Our Fontan Journey
So, here it is. The long version of the story, our Fontan journey, notes for moms in the heartland, and what's next.
It was in the early afternoon that Dr. Spray came out to tell us all had gone well. We went back to see Paul and I was cautiously optimistic. He woke up, asked for ice and squeezable fruit. The attending came by and said that he was doing so well we may be going to the step down unit in less than 24 hours. He was breathing funky- like fast and weird. I asked about it and they said it was normal (it is-its called tipping and can happen from anesthesia). I asked to see his post op X-ray and noted that it looked different- not horrible but just different. I kept asking over and over- assuming something had to go wrong. You see, for as long as Paul has been alive I have had this sense that we were dealing with more than just hlhs. This sneaking suspicion that something wasn't quite right. Paul's whole life he had a chronic cough, weird breathing, funky episodes of desaturating, and was hit hard with viruses. Before the Fontan I prayed to God and I said: "You and I both know something's about to go down. If it's that I have been crazy all of these years- show me that by letting him rock this surgery. After all, I will own that in some weird way I'm just crazy from all of this and that's what's happening. If it's true- if I really am crazy- I'll go home and go to more counseling and compartmentalize these feelings really well just as I always have. If my feelings over the last years have been about the fact that somehow I just knew you would take Paul from us- do that. I know he'll be safe with you but don't put our family through months in the hospital, just let him go in peace on the operating table. And God- if he's going to have PLE or plastic bronchitis or some crazy complication reveal that to us immediately. Don't send me home with a baby that's a little off and may have some lung issues (pb) or tummy issues (ple)."
I was 33% sure one of those three things would happen- 1/3 rocking it; 1/3 passing on and 1/3 crazy complication (and 1% not knowing at all) Creepy, right? Spell binding, amazing and a little weird. It gives me chills thinking about it now. My intuition was so powerful; God had been preparing me for this all along.
We went to a private icu room and immediately things started to change. He had fallen back asleep and wasn't waking up quite right, he was swelling. Not normal post surgery swelling. One side of his body was swelling and the other was not- a clot maybe, just the way he was lying maybe... It got worse- like something out of the twilight zone worse. By 12am his whole left side of his head and under his arm looked like another person was growing off of him. That night and into the morning a reinitbation, a head ct scan that revealed a small stroke, a cath, a stent in the artery..."this may fix things," they said. It usually fixes things.They were wrong. He couldn't come off the breathing tube. He crashed that night and had 6 minutes of CPR. I was sleeping, about 8 blocks away and he almost died- I raced in the middle of the night to the hospital like something out of a movie. I told him to let go if he needed to. By morning it happened again- he started to crash and then they pulled out something from his breathing tube that some of them were calling a "cast". A cast. A cast is like a huge mucous plug- it's a fibrinous piece of material that's rubbery and looks like a little tree (it is actually formed like the bronchi that is in the form of a tree) that blocks the airway. Casting= super rare complication called plastic bronchitis. Never in the history of the fontan surgery has plastic bronchitis developed this soon after the fontan- even then it only effects a very tiny group of kids and not in this way- they don't get sick this quickly.
During the net few days docs would extract 2 more casts from Paul's breathing tube. Docs ordered treatments for plastic bronchitis to begin. A precursor vest, tpa, albuterol, saline, doronsase- you name it they threw the book at him. One doctor (Roxanne Hirsch) stayed up with Paul one shift for 24 hours- adjusting his ventilation settings just to get him to a place where we were not about to lose him again. We were helpless and they- the best doctors in the entire world- the doctors that are never stumped, were stumped.
If something has never ever happened in medicine the likelihood of it happening is like basically nothing. It came time to make a move- the longer Paul was intubated the worse he was getting. The Fontan circulation depends upon passive blood flow to work and the ventilator works against that- increasing pulmonary pressures. High pressures are no good in anyone but especially not in these kids. Pressures of less than 15 are ideal- Paul's they'd guess- were in the high 20s. The higher the pressures the worse he'd get and the more support he'd need and that would increase pressures and so is the cycle of the intubated Fontan patient.
We were going to try to extubate him- just pull the tube and see how he did and then a course correction took place. The swelling that Paul experienced was likely due to lymphatic drainage. A procedure done in only 6 other fontan kids would be preformed on Paul. The theory is that as pressures increase, lymphatic production increases. Plastic bronchitis is lymphatic leak into the lungs. In this procedure they plug the leak with a special glue called lipidil. If they could find and plug the leak the system would reroute and drainage into the lungs could be stopped-- maybe forever maybe for a day or two maybe for years. If they couldn't find the leak, they'd take down the fontan thereby decreasing pressures and preventing further casting.
That morning Paul made history as the first child to undergo a lymphatic angiorgram who was that sick and the seventh in the world to have it period. It was a long shot but we had to try. Several hours into the procedure our cardiologist came to talk to us. They had tested Paul's lymphatic system and learned that it was extremely abnormal. To start, his thoracic duct (the main thing that lymph fluid lows through) was occluded. As in not a part of the equation- not working. They used a special dye to determine where and how fast the fluid was going. His lymphatic production was much more active than it should be and it was leaking into his pulmonary system. Nothing was spared, his lungs were flooded with lymphatic fluid. For all the other children they had done this on, they were able to find one or two places where a leak was occurring and plug that leak. Paul had more than one or two places that were leaking- he had more like thousands. It was something that had never been seen before. The doctor pulled up his phone with a picture of the procedure and Paul's lung beds, "everywhere you see white is lymphatic fluid" he said. The whole film was white- the entire picture was the color white.In that moment a calm came over me like I can't describe. There it was-my years of worry staring me in the face in a sheet of white on a little picture on a phone. There it was- that was it- that's what we've been fighting against. That explains Paul's random cough, crazy breathing, asthma- everything. "He's been leaking this fluid into his lungs his whole life" I said..." it's not unlikely" said the doctor. They sprayed the entire lung beds with lipidil in the hopes that it would find the leaks and plug them. Would it work? Maybe- we had to try before bailing on the Fontan.
Did it work? Well, maybe. Paul was extubated a few days later. Paul came off oxygen. Paul started playing and talking and running around. Paul continued to cast- was it old casting? New casting? In between casting? Hard to say. I continue to pull cast like material from his gtube via aspiration that he presumably coughs up and swallows. He's still on a ton of support- breathing treatments, shots, medicine, lots of diuretics, special formula, low fat diet etc.Did it work? Maybe...Paul is throwing mandarin oranges on the floor as I type this and insisted on purchasing a little mermaid sweatshirt yesterday (insert another blog on the importance of fostering confidence in gender identity) and is well enough to have "broken" his toe. His X-ray is clear, heart function is good, sats are fine and he's happy as a clam.
What's next? Medically I don't know... They are not even "sold" that this is all actually plastic bronchitis. We may never know what is really happening/ what really happened and I am not sure it even matters. Maybe we will have a transplant, maybe nothing at all, maybe another intervention. From a living life perspective what's next? We have been cleared to come home to Nashville and live life with our toddler and baby. We are coming home, maybe we will move to Philadelphia in the future but for now home will be right back where it all started. There is nothing that we are doing in Philly that we can't do in Nashville and our home team is more that equipped to handle everything. This year Paul's plans include- High Hopes preschool, tube weaning, pulling his sister's hair, the park, friends, family, a real Christmas tree, therapies, south end church, doctors visits, laughter, movies, travel, toys and love.
What did I do to deserve this? I think about that all the time but not in the usual way of thinking that phrase. What did I do to be trusted with this little soul? What did I do to deserve this man and little girl that love me so much? What did I do to deserve the outpouring of love and support of our parents and from the medical, online, local and heart communities? What did I do to deserve you Diana? Chandler? Allison? Jen and Lee? Jen and Jeff? Beth? Nick? Christian? Tommy and Stacie? Stacey? Jen Fultz and so many others...What in the world did I do? I don't know, but I do know this- I am God's daughter, and He picked Dan and Paul and Georgia for me and me for them and I couldn't be more thankful. I'm not anywhere close to deserving His grace and this life but I am so thankful to have it and plan to live it as fully as possible. All the while, I will never forget the amazing gifts I have been given.
I still struggle to be the version of myself that the world deserves to see. I still can be horrible to people when I am grieving as a means of self protection. I am demanding, anxious, unrelenting and have unreasonable expectations sometimes. I eat cookies - like all the time- to cope with stress and some days I hate being alone. I talk way too much and intellectualize everything. I often wish we had more money and a bigger house and I used to question when children died in the heartland why God needed so many sick babies...I am perfectly imperfectly made but for that matter so are we all- i'm such a work in progress and this journey with Paul has shed light on that.
As my friend the infamous Miss Kitty who has a few years on me and is, as such, wiser than I am often says to me "you have it all." Indeed I do. The truth is this, my measure of success as a mother, wife and human being is about nothing else other than how well I love. I am so lucky to get to love as I do, who I do...
To my sisters in the heartland...I am no expert but I am a heart mom, too. When things like this happen in the heartland I find myself scanning the blog or update for what's wrong and thinking "will this happen to Paul?" And then praying for the baby. In an effort to ease some of the anxiety that you may be feeling, I put these notes together for you (they would be what I needed to read if I were you)
1) Pay attention to your feelings but don't let them overwhelm you. My gut was different than anxiety that something was going on with Paul. Even though I was "right" and this perhaps seems like some sort of evidence that all worry results in crisis-it isn't. Not even close.
2) Even though my feelings were spot on two things should be noted a-Paul was not in immediate danger during his period as a Glenn so nothing needed to be done right then anyway and b- I compartmentalized those feelings to be a good mom to Paul most of the time. The times I didn't set it aside were days of unease, googling, doctors visits, constant monitoring, and tears- those are not the days I remember best as a mom. The days where I set stuff aside and just lived life were the days I cherish and will continue to cherish.
3) the lymphatic system plays a huge role in our hemodynamics. They have only been studying this system for under a year. The theory at chop is that lymphatic abnormality is present in many kids and for a few of them is what results in ple and plastic bronchitis. This happens in a very very very small percent of kids and there are ways to treat it medically, surgically, and through transplant. This does not mean that every hlhs child with a lymph node that is swollen is doomed to a fate of complication- far from it. Nor are all kids with a chronic cough subject to a similar fate. I do think if you experience ple or plastic bronchitis complications you should connect with docs at chop- they are amazing and if nothing else will talk to you about what you are doing to manage issues as they arise.
4) Our kids will tell us if something is wrong. Paul's course of complication has not been subtle at all. I didn't need to watch his every breath because it was obvious to me that something was up when it was up.
5) every single medical professional that has cared for Paul has his best interest at heart- these people have moved mountains, lost sleep, prayed, called, loved, and spent countless hours with us to treat our whole family. Most notably Dr. David Parra and Karen Kohl Vanderbilt and Dr. Jack Rychik and Katie Dodds at chop are our quarterbacks in this game.
6) Now, stop reading this... Yes, you! Put down your phone or whatever and go do something beautiful- God knows you deserve it
7) I am certain that we will all be ok- no matter what happens- as sure as day turns into night and night to day-we will all be ok.
Congenital heart disease effects up to 1 in 100 children each year. It is the most prevalent and under funded issue in pediatric health care. Consider donating time and resources to a childrens hospital, the childrens heart foundation, or sisters by heart.I am humbled and grateful to each of you for reading this and loving us.