Steven and I went up to Philadelphia on Sunday, April 17. Kayla Ball (a girl from our church who has babysat for us since Hannah was a baby) kept Hannah and Benjamin for us while we were gone. They were so excited to stay with Ms. Kayla and funny Mr. Ryan (her husband). We were so thankful that again we could leave them with people who loved them and kept them happy and busy while we were away. Thanks to Kayla and Ryan for keeping them for us. Thanks also to Lisa Heller (Kayla's mom) for letting them come over and play at your house and jump on your trampoline.
The first appointment we had on Monday was with the cardiologist. She looked at his echocardiogram from his discharge from the NICU and said everything looked great. We told her about the blue spells he had been having, and she said that it sounded like it was his acid reflux causing it. She said the surgical team would deal with that part of his treatment. She listened to his heart and lungs and said everything sounded great. We will see her again in November and they will do another echocardiogram and an EKG then, which is just standard for the Pulmonary Hyperplasia Program he will be in (I will explain more about that program later).
Next we saw the surgeon who performed his surgery, Dr. Adzick, and his medical team. His nurse practitioner talked to us first. We told her what had been going on since we were home. Then she shared what we told her with Dr. Adzick, and he came in to exam Jeremiah. They both said that the reflux was causing the blue spells. They said that they would modify his medications to help with the acid reflux. They decided to take him off of the Nexium (which is what they switched us to when we stayed overnight in the hospital at CAMC). He was put back on Zantac and they added Prilosec. They are two acid reflux medicines, but they both work in different ways. The nurse explained to us that it is not unusual for babies with CDH to be on multiple medicines for acid reflux. Acid reflux is very common in babies with CDH and can be very severe. From talking to us about his symptoms and watching Jeremiah after he eats, multiple doctors that he has seen say that he has severe acid reflux. Once the acid reflux gets under control the doctors are hoping the blue spells will stop. Dr. Adzick examined him and was happy with how he was healing. He brought up how well he did in the NICU, and he told us that Jeremiah is "probably some kind of record breaker". This is from the doctor who has been at CHOP performing these surgeries since the early 90's. Hearing that from him just confirms how much of a miracle Jeremiah is. It also is a confirmation of the power of prayer. The one thing Dr. Adzick and his team are concerned about is Jeremiah's weight gain. He is gaining weight pretty consistently, just not as fast as they would like. We talked to the nutritionist on the team about it, and she gave us some good steps to take with feeding him that should help. Dr. Adzick also decided that he wanted to see Jeremiah again in a month. So on May 23rd we are going back to CHOP to see the surgical team again.
Next appointment was with the pulmonologist. He talked to us in detail about what had been going on with Jeremiah since birth and he examined him. He was of the opinion that the blue spells were caused by the acid reflux as well. He explained very well why he thinks the blue spells are happening. When Jeremiah has acid reflux come up it makes him upset and makes his blood pump more. When his blood is pumped faster (when he is worked up or irritated) some of it does not get oxygenated because of his low lung function. He is also shunting in his heart because of a small hole in the septum of the heart that all newborns have. Most of the time the hole closes as the baby gets older, but in about 25% of people it doesn't. In most all cases when it doesn't close it isn't a problem, and there are adults who still have the hole but are fine. But in Jeremiah's case it is a compounding issue. The pulmonologist said it was taking part in causing the blue spells as well. So they are just going to keep an eye on it. The cardiologist said the hold was very small, so she did not forsee the need to fix it. If it does have to be fixed in the future it will just be done with a catheter. The pulmonologist also added another medicine for Jeremiah to take. It is called Bethanechol and it helps the stomach process the food faster. That way the food does not sit in the stomach as long and cause acid. It also strengthens the muscle in the esophagus.
We left the hospital happy with our appointments and the answers we got. We are thankful for CHOP and that we chose them for not only the birth, but for his after care as well. They have dealt with hundreds maybe even thousands of babies after CDH surgery over the years, so they know what works and what they need to be doing to thrive. We are hopeful that the new medicines will make a difference and help him not be so miserable after eating.
Many people have asked questions about how often we will have to go back to CHOP for appointments and for how long. Jeremiah will be taking part of CHOP's Pulmonary Hyperplasia Program. This program is the only one like it in the country for children with pulmonary hyperplasia. Pulmonary hyperplasia is basically incomplete development of the lung and Jeremiah has it because of the CDH. If pulmonary hyperplasia gets bad enough it can cause pulmonary hypertension, which affects the heart. That is why he will be seeing a cardiologist over the years. The PHP (pulmonary hyperplasia program) follows children with pulmonary hyperplasia caused by CDH and other birth defects from birth till they are 18. After this year, we will just have to go up to CHOP for the program once a year. Since he is doing so well and right now shows no signs of pulmonary hypertension, we are hoping that maybe he won't have to be in the program until he is 18. He will be in the program for many years, though. The pulmonologist told us that pulmonary hypertension is best diagnosed around the age of 8 or 9. This is because the lungs continue to develop up to the age of 8. So we will definitely be a part of the program until he is 10 or so. When we go to CHOP for the program Jeremiah will see many doctors all in one or two days. He will see his surgical team, the cardiologist, the pulmonologist, the nutritionist, a speech therapist, a physical therapist, and a pediatrician to follow his development to make sure there are no developmental delays. They will do blood tests, chest x rays, echocardiograms, and any other tests that they deem necessary on these days as well. Our first PHP appointment is the first of November. This time it will probably be two days of appointments because of the amount of tests that are going to be done. Also, he will be seeing the pulmonologist here in WV every year 6 months after his PHP appointment at CHOP. He may also have to see a cardiologist here. That is going to be what his continued care will look like for the future.
Since Jeremiah's appointment at CHOP he has been to see his pediatrician here twice. He is still going once a week to get his weight checked. At each appointment he has gained weight very well. He gained 7 ounces the first week and 6 last week. The pediatrician is very happy with his growth and his overall health. He has not had any more prolonged blue spells. He still gets blue around his mouth a little bit after he throws a fit or after bad reflux, but it doesn't last as long and hasn't been happening as often. I think the medicine is helping, but he is still very uncomfortable after eating and refluxing quite a bit. I hate seeing him so upset after eating. Many times he just starts crying, arching his back, and wiggling uncomfortably during breast feeding. Pray that his acid reflux get better and the medicines do their job. Also, we are having trouble getting him to take his medicine because it tastes so bad. He hates it and screams, cries, and spits it out when we give it to him. We have tried flavoring it, putting it in breastmilk, and putting a hole in his pacifier and giving it to him through the hole. Nothing has worked. So we just give it to him little by little and do our best to make sure he doesn't spit it out. Pray that we get all of his medicine in him when we give it to him. He is taking medicine before 7 out of 8 of his feedings. Thanks for asking about Jeremiah and keeping up with his progress.
