A definition of Juvenile Scleroderma in simple, easy to understand language

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Mother and Daughter’s Journey -
Amber’s Stem Cell Transplant

Amber is a 15-year-old teenager who wants to hang out at the malls with friends. She has a whole new interest in music, clothing, makeup, and boys. She wants to be in school everyday talking and chatting with her schoolmates. There is only one thing wrong. Amber is sick with *MCTD. She is a teen trapped in the body of a 70 year old. Her knees, hands, back, arms and ankles are always in pain from arthritis. Her eyes and skin cannot handle the sun. Her teeth are yellowing and her gums are receding. She can't walk ten steps without losing her breath. Her skin is so tight on her hands that her fingers are curling and her legs hurt because the skin is so thick.

She sees so many doctors, friends and family can no longer keep up. Her list of medication is longer than both her grandparents put together. She was taking naproxen for arthritis, adalat for Raynauds, synthroid for her damaged thyroid due to meds, valporic acid for seizures, celexa for depression, prevacid for esophagus damage, domperidone for motility, a laxative to help keep her regular due to meds, ventolin, both discus and mask, to help her catch her breath, and imuran to slow the progression of scarring.

She has been through pulse cyclophosphamide. It made her so sick that she was in the hospital 4 days at a time twice a month. It was getting to be too toxic for her. It didn’t work and so to her it was all for nothing. Prednisone, well there is a drug that Amber hates very much. She has been on it for the last 3 years with doses ranging from 60 mg- 5mg, up and down all the time. This made her swell up so much. Her face was so swollen it hurt and her feet hardly fit in shoes anymore. She was also on bi-weekly pulse steroids, taking 1000mg in an IV. This made her hurt so badly that they had to give her liquid codeine every 4 hours. This scared me. I did not want Amber on the codeine so the pulse steroid treatment was stopped. There were also weekly injections of methotrexate. The methotrexate did nothing for Amber except to give her a rash all over her face. All if these treatments needed needles and now Amber is terrified of needles. Her emotions went from sadness to fear to rage. There was a lot of anxiety to deal with. Amber has a good counselor that helped Amber learn to deal with and control the situation.

Unfortunately, the illness was misdiagnosed for 5 years, leaving severe scarring in Amber's lungs. For the last 3 years the doctors have been trying to get Ambers illness under control. The worse damage has been to hers lungs. She has less then 40% of her lung capacity left. One lung is worse. All of this damage is irreversible. Though they have managed to slow it down a little, they haven’t been able to stop it. She can't take a nice deep breath. She coughs when trying to use her inhaler. She cannot walk through the mall and it is hard for her to dress without losing her breath. Even talking to her friends causes Amber to lose her breath. This makes her very tired all the time, unable to be a kid!!!

We recently saw her pediatric rheumy and after he examined the tests he told us about a procedure called stem cell transplant. He said that we needed to do it as quickly as possible so that we could maintain the quality of Amber’s life. He also told us that left the way it is Amber would eventually have to have a lung and heart transplant. This scared me so much to hear this. He then sent us to an adult rheumy who specializes in scleroderma and has done a stem cell transplant on a patient. We went to see him and he agreed that Amber would be a great candidate for a stem cell transplant. He told us of the patient he did the transplant on. It has been 3 years and the patient is still in remission. This gave both Amber and I hope. We talked and both agreed that the stem cell transplant is what we would try next.

The procedure was done at Toronto's Sick Kids. Feb. 4th we had a videoconference with the doctor, his nurse, and a social worker. Dr Doyle gave us information about the procedure. This was the first time this procedure was done in Ontario on a child. He explained that there was a 50/50 chance that there would be some kind of improvement and that it could be temporary. If improvement is seen for 2 years, odds are that it will continue to get better. We are nervous and scared but if this works the doors of the world will open for Amber. If not we will deal with whatever happens. What I do know is that without this treatment, there is nothing that will stop this illness from taking over first Amber's lungs and then her heart. I like the 50/50 chance better then watching her go through this illness.

She would be 4 weeks in and out of hospital before transplant, 5 weeks in hospital, and 4 weeks after as an outpatient again. So we were to leave Feb 21 and be back around May. When we get home the cancer center here will continue to care for her as an outpatient.

He also told us about all the medications that she would have to take. There was neupogen (GCSF) to make stem cell counts go up, thymoglobulin (anti-T cell antibody) to destroy the T-cells, cyclophosphamide, septra, penicillin, antifungals, antibiotics, and nausea medications. There are others too. I would write them out but I don’t know how to spell them.

The procedure was described. Amber’s port would be changed into 2 central lines, which will be used to access her blood and t-cells. Then 2 days of cyclophosphamide. After this, neupogen is given for two weeks as an outpatient to increase stem cell counts. They start collection when the counts have risen. Then they will hook up the central lines to an apheresis machine for 4-6 hours for 2-3 days. With this she may need to get a transfusion for platelets. Calcium levels may drop during this and may cause seizures. All of her other meds will also be affected. They need to collect 10,000,000 stem cells per kg. Fifty percent of them will be lost. Then she will stop imuran. She will have to get an Echo PFT (pulmonary function test) and GFR (kidney test). Then the transplant began. It starts with a high dose cyclophosphamide for 4 days and thymoglobulin for 3 days to destroy the immune system. They wait 8 hours and then transplant Amber’s harvested t-cells.

She will then be in isolation, allowed only 2 adult visitors, to protect her from infections. A gown will have to be worn and hands washed very well. Food will have to be thoroughly cooked, no fresh fruits or veggies. She will probably be fed through an IV. She will be nauseated and may get painful mouth sores. She may also have sores in her stomach. They treat this with morphine. She will be most susceptible of infections from the gut. Almost everyone gets a fever. The biggest risk is infection, but 97% of kids do well with antibiotics and antifungals. After 10-14 days the white blood cells will show again. About 14 days after the transplant the platelets will start to be produced. They will continue to increase until Amber can be released. She’ll be monitored closely as an outpatient.

Septra or pentamine will be taken for 6 months after to prevent pneumonia; penicillin is given to prevent pneumocous infection for 1 year. There will be a 50% chance of getting shingles for 2 years after the transplant. There is a 5% chance of getting cancer and a very high risk of infertility from the cyclophosphamide. Because of the damage that has already been done, even with remission she will be at a very high risk of having early heart and lung disease in her early adult years. We agreed to send all data to central registry and to share any information that may be helpful for other children like Amber.

The procedure was begun as planned. There were only a few minor differences. First Amber only needed the G-CSF for 3 days. Her WBC (white blood count) went from 0.8 to 28 with the norm being 4.0-11.0. Amber lost all of her hair and was very sad and upset about that. We made a game of it, using the hair for beards and moustaches. Boy, did we ever laugh! It was truly amazing when they harvested the stem cells. They hooked her up to her double lumina, one line for going into the machine, the other going back into Amber. I watched what the machine does. It takes the blood from Amber sends it through a lot of tubing into the machine, which spins it, and every now and then it harvests the stem cells and hemoglobin. You can actually see the stem cells separate! Now that that was done they began killing her old immune system. They used large doses of chemo and thymoglobulin. This is a very harsh drug and it gave Amber a lot of breathing problems. They also give her a mixture of meds that are used on all bone marrow and stem cell transplants; acyclovir for sores such as the herpes, dexamethasone a steroid, fluconazole for fungus which is a very common factor in these kids and is usually one of the things that causes fevers and such. Demerol was given to help her body relax so it wouldn’t reject the thymoglobulin. Amber really liked this one because she felt absolutely no pain for the first time since she was six years old. They gave her adivan, ondosetron and gravol. They gave her benadryl whenever she had breathing problems. She gained over 2 lbs overnight from fluid retention so they gave her a diuretic. Amber was beaten up from the inside after 3 days of thymoglobulin and an extra day of chemo. They had to stop the naproxen for Amber's arthritis and put her on morphine because her platelet count was too low. They had to give her a few blood transfusions to bring them up. She looked good after that. Her oxygen saturations came up to a 100%, something that has never happened before. This lasted for the day and then they went down into the 60's. They got her back up to the 80's with oxygen. Man, I was scared but always stayed strong for her and always insisted on her smiling everyday.

The day they gave Amber back her stem cells they called Day 0. The stem cells came in 3 huge syringes, which they gave through IV. A few days later Amber started to get a fever and they started her on all the preventative meds. They checked her for fungus but it all came back fine. Amber’s blood counts all dropped down well below the normal levels. Well these did not stay down long and her counts climbed quite well. They told us she would be in the hospital at least 4 weeks after the transplant. Well did Amber ever prove them wrong! Amber did so well that after only13 days they were telling her she was ready to get out of the hospital. It took a few days for all the doctors to see her and for some tests. Amber also decided to help research a new breathing test that will help eliminate having to get so many CT scans. And then she was a free bird.

Amber was a true trooper through this all and she smiled everyday. The big factor that got her out early is that she never stopped eating or drinking even though they were giving her nutrients, vitamins and a lipid fatty food source. Now Amber's only job is to drink 2.5 liters a day and to get plenty of rest. She is happy to be home and I know she will have a much more speedy recovery here. She will be on Penicillin for 1 year and septra for 6 months. After that she has to go and get immunized all over again but I am leery of that. Why mess with this new immune system?

It was a journey and we flew through it in the clouds amongst all the prayers for those who care. My daughter Amber Mae Woods is a HERO. She fought a battle and she won. She is also my HERO and I am so proud to be her Mum.

*MCTD: mixed connective tissue disease, a disease characterized by features of lupus, scleroderma, and myositis.

Jemz New Juvenile Scleroderma Website

Though I get tired a lot and am in pain most of the time, I try to do stuff that I like doing but I can't do sports or anything like that anymore. I used to play football before I became really ill but now I can't because I end up in pain and out of breath. I have to take lots of tablets as well as the stuff for pain which I take most days, and I have to have a blood test every month. I don't really let anything bother me anymore, when I was younger I used to cry all the time because I was scared I was going to die and that sort of thing. But I'm not really bothered now I just act as normal as I can, my Mum has always brought me up to be positive, not to worry about things I can do nothing about and as she says there are always people worse off than you aren't there. I look at myself as being unique one in a million.

I have created a juvenile scleroderma webpage to help spread awareness and express how I feel and what I go through with having juvenile scleroderma. Please take a look at my website at  Jemz Page 

Thanks!

~Jemz
United Kingdom

Juvenile Scleroderma Couldn't Stop Gymnastics Champ!

Marie Lucas, 14 year old 8th grader won first place in Level 7 competing in the Washington State gymnastics championship.

Marie's doctor diagnosed her with Juvenile Scleroderma. She fought back! She continued going to the gym, even if all she could do was stretch or ride a stationary bike.

This is her third state championship for the fourth-year gymnast.

Marie Lucas is a champion again!

Hilary Donates to help other kids in need!

Hilary Abigail Morrissey is a girl who inspires hope in the hearts of those who have none. Hilary, who has scleroderma, a swallowing disorder, and acral bone dysplasia of the hands and feet, knows what it is like to feel discouraged.

She also knows that every child is special despite challenges they may face. Rising above her difficulties to model true beauty, Hilary will be giving the gift of her own hair to Wigs for Kids, and organization that helps children who have lost hair through illness. Though she is only ten, Hilary has wisdom beyond her years and a heart that throbs for others.

"I want to donate my hair because I know how it feels to be different," Hilary says. "It is a good thing to do to donate your hair to make someone else feel happy about themselves when they are going through health problems. I feel really, really good inside being able to help another person. My life does have purpose!

Mother's Day 2003 was on Sunday, May 11th which was also my 13th birthday. My mom has multiple sclerosis which is getting worse and she was having more health problems, so I cut off my hair so she would not have to french braid or fix my hair everyday. I thought she could use a vacation from always helping me with my hair, plus by donating my hair again I would also be helping a child feel better about what they looked like.  So I felt like I was helping two people at one time.

Since November 1st, 2000 I have donated 35 inches of my hair to Wigs for Kids. It is my hope that others may donate their own long hair to Wigs for Kids or to Locks for Love to help people of all ages feel better about themselves when they may not feel well or may feel different. I know what it feels like to look different.

I am growing my hair out again and hope to make a third hair donation sometime in high school.

Hilary always had the passion to help others. She loves donating her hair to Wigs for Kids and Locks of Love. Hilary needed to come up with a 12 month Girl Scout Project to earn her Girl Scout Silver Award. She wanted to continue to help others by donating her hair and at the same time spread awareness about juvenile scleroderma. So, Hilary, decided she would start up a hair drive in her community. She realized why stop at our local community. Why not expand it to other areas of the country. Hilary would like to challenge others to donate their hair to Wigs for Kids or Locks of Love. To read more about Hilarys project check out her website Small Hands/Big Hearts.

Brenda follows her Dreams!

I am 13 and I was diagnosed with linear scleroderma when I was 2 years old. I hope some day I will not have to take shots every week. I play like no other kid, so thats cool. I like to play basketball, and I love to write story's. I hope I grow up and follow my dreams.
~ Brenda


For more information on Juvenile Scleroderma, contact:

Juvenile Scleroderma Network, Inc.
1204 W. 13th Street, San Pedro, CA 90731

Tel: (310)519-9511 (Pacific Time)
24 Hour Support Line: 1-866-338-5892 (toll-free)

Speak to another JSD parent for emotional and logistical support provided by home-based JSD volunteers. For medical advice, please contact your child's physician.

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