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.  

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.

A Full Week

It is the end of another busy week, exhausting week. We had appointments everyday this week (some days multiple), except for Friday. Most of them

were various therapy appointments, with IVIG and some research lab work thrown in the mix. Health-wise Elias is doing well for the most part. We just finished another 3 week course of vancomycin (second course in 6-1/2 weeks) on Monday for C-diff and already we are seeing the beginning symptoms of a flare up emerging. I imagine Monday morning we will be calling GI for the next plan to try and beat out this very chronic issue. There has been some conversation between Elias GI doctor, His BMT doctor and the chief doctor of infectious disease on alternative treatments. Probiotics have been mentioned in the past, but BMT would rather try other options first. We do not fully understand the reason behind the reluctance, but she is a VERY good FA doctor that we trust explicitly. She is not 100% opposed, but would rather not jump right in if there is an alternative, which apparently does exist. So we will place this in their very capable hands and see what comes next.

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.

Visits, Meetings, and Day to Day

This week Katharine’s grandparents from Florida came to visit. This was the first time in several visits that Elias has not been in a hospital. Actually, he was home one other time, but was very sick and we ended up being admitted that day. It was great for them to see Elias thriving and happy oh, and walking all over the place! He had such a good time being a little ham and showing off. We are thankful for that opportunity.

Just a few items to quickly update everyone with. As we just mentioned Elias is officially a walker and quite good at the recovery and balance. He is loving his new found freedom. This past week we had our transitional meeting with the early intervention team and the public schools. Hard to believe we are already talking about school for Elias! He is slated to begin pre-school next fall, which is a half day program at the schools. Elias will more than likely be home-bound because of his continued immune suppression issues as well as the risk with his airway. The pulmonologist will evaluate again in the summer, but is not optimistic that status will change. At least it wasn’t a stern “NO!” Given that likelihood we discussed that with the school. They are able and prepared to send a teacher to our home for Elias in addition to continuing OT, PT, and Speech at a more limited level than we are receiving now. Their official assessment of him will be in February with a formal Individual Education Plan (IEP) meeting in April. It all sounds very accommodating and well supported. We have been told by several people that we are in a very good school district. If that holds true we will be very fortunate and blessed.

Elias has had some issues this week. He has tested positive for C-diff, so we are treating him with vancomycin again. Then we thought he was catching a cold, which would not be good for many reason. Fortunately it seems to have vanished as quickly as it appeared. We have started to see the suction need increase though. Last evening over a six hour period I suctioned him over thirty times. That is a lot of suctioning, but ‘tis the season! Last but certainly not least we are preparing for Elias next surgery very soon. This will close the colostomy! Hurray! We will be very happy to see this go away and it will be another huge step and change for Elias! We are hoping that things go well with the transition. We are not certain how long it will take for us to determine his continence success, but that is one concern that still exists. Tagged on to this surgery will be several other doctors doing various procedures. We appreciate all the thoughts and prayers in advance for a quick and smooth recovery for our strong little man as he endures yet another round of surgeries.

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.