The Penn State Children’s Hospital Approach to the Pulmonary and Cystic Fibrosis Program
The Pediatric Pulmonary (lung) and Cystic Fibrosis Program helps children with a wide range of lung problems. Our experts are here to help improve your child’s breathing and quality of life.
We treat children of all ages - from infants to teens - with sudden (acute) and ongoing (chronic) lung and breathing problems. After your child's first visit, we will assign a primary attending doctor or an advanced practice provider to work with your child and family during treatment.
Our experts provide personalized treatment plans and programs, including:
Chronic lung disease of prematurity, or bronchopulmonary dysplasia (BPD), is one of the most common complications of premature birth, especially those born less than 32 weeks of gestation. Infants born prematurely often develop lung disease, which can require prolonged hospital care in the neonatal intensive care unit (NICU). The respiratory care and other support they require changes throughout their hospitalization. Depending on the degree of lung disease, some premature babies with lung disease may be discharged home on supplemental oxygen and some require other forms of respiratory support. Once discharged, these infants will need close monitoring by an experienced team for growth and development and medical needs, since they are at high risk for hospitalization until two years of age. Some of these premature infants with lung disease may also develop a more serious complication, called pulmonary hypertension, that requires more aggressive management. Studies have shown that preterm infants with BPD continue to have abnormal lung function into adulthood.
The Penn State Health team of pediatric pulmonologists has developed the BPD Program, a comprehensive multidisciplinary program with a focused team that includes attending physicians from the specialties of pediatric pulmonology, neonatology and pediatric cardiology. This team also includes members from the home ventilator program team, NICU nursing, respiratory therapists, a speech therapist and a radiologist who meet on a weekly basis to provide continuity of care to this vulnerable population. Once these infants are discharged, they will continue to be followed. We have several specialty multidisciplinary clinics to serve this population.
Children with Home Ventilators
The Pediatric Home Ventilator Program at Penn State Children’s Hospital is dedicated to the care of children with breathing problems that require long-term mechanical ventilator support. We care for children from infancy through young adulthood.
Our team of medical providers assists to transition children from the hospital to home with ongoing follow-up care after the child has been discharged. Our goal is to provide comprehensive and coordinated care for children who require respiratory support. Conditions we treat include:
- Apnea: a temporary stop of breathing. Can be due to abnormalities in the airway or to brain malformations.
- Bronchopulmonary dysplasia (BPD)/chronic lung disease of infancy: lung disease of premature infants where the lungs have not fully developed.
- Hypoventilation: breathing that is too weak or slow to meet the body’s needs (congenital central hypoventilation, acquired central hypoventilation, Chiari malformations, other brain malformations).
- Neuromuscular disorders: diseases causing weak muscles where patients do not have enough respiratory effort to breathe properly and efficiently (muscular dystrophies, spinal muscular atrophy, spinal cord injury).
- Restrictive lung disease: inflation of the lungs is prevented by something compressing or pressing upon them. This could be due to abnormalities of the rib cage or spine (scoliosis, kyphosis), excessive muscle activity (cerebral palsy) or increased abdominal pressure.
- Tracheomalacia/bronchomalacia: the walls of the trachea (windpipe) or bronchi (branches from the trachea to the lungs) are soft or floppy, leading to narrowing of the airway during exhalation (breathing out).
Our ventilator training program
Our program educates parents and/or other caregivers in the care of their ventilator-dependent child at home. This program is designed to take six weeks from the time of tracheostomy tube placement. We require that at least two caregivers be trained in all aspects of the child’s care. Our education includes written materials, videos, hands-on learning and simulation lab training.
From hospital to home
Our inpatient team consists of pulmonologists; nurses; nurse practitioners; physician assistants; respiratory, physical, occupational, speech and music therapists; nutritionists; care coordinators; and social workers who will work to provide you with the skills necessary to care for your child. Our team also assists in coordinating all services that are needed to take your child home. This includes obtaining in-home nursing services, setting up the medical equipment for your home and coordinating all outpatient follow-up appointments and other outpatient services, such as Early Intervention (community-based therapy services for your child at home).
Once you are home, you will continue to have close follow-up with our home ventilator team, which includes our nurse practitioner, care coordinator and pulmonologists. We will follow your child closely in our outpatient clinics, as well as be available for you 24 hours per day via phone, email or our patient health portal. We will continue to help you obtain whatever services your child needs to be able to stay at home.
Patient and Caregiver Education
Our team provides education on the correct use of:
- Nebulizers and nebulized medications
- Metered dose inhalers (MDIs) with spacer devices
- Dry powder inhalers (DPIs)
- Vests for high frequency chest wall oscillation
- Chest physical therapy (CPT)
- Airway clearance
Pediatric Sleep Issues
If your child’s lung and breathing problems are causing sleep issues, your specialist may recommend a sleep study at Penn State Sleep Research and Treatment Center. We care for all types of sleep-related health conditions, including:
- Sleep apnea (short periods of not breathing during sleep)
- Apnea of prematurity (short periods of not breathing in infants)
- Insomnia (trouble falling or staying asleep)
- Restless sleep
- Narcolepsy (trouble staying awake)
Our specialists include:
- Pediatric pulmonologists (lung doctors)
- Pediatric sleep doctors
- Certified pediatric nurse practitioners (nurses who can check patients and write prescriptions)
- Social workers
Our Experts in Pulmonary and Cystic Fibrosis Care
The Pediatric Pulmonary and Cystic Fibrosis program helps children with lung conditions, including cystic fibrosis.
Why Choose Penn State Children's Hospital for Care?
We are committed to providing outstanding patient care and researching lung diseases and treatments. Our doctors take part in clinical research studies that might be available to your child.
We provide care for:
- Aerodigestive problems (breathing, swallowing or digestive problems along with lung problems)
- Bronchopulmonary dysplasia (BPD)
- Congenital lung anomalies (lung problems present at birth)
- Cough and wheezing
- Cystic fibrosis
- Interstitial (forming) lung diseases
- Ongoing (chronic) cough
- Ongoing (chronic) lung diseases
- Ongoing (chronic) respiratory failure/insufficiency
- Neuromuscular respiratory diseases (breathing problems that may come with brain or muscle diseases, such as muscular dystrophies and myasthenia gravis)
- Noisy breathing
- Recurrent pneumonia
- Sickle cell-related lung disease
- Sleep apnea (breathing problems during sleep)
- Sudden (acute), ongoing (chronic) and frequent (recurrent) pneumonia
Support groups provide children and their families an opportunity to connect with others in similar situations. Learn more about the support groups offered at Penn State Children’s Hospital.
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