What is scleroderma? Author whose diagnosis left her with claw-like hands given years to live
A young woman diagnosed with a rare disease that left her with “claw-like” hands and extreme pain was given only 10 to 15 years to live at the age of 27.
Claudia Sultan, an author and project consultant, sat at her desk a decade ago and noticed her fingers turning blue and “icy cold.”
Claudia, 37, from Wollongong, New South Wales, assumed she was just feeling the effects of winter – but now knows it was the first sign of a much more sinister diagnosis.
Claudia suffered from scleroderma, a rare autoimmune disease that floods the body with too much collagen and leads to hardened skin, tighter connective tissue and constant pain.
“I had just gone outside for a bit, pulled back in and noticed my fingers were throbbing in pain and turning from blue to red and then white,” Claudia told FEMAIL.
Claudia Sultan was diagnosed in 2013 with scleroderma – an autoimmune and rheumatic disease in which the body produces too much collagen
The unusual symptom lasted for two weeks before she visited the doctor who misdiagnosed her with Raynaud’s disease – a condition that causes parts of the body to feel numb and cold.
Over the next few weeks, Claudia found herself unable to move her elbows, struggling to raise her arms above her head, and unable to even make a cup of tea. She also fell down the stairs when she got off the train because her knees were ‘cramped’.
The chain of events led Claudia to her eventual diagnosis of scleroderma can harden tissue around vital organs, such as the lungs, heart, and kidneys.
Prior to the diagnosis, she noticed her fingers turning an icy blue and throbbing in pain. In the weeks that followed, she could no longer move her elbows
Claudia was told that life expectancy for patients suffering from the disease is 10 to 15 years from diagnosis as the condition begins to harden essential organs, turning her world upside down.
Claudia has claw-like fingers (pictured)
“My mom went with me to the doctor that day and I was in complete shock,” she said.
“Being told how long I might have to live was very damaging and completely broke me. I had also never heard of scleroderma and immediately googled it.’
As her body produced an influx of collagen, Claudia’s face felt increasingly toned and firm, preventing wrinkles from forming.
“I wish I had wrinkles instead of scleroderma,” she said.
“I felt trapped in my body and disconnected from myself. It’s like I woke up one day and was a completely different person.’
The cause of the disease is unknown and there is no cure, despite 6,000 Aussies being diagnosed each year.
What is Scleroderma?
Systemic sclerosis (SS), also known as scleroderma, is an autoimmune disease that causes atypical growths of connective tissue.
It results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body’s connective tissue, including your skin.
Connective tissue gives your tissues (organs and muscles) strength and shape. With SS, your tissue becomes thick and stiff, leading to swelling and pain.
It also causes changes in the texture and appearance of your skin due to increased collagen production. Collagen is a component of connective tissue and hardens in this disease.
It can affect your blood vessels, muscles, heart, digestive system, lungs and kidneys.
The symptoms and severity of the condition vary from person to person based on the systems and organs involved.
Doctors aren’t exactly sure what triggers this process, but the body’s immune system seems to play a role. Most likely, scleroderma is caused by a combination of factors, including problems with the immune system, genetics, and environmental factors.
It is much more common in women than in men.
Claudia was prescribed medication to reduce symptoms and control the disease, but “nothing worked” and was told to “live with it.”
“My skin started to get really hard and thick and it physically changed my hands — my fingers now curled up like claws,” said Claudia.
But she didn’t give up hope.
A colleague advised a specialist at St Vincent’s Hospital in Sydney, who claimed he could “reverse the condition by 50 percent” through a stem cell transplant.
She decided to go ahead with the treatment – a process in which her own stem cells are collected, treated and put back into the body, while removing any damaged cells.
“I went through a period of self-loathing and couldn’t accept that fate. I always asked myself “why me?” I didn’t want to believe it,” she said.
Although the stem cell transplant was painful, it was 50 percent successful. This meant that her symptoms were reduced by half.
Both before and after the transplant, she underwent six sessions of chemotherapy with various side effects, including hair loss, insomnia, infertility, nausea, and weight loss.
“It took me about a year to get back on my feet,” Claudia said.
Unfortunately, Claudia relapsed in 2018 and the disease focused on her lungs. As a result, she now has a persistent and chronic cough.
“I felt trapped in my body and disconnected from myself. It’s like I woke up one day and was a completely different person,” said Claudia
Claudia said she had to adapt to her “new way of life” by learning to accept herself and her challenges.
“I had to face my insecurities and stop doubting and comparing myself to others. I had to reject negative thoughts and find happiness in the smallest things. There was a lot of trial and error,” she said.
Now it comes down to managing the condition as best as possible.
Claudia shared her experience in her memoir ‘A Life With Uncertainty’.
You can buy the book here.