As defined by PubMed Health, Protein Losing Enteropathy (PLE) is an abnormal loss of serum protein from the digestive tract or the inability of the digestive tract to absorb proteins. PLE is a rare, but well-known complication of HLHS, which may develop in some children weeks or years after the Fontan procedure. Symptoms include swelling in the abdomen and feet, diarrhea, fever, and abdominal pain. Complications include malabsorption due to diarrhea, an impaired immune system which increases the chances of infection, and low cardiac output.
Sadly, PLE is associated with an alarming mortality rate. The 2007 study, Protein Losing Enteropathy after Fontan Surgery - Clinical and Diagnostical Aspects by Tarnok, Bocsi, Lenz, and Janousek, from the Department of Pediatric Cardiology, Cardiac Center Leipzig GmbH, University of Leipzig, Germany, states that between 2,000 and 5,000 children have been diagnosed with PLE. Of the children that have major cardiac surgery, about 7-10% becomes afflicted with PLE. Of these, there is only a 50% survival rate.
The cause of PLE in post-Fontan children is unknown. There are many studies on this condition suggesting the use of various medications such as steroids and diuretics for treatment, but further research is necessary to better understand this condition.
Currently, the Children’s Hospital of Philadelphia, through its Single Ventricle Survivorship Program, follows a treatment protocol that has proven effective in managing PLE. The use of Sildenafil (Viagra®, Revatio®) lowers pulmonary flow to improve cardiac output after the Fontan operation, in addition to the use of the steroid Entocort, to reduce bowel inflammation.
Talk to your child’s doctor to learn more about PLE and the symptoms of this condition.
If your child has been diagnosed with PLE and you are interested in connecting with a support group, please contact the Congenital Heart Information Network for more information.
My daughter died of Protein Losing Enteropathy about 7 weeks ago at CHOP. It's a terrible complication from the Fontan surgery and having a single ventricle physiology. Dr Rychik at Children's hospital in Philadelphia is certainly the doctor to see or consult if your child is diagnosed with this. Single ventricle survivorship program is great
ReplyDeleteI am so very sorry for your loss. I imagine there are no words that bring comfort at this time but I am sending many prayers and positive thoughts to your and your family.
DeleteI must admit, PLE is, and always has been a fear of mine. Children with HLHS may survive three very complicated surgeries but they still face many obstacles ahead. I have heard many great things about Dr. Rychik. He is very well-known in the field. Thank you for sharing your story and your thoughts on the Single Ventricle Survivorship program.
As heart parents we must continue to support one another and spreading the word about CHD is one small way I feel I can help another parent. The support I have received has certainly brought me through difficult times.
Be kind to yourself and allow yourself a little peace today,
Julie