Women in their childbearing years are the targets of a rare lung disease most of us have never even heard of called LAM — now, for the first time ever, patients have an approved treatment to keep them on their feet.
There’s very little that can slow Alison Hedervary these days, but for years, she would get short of breath with any activity. Doctors weren’t concerned until her lung collapsed.
“I was actually unloading my parent’s dishwasher and felt a pop in my back,” she remembered.
Hedervary has LAM — a rare lung disease caused by a genetic mutation. It strikes women ages 20 to 40. With LAM, abnormal cells grow out of control in certain organs. These cells also carve out holes in the lungs making them susceptible to collapse.
Robert Kotloff, MD, Pulmonologist at Cleveland Clinic told Ivanhoe, “There is a strong suspicion that in some way the disease is mediated by estrogen and certainly in young women there are high circulating estrogen levels. The other evidence for this is that LAM will often worsen during pregnancy.”
Hedervary is a nurse at the Cleveland Clinic where she was diagnosed and treated with a drug approved by the FDA in May as the first treatment for LAM. Rapamune was originally developed to prevent rejection of transplanted organs.
“It’s a drug that works by turning off the switch to cell growth,” Dr. Kotloff explained.
Hedervary told Ivanhoe, “I haven’t had any problems. I’m not short of breath. I can walk up a flight of stairs and breathe.”
In fact, her lung function right now is 92 percent, which is considered normal. Doctors say this medication is not a cure, but can stabilize this otherwise progressive disease.
Rapamune received orphan product designation because this is such a rare disease. Lung experts are hopeful this treatment and others on the horizon will stop LAM from being lethal.
Contributors to this news report include: Cyndy McGrath, Supervising Producer; Wendy Chioji, Writer; Christine Rifkin, News Assistant and Erik Laidroo, Editor.
