to: HLHS Listserv
from: Krey and Lindy Price
subject: Fontan Report
date: 10-15-01

Thought we’d check in and give a report on Jaedin’s summer, which involved a little more hospital time than we originally anticipated.  Looks like this e-mail got pretty long - I apologize for all of the detail, but here goes:

SEATTLE.  Jaedin has HLHS and had his previous surgeries (balloon catheterization, Norwood, open-heart atrial septectomy, Glenn) in Seattle.  He had been doing great since the Glenn (12/99), but our cardiologist had been keeping an eye on some trouble with his diaphragm as well as leaking aortic and mitral valves.  The problem with the diaphragm managed to correct itself over the period of about a year after the Glenn.  The aortic valve leakage, however, continued to be a problem, so we started thinking about an additional surgery to repair it.  The left ventricle doesn’t really contribute to circulation after the Norwood operation, but the problem here was backflow through the ventricle.  Dr. Duncan had performed Jaedin’s previous surgeries in Seattle, but we got word in April that he had left for Cleveland. 

THE DRIVE.  Our cardiologist referred us to Dr. Hanley at UCSF (and soon to be at Stanford), and we scheduled the valve repair for August.  About two weeks before we had to leave for San Francisco, we got the word that insurance coverage had come through – that was a big relief, since technically we weren’t supposed to be covered out of state through our HMO.  We packed up and made the long drive to the Bay Area (which is actually where we first met 6 long years ago).  We couldn’t have imagined back then we’d be returning under these circumstances, but despite it all, it was good to be back and see old friends again. 

CATH.  We went in for a catheterization on August 6, three days before the surgery date.  They were hoping to get us out the same day, but it turned into a 2-day stay.  They found some additional blood vessels that had formed and needed to have coils inserted into them.  All of the measurements during the catheterization indicated that he was a good candidate for the Fontan, so we decided to have Dr. Hanley proceed with the Fontan as well if things went smoothly with the valve repair. 

FUN.  We had family come from out of town to help us out with our other son (Cambren is fourteen months younger and three pounds heavier than Jaedin).  We had a day to relax before the surgery, so we took the kids to Marine World in Vallejo.  They must have ridden the train ride ten times or more, and they loved the dolphin shows, too.  Parking in San Francisco was a nightmare, and we couldn’t get into the Ronald McDonald house, so we stayed in a motel nearby.  One bonus was that it was near the beach, and the kids LOVED the waves. 

GOING IN.  We were the first case the day of the operation, so we went in bright and early.  The streetcar went straight from the motel to UCSF, and Jaedin got a kick out of his “train” ride to the hospital.  He was in a great mood – he carried his little Fisher-Price medical kit with him the whole way.  When we checked in, he was flirting with all of the nurses as always.  It helped to see him go into it so happy – he waved to us without any concern as they carried him off to the OR, and then the waiting and pacing began for us. 

THE WAIT.  We had been spoiled by the periodic updates we used to get in Seattle through a pager – this time, it was just one long wait.  Finally, I ran into Dr. Hanley in the hallway as he was coming out of the OR.  Everything had gone well – he had repaired the aortic valve and had been able to slip in the Fontan while Jaedin was coming off of bypass.  I thought it would be a huge relief to hear that, but I knew there was still so much more involved in the recovery that I found it hard to feel relieved.  Before the surgery, everyone had thought that the leakage through the aortic valve was a result of a tear from the balloon catheterization Jaedin had had at a few days old, but when Dr. Hanley got a good look at it, it turned out the valve had a deformity.  It was described to us as being two quarters and a half, rather than three equal flaps in the classic Mercedes symbol shape.  The two quarters got sewn together, so now it is essentially two halves.  The repaired valve prevents backflow without completely sealing off the left ventricle, which was the original goal.

A SCARE.  They brought him down to the ICU late that afternoon, and after about an hour of setting up, we were able to join him in his room.  They had him on a pacemaker, which was a new thing for us – he had never come off a surgery on a pacemaker before.  Not too long after we got there, we watched his heart rate start flying on the monitor.  150…200…250 – a whole team converged on the room like in a scene out of ER.  They were trying to adjust the pacemaker rate to pass up his own rate and then scale it back gradually – he hit 300 before they were able to bring him back down.  When they yelled for someone to grab the paddles we about lost it.  We just stood back and prayed – not much else we could do at that point.  Twice more he did that (each time without going into fibrillation) before things stabilized.  The paddles were just precautionary, and Dr. Hanley came in later on and told us he wasn’t concerned – that it’s just something they have to do sometimes to get the rhythms straightened out.  Still, I imagine you can’t keep up 300 beats a minute for long, so we were relieved that was the end of it.  Day 1 and 2 after surgery were fairly uneventful – just a lot of monitoring while Jaedin was out cold and intubated.

HEARTSTRINGS.  Dr. Hanley wasn’t completely satisfied with Jaedin’s saturations, and he thought maybe closing up the fenestration would help.  We had been told that in some kids, the fenestration closes on its own eventually, but that we may (50/50 chance) need to go back in for a catheterization six months to a year later in order to get it closed.  During the surgery, Dr. Hanley had embedded a little string just below the incision that could be used later on to cinch up the fenestration if necessary.  On Day 3, they did a bedside operation and made an adjustment to the fenestration.  They pretty much closed it off, and the saturations came up a little.  They were expecting a bigger jump, though, so they started looking into other issues. 

DIAPHRAGM.  They backed off on the drugs a little after extubation (Day 4), and it was good to have him looking at us again.  Things had begun to stabilize, and Cam went home with his grandparents.  With Jaedin breathing on his own, the chest x-rays showed that his diaphragm had taken another hit during the surgery.  The same side that had been giving him trouble before was at it again.  One side essentially fights the other by rising while the other side is falling.  In anyone else, it wouldn’t be such a big deal, but in single-ventricle kids, they need all the air they can get with each breath, so it’s really working against him. So now they were thinking the saturation problem may be due to the problem with the diaphragm.  As long as it doesn’t visibly affect him, there probably isn’t anything they would do about it.  One option is to surgically tack down the misbehaving diaphragm so that it doesn’t oppose the other.  It still wouldn’t be helping much, but at least it wouldn’t be fighting against the other one.  Jaedin seems to be acting just fine, though, so I don’t think we’ll end up doing anything about it.  His baseline runs around 92 now, and it seems like that’s just where he wants to be.   

NEW SOLUTION.  After over a week in the ICU, he was still on the pacemaker.  They were waiting for his underlying rhythm to take over, but it was still too low every time they tried turning down the pacemaker.  We had heard that after two weeks or so they start talking about a permanent pacemaker to send you home with, so we were getting real concerned.  He wasn’t eating, either, at least not anything other than Popsicles.  Then I tried giving him some ice cream.  He immediately threw it up, and his heart rate jumped up to just above the pacemaker rate.  From that point on, it never dropped below the pacemaker again, so they took him off it a few days later.  Maybe we stumbled onto a new solution we should submit to some medical journal.  His liver also took a hit during the surgery, but it gradually improved during the recovery.

THE ZONE.  Things kept improving, and he was getting weaned off the gadgets one by one.  He was so sick of the ICU and so anxious for a change of scenery, though.  It was really getting to him.  They were having a real tough time getting any blood through the IV’s.  Each draw dragged on and on with multiple attempts – it was a struggle for everyone and left Jaedin in “the zone” for hours, playing with his fingers with a blank stare - not acknowledging anyone.  I think it was Day 10 before he even cracked a smile – and that was from something he saw on a video, he still refused to acknowledge us.  Finally they let us put him in a cart and push him around the ICU.  That helped his spirits a little, but moving around was painful for him because of his chest tube.  He had gotten rid of two of the three original tubes real quickly, but he was having trouble with the last one – it just wouldn’t quit draining, so they left it in for quite a while.     

THE FLOOR.  Day 11 they pulled out the last chest tube and we finally got out of the ICU.  He was able to stand up a little, but still very cautious and timid about everything.  We set up his train set on the floor, and he spent most of his time pushing his trains around.  It was nice to see him slowly start to acknowledge us again.  From there things improved so quickly that we were able get him cleared for going home the next day.  We did end up getting into the Ronald McDonald House halfway through our stay – that was a big blessing.  It’s a couple of miles from the hospital, so it wasn’t real convenient, but we hadn’t left the hospital complex in almost a week, so it was good to have somewhere to go and sleep as we traded off shifts.  So anyway, we cleaned up at the Ronald McDonald House and got ready to leave the next morning.

GOING HOME.  We took our time on the way home and ended up doing it in three legs, since Jaedin was still pretty uncomfortable in his car seat.  The car did great with our brand new radiator.  One complication I forgot to mention was a blown radiator in our van on Day 5 that left me stranded in the East Bay – but that’s a whole other story I won’t go into.  We thought Jaedin and Cam would be happy to see each other again, but after a quick (forced) hug, they were both screaming at the top of their lungs with a death grip on the same toy train engine.  We realized that they had both been getting spoiled separately for a while, so they had forgotten what it was like to compete for toys and especially for attention. 

FOLLOW UP.  We had some follow-up visits over the next couple of weeks with Jaedin’s cardiologist in Seattle.  He was tracing some pleural effusion that was starting to worry him, but it diminished after a few weeks.  Jaedin was getting back to himself, and his incision was all healed up.  I had to go to a conference in Utah for work (I was lucky to still have a job after all the time off), and since I’ve got family there, we were debating whether to bring Jaedin along to see everyone.  The cardiologist felt that the high altitude might be a little tough on Jaedin at that point, so we decided against it.  Jaedin’s last checkup was great, though, so we felt comfortable dropping him off at Grandma and Grandpa’s place in Eugene, OR while we went to Utah for a few days. 

UTAH.  The day after we got to Utah, we got a call that Jaedin had a fever - it went away the next day, so we didn’t think too much of it.  We had left our sat meter with him, and he was holding right at 92 as always, so we weren’t too concerned.  Then the fever came back again and we started getting worried.  His grandma told us he just wasn’t acting like himself.  We had her take him into the ER – they did some blood tests, but couldn’t see anything wrong, so they sent him home.  His sats were still fine, but he still was just lethargic, and his fever was getting worse.  So we decided to cut our trip short and head back.  Meanwhile it was the aftermath of the September 11 chaos, and no flights were going, so we ended up driving straight through – we were still a couple of hours away when we got word that the blood tests showed that he was fighting an infection.  His incision site, which had looked good the night before, was now turning red and swelling up.

DOERNBECHER.  We went straight to the ER in Eugene, where they put him on antibiotics.  His saturations were dropping, the lump on his chest was getting bigger, and he was just miserable.  They weren’t really sure what to do with the incision, since noone there was really familiar with HLHS – so they sent us up to Doernbecher Childrens in Portland (100 miles away) in an ambulance.  By this time, it was 4 am, and when we got to Doernbecher, his chest was bulging.  The doctors looked at it and decided to take him right up for an operation.  Meanwhile the bulge had started draining - we knew he was fighting a major infection, but we didn’t know how deep it was.  The uncertainty about what was going on with Jaedin, combined with the long drive, no sleep, and all the other tragic news coming over the radio, left me about as drained as I had ever been. 

NOT AGAIN.  They took him into the OR to open up the incision again and see if they could tell how far it had spread.  That was scary, since for all we knew, they might come back and tell us it had spread all over his heart.  Luckily, though, Dr. Ungerleiter came back and told us it looked like the infection was pretty much contained just below the incision, and they weren’t going to go any deeper at that point.  The string that was attached to the fenestration did look infected, so they took that out.  Also, some of the coils that had kept his sternum together while it healed were infected, so they took those out as well.  They decided to leave the incision open this time and let it heal on its own over time.  It was tough to see, since without the coils his sternum was separated – reminded us of when they brought him back after the Norwood with his chest open. 

IV.  They got all the results from the lab and were able to get a more targeted antibiotic.  The antibiotic masks any sign of infection, though, so there is still a chance that the infection is deeper in the heart.  The only way to find out for sure is to see if anything flares up when they take him off the antibiotics, which could be as long as a year from now.  If the infection got into the Gore-Tex, it’s pretty much impossible to get rid of it, so there’s a possibility they would have to redo the Fontan to replace the shunt.  We’re obviously hoping it doesn’t come to that, and all signs right now point to a full recovery.  The speculation is that the infection may have begun on the fenestration string, but there really is no way of determining the specific source.  All you can do is throw antibiotics at it.  They put in a PIC line for the IV antibiotics a few days after the operation.  It was a struggle to get it in, but once it was there, it sure made things a lot easier.  They were able to do most of the blood draws through the PIC line, so that saved him lots of grief.

HOSPITAL FOOD.  After a few days he finally started eating again, but he had developed some really strange tastes to make his hospital food more interesting.  My favorite was the fruit loops in chicken noodle soup – he insisted on it, so I thought, hey it’s the one thing he can have control over, so why not.  And he loved every bite.  It was tough to see him back in the hospital just when we thought he was done with all of that.  We spent five days or so in the ICU and then moved to the floor.  Once we were on the floor, the main thing they were waiting on was for us to get comfortable with the dressing changes and the new routine.  The opening was huge, and I didn’t think there was any way it could close up on its own, but the doctors were thinking it would only take a month or two.  We passed off on our dressing changes after two days on the floor and drove up to Seattle to meet with the home health nurse.  She walked us through the IV lessons and gave us a couple of big boxes of supplies (just one week’s worth!)  They want him on IV antibiotics four times a day for about six weeks, and then they hope to switch him to oral.  The toughest part about the new routine is the 3 am dose of antibiotics, especially since we have to flush it before and after, and the actual infusion can take up to an hour. 

ROUTINE.  We were worried how all of this would affect Jaedin psychologically.  He had already been through so much this summer, and now he hated his new meds and would cry every time we would start the infusions.  After the first couple of days, though, it was miraculous how he adjusted to his new routine.  He actually looks forward to his infusions now, and he’s proud of his pump, which has become his toy rocket ship all covered with stickers.  We made him a special little backpack carrier that he takes along with him.  He does his own flushes by pushing on the syringes himself, and he takes his meds voluntarily again.  The dressing changes got less and less painful, and sure enough, his incision is pretty much closed already.  We hope to get him off the IV next week.  I couldn’t believe how he adjusted to everything and got back to his own happy self so quickly.  Seems like in the big plan these kids get an extra dose of resilience (and hopefully some amnesia as well).

MOVE.  Another tough part of all of this is the timing, which hit right in the middle of a job transfer and family move to Portland (we’re finally closing on a house this week!).  You can imagine the insurance issues that come up when you switch in the middle of home treatment…  We’ll be switching to the clinic at Doernbecher permanently now, so it will be good to finally get some consistency going in Jaedin’s treatment.  With all of the facilities that have been involved so far, we’ve had lots of opinions as far as medications, doses, desired PT/INR and sat levels, how to approach the diaphragm problem, the antibiotics, and everything else.  It’s time to settle down now, and it sounds like Doernbecher is a good clinic for long-term care, so we’re glad to be in good hands.

WRAP IT UP.  So anyway, sorry this got so long, but I thought I’d pass on the details in case something here applies to any of you.  The moral of this story is to watch out for low-grade fevers that don’t have any other symptoms along with them, even if you think you’re in the clear after the operation.  They always tell you that any operation comes with the standard risk of infection – now we know what they mean.  We’ve met some of the other people on this list during our hospital stays, and it feels like we already know your special kids.  It’s great to have this group to share experiences with.  It helps prepare you to see what you might be up against, and you see you’re not at all alone.  In the last few months in the hospital we’ve seen kids that had strokes during pretty routine procedures, and we’ve seen other kids breeze right through really complex operations – you just never can know what to expect.  You see some kids come and go while you’re stuck there and you feel unlucky or envious.  Then you feel lucky and guilty at the same time when you get to go home and come back for follow-up a few weeks later to see other kids who never left.  But we all face very similar challenges and emotions with these kids, and the main thing is to be grateful for what we have and to keep hoping for the best with our kids.  If anyone else has experience with infections or misbehaving diaphragms, we’d love to hear from you.  Take care and keep up the good work.  God bless,

Krey and Lindy Price
parents to Cam (1 1/2) and Jaedin (almost 3, HLHS)