Mom first became ill in the fall of 1998. She started to develop a lot of respiratory infections, acid reflux, fatigue, and shortness of breath. I was student teaching and preparing to graduate from college at the time. While I was concerned about her, I had no idea that these were the first symptoms of scleroderma. She told me later that she had also noticed some tingling in her fingers and some blue discoloration (Raynauds) when they were exposed to cold.

A chest x-ray by our family doctor revealed that Mom had a calcium deposit in her lung, so she was referred to a pulmonary specialist. The specialist told her in January, 1999, that blood tests had revealed that she had some sort of connective tissue disease. Scleroderma was one possibility that he mentioned. When Mom told me the news, I started to cry. I remembered from childhood what scleroderma was and what might happen to my mother. She assured me that I shouldn't worry and that everything would be all right. She secured an appointment with a rheumatologist for March 17, 1999.

Mom began to develop other symptoms as we waited for the March appointment. Her legs, feet, and hands swelled and ached, filling with fluid. Also, her shortness of breath became more pronounced. I started to read internet articles voraciously and became more and more convinced that she had scleroderma. When the day of the appointment arrived, Mom left work at noon to go to the rheumatologist's office. When she arrived, she was told that she did not have an appointment scheduled. All of her clinical information and lab reports had been sent, and she had even called to confirm the appointment. However, someone on the clerical staff had not written her name in the appointment book. She was sent away and told to come back in two weeks. When she returned, that appointment was also postponed. She did not see the rheumatologist until early April. The waiting and uncertainty had been devastating for her, and this mishandling of her appointments only deepened her anxiety and depression.

The rheumatologist first diagnosed her with CREST but insisted on a lung biopsy to make certain she had no lung involvement. Instead of taking into account her obvious symptoms of lung involvement, he tried to make the illness fit his diagnosis instead of diagnosing her symptoms as diffuse. The biopsy required five days of hospitalization and a month of recovery at home. The procedure took an extreme physical toll on my mother. She weakened considerably and lost forty pounds in two months. I don't think her body ever recovered from the effects of that biopsy.

When the rheumatologist pronounced the biopsy results inconclusive, we promptly found another rheumatologist and scheduled an appointment after Memorial Day. By now, my mother was so weak and short of breath that she had to ride a shuttle from the parking lot to the clinic's entrance. This second rheumatologist, competent but impersonal, diagnosed her correctly with diffuse scleroderma and started her on Cytoxan (cyclophosphamide) for her interstitial lung disease. Mom lost much of her hair (cytoxan is a form of chemotherapy), but her pulmonary functions stabilized and her skin softened slightly. She still had fits of coughing and was on a restricted diet, but she felt better and more optimistic than she had in months. She couldn't travel or work in her garden the way she liked to in the summer, but she continued to work full-time and to plan for the future. Her gastrointestinal tolerance improved to the point that she could eat Chinese food again (her favorite). Once again, I began to think of our future.

Suddenly, in September, Mom took a downhill turn. She became unable to eat again, and her blood pressure dropped so low that it wouldn't register on the monitor I had persuaded her to buy. She was hospitalized for severe dehydration, and we were told in general detail that the disease was affecting her liver. She was released from the hospital, but she could only manage to eat pudding, applesauce, mashed potatoes, or other soft, bland foods. She continued to lose weight and lung function, but her rheumatologist simply suggested that she "get lots of rest and drink Ensure." She visited her family doctor again, in effect, to plead for her life. He realized how ill she was and consulted with a team of doctors at a local hospital. They discovered that Mom had a pericardial effusion (fluid around the heart) and admitted her immediately into the cardiac care unit. Finally, she received the attention she desperately needed. An echocardiogram (ECG) revealed that she had pulmonary hypertension.

A respiratory specialist arranged to have her admitted to an experimental treatment program for pulmonary hypertension in which a new drug, Flolan, was released into the body 24 hours a day through a pump implanted in the abdomen. A new rheumatologist arranged for her to start taking gamma interferon for the scleroderma. They removed the fluid around her heart with a small incision and drain tube. We thought she would stabilize enough to start the treatment for hypertension and then to begin fighting the scleroderma. She was too weak to survive a stem cell transplant or a heart/lung transplant.

Mom never recovered enough strength to survive these treatments. On October 24, 1999, after two weeks in the hospital and less than six months after her diagnosis, Mom died of respiratory and cardiac failure. On the day she died, she was uncomfortable and irritable all morning. Her blood pressure dropped, and she said her feet and hands were numb. I rubbed her hands and feet to try to make her more comfortable. The nurses asked us to leave so that she could rest. Mom told us that she loved us. Forty-five minutes later, she was gone.

My mom loved to laugh. She worked almost twenty years for a major health insurance company. She was surrounded by friends who respected and loved her warmth, humor, and frankness. She worked hard and gave her best at whatever she set out to do. She was a faithful and generous friend and a wonderful wife and mother who made home a place of love and rest. She loved to paint and to plant flowers and garden vegetables. She was a wonderful cook and a shrewd investor of money. Most of all, she was the wisest person I ever knew and always had the ability to say just the right thing whenever I was confused or needed advice. She supported and loved me with every action and word. I am so sad and disappointed that my children will never know what a wonderful person she was. I will think of her and miss her every day that I walk on this earth. I believe with all my heart that, because we both have believed in Jesus' sacrifice and offer of eternal life, that I will see her again.

I hope that this tribute will grant others some insight into the illness that is affecting them or their loved ones. I also hope that my mother's story will teach people to be completely informed about their illness and assertive about medical care. This experience taught me that each person must be his or her own medical advocate and must fight for the treatment that he or she deserves. My mom was the most courageous person that I have ever known, but I think she was afraid to know the full extent of her illness and, as a result, could not demand correct treatment from her rheumatologists. Read everything you can and explore every available option that you have as a patient. If my mother's story prompts any person with scleroderma to demand better care for him/herself, then our loss will benefit someone else who needs more thorough treatment. Please do not let fear of the unknown rob you of your health and, quite possibly, of your life.

Submitted in loving memory of Georgette Stevens (1952-1999)

Jackie V, North Little Rock, Arkansas - jackiev@cyberback.com

copyright 1999 Jackie Vaughn                                                 Return to Tributes