Rare Diseases Day

Kris and I Two Days Before Transplant Surgery

Happy Leap Day! Today is also Rare Diseases Day, a day when we who have a rare disease, can feel proud and special. You may hear us say things like "Mine is rarer than yers" or "Maybe, but mine is easier to pronounce".
In the USA, a disease or disorder that affects fewer than 200,000 Americans at any given time is defined as rare.
I'm 'blessed' with a rare disease with many names. Wegener’s Granulomatosis A.K.A. Granulomatosis with Polyangitis (GPA)/Midline granulomatosis/Necrotizing Respiratory Granulomatosis7Pathergic Granulomatosis etc. etc. The prevalence of GPA in the United States is estimated to be 3 cases per 100,000 people. So statistically there should be 3 or 4 of us in Sioux Falls, SD where I live, which would make a rather small support group.
GPA was first described by a German pathologist Friedrich Wegener (pictured below),. In 1989 the American College of Chest Physicians (ACCP) gave Wegener a “master clinician” prize. That prize was rescinded in 2000 when a Mayo Clinic doctor discovered his Nazi past and connections to experiments on Polish Ghetto prisoners. This also triggered a movement to change the name. Currently GPA appears to be the lead horse in this race.
I'm so proud. wink emoticon
GPA vasculitis destroyed my kidneys in March of 1974. My sister Kris, (organ donors and the families of deceased donors are the best people in the world) gave me one of hers in Oct. 1975. In 2013 it became apparent that I was having a GPA relapse, this time as a fibrosis in my lungs (interstitial lung disease). So far the kidney is fine, but my lung function is significantly diminished. I'm currently on a treatment regimen that should induce a remission. We'll know more in a few weeks. In the meantime I'm still 'livin on bonus time'.

Dr. Friedrich Wegener

http://www.rarediseaseday.org/
http://emedicine.medscape.com/article/332622-overview