What we thought:
During his May admission, once his heart rhythm was more stable, the focus shifted to his growing abdomen. After an abdominal ultrasound he had a liver ultrasound. Then he had a more specialized liver ultrasound. Then he had a CT. Then a liver biopsy. From what the team had seen on ultrasounds and the CT, they were fairly certain he had an advanced case of liver cirrhosis. They wanted the biopsy done to confirm that and to show what stage of cirrhosis he was at. Liver cirrhosis is a disease of liver damage from a variety of causes which lead to scarring and liver failure. There's no reversal of the damage done from cirrhosis. Kids with Eli's type of circulation (also known as Fontan circulation) are known to have liver problems later in life, but cirrhosis is rare. His gastrointestinal team, cardiology team, and the hospitalists had never had experience with a Fontan kid having cirrhosis. The research they did yielded very little help. It looked like Eli was once again blazing his own medical trail and doing things no one else has done.
{Let me get off track here just a bit and say something about his heart and treatment so far. The heart surgeries Eli has had done are still relatively new in medicine. Twenty five years ago, most babies born with his defects were sent for a transplant right away (which gets rid of the defective heart, but brings about a whole lot of other problems and issues to deal with in addition to the fact that a transplanted heart doesn't last as long as a natural heart....studies estimate a heart transplant averages 13 years). If the baby wasn't a good transplant candidate, then they were made as comfortable as possible and let nature take its course. As time passed, less and less baby hearts were available. Why? Because of stricter car seat laws. We were keeping our kids safer in our vehicles (which is a VERY good thing!) which meant fewer infant/child deaths which in turn meant a major decrease in the availability of donor hearts. Some surgical options had been developed and trialed in heart defect children, but weren't routinely used. With transplant options becoming less prominent, these surgical options became more prevalent. My point with all this is, there are not a lot of adults living with Fontan circulation. Most of the patients are still children. We don't have a large medical base to study the long term effects of Fontan circulation on other parts of the body. So although we do know liver disease is common with Fontan kids, there's still a lot of research going on to discover other negative effects or problems the Fontan will bring. However, we don't regret undergoing the Fontan. The goal for Eli has always been have this series of surgeries which is believed to help get him to his late teens/early adulthood before needing a heart transplant.}
Back to now, we don't have other cases or research to compare Eli to. But the fact remains he is suspected to have severe liver cirrhosis based on his radiology studies. The CT and ultrasounds showed that his liver was hardening and had many nodules in it. The good news is that the ducts going into and out of the liver showed good flow and his liver function tests were good. But we've always known Eli's body compensates for many defects very well, so the fact they thought he had those good things didn't mean the bad things weren't happening. One doctor even had a discussion with me about the immediate plan for Eli once the liver biopsy results were in. This talk was only two days after the biopsy when we had no more information than we did two days before the biopsy, so in hindsight this talk was a very premature discussion that I now believe she did not need to have with me, but I'm sharing here to further explain how serious and worried we were at the time. She said Eli would need to be listed soon for a liver and heart transplant. The reason for both was to have both organs come from one donor which would greatly minimize the risk of rejection. If he were to be listed, it would happen in the next few weeks (as in yet this summer). PMCH does not do pediatric transplants. In fact, if Eli needed to have both done, not many places do that. We were likely looking at going to Cincinnati or Philadelphia for an undetermined amount of time for him to be listed, wait for the perfect donor, then have the surgery, and begin recovery from that. So not only were we facing a very uncertain medical future for Eli, but we thought our family would be further broken up for a long time as one of us (probably me) would move with Eli and the other stay with Zachary and Charlotte. All that in addition to having just moved and our old house still being on the market (had an offer 11 days after listing. After working on that deal for six weeks, it fell through just prior to closing so it is back on the market and we technically still have two homes).
And that's not all that was going on. When Eli was to have the liver biopsy, he also had a lymph node biopsy. Yep, more concerns about Eli's health. Through all the imaging that was done, it was discovered that he had several enlarged lymph nodes. The teams wanted to biopsy one of those to rule out lymphoma. That's right. There was a slight concern that Eli could be battling cancer of the lymphatic system. Overwhelmed doesn't begin to describe the emotions Bryan and I were feeling. There was too much going on, too many unanswered questions to explain publicly at the time.
What we know:
On Wednesday, May 23, Eli had a big day. He had a heart cath, liver biopsies, lymph node biopsies, and paracentesis. So he had a cardiologist, an interventional radiologist, a pathologist, and an anesthesiologist in the procedure room in addition to the staff of nurses and technicians necessary to accomplish everything that needed to be done. None of his procedures were actually that difficult or dangerous for him, but just scheduling all that together is so complicated. Major kudos to the hospitalist staff and others involved who made it all happen for our little guy. He tolerated everything very well. We had the heart cath info immediately from Dr. Parikh and it was all good. Eli's Fontan was intact and working just as it should. Eli has several (many, many!) collateral veins that have grown off of some of his major vessels. Sometimes those are closed off with coils, but Dr. Parikh said Eli's are too small and too numerous to do. Also he said if the body grows them and we close them off, the body will grow more. He doesn't believe these are hurting Eli and saw no reason to spend hours coiling each of them, even if he could. Any other problems he saw from the cath were things that he thinks can be corrected or at least improved with a pacemaker. Overall, it was great heart news. We knew the biopsy results would take many days to come back so we were happy with what we knew.
A few days after the biopsies were done, he was sedated again. He had more fluid removed from his abdomen for testing as well as drainage of an abscess located deep in his abdomen. We had to be sure he didn't have any active infection going on before proceeding with a pacemaker in the future.
As answers came in we confirmed Eli has no active infection going on in his body. At an appointment with Eli's regular GI doctor (not the one who had the transplant talk with me), we got the biopsy results. We actually had the results before that so I had a good idea of what was or wasn't happening, but we didn't have results as far as how they pertained to Eli and what the next step was, so we didn't share them yet.
The liver biopsy showed liver congestion and mild fibrosis...NO CIRRHOSIS!!!! Fibrosis is scarring of the liver, but it's an early stage and Eli's is classified as mild. What damage has been done is not reversible, but if we can alleviate and control some of the liver congestion, then the progression can be significantly slowed. So he does have liver damage, but honestly the news we got is basically best case scenario. I made it a point to discuss with her the conversation had with the other doctor about transplant and she confirmed my thoughts that we are no where close to needing to go that route for Eli. Although it may be in the cards for him one day, it's not anything to worry about at this time or likely even in the near future! The lymph node biopsy also did not show anything of concern so we were blessed with amazing news on many of our fears!
What's next:
It is believed that the liver congestion and resulting fibrosis are related to Eli's heart rate and rhythm. He is still in his slow junctional rhythm and also has some breakthrough SVT. Everyone has agreed he needs a pacemaker, but cardiology did not want to do surgery to place one until we knew he had no infection and what the status was of his liver disease. Now that cardiology has clearance (and even encouragement) from Infectious Disease and Gastroenterology, they got to work getting surgery set. Of course it's not that simple as picking a date on the calendar. I do not envy the scheduler in their office. This is the third time this poor person has had to coordinate all this for him. As of now, he is set for pacemaker placement the week of July 23. The day before his pacemaker placement, he will undergo a few tests. One will be another paracentesis to drain fluid from his abdomen. We'll talk about that more a little later. The other will be a test by Dr. Steinberg, his pacemaker doctor, to further investigate Eli's heart rhythm. He wants to see if there's a chance Eli could have a pacemaker with just one lead. The significance of this is pretty major. If one lead would work for Eli, then the surgery to place it is a lot easier than if he needs multiple leads. Of course we want what's easiest for Eli, but what's easiest now may not be easiest in the long run. I'm curious to see what will happen.
While Eli was in the hospital in May he had three paracentesis procedures done. Nearly seven liters of fluid was taken from his abdomen. Because test results were still pending, nothing was done about the cause of the fluid, so we knew it would come back. And it has. At his GI appointment last week the doctor said he needed it drained off. She waited a while to see if we could coordinate with cardiology to have the paracentesis and pacemaker closer together. Now we know pacemaker can't happen for another month, so he needs to have a paracentesis soon. Actually she's recommending three between now and pacemaker placement. He has a significant amount of fluid to remove and he will likely accumulate more as time passes. When they took four liters off the first time, he did not do well that night. We don't want to wait and do a large amount the day before pacemaker surgery and risk him crashing and not being stable enough for surgery. That's why she wants to do three between now and then, so they can remove smaller amounts each time.
Eli is scheduled for tomorrow (June 26) to have a paracentesis. He has to be checked in at 6:30am so he and I will leave town a little before 4am. We opted not to spend the night up there somewhere, it just makes more work and Zachary has a ball game we'd like to see tonight. After the procedure, he will be admitted so they can monitor him closely. The plan is to just stay the night and come home on Wednesday. I will pack for multiple nights! He will have another paracentesis done in early/mid July, then a third one the day before pacemaker surgery.
The thought process now is that once his heart is in a regular rhythm with a higher rate, it can decrease the amount of liver congestion he's experiencing, thereby decreasing the progression of fibrosis.
We would appreciate your prayers tomorrow as he undergoes this procedure, for safe travels, and for Zachary and Charlotte. Zachary understands what is going on and is doing okay with it, but Charlotte is not pleased to hear about Mommy and Eli leaving again, especially overnight. She will be fine, but it's a lot for her to take in.
Thank you all for your prayers! Eli is proof they work! In closing, I'll add a few pictures of events that have happened since we've been home. We have been BUSY! Eli has had baseball, Zachary has had baseball, Bryan's sister got married, we've had a family reunion, and we're still trying to unpack from the move. Busy times! But always time to cherish Every Little Beat...
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| Eli and his little league team after their last game together |
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| Receiving his medal from the league president |
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| Not really sure what these three were up to, but they're so cute! |
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| We went shopping for me to get a dress for my sister-in-law's wedding and these two had to have a ride :) |
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| Eli with my mom and sister at the Summer Social |
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| Playing ball in the yard one day, these two had to have a rest....on Aunt Kendra |
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| Throwing coins in the fountain at St. Vincent the day of his appointment |
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| Playing with kinetic sand in the kitchen. Not really sure why his foot is in it. |
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| Eli receiving a special blessing from the pastor who performed Kendra's wedding ceremony. He listened intently but did not put down his bag of chips. |
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| At the rehearsal. From left to right: Alyssa, Charlotte, Eli, Gunner, and Zachary. All five were flower girls and ring bearers for the ceremony. |
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| Taking pictures after the wedding. All five kids were very good through the ceremony and getting pictures after. So proud of all of them! Then they danced the night away and provided lots of entertainment! |
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| Not an official photo, but the Veale family in full force. We were blessed with a beautiful evening for the wedding. The temperature was good, the rain held off, and it was gorgeous. |
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| Bryan, Eli, and family friend Tom. |
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| Zachary, Ashley, Eli, and Bryan having some fun at the reception |
