10 | Our Fighter
Shirley Choi
About Scleroderma
Scleroderma is an autoimmune disease that is characterised by the hardening and tightening of the skin. It is a disease of vascular and connective tissue where the skin and many other parts of the body can become thick and hard. There are two kinds of scleroderma: localised and systemic. The localised type tends to be milder and affect mainly the skin and subcutaneous tissue, while the systemic type may affect the internal organs as well as the skin and may be life-threatening. With it being an extremely rare disease, scientists are still unable to provide an answer to its cause of collagen overproduction.
It has been more than two decades since Shirley, at the age of thirteen, was diagnosed with frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre), which is a kind of localised scleroderma. Since then, scleroderma has drastically flipped her world upside down. For the first few days when she experienced symptoms, there were linear red patches on her forehead and thickening of skin like scales on fish. Gradually, the symptoms spread up to the scalp and down to her nose, causing the death of some follicles and a linear band of atrophy and a furrow in the skin and cartilage. To a teen girl who usually cared much about her appearance, not only did this condition affect Shirley’s physical health, but it also greatly damaged her self-esteem and confidence at that time.
About Scleroderma
Cause
Autoimmune disease; immune system attacking the connective tissue under the skin and around internal organs and blood vessels
Inheritance
Possible but rare
Occurrence rate
3-5 per 100,000
Symptoms
Hardening and tightening of patches of skin, blue and numb fingers or toes in cold temperatures
Effects
Affects their physical appearance, and damages internal organ functions, such as lungs, intestines and esophagus
Treatment
Supportive treatment such as immunosuppressing drugs and corticosteroids
"Healthcare services should not be evaluated in terms of cost effectiveness; rare disease patients deserve the right to receive equal medical resources and necessary treatments.
Overcoming hardships
Having to bear with this condition for more than twenty years, Shirley still has not managed to find another person that shares the same disease as hers. This disease has been negatively affecting her daily lives in terms of job seeking, social interaction and even romantic relationships. Due to the fact that scleroderma is incurable and the affected skin and follicles cannot turn back to normal, she felt as if she suffered from an incurable deadly disease. In an attempt to keep an appearance that looks ordinary, she has undergone quite a lot of plastic surgeries, but at the same time, she has experienced countless discrimination and taunts. Apart from that, she received chemotherapy because of a kind of rare cancer of the lymphatic system – Burkitt’s lymphoma, which is again a deadly autoimmune disease. One morning in 2018, she had a haemorrhagic stroke and nearly lost her life because of the uncommon cavernous malformation in her brainstem. When all of you readers are wondering how she managed to go through all these misfortunes, she has made it, again and again. Despite all these hardships, she was able to overcome what was thrown at her. Her faith in God and her life experiences have crafted her into a stronger person, and made her learn to cherish whatever she still has and realise the meaning and value of life in her own unique way. She is currently actively participating in voluntary work, sharing her experience about how she has coexisted with her diseases and contributing to society by helping patients and the disabled.
Words from God
But he said to me, “My grace is sufficient for you, for my power is made perfect in weakness.” Therefore I will boast all the more gladly about my weaknesses, so that Christ’s power may rest on me. (2 Corinthians 12:9)
Words to the government
Up till now, Shirley has not found the medication that cures scleroderma. Without the suitable treatment plans available, her condition continues to worsen. She thinks that even though multiple plastic surgeries could help her try to ‘look normal’, it is not pin-pointing to the root cause of the disease and the problem. While she understands that there are limited government resources, it is her hope that the government will be willing to set up a rare disease clinic to gather all the patients with rare diseases together and concentrate resources on conducting scientific researches through data collection and data analysis so that medical professionals could be trained to know how to have consultations with the patients with rare diseases. She emphasises that all patients should be treated equally and patients with rare diseases also deserve the right to receive attention from the government, no matter how rare the diseases are. She hopes that the government should not allocate resources from the business perspective only because life is invaluable and patients with rare diseases also deserve treatment like patients suffering from flu do. This way, no patients will be left behind.
‘I made it through again and again; God's grace is indeed sufficient. I hope someday I can find another scleroderma patient, so we can fight hand in hand against this disease.’