Today was a good day. The only time he needed the Roc to get paralyzed was at 7:45 this morning. So far, that makes for a great day! He has had several episodes where his stats (% oxygen in his bloodstream) drop but then recovers on his own with the help of us talking to him, rubbing his legs or playing his glowie singing toy. It really comes down to his temper, he gets mad and when he gets mad he bears down and it closes the vessels in his lungs and his sats drop. So, keeping him calm and mostly sedated has helped.
The doctor came in this morning and we talked about timing. The question of the century. She said there is no way that she could tell us (of course). She said that the better question is to talk about goals for the week. That way it is week to week. This will be a long road and there is no way they can give us an idea of how he will do. In her opinion, she did say that he will need to go a week or so without any crisis episodes before we can even talk about weaning the vent. He will also need to be awake and not getting pissed and bearing down, that will be the tricky part. He has a temper, he got that from me… oops. Each rounding pediatric intensivist has a different process and so it will depend on who is rounding that week but it will be something close to that for timing.
The NJ was clogged and so they came in and had fluoroscopy come in and look at it. It ended up being wound up and so they were able to adjust it versus replace it. We started feeds at 5ml an hour and he hasn’t pooped yet so we will see what his stomach is measuring at tomorrow. They measure around his stomach every few hours to see if he it is distended. That is how to tell if he is handling the feeds well.
We met with Dr. Shepard, one of the cardiologists and talked about the TEE heart procedure he had done yesterday. It gave them a good view of the heart and it wasn’t as awful as they thought it was. They think that the extra cluster of muscle isn’t affecting him as much as they originally thought which they did already think but good to have confirmation. The cardiologists and the intensivists are all saying that it is equal parts the heart and the lungs.
The pulminary hypertension means his lung pressures are bad and the thick right side of the heart means the blood isn’t pumping well. He is on several meds for each and so hopefully they will all help him to stop with the crisis. He started another med today on top of the sildenafil to help with the hypertension, that will take a day or 2 to really start helping him.
His fever was much better by this afternoon but this morning when we met with the rounding doctor we were discussing his platelets and his hemoglobin. His hemoglobin is low again even after a blood transfusion just a few days ago. We were concerned about a brain bleed because they have basically done an ultrasound and/ or x-ray on the rest of his body so we got a head ultrasound done. It came back clean, which we suspected it would. The next step would be a CT scan and we aren’t there yet. They want to see if the platelets come back up on their own. We will probably need to give him another blood transfusion but they are so so worried about his fluid intake because when he is fluid positive like he has been for several days (receiving more fluid than he is outputting) his crisis are worse, when he is negative, like today, they are better. Obviously, blood is fluid and so it makes it worse. He is -230 ml so far today and still puffy but handling it better than when he is positive.
The doctor also mentioned that she has noticed that kids with genetic abnormalities can have higher temperatures and run low grade fevers for no reason. I am curious the research on that. Maxley is only the 21st case of his genetic abnormality but there are similarities with lots of kids with generic abnormalities. For example, a lot of them have heart conditions, developmental delays, etc.
On Saturday morning he developed a small rash looking thing called Petechiae on his ribs. They look like tiny broken blood vessels. I have seen them before around eyes after vomiting. It is not concerning it is just from his low platelets so they aren’t concerned but I forgot to write about it before.
