Advanced Lung Disease
The Advanced Lung Disease program consists of programs for cystic fibrosis, pulmonary vascular disease, interstitial lung disease and neonatal lung disease.
What is Advanced Lung Disease?
Utilizing a team of experts, Nationwide Children’s Hospital has assembled an organized program to assist referring health care providers in caring for patients with progressive lung or heart-lung disorders.
Under the Department of Pulmonary Medicine, the Advanced Lung Disease program consists of programs for cystic fibrosis, pulmonary vascular disease, interstitial lung disease and neonatal lung disease. Physicians see patients throughout all stages of care from early to late-stage diagnosis.
The goal of the program is to collaborate with physicians in medically managing their patients and if needed, expedite a patient’s evaluation for lung or heart-lung transplantation.
If transplantation is deemed necessary, the team at Nationwide Children’s Hospital has a “toolbox” containing a full spectrum of mechanical assist devices, designed for short- to long-term use, in order to reduce morbidity and improve patient survival. Devices used to bridge pediatric patients to transplant include ambulatory ECMO and artificial lung technology.
What are the Symptoms of Advanced Lung Disease?
Dyspnea is a very common complaint in pediatric, adolescent and young adult patients with pulmonary disorders. We assess dyspnea following guidelines determined by the New York Heart Association (NYHA) Functional Classification, which uses four categories based on physical activity limitations:
- Patient Symptoms: No limitation of physical activity. Ordinary physical activity does not cause undue dyspnea, fatigue, or palpitations.
Class II (mild)
- Patient Symptoms: Slight limitation of physical activity. Comfortable at rest, but ordinary physical activity results in dyspnea, fatigue or palpitations.
Class III (moderate)
- Patient Symptoms: Marked limitation of physical activity. Comfortable at rest, but less than ordinary activity causes dyspnea, fatigue or palpitations.
Class IV (severe)
- Patient Symptoms: Unable to carry out any physical activity without discomfort. Symptoms of dyspnea, fatigue or palpitations at rest. If any physical activity is undertaken, these symptoms are increased.
For any patient that is NYHA Class IV or Class III unresponsive to standard therapy, our team may provide services that could either assist in medical management or may expedite their evaluation for lung or heart-lung transplantation.
Advanced Lung Disease Diagnosis Paths
The initial evaluation and treatment focuses on therapies available for the specific lung diseases classified as:
- Congenital lung disease — Airway disorders, congenital malformations of the lung and surfactant deficiencies
- Obstructive lung disease — Alpha-1-antitrypsin deficiency, bronchiectasis, bronchiolitis obliterans and cystic fibrosis
- Restrictive lung disease — Interstitial lung disease and neuromuscular disorders
- Pulmonary vascular disorders — Pulmonary arterial hypertension, congenital heart disease, pulmonary vascular and veno-occlusive diseases, sarcoidosis and connective tissue diseases
The work-up and treatment of these advanced lung diseases follows along a continuum. At some point, the dyspnea reaches levels refractory to typical medical management that may limit the patient’s ability to participate in activities of daily living. At that time, we can evaluate a patient for lung or heart-lung transplantation. For patients with advanced lung disease including acute respiratory decompensation, our Ambulatory ECMO and Artificial Lung Program is available, and we have the capability of transporting patients on ECMO if needed.