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Johns Hopkins Pediatric

A Rare Encounter in Pulmonary Rehab

June 6, 2014

In managing a patient with LAM, Sam Mayer worked closely with specialists across the spectrum, including pulmonologist Jason Mock, to craft a safe rehab plan.

In managing a patient with LAM, Sam Mayer worked closely with specialists across the spectrum, including pulmonologist Jason Mock, to craft a safe rehab plan.

Peggy Chung entered her 21st week of pregnancy with a low-grade fever. Within a week, she developed high fevers and extreme fatigue. Her physician diagnosed her with pneumonia and prescribed antibiotics, but they failed to alleviate her symptoms. Her husband later brought her to an emergency room at a community hospital in Frederick, Md., where she was admitted out of concern for her pneumonia. Within a few hours her health deteriorated rapidly, landing her in the hospital’s ICU. The next morning she was rushed to Johns Hopkins Hospital.

Chung, 37, soon went into severe acute respiratory distress and required 100 percent oxygen. Imaging revealed diffuse bilateral infiltrates and a pneumothorax. Within several hours of admission, a bronchoscopy showed diffuse alveolar hemorrhage. Chung also tested positive for influenza A. An obstetrics team followed her with daily fetal heart monitoring, working alongside pulmonary and infectious disease experts. Other troubling problems arose, and she failed to improve after eight days. A CT revealed numerous bilateral cysts on both lungs. Then as Chung developed pre-eclampsia, the decision was made to deliver her baby at 24 weeks. Her condition improved almost immediately postpartum, though she’d need additional monitoring and rehabilitation.

After considering Chung’s age, gender and imaging, her clinical team reached the conclusion that she had lymphangioleiomyomatosis (LAM). The rare disease (estimates range from 1,500 to 10,000 cases worldwide) often strikes women in childbearing age. Pregnancy—with its hormonal blast—can trigger a flare-up. From a rehab standpoint, says physiatrist Samuel Mayer, the challenges in Chung’s case were daunting, given her diffuse cystic lung disease, pregnancy and flu symptoms. On top of that, he says, there was an emotional toll: She and her husband had to balance the care of a premature newborn.

 “But we’re well positioned to manage patients with rare diagnoses like LAM,” says Mayer, noting a broad spectrum of specialties available to support medical and psychosocial needs. In this case, aid came from a child-life specialist, infectious disease physician, pulmonary experts and pediatric intensivists, among others.

Simply getting patients this compromised to sit up builds endurance, says Mayer, as does teaching them to breathe more efficiently. Concomitantly, respiratory therapy, controlling secretions, improving nutrition and inhalers can ease breathing.

In Chung’s case, rehab had to be delayed, explains pulmonologist Jason Mock, because she needed to lie on her stomach for two weeks while intubated to alleviate her ARDS. “With large tubes in her groin and chest and because of the severity of her illness,” he says, “she couldn’t receive traditional rehab.” Yet, once off of extracorpeal membranous oxygenation (ECMO) after four days, she began to improve dramatically, says Mock. Recalling the “amazing” moment he watched Chung walk from the NICU after seeing her baby, Mock says “she clearly benefited from inpatient rehab.”

On day 61 at the hospital, Chung was discharged without oxygen. She has little recollection of her stay. But she no longer gasps for air, she says. Rehab, she adds, has helped her improve her speech, walking and climbing stairs. These days, Chung and her husband are busy preparing for their daughter’s homecoming. Though still being monitored in a NICU, the baby is making steady progress. Looking back, Chung says her hospital experience was both happy and sad. “I’m still alive, so I’m OK with all of it now.”

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