“What is Scleroderma? How you found out about her condition? Can it be cured?“
Most common questions I get whenever my friends or followers know about my daughter’s condition. Penning this down on my blog and the journey of fighting this battle, letting my girl know she has come a long way, she’s doing it very well, and we will overcome it as a family.
What is Scleroderma?
Scleroderma is an autoimmune connective tissue and rheumatic disease that causes inflammation in the skin and other areas of the body. When an immune response tricks tissues into thinking they are injured, it causes inflammation, and the body makes too much collagen, leading to scleroderma. (from google)
Because if it attacks, her affected skin becomes hardened and the hardening part will affect her growth, especially since she is still at her growing age.
Although her arm can see a little unevenly, and at some angles, it looks like she has a muscle, you wouldn’t know it’s Scleroderma unless you touch and feel it.
How do we find out?
Around Late 2017, one day she complained about her arm getting 1 patch of redness and is in pain. when I touch it, it feels warm. So I observed for the next few days, the affected part wasn’t painful and was warm. I also monitor if it’s growing in size.
1-2 weeks later, she was complaining about tummyache, which I thought she might have appendicitis, so faster brought her to KKH A&E. Luckily, it was just a normal stomach virus. Since I was already at KKH A&E, I asked the Dr to see her arm. The A&E Dr was very nice, and straight away arranged an X-ray, but the results were uncertain. So they arranged a specialist appointment for her under Rheumatology ＆ Immunology.
Can It Be Cure?
Currently, there is no medication or way to cure Scleroderma. What we are doing now is to prevent any new symptoms and whatever the conditions may bring.
The only thing we really feel thankful for is she got diagnosed early, so her growth wasn’t badly affected.
2017 – The Journey Of Testing & Diagnosis
During the 1st visit, they informed me about the possible diagnosis, test and biopsy to do before they can 100% confirm and proceed to the treatment.
Even as a nurse with medical knowledge, it’s still not an easy journey for me and Yi He. Especially when she is only 6 years old and I was pregnant with didi. (But Didi’s arrival was our biggest pillar of support and her joy at that point in time.)
After lots of tests and monthly visits to the Dr, they diagnosed her with Scleroderma and refer to Prof Tash who is an expert in this field. To this day, I’m very thankful and appreciative to the KKH team for their expertise and no time to waste (because I’m referred directly from KKH so she’s under subsidy) in getting her diagnosed and treated because it’s a time-sensitive condition.
In less than a year, the condition also affected her right fingers.
Jan 2019 – Her 1st Admission
They admitted her due to suspecting either fat necrosis or deep morphea at both of her cheeks. We can’t have a concrete diagnosis because is too deep inside, ultrasound can’t reach and we don’t want to do a biopsy on her face. So we admitted her for methylprednisolone treatment.
Before this admission, she has already started daily prednisolone and once-a-week methotrexate injection. Giving her weekly injection was torturing because it was painful for her and the side effects of the drugs (vomiting and headache) make her feel terrible. Most of the time she will start vomiting even before the injection. Prednisolone also gives her a chubby face which we keep encouraging her, telling her it’s ok and she looks cute.
In 2021, she stops her weekly injection because she needs to move on to the other medication. And thankfully she’s happy with the oral medication.
Fast Forward to 2023.
It has been almost 6 years since she has been taking medicine (currently taking Cellcept) and during one of the checkups in Dec 2022; the Dr suspected her to (may) have En Coup De Sabre.
“The scleroderma en coup de sabre is a variant of linear or localized scleroderma that occurs preferentially in children. It may involve all of the hemiface, extending from the scalp to the nose, lips and tongue. Its etiology is unknown, but probably it is of autoimmune nature.”
I broke down again. At first Yi He doesn’t know exactly what is happening, but when she knows that she needs to do a series of tests, including an MRI scan and needs to be admitted 2 weekly for medication infusion, she also cried on the spot. I told her it was okay, we are all helping her, and we got medicine for her.
But when I went back home, I cried my lungs out on that day. This time round was as bad as previously. I pick myself up quickly the next day. I can’t remember how many days, or nights, I cried when she was 1st diagnosed. My hubby was very strong. He said if anything happens, there is still plastic surgery, Botox or fillers for her. Apparently, he did all the research work before we reached home.
Even though it’s just suspecting, but we are not taking any chance and went ahead with the treatment. It was not cheap. The drug cost $8k plus, and there is no government subsidy on the drug. Thankfully, our insurance provides us with coverage. So at the time of writing, she already completed her 1 first month of 2 weekly medicine infusions (Abatacept) and also started steroid medication again.
Coping with it
It’s a long and painful journey of diagnosing Scleroderma because it’s a rare condition and it doesn’t have any symptoms at all. Her arm’s redness just feels like an insect bite, although a bit hard but not swollen and not itchy. The pain and warmth when touched only happened once.
I couldn’t find any suitable support group for kids who had Scleroderma in SG but found one from overseas. She also made a friend from the USA who has the same age and condition as her. Mental support has been great so far and the KKH team that has been taking of her is also great. All I can say is we are very blessed despite all these difficulties.
Although there is no specific diet for her condition, she’s taking medication daily and with all the strong medication she’s currently taking, cooking home-cooked food almost daily becomes one of my motivations. We also have to ensure she keeps herself active, also to make sure her joints are not experiencing any stiffness and preventing it too.
If you have any more questions regarding her condition or if your child also has just been diagnosed with the same condition, I’m happy to connect. Hopefully, I do not wish any child or parent to go through this.