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Experts at our Pediatric Cystic Fibrosis Center are committed to delivering the highest-quality treatment and supportive services to children diagnosed with cystic fibrosis or primary ciliary dyskinesia (PCD). Accredited by the Cystic Fibrosis Foundation for our expertise, our program aims to improve the quality of life of every child in our care.

Cystic fibrosis is a genetic condition that causes mucus to be thick, sticky, and prone to harboring bacteria that can cause respiratory infections. The mucus also affects the digestive system, making it difficult for children with cystic fibrosis to absorb the nutrients they need. PCD, also a hereditary condition, causes breathing difficulties that begin shortly after birth, a year-round wet cough, and recurrent respiratory and ear infections.

A sweat test to look for elevated levels of chloride, as well as genetic testing, are used to diagnose cystic fibrosis. To diagnose PCD, we use genetic testing and nasal nitric oxide (nNO) measurement, which determines the amount of nitric oxide in the sinus cavity. Children with PCD tend to have a significantly lower nitric oxide level than those without the condition.

Treatment for Cystic Fibrosis and Primary Ciliary Dyskinesia

Our specialists, part of Hassenfeld Children’s Hospital at ºÃÉ«tv Langone, work with you and your child from infancy to adulthood to develop an individualized treatment plan. We follow a team-based method of care that involves the collaboration of pulmonologists, nurses, pharmacists, respiratory therapists, physical therapists, dietitians, and social workers.

Treatment for cystic fibrosis and PCD focuses on monitoring the airways for signs of infection and prescribing medications that keep the airways clear and healthy. Chest physiotherapy is an important part of the treatment plan. This technique, in which gravity and percussion are used to clear the airway, is used daily at home.

Children with cystic fibrosis also need help preventing and treating constipation and intestinal blockages, and dietary guidance to ensure they are getting the nutrients they need. We also work closely with the Pediatric Diabetes Center, as children with cystic fibrosis are at risk for developing diabetes.

Partnering to Provide Comprehensive Care

We collaborate with Hassenfeld Children’s Hospital’s behavioral health experts to support families affected by cystic fibrosis and PCD. We also teach ways to ease your child’s anxiety about medical procedures or swallowing pills.

To minimize the risk of infection from patient to patient, we follow strict infection control guidelines in the hospital and in outpatient settings. When caring for children with cystic fibrosis, staff members are required to wear gowns and gloves, and rooms are thoroughly disinfected to prevent the spread of bacteria that can cause infection.

As your child grows, we coordinate and transition care to ºÃÉ«tv Langone’s Bronchiectasis and Nontuberculous Mycobacteria (NTM) Program. To help make this process as smooth as possible, our social workers meet with you and your teen to make sure you are both ready for this transition. We also help prepare your teen to be responsible for managing daily treatments.