Important research for Fontan, Protein Losing Enteropathy (PLE), and Plastic Bronchitis (PB) Patients
If you have had a Fontan (even if you haven't had PLE or PB), please take a few minutes to fill out our anonymous survey. The information you provide will help to lead us toward a cure.
The Fontan palliation is the final planned surgery in the repair of a single ventricle-type of heart defect. Despite the best efforts of those who care for patients who have had this surgery, a small number of patients develop poorly understood and difficult to treat complications including Protein Losing Enteropathy (PLE) and Plastic Bronchitis (PB).
Protein losing enteropathy (PLE) is the more common of the two. It can occur at any time following the Fontan surgery. In children who have PLE, proteins in the blood are lost into the patient's intestines resulting in diarrhea which may be quite severe. The loss of proteins can lead to swelling of the arms, legs and abdomen. Fluid may also collect in and around the lung leading to difficulty breathing. Patients with PLE may have frequent infections due to problems with their immune system, and their growth is often poor. Often, they require aggressive treatment including frequent hospitalizations.
Plastic bronchitis (PB) is another severe complication that can occur at any time after the Fontan surgery. Patients with PB can develop casts – rubbery branching structures made of protein – that fill and block their airways. This can make breathing very difficult, and patients must cough up the cast to clear the airway to the lungs. At times, casts can form that are too difficult to cough up.
We do not know exactly how or why PLE and PB occur. We believe that many factors may lead to PLE and PB and that the causes may vary some from one patient to the next. The key to treating these difficult but rare conditions will be to learn as much about them as we can from as many different patients as possible. We will try to find things that patients with these conditions have in common and determine what may be the most important areas for future research and for the development of new treatments.
At the University of Michigan C.S. Mott Children's Hospital Congenital Heart Center, we have made a strong commitment to improving the care of patients with heart conditions such as single ventricle heart defects. An important part of that commitment is to develop new treatments for some of our most challenging problems. We have therefore made learning more about PLE and PB a priority for our program. Our goal is to identify causes and potential treatments to improve the lives of our patients.
Since these are relatively uncommon conditions, we hope to learn from patients around the world, please take the suvey on top of this page to let us know:
- what has led to their PLE or PB
- how it is being treated, and
- what has been successful (and what hasn't)