(From the January 2015 Issue of PediatricsOnline)
The new Pulmonary Sickle Cell Clinic at Nationwide Children’s Hospital is wrapping up its first year of treatment for young patients with sickle cell diseases and approaching the six-month mark of care for adolescents and teens. The placement of a dedicated pulmonologist in the Comprehensive Sickle Cell and Thalassemia Program allows patients to be seen by a lung specialist during regular visits with their hematologists. The twice-monthly clinic aims to reduce loss to follow-up of patients referred to pulmonologists and to improve collaboration among patients’ caregivers to optimize treatment and improve outcomes.
“We previously had a high no-show rate for patients with sickle cell referred to pulmonology for separate appointments,” says Benjamin T. Kopp, MD, the clinic’s dedicated pulmonologist. “We’re capturing more patients with me embedded in the clinic, but more importantly, we’re able to consult on their care as a team.”
The Comprehensive Sickle Cell and Thalassemia Program includes hematologists and a pulmonologist, as well as experts in dentistry, psychology, social work, genetics and acute care. To monitor improvements in patients’ care and outcomes since the addition of a pulmonologist to the clinic, data collection is in progress, with several studies also planned. Initial clinic experiences indicate that follow-through with pulmonology visits has already been improved. Measurements of health outcomes, such as breathing-related visits to the emergency department and episodes of acute chest syndrome — a vaso-occlusive event affecting the lungs of sickle cell disease patients — will help the clinic practitioners determine the effectiveness of this new care model.
“Theoretically, having a pulmonologist embedded in the clinic is the best care model,” says Dr. Kopp, who recognizes the positive impact that other dedicated specialists have made on patient care in pulmonology clinics such as the Cystic Fibrosis Clinic. “Patients prefer it and are more likely to attend when they have a regular provider they know well. By combining visits and staying in one place we get around those barriers and also make the pulmonologist a regular and familiar part of the patient’s sickle cell care team.”
Although most pulmonologists receive limited training in the management of sickle cell-related lung complications, Dr. Kopp believes they are crucial members of the patients’ long-term care team.
“There are several groups of sickle cell patients when it comes to lung care, and they often overlap,” Dr. Kopp explains. “Many patients with sickle cell have asthma or recurrent wheezing, and others have recurrent episodes of acute chest syndrome that may include wheezing. All of these children are also at risk for sleep disordered-breathing, deconditioning and long-term pulmonary hypertension and oxygenation issues.”
Estimates in the literature of children with sickle cell disease who also have asthma range from 2 to 50 percent, while 50 percent have at least one episode of acute chest syndrome in their lifetime and 30 percent eventually develop pulmonary hypertension.
“We hope that having a pulmonologist more regularly involved in the care of children with sickle cell disease can make a big impact on their health now and for the long term,” Dr. Kopp says. “Any opportunity we have to improve the likelihood that these individuals receive the best care possible is worth it.”