Systemic amyloidosis patient to receive triple organ transplant

Oct 28 2010

In 2005, then 40-year-old Walt McGuire was playing hockey twice a week, working full-time and chasing his young daughter. Then one day, he noticed after one trip up the stairs to catch her, he couldn't catch his breath. A few days later his legs were swollen. After numerous trips to different doctors, McGuire finally was diagnosed with a rare blood disorder called amyloidosis.

Now, McGuire will be the first systemic amyloidosis patient in the world to receive a triple organ transplant as a result of his disease.

Amyloidosis is a condition where proteins fold in an abnormal manner. As a result, these proteins can travel to the heart, kidney, lung and spleen and severely damage these organs. This condition can present as carpal tunnel syndrome, heart, kidney or liver failure. Other symptoms include weight loss, nausea, and vomiting. It affects between five and 13 people per million population per year and some can have the disease many years before it is diagnosed.

"Mr. McGuire has the most common form of this disease in the United States called systemic AL(light-chain) amyloidosis," said Dr. Horacio E. Adrogue, medical director of the Methodist Transplant Network, a part of The Methodist Transplant Center in Houston. "This means a type of protein that comes from bone marrow cells is not being produced correctly, not folding properly, becoming insoluble, getting stuck in the organs and destroying them."

If a person with this condition receives a bone marrow transplant and chemotherapy the disease can be turned off.

"You then have to deal with the consequences of the disease," Adrogue said. "For example, if the kidney is damaged, the person can receive a kidney transplant and they can do quite well."

There are two other major forms of amyloidosis. AA (associated) amyloidosis, which is associated to chronic diseases such as Crohn's Disease, tuberculosis and familial Mediterranean fever, and ATTR familial amyloidosis, a condition where an enzyme in the liver is not being produced correctly. People with ATTR amyloidosis can have their liver removed, receive a transplant, and live a normal life.

"We have created the Amyloid Clinic, a multi-disciplinary, multi-specialty clinic at Methodist in an attempt to diagnosis this condition much quicker," Adrogue said. "There are only two other facilities in the country with similar clinics. Our goal is to try to diagnose patients quicker before their organs are damaged.

SOURCE The Methodist Transplant Center