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    Posted January 23, 2014 by
    Cincinnati, Ohio
    This iReport is part of an assignment:
    Living with a rare disease?

    aggressive systemic mastocytosis

    I do not suffer from a rare disease but my late husband (Kevin Medley) did. He had "aggressive systemic mastocytosis". He died at the age of 49 on September 7, 2012 leaving me to raise our two boys ages 12 and 9 alone.
    My husband had been sick since 2009 but no one can pinpoint exactly what was wrong with him. RA, lupus, ITP, etc, etc. He went from doctor to doctor, specialists to specialists. His symptoms suggests many other diseases but when additional tests to confirm were done, the results were negative. Many specialists have scratched their heads with regards to his case.
    Finally In 2010 after a bone biopsy, his hematologist (Dr. James Essell of the Cincinnati Jewish Hospital Bone Marrow Transplant Unit) finally diagnosed him with "aggressive systemic mastocytosis" and referred him immediately to the Mayo Clinic. I remember asking "He has WHAT!!!???". Dr. Essell warned us about "Googling" it ... he was right. It was depressing to read the very limited information available. Here's a quote from NIH article "Aggressive systemic mastocytosis (ASM) is a clonal mast cell disease characterized by progressive growth of neoplastic cells in diverse organs leading to organopathy. The organ-systems most frequently affected are the bone marrow, skeletal system, liver, spleen, and the gastrointestinal tract.". The Mayo Clinic website simply puts it as "a disorder caused by a genetic mutation that results in an excessive number of mast cells in your body. Mast cells normally help protect you from disease and aid in wound healing by releasing substances such as histamine and leukotrienes. But if you have systemic mastocytosis, excess mast cells can build up in your skin, around blood vessels, in your respiratory, gastrointestinal and urinary tracts, or in reproductive organs."
    My husband travelled to the Mayo Clinic regularly for evaluations. His treatments (chemo-type) were all done at Jewish Hospital in Cincinnati. Before this disease, my husband was healthy and in good shape. He was active. He enjoyed spending time with the kids. We enjoyed travelling and being outdoors. As his disease progressed, his energy level plummeted. The treatments wore him out. He was in pain. He was bleeding constantly. There were times when you look at him, you would have thought nothing was wrong with him. But his inside was deteriorating. No matter how bad he felt, he rarely complained. He kept working, he tried to be involved with the kids activities, he kept going.
    In late July 2012, we took the whole family to the Mayo Clinic for his regular check-up. We wanted the kids to know where dad goes regularly. We were going to make it part of our family vacation. After his appointment, we were going to the Badlands in South Dakota. We did not make it. His biopsy showed that he has full blown AML. His Mayo doctors sent us back to Cincinnati to start chemo therapy immediately and hopefully be able to have bone marrow transplant. He was admitted to the hospital the day after we returned to Cincinnati, August 3. He died September 7.
    There is no cure for this disease. There are a few treatments but the success rate is low. It may be too late for my husband, but finding a cure or better treatment options will benefit others who are suffering from this disease. Please check out this website for more information: www.tmsforacure.org.
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