According to a story from the Philly Voice, it has been nearly nine years since Dr. David Fajgenbaum was first diagnosed with idiopathic multicentric Castleman disease. This immune system disorder can be potentially fatal, and Dr. Fajgenbaum struggled through several life-threatening moments during the first few years of his diagnosis. However, he also was able to play a major role in finding a treatment that has allowed him to live free of symptoms for the last five years.
About Idiopathic Multicentric Castleman Disease
Idiopathic multicentric Castleman disease is a subtype of Castleman disease that is characterized by dysfunction of vital organs and enlarged lymph nodes that display distinctive features upon microscopic analysis. It is one of three types of Castleman disease. As an idiopathic illness, the causal mechanism of the illness remains unknown. Idiopathic multicentric Castleman disease can cause a number of symptoms such as enlarged lymph nodes, liver, and spleen, fever, night sweats, fatigue, weight loss, coughing, shortness of breath, fluid buildup in the limbs, abdomen, or lung lining, and small skin tumors called cherry angiomas. Treatment options for this form of Castleman disease may include medications such as siltuximab, tocilizumab, high doses of steroids, and chemotherapy. Treatment may often be required indefinitely because this disease relapses frequently without it. To learn more about idiopathic multicentric Castleman disease, click here.
Finding a Treatment That Works for Everyone
Dr. Fajgenbaum was one of the unfortunate patients that did not respond to treatment with siltuximab or other similar drugs. As it turns out, this drug is really only useful for about a third of patients with idiopathic multicentric Castleman disease. However, the doctor is planning to conduct a clinical trial for the drug that has been helping him: sirolimus.
Dr. Fajgenbaum starting taking sirolimus on an experimental basis after his last dangerous hospitalization as the result of his illness. At the time, there was no evidence that the drug would actually work. The only hunch that he had was the fact that the disease triggers an abnormal immune system response and sirolimus, a compound produced by the bacteria Streptomyces hygroscopicus, had immune system suppressing properties.
Since he is a patient researching treatments for his own disease, Dr. Fajgenbaum has a uniquely powerful perspective that allows him to relate to everyone else involved– especially the other patients that he is trying to help. Enrollment for the sirolimus clinical trial is expected to begin this spring.
At Patient Worthy, we have written several articles about Dr. Fajgenbaum’s remarkable story of research and recovery. If you would like to read more about it, check out the links to other stories below.
A Doctor With Castleman Disease is in a Race Against Time to Save His Own Life
One Doctor’s Fight Against His Castleman: This Time, It’s Personal
University of Pennsylvania Football Team Kicks off Fundraising for Castleman Disease Research
How Collaboration Accelerates Cures
This Amazing Doctor Is a Hero Castleman Deserves
