The past year ...
My daughter Ava is 12 years old. She has a smile and laugh that will make you smile, even after a long, tough day. She loves to dance ( ballet, tap, and jazz) and just celebrated her 5th of dance and received a special trophy.
She also has Hypoplastic Right Heart Syndrome. She has had two open heart surgeries, one at 4 1/2 months, the other at four years old. She has always had complications after each of her operations, resulting in several re-hospitalizations after each surgery. Last year Ava was hospitalized for PLE Protein Losing Enteropathy. PLE is a rare complication, which is a result of her previous open heart surgery the Fontan. It causes severe diarrhea and results in protein loss from the body when this occurs, and it makes it difficult for her body to absorb protein and for her to grow and gain weight. Ava has not increased in height or gained any nutritional weight in almost two years. Last year (2018), she was hospitalized three times for uncontrolled symptoms of the PLE. When she has a flare, she gets horrible stomach pains, headaches, and diarrhea sometimes 4-5 times within a few hours. Each hospitalization last year was progressively longer. In May it was five days, July 2 weeks and then the longest was 3 1/2 weeks, September into October.
It was during this last hospitalization that they tried every therapeutic intervention that they know to help stop the symptoms. Ava had IV Lasix, which was unsuccessful, The hospital placed a PICC line( IV line directly into her heart) which they administered Milirone (given to help the heart function better, for kids in heart failure), that didn't work. She was on TPN (nutrition by IV) and on fluid restrictions, which was very difficult for her. So much, so she tried to sneak water by trying to drink the water directly from the faucet after she went to the bathroom.
At this point, the doctors told us that she was not strong enough to survive a heart transplant, which was the only other option they had for Ava. The doctor asked us if we knew we only had a year left with Ava, would we want to go home or spend it in a hospital. Imagine being asked about your child. Our answer was we want, and more importantly, Ava wants to fight!
They then tried steroids as a last ditch effort, this has worked so far, and she is on a low dose of daily steroid. The long term side effects are bone loss, as well as difficulty in growing and weight gain to name a few were some of the severe side effects that she is experiencing, and will continue to suffer. As I stated earlier, she has not gained weight or height in almost two years. Ava was an 11 1/2-year-old being the size of a 6-7-year-old, not to mention she already has osteopenia (bone loss).
In February of this year, we left our comfort zone of Boston Children's to (where we have gone for over 12 years) to go to Children's Hospital of Philadelphia CHOP. They have an intervention to help put kids with PLE into remission. The procedure did stop the PLE symptoms, and she has been off steroids for almost three months. She is no longer on fluid restrictions, and have been slowly increasing the fat in her diet, which will hopefully allow her to grow, gain weight and thrive.
So far things are going well still in remission from the PLE, slowly getting back to school as was out most of the year due to her weak immune system. We don't know how long the intervention she had at CHOP will continue to work; some of her MDs are skeptical. , but we (my wife and I continue to hope and keep her as healthy as she can be.
She has so much life in her and so much love to give. Please help give back to the hospital that has and continues to help Ava to grow and thrive, to allow her to do the things she loves, dancing, riding her bike. Texting with her friends and just living life to the fullest.