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.