Our son Magnus was born November 19, 2009 with a serious congenital heart defect, HLHS. I set up this blog to keep our family and friends updated on his condition, and to connect with other families dealing with HLHS.
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.
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.
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.
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.
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.
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.
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.