Hard word – hard disease

Today is World Scleroderma Day, and on this day of June 29 1940, a talented Swiss-German Artist Paul Klee died of Scleroderma. Klee was diagnosed with Scleroderma in 1936, and with rapid advancement, sadly diffuse systemic sclerosis took away his last breath and his life.

Klee’s symptoms started with fatigue, a skin rash, shortness of breath on exertion, difficulty in swallowing and pain in the joints of his hands. As far as we know, Klee’s hands were not as drastically affected, even though both his organs and skin were involved.  The artist appeared to rise above his enduring pain to forge ahead with his passion for developing his artistic talent.

Opinion is that his works after his diagnosis of Scleroderma displayed a sense of sorrow and darkness, depicting his agony. One of the most significant pieces to demonstrate this is ‘Tod und Feuer’ (Death and Fire). If you look closely, you can see the actual words of ‘Tod’ (Death) spelled in the figures face. The red (Feuer) also suggest the inflammation and burning pain of Scleroderma. There is also a slight representation of a Scleroderma face, a very tight mouth, a thin (pinched) nose, with a pale hue almost representing shininess.

We know that Klee’s art was not well received by the Swiss authorities, and was often referred to as ‘degenerate art’, however to me it clearly shows his inner being, his humility and towards the end the sense of suffering he felt when Scleroderma took his life.

Just like any other disability, Scleroderma does not always appear the same in each and every patient. It does not mean that we will all have fatal consequences of the illness, like Klee, nor does it mean that we will all live our lives with the same symptoms. One thing is certain however, and that is the uncertainty and fear of where this illness might take you. So in honoring Klee, I’d like to share with you some of the common symptoms of Scleroderma and how it can present in some patients.

Scleroderma is a hard word. So often I am asked to repeat it when I say it, and more so I am asked to spell it. I always find it easier to break it down, Sclero – hard, Derma – skin. Almost all patients with Scleroderma will have thickening and hardening of the skin. Skin can appear shiny because it’s so tight, and movement of the affected area may be restricted. In addition, this can lead to skin ulcerations which again, may or may not lead to amputation of toes or fingers.

One of the earliest signs of Scleroderma is an increased response to cold temperatures or emotional distress. This can cause numbness, pain or color changes in the fingers or toes. An example of this one in regards to no-one is the same, is that Raynaud’s phenomenon does not affect me. I have never had the experience of my hands and feet changing color with extreme cold. If I had a dollar for every time someone asked me if I was cold, I’d be a rich woman!

Then there are the digestive issues. Scleroderma patients can be prone to acid reflux, which can damage the section of esophagus nearest to the stomach. Some people with Scleroderma may also have problems absorbing nutrients if their intestinal muscles aren’t moving food properly through the intestines.

And let’s not forget the emotional aspects. In the early stages of Scleroderma you have no idea of what will happen and how you will feel as each new day begins.  It is not a well known disease, nor is it greatly understood. Getting the right diagnosis can take several years, leaving you with many unanswered questions, feeling like you are living life on a roller-coaster.

Finally, but in no way the least important, is that Scleroderma can affect the function of the heart, lungs or kidneys. These can and often do become life-threatening.

Today, we remember, we celebrate, and we acknowledge all that have passed from and continue to endure Scleroderma. We are here to raise awareness, enable research and create a supportive environment for patients, family and friends with Scleroderma. It is not only a hard word, but it is a hard disease.

Today, I ask you to share my story, or a story on Scleroderma to help us raise awareness. We no longer want it to be a hard word. Let’s move into people’s vocabulary, and better yet into regular conversations among your family and friends. 

On the final note of Paul Klee, and the memory he created for us to celebrate World Scleroderma Day, I’d like to share the words on his tombstone which I find reflective and moving:

“I cannot be grasped in the here and now, For my dwelling place is as much among the dead, As the yet unborn, Slightly closer to the heart of creation than usual, But still not close enough.”