CAMP recap

A lot of people have been asking how the evaluation at CAMP went (Center of Autonomic Medicine in Pediatrics at Children’s Memorial Hospital in Chicago, IL). It was a very long and hectic week, a lot of changes were made and unfortunately, some unforeseen events happened immediately after we returned home. Aside from a few Facebook updates, I haven’t had a chance to gather my thoughts and write a detailed blog post. So let me try to sum it all up now.

The drive to Chicago: We left Friday, February 10^th around 5pm. My husband, Wyatt and I in our SUV, and Grandma and Grandpa followed in their rental minivan with our other three kids. Why the family trip? Well, we figured an actual vacation is not in our near future so we might as well make the most out of this trip. My husband has family near Chicago so it would be a chance for the kids to spend some time with everyone and hopefully see some snow. We wanted to drive overnight in the hope that the kids would sleep through most of the 20hour ride. As usual, kids have their own ideas and with constant stops for diaper changes and breath holding episodes, we ended up having to take a quick nap at a rest stop to push through till Indiana where we stayed, or I should say crashed out, with relatives for the remainder of Saturday. Sunday we then took on the remaining 4 hour drive. I have to mention here that stopping at any time anywhere with all of Wyatt’s gear is an undertaking every time. It’s never just a quick unbuckle from the car seat, switch that diaper and of we go. Unbuckling him and taking him out of the car seat is a challenge because of the vent circuit always getting in the way or getting snagged on something. And then there is also the Pulse Ox wire, and if it’s feeding time, then the feeding tube is yet another obstacle to fuss with. Once we arrive anywhere for a longer period of time, we then also have to unload and set up everything which takes a good 30 minutes.

Camp day 1: Everything was really hectic and disorganized. Wyatt was supposed to be admitted around 2pm. We were staying with relatives 30 minutes outside of Chicago the weekend prior to admission to the hospital and a friend graciously offered us their condo in downtown Chicago for the nights that Wyatt would be inpatient.  The condo was about 10 blocks away and with traffic in and around town, we left early to check out the area. Once we got to the hospital we had to go downstairs to admissions to fill out some paperwork. Since we arrived early we had to then wait and decided to grab some lunch. My first impression of Children’s Memorial Hospital wasn’t great. I felt claustrophobic in that building. It’s an old building with low ceilings and small rooms. When we were told a bed was available, we were taken to the PICU (Pediatric Intensive Care Unit). That’s where Wyatt was supposed to spend the first night to be observed. It didn’t sit right with me that he was in the PICU though. I understand that is the one place where he’d get the best nurse to patient ratio so therefore he’d be observed closely but at the same time, who knows what all those other sick kids were there for and what type of potential infections and illnesses were just lingering around the corner. Wyatt was hooked up to all the monitors to keep an eye on his oxygen saturation, heart rate, co2 levels, as well as frequent blood pressure and temperature checks, and weighing every diaper for input and output comparisons. It was basically impossible to pick him up and hold him with everything he was attached to. Of course with all that stress and us not being able to comfort him, he went into a few breath holding/cyanotic episodes and I was ready to cry because I felt like we are voluntarily torturing our son. Toward 5pm Dr. Weese-Mayer came into the room and we went over his medical history and a bunch of clarification questions. Thankfully we had a good night nurse and after a long debate between my husband and I, we decided that it’d be best if we both went back to the condo to get some sleep since we knew we’d have a long week ahead of us. So around 10pm when Wyatt finally fell asleep we were able to leave.

CAMP day 2: It took me a minute to realize where I was when the alarm on my cell phone went off at 5am. I had a hard time falling asleep that night wondering how Wyatt was doing and whether we made the right decision to bring him here for evaluation. With a quick Starbuck’s run, we grabbed a cap and arrived back at the hospital at 6am. Around 7am Wyatt was moved from the PICU to 9West (a higher floor for ventilated kids) where he would stay for the remainder of this hospital admission. That room was even smaller (semi-private) than the PICU room and not very clean. We were told that around 9am the CAMP staff would come and bring Wyatt to their lab. Here I was imagining that CAMP would be such a great impressive program with its own hospital floor because that’s how Dr. Weese-Mayer described it (“our floor”). The CAMP lab was great and spacious and clean but it was literally just three conjoining rooms that Wyatt would be brought to from 9a-4p (the day room) and again from 9p-6a (the night room) while being observed by the CAMP staff from the third adjacent room with a bunch of cameras and monitors. In between the CAMP lab, he’d still have to go back to his room on 9West. Each time going back and forth meant unhooking all of the leads used at CAMP (and there were SO MANY leads on his head, arms, back, stomach, plus the Holter monitor), rolling him in his hospital bed back to his room and then hooking him back up the monitors there. Of course, moving Wyatt at 6a and 9p meant he’d always get woken up when he’d normally be sleeping which messed up his whole daily schedule and didn’t put him in a good mood. That in turn then meant that it was very difficult to get him to cooperate during the evaluations and was having more episodes. Unfortunately the back and forth was going to be a daily hassle we simply had to accept. One of the first things that the CAMP staff decided to do was to change Wyatt’s trach from his 3.5 NEO flextend cuffless Bivona to a 3.5 NEO tight to the shaft cuffed Bivona which they intended to only inflate with 1cc of sterile water and to do so only while sleeping. We voiced our concerns right away that a TTS trach would not work for Wyatt because he has no neck yet and it’d be a source of irritation and would limit his head movement. The reason behind the trach change we were told was that he’d be better ventilated and the co2 reading would be more accurate. So we decided to give it a try and see how it goes. (I’m going to try my best to stay objective here and not give away our current opinion on decisions that were made at CAMP. I’ll save that for the next blog post.)A lot of ventilator setting changes were made without much explanation except that it was done to optimize his ventilation. The goal was to come up with a vent ladder system to would allow us as the parents to make ventilator setting changes at home based on whether Wyatt was awake or sleep and based on his co2 readings. The main conversation topic of course were Wyatt’s severe breath holding/cyanotic episodes that always ended with deep desaturations and him passing out. Previously we had been told that there is no need to ambu bag him during these episodes because his airway is completely closed off, bagging him would put too much pressure on a closed airway that it could do more damage than good, and that by the time he passes out and relaxes, the vent can finally kick in and do its job again. CAMP now decided that we need to bag him with O2 during each of those episodes to help him recover faster. We were told that these episodes can severely compromise his neurological status and that we need to at all cost prevent them from happening. I have to mention here that at that point in time we were already observing that Wyatt was having a lot more episodes than his norm at home. We mentioned our concern to the CAMP staff and explained that we were aware of the severity and the need of prevention and that at home we had narrowed down what situation or behavior is most likely to cause an episode and we had effective comforting measures to prevent these episodes 90% of the time. The response we received was that they weren’t too concerned that he was having more episodes than baseline and that it’d be better for the CAMP staff to see him at his worst because if they can optimize his ventilation at his worst than we’d be well set for managing him at home.

CAMP day 3 and 4: I don’t remember exactly what happened on what day so bare with me. CAMP informed us that their monitoring revealed that Wyatt was having random desats even as low as in the 40s while awake and happy and without any physical signs or symptoms. Based on that information combined with his breath holding episodes when he gets mad, they put him on 7L of oxygen continuously while awake to give him a higher blood oxygen reserve in the hope that he won’t desat so low and recover faster. Those dips in saturations didn’t make any sense to us as we had never seen that happen before at home. At the same time we started to panic thinking how are we supposed to get Wyatt back home on 7L of continuous oxygen over a 20+hour drive when he didn’t used to need any oxygen at all. A portable oxygen concentrator can’t give as high as 7L and 7L continuously equals a ridiculous amount of O2 tanks to be traveling with. It all seemed really surreal.  Then the team also informed us that they found a 2.9second pause in Wyatt’s heart rate during the first night in the PICU. Spontaneous prolonged sinus pauses are a risk factor for CCHS patients and over 30% of CCHS patients will end up needing a cardiac pacemaker at some point in time. The cutoff for recommending a cardiac pacemaker is 3.0seconds. Needless to say Wyatt was right on the edge of the cutoff and that was a concern. To some degree we were doubting that the monitoring in the PICU could be trusted since leads can easily come off partially and give false readings. But then we also got anxious to hear the results of the 72hour Holter monitoring that is routinely done at CAMP. Meanwhile, my husband and I kept trying to make sense of it all. Why would Wyatt suddenly have these random desats and need oxygen when that was never the case before? We also noticed that his resting heart rate during sleep was higher than usual. The first couple days we thought it was the stress of traveling 20hours and going through all these assessments but something just didn’t feel right. I have no other way of explaining it but that Wyatt simply was too calm when awake but then also had far too many episodes when he got upset. We again spoke with CAMP staff about our concerns that Wyatt is just not acting like himself. We explained that we were convinced he was getting sick but CAMP tried to reassure us that his behavior could simply be different now that his ventilation is optimized and he’s had a few days of having to calm himself down by being in the bed by himself instead of us holding him for comfort. I can’t tell you how many times we went back and forth with them. We told them, he doesn’t normally just slouch over in the high chair and just sits there, he also doesn’t normally just lay in bed all chilled out. Typically he’d be pulling on wires, kicking and grabbing things, playing, laughing and only having an episode or two when he’s overly tired at the end of the day. Something just wasn’t right and it wasn’t just the stress of everything that was going on.  And it certainly didn’t sit right with us that our concerns were just brushed off. Yes, CAMP is the expert on CCHS and is amazing at optimizing ventilator settings and making them applicable to different activity levels but we were getting the impression that to them it was all about getting all their evaluations and numbers in those 5 days, not much else. So no surprise, they felt that Wyatt was severely developmentally delayed as he was not interacting much nor did he want to play despite us reassuring them that he does all those things at home. We even had our local therapist fax them Wyatt’s recent developmental assessment that was performed. Another issue we kept running into was the TTS trach. Wyatt was not comfortable with it and every time he moved his head the circuit would come disconnected, even when trying to use twill to keep it in place. Since he has no neck, it then also made it very difficult to get a hold of the trach to reconnect the circuit and it was aggravating him further every time that happened. We told CAMP every single day about these issues and that this type of trach would not work for Wyatt. We told them that we are not against a cuffed trach but that it had to be a flextend. CAMP staff replied that a cuffed flextend trach is a custom order so therefore the TTS was our only option at this time to be able to use the cuff and optimize his setting  and that Wyatt would get used to it over time. In not so nice words I told them that at home I would have already switched him back to a flextend trach to ensure his comfort regardless of the cuff or slightly more challenging vent adjustments. Things kind of escalated by Thursday. The CAMP staff acted like they were convinced that we were against their recommendations and in turn we were frustrated with them that they didn’t value our parental input. We were over being told numerous times that simply blowing in Wyatt’s face would stop his episodes as if we had never tried that before and as if we were just sitting back relaxed when Wyatt has an episode. I got so angry at them for talking to us as if we were careless and weren’t aware of the potential neurological damage that each episode can cause. We ended up insisting on a sit down with Dr. Weese-Mayer to go over what medical discoveries were made, what the recommendations were and to simply clear the air. We were not against CAMP or their recommendations and we value their input. But we took offense to them trying to tell us what is normal and not normal for our son, to them telling us that we shouldn’t hold our son that much to comfort him and that he shouldn’t sleep in the same room with us at home. I really don’t care if you are a world famous expert but you are not going to sit there and tell me how to parent my child. You know absolutely nothing about me or my parenting style. Anyone who knows us is aware that we do not spoil our children and have high expectations of independence and responsibility from each one of them. Wyatt is no different. We simply have to prioritize. If holding him for comfort keeps him from having breath holding/cyanotic episodes while he is still too young to have other means of communicating with us, then so be it… While we’re on the topic of frustrations, we had a beef with the nurses on 9West also, mainly the night shift. Since Wyatt was only officially in his room on the floor from 4p-9p and then again from 6a-9a, some nurses were acting like they were only responsible for hooking up his feed. One particular incident that could nad should have been prevented pushed me over the edge. Sometime between Wyatt getting a trach change done at CAMP to upsize to a 4.0 NEO TTS cuffed Bivona trach during the day and him coming back to his room in the late afternoon, no one cared to check whether his trach tie was on tight enough and he decannulated with the cuff inflated. It was so loose I was able to put 4 fingers between his neck and the trach tie. And since his room was so unorganized and the emergency trach and syringes to deflate his cuff were in a spot that no one could easily reach, we had no choice but to reinsert the cuffed trach and then yell at everyone afterward. Not only were the emergency supplies not accessible but his nurse for that night wasn’t even anywhere to be found. Another nurse and RT came in the room to assist. Of course that incident caused trauma to his airway and he had a fair amount of blood in his secretions the next morning. So we ended up talking to the nursing director for 9West.

CAMP day 5: The last day of CAMP was just as unorganized and hectic as the first day when Wyatt was admitted. Everything was so rushed. CAMP picked Wyatt up earlier that morning even though the nurses and RTs on the floor were not even ready. The good news was that they had weaned Wyatt down to 2L of oxygen while awake and they were able to get Apria to help us with renting an oxygen concentrator and several O2 tanks for the few days we had left in Illinois after discharge, as well as for the 20hour drive back to Florida. While CAMP was testing Wyatt’s ventilation needs in the car seat, we talked with Cardiology to discuss the Holter results. Fortunately they didn’t find any other prolonged pauses but they did recommend to be proactive and get a 72hr Holter done every 3-4months. Endocrinology came to speak with us as well because there were discrepancies in the growth charts sent from Wyatt’s Pediatrician vs his Pulmonologist. We explained to them that we didn’t feel like his growth measurements were trustworthy because he would almost always go into an episode when the doctor’s office tried to take his height and weight, therefore, he wasn’t relaxed enough to stretch out for a good length measurement. We have noticed slow progress in Wyatt’s growth in regard to his length but aside from keeping an eye on it and making sure we get good measurements, Endocrinology explained that there isn’t really much that would be done until age 1. Another thing they wanted us to keep an eye on was his blood sugar level because they had one low reading in the five days at CAMP. They do believe that since it was only one low reading that it was probably due to a procedure mistake. But just to be on the safe side, they want us to check it daily for one week after his longest time period without food. The sit-down with Dr. Weese-Mayer summed up what the specialists had already said and she urged us to get home nursing again. Her primary goals were to control the breath holding episodes and to intensively work with OT,PT and Speech. She again went into her ideas of parenting techniques which we this time chose to ignore instead of getting angry again. What was frustrating to me was that even our final sit-down with her was spontaneously ended by the CAMP staff wanting to take Wyatt back to his room. The day ended by us waiting for the delivery of the O2 tanks and getting discharged. I can’t speak for my husband but I left feeling really overwhelmed without a clear understanding of what was actually accomplished in those five days other than identifying a lot of potential risk factors that may or may not happen in the future.

Driving back home: Simply put, it was a disaster. The plan was to leave early Saturday morning but the rental minivan that my husband’s parents were driving with our other three kids broke down. After getting all that sorted out, we finally left Saturday afternoon. Wyatt had diarrhea and kept going into back to back episodes every time he was awake no matter what we tried. We were even having trouble keeping his co2 level in range despite using the newly established vent ladder. Having to ambu bag your own child multiple times in the car during each episode is mentally draining. No one spoke up about the thoughts going through our minds but we all knew that our concerns of Wyatt being sick were true. We also knew though that our only option was to get home as quickly as possible to see our Pulmonologist who knew about CCHS and has seen Wyatt before. I was terrified that eventually he would have an episode that he wouldn’t simply recover from just by bagging him with O2. We ended up stopping at a hotel overnight and then driving the rest of the way on Sunday. We finally got home late Sunday night, Feb 19th.