Last leg of the marathon.

No one warns you of the "post-transplant" period. They don't tell you that your child will have even less of an immune system then they had before transplant and that it will feel like an eternity until any cells begin to trickle in. They don't tell you to expect numerous complications and bumps in the road. I wish we were warned that the worst was yet to come.. Not that I would have believed them.

Our Immunologist had come in to go over Emie's 100 day Chimerism/Immune Panel a while back and said she's heard lots of families describe this awkward period as the last leg of a marathon. I couldn't describe it any better. I feel like we've been running full tilt for so long and the end is still not in sight. We are completely and utterly exhausted- physically, mentally, & emotionally. If you were to ask me before diagnosis if it were possible to survive all we've been through I'd say not a chance.

Since my last update - Em's new PICC line was working great. We were discharged home and were in and out of clinic what was suppose to be twice a week. After a couple visits they noticed his urea/creatinine (markers of hydration and kidney function) to be high and his bicarb (acid-base regulation) was extremely low. These are indicators that his kidneys were unhappy. They tried treating it with saline boluses and eventually tried a few doses of oral bicarb. His numbers continued to worsen and we were readmitted after being home for less then 2 weeks. He was hooked up to continuous fluids and bicarb supplementation to try and get his numbers up. They also tested his urine for bacteria which could be affecting his kidney/making his numbers off. They were unsuccessful with the catheter so a bagged sample would have to do. It came back positive for a bacteria but its hard to know if it was contamination or not. He was started on a week of antibiotics which would worsen his already loose stool and make his kidney even angrier. Em was hooked up continuously for a week in hospital before his numbers began to improve. They started letting him be unhooked for a couple hours a day and working up as long as his bicarb stayed at an ok level. By the time Halloween came we were able to leave on an 8 hr pass :). Em was able to experience Halloween and it was magical. He was SO excited every time the doorbell rang. He would grab a hold of our fingers and race to the door giving the kids the biggest smiles as they collected their treats. Em dressed up as a little monster and we took him out to a few of the neighbours houses (keeping him at a safe distance). I've never seen such excitement on his face, it was priceless :).

A few days later they trialled him on an overnight pass. It was so nice being able to put him to sleep in his own bed and spend the night as a couple for the first time in weeks. I can't stress enough to everyone to not take for granted the little things in life. We went in the next morning to check his levels and they had dropped from 19 to 12 (Normal levels are 18-26). It was absolutely devastating. We were back at square one with no end in sight. They took away our passes once again and Em was hooked up 24hrs a day.. This was one of my lowest lows. To make things worse the Oncologist on was insensitive and made a tough situation even more horrible. It took a couple days to get his bicarb back up to the level they wanted but the Oncologist refused to let us out even for walks.  He said he felt more comfortable being able to monitor us. Another week passed until they allowed us to go out on a 2 hr pass. We did that for a few days and then they allowed us out for 4 hours at a time. These small escapes are what kept me sane and able to survive this never ending hospital stay. Grandma came to visit and within this period Emerson celebrated his day+100 post BMT. We brought cupcakes to the unit and did crafts in the room until we were able to go home on a pass. It was a really nice day.

What is the significance of day+100 post BMT. It really only meant being able to wean a couple of the medications Em was on. We had thought it meant that we were out of the woods for acute GVHD but were told otherwise. I guess they have seen cases of GVHD post day 100. It was fairly anti-climatic. They sent off his immune panel (breakdown of the specific immune cells growing) and chimerism (tells what percentage of the immune cells are from the donor or from Emerson). The results came back and those were also anti-climatic. He still had less cells then he did before the transplant however there were a few immature t-cells which weren't present before. We were told that this is exactly what we should expect at that point but in my head I was hoping for so much more. I wanted them to tell me he was fixed, that we wouldn't need to worry any longer. His chimerism results were more assuring. He was 100% donor cells for his t-cells and b-cells meaning the transplant had taken and the chemo did what it needed to. They will now draw an immune panel/chimerism every 30 days.

In the next couple weeks we kept increasing the length of our pass. We were now only spending our evenings/sleeping at the hospital. As tough as this was it was so much better then being stuck there 24hrs. Emerson was feeling great and was so happy and "normal" at home. I feel like he learns something new each day and is developing so quickly now. On November 26th Emerson decided it was time he walked on his own :) He had been taking steps alone between us since before his transplant but that evening he started to walk from room to room upstairs all on his own. I can't begin to describe the joy and pride that was oozing out of me. That little boy amazes me so much everyday with his strength and happy-go-lucky demeanour. Things finally started to feel like they were going in the right direction. His bicarb level had been stable in the 20's even with us weaning the length of the infusion. They had come to the conclusion that his kidneys were still under stress from the injury they underwent with the immune suppressant drug in combination with dehydration from his persistent loose stool. Things were falling into place and we began discussing discharge home on oral bicarb. They were still being very cautious with weaning the bicarb and were going at a very slow rate. We were down to a 4 hr infusion and three oral doses. This allows us to spend our nights at home and just come in the morning for the infusion. On Dec 2nd we woke up at 3am to find Emerson had soiled his jammies up his back and down THE ONE SLEEVE that had his PICC line....... I still to this day do not understand how this happened or how we can have such bad luck. We called the Unit and they walked us through a sterile cap change at home. I barley slept that night re-living our last septic attack and hospital stay. I felt like crawling into a hole. I couldn't go through that again. Em woke up in a good mood and we went in for our bicarb infusion the next day. The nurse drew off a little extra waste that morning as a precaution and everything seemed to be ok. We went home and Em had his nap. He woke up super flushed and without fail he had a temp. I was dizzy with worry and hatred at myself for doing the cap change.We brought him in and the nurse took his temp and got 37.4 (normal). I started sobbing.. I couldn't believe it. I still had an awful feeling that something wasn't right. We stayed a few hours and he had a low grade temp of 37.6 but nothing over 38. We went home and tried not worrying too much. Em went down at his usual time but woke up at 10:30 pm and he felt really warm. So off to the hospital we went. He was febrile this time. They drew blood cultures and began him on antibiotics. No one slept that night as Em was crying in pain and shaking. We were re-living our worst nightmare. Em was septic for the second time in months. I still don't understand what we did to deserve the hell we've been through. I don't believe everything happens for a reason, there is no reason on earth good enough that this sweet boy has had to endure what he's been through. The next morning (Friday) Ems blood pressure was super low. They gave two fluid boluses and consulted the STEP team. Things were not looking good and we were told Em was one bad blood pressure away from being admitted to the ICU where he'd be sedated, intubated, and given an arterial line. This would be so they could administer medications that would help his blood pressure. On top of everything else, that afternoon Emerson's PICC line stopped working.. They still don't know why but think it might have been from the bicarb reacting with one of the antibiotics given even though they were extremely careful.. So Emerson was now septic with no IV access.... Em was poked too many times for blood work and they needed an IV asap. I requested that Anesthesia come to place the line because of the trouble we've had in the past. They came and got the line the first try, THANK YOU! Things started to stabilize a bit more. His pressure was still on the lower side but he had perked up and was wanting to sit and play. We narrowly avoided the ICU and Em was back to himself the next morning. The antibiotics did their job and cleared Ems blood quickly of the bacteria and he was feeling better. The IV that was placed was holding strong but this meant Em still needed to be poked peripherally everyday for blood work. We needed a central line, bad. Monday came and they had a cancellation in the operating room. Em was able to get in for another PICC line to be placed! AND not be red listed!!! The line was placed without complication and the oncologist was so happy with the way things were looking that he allowed us to go home on an overnight pass! Things had gone from horrific potentially ICU admission to back on pass in 3 days. It was truly a miracle.

We were home for Christmas :) JPs entire family was able to fly in and spend a few days with us. Emerson was over the moon excited to be around his aunties. It was so so nice to have everyone together. My mom and family came up just after Christmas to spend time with Em. The love was non-stop and Em could not have been happier. As lovely as the holidays were I felt a sense of relief as they ended. I said goodbye to the most horrible year and welcomed 2016 with open arms. This year HAS to be better then the last.

They had drawn off Emerson's 4 month post BMT immune panel/chimerism while we were still in hospital and we received the results a bit ago. Emerson FINALLY HAS T-CELLS!! (que happy dance) His CD4 count (the immune cells that he needs to fight viruses) at 100 days were 40 and only one month later were up to 270! (normal is 400-1600). We had noticed Em's nose wasn't runny any more. They did a swab and it came back negative. He cleared his cold viruses!!!! He was cold free for the first time since we was months old!!! I feel like things may finally turn a corner. Now all we need is the damn noro-virus to scram.

With this good news came bad news. They informed us that his liver enzymes (a marker that the liver is under stress) were climbing. Normal is under 50 and his were in the 500's. These can climb for many reasons. In Ems case it could be a virus or GVHD of the liver. Both of these causes should manifest differently though. If it were viral you'd expect him to show symptoms of being ill, fever ect. and if it were GVHD his bilirubin would also be elevated but his was normal. They did an ultrasound of his liver and it came back normal. They then sent off every viral study they could think of as well as a few other liver markers. So far everything has come back negative or normal. They are completely clueless as to what could be making his liver so upset. His numbers now are around 1000. Our only option now is to have another surgery and do a liver biopsy. This will tell us if it is a virus we haven't been able to test or if it is an unusual case of GVHD. The operation is tentatively scheduled for Friday or Monday. This will mean another night in the hospital but hopefully it will also mean an answer. The constant stress and worry we've been living under has been unbearable. I dream of the day where we can stop worrying over every little thing and just enjoy life with our little boy.

I want to thank our families for helping us survive the last few months. We would not have been able to get through the last while without you. Whether it be putting your own lives on hold so you can come stay with us while admitted in hospital  so JP could work to the emotional support you've all provided. We are so luck to have you all.

Thank you for everyones support and prayers. Please say a little one that we find some answers to Ems elevated liver enzymes and for his cells to grow, grow, grow!

D,xo