One of my favorite children's stories growing up was that of Peter Pan. Beyond the story's most notable characters of Peter Pan, Tinkerbell and Captain Hook, there is one who heightens the calamity and brings on giggles of anticipation from my kids every time he makes an appearance. His name is Tick Tock the Croc and this character lives for one motive.. to get one more chance to eat Captain Hook after getting his first taste when he bit off Hook's hand in an earlier altercation. He has an insatiable appetite, even having swallowed an alarm clock in his quest to be satisfied. With this clock somehow permanently part of his gastric system, Tick Tock can be heard ticking from a distance as he inches closer and closer to the prize he hunts: Captain Hook. Well, I share all this with you because our sweet boy has adopted his own Tick Tock in the form of his recent implantation of the permanent gastric stimulator. We hope it will be as determined as the Peter Pan character at meeting it's objective and taking out the villains in Sammy's daily life caused by his gastric paresis and dysmotility.
Sofia was actually the one to give his medtronic device a name.. similar to a pacer, there is a clock of sorts working to give off calculated electrical discharges at timed 3 second intervals into the outer lining of his stomach muscle. The makers of this technology can not fully explain why it works and scientific analysis is still in its beginnings. These devices have long been prescribed and allowed as a form of enterra therapy under a humanitarian use waiver by the FDA. You can imagine we were skeptical when first offered this as a possible solution to his seemingly constant and increasingly worsening problems associated with his slow gastric emptying and motility. Because Samuel had a nissen fundoplication done at 2 months of age to prevent any type of reflux or aspiration of stomach fluids, he has never vomited or refluxed in the typical fashion. He instead would writhe with feedings and when his g tube was vented, large amounts of stomach contents and sometimes intestinal contents and bile would come out into the open syringe. At first these episodes were few and far between but over time they were becoming an exhausting and debilitating part of his condition and pointed towards signs of disease progression that might mean more interventions in the future. Interventions such as permanent central line feedings which had only been needed for short periods in his past. I have blogged in detail however at the remarkable positive changes we experienced with the trials of the stimulator over the last four months.
So to bring you up to speed on the events of the last month.. After returning home with his last temporary stimulator, things went well for about three weeks and then we needed to head back in mid November for a check up with his surgeon. Four days before I started noticing some changes in Sam for the worse and on the drive down he became unable to tolerate the usual feeding amounts. The Dr found that the leads were faltering and removed the stimulator in his office. He then wanted one more trial at home for one week with no stimulator in place at all. Now he always reminds us that most patients continue to feel positive impacts for days or evens weeks following its removal. We think it started to cause him significant trouble within three days. By the Monday following our appointment Sam was running fever, lethargic, acting "off" and then dropping oxygen saturations even while on oxygen and while not dealing with any respiratory distress. Sam is not used to higher fevers as he is usually in pretty bad shape with low grade fever and we have to act aggressively to get ahead of what is plaguing him. We figured it was either A. a bacterial infection like ears which we could not find, B. a virus we picked up somewhere. or C. his bodies way of alerting to us that the body was not meeting the bodies metabolic needs due to the stress of the losing the stimulator benefits. We just could not know. Now I would suggest we should have done blood work or cultures that could have shed more light but I think our Dr from a practical position could not see how it would change what we were doing to support him, which was 10 day antibiotics (cefdiner) and lots of fluid and rest. The surgeon might have disagreed.. as he did not want to go to surgery not knowing the prior status pre antibiotics.. and I could see his point. Anyway, we ended up pulled off the antibiotics on Day four when he broke out in a rash. Now then there was the question of whether rash confirmed that it was a viral process going on or a reaction to the antibiotics. As the weekend came round and we were still scheduled for surgery on Tuesday, I worried we would be postponing. Sam ended up making a turn and with no fever by Friday evening, we were on for admission monday, surgery tuesday. The girls were out for Thanksgiving break and we made the plan to all go down together and support Sam through what they said would require three hours in the operating room and up to a week in the hospital.
My In- Laws lended us their spacious conversion van and we got Sam admitted on Monday. I left Jason there to get him settled in while the girls, Max and I found a hotel since there was no space at the Ronald McD house. It is no easy feat at night to unload a van and check in to a hotel with two bickering girls tired from a long day in the car and one hungry baby in tow! God gives us strength in the most curious of situations. I have to say that my girls learned real quick what I needed in way of their help. ;) I was so pleased though with the place we stayed cause they had a nice breakfast in the lobby that was healthy and the girls relaxed by a roaring fire while I took in all the details about Sam down in surgery that next morning. I was a little tenser because usually I am the one there to send him off but at the same time, I was glad Jason could be more involved in the caregiver role within this capacity because then he better understands what it is like for me when he calls from home and I am inpatient with Sam. Until you are in each other's shoes, this can be a delicate area of your coping as a couple. Luckily, we have always respected each other during these difficult periods and it has made all the difference. I have met so many families that fall apart when under the duress of having a medically complex child or stressful situation.
Then just to make things that much easier, in came our dear friend Amber and her family with rescue offers of taking on my kids while we were staying that day close to Sam in recovery. They even took Max which took a lot of convincing because I could only imagine how she and her husband would juggle their own three with my extra three. I mean, they have a complicated little guy of their own and enough equipment and oxygen and meds etc to run circles round what most kind neighbors would have in offering to keep a few extra kiddos.. but God must have been laying on them because she would not take no for an answer and soon all the kids were in their backyard and watching movies, having a grand old time. They even kept Sofie and Max through the night while I took Bella to her sleep study which was being done to check out some respiratory and daytime focus concerns.
Sam was in the OR longer than expected but they were having to work with a small abdominal space and they had told me ahead that this might be the case as he was the smallest they had worked with. His buddy Nate was not much bigger and about a year older and they ran into the same issue with him. So he was in for almost five hours. His recovery started out with good vitals, a lot of pain controlled by meds and blood suctioned by NG tube. His incision looked good and vitals were good in that first day. Second day was much rougher and although I expected that, I don't think we expected as much in the way of airway swelling concerns as he experienced. He was in distress and pulmonology was quickly brought on board but he responded well to the epinephrin and prednisone steroids they jumped up to get ahead of these issues. Later that day he actually had a pretty significant Brady event that had Jason and the nurse responding aggressively when he turned blue and stopped breathing at one point. This was something I actually was prepared for as these autonomic events seems to become more frequent when he is taxed and exhausted..metabolic crises of sorts.
By day three, things were looking up and we shared a Thanksgiving Dinner together around his hospital bed.. styrofoam meal and all. It was very graciously brought to all the families in the children's ward by Parkside Baptist of Gainesville.. what a kind ministry to share a meal with families by bedsides of loved ones when nothing in the hospital was open besides a vending machine. It may have been the most special Thanksgiving ever for this family.. just being together and supporting our little trooper. At that point Sam began asking for food and was eating jello and shaved ice in tiny amounts. He started back slow formula feeds and myself and the kids stayed in the ronald mcd house awaiting word on when he might get to go home with short visits to swap out sitting by Sam. It was on the weekend that we noticed he needed less morphine and then down to none. At the same time, however we watched him experiencing low heart rates, prolonged all the time and not just as a brief Brady episode. He usually has a heart rate around 100 to 140 when awake and it was trending in the 50s and 60s. No origin was attributed to this change but by Sunday afternoon when he had his first BM and was cleared to go home, he started having higher heart rates. We felt better making the trip.
At home Sam is doing well.. far better than we ever expected. He had a few Bradys on his first few nights home but respiratory has been great. His incision which was fully open to the abdomen is healing well and the box which was pocketed under his skin is not bothering him either. He has a few times tried to pull at the skin glue and we are trying to train him on safe ways to move and do things with the new device. He started back to therapy and they are going to incorporate how to best work around it as well. One thing I have to learn is all the things he should avoid contact with like cell phones and radios, microwaves and security devices. These can not only interrupt with the integrity of the device but some can cause physical harm. tHe device feels weird when you touch it from the outside but he doesn't seem to even notice it. His stomach is very distended and we re trying to keep him from jack knifing or twisting too much. He talks about his Tick Tock and the girls tease him that they can here him ticking down the hall.. of course it is like a pacemaker and does not make a peep.
On the agenda moving forward will be follow ups with most of his specialists coming do.. plans to maybe see my mom's family before Christmas and maybe setting up to meet with the Houston Drs at the mito clinic. I had put this off cause I just could not get all his records organized and sent over till now but things should get easier in days ahead. Sam is just showing us such positive improvements with both this stimulator and the supplemented oxygen he is now wearing through the night and intermittently through the day. I did get a copy of his sleep study results on this trip and he experienced 31 apneas, only 5 of which were obstructive. He had 81 oxygen desaturations and recovered well with oxygen placement. I couldn't believe how concerning this was as that was as that night he was doing great and feeling wonderful.. what must it be when he is sick? yikes.
So the last news I wanted to share is difficult. I am praying for a family we have followed for some time whose child had mito, complex 1 and was two days shy of his 6th birthday when he passed away suddenly. In many ways, I thought this child less complicated and was shocked when I found out he passed away this week. This is such sad news for his loving family and friends. Our friend Amber knew them personally and from reading their blog I felt an instant kinship to this sweet mom. I pray peace for them and God cover them with love and support. I cant even imagine this loss. I know I cant live in fear but it is terrifying to understand the tragedy this disease can purvey on little children.
Time to get moving as I am way behind on everything. Will be a busy weekend..Tick.. Tock..!