Conditions and Treatments 

Within our program, we focus on diagnosing and treating children with advanced pulmonary hypertension or developmental lung disease.

Understanding pulmonary hypertension and developmental lung disease

• Advanced pulmonary hypertension. A condition where there is high blood pressure in the lungs. Pulmonary hypertension can be genetically passed down within families, or it can occur without a known cause. It can also be associated with another condition, such as congenital heart disease, connective tissue disorders, or lung diseases. Severe pulmonary hypertension can cause the right side of the heart to work harder to pump blood, which can lead to right-sided heart failure.
• Developmental lung disease. A broad term that describes different types of lung disease. Lung disease can be inherited, result from an autoimmune disease, or start after chemical exposure or infection. In severe lung disease, the lungs become scarred and stiff, making it hard to breathe and difficult for the oxygen-rich blood to move from the lungs to the heart and the rest of the body. Damage to lungs may not be possible to heal. In our CEAL program, we treat complex, advanced lung disease that isn’t responding to traditional treatments. For many of the children we see, we are the family’s last option while they await lung transplant.
• Interstitial lung disease. Interstitial lung disease (ILD) is a general term for a group of rare lung disorders that make it hard to breathe. Many of these diseases can be difficult to diagnose and manage, and they can affect people at any age. At our Center for Advanced Lung (CEAL) Therapies, we collaborate with pediatric experts in interstitial lung disease who understand the intricacies of these rare disorders in children.

Treatments

We are one of a very few pediatric centers in the nation to offer a combination of unique therapies. These include providing a bridge to transplant (getting your child strong and ready for transplant), providing lung and heart-lung transplants, and/or performing Potts shunt surgery.

Bridge to lung transplant therapies

With our bridge to lung transplant therapies, our goal is to help your child survive to lung transplant and become as healthy as possible while awaiting lung transplant. The healthier your child is going into lung transplant, the better she or he will adjust to new lungs and recover. Bridge to lung transplant therapies include: 

• Medicines. We provide the full range of medicines for pulmonary hypertension and lung disease, as well as new, innovative medicines.
• ECMO. Extracorporeal membrane oxygenation (ECMO) is a lifesaving device that serves as an artificial lung in emergencies, but it’s not meant to be used long term. For this reason, we aim to get your child off of ECMO and onto a mechanical circulatory support device to empower him or her to recover and feel better.
• Mechanical circulatory support devices. These devices are ideally used before your child’s health becomes critical, helping to avoid some of the late-stage complications that can come with lung or heart failure, such as failure of multiple organs. These devices allow your child to move around more freely and breathe on his or her own without a breathing tube attached to a ventilator. Being able to walk around and breathe independently greatly improves your child’s strength and overall health. Our surgical and medical teams will work closely together to determine the right type of device to place in your child so that he or she can gain strength while awaiting lifesaving lung transplant.