Shaken not Stirred

Man does it ever seem to be more difficult to find time to update lately. It seems like the longer we go between updates the more difficult it is to put it all together. More on that later. Yesterday there was an earthquake in Virginia, you all undoubtedly know this. We have a great deal of friends in family in that area that were thankfully unaffected and safe. We also had tremors here in our area as a result, but nothing too bad. Crazy stuff! Shortly after the "earthquake" we had to make our way to the hospital for a Pulmonary appointment. This was a routine follow-up because of Elias' hospital stay a few weeks ago. Pretty much status-quot, which is always welcomed. Dr Wood did comment on the new adrenal insufficiency. Again he is so amazed that Elias has yet another affecting diagnosis, that there isn't a system or organ in this boy's body that isn't impacted in some way from Fanconi Anemia, yet he thrives and out performs expectations. No one is complaining of course, but it is amazing. He is interested in watching how Elias does over the next 4-6 months with the recurrence or absence of  his spontaneous breathing stoppages. Will the Keppra (anti-seizure medication) have any impact on that or not? He will be working along with neurology to evaluate. Katharine took the opportunity to ask him about the possibility of letting Elias have anything by mouth, knowing what the answer would be but you have to ask you know. He chuckled a bit and hung his head and said, "I don't want to answer that." He still feels it is too dangerous a risk, especially since there has only been marginal improvement in the airway compression along with the lack of growth to assist that. It is fair and we expected that as we said, but again if you don't ask....

Speaking of the Keppra - we have started to see a diminished amount of side-effects with the adding of the Vitamin B-6 along with the natural adjustment. So that is a positive sign. The daily steroid that endocrinology has ordered also seems to be having a positive impact. He has been on that 3 times a day for just under a week and we can already tell a difference. On a not so positive note, Elias' IGG level has plummeted. These levels correspond with immune system function. He dropped close to 200 points to the low 600's. He was around 890. Normal ranges for this is 400 as an accepted low and 1200. He has been steadily declining since May from 920 to 890 and now the huge sudden drop has the doctors a little concerned. We already have gone back to extra precaution mode with him and will continue that for awhile. He will be getting IVIG much more frequently it seems. We got a call yesterday from the FA clinic saying they wanted him in for an infusion this week, but were okay with waiting until next week if need be just no later than that. I think the bigger concern at the moment is we are getting ready to head into cold/flu season and with him being more compromised than his normal is a touch concerning. So they want to figure this out and get his IGG numbers back up as quickly as they can. There will be more on this in the near future we are sure. One final item to quickly note is we are moving forward to see the Infectious Disease (ID) team in order to try and get a better grasp on the c-diff situation. GI is just not doing much to follow-up on this, and he has been put on the same course of vancomycin for the dozenthish (is that even a word?) time. A few of his doctors were NOT happy to hear he was being treated again, because the symptoms were not there 100%. We agreed.  So we are looking for another opinion, especially after the conversation we had with ID when he was being evaluated during his admission.

Overall Elias continues to improve and recover. We are settling into his new routine, which has become a bit more intense as we have increased from 5 daily meds to 9 (most 2-3 times per day around the clock) and 16 overall with the "as needed" and emergency medicines. Kind of reminds us of our first months home. We switched pharmacies 3 weeks ago, they already know my name and face we have been in so many times. We continue to be thankful for how well he is doing all things considered. Especially after being reminded so much lately how unlikely it should be and how incredibly complicated, medically he continues to become. Thank you all for the continued support and prayers. They are obviously being answered.  

GI Appointment Review

We took Elias to see his GI team last Thursday. They are the team responsible for managing Elias feeding and overseeing his growth. Unfortunately the politics of the hospital plus the fact that Elias is part of the Aero Digestive Team prevent him from seeing the Fanconi Anemia GI specialist. It is a long complex and idiotic issue. It actually took the FA dietician pushing the dietician that is part of this team to actually set an appointment. So many thanks to her for helping us rage the battle and winning. FA children can not be managed on paper and that is what this team is trying to do. It is even easier now that the hospital is on a new computer system that allows all a patients information to be access across multi-discipline channels without waiting. On paper this is an amazing tool, but we refer to this system called EPIC as EPIC Fail! This is mainly due to the fact no one truly knows how to use it properly. Each doctor, nurse, or support staff learn the minimum and still struggle with it. That in turn leaves information out and prevents the most current information from being accurate. We know this because every clinic visit we have to make the same changes repeatedly.

So on to the appointment...

Bill Nye the Science Guy Time

Image via Wikipedia

Not a whole lot to report in with this update. We have been having some unmentioned issues with Elias feeds. Primarily that he almost to the point of predictability is retching and at times vomiting toward the end of his feeds. we have watched and tried to figure out a pattern to this, i.e.; activity level, positioning, etc. but have not come up with anything that nails it. Katharine believes that it is a volume issue. That we are giving him too much at once. Sort of like overstuffing him. Since he can not communicate to us yet about these things we have to trial and error. It would make sense, if we eat too much we get sick. There are times though that he will not do it, albeit rare. Tomorrow and Thursday we are going to conduct an experiment. Feed him four times versus three for a shorter time and decreased volume. Hopefully we will no longer see the behavior and we can then address the why. If he does continue to retch and vomit then we will be heading back to GI to try and determine what the issue might be.