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.  

Neurology & Endocrine Follow Ups

We had been holding off on an update to get results from a few things, but it appears they are taking longer than expected to complete. So we will update on what we have from the follow-up visits last week. We conveyed our observations and concerns over the possible side-effects Elias has been exhibiting from the Keppra, his anti-seizure medication. Neurology agreed that these were likely linked and have added vitamin B-6 twice a day to his medication regiment. There has been some success with counter-balancing the Keppra with this vitamin. It is something we have to crush to be able to give via G-tube, so there is a small process to prepare it. So far it hasn't been too bad and our biggest worry was clogging, but it seems we are crushing it fine enough. We also changed the emergency medication and protocol for if he actually has a seizure again. We will be giving Midazolam. After we draw it up from the vial we will remove the needle and actually push the medicine into the buckle of his mouth - pocket between the gum and cheek. This will allow for quicker absorption into the blood stream. We are also going to give this the next time Elias spontaneously stops breathing to test if the clusters continue or we isolate the behavior. This will help to figure out is that issue is seizure related as pulmonary suspects. The biggest surprise from the appointment was to hear that his neurologist still feels strongly that Elias had meningitis despite the very well reasoned logic from Infectious Disease (ID). To recap, ID felt that Elias recovery time alone was too brief, there were no signs or symptoms prior to the seizure, and a seizure is not the initial presentation of meningitis. Furthermore looking more in depth at his blood work, there were not any of the other tells you typically see. Impressively his Neurologist brought up the point that Elias is far from normal when it comes to lab work, symptoms, and recovery. This makes for a very strong argument against ID's thoughts. Flashback to July 2008 when the tracheostomy and G-tube was placed. Elias was just a bit off, and it was a Mother's intuition nothing physical that made Katharine press the doctors to run tests the night before the surgery. His blood work showed no signals and his physical exams were fine as well. We found out that Elias had full blown pneumonia that went undetected because there were not any "typical symptoms." Elias had to battle for his life after crashing on the operating table. So what does all that mean....well medically, nothing. It means we don't truly have an answer and must proceed as seizures are a new problem that will strike randomly. It also reminds us and deepens the vigilance we use becoming concerned at the smallest of changes. In you or I it would present much stronger, in Elias it may appear business as usual. All-in-All we will simply keep doing what we have been doing, successfully. Before this we had kept Elias out of the hospital for an illness since April 2010, when he had a MRSA blood infection. That includes 2 winter seasons unhospitalized (ie; cold/flu/rsv hell) for a trached child that is amazing, as the doctors reminded us.
We are still awaiting the endocrinology results in reference to the possible adrenal insufficiency or dissolved regulatory response. This may mean another medication daily, a steroid instead of the stress dosing we are set-up with now. We are also going to be checking his blood sugars a few times a week for 4-6 weeks just to make sure things are working as they should. They do not feel this is an issue, but this will make them more comfortable in evaluating the big picture from an endocrine stand point.

We are beginning to see Elias level out a bit and regain some of his energy that has been difficult to watch. He has had a good week overall with the amount of time he will play and how he has felt. Part of it is adjusting and let's face it Elias was very sick so recovery will take time. The doctors did a wonderful job disguising the seriousness of things while he was admitted - giving it due attention mind you, but keeping our stress down and concern level low. However, now that he has improved they have not been shy telling us how very concerned they were and sick he was. Dr. Mehta, his bone marrow/FA specialist commented to us in clinic she has no idea how we handle it, but she is glad that we manage somehow for Elias sake. That is just it, it is for Elias' sake. She also reinforced how challenging and unique he is medically on all fronts. We appreciated the compliment and vote of confidence, which several of his doctors have made over the years. We told her that it helps to have great doctors caring for him and talented enough to handle his challenges and be pragmatic with his plans. They have provided us with the tools and we have embraced them. Without them, along with supportive family and friends we would not be half as strong as we are. It serves as yet another reassurance that we made the best decision coming here for his care and reminds us that he has some of the top doctors in the world - for their fields - working with him. We couldn't ask for more. Thank you for the continued prayers, support, and uplifting motivation. As I posted on Facebook this morning, "Tomorrow will be a better day because we are privileged to be granted the opportunity."

Medical Regrouping and Pre-school considerations

After last weeks chaos medically we have finally regrouped and are working towards a solution. Here is what we learned. The C-diff is still active so we must go back on the vancomycin for a month this time 4 times a day. Afterward we will taper or pulse dose. With the feeding issue that had disappeared returning GI wants to push the timeline up on the surgeries so he can do a scope in the O.R. and try to figure out what is going on in there. This could be as early as the end of this month.

Getting Back On Track

Things here have been busy. There have been a lot of follow-up and make up appointments to cram in before Christmas. The platelet antibody test came back positive, which is bad. It does provide an explanation for the dramatic drop. The good news is the counts are trending up despite the antibodies and IVIG is the first treatment, which Elias is already getting. The FA team is just going to continue to monitor his counts and increase his IVIG if needed.

Elias has improved a great deal, mostly in the last few days. He is beginning to get his energy back and becoming more active. The smiles, fishy kisses, and raspberries are back stronger than ever. He is also beginning to get back to walking. Wednesday night he was so determined and it was like watching him take his first steps all over again. He still is not walking as easily as he was before surgery, but her is able to walk across the room and spin around as well. When he stops it is more from fatigue than not being able to keep his balance and footing. That frustrates him a little, but he rests and then pulls himself up on the nearest stable object and goes again. We are glad we have not had to prompt him to do this and he is working this out on his own and at his pace. It shows his determination and will to conquer.

We have another busy week ahead with several appointments in the mix. It has been exhausting to be quite honest. We are still trying to recover just from being in the hospital so long and now this crazy intense schedule we have going on. Last week Elias also tested positive yet again for his chronic C-diff. Back on the vancomycin he goes. They are going to try a taper off it this time to see if that helps keep it at bay longer. We also started Diflucan to see if we can control and eliminate the feeding issues we have been battling where he sweats and retches with occasional vomiting. His GI doctor has a theory that with all the varied antibiotics Elias has had to be on almost constantly along with the colonization of different things in his body, that might be the cause. The Diflucan will treat certain types of those most commonly known to create issues like this. If after some time we are not seeing improvement they will likely scope Elias’ esophagus and stomach. Let us hope the Diflucan is the answer. We also had a day with about five apnea spells. Fortunately, they were relatively easy to manage and bag him back without having to go on oxygen. We have learned to just accept these as a stress in our life that isn’t going away anytime soon. All in all we are just staying busy, trying to get rest when we can, and generally trying to keep our head above water. We hope everyone’s holiday season is going well.

How Much Can I Cram in One Week?

Fire the idiot that does our scheduling! Oh wait, that’s me! What was I thinking? How much can we cram in on one week? I have no idea how I managed our schedule to get so out of control this week. Well this week has been a very hectic one. Elias had Speech, Occupational, Physical and Aural Rehab Therapies through the week. We also had IVIG Friday and of course, what is a week without a new problem or issue to contend with? Not to mention Katharine having to work an overnight shift at work, and having some doctor appointments of he own and I had a meeting with our nursing agency case manager. We are very glad this week is behind us, but the schedule doesn’t seem to be getting less chaotic anytime soon.

A Week of “New”

 

Elias has an odor from his trach tube. That is never a good thing and usually an early indicator of something bad. However and thankfully he was presenting with none of the other usual suspects of symptoms. We still felt the need to contact Dr. Wood, Elias’ pulmonologist. Our thought was possibly that the pseudomonas he is colonized with might be flaring up. Dr. Wood agreed, but since there were no other symptoms it wasn’t worth doing a culture. We would just treat this presumptuously, as Dr. Wood put it, because Elias can not be trusted! Especially when it comes to that tenuous airway, again his words. Elias amuses him and challenges him at the same time and he has told us on many occasion that he sleeps better knowing we are his parents. We appreciated that compliment coming from one of the worlds top pulmonary doctors very much! So we are giving him Gentamicin for the next 10 days in hopes that this clears up the issue.