The Face of Scleroderma

My first post on Scleroderma was an attempt to raise awareness for this unfamiliar killer.  This post will put a name and a face on this devastating disease.  The name is Rachelle (Shelly) Sandra Hollander, and she was my mother.  She lived with this disease for twenty-six years.  It finally killed her on October 16, 2006.  Scleroderma is ruthless.  It is sadistic.  Worst of all, it destroys lives. 

Shelly was diagnosed with Systemic Scleroderma in the 1980's when she was in her early 40's.  The first symptom was a persistent cough and poor circulation in her extremities.  After several misdiagnoses, she was informed that she had the fatal form of the disease.  Shelly was given five years to live.  The doctors underestimated her strength, determination, and her will to live.  She faced every new obstacle with grace and courage.  And the obstacles were brutal.

As the years went by, the cough became much worse.  Shelly could barely laugh anymore because, when she did, she started coughing so badly that she could not catch her breath.  The skin on her face was beginning to get pulled tight, which, to her embarrassment, made her smile appear deformed.  The skin on her fingers and toes were being pulled so tightly that they began to bleed.  Her fingers became so deformed that she had difficulty doing a lot of the everyday things.  Shelly was no longer able to use a can opener to open the cat and dog food cans for her precious pets.  She could barely turn the pages of the magazines that she had loved to read.  She could not stand the cold weather anymore because of the poor circulation in her extremities.  All the while, the cough continued to get worse. 

Shelly was taking numerous medications each day.  There was Nexium for the persistent heartburn caused by the scarring of her esophagus.  There was Oxycodone for pain, Xanax for anxiety, prednisone (a steroid) for her breathing, Procardia (a calcium channel blocker) for pulmonary hypertension, and Albuterol for asthma-like symptoms.   These are just the ones that I know about.  I am pretty sure that there were more.   None of these were meant to cure her, or stop the disease from progressing.  They were all just supposed to help her get by.

Walking became more and more difficult with the progression of the disease due to the damage to her lungs.  A CT scan showed that my mother's lungs were as black and damaged as that of a life-long smoker, despite the fact that she had never smoked a cigarette in her life.  Shelly had more and more difficulty eating because her esophagus was narrowed due to the scar tissue caused by the scleroderma.  Her heart was also greatly damaged by the disease.  A few years before she died, Shelly's doctor suggested she go on the list for a heart/lung transplant.  She would not hear of it.  I am not really sure is she was against it for religious reasons, or if she was just getting tired of fighting.  But fight on she would, for several more years.

For a few years, towards the end of her battle, Shelly would have to be connected to an oxygen machine when she was at home.  After a while she could no longer breathe without it, and had to carry a portable machine everywhere she went.  She spent more and more time in bed, and less up and about.  Perhaps the worst thing that Shelly had to endure was the pain from her fingers and feet.  The skin was being pulled so tight that it was cracking.  Her fingers were raw.  You could barely touch her hand without her recoiling them in agony.  Creams, lotions, prescription medications - none could help alleviate the pain.  Eventually we had a twice-daily ritual of wrapping her fingers in bandaids and gauze pads.  Having them covered seemed to offer some slight relief.  There are even more symptoms that I can tell you about.  Here is a list of possible symptoms that a Scleroderma patient may experience, according to the Cleveland Clinic.   My mother had them all.

·         Swelling of the hands and feet

·         Red spots on the skin

·         Excessive calcium deposition in the skin

·         Joint contractures (rigidity)

·         Tight, mask-like facial skin

·         Ulcerations on the fingertips and toes

·         Pain and stiffness in the joints

·         Persistent cough

·         Shortness of breath

·         Heartburn

·         Difficulty swallowing

·         Digestive and gastrointestinal problems

·         Constipation

·         Weight loss

·         Fatigue

·         Hair loss

·         Possibly Raynaud's Disease (narrowing of the blood vessels)

·         Possibly Sjogren's Syndrome (chronic dryness of the eyes and mouth)

Then there were the side effects of all of the medications that she was on.  The worst side effect was a result of the steroid Prednisone that she was taking to help her breathing.  Prednisone, if taken for an extended period of time, can weaken the bones.  A few weeks before my mom passed away she complained of having an even harder time breathing than usual.  She was in terrible pain and could not get any relief, even when she was completely immobile in bed.  We took her to Stony Brook University Hospital on Long Island and it was discovered that her cough had caused a few of her ribs to collapse, leaving them pressing against her lungs.  The prednisone was to blame for making her ribs so weak.  Shelly would leave the hospital after a few days with nothing more able to be done other than more Oxy. 

Now, I don't want to give the impression that, with all of this physical and mental pain caused by Scleroderma, that life was not worth living for my mom.  She would have told you that she still cherished life.  She had so much to live for.  And don't forget, Shelly was a fighter.  She had two daughters and four grandsons to love.  And she had lessons to teach.  She taught me what real courage is.  She taught me never to take anything for granted.  And she taught me to laugh, despite everything.  So we tried to joke about Scleroderma as much as we could.  I would tell her that she would make the perfect criminal since she no longer had fingerprints.  She would tell me that she would always look years younger than her actual age because the skin tightening on her face smoothed out any wrinkles that she may have had otherwise.   Some days we were able to joke about the disease.  Most often, not.

Shelly died on October 16 at around 6:00 AM.  I got the call from the hospital while I was taking a shower.  For the past two days I had been working with my sister to have my mother brought home with a full-time nurse.  I knew that she wanted to be home, although she was almost completely out of it by this time.  She had been in and out of the hospital for months by this time.  I did not want her to die alone, but she did.  We could not get her home in time.  That is one of my greatest regrets.  Of course, I visited her at the hospital every day, but that does not even ease the pain of her being all alone in a cold hospital room when she passed.  The actual cause of my mother's death was listed as Pulmonary Hypertension, but the underlying cause was Scleroderma.

I write this blog in the hope that you will want to help by donating to this cause.  In the hope that it is more real to you now that the disease has a name - and a face.  The face of Shelly Hollander - my mother, and an inspiration to us all.  Please visit http://www.scleroderma.org/ to help eradicate this unfamiliar killer.