Time sure flies when your busy! Next thing you know it's been a whole year since your last blog update. Sorry friends and family, new and old! For those of you who are new to Audrey let me give you a brief introduction. She is a sweet six year old girl who loves anything pink and sparkly! Even at the age of six, when it's time to leave the house she puts on her Cinderella heels and piles on the "jewelry" making sure to grab a purse filled with "treasure." She's that kind of girl :) She also happens to have mitochondrial disease which has stolen pieces of her body and life over the last 4 years. This terrible disease has lead to severe GI dysmotiliy resulting in an ileostomy and total dependence on IV nutrition, neurogenic bladder requiring a mitrofanoff, intermittent hypoxia requiring intermittent oxygen, dysautonomia, adrenal insufficiency, POTS, hypoglycemia, immune deficiency, learning disabilities and so much more. Feel free to scroll back over previous posts for more details :) Now, lets see if I can fill in the gaps and catch you up to sweet A today :)
January 2015....the new year started strong but her nemesis of low blood pressure overnight was prominent. Audrey suffers from dysautonomia. This is best described as everything your body should do automatically doesn't work quite right for her. You can tell during the summer when it's hot out, her body is hot instead of cooling down. During the winter when it's cold, her body is cold instead of warming up. Overnight when her body is resting her autonomic system seems to rest too. Unfortunately that means her blood pressure drops dangerously low and we can't wake her up. She resembles an unconscious person with no movement, her skin is cool, her color is grey. Disconcerting to say the least. It also stumps her doctors because there are very few medications on the market meant to increase blood pressure, especially in a five year old! So January saw a small hole in her line which required a quick trip to the emergency room for a repair. While waiting for her repair, she fell asleep and the ED staff witnessed her sleepy autonomic system. This earned her a couple night stay in the PICU. Once again, her doctors were stumped and so we returned home without any solutions.
February was much the same. Her doctors were getting more desperate at this point and thinking of trying newer medication which had not been tried in children her age. They were also discussing off label uses with medications such as caffeine. We were giving as much as 400mg of caffeine at bedtime in an effort to increase her blood pressure...all that was happening was a bad tummy ache. Aside from her overnight drama, keeping myself up, she was doing fairly well during the day. She had been out of the hospital since November and had only had a quick colonoscopy with her surgeon in December. She was otherwise "stable."
Stable in mito is so misguiding. It lures you into this false sense of security that maybe everything will work out. Maybe she will be the one to beat the odds. This next part will be long and detailed. I haven't written these experiences out and only my best friend knows the terror and horror I felt and still flashback to all these months later...
March started off great! Her best friend was staying with us. We had a couple snow days which were great fun. Things were going well. Still dealing with her pesky BP at night but no major changes. On March 10, her GJ tube was accidentally pulled out. No big deal! It had been six months since her last change and by the looks of this fuzzy, black tube it was way over due! She had it switched out on March 11, as painlessly as possible for someone with chronic intestinal pain, and we thought all was well. The night of March 12th Audrey said she wasn't feeling well. She quickly wasn't looking herself and her heart rate was rapidly increasing. As I ran around the house packing for the hospital her fever started. I rushed her to her hospital. Though she was sick, she was just stable enough for me to make the extra trip to her hospital, to the doctors and teams that know her best. She was taken straight into the trauma room because by then she was so lethargic they were doing jaw thrusts to try to get some sort of response from her. Working of the assumption she was septic, cultures were taken and heavy antibiotics were started. It was already Friday the 13th at this point. She finally stabilized enough they sent her to the floor and she looked ok the rest of Friday into Saturday. Saturday early evening her temperature respiked and she quickly decompensated. By change of shift, her nurses that knew her so well were calling a rapid response to the ICU and we got the news that her latest blood cultures were growing yeast. Anyone with a immunosuppressed child and a central line knows to fear that organism. If there is one thing that will steal your child from you, it's fungal sepsis. So she was sent to the ICU were the doctor reassured me she wasn't "too" sick so hopefully a couple days on antifungals and she would be back to her regular floor. She remained stablily sick if that makes any sense. Not getting better and maybe getting a little worse each day. By Monday her fever was difficult to control even with the cooling blanket and IV tylenol. She was working a little harder to breath and she was on increasingly more oxygen. By 3am Tuesday they were trying bipap which failed and at 7am she was being intubated.
So I have been a nurse for over 10 years. I have seen more intubations then I can count. Nothing prepared me for that moment when her amazing fellow walked in and said, "I'm sorry your daughter is not doing well." I sobbed like the mother I was, forgetting all of my nurse training, feeling terrified for my little five year old surrounded by so many people trying to keep her alive. I held her hand as meds were given and she was quickly intubated and placed on a ventilator. I prayed for her to continue to fight and begged her not to leave me. The next two weeks were the scariest I have ever lived. I saw my daughter with oxygen levels at 18, far below the 100 that she should be. I saw her medical team exhausted and running out of options. She failed different modes of ventilation, she showed no improvement with other gases such as nitric oxide. She stopped responding to her sedation medications so she coughed and gagged for hours on end. We knew she had developed ARDS and that her lungs were very sick. What no one knew was whether or not she would be able to recover. In between her coughing and endless amounts of medications being given, she was responding to me. I had a couple of little smiles from her which gave me hope that she was still there, still fighting. Finally after two weeks, we were out of sedation options. It was either try to push her lungs off the ventilator or put her in a medically induced coma and lose what precious few gains had been made. We opted to try to push her body. To everyones great surprise, she started to improve. Every day I anxiously listened to rounds, hoping to hear, this was the day she would be extubated. Finally, through my begging and pleading, the doctors gave her a try at extubation though she wasn't quite where they wanted her to be. After 20 days on a ventilator my girl was breathing on her own!!! Within 24 hours she was out of the ICU and back to her regular floor. A changed girl for sure, but I knew she would recover. She was too weak to hold her head up. She was on 2 liters of oxygen continuously. She had lost all her muscle mass in her legs and arms. Within a week, she was strong enough to sit up and she had been weaned off the last of her sedation medications so she was sent home!!! My sweet girl showed everyone just what a strong fighter she really was. That being said, I know my girl is truly and miracle. Without the endless prayers from family and friends she would not be where she's at today.
Unfortunately she was only home for a week when she was readmitted for a UTI. Then she was home another 10 days and was readmitted with a blood clot in her left jugular. Yet another medication to be added, now she had to follow with hematology for blood clots and anticoagulants. But through this time, she continued to get stronger. She was walking on her own, she was building muscle, and six weeks after she was ill, she returned to her precious ballet class.
The following months were times of hope. She was able to perform in her ballet recital. She restarted her IV immunoglobin to stop the relentless infections that were threatening her. She had her gallbladder removed in June and then...nothing. Absolutely nothing happened all summer! She played with her friend, she went swimming, she laughed and loved and was far way from the hospital and doctors. It was a time of healing for Audrey and myself. We enjoyed every minute with her having been so brutally reminded that her life is so fragile.
September continued our feelings of hope. Audrey turned six! She grown so much and every year we celebrate is a year mito did not win. She was able to start kindergarten which she had been looking forward to. September and October were filled with endless assessments by therapists to fine tune her IEP which is still a work in progress. Audrey's brain works very differently and it's hard to find the best ways to help her learn. It's not that she can't learn or be taught but it's a matter of finding the key that allows her brain to understand. This still is, and will likely always be, something we are struggling with.
This brings us up to today. Audrey's last unplanned admission was in April. She's had two planned stays, one in June and one in September, that were one night each. She continues on her immunoglobin and has done amazing! She is off all extra oxygen during the day and her lungs are pretty much back to where they were before her ARDS. Her blood pressure continues to be an issue. She is on crazy regimen now that changes how we manage her every night. There are three different medications we rotate around to be given at bedtime. Giving any one of the medications too frequently allows her body to become accustomed and efficacy is lost. We continue to battle her sleepy gut which still does not want any food in it. We are trying to preserve her liver and so have taken her off TPN for four hours a day. This has been wonderful for her and she loves running around during "free time." To say that life is easy or she is easily managed would be a great lie. However, in the face of all that happened this year, I would gladly spend every night awake keeping her autonomic system active, just to see her sweet smile every morning. This next month has us facing yet another new problem. Audrey has narrowing in her great vessels in her chest that are going to require intervention. This intervention is very scary and highly specialized as it is placing stents inside her little six-year-old veins to open them up. I have faith in her medical team that they will do their very best to ensure she pulls through safely. I also know just how strong she is and she will continue fighting with every cell in her body. So long as she is up for the fight I will be right along beside her, holding her hand.