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Patients with rare lung disease face agonizing treatment dilemma
Date:4/17/2014

in selected patients slowed the progression of the disease and was associated with a reduction in symptoms and improved quality of life.

Sirolimus sometimes is given to lung transplant patients to prevent rejection. But two studies found that sirolimus caused a serious and sometimes fatal complication called anastomotic dehiscence, which impairs wound healing if the drug is given around the time of the operation. Consequently the connections where the donor's lung are sutured into the recipient break apart.

Sirolimus (trade name, Rapamune) has a relatively long half-life, meaning it stays in the body even after a patient stops taking it. Consequently, many physicians do not prescribe sirolimus in patients who are waiting for lung transplants.

However, some LAM physicians have begun prescribing everolimus (Zortress), which has a shorter half-life. Once a patient undergoes a lung transplant, the patient is immediately taken off the drug. In an informal survey, Dilling found that physicians who have used this strategy report no dehiscence problems. Moreover, a recent animal study at the University of Cincinnati found that if sirolimus treatment ceased immediately, wound healing was not impaired. And an earlier study of pulmonary fibrosis patients who took everolimus found that among 11 patients who had lung transplants, there was no increased incidence of wound-healing/anastomosis problems.

Historically, Loyola physicians have not prescribed rapamycin-class drugs to LAM patients who are on the waiting list for lung transplants. "However, in light of recent evidence and experience, we are reconsidering this policy," Dilling said.

Laura Lentz, chair of the board of directors of the LAM Foundation, said that in raising the topic, Dilling "is providing a service that is going to make a big difference for women who have LAM."

Lentz agrees with Dilling that more research is needed on rapamycin-class drugs in LAM patients
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Contact: Jim Ritter
jritter@lumc.edu
708-216-2445
Loyola University Health System
Source:Eurekalert

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