The Story of A Survivor - by Linda Gonzalez

This is the story of a survivor. Even I find it hard to believe when I think back to that crisp fall day in 1982 when Dr. Siegal, his face filled with compassion, told my family "There is nothing I can do for Linda…she is going to die." I had been very ill for about four years by then. Dr. Siegal explained that the massive doses of steroids that kept me alive were now destroying my body—-congestive heart failure, a malfunctioning pancreas, high blood pressure, diabetes, osteoporosis, Cushing Syndrome… and the list goes on. After multiple misdiagnoses at major medical facilities around the country, only recently had my illness been pinpointed to Primary Immune Deficiency. Dr. Siegal, a leading immunologist at Mount Sinai, was my final hope.

Pressed by my anguished family to pursue any treatment that could possibly extend my life, Dr. Siegal reluctantly arranged for an experimental Laminar Flow Unit to be reopened. Originally developed to help children with Leukemia, the Laminar Flow Unit had proven unsuccessful for that purpose, but Dr. Siegal understood that we were willing to try anything that might possibly add time to my precious life. I agreed to spend the next four months in this unit - a "room" measuring approximately 6' X 8' made up of plastic walls and powerful air blowers that created a sterile environment. It contained a bed, a chair, and a small table that served as a night stand. No one but doctors and hospital staff members were allowed to enter.

The sterile environment of the Laminar Flow Unit kept me free from infections while I received IVIG infusions to provide the antibodies that my body no longer could make. My immune system began responding and getting somewhat stronger, but with no way to exercise in this restricted space, my leg muscles atrophied dramatically. My weakened legs were no longer able to support me and a bad fall collapsed three vertebrae in my spine and broke several ribs. Faced with the grim prognosis that I may never walk unassisted again, I left the Laminar Flow Unit confined to a wheelchair.

The IVIG infusions continued, but my hospital stays continued to be more numerous, averaging fifteen or more a year. The treatments miraculously kept me alive for the next several years, but twice during that time major complications threatened my life. First, in 1984, a viral infection caused my heart to enlarge. The only treatment was a heart transplant for which I was not a candidate. With no medical explanation, my heart mysteriously returned to normal size and with the help of a pacemaker, I survived. And again in 1987, fighting a devastating blood infection, I was put on a respirator and received Last Rites. Again, I survived.

Ravaged by years of illness and daily doses of steroids, my body changed so dramatically, distant friends no longer recognized me. The image of a stranger, first one, then three, then five inches shorter, with a humped back, extended stomach and bloated face reflected back in the mirror. Hiding behind humor, I began to call myself "Miss Piggy."

By the end of 1989, it was clear that the infusions were no longer helping my failing immune system. Once again, I turned to Mount Sinai, and was put on a series of experimental drugs. Each drug provided new hope for more time but one by one they failed. Just when there seemed no one left to turn to, I had the good fortune of being referred to Dr. Cunningham-Rundles at Mount Sinai to take part in a study she was conducting to measure the effects of the drug Interleukin-2 on Primary Immune Deficiency. Miraculously the drug did enhance the function of my immune system and provided the turning point my family and I had prayed for twelve painful years.

Requiring the Interleukin-2 injections weekly, I traveled each Tuesday to the Jeffrey Modell Infusion Room at Mt. Sinai Medical Center. It was there that I had the privilege of meeting Fred and Vicki Modell, whose tireless efforts and boundless generosity raised countless dollars to provide the much needed research on this mysterious and deadly disease. Their Jeffrey Modell Laboratory, headed by Dr. Cunningham-Rundles, is the cornerstone for the advances that have been made toward finding the cure for Primary Immune Deficiency.

Interleukin-2 combined with the support of Dr. Cunningham-Rundles and the Jeffrey Modell Foundation has changed my life over the past six years. No longer plagued by constant infections and frequent hospitalizations, I now have the energy to reach out and help other people suffering with Primary Immune Deficiency deal with their illness, as I did, one day at a time. The gratitude I feel for the dedication of my family, friends, Dr. Cunningham-Rundles and the Modells is hard to put into words. I can only hope they can see it, as I share with others the gift of hope they so generously gave to me and the light that shines in my eyes when I hold my first grandchild. Without their support and encouragement I would never have reached this moment. It is truly due to them that today, I am, indeed, a survivor.

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JMF UPDATE is published by the Jeffrey Modell Foundation, Inc., A non-profit Research Foundation. The mission of the JMF includes Research, Physician Education, Patient Support, and Public Awareness. Address all inquiries to the Jeffrey Modell Foundation, Inc., 43 West 47th Street, New York, NY 10036 or call (212) 575-1122. Copyright 2002, The Jeffrey Modell Foundation. All rights reserved.