be in the middle of prayer or devotional!) - he calls me right back. Russell tells me they are standing in line at breakfast and he turns around to see Kealy and notices right off 'he can't see her indention' - which is/was pretty prominent. He mentions it to her, she goes and looks - and calls me. WOW

So now I have pic's as you can see. It is still faintly there, but just from the photos taken on Sunday [3 days ago] LOOK AT THE CHANGE!!! In bad times and in good times - God is always good. So whether temporary or long term - I give HIM the Praise.

For it to look this good from the side - which a side shot usually makes it look worse....speachless.

Just look at the shot of her the day she left for camp (on the last blog) - HUGE DIFFERENCE.

Catching up....

2009-07-06T10:03:00.016-05:00

Summer seems to be flying by!

Kealy went to her June doctors appointment and all went well. For the 1st time since this started we all seem to be in unison that there has been no change in the previous 2 months. So this is very good news :)

So hopefully, prayerfully she is in remission.

She will be off of prednisone in 2 more weeks!

YEAH TO THAT IN ITSELF!!

She had blood work done while at UTSW. I have not heard back on that, so assume all is well.

We stayed with her Great Aunt Dolly and Uncle Mike that live in Wylie for a couple of days. It was nice to get to actually visit with them and not feel in such a rush when we go. I am learning my way around Dallas, so I don't feel so uptight when I go now. Kealy was also able to take a friend, Stephanie with her. They seemed to have a good time and it was her first time to go to Texas. [maybe I can post a pic later of them by the Star that greets you upon entering TX]

Looking at the picture above I posted [and the reason I posted it] I see that faint line on her chin again...? It's a mystery.

The reason I colored the other one blue in the background, she seemed to blend into the wall that was behind her.

She has been in a really gripey mood towards me lately, more than others - not sure whats up with that. Being the mom and the one that is around her most etc, I just seem to get the brunt of it all.

Kealy is slowing taking the weight off. Gone to camp for the next 2 weeks, so it will be interesting if that accelerates that process. And of course I worry, I don't want her to get too much sun or get too hot - knowing the medication she is on etc.

Our little friend Hank [his mom found me on the interent when I was searching for information on Kealy's condition] is recovering from facial surgery. Continue to keep him and his family in your prayers. It is a hard road to go. Any surgery on a child is hard and then on Mom because she gets the emotional hurt thrown at her. You can see a link to her blog at the right.

Worth Sharing

2009-06-22T15:18:00.004-05:00

This article and news clip is of one of the families that is in the Romberg Group I am a part of :

http://www.9news.com/news/article.aspx?storyid=117172&catid=188

Another friend I have met over the internet due to Kealy's condition, her son is getting ready to in a couple of weeks have facial surgery by this same doctor. Be praying for little Hank.

Also, when we went back to the doctor on the 17th Kealy still had one toe that was still infected, so she was put on 5 more days of antibiotics.

And we prepare to go back to Dallas, leaving out on Thursday to see Dr. Jacobe. I am at a loss of any new questions. Just seems to be a waiting game - will it progress, will it not. I think it has remained about the same since our last visit 2 months ago - which is a good thing. Maybe not deeper, just a little wider. Which reminds me we forgot to take her photo for this month - need to get that done.

Kealy has started to take some of the weight off she gained. It will take a while, but I think she will get there. She has had a busy summer and the future is still looking busy, 2 camps back to back...she just might get a little home sick. :)

And a side note, not in regards to Kealy - but her sister Raina, may have early signs of glaucoma and she is only 20. The doctor said the pressure in the eye is usually around 30 something and hers is 62 or 65....so I will be going to the doctor when she goes back in July to have it checked again and then maybe on to see the optomologist that Kealy sees for her LS.

1 Peter 5:7 (NIV)
Cast all your anxiety on him because he cares for you.

May's update

2009-06-04T09:41:00.016-05:00

May was a busy month:

5/4 - blood work done

5/11 - Kealy's choir put on a show.

5/13 - Doctors appointment

5/14 - Doctors appointment

5/16 - Her most favorite brother graduated from college.

5/18 - MRI of her head and ankle

5/21 - School was dismissed for the Summer!

5/25 - We had a wonderful day at the Zoo [Memorial Day, we do remember!]

5/28 - We all went to Jenks Riverwalk to see the free concert by Francesca Battistelli and Jonny Diaz

~ Let's see, the blood work was okay (triglycerides still high), choir performance was great, doctors appointments went well. They did increase her Concerta which was needed. Wade graduated with honors and 2 degrees. MRI machine broke down while she was being scanned, so she was moved to another one. Results were: No change inside her head - that's good news, but her ankle which still gives her some problems from the sprain back in February has a bone bruise on it. Her doctor said that she could recommend her on to an orthopedic doctor, but she along with myself think it is due to the prednisone and once she is clear of it, then hopefully it will heal up completely.

Speaking of prednisone, as of June 01 she is on only 10mg every other day...YEAH. We are working our way off that stuff. And I say 'our' because it pretty much affected the whole family. And we are starting to see that her ferocious appetite is starting to tapper off. A good thing!

Photos last month were taken a couple weeks past the scheduled date, for some reason we couldn't motivate ourselves to snap them. And they really didn't come out so well, as we did it on the spur of the moment so as not to have any reference.

Summer is upon her, she has been staying with Raina in Tahlequah for a week now and I think is ready to come home. She did get to help sis move into a new apartment. And she isn't too sure she likes her sister having a boyfriend LOL, a little jealously has been going on. It has been busy at home though for me so it doesn't seem like it has been a week, but it sure isn't the same when Boo isn't home. And talking to her on the phone last night I could tell she has picked up a cold, so that always concern's me because of the chemo drug she is on [Methotrexate] lowers her immune system. But I know in the end everything is in God's hands so we will all be okay.

Free to be a More Beautiful You

2009-05-11T09:11:00.006-05:00

http://kxoj.com/you

http://new.music.yahoo.com/videos/--205815479

For KealyOna, with love Mom

Terry Bullard says:
May 8, 2009 at 3:32 pm
~*~My daughter, KealyOna is showing me what it is to be FREE TO BE A MORE BEAUTIFUL YOU!Truely.Tears come to my eyes when I think of it. Since June 2008 she has been struggling with a rare disease at only 12 years old then - now 13. What a hard age anyway. What she has is called Linear Scleraderma En Coup de Sabre, and one doctor said possibly also Parry Romberg Syndrome. It has caused indentions in her head and face and hair loss. Talk about a lot to deal with at that age then add this. The medications she currently take also add weight and mood changes……and tend to make her tired. But she is a true trooper. She often tells me people can love me for who I am, and if they can’t see past the physcial stuff then it’s thier loss. Kind of like a Susan Boyle story. Kealy loves to help at church with the young children and they love her, it’s pretty amazing. You can read more on her story and this disease at my blog. This young lady deals with many issues, but she is always bouncing back and up for the next challenge. Life hasn’t and won’t always been easy, we realize that - but we all know with God we will get through.I love her.She is true beauty.~*~

Hurting

2009-05-05T13:27:00.013-05:00

We're hurting and we don't want to talk about it. Add to that the side effects of the medications she is taking and yesterdays session with the therapist sent us all reeling (except Jerry Don - because he doesn't go). I was a tear drop away from having to take her inpatient and it's not something I could have done. But in the end, her therapist feels Kealy made a break through (which is what happened the last time we had a huge turnaround - May 2007) so pray for her, pray for us all.

Website that talks about Prednisone side effects:
http://vasculitis.med.jhu.edu/treatments/prednisone.html

Kealy started on Prednisone Dec. 22, 2008 [15mg - 1 x a day] 3 weeks or 18 days
Jan. 8, 2009 she went up to 30mg 1 x/day 5 1/2 weeks or 40 days
Feb. 17, 2009 she went down to 20mg 1 x/day and 30mg 1 x/day - alternating every other day 3 1/2 weeks or 26 days
March 15, 2009 she then went to 20mg 1x/day 4 weeks or 29 days
April 13, 2009 again alternating every other day - 20mg then 10mg 1x/day 3 weeks or 20 days
May 2, 2009 she is currently down to 10mg 1x/day

[side note - now that she is down to 10mg and continuing to decrease, she should start seeing some of the weight come off]

The side effects of prednisone include adrenal suppression, which can occur with periods of prednisone use to surpass seven days. Adrenal suppression refers to the body’s inability to synthesize natural corticosteroids, resulting in a dependency on the prednisone taken by the patient. This is why doctors do not recommend the cessation of prednisone when the patient has taken it for longer than seven days. They reduce the dose gradually over a few days in the case of short-term prednisone use, and over weeks or months in the case of long-term treatment. Stopping prednisone treatment abruptly can cause the life-threatening Addison’s disease, in which in the body no longer produces sufficient amounts of adrenal steroid hormones.

Other short-term side effects of prednisone include insomnia, euphoria and, in some cases, even mania. Using prednisone for long periods can cause side effects such as Cushing's syndrome, weight gain, osteoporosis, glaucoma and type II diabetes mellitus. Upon withdrawal of prednisone after long-term use, patients also suffer from depression.

Prednisone use of any period can give rise to many other side effects such as unnatural fatigue or weakness, abdominal pain, blurring of vision, peptic ulcers, infections, pain in the hips or shoulders, osteoporosis, occurrence of acne and sleeplessness. Some of the less serious side effects of prednisone include weight gain, stretch marks on the skin, swelling in the face, nervousness, increase in appetite and hyperactivity. Doctors treat these side effects of prednisone symptomatically, since it is not always feasible to stop prednisone administration even when severe side effects occur. These are situation where they have to weigh the disadvantages of using a drug against the advantages and make an informed decision. One must remember that while prednisone definitely does give rise to many side effects, it is also a life-saving drug.

restlessness
depression
anxiety
increased hair growth
irregular or absent menstrual periods

This video is not of Kealy, but it reminds me of and shows the stages she is going through:
http://www.youtube.com/watch?v=Rc996KPlUKM&feature=player_embedded

Now add to that kids calling you names, 'you and your diseased head'.......RIPS MY HEART OUT!......your fat......AND then the fact that your kind of out of control due to the medications and when one already suffers from ADHD and Mood Disorder - life sometimes is not fair nor easy. But right good choices still have to be made and it's hard.

For the kids out there that make fun of her and don't have a clue what it is to walk in her shoes.......I just want to scream WAKE UP and GROW UP!

God help us all and I know He is seeing us through. My hope rests in Him.

It's a roller coaster.........

Psalm 127:3 "Children are a gift from the LORD; they are a reward from him" (NLT).

Search me, O God, and know my heart; test me and know my anxious thoughts. See if there is any offensive way in me, and lead me in the way everlasting. -- Psalm 139:23-24

~*~

Kealy was blessed before therapy, Kay G. from church took her into her home and cut and styled her hair - it was wonderful. She did an awesome job and Kealy was able to get some bangs. :)

Regarding Kealy's trip to see Dr. Jacobe in Dallas 4/24/2009

2009-04-27T11:03:00.011-05:00

Alrighty! KealyOna and I made our first trip to Dallas on our own and we didn't get lost - though we did happen upon the very last road by accident (or though we felt like it anyway) we were looking to make a turn and the road actually just ran straight ahead into the next one. So all worked out great. It's much better to drive into Dallas late night then at rush hour!!!! But one question - does construction ever go away? I thought Oklahoma was the only state in a perpetual state of road construction! It's nice the hotel is on the same street as the doctors office.

1. She requested that Kealy have a MRI of her head soon (last one she had was in October before we saw the doctor in Philadelphia). She said she was checking for deeper inflammation and other changes.

2. She wanted Kealy to continue to come off the Prednisone, in about 2 weeks we will drop to 10mg for 1 month and continue to come off of it gradually. They (which usually there are about 5 doctors that see her) also said there maybe a chance she would have to go back on the Prednisone - this is NOT an option we will do again and I let them know, something else would have to be tried other than that. Our hopes of being off of it by June have been dashed.

3. Kealy's Methotrexate is to increase from 15mg to 20mg every Monday - starting today.

4. She wanted labs done 1 month from today.

5. She also looked Kealy over for Livedo Reticularis, (as I came across this by chance as one of the ladies in one of the groups I am in has it and sent a link - which resembled Kealy's skin in many places) - she said it does look like it, but didn't confirm or say she didn't have it...only that if she did it wasn't a real concern.

6. She wanted to see her back in 2 months. She felt like one indention had improved and the rest had not, but gotten worse. I mentioned that her face seemed to droop a little just slightly on one side and that I can only see this when she tilts her head back - later while looking at her she said she could notice it, ever so slight as it is.

7. Kealy sprained her ankle in Feb. - it is still causing her pain, so Dr. Jacobe said it would be good just to have an orthopedic doctor check it out. [Prednisone can interfere with bone healing and even cause bone loss.]

~ #8: I forgot on original post.....Kealy said when her mouth is dry she feels like she is drooling, she mentioned this to the doctor, but she didn't really have a reply. Her dad noticed she kept wiping her mouth/chin after picking her up from school or she said it keeps feeling like she has drool - but nothing is there.

***********************************************************************************

Today's followup:

Kealy has an appointment with her pediatrician tomorrow to check her ankle. And the pediatrician will also get her set up for the MRI. We will know more on these two things tomorrow evening.

Side note: She has been having headaches again. :(

2 Corinthians 1:3-4

2009-04-23T08:50:00.004-05:00

"Praise be to the God and Father of our Lord Jesus Christ. God is the Father who is full of mercy and all comfort. He comforts us every time we have trouble, so when others have trouble, we can comfort them with the same comfort God gives us."

Bloodwork & Photo update

2009-04-22T14:08:00.020-05:00

Kealy had her blood work done the other day - lipids, cbc & chem (14?)

Everything was good EXCEPT her triglycerides, normal is 30 to 125 - hers was 170.

SO we have tried getting her (as much as possible) to stick to a low carb diet - which I told her 'lets just try to stay away from sweets and starches'. It is a struggle. Besides still feeling hungry all the time - which is getting better, she is showing some control. Mood's are another thing too. It's pretty much a daily argument about something or she is crying over something. But, as long as everything is going along the way she thinks it should be - we are okay. LOL

It's an up and down cycle, I will be glad when we are back to a steady normal plain.

Her ankle is still bothering her from when she sprained it [back in the middle of February]. So we will be bringing that to the attention of the doctor. She will see Dr. Jacobe on Friday. I get to drive her down Thursday night on my own and then back to Tahlequah on Friday that evening for Wade's (he older/only brother) honors presentation at college. We will put a lot of miles on the car & ourselves in 2 days!

There has been a ton of discussions going on with the Romberg group I joined. Very interesting and makes one continue to think. Besides being autoimmune I am starting to think it is also some sort of vascular problem, one of the ladies posted this link for Livedo Reticularis: http://www3.dermis.net/dermisroot/en/42784/diagnose.htm the other day.......and I have always thought Kealy's legs when you look at them closely look different - and this to me is what her legs look like (I am also bringing this to the attention of the doctor). If Kealy gets a blister or a bite on her foot we really have to watch it close (this going back to the time she was just walking). She has had two occasions where in just a days time she had infections spreading up her leg and had to receive antibiotic shots + medication.

She is also to see Dr. Adelson on the 29th. When he called about the blood work results, he of course asked how she is doing. She is great actually - but.....when you look at her the visible part on her face doesn't seem to have progressed (though at times it looks deeper, and other times less noticeable); but what is going on with her hair and scalp is another thing. She has continued to loose hair in her 2nd indention and now at the crown (top back) of her head. And bless her heart just to feel her head is alarming. This she doesn't let me do hardly at all. She doesn't want anyone touching her head. Which I do not blame her.

Kealy colored her hair the other day (by herself). It's looks really nice. And she thought about bangs, but with everything going on, decided against it. With hair manipulation, loosing anymore might not be a good thing when trying to cover spots.

She is down to 20mg / 10mg of Prednisone alternating every other day.

A note about her choir competition - she did great [ they score them between 1 and 5, 1 be the best and 5 needing lots of work ] ~ Kealy got a 1 on her first solo! She sang 'Beautiful Saviour'. We are very proud of her. If she will stick with it I think she will really enjoy this all through the rest of her school years. The choir also sang a song in Hebrew I think, it was pretty cool.

April Photos:

The flash blended the LSES out. (a good thing but not for reference purposes)

Her face on the Right side as you look at it seems to droop down somewhat to me,

[not noticeable on the forward shot].

The eye seems to droop and her nose looks smaller on that side.

This is one of the few shots of the 2nd indention.

This is a shot of the back, where she thinks it is,

I don't but she wouldn't let me locate it.

So we do with what we can get!

If you stop by to read the blog ~ I would appreciate you leaving a note on the guestbook for Kealy, it lifts her spirits to hear from others. Just encouragement or just to know someone out there knows what she is going through and can sympathise OR to know that 'putting herself out there like this' is helping others.

THANKS & God Bless!

Ophthalmologist Visit today

2009-03-31T13:23:00.006-05:00

Just a picture from our Oklahoma Blizzard on Saturday March 28th, 2009
we received about 7 inches of snow at the house that day!

All is clear, no inflammation - yeah. He also checked her eye pressure and it was good too. The doctor stated when you are on prednisone it can start affecting your eyes, cataracts and such - this is why they did the pressure check. All was good with her pressure too! So far the eyes have not been anything we have had to worry about - other than praying it all stays clear and healthy. Vision is also good. ~Back to school for the day (she has a choir competition tomorrow and also will sing her first solo!)

This is a picture taken Sunday, we are making a scrapbook page for the little boy in Thialand that we sponsor through Compassion International.

March update

2009-03-24T15:13:00.009-05:00

The months go by slowly.

It seems like it is going to be forever before Kealy is off of the prednisone. It has changed her looks so dramatically. Bless her heart. But she treads on. Her attitude is - people either like me for who I am or they can move on. She is much better at that then I am, I want to feel like I fit somewhere. I have been reading a friends blog, she just started - and it has been pretty overwhelming emotionally because she is and has been where we have been and are. Though they are not looking at treating their son with medications, but looking at surgery - which may be something Kealy does down the road.

She had a charlie horse in her leg last night and this morning, so I told her to eat banana's at lunch today. And she has a small rash we are keeping an eye on. And the MOODS oh, they come and they go.

Also I joined the Parry-Romberg group, I recommend this group it has been a great help and support: http://www.geocities.com/HotSprings/1018/index.html

If you want to join the group - sign the guest book and Marilyn will help you through the rest off it by email - so be sure to list your email address. They are having a get together in July, I wish we could afford to go.

And just an odd tidbit - I was watching 'Say Yes to the Dress" the other night and I believe that one of the brides to be on the show has PRS. Called Kealy into see and she agreed. Or maybe it was something else. But it was like hey! a kindred spirit. LOL

Just another site I found with information I thought helpful.

http://www.patient.co.uk/showdoc/27001298/

I use to spend a lot of time researching all of this on the web, everyday.....now I do it usually a couple of times a week to see if there is anything new out there.

Lastly, pictures of change once again: 2007 when she just had hair loss.

Then this months reference photo, it doesn't look as deep, but I believe that is because we have reached the prednisone 'moon face' stage:

Just came across this site....

2009-03-04T13:33:00.007-06:00

http://www.hss.edu/conditions_scleroderma-coup-sabre-parry-romberg-syndrome.asp

I thought this site was insightful (no pun intended). As it seems confusing between en coup de sabre and parry-romberg syndrome.

Since only one doctor has mentioned Kealy also has Parry-Romberg Syndrome, I didn't pay much attention to that until of late. But the two seem so intertwined it's hard to tell where one begins and one ends. In this article he states that almost all times that if you have PRS that your tongue will show involvement - as yet Kealy's does not show any signs in her tongue. Her teeth are delayed a year in growth though and he doesn't mention anything about teeth in the article.

He also mentions the eye - and she goes back for her 6 month check up with the optomolgist at the end of March.

She has the moon face which comes with the prednisone. I don't think we will ever do the prednisone again and I will be glad when she is all the way off of that stuff. She is constantly hungry and it hasn't tapered off any with just the slight decrease every other day of the medication. Every day is a constant arguement over telling her no she can't have something else to eat.

Thanks to Terri in California, I was able to look into a good facial surgeon if that time ever comes. Her son has PRS and he is very young.

Just on picture to share - it's over a month old, but she is still a cutey!

Mostly I just wanted to make note of the site listed above.

Take care!

Dallas, decreasing and doctors...

2009-02-20T14:13:00.014-06:00

We made our trip to Dallas for Kealy to see Dr. Jacobe. 2/13 [she goes back in 2 months]

Pretty uneventful, I guess.......but she has been able to start decreasing the prednisone - WHICH we are happy about. It will take 4 month's or more to get her off of it, but that's okay. And the other thing I almost forgot, because we already have an appointment set is she wanted her to see the optomolotist again soon. And wanted to be sure she had, had a CT & MRI done (which was done in October) - not sure when it will be needed again. It has affected her skull, I will have to see some day if I can scan a picture of the CT that shows this.

The doctor feels that it has stopped spreading, -we aren't holding our breath (we would probably pass out). It may not be any farther down, but it does look deeper to me, as still seems to change a bit from time to time. Looking worse sometimes and better others.

Poor girl - she looks like an old person when it comes to medications - one of her friends came over the other day and said "that's all Kealy's medicines?!" Sorry to say yes. And they added 'calcium and vitamin D' to her regime. This for her bone health.

Medication run down:

AM daily:

20mg [2 pills]/ 30mg [3 pills] prednisone (alternates every other day for 1 month - then drops to 20mg for 1 month solid)

Concerta [1 pill]

Folic Acid 1mg [1 pill]

PM daily:

Abilify [1/2 a pill]

Vitamin D and Calcium [1 big pill, she will tell you]

{1 day each week she takes 15mg of Methotrexate} [3 pills in the am/3 pills in the pm]

Speaking of which she sprained her ankle at school the other day. Not to bad. After a day on crutches she was ready to deal with the pain and work on walking! Crutches like braces might sound like fun, but once experience are a huge pain.

Going off the prednisone will help her loose the weight, I have never seen anyone that is hungry like she is all the time! I will be glad for her when she is off of that stuff (oh and the moods - owe!).

If you notice the hand prints on the wall behind her that's back from when we did foster care, each child got to stick their hand in paint and decorate our wall. Sometimes I just walk by there and pray for one of them, or all of them, it's a nice reminder. WE ARE ALL LOVED.

And not in regards to the LS - but she changed and started at a new school, and so far is loving it. From her previous school which had under 400 students from Pre-K to 12th grade; to a much larger one where there are as many 6th, 7th & 8th graders as were in her other school all grades combined. But she has adjusted well.

She has had several people ask her about her head and a parent that wanted to look it up, but she just tells them what it is and goes on. She seems to have some friends that are very nice and have taken her in per say...

Also, I have started feeling a bit more relaxed over all of this - I know it could have been or could be way way worse......but she is still my child and just wanting to make sure everything was being done that could be and should be, plus seeing her go through changes physically, emotionally and dealing with all of that makes a person 'like me' I should say, a bit stressed out.

Doctors appointment update...

2009-01-24T22:53:00.007-06:00

She went to see the doctor on Friday 1/23. Everything is going okay. He said he thought the inflammation had stopped because the main indention on her head is not red (or it wasn't at the time she was at the office). He is not going to increase any of her medications at this time (15mg total of Methotrexate once a week; 30mg Prednisone daily; and her Folic Acid 1mg daily) - he said he would wait until she goes to see the doctor in Dallas again (which is set for 2/13) before he makes any further increases to see what she has to say. I must say he has been a great doctor to work with. He is also going to a conference in March and will be looking to see if he can obtain a light unit (which we think is awesome). And will also get to visit with Dr. Jacobe at that time in person (or so he thinks). There is a question as to if the second indention has started to show on her forehead (this maybe a normal line having to do with the skull - or with how her face has puffed up from the prednisone & weight gain - lets hope that is it). The doctor said to have Dr. Jacobe look at it when we went. Dr. Adelson did purchase a camera and took photos of Kealy for reference, he told her if she ever saw them not to be shocked by them (as he took them at an angle which really highlights the indention) but I don't think he realizes she looks at it everyday. It's there staring her in the face. She seems to get a little tired more now. Friday after the doctors she was feeling a little under the weather & tired so she went home and slept for a couple hours or so. But was feeling better around 2pm. She tends to take a nap when she gets home in the evenings at times or goes to bed early. Which is okay!

She had therapy on Monday too......this is for the ADHD/Mood Disorder; BUT is really a blessing to help her open up more about the LS and how it is affecting her. She tends to tell me everything is fine. It's hard to see your child cry and NOT be able to fix it. She has this fear (I think it stems just from the fact of what she has is so rare) that she is going to die from it. I constantly reassure her she is NOT. We have made some friends over the Internet (hi Sarah and Hege) that also suffer with LS; I reminded her that Sarah has had what Kealy has for several years now and she is fine (in a manner of speaking) AND Kealy just opened up that She is not fine, she hurts physically and emotionally and that people laugh at her and make fun of her and that she hurts it makes her hurt. Which came out in an angry out burst, which led to her crying and not talking about it anymore at that time. The doctor [which we love] was going to talk to her some more about it and how she can find ways to talk to me about it - BUT Kealy had some 'healies' on that her cousin gave her and fell hurting her wrist (not severely) right when I left. So I had to return and they didn't get to have their discussion, guess it will take place the next time we go.

The rash she had that one morning has not been back. The only other thing to mention is that she has been more moodier than usual, this I believe to be from the Prednisone. And she talks in her sleep a lot now.

Just some notes

2009-01-14T12:29:00.005-06:00

This is mostly for my own memories sake.

Kealy woke up with a slight rash beside her LS (on each side) -red and alittle bumpy, her checks were a little flushed, but not warm to the touch. The coup de sabre looked more blue than usual. She said she had no physical symptoms. By the time we were to the school it was looking better. So who knows what that was about.

She didn't take her medication yesterday morning/Tues. (I asked, she said yes I guess thinking she would and got side tracked & didn't). Argh [so she missed her foclic acid, concerta & prednisone].

Her ankle has been hurting her, she said she hurt it playing basketball the end of last week.

They won thier basketball game Monday and lost last night. She did score 2 points in her game last week! (I might have already mentioned that, but it's pretty exciting for her) :)

Pictures from 12/20/08 for reference:

Just some fun pics

2009-01-06T14:52:00.007-06:00

This is her new 'do', with the highlights of blonde and red...now if she will just leave it out of a pony tail!

Kealy being Kealy - she got to spend a couple days with her sister at her apartment in T-quah and had a great time (just the 2 girls doing girly things).

I like this one - the LS is barely visible (wouldn't see it if you didn't know it was there) and Raina colored her eyes blue : ) . I am fond of black and white photos.

She looks like 'm a r v e l o u s'. : )

Medications Increase, etc.

2009-01-05T10:15:00.009-06:00

Today Kealy starts on 15mg of Methotrexate (7.5mg in the am and 7.5mg in the pm) on Thursday her Prednisone will increase to 30mg daily.

Her hair loss has increased, more so in the second indention than the first one - and the 2 indention's have joined at the crown of her head, causing it to feel kind of flat. I was unaware of this until I walked by the back of the couch while she was sitting on it and gently as a mom will do rubbed her head (cause I love her) and was stopped in my tracks. She notices these things and doesn't always tell me. The indention down her forehead has made it's way to her nose - we could see the outer indention (which is what we say is surface) and then she can feel with her hands how deep it really is and said it has made its way down just a slight bit more. It still changes when looking at it from day to day, some days not so bad and other days it looks really deep. Her face also gets red for no reason sometimes and it will get hot. She had some more headaches in early December, but none (that she has noted to me) of late.

She had more blood work done on 12/31 and goes back to the doctor on 1/23.

Her brother is home from college and has a cold which concerns me for her. So pray she doesn't get his illness. We are hoping it is just allergies! He has struggled with those all his life.

A friend and her family came over for New Years, she hadn't seen Kealy in a while and asked what she had surgery on her face for? Some people think it is a scar. Unless they ask we usually don't say anything.

I also want to Thank Hege and Sarah for conversing with me and Kealy - it does help to know there are others out there. This is a disease since it is so rare, makes one feel all alone.

Merry Christmas!

2008-12-24T09:19:00.007-06:00

"For to us a child is born, to us a son is given, and the government will be on his shoulders.
And he will be called Wonderful Counselor, Mighty God, Everlasting Father, Prince of Peace" (Isaiah 9:6 NIV)

************

Just a brief update; The MTX was doubled, and so far so good, no real side affects. The prednisone will double soon. I feel like we are starting to feel some of the mood change affects it can have, last night was a very rough evening - to say the least Kealy & I both left the Wal-Mart before shopping was done and with nothing. She did get her hair cut earlier in the day (pictures later) short in back, longer on the sides and I allowed her to have highlights put in it - reds and blonde's....it looks really cute! So far she is doing okay with the weight gain. Not to say she hasn't gained any, but not noticeable yet. Oh and the doctor said she could have a hamster....so she picked one out for Christmas on Saturday - her name is Bella Ann. Lastly, we took her monthly photo so we could keep a journal of any changes - I won't be posting them as I don't think Kealy would feel comfortable with them up on the Internet. She continues to loose hair (telltale sign is the shower wall) and the indention appears deeper at times, maybe a little farther down onto her nose - it changes so it's hard to gauge. Lastly, I get a little aggravated with her.......she would insist that she had to wear her hair in a pony tail (hopefully that will change with the new do) even though she knew it was making her hair loss worse [some habits are hard to break] and she does 'fair' with keeping her hands sanitized with antibacterial gels. This mostly takes place if someone is there to remind her.

***************

May you all be blessed with a Merry Christmas

and a

Prosperous New Year

Okay

2008-12-17T16:18:00.004-06:00

She had her appointment today. The spot is still there, but appears to be superficial - so he gave her some cream to put on it. The place beside her eye and the bumps on her head have cleared up in the last couple of days so that was good. She had blood work done. And will need it again in 2 weeks. Her dose of MTX or Methotrexate will increase the next time she takes it, Prednisone the same - and he talked about going to a pulse dose steroid in the future. We will see what happens. Everything seems to be going along pretty well. Lastly the cream she was putting on every other week was causing her skin to peal up or shed in chunks at time so he said that she could stop using it.

Physical Stuff...

2008-12-15T12:35:00.008-06:00

Let's see, over the weekend first Kealy noticed a spot on her back - she was upset (she is always afraid she is going to get bit by a spider), I kind of got upset thinking maybe a plaque type spot - but then I try to be more rational and think it's probably just a dry skin patch. She also has a spot by her eye that itches, can't really see anything there just that it is irritated. I did email the doctor and sent some photos of the spot on her back. Next day she notices a small set of bumps on her head by her hairline and indention that itch somewhat and look like a cluster of pimples. Not sure what they are.

She has an appointment to see the doctor this week.

Kealy also had a visit with the dentist last week. Her teeth were all good (no cavities! yeah) but she is about a year behind in there growth. She has 2 baby teeth yet to loose, and 2 adult teeth waiting to come in. Other than that her teeth are pretty straight.

We also met with her teachers last week and reviewed and updated her IEP (individual education plan). They have been very good to work with.

Treatment Started...

2008-12-10T11:19:00.009-06:00

Just a brief update:

Monday 12/8 she started the Methotrexate (5mg) that night. [she only takes this once per week]

Since her daddy works nights I had her sleep with me, just to make sure nothing went awry.

I think she, like babies look so sweet when they are asleep!

Tuesday 12/9 she took her Prednisone (15mg) in the morning with breakfast. [she takes this daily]

{she slept a lot when she got home from school, not sure if it is the medications or not}

I have mixed emotions about all this, fear of the medications and what if they don't work - to put her through all this, but then I also feel I can't set back and try nothing.
She said Monday night while in bed, 'Mom what if the medication doesn't work, what will happen to my face'.......I just have to tell her our prayers are it will, and what ever happens WE will deal with it.

Photos from the Philadelphia Trip

2008-12-10T10:38:00.011-06:00

Kealy at the Liberty Bell (like you can't tell). This is a park now, but our guide told us many soldiers are buried there,...

She also said President Washington and President Lincoln both gave speeches from the balcony of this building....

Jerry Don (her Dad) and Kealy in front of many Historical sites - the lady we took our carriage ride with is actually passing back behind them (this was taken before we took our ride with her)...

Kealy entertaining with card tricks (she did this for anyone who would take the time to watch, even on the plane!) I like this picture of her, nice smile! : )

Lounging at the aLoft Hotel....too bad it was a little too chilly to stay outside.

On the plane...

On the shuttle...she thought that was cool (funny)

Anticipation....Treatment starts Monday

2008-12-03T10:04:00.019-06:00

"I love the man that can smile in trouble, that can gather strength from distress, and grow brave by reflection." --Thomas Paine

Isn't she beautiful! : )

Just a few updates: Treatment will begin Monday 12/08/08! Finally!!

She is going to switch from taking her Focalin XR for the ADHD to Concerta; she took Concerta when she first started on ADHD medication - AND had NO appetite, so I am hoping that this may help even out the Prednisone and her weight gain will not be so bad. It will be a wait and see process if it works as we hope in theory.

We have been dealing with the weight issue of late [just the thought of it has it's affect], I had purchased her a couple of jogging outfits for her to put on after she came home and showered each day........(well I bought them in her size, but in the plus division to give her a little room to prepare for the prednisone changes so she could be comfortable) She took them back to her room and I knew once I heard her crying she read the label, sad how a word can affect us so negatively. And so she came out crying and upset about that, and bringing up the time her Grandma called her obese.........PEOPLE PLEASE DON'T SAY NEGATIVE THINGS TO OTHERS THINKING THAT WILL SPUR THEM INTO DOING WHAT YOU FEEL IS CORRECT OR NORM (I too am bad about this sometimes! self correction needed) - It only brings lasting hurt because words can never be taken back. NOT everyone understands or knows that a lot of the changes Kealy goes through in her life are brought on by medications and that is something she has learned to deal with I just wish everyone else would.

Lastly, she is wanting to transfer to another school next year so she can take chior (she does love to sing and is pretty good at it) - so I mentioned it might not be to easy making a change at this point in time (kids can be cruel), I let her know that her classmate right now understand and know whats going on, but a new school would be all different and they would not know and it could be hurtful - BUT she said she is ready to face the challenge [of course it didn't go so sweetly, a little anger some tears and 'see this thing is ruining my life!' - but I told her it only will if she lets it, she determines her path and she was good with that and still wanted to change schools.]

Boo and Bell

KealyOna and Raina

Boo's Baptism [July 20, 2008]

2008-12-02T15:36:00.004-06:00

And we know that in all things God works

for the good of those who love Him, who

have been called according to His purpose.

~ Romans 8:28

[just thought I would share one of the most important events in her life]

Prednisone [and my folder]

2008-12-02T14:35:00.005-06:00

As mentioned before, this medication is a concern. Since the side affects can be disturbing, especially for a teen girl, I have made mention of it to her therapist and school so we can all help Kealy as she goes through this know she is loved and there will be an end to it all. Last night I went over the side affects with Kealy once again, and discussed how we would try to handle her diet to keep matters in check:
No extra salt
Try to stay away from sugary, starchy foods and candies (VERY HARD)
Exercise
Eat small amounts through out the day - instead of big meals
Eat foods the body has to work harder at breaking down

So besides the water retention and weight gain, I made her aware that she could deal with acne and mood swings. I let her know there maybe other things that we haven't talked about, so let me know if something is up and she doesn't feel right. I was able to get some helpful information from many sites but this one was on of the best:

http://www.gihealth.com/html/education/drugs/prednisone.html

So be praying for her, I know this will be a struggle.

One of these days I will have to take a picture and post of 'my binder'; it started out as Kealy's old Care Bear folder from school I started sticking stuff in and grew into a 2" binder......Kealy use to say 'mom get your folder' making fun of me - she knows what it contains too, so it angers her sometimes and she has threatened to throw it away (hoping in a way that if she could that she could be done with it all) I Don't Blame Her! But it has information from each doctors appointment, information for the school and to the school, information I have printed from the web on LS, coup de sabre, Romberg syndrome, morphea and also information on medications used to treat them [ what has been successfull, what has not ] and then further medical information on the medications she is to take.

WE must be willing to get rid of the life we planned,

so as to have the life that is waiting for us.

~ Joseph Campbell

12/1/2008 Thanksgiving weekend has passed...back to the doctor

2008-12-02T11:00:00.023-06:00

WED 11/26

Kealy was off Wednesday along with her brother and sister who came home from college for the break. So on Wednesday her sister Raina took Kealy shopping. Their favorite thing to do. And they had a great time together. That is always a blessing to see them enjoy each others company and talk.

THURS 11/27

Thanksgiving was great, always good, yummy food AND too much of it! Kealy had a busy break. Thanksgiving day we celebrated at her dad's parents house. I notice a line on her chin. Raina too see's it. I mention it to Kealy - she tells me to not tell her stuff like that anymore until we are at the doctors. I can't blame her. We will watch it.

FRI 11/28

The day after Thanksgiving - It's tradition.....the TREE goes UP, and Kealy officiates that and puts it together, then everyone puts on their ornaments. Dad does the lights and tops it off with the angel at the end. I put on my angels and bows. Kealy, Raina and Wade put on their ornaments (photos - 1 each year until they graduate high school).

SAT 11/29

We spent part of the day celebrating Thanksgiving with my family at my parents house (which is right in front of us). My niece was ill, so in order for everyone to enjoy the day - AND KEALY NOT CONTRACT ANY OTHER VIRUSES AT THIS TIME........she agreed to wear a face mask.

Food again was awesome. Kealy made 2 cream cheese pies, and some peanut butter cup cupcakes and helped me with making a pecan pie and mashed potatoes (she was a great help at peeling all those potatoes).

Later that evening Raina took Kealy to see TWILIGHT. She loved it.

SUN 11/30
To church to worship. Kealy does a great job with the children, she is often asked by the adults to help them with their classes and with children's worship. She is always happy to oblige and you can tell the kids love her - they flock to her. It was so cute one day when one of the little boys turned around in his seat and yelled 'Kealy' then shot her the 'I love you' hand gesture. : )

*******************************

MONDAY 12/01/2008

Back to school. We have started preparing her for treatment. What it will entail. Letting the school know, etc.

Dad picked Boo up just a little early at school for her doctors appointment today - to see if she is healthy enough to start treatment. At the doctors office, the infection appears to be gone, just a little fluid left on each ear, but okay. She is given the go ahead to start. He doctor starts letting her know what she needs to start doing to keep from getting further infections, or trying to prevent as much as possible:

Wash hands OFTEN
Use antibacterial gel
Move away from people that are ill or coughing
DO NOT SHARE ANYTHING!
Shower daily will help
Change her clothes right when she gets home from school
Wash your hands OFTEN
DON'T Touch Your Face
Do not drink after anyone, back up to Do not share anything!

After that she was able to get a flu shot, the last time we were in I said no because I wasn't for sure if she should get it, but she could and did - the shot kind, not the live virus type.

Emailed Dr. A that she was cleared to start treatment, he said lets give it a week and then start. Not sure why, maybe because of just getting the flu shot. And he is concerned for Kealy because of the prednisone side affects (as we are too) because of her age and being a girl - it's a hard time anyway.

Photos of Change...taken 11/19/2008

2008-11-21T14:59:00.002-06:00

Her mug shot! : )

She was trying to be funny (and she was pretty much)

You can see part of her hair loss; the indention

And from these photos we noticed something going on in her eyebrow to the left as you are looking at it.

On this one you can see how it is indenting her

forehead, the main one and then slightly above the

one to our left as we look at it, the towel kind of covers it slightly.

AND IT WOULDN'T BE KEALY IF YOU COULDN'T HAVE SOME 'FUN' WITH IT.

SO ~ROCK ON~ DUDES!!!!

Psalm 139:15b-16

2008-11-21T12:59:00.000-06:00

When I was woven together in the depths of the earth,

your eyes saw my unformed body.

All the days ordained for me were written in your book

before one of them came to be.

1 week since Dallas

2008-11-21T10:47:00.000-06:00

It's been a week since Dallas, Kealy still can't start treatment because she has a double ear infection and is currently on antibiotics. Once that clears up and she is given the go ahead the treatment we were notified would be:

1. she will start out on 30 mg prednisone on going (I thought for 3 months) but just got news that it may be 4 - 6 months. Let's pray not that long.

2. Methotrexate starting out at 7.5 or 5 mg one time per week building up by 5mg a week until she reaches 15 to 20 mg once per week. She will need a blood test every time she increases a dose to test her liver function and white blood cell count. MTX I am told is long term, 2 years or more.

3. She will continue using the cream as noted before.

If the doctors find that this regimen is not succeeding then she will start UVA1 - which we are not sure how that will work since the distance is so far and she would need to do it 2 to 3 times per week for 45 treatments. So another prayer [pray that everything before mentioned gets her to the point she needs to be]!

This website has some good information and describes the different medications toward the bottom of the article. http://www.emedicine.com/derm/TOPIC272.HTM

Note Kealy is staying active, she is not letting this slow her down a bit. She loves playing basketball and gets upset when she has to miss practice or a game due to doctor visits etc. We did decide that she should not wear pony tails anymore - as it causes her hair to fall out that much quicker!! So we picked up a sporty headband - go Charles Barkley! (I always remember him in a headband for some reason : )

Physically she has not been ill from the LS, just headaches and sensitivity in the area where she has the hair loss and indention's.

I have read where some with LS - coup de sabre have had or deal with seizer's, problems with their eyes and dental problems.......so far none of that with Kealy [Thank my Lord!]

HAPPY 13TH BIRTHDAY!

2008-11-20T16:41:00.000-06:00

I also might add - among all the doctors visits and trips.............Kealy turned 13.

She was so excited she got and ipod for her birthday (I thought it might come in handy with all thats going on). And over the weekend she had a friend come over for cake & festivities, she spent the night and they went to the Purple Glaze to do ceramics and mosaics. Loads of Fun!

Love you Boo!

Dallas Doctor Visit

2008-11-20T15:29:00.001-06:00

We make it to Dallas (the GPS and maps on the iPhone are great - Thanks Wade)!

The doctors visit was good. I think Kealy said 'I saw 7 doctors'. We did not see anyone with coup de sabre while there though. Dr. J was very informative, and went over most everything I had already been reading, but it was good to hear it from the doctors mouth.

*Not much is know about LS
*They don't know what causes it
*It is not contagious
*There is no set treatment for it and treatment can vary between individuals
*It is basically collagen that has went wrong, that hardens which causes the hair to fall out and the skin to tighten
*Talked about treatments; she thought the best out come for Kealy would be:
Prednisone for about 3 months and Methotrexate for long term 2 years or so
She said she has not had much success with UVA1 on coup de sabre because of the density of the skin on the head, but would look at it in 3 months and maybe reconsider
*One of the other doctors prescribed a cortizone ointment, she puts it on 2 x/day every other week for 3 months

While there Kealy agreed (we actually discussed this before getting there) to be part of a research project. So she had LOTS more blood taken.

She seems to be taking this all in stride. Though she has her moments at times, she deals with this very well. A nice distraction at my aunts house was her dog, he was fun! But right after the appointment we had to head straight home [Jerry Don had to work that night].

Dr. J said she would work with the doctor in Tulsa and let him prescribe the medications as Kealy will need frequent blood work to make sure everything stays okay with her. Kealy will go back to Dallas to see her for follow-up in 3 months.

Back from PA

2008-11-20T15:16:00.000-06:00

We get back from PA on Saturday, tell them at church Sunday we have a plan (or so we thought). I start researching the medications and I am not happy with what I find, besides reading over and over again that results are inconclusive, no real evidence found that it does anything (very few cases I read had positive mention) and the side effects.....renal failure; I decided to call Dr. A and see what he thought. He was surprised at what was prescribed, and I asked about seeing another specialist for a second opinion. He agreed. And recommended a doctor in Dallas. Not that I have more knowledge then any doctor, but it's my daughter and I want to feel and know that what is done to her is right for her.

We started working on getting an appointment, quickly with his help and working with the insurance to get the doctor in our network so the appointment would be covered. It all worked out. GOD is SO GOOD. An appointment is set for November 14th - my aunt lives near Dallas so that was a blessing, we got to have a short visit with her and spend the night at her house.

In the meantime Kealy caught a cold, her grandma had it, her sister Raina too, then me - so I guess it was inevitable that Boo get it.

Philadelphia 10-24-2008

2008-11-20T14:17:00.000-06:00

We got to PA on Thurs. found our hotel [the aLoft - very nice hip place] and did a search for the doctors office so we would know where to go in the morning.

We highly recommend the talking GPS for directions - it was awesome!

Traffic wasn't as bad as we had anticipated and travel was pretty easy (per say). Philly would be a great place for a historical vacation! And guess what the world series was going on at the time we were there - and the Phillies won! Kealy enjoyed the trip - she, along with her dad & I took a carriage ride around the historical sites. That is a great way to get good information and see things in a hurry. Took photos of course and saw the Liberty Bell among other things.

The doctors visit to the Scleroderma Center. We arrived early........and what I do now is look at people to see if anyone has what Kealy has - on TV, when I go to town, any where......I have yet to see another person (besides the images on the internet). We did see some individual's that appeared to have the systemic form of scleroderma (I thank God truly that is not what Kealy has). But no one with coup de sabre. Anyway back to the visit, all the paperwork is filled out, we have film's in hand and are ready - she is ready just to get it over. Finally she is called back, an intern comes in first, beautiful girl - she goes over everything, asks the questions, looks Boo over and finally the doctor comes in. Looks her over, confirms everything, states that it is very agressive and that we have caught it early also mentions that she has Romberg Syndrome. He wants to treat her with steroids and D-Penicillimine (I have not come across D-P.. in my readings much, so I wasn't to informed on it) - what I had read a lot about was steroids and Methotrexate and UVA1 therapy........so I ask about that. He said they have not had much success with those type of treatments. So I am thinking she is getting something new and better. He wants to see her back every 6 months.......sorry I say we can't afford that (the $1,900 bill for that trip is yet to be paid - but we'll get it done). So, we will see what we can do. In the meantime plans are for the doctor to work through / with the doctor in Tulsa. So before leaving photos are taken of Kealy, I might mention another doctor came in too to see her. I think Boo likes all the attention, just too bad it comes with LS.

And incase I forget by the time we made it to start seeing any of the doctors Kealy had a 2nd indention start, and she thought another one at the back (but we have come to think that it was just her normal head). When we were in PA the hair loss was barely noticable in the 2nd indention.

In a Hurry......!

2008-11-20T13:31:00.000-06:00

I must say our insurance has been very nice to work with - Kealy's case manager has been helpful for sure.

Before going to PA, Kealy needed to have her CT scan, x-rays and MRI done. SHE WAS A TROOPER THROUGH IT ALL!!!!

The CT Scan was done on Oct. 21st (and the neck x-rays) - she couldn't eat anything after noon until after the CT was done, which was scheduled for 4:30pm. She had to have a temporary stint (I think that is what it is called) and then when they were ready to put her into the scanner they hooked up the dye. She wasn't happy about that and some tears made there way through - but she did great and it didn't take long.

Oct. 22nd she had her MRI done, she also had to get an injection for this one too (I was unaware of that) but she did great. Dad took her for this trip.

She got her copies of all the films.

On Oct. 23rd we boarded the plan to head to PA. Kealy's first flight. I was a little nervous for her because she has had car sickness before, so I was expecting maybe she would need a barf bag! BUT, SHE DID GREAT - DIDN'T BOTHER HER A BIT!

Second Dermatologist - 6th Doctor

2008-11-20T12:06:00.000-06:00

October 13th, 2008 - The main doctor we had been waiting on to see, the second dermatologist. He came in along with 2 interns. Even though I was holding my breath, because I had called the doctors office and asked if he treated any patients with LS, I also sent him an e-mail (I found him on the internet) and never heard back - and after the last few doctors we had been too my confidence was fading. Thats why we were looking for help else where. I also called the local scleroderma group, asking who they used as doctors or recommended, the lady I talked to only knew of one other person (a grown lady) that had the type of LS Kealy has. She told me she had heard the doctor we were going to see was pretty good.

If you want someone to treat cancer you go to a cancer specialist, not a foot doctor. You want someone to know what they are dealing with.

Dr. A (as I will call him, not sure if the doctors would like their names plastered on the internet) - seemed to know more than anyone else, but he still did not deal with this on a regular basis. Now I don't think anyone does. He confirmed the diagnosis. He also did a punch biopsy, which Kealy handled pretty well, I was proud of her. For those that don't know what that is: they numb an area of the LS, then they take a punch and by hand push it into the head and retreive a portion of the scalp and skin layers about the size of a small eraser. Kealy didn't really feel anything she said BUT hated the sound it made! And her head bled pretty good for a bit. It took at least a month for the spot to heal, probably because of the LS. It confirmed the morphea. We left the doctors office, being told that a decision on what to do would be made after the biopsy results came back. I had already started working on getting her into the specialist in PA.

Dr. A had called with the results of the biopsy and wanted to take Kealy to a forum of doctors to have them all review and view her case and then decide on a treatment plan. I told him that we had an appointment to go to PA to see a specialist in Philadelphia - but since what she had was so rare it would still be a good idea and that I thought Kealy would be up to letting other doctors see her. While talking with him he had mentioned some other doctors that he would take his daughter to if it was his daughter. One of those I had come across in all the litature I have been reading. I would get back with him when we returned from PA.

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Prayers

2008-11-20T11:50:00.000-06:00

After the first visit with Kealy's pediatrician - we asked for prayers. From our friends, family and church family, also co-workers......we know those continue to be offered up to our Heavenly Father on Kealy's behalf and WE THANK YOU!

Quick Run Down of Next Few Doctors Visits!

2008-11-19T15:56:00.000-06:00

We couldn't get into see the next dermatologist until October 13, 2008........long wait, and I am not a patient person per say!

I called the derm.1 to see if Kealy should play basketball, not knowing and she said NO not until she sees a neurologist. I also mentioned she was having headaches again, so she said she would need to see the neurologist for that too.

3rd Doctor: Optomologist - he had never heard of LS before, and had to go back to the medical books to see what he was suppose to be looking for when examining Kealy. Her eyes were clear, no inflamation, her vision was good and the muscles were good. So that went smoothly. Oct. 3, 2008

4th Doctor: Rheumatologist - he said he had only seen 2 other patients with LS also. He mentioned that it may be treated or just let it run it's course. He also said it would be good to find a clinic or specialist that dealt with this - AND so I started searching. Kealy also had blood work done on this date. [which I was told came back normal, which is not unusual for LS] October 7, 2008

Searching: I used www.sclero.org site and the www.scleroderma.org site. Shelley Ensz with the www.sclero.org site was the MOST HELPFUL PERSON!!!!! I highly recommend that site.

I grouped together all the specialist clinics and called one in Houston, they didn't treat children, I called the next closest one in Colorado they too didn't treat children. So I mapped them on a map and the rest of them were located back east - I picked one in PA which seemed to be the most knowlegeable and listed treating many people with scleroderma and Linear scleroderma. Fired off an email, not expecting to really hear back - but I did. When it was mentioned previously to 'possibly let it run it's course, I kind of freaked'.....and so was trying to find the best, since it seems that this is pretty rare and not much is known about it. The doctor in PA said you don't let it run it's course and would be happy to see her and get her in quickly.

5th Doctor: Neurologist - he didn't know what LS was either and had to go back to his medical books. He seemed to think the headaches were caused by posture (I mentioned twice, she never had headaches until this started). So he referred the MRI, CT scan and x-rays of her neck (for posture problems!) back to her pediatrician. Oct. 8, 2008

2nd Doctors Visit....

2008-11-19T15:43:00.000-06:00

9/11/2008 - a sad day to begin with.

[note before going to this doctors appointment I called stating I believe my daughter has Linear Scleroderma, does this doctor know what that is and does she treat it....of course I was told yes]

We get to the appointment. Kealy is getting nervous, I can tell because she starts getting very verbal. Sometimes not pleasant.

The doctor comes in, and looks at it and straight off states it is coup de sabre............well I thought good it's not what I thought it was............only thing is I was pronouncing it wrong or differently. My husband knew right off, you mean strike of the sword - and I thought 'oh crap'. Yes she said, I said - then it is linear scleroderma, she kind of looked at me in surprise (so I had to tell her I looked it up on the Internet and thought I knew what she had). This doctor had only seen it one other time, back 20 years ago when she did her clinicians. She went to get her medical book and looked it up, and then came back to show us and photos of it. SO SHE DIDN'T TREAT PATIENTS WITH IT and we were referred onto another dermatologist.

Before going back to school, we decided a fun hair cut would be just what Boo needed. And it turned out soooo cute!

Some of the first websites I came across.....

2008-11-19T15:34:00.000-06:00

http://dermatology.cdlib.org/131/cases/NYUcases/111505_8.html

http://images.google.com/imgres?imgurl=http://www.scleroderma.org/images/medicalimages/2004/OralDentalFacial/Case2.jpg&imgrefurl=http://www.scleroderma.org/medical/facial_articles/Shulman_2000.shtm&usg=__il9SCHqhfpG3e67eUnWwWgKZJUs=&h=453&w=200&sz=20&hl=en&start=3&um=1&tbnid=caUpounGj60_bM:&tbnh=127&tbnw=56&prev=/images%3Fq%3Dlinear%2Bscleroderma%26um%3D1%26hl%3Den%26rlz%3D1T4ADBS_enUS267US267%26sa%3DN

http://www.jrheum.com/subscribers/03/12/correspondence.html

Psalm 139:14

2008-11-19T15:28:00.000-06:00

I praise You because I am fearfully and wonderfully made; Your works are wonderful, I know that full well.

1st Doctors Visit

2008-11-19T14:58:00.000-06:00

The first visit we made was to Kealy's pediatrician on 8/20/08. She has been a marvelous help to our family through the last several years. She looked at it and had never seen anything like it. And was quick to refer us on to a dermatologist. It took almost a month to get into the dermatologist. In between time, myself and a co-worker are looking up on the Internet: hair loss and indention in head, and googling any like phrases - scanning through images..........that's when she came across it, it was a busy day when she mentioned it, so when I got around to looking at the images I just saw the ones showing the hands (which I thought that's not what Kealy has, it's affecting her head). Later I came across what she had found, I just didn't at the first time around link to the site and see the 'rest of the story'. I came across the en coup de sabre images and pretty much had a gut feeling that was it. For those mom's out there - yes I cried.

How did it start?

2008-11-19T14:05:00.000-06:00

How did the LS start? Not sure if we really know. About the middle of 5th grade, say January 2007 - she started having just a small area of hair loss. The doctors and I attributed this to medications she was on at that time (lithium and seroquel) and so she was removed from those medication's - which was a good thing. The hair loss stayed the same the rest of the year and through 6th grade, so basically for a year and a half no real changes - just an area about the size of quarter.

But then during the summer of 2008 it started falling out. And now that we think back, during May she also started having headaches and started the girly thing (if you know what I mean). I am sure she is going to say MOM if she reads that - but I think it is important.........possibly a catalyst to the start of the LS. Some of the other stories I have read have had this same thought. But no one seems to know what causes Scleroderma of any type.

At the first of June 2008 we noticed just a slight indention in the hair loss area, about the size of a finger tip. We asked one of her doctors about it, they thought it could just be a normal thing. We just kind of disregarded it....for a while. But by the first of August a slight indention could be seen on her forehead. That's when the doctors appointments start!

Breif mention...

2008-11-19T13:55:00.000-06:00

I only mention the ADHD and Mood Disorder lightly, they in themselves are their own story/journey and the focus of this blog is to bring some comfort or knowledge to someone out there like myself that has been searching the internet for information on Linear Scleroderma / En Coup De Sabre / Romberg Syndrome. Kealy does take Focalin and Abilify that manages everything well - we have yet to see how or if there will be any problems with treatment for the LS along with these medications.

Her Beginnings

2008-11-19T13:36:00.000-06:00

KealyOna came to live with us at 5 weeks old, right around Christmas time. I picked her up from her previous foster mother at Wal-Mart........so she is my gift from God. Thats what I tell her. We gave her the middle name Joy because she is a joy, and it was the time of year and I always thought the name had a cool motto: J-Jesus; O-for nothing, zero and Y-You. Nothing between Jesus and her. Finally by the age of 2 her adoption was able to be finalized!

She proved to be a smart little girl, talking early, working the computer (using the mouse and putting her own DVD programs in/out) and writting a page full of little '0' and '/' across the lines of papers. And she loved to line up domino's.

Preschool started, and by the time 2nd grade started we were looking for help. Kealy ended up having ADHD and Mood Disorder (this took time to figure out). Finally by the 6th grade we were on medications that truly made a difference. And with the help of 2 doctors she likes progress has been made. Everyone that meets Kealy usually remembers her - and now that is not because of trouble, but because of her bubblely helpful loving personality.