The last couple of weeks have been a lot; I am not even sure where to begin. I guess I will start with the good. I ended my last post, before this mess, with us off to a friends' place on the lake where Eric was looking forward to tubing. We were supposed to be there for a week, but only ended up staying half that long. Eric was so looking forward to tubing, swimming in the lake and playing Telestrations, and he did get to do all of those things, though not quite as much as he would have liked.
On Wednesday, Eric and I took a float plane to Vancouver for his scheduled platelet transfusion. It was a beautiful day and a smooth ride. We got to the hospital and since we had time, he actually got 2 units of platelets to prepare him for tubing the rest of the week. He was still coughing and hoarse at the appointment. I didn't really feel like he had gotten any better or worse since we'd been in clinic the Friday before. His Oncologist wanted an oxygen saturation monitor on him to make sure that he was still getting enough oxygen. It was on the whole appointment and his saturation looked pretty good, so we headed back to the lake.
That evening Eric was struggling. He was having trouble sleeping and said he couldn't really catch his breath. We had a very long day and he stayed up too late, so I figured he was just worked up from that. In the morning, Chuck went golfing in Sechelt and I slept in. I had a cup of tea on the deck and Eric was playing on his iPad, but he told me he didn't feel like he could breathe. He'd had that O2 probe on all day the day before so I knew he was fine, but thought maybe we could use some reassurance. I called the nearby Pender Harbour Health Center and asked if we could come in for an O2 check. They agreed so we left Joel behind and Eric and I drove to the clinic. I truly thought we were just going for a quick check and heading back to the lake. I didn't bring a thing with me, only my purse. Luckily I had told Eric that he could bring his iPad in case there was a wait at the clinic. We arrived just before noon (their lunch break) and went right into a room. The RN there put an O2 probe on Eric and she saw his oxygen at only 75%. She got an oxygen mask on him quick and he perked up. From there, I called Eric's Oncologist to ask what he'd like us to do. Between the Oncologist and the RN, they decided that we should go to the Sechelt Hospital via ambulance, but no lights or sirens. We were thinking it was the cold, maybe exacerbated by the forest fire smoke in the air. We figured a breathing treatment, like for an asthma attack, and we'd head back to the lake. I didn't even call Chuck.
At the Sechelt Hospital ER, they did start with nebulized ventolin, but it didn't really seem to be helping and was making Eric feel light headed and nauseous from the racing heart side effect. They did a chest x-ray there as well and the doctor spoke with our team at BC Children's Hospital before coming to tell us the plan. There was a new finding on the x-ray: air in Eric's chest cavity outside the lungs, and they wanted to move us to Vancouver. She says the good news is that we get to go by helicopter! At this point, I was still not worried. Apparently my baseline is so broken from the last almost 4 years that an air ambulance doesn't even get my adrenaline going. By this point I had at least called Chuck, but that was mostly just so he could get the keys from me to pick up the car from the Health Center on his way back to the lake. When I learned we were going to Vancouver, I called him again and asked him to pack bags for Eric and I and zoom back to Sechelt. It's about 45 or so minutes and the air ambulance was going to be about the same so it was 50/50 whether I would have only my purse or if I would have a toothbrush and underpants also! Luckily Chuck arrived just as the helicopter touched down. Unfortunately, he packed so frantically that I did not, in fact, have any underpants, but I did have a bathing suit!
The air ambulance was actually really cool since they had to fly at low altitude, less than 500 feet, because of the air in Eric's chest. We had the most amazing views flying low over the ocean and it felt like we could practically reach out and touch the buildings downtown. There's a helipad on the grounds (not the roof) of BC Children's and Women's Hospital, so then you actually go by ambulance around the building to the Children's ER. We arrived at 6pm and went in different doors than normal, but once inside it was all familiar again. The ER doctor came right in and did an assessment. Eric continued to use oxygen on and off as needed while resting with his iPad. We were waiting for the Pediatric Intensive Care team and the Oncology team to do an assessment to determine where he would be admitted. The Oncology team came and felt that he could be treated on the Oncology ward (T8). Duh. Of course he could. He wasn't that sick, just needed a little oxygen and he'd be fixed right up. Eventually the Intensive Care team came by as well and they did say they felt he could be treated on the ward. Again, duh. I did learn a new word from them though: pneumomediastinum. That was Eric's diagnosis from the x-ray. It is air in the chest cavity between the two lungs.
We arrived on T8 (the Oncology floor) around 11:30pm. It took some time to get settled, but eventually Eric fell asleep. I sat and watched him breathe for awhile and then tucked myself into bed. I didn't have my mattress topper so put down 5 of the thick hospital blankets as a cushion. It didn't work. I fell asleep for about 20-30 minutes but I woke up to Eric coughing. Within 30 minutes Eric was working really hard to breathe. It was very noisy and his entire chest, abdomen and shoulders would curl in, then raise up and then come crashing down, including his head bobbing like he was falling asleep, but that wasn't the problem. Eric's face was also extremely swollen, like stung by a million bees swollen. Believe it or not, this is when I started to worry. A little about the breathing, but a lot about the swollen face. I guess it was finally the cue for my brain that something was not right here. The T8 doctor and a respiratory therapist came in. They tried more inhalers but they weren't working. The intensive care team was called for another consult and a portable x-ray was ordered. Next thing I knew a bunch of people from the PICU (Pediatric Intensive Care Unit) were here to move Eric downstairs to T4, the PICU.
It was our first time in the PICU. Actually we'd had a lot of firsts in the less than 24 hours that had passed. First ambulance ride, first helicopter ride (for Eric, first air ambulance for me) and now first visit to the PICU. At this point, I was done with firsts, but unfortunately we were in for a few more. By this point it's the wee hours of Friday morning. The PICU room is large and has a recliner and parent "bed" just like upstairs, but it also has a lot of fancy equipment and is a fish bowl. Most of 3 of the 4 walls are glass looking out to the hall and also into other rooms. Eric asked me for some water and I gave him a sip. Someone came from outside the room and said to hold off on the water for now please because he might be having surgery?!?! Eric was able to sleep for another couple hours while I sat in the chair and watched him. An anesthesiologist came in early in the morning, maybe 7am-ish, to tell me that they were going to take Eric to the OR to insert a breathing tube and, while in there, ENT was going to do an MLB (microlaryngoscopy and brochoscopy) to see if they could find the cause of the pneumomediastinum. The anesthesiologist told me that I could not come with Eric. This is when I completely fell apart. I have always been allowed to go into the OR, dressed in gown, booties and hairnet, until Eric is asleep. I was worried that he would be afraid because he finds the OR intimidating with its size and all of the equipment, and this was all happening so fast. Somehow one of the Child Life Specialists from T8 managed to insert herself right into the OR. She didn't ask, she just went, and I was so grateful that Eric had a familiar face with him. I got a phone call during the procedure that Respirology was going to attempt a lavage (clean out) of the lower airways.
Eric came back into the room, still sedated, and now intubated with a breathing tube. Our social worker had come to sit with me while he was in the OR and I was happy to have her there when he came back. It is quite shocking to see your little boy with a breathing tube. Though it was actually a relief to see him breathe without all that effort. In addition to the breathing tube, he came back with a peripheral IV in each hand and an arterial line in his right wrist, and they inserted an NG tube for nutrition as soon as he was back. All the teams from the OR (Anesthesia, ENT, Respirology) along with the PICU Attending gather in the room for a "handover" which is sort of like a debrief. I learned that Eric had treacheitis, that his airway was 60-70% blocked by some gunk (my word) and they even showed me a video from the bronch (eww) that they had attempted a lavage but the gunk caused bleeding when they tried to remove it, and that they had taken samples in an attempt to identify the gunk. They expected it was an infection of some sort, but also wondered if perhaps there was a small perforation in the trachea causing the pneumomediastinum and subcutaneous emphysema. That was another new phrase I learned that means air bubbles collected under the skin. In Eric's case the pneumomediastinum was also sending air up into Eric's neck which is why he looked so swollen. The doctor told me it felt like rice krispies under his skin! On the plus side, Eric's lungs were functioning fine. They inserted the breathing tube a little deeper than normal to bypass the tracheitis and wanted to give about 72 hours for the trachea to heal. The plan was to go back to the OR on Tuesday, have a look around, and hopefully extubate. Eric's Oncologist came to see us a couple of times on Friday, but then left on vacation for 2 weeks. Terrible timing!
The next few days are a bit of a blur. Eric woke up shortly after the debrief and went straight for the tube so they had to use restraints on his wrists. They obviously increased the sedation as well, but they were having a really hard time keeping him appropriately sedated and he would sit bolt upright and become quite agitated. They were worried about the safety of the tube, so ended up adding a paralytic to the mix as well. It was very difficult for me to watch the inadequate sedation. Sunday night was particularly rough and I remember thinking one more night, I only have to do this for one more night, and then he will be extubated Tuesday morning.
During this blur, Infectious Disease (ID) stopped by to let me know they had found fungus in the samples that were gathered in the OR that first day and were going to start him on an anti-fungal but that it would take a little longer to determine what specific fungus it was. A day or so later they came back to let me know that it was Aspergillus Fumigatus, a very common fungus that is all around us. For those of us with healthy immune systems, you probably aren't even aware that it is all around us, but since Eric is immune compromised it was able to take hold. The ID Doctor said that Eric has probably had this for months! He was on a prophylactic anti fungal for a long period of time for this reason, but it was stopped because it interacted with a chemo med that he was on only briefly in the winter or spring.
Tuesday morning they took him back to the OR. The plan was to have a look around, take tissue samples (they'd only taken sputum samples the first time) and hopefully extubate. While he was sedated for this procedure, they also took him for CT to identify any dissemination of the fungal infection, beyond his trachea. They did find 2 spots on his lung and 1 on his liver, but his head, sinuses, etc looked clear. About 5 minutes before Eric returned from the OR, the nurse let me know that he was on his way back and he was still intubated. I guess I hadn't really considered the possibility that they wouldn't extubate him and I was a mess. This was the start of a very bad day. The Respirologist on service for the week was the same one that treated Eric's pulmonary GVHD and it was nice to have a familiar face. He came right over to me before the debrief/handover and said that Eric just needed a little more time. To which I, of course, replied "how much time?" He said that he could take a look, bedside, on Thursday and potentially extubate then. In the debrief, he also mentioned that it was an "atypical presentation" of the fungal infection. I think by this point they had decided there was no perforation and the extra air in the chest cavity and neck was due to the fungal infection. After rounds that day, the Oncologist that was on service for the week came by the room. She works as both an Oncologist at BCCH and at Canuck Place Children's Hospice. She was wearing her BCCH hat that day but I can never forget her other hat since having to have a conversation with her in that capacity a couple of years ago. She put on a serious voice and told me that Eric was "very, very sick" and then told me that the lesions they'd found on his lung and liver could be leukemia. I was flabbergasted. This hadn't even crossed my mind as a possibility. Also isn't leukemia a blood cancer? How could there be lesions? She said that sometimes it does present in deposits. WORST DAY EVER.
Later that same day, the Infectious Disease Doctor came by and told me that it was fungus. It was all fungus. No leukemia. I sobbed and had to tell her they were tears of joy. Apparently this was only the second time in her career that someone wept with joy finding out that their child was riddled with fungus. I am still really mad at that Oncologist; there was no need to tell me what the lesions "could be" when we had definitive answers within 2 hours. Fungal infections are not easy to treat and the anti fungals do not work quickly, like an antibiotic does against bacteria, but fungus seems doable; more cancer seemed insurmountable. They added a second anti fungal to treat it and removed all of his chemo meds in order to stop suppressing his immune system so that it has a chance to fight the infection. Obviously, this made me very uncomfortable, but I discussed with a different Oncologist who I like and trust, and she told me that it's a balance and right now the fungal infection is the more acute, life threatening problem so we need to address it with everything we've got, but we will adjust as the situation changes. Luckily, Eric's blast count was very low at that point, sitting around 0.2 and then dropping to 0. Additionally, now that they knew the deposits along his trachea were, in fact, just an atypical presentation of the fungus infection, they decided that instead of taking a bedside look around on Thursday, they would instead go back into the OR and clean up (debride) all of it. In the meantime, we continued to limp along with poor sedation, and it seemed like a new sedation plan every 12 hours as the PICU staff changed.
On Wednesday morning, I cried during rounds and said they needed to do better with the sedation. I didn't love the tube, but really was okay with Eric needing more time to heal, if only they could keep him sedated! Instead, they went the other direction and ended up peeling back all of the sedation and adding some anti anxiety meds. Eric woke up, with his breathing tube. He was curious about it, and touched it, but he didn't try and pull it out. I explained to him where we were and why he had the tube, and I had to do that a few more times as the sedation fully left his system since it also affects memory and, luckily, Eric was not aware of having had the tube for many days. We learned to communicate with him typing on his iPad, which also got better as the sedation left his system. I was able to personalize recordings on a button and made one that said "Mama" so he could get my attention if needed. We did have a speech language pathologist stop by, but she was mostly just going to set us up with the stuff we already had for communication. It was really, really hard and very tiring, but so much better to have him alert and relatively comfortable than what we had before. It is very rare for adults, let alone children, to be intubated and awake. They were so impressed with Eric. One of the Charge Nurses asked if they could take a picture for their staff education sessions.
Eric kept asking when the tube would come out and I kept saying tomorrow/Thursday/at noon. Unfortunately, on Thursday, he again came back from the OR with the tube. However, this time, it was because they cleaned up all the junk, and they were able to get it all!!! They said his trachea had been 60ish% blocked and the top third of his left bronchus was completely blocked, but now it was all patent (clear.) Since they'd been digging around in there, the area was inflamed, so they wanted to wait 24 hours before extubating.
Friday morning, one week since the tube went in, and it was finally coming out!!! They had initially said 11am and then noon. Eric was so annoyed. He said (typed) "it's already been a couple days!" I figured I would just roll with that and not bother letting him know that it had been a week unless he asked. The tube finally came out and I was emotional just to hear his voice for the first time in a week! Eric had his request of a cold and bubbly Sprite in the fridge and ready to go when they told him that he couldn't eat or drink for 2 hours. He was not impressed! They did let him have ice chips and an hour later I saw the doctor in the hallway and asked her and she said he could have his Sprite. After that he had some yogurt and jello and soup. We were just supposed to follow his lead and go slow, but he chose all soft foods that first day. I asked for physio to come by that afternoon. After a week of being bedridden I knew we would need them and didn't want to wait for the weekend to ask when there is only a physio or two around for the whole place. Eric did some light standing that day. When he commented on the challenges of standing, the physio said well you haven't done it in a week! She ripped that bandaid right off! Eric was very surprised and a bit teary at having lost a week, but I explained that it was the meds that made him forget and he seemed to just accept that and move on.
The following day, Saturday, around noon, Eric was moved back up to T8, our "home" floor and boy did it feel good to be back here. Everyone knows us and they were all saying how happy they were to see Eric back and looking so well. On T8 we have a bathroom in our room, unlike in the PICU, so Eric's big goal for the day was to walk to the bathroom. When it was time, I was ready to support him and his IV pole but he basically just told me to get out of his way and motored himself to the bathroom unassisted. I asked if he wanted a chair to sit on to rest while he brushed his teeth, but nope! He walked there, stood and brushed his teeth and walked himself back. What an impressive kid! Other than short walks to the bathroom, our goal for the weekend was to stay out of the PICU and Eric nailed that too. His oxygen saturation was good, and he was able to eat and drink and swallow meds.
Stay tuned for next steps ;)
