Well, we survived!! Today was Lily's first visit to the Single Ventricle Survivorship Program at CHOP. I can't say enough how lucky our heart kids are to have a program like this at our hospital. What other programs don't realize is that single ventricle defect kids can have lasting effects on other systems in their bodies due to the type of circulation they have. These effects can be very damaging if not evaluated and treated early in their progression. Because of this, the children in CHOP's SVSP program are evaluated by a team of physicians in various specialty areas including Immunology, Gastroenterology, Endicrinology, and Cardiology. They work together as a team to treat each patient and work with the families as the kids grow into young adulthood and eventually transition to an adult congenital heart program.
Lily was first evaluated by Immunology. They follow the kids for any protein losing conditions as well as any other immune system or allergy disorders. Dr. Heimal feet that Lit looked great, no indication of PLE or any other immune deficiencies.
Next we saw Dr. Iyer for Lily's routine pacemaker check. She has about 3 years left on the battery. Not sure whether it will be replaced then, of just removed. We will reevaluate her status once we get closer. We also saw Dr. Ravishankar who will be Lily's new primary cardiologist since our Dr. Donner retired. She was so excited to see Lily as she hasn't treated her since she was an infant in the CICU. She is just as loving and compassionate as I always remembered her to be, and extremely knowledgeable in the treatment of HLHS kids. We are very lucky to have agree to take Lily on as her patient. We will see her again in 6 months for a regular cardiology followup.
We then saw Dr. Mancilla with Endocrinology. They follow the growth of these children. Lily's DEXA scan showed lower than normal bone density and a low PTH. She sent us for repeat blood work, and a repeat urine. If the levels come back low again, she will start Lily on a Calcium supplement daily to hopefully boost them up.
Finally we saw Dr. Rand with Gastroenterology. Their main focus is the liver. Children with single ventricle Fontan circulation tend to have blood that pools in the liver. This can lead to scarring and eventually cirrhosis. They would like to do a liver biopsy on Limy sometime in 2019 to rule out cirrhosis. The good news is that now her liver looks great. It is normal size, soft, and liver studies are normal! All great news!!
The doctors mentioned that Lily looks like a "perfect Fontan patient." They were excited to hear that she is active....dances, does gymnastics, and participates in gym class with the other kids. They switched her liquid Enalapril to a 5mg tablet twice daily, and also added a twice daily aspirin regimen. This will be new for us, but something that has been discussed in the paste a
All and all, it was a very positive visit and we don't have to come back to SVSP clinic for another 2-3 years. Just regular cardiology followups. Below are some pictures from our day.