SCLERODERMA- your opinions please
Posted 05 November 2008 - 07:00 PM
I am Dr. P. Damayanti, gyenaecologist, Dr. Maheedhar, orthopaedician, parents of ashritha. We both are staying in a small town in andhra pradesh part of India. We have 2 lovely children, one daughter and one son. Son who is 9 years old doing 4th standard. My daughter who is very pretty, beautiful till the age of 61/2 years. At the age of 61/2 - 7 years. We have notice hyperpigmented area over left cheeck. We did not consider it seriously. Six months later she had fever with myocarditis, she was ill for 15 days. Her ANA is positive. rest of the blood reports are normal. With in a couple of months later she has been diagnosed as overlap syndrome. Over a period she has progressed to scleroderma alone. Treatment given is
- Oral steroids (Wysolone) for about one year. She developed cushingoid features. So on advice we have stoped steroids.
- Tab: Methotrexate 7.5 mg weekly for about nearly 4 years. At present she is not on methotrexate.
- At present she is on mycophenalate (Cellcept) M.M.F 250 mg 2 times daily since 9 months.
As per Rheumatologist her condition is worsened, so he started on steroid pulse therapy that is 625 mg of steroid for day 3 days in a month for 6 months.
With all this she does't seem to be alright.
Age is 11 years, weight is 23 kgs. At present she has the following problems
1) Inability to lift neck suddenly not all the time esp.. when she is getting of from chair. After some time settles down.
2) Indrawn mouth exposure of teeth and gums, can't open the mouth completely.
3) She can't turn the head back.
4) She can't lift hand , she uses left hand to lift the right hand to feed.
5) Elbow: She can't extend it completely.
6) Fingers: Sclerodactyly
7) She walks on toes.
8) She can't squat in bath room as well as on the floor.
9) She can't climb stairs
10) Reflux Oesophagitis
What she can do is
1) She can climb down the steps
2) She will sit on the chair with my help
3) She goes to school, in fact she is very good in studies
4) Restricted mobility because she gets tired very easily, so most of the free time she will watch T.V
She gets ulcers over the joints, the scab which forms is very thick does not fall easily.
I would say she is partially crippled.
I will enclose the photographs in my next mail.
Kindly give me your suggestions regarding it.
Dr. P. Damayanti, M/o. Ashritha
anybody with similar complaint ,improved after medication,prognosis,newer medication,prophylactic treatment ,STEM CELL TRANSPLANTATION PLEASE DISCUSS/REPLY.ANY OTHER INFORMATION PL INFORM.
Posted 05 November 2008 - 10:54 PM
Another useful page is the one on Emotional Adjustment and Scleroderma.
Please feel free to ask any and all questions that may arise and we will try to answer.
(Retired)ISN Sclero Forums Support Specialist
(Retired)ISN Sclero Forums UK Chat Host
International Scleroderma Network (ISN)
Posted 06 November 2008 - 06:41 AM
I am back,I have following questions
1 . painful ulcers at the tip of the digits. over the joints. 2.riflux 3. corns at theplantar surface of the foot 4 .any advance treatment like stemcell transplantation.5.anypossibulity for remmission since she is only 11yrs.
ashu 's mother
Posted 06 November 2008 - 08:33 AM
I can't give any advice on Stem cell transplant but wanted to welcome you. It sounds like your daughter needs some drastic measures taken. I sure hope someone chimes in from the USA.....there are several people that have had Stem cell transplants here and are doing OK now.
I have an son who was diagnosed with UCTD/sine Scleroderma when he was 18 years old. His issues were all internal and after 9 months was put on Plaquenil. He is doing fine now at age 20. I will keep you in my thoughts that your daughter gets the help she needs.
Take care, Everyone.
Posted 06 November 2008 - 10:41 AM
I'm sorry to hear of your daughter's illness. I would strongly recommend consultation with a scleroderma expert. You would need to make sure the center is able to treat children. Our worldwide listings for scleroderma experts is at: Worldwide Scleroderma Experts.
Here are some links related to your last post:
Stem Cell Transplants
Please give your daughter an extra warm hug, just from me!
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099
The most important thing in the world to know about scleroderma is sclero.org.
Posted 07 November 2008 - 06:23 PM
I recieved a stem cell transplant at Duke University Medical Center in March 2007. It was part of a clinical trial known as the SCOT study. I can tell you that the trial is looking for people in the early stages of the disease that have diffuse scleroderma. It is a random trial meaning if qualified for the trial, you are randomized into either the stem cell transplant arm or the cyclophosphamide arm. which require regular infusions. I can tell you the stem cell transplant has been very successful in my case. However, it is a difficult process and I am uncertain if the trial includes children. There are other trials going on both in the United States and elsewhere. Northwestern has a trial going on as well. The SCOT study involves both total body irradiation and chemo. Northwestern does not use any radiation. I would be happy to discuss my experiences further with you if you wish. I don't believe stem cell transplants are being offered for this disease outside a clinical study at the present time. Obviously a transplant should only be used for those who have a very aggressive disease process. Shelly's recommendation to consult an expert in this field is a good one.
Here is a list of current trials for which I am aware. Best of luck to you. I wish you and your daughter the very best.
Posted 07 November 2008 - 08:47 PM
Am so sorry to hear about your daughter and wish I had some answers for you. The links Shelley and Jefa left for you should help answer some questions.
I am not a doctor but do you do gentle passive range of motion to exercise her extremeties and she might actively be able to do some of them too while she is watching TV. Exercise is very important in preventing some skin and ligament tightening. Lotions for her skin would be soothing and maybe help to keep the skin more supple. Gloves or mittens may help to keep her hands warmer. Maybe your doctor and you can judge on what type of passive exercise she would be able to tolerate.
As far as medications, there are many but what will work better I do not know. Wish you well. Keep us posted.
With gentle thoughts.
Posted 08 November 2008 - 04:06 PM
My heart pours out to you and I wish you all the best. I hope you get some answers and some results soon!