Scleroderma: An introduction

Welcome to ImperfectlyWhole. I’m a young woman living with Diffuse Scleroderma.

ImperfectlyWhole is a blog about scleroderma where I’ll be sharing my experiences living with the illness as well as some useful information regarding the management of it.

“Scleroderma, which means “hard skin,” is a rare disease that affects the skin and connective tissue (fibers that provide structure and support to the body). There are two types of scleroderma – localized and systemic (sometimes called systemic sclerosis).” Arthritis Foundation. https://www.arthritis.org/diseases/scleroderma .

Systemic scleroderma can be broken down into two main types: diffuse and limited. Cleveland Clinic.org https://my.clevelandclinic.org/health/diseases/8979-scleroderma-an-overview

The symptoms of this disease vary with each patient and consequently the treatments are not always the same for everyone.

There are numerous sites on the internet with information about scleroderma. I have put a few links below of some that I have visited.

An introduction: How did I get here

I was diagnosed with Diffuse Scleroderma in February 2018 at Groote Schuur Hospital in Cape Town. This is the kind of Scleroderma that is identified through symptoms of an acronym called CREST (Calcinosis, Raynaud’s Phenomenon, Sclerodactyly, Telangiectasis) – which I will write about in detail in the next blog posts.

In December, the previous year, my husband and I had travelled to Norway from Mpumalanga. We had experienced the harsh coldness of Norway, which was the type of weather we were not accustomed to. And when we returned the same month to Mpumalanga, we were greeted by the unbearable December heat. A few days upon our return, I experienced a prolonged swelling on my feet and I dismissed it as a combination of long flights and the experience of the two extreme weathers of Norway and Mpumalanga in one month. However, in January, when we travelled back to Cape Town, where we lived at the time, my feet had not stop swelling. I did not particularly experience pain, but discomfort whenever I walk.

We finally went to see a Doctor and after a quick assessment he mentioned that it could be water retention and prescribed water pills. A week into taking those pills, I started to feel ill. My days were filled with fatigue, light-headedness and constant vomiting; then I started to experience excruciating abdominal pain. Upon another consultation with the doctor he discontinued my use of one of the pills and offered me different ones.

During that time, the swelling had stopped and I assumed the problem was sorted and I was just now having a bad reaction to the medication. Another week would come and I would feel worse. On my own accord I decided to stop taking the pills, but I still did not feel better. I was getting worse and could not keep any food down as I ate. I purged everything from food to water. Sleeping became unbearable and almost an unattainable goal. Some days my husband would find me asleep on the bathroom floor, laying there, weak from all the purging and the excruciating abdominal pain.

One evening we decided that in the morning, we would drive to the emergency room at Groote Schuur Hospital.

The following morning, we arrived at the busy emergency room and I was sent  to a nurse who took my vitals and recorded all the symptoms that I had.

Moments later, we were sent to see a doctor who would draw some bloods for tests. In the doctor’s room, during a physical check of my body, I was asked all sorts of questions that were seemingly confusing to me; I asked myself “what do these questions have anything to do with my swelling feet? Or the abdominal pain and the vomiting?”

Dr: “Have you travelled recently? Are you sensitive to the sun? do you get burned easily and harshly? Have you noticed any changes in your hands during cold weathers?”

I was surprised and relieved, as someone sitting across a psychic listening to them profess exactly what has been happening in their life. I began answering all his questions, backing them up with back stories.

Me: “Yes, I had travelled recently”. “Yes, recently my chest area has burned badly and it looks weird”. “Oh my goodness yes, in July after work I was walking to the bus stop and my toes felt very cold and then painful. My fingers were feeling cold too and then turned pale yellow and after a few moments a bluish, purple colour and instant pain overcame them. I took a picture of them and sent it to my best friend and showed her”. I took my phone and showed the Dr the picture of my hands. He looked at it and proceeded to write some notes.

More bloods were drawn for more tests. And moments later I was told to wait in another room for a specialist who will talk to me. I later learnt that the specialist is called a Rheumatologist: “a specialist who has expertise in diagnosing and treating arthritis and other musculoskeletal conditions and autoimmune disease(s)” www.rheumatology.org.au

She confirmed the diagnosis after further examining my skin, the burns on my chest and my hands. She was glad that I had an early detection of the disease, this would help both me and her team in treating the illness effectively.

My early signs of scleroderma:

1. My skin had tightened around both my armpits, making it hard and painful to raise my arms in a full stretch.
2. I had a round patch of hard skin on my right arm.
3. The turning pale yellow, bluish-purple colour on my fingers and toes in cold weathers/areas.This is called Raynaud’s Phenomenon: “a disorder that causes decreased blood flow to the fingers. In some cases, it also causes less blood flow to the ears, toes, nipples, knees, or nose. Spasms of blood vessels happen in response to cold, stress, or emotional upset”. hopkinsmedicine.org
4. The swelling of my feet and slightly on my knees

Here is a list of helpful sites to learn more about Scleroderma:

1. The Scleroderma Foundation http://www.scleroderma.org/
2. The Scleroderma Research Foundation https://srfcure.org/
3. More Than Scleroderma https://www.morethanscleroderma.com/
4. Johns Hopkins Scleroderma Center https://www.hopkinsscleroderma.org/
5. Arthritis Foundation https://www.arthritis.org/diseases/scleroderma