Loyola treats rare lung disease LAM in women

Six years ago after the birth of her first son, Laura Lentz started experiencing some minor asthma-like symptoms.

"I thought I was just out of shape from pregnancy," said Lentz, 37, a resident of Evanston, Ill. "I didn't know what was going on but it wasn't bad enough to do anything about it."

Two years later, after the birth of her second son in 2007, Lentz's symptoms became a great deal worse.

"I was having severe shortness of breath and stomach pains," Lentz said. "I ended up in the ER and they took a CT scan of my lungs and found some cysts. That's what eventually led to my diagnosis."

Lentz was diagnosed with a rare lung disease known as lymphangioleiomyomatosis (LAM).

"It was very hard because they didn't tell me much more about LAM," she said. "I went to the Internet and looked it up and what I found was pretty traumatic."

In patients with LAM, abnormal cells start growing out of control in the body, including the lungs, lymph nodes, lymph vessels and kidneys. Over time, the abnormal cells form cysts that can invade the lungs, destroying and replacing normal lung tissue. Symptoms include shortness of breath, persistent cough, collapsed lung, chest pains and fatigue.

LAM is progressive and frequently fatal disease that almost exclusively strikes women, usually during their childbearing years. There is no cure for the disease and death comes from respiratory failure.

However, with early intervention and treatment, a LAM patient's length of survival and quality of life can be improved and her lifespan dramatically extended. It is a rare disease that was once featured in the popular television drama "House."

Due to its rarity, LAM patients have had to travel hundreds and sometimes thousands of miles to find physicians who are knowledgeable enough to care for the condition, which is frequently misdiagnosed as asthma, emphysema or bronchitis.

"I had to travel east as far away as Cincinnati, Ohio and Bethesda, Md., to find a clinic and physicians," Lentz said.

Fortunately, Laura has finally found the care she requires much closer to home after Loyola University Medical Center became one of a network of 16 clinics across the nation to diagnose and treat LAM. It's the only designated LAM clinic in Illinois.

"At Loyola we provide a breadth of experience and skill in specialty areas that pertain to LAM," said pulmonologist Dr. Daniel Dilling, medical director of the LAM clinic and of respiratory care at Loyola. "We can manage the spectrum of LAM patients' problems - all the way from minor shortness of breath to managing involvement of other organs or of a collapsed lung and even to lung transplantation, if necessary."

Loyola's LAM clinic is led by a number of pulmonologists, radiologists, pathologists, interventional radiologists and cardiothoracic surgeons, all of whom work together to diagnose, treat and manage the complications of the disease. They are supported by respiratory therapists, nutritionists and physical therapists. Patients at Loyola's LAM Clinic also will have access to a variety of clinical trials designed to find treatments for LAM.

"The LAM patients in the Chicago area have been eager to have a LAM clinic in their area," said Jill Raleigh, executive director of the LAM Foundation, the national organization that designated Loyola as a clinic for the disease. "In the Chicago area, we have a large patient population considering how rare the disease is."