Recovery

We're hanging out with Magnus in the recovery room. He's still fairly groggy from the anesthesia, but generally seems OK. He has to lie completely flat for 4 hours after the procedure to prevent bleeding where they put the catheter in. The goal is to get up and walk at 7:30 p.m.

Mixed results

The cath Dr. just came back to talk to us, and the results were mixed. The good news is that blood flow to his right lung, which was nonexistent in August, has normalized, and also that they didn't need to do any interventions, which means that we should be able to go home from the hospital tonight. The bad news is that his pressures are actually a little bit higher than they were in August, and the culprit seems to be that his heart ventricle is not relaxing as much as it should, which is making the pressure higher in his Fontan circuit. There are some options for managing this with medication. The cath doc said that they would most likely consider a higher diuretic dose, an ACE inhibitor (which he's been on before), and the dreaded Remodulin (this is the medication that must be administered via a subcutaneous pump). We haven't actually talked to our cardiology team yet, though, so it remains to be seen what will actually be recommended to us.

So, not as good as we'd hoped, but not as bad as it could have been.

He's off!

I dropped Magnus off in the cath lab around 12:30 p.m. I stayed there until he had fallen asleep. After that, Iggy and I proceeded immediately to the cafeteria, as we'd been NPO all morning.

Here are a couple of photos from this morning:

In the waiting room.

In pre-op, demonstrating how silly he was going to be after he got his Versed.

Still waiting. After being told that they were ahead of schedule, we were then told that they were now behind schedule because although they were done with the first case, they didn't have a bed for that patient in the part of the hospital he/she needs to go to next. Which is just how things go in the hospital. We're hoping the anesthesia team shows up soon. Magnus just told me that he's hungry, but other than that has been in pretty good spirits.

The cath NP told us she thinks there's an 80% chance we'll be staying overnight. We did pack with that expectation, but I'm still hoping we can get out of here today.

Cath day again

After twice rescheduling due to illness, we are now in pre-op waiting to go back to the cath lab. You never know when you'll actually get to go back when you're second case, but so far things seem to be on schedule (KNOCK ON WOOD). Will update throughout the day!

To be continued...

Alas, we are going to have to reschedule our cath; Magnus has a cold and that can affect his pressures and they want to get the most accurate info possible. The rescheduled cath will probably be in about 4 weeks.

Things are good

I know I have been terribly delinquent in my posting here! This is mostly because Magnus has been doing great.

After Magnus was discharged from the hospital at the beginning of September, we started seeing the pulmonary hypertension team at Stanford once a month. They really wanted us to start Magnus on Remodulin, but instead, we decided to try giving him an oral drug, Bosentan (Remodulin has to be administered intravenously or through a subcutanous pump. We wanted to avoid the pump route if at all possible). The team gave us the OK to start with Bosentan, but they were not optimistic and said that they thought it had maybe a 2% chance of bringing down his pressures. We started on a half dose of Bosentan in September, and at first saw no big change. In fact, we actually ended up being briefly re-hospitalized in October because Magnus developed an upper respiratory infection that seemed to be not a big deal, but then he woke up in the middle of the night gasping for breath. It was extremely scary. We drove him to the UCSF ER, where they gave him breathing treatments, which helped tremendously, and then they kept him in the hospital for a couple of days (really, after the breathing treatments he was perfectly fine and could have gone home from the ER, but better safe than sorry).

Right after that, we moved up to the full dose of Bosentan, and much to our surprise, Magnus showed marked improvement! Overnight, his stamina was dramatically improved and his appetite increased. His teachers and therapists (who did not know about the new drug) all commented about his sudden improvement. We went trick-or-treating on Halloween, which in San Francisco means going up LOTS of stairs, and he walked for more than an hour without complaint, which was just unbelievable to me.

His oxygen saturations also dramatically improved along with the clinical improvements we were seeing. In fact, his oxygen saturations were so high, the team was worried that his fenestration* might have spontaneously closed (an echocardiogram subsequently ruled out that possibility). Since then, he has maintained this improvement and has been growing and gaining weight like crazy. I also think that his paralyzed right diaphragm may have healed, at least partially; when I put my hand on his right side, I'm pretty sure I can feel his diaphragm moving, whereas I couldn't before.

We are scheduled to go back down to Stanford at the end of next week (3/27) for a followup cardiac catheterization. They will measure his pressures and will be able to definitively determine whether his diaphragm is functioning. I'm a little bit nervous, as always, but I will be absolutely shocked if his pressures don't show a dramatic improvement. I will be sure to update here as soon as we have any news!

*The fenestration is a "leak" that they put into his circulation in order to reduce the blood pressure in his lungs. He needed it because of his past and present tendency towards high pulmonary pressures. The higher oxygen saturations suggest that he is using the fenestration less. The hope is that we will eventually be able to close it. That would be good because until that happens, he will need to be on a strong blood thinner called coumadin. Being on coumadin means that he needs to have weekly blood tests, and also puts him at risk of heavy bleeding if he were to have an accident.

Sick

Magnus was back at school all last week, and brought home a beginning-of-the-year cold to share with all of us! We were worried, because that is the last thing his lungs need right now in their fragile state. Magnus was pretty sick for about 3 days with a runny nose and coughing and then seemed well enough to go back to school on Tuesday of this week. I seem to have gotten much sicker than he did and at this point have completely lost my voice, so I'm not sure how I'm going to be able to teach classes today!

His doctor had wanted us to bring Magnus in for a follow-up chest X-ray, especially since he's been sick. We did that last night, and the Dr. e-mailed me to say that she thinks he might have some fluid on his right lung again, but that she wasn't sure because she hadn't seen his last X-ray for comparison. I guess it's at least good that if there is fluid, it must be a pretty small amount, and hopefully it will be unchanged from his last X-ray. If it does look worse, I'm not sure what they will want us to do...maybe more diuretics. Nobody has said the "h" word yet, but I'm assuming it's not off the table. Sigh.

Deja Vu

Magnus is out of his cath. We got to go back and see him around 7 p.m. He was awake and asking for cookies (they didn't want to give them to him right away in case his stomach was still funny from the anesthesia).

The news we got from the cath was not great. His Fontan pressure is too high. It is 20. Optimal is 10–15. This is bad for the long-term viability of his Fontan circulation. The pulmonary hypertension doctor wants to be aggressive and put him on a new medication. Ordinarily, this would be no big deal, as he already takes many medications, but this is a medication that must be injected; in his case they are proposing that he get a subcutaneous pump like an insulin pump. Because he's small, it would go on his leg rather than his abdomen. Needless to say, I have some reservations about this.

It all kind of feels like deja vu. After his last open heart surgery, the Glenn, we went through something very similar. His Glenn pressures were too high. So, his doctors wanted to be aggressive and they put him on oxygen 24 hours a day and on Viagra. We were on oxygen for 7 months, it caused huge disruption in our lives, and it ended up not really doing anything. What did help was the Viagra (but it took a long time to start working), and also just growing and giving his body some time.

Anyway, my goal for tomorrow when I go back to the hospital is to talk to the doctors about alternatives and to try to do more research between now and then.

ETA: I forgot to mention that when they tried to drain the fluid from his "effusion" there was none. I mean, there used to be an effusion there, and the diuretics cleared it up and what had appeared to be remaining effusion was actually "pleural thickening," which I guess means thickening of his pleural membrane. I didn't think to ask about the clinical significance of that, if any. But at least he didn't get a chest tube.

Long cath

Magnus went in for his cath at about 3 p.m. They said it would be about 2 hours, but they are still in there. We just got something of an update: they were able to drain the fluid from his effusion without putting in a chest tube (this is good), but the bad news is that his Fontan pressures are high (this is bad) and they are coiling off some collateral vessels (also bad). One of the doctors just called Iggy and said that he was probably going to be going back to the ICU, but didn't explain why and I didn't get a chance to talk to her. The person I spoke to in the cath lab also said she thought they might be done pretty soon, but she didn't know much about what was going on.

Cath day again

I had to go teach my class yesterday morning, so I was gone from the hospital for most of the day while Iggy came down and stayed with Magnus. When I got back, I was surprised to learn that our plan of waiting for the effusion to go away and then going to the cath lab had been discarded and that instead, he would be going in for a cath on Tuesday afternoon.

I was confused, but finally someone came and told me that this new plan was because the diuretics didn't seem to be having the desired effects on the effusion. This is concerning because it may be indicative of problems with his hemodynamics. That's why they are doing the cath today. However, it could also just be a consequence of his paralyzed diaphragm. They also said that although they were going to try to drain the fluid from his effusion with a needle that they might need to put in a chest tube, too.

He's supposed to go back to the cath lab at 2 p.m. Here's hoping for good news and no chest tube.

Day 4

This morning started out pretty well. Magnus slept through the big earthquake we had last night (I most definitely did not) as well as the 2 vitals checks and a mid-night diaper change. I had managed to talk the doctors out of doing any morning labs today, so we didn't have a blood draw. After breakfast and meds, Magnus had an X-ray and we went down to the outdoor courtyard and played for a while. When we came back, we were right in time for the team to round on him, but we unfortunately learned that his X-ray looked about the same as yesterday, or if anything, a little bit worse. His fluid balance had been even over the last 24 hours (he was taking in the same amount as he was peeing out, so basically no progress). They decided to add a second diuretic to try to accelerate things. The lack of progress was a little disappointing, but then my cousin Chris came to visit us and even brought us some non-hospital food for lunch. We went outside to eat, and showed Chris the train and thoroughly enjoyed our visit. Then it was time to go back to the room for his afternoon IV diuretic dose and ugggh, his IV had gone bad. The vascular access experts are not here on Sundays, so after they took his old IV out, they let us go to the playroom while they tried to find someone good. So, they tried. Twice. It was bad. There was a lot of rooting around. Magnus was very brave. So brave that as soon as it was over, I took him down to the vending machine to get a Rice Krispie Treat. Luckily, we did then get a reprieve and they're going to wait until tomorrow morning when the vascular access team is here to try again. The down side of that is that this may delay the resolution of the effusion, but I feel OK with that tradeoff.

Here for a while

Well, for a while today it was looking like this would be a nice, short, easy hospital visit. As of this morning, we were told that if his effusion was looking better, he'd be switched to oral diuretics and then if things continued to improve that we would be home on Sunday. But, that was before the hospital doctors talked to the folks on the pulmonary hypertension team, who are not here on the weekends, but who called in to say that they wanted Magnus to stay on IV diuretics until his effusion is completely gone and then they want him to have a cardiac catheterization after it's gone. This is all very frustrating for a multitude of reasons; first, because although we've been in the hospital for more than 2 days now, nobody has come to talk to us about why this effusion developed and what it all means. I can intuit that because the PH team is advocating a cath and that they originally were talking chest tube that they are quite concerned about the impact of the effusion, but I don't totally understand why (they did mention in clinic that having an effusion can affect the hemodynamics of the Fontan circulation, so I guess they want to do the cath to see if his pressures have gotten screwed up and/or he has grown any new collaterals, but since we haven't actually gotten to talk to any of those doctors I have no idea!) And then second, we have to figure out how to integrate this unplanned hospitalization into our lives...Magnus will miss school but Iggy and I really can't afford to miss work, so I honestly don't know how we are going to make it through the week. We're going to have to rent a car, I guess? We will figure it out, we always do, but I just have no idea how right now.

On the bright side, Magnus is in very good spirits, and they are letting us be off the monitors except for checking vitals every 4 hours so we can roam around the hospital at will.

In the hospital

Sorry for the lack of updates here! To make a long story kind of short, because it is very late and I am exhausted, Magnus has been admitted to the ICU. At first, they were going to put in a chest tube (which is pretty much what it sounds like...they poke a hole in his chest and put a tube in to drain fluid from around the lungs). But then they changed their minds and decided that they were going to try IV diuretics first. I have no idea how long we will be here or what the significance (if any) of this development is. I have been at work all day and drove straight to the hospital when my class got out at 8:30 p.m. (in a town about an hour away from here). Of course he's been asleep the whole time I've been here, but it's such a huge relief to finally be with him!

Just when you think it's over...

Magnus and I went down to Stanford on Monday for a checkup with the Pulmonary Hypertension clinic. It was the first time we'd been back to the hospital since the week after Magnus was discharged, and I could tell that being back in the building brought back some memories for him. In particular, he seemed to be struck by how much better he was feeling than the last time he'd been there. He started running down the hall saying "I can go fast now! I don't need a car anymore!" (when he'd been inpatient, he was too weak to walk very far so we went everywhere in a little toy car). We checked in at the Heart Center clinic and then proceeded to wait until 90 minutes after our appointment time until they called us in, which was unpleasant, but we're used to waiting around for medical appointments.

They checked his oxygen saturations, which looked great, much better than they'd been at his last two cardiology appointments. The appointment itself went smoothly. We actually saw three doctors, including one whom we'd seen in the hospital, and we discussed his medications, but ultimately decided to keep everything the same. They did want to check on one thing, though: on our way out, they wanted us to have an X-ray to check on the status of his lungs. When he'd been discharged in late June, he still had some fluid buildup in his lungs, but the doctors were pretty sure that it would resolve in time. In our clinic appointment, though, they said that it still felt like he had some fluid in there. So, we went for the X ray and headed home.

On our drive home, the doctors called me and said that not only did he still have fluid in his lungs, it was actually significantly worse than it had been when we were discharged. We would have to double his diuretic dose and bring him back down to Palo Alto on Thursday. So, right now, Iggy and Magnus are on their way down there for another X-Ray and some bloodwork. If his lungs look better, they'll be home shortly after that. But if not, Magnus will have to be re-admitted for IV diuretics and possibly a new chest tube, which would be horrible.

I'm not there with them because I have class this morning, and it's my first week of classes, so I absolutely can't miss work. I actually have an evening class tonight, too, so if he is hospitalized, I actually don't know how I'm going to get to my class and then down to the hospital. For now, I'm just keeping my fingers crossed that I won't have to worry about it.

"Recovered"

As of yesterday, Magnus is 6 weeks post-op and thus is considered to be officially recovered from his surgery. Now we can pick him up under his arms, give him a proper bath, and let him climb things and pick up heavy objects.

In many ways, he does seem healed. He had his first day back at school yesterday, followed by a 2-hour playdate. I was hoping this would leave him so exhausted that he would peacefully drop off to sleep at 7:30, but it took an hour of struggle before he fell asleep at 9 p.m., as per usual. His strength is almost back to where it was and certainly dramatically better than when he first came home from the hospital and couldn't sit down or stand up on his own. He still complains loudly about being expected to go on long walks or to go up and down stairs, but I think it's mostly psychological, and he's able to do it.

Areas we're still working on include eating and sleeping. On the plus side, it's been 2 weeks since he last puked, but he's still not showing much appetite and still prefers to get most of his calories from chocolate milk and yogurt. I guess we're moving in the right direction, but progress has been slow. Luckily, he hasn't lost too much weight and is only down about 2 or 3 pounds from pre-surgery. As for the sleeping, I think the problem is a combination of a few factors: being in the hospital for 19 days where they need to check your vitals every few hours day and night, having bad dreams, and post-surgical itchiness. At any rate, for whatever reason, Magnus has not slept through the night since coming home from the hospital more than 3 weeks ago, and typically he wakes up a few times each night and needs a parent to soothe him back to sleep, preferably Mama. I think this is slowly getting better, too, or maybe I am just getting used to being exhausted all the time.

We had a follow-up cardiology appointment and echocardiogram this morning. I was nervous about this appointment, mostly because of the fact that his appetite is still bad and that had me worried that he might be having liver problems. The echo tech was acting extra nice to us (he is always nice, but seemed extra nice today) and then THAT made me worried that he was being nice because he felt sorry that he was sending us off to the cardiologist to get bad news. But everything went OK with the cardiologist. His oxygen saturations were a little higher than last time at 84, which is not spectacular, but fine for a fenestrated Fontan. I was also worried about his weight but that wasn't terrible, either. One piece of semi-bad news was that they still don't see any signs of movement in his right diaphragm, which was paralyzed during the surgery. It may be that the nerve just needs additional time to heal, or it is also possible that it will not heal. The more time that passes without any sign of recovery, the greater the chance that it will not heal. It's not a disaster if it doesn't heal, but that would mean that his right lung would always have suboptimal function, which in turn makes things harder for his already compromised heart. Anyway, there's nothing we can do now except wait. Hey, it's not a real pediatric cardiology appointment if you don't leave feeling vaguely discomfited about something, right?

Overall, though, it really does feel like Magnus, and our lives, are getting back to normal.

Home

(Above: exiting the hospital after 19 days, Below: welcome home sign by our neighbor Audrey)

We are home! We got home around 1 p.m. on Friday, and there was so much to do! Presents for Magnus to open, unpacking and going through mail for me (I hadn't been home in 20 days!), a new medication regimen to figure out. After surgery, you're usually on some extra meds for a while. This time it wasn't too bad, although we did switch from being on 2x a day meds to 3x a day meds. Hopefully, as he recovers, we'll be able to drop some of the extra meds and will be on fewer than before surgery.

I can't believe how exhausted I've felt since we got home. I guess I was having an extended adrenaline rush the whole time we were in the hospital, and now that's over. It helps that my back and neck are starting to feel normal again after a couple of nights of sleeping in an actual bed!

Magnus is happy to be home, but the hospital has taken its toll on him, too. He's quite clingy and gets anxious if I am too far away from him. He's been waking up several times each night. He also has lost a lot of physical strength from all that bedrest. Ordinarily, when kids have a Fontan, they like to get them up and moving not too long after surgery, but in his case, he was tethered to the CPAP and nitric oxide machines for a couple of weeks, so he really spent a long time lying down. I knew that when he came home that his sternum would still be healing, but I was surprised by how weak and stiff his legs are, too. Going up and down stairs is difficult, and he's having difficulty with transitioning between sitting and standing. He needs help to get up after sitting on the floor or to sit on his potty chair. I'm sure that stuff will come back to him pretty quickly, but it's just a shock to see how quickly those muscles went!

Another challenge has been eating. The Fontan is notorious for the "Fontan gut" most kids experience. The surgery reroutes blood flow through the entire lower part of the body, including the gut and the liver, and as a result, bloating and vomiting are common in the weeks after the surgery. We've had some issues with puking, gas, and constipation, and Magnus is still very skittish about eating anything. Luckily, he does seem to be tolerating chocolate milk, supplemented by a few cheese puffs here and there

Being home has also finally given me the opportunity to start to reflect on the big picture. This surgery was a gamble. Magnus was considered a high risk Fontan due to his elevated pulmonary pressures, and there were many people who suggested that maybe he shouldn't have the surgery due to the risks for his particular anatomy. Although he did have complications (the paralyzed diaphragm and collapsed right lung), he actually had a relatively easy time with fluid buildup and seems to be doing pretty well with the Fontan anatomy so far. It was almost 5 years ago that we received Magnus's diagnosis and were told that a series of three surgeries would be our best option for him to survive. Now we are done with those surgeries! There will likely be more in the future, but hopefully not for a long, long time.

It's official

Magnus's INR was 1.7 this morning! WE ARE GOING HOME! They took out his chest tube sutures and are about to take out his IV. (When they take out your IV, you know you are on the way out the door).

Also, this morning I went down to the cafeteria and bumped into a couple we had met up in the ICU. Their 14-year-old grandson had been very sick and on ECMO. Today he is getting a new heart!

It's a good day.

???

Good news: Magnus's INR this morning was 1.6!
Bad news: Although the nurse practitioner told us yesterday that an INR of 1.6 would be sufficient for discharge, today we have a new NP, who says that the old NP's notes say that we have to be 1.7–1.8 to be discharged!

The new NP is currently following up with the surgeons.

You guys are still here?

We've heard a lot of that today. And yes, we are still here. Magnus's INR this morning was 1.4. The good news is that the surgeons have backed off in their requirement of 2.0 and said he needs to be 1.6 to go home. We were 1.2 yesterday, so hopefully 1.6 tomorrow.

Magnus did not sleep well last night. We have a new roommate, and he has a very large family who like to visit. As a result, Magnus was pretty grumpy all morning although he did end up taking a nap in the afternoon. Iggy was also able to come down for most of the day, and in the evening we had a visit from our friends Katrin and Finn.

Overall, though, we are both pretty sick of the hospital and can't wait to be out of here!