Pediatric Pulmonology and Cystic Fibrosis

Ask the Experts

kier1 Catherine Kier, MD
Division Chief, Pediatric Pulmonology 
Stony Brook Children's Hospital

What could be more important than every breath your child takes? Stony Brook Children’s Hospital’s pediatric pulmonology services help Suffolk County children with everything from asthma and chronic lung diseases to cystic fibrosis and sleep disorders. Here, Division Chief Catherine Kier, MD, talks about new ways Stony Brook Children’s is helping the children (and their parents) breathe easier. 

What is pediatric pulmonology? 
Basically, pediatric pulmonology deals with all types of respiratory diseases that affect children — from the common cold to inherited chronic diseases. Here at Stony Brook Children’s, we offer the latest protocols and state-of-the-art technology to ensure that the children of Suffolk County have access to advanced pediatric pulmonary care. In addition, we have a fully accredited pulmonary function laboratory for assessment and diagnosis. This includes full pulmonary testing, spirometry, exercise and cold air challenge, allergy skin testing and allergy blood testing.

How does the pediatric pulmonary team work with children? 
We take a completely comprehensive approach. Each child’s team is led by a board-certified pediatric pulmonologist and can include a pediatric pulmonary nurse practitioner, nurse, respiratory therapist, pulmonology function technician, sleep technician, social worker, metal health coordinator, dietician, physical therapist, pharmacist and a genetic counselor - who all care for patients and their families. Together they make an initial assessment and diagnosis, develop a plan of treatment, perform interventions and provide follow-up care for as long as the child needs it. The care is highly individualized, and is frequently adjusted as the child grows and needs change.

Stony Brook Children’s has a Cystic Fibrosis Center — how does that help the children of Suffolk County? 
Stony Brook Children’s Cystic Fibrosis Center is just one of 130 centers in the country accredited by the Cystic Fibrosis Foundation. This means that it has gone through (and continues to go through on a regular basis) a rigorous evaluation that examines every aspect of care and services. It is also one the select cystic fibrosis centers that is part of the CF Learning Network. Members share quality improvement initiatives, evidence-based care practices and results of ongoing studies designed to provide better patient care. 

In fact, Stony Brook’s Cystic Fibrosis Center is serving as a model for other centers focused on a specific disease. They look to our multidisciplinary approach, our strong research focus, our state-of-the-art technology and the long relationships we build with patients and families, as those with cystic fibrosis live longer lives.

Is that one of the biggest changes — the greater lifespan of patients with cystic fibrosis? 
Absolutely. Life expectancy has increased dramatically and continues to rise. For example, the median age of survival for an individual with cystic fibrosis in 2003 was 33.4 years. In 2019 it rose to 46 years. That’s remarkable: a more than 12-year increase in a decade.

What can these changes be attributed to? 
A better understanding of the disease process, more proactive care, a multidisciplinary patient- and family-centered approach, and ongoing research strides. Recent medications called CFTR Modulators have been approved by the FDA, which have created great excitement in the cystic fibrosis community. They are a class of drugs that target the production and function of the CF protein, and patients eligible to take this drug have noted significant improvement in symptoms and quality of life. The goal of the Cystic Fibrosis Foundation is for research to continue to bring newer therapies to all CF patients. The challenge right now is the high cost. Our team at Stony Brook works tirelessly to get these medications to our community. We liaise with insurance companies, the drug manufacturers and other organizations to help make these drugs accessible and affordable. If there is something available that we know will help our patients, we do everything in our power to ensure that we can get them to them.

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For more information about Stony Brook Children’s, call (631) 444-KIDS.

All health and health-related information contained in this article is intended to be general and/or educational in nature and should not be used as a substitute for a visit with a healthcare professional for help, diagnosis, guidance, and treatment. The information is intended to offer only general information for individuals to discuss with their healthcare provider. It is not intended to constitute a medical diagnosis or treatment or endorsement of any particular test, treatment, procedure, service, etc. Reliance on information provided is at the user's risk. Your healthcare provider should be consulted regarding matters concerning the medical condition, treatment, and needs of you and your family. Stony Brook University/SUNY is an affirmative action, equal opportunity educator and employer.

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