End-stage lung disease is the most severe form of a lung disease. When a specific lung disease progresses to the point that lung function is seriously compromised, the disease is considered "end-stage." Most patients are diagnosed with a specific lung disease long before it progresses to end-stage.
A lung transplant can be an option for some patients with end-stage lung disease. The Lung Transplant Program at Brigham and Women's Hospital (BWH) offers single and bilateral (double) lung transplantation for appropriate candidates with a variety of end-stage lung diseases, including
- Chronic obstructive pulmonary disease (COPD)
- Interstitial lung disease/sarcoidosis/ idiopathic pulmonary fibrosis
- Lymphangioleiomyomatosis (LAM)
- Pulmonary vascular disease
- Cystic fibrosis
- Pulmonary hypertension
Learn more about lung transplantation.
- Symptoms of End-Stage Lung Disease
- Diagnosis of End-Stage Lung Disease
- Treatment for End-Stage Lung Disease
- End-of-Life Care for End-Stage Lung Disease
- What You Should Expect
- Multidisciplinary Care
- Appointments and Locations
End-stage lung disease patients experience acute and severe symptoms. These symptoms often include:
- Shortness of breath (dyspnea)
- Blue fingernails, lips or skin because of decreased oxygen levels
Your physician will take a complete medical history, measure how well your lungs are working and conduct other tests to determine if you have end-stage lung disease. This will help your physician to determine the severity of your condition and develop the most successful plan for treating and managing your symptoms.
Pulmonary Function Tests
Pulmonary function tests measure how well your lungs move air in and out of your airways. These tests include spirometry and peak flow monitoring. If your lung function is 30 to 50% below normal, you may be diagnosed with end-stage lung disease.
The treatment of end-stage lung disease is designed to slow disease progress and lessen the severity of symptoms. For patients requiring long-term ventilatory support through tracheotomy or non-invasive ventilation (BiPAP, CPAP), our team of physicians, respiratory therapists, and pulmonary rehabilitation specialists work to improve quality of life, minimize symptoms and complications, and optimize functional capacity, with a goal of independence from mechanical support.
Other treatments can include:
ECMO (extracorporeal membrane oxygenation) and Mechanical Circulatory Support
For patients with severe lung and heart failure, we can provide mechanical support that bridges patients until they recover or additional advanced therapy is successful.
Depending on your specific lung disease, its stage, your age and a variety of other factors, your physician may prescribe bronchodilators, steroids or antibiotics to help reduce airway inflammation and prevent exacerbations.
If you do not have enough oxygen in your blood, you may need supplemental oxygen. Devices are available that deliver pure oxygen to your lungs. Many devices are small, portable and convenient to use.
Read more about the benefits of oxygen therapy.
You can help manage the symptoms of end-stage lung disease by:
- Quitting smoking and all tobacco products
- Avoiding any known irritants that may cause lung inflammation
- Getting an annual flu and pneumonia vaccine
- Preparing for emergencies and periods of exacerbations
- Meeting with your healthcare team frequently to monitor your health
- Receiving emotional support through therapy or support groups
It is important to discuss your end-of-life wishes with your healthcare team and family. Your plan may include palliative and hospice care to help manage severe symptoms.
The Palliative Care team at BWH includes physicians, nurse practitioners, social workers, clergy members and a personal case manager to manage pain and other symptoms, as well as promote open communication about care. The Partners HealthCare Bridge to Hospice Program provides immediate home care to improve quality of life and comfort.
When you become a patient, our team of experts will develop a personalized, multidisciplinary care plan based on your disease and current research. Your health will be closely monitored and managed to slow the disease progress and alleviate symptoms. You will also have access to patient support groups.
Collaboration between pulmonologists, thoracic surgeons, physical therapists, social workers, nutritionists and dedicated nurses provides comprehensive, expert care for patients who have end-stage lung disease. Our multidisciplinary team members are recognized experts and have created one of the most advanced programs in the country for end-stage lung disease.
Visit the Kessler Health Education Library in the Bretholtz Center for Patients and Families to access computers and knowledgeable staff.
This page was last modified on 6/23/2016