Sorry for the lack of updates

It was a busy end of the week.  Isaiah was only in the hospital for 24 hrs and I have to say it was the most productive short stay I have ever had with my kids.  Everyone was impressed that it had been 3 yrs. since Isaiah was inpatient.  They are all so used to seeing Avery that everyone seems to forget my other guy at home.  Well it was time to focus on Isaiah's needs and have drs. take his cycles seriously.  That was the purpose of this admission and it worked.  He was nearing the end of the cyle so he did not even look that bad but they thought he did (good job Zay). Although they did think he was handsome :)

By the morning we had seen Neurology, Metabolism, GI, Nutrition, Pallative Care, and Complex Care team.  They all came together to decide the best meds for preventive (daily med.) and abortive meds (at the start of a cycle).  All were in agreement that the frequency of his cycles were just not good.  Isaiah has tried just about every med. over the past four yrs. and nothing has really touched him.  With him having mito. it also restricts some of the meds. he can try.  Depakote being one of them.  The jury was out on this one b/c it could potentially damage the liver pretty quickly sometimes reversible and sometimes not.  So we all agreed that it was a total last resort.  In the meantime we are trying propanodal (beta blocker) as preventive and Imitrex as abortive but it can only be given once a week.  He will also remain on compozine and diastat  as abortive.  Today was our first trial of the Imitrex and I am not impressed but we will see. 

Next is his diet (on a postive note he gained 4 pounds he is finally in the 40s).  They will reduce the fats that are in his formula.  With the boys new diagnosis it is now thought that they need much less fats then they are getting.  We have a ton of appts. coming up to keep on track with what is going on.  All good things.  I am just glad is was only a 24 hr. visit b/c Isaiah is just plain wild.  I swear he might have even eaten his hospital bracelet, LOL.  He kept getting his skinny legs stuck through the slats of the crib.  I was worried he would break them.  It is very stressful being inpatient with him considering he is just about my size. Plus he was getting depressed b/c he cannot roll around :(  Avery is easier b/c he doesn't move (definetely has its advantages).

Meanwhile, I also had the drs. talking to me about Avery.  Last week he had a blood clot in his central line.  This is very concerning b/c he has quite the history of blood clots which have limited his line access to only the right side.  If this side ever got clotted we would be totally screwed.  The team had not heard of blood being clotted at the end of his line b/c he gets heprin regularly and his TPN is constantly running through it.  This weekend a nurse is coming to put TPA (extra strong heprin) in his line to make sure he has not clots.  It is just nerve wrecking b/c he is doing so well and something like this could really set him back.  I am just glad we are being proactive about it.   He has been junky more than usual and requiring bipap at least 3 nights a week.  He also has been having alot of very fussy days.  Things change from day to day with this guy but I am just grateful he is home. 

This update turned out to be much longer than I thought.  So thank you if you have made it this far.  Sometimes a couple of days in this house turns out to be novel of events.