Life continues to be so busy! I'm not a great blogger as life seems to get in the way and before I know it 6-8 weeks have gone on since my last entry. So here is a summary of what's going on with us and our sweet girl!
We are adapting to life with a feeding tube. Though I have taken care of babies with g-tubes for years during my time as a NICU nurse and it isn't uncommon for our Cranio kids to need feeding tubes at least temporarily, it's completely different living with one. The day to day cleaning, problem-solving, preparing and planning is just more than you could ever anticipate.Since the tube was placed it has never been comfortable. Whenever it moves or the the tubing moves or any care has to be completed she kicks and screams like she's in terrible pain. At first we thought it was just post surgical pain. Though it isn't usually thought of as being a super painful surgery, every child is different and maybe our girl has a low tolerance level. But as the weeks have gone on the doctors are stumped. We went to the pediatrician and back to the surgeon. The site looks great, there's nothing wrong with the tube, no one knows why it hurts her. Her in-home therapist think it has to do with some sensory input. For some reason she seems to be really bothered by things touching her and maybe this is why she is feeling pain from something that is never more than slightly uncomfortable. Maybe this is true. We've spent months working with her in therapy so that she is able to touch play-d'oh and play with sand. I hope that this is something she will overcome.
Thankfully, we have found some help on facebook! Little did I know about the large world available to parents of special needs children on Facebook. Many enterprising mothers and pediatric nurse have created and sell items that make life easier. We found the site for Belly Buttons and were lucky to be able to order 2 g-tube belts. These are cloth belts worn around the waist over the g-tube site which hold the g-tube secure and keep the tubing in one place. With the belt on the g-tube doesn't spin and the tubing doesn't pull and finally Audrey is comfortable with her tube! This site also sells (as do many others) g-tube pads! What an amazing invention! Instead of piles of spilt gauze and tape, these talented individuals sew little spilt disks of flannel, cotton and absorb able material which stick together with either snaps or Velcro. They come in a variety of patterns and colors including various princesses, pink, hearts etc... Finally with this small thing Audrey has control of one small aspect of her tube, if only which pattern to wear.
Aside from the care of the tube, the use of the tube has been another learning curve. As we have now figured out, when Audrey is tired her little body conserves energy by not processing food. So the days that she is very tired her feeding pump backs up and we have to vent/drain her tube often being surprised as hours of feeds come pouring out. At least now we can see this pattern so when we wake her up in the morning and see how tired she is we know what to expect for the day.
We were able to see the metabolic nutritionist in February. We were relieved to see her weight had improved to where she was before she was sick. We found out that she should be on a low fat diet as her body may have difficulty breaking down fats. So my skinny 2 year old with a feeding tube gets low fat foods, how ironic. We also needed to change her formula to Vivonex from Elecare as it has lower amount of fat but same amount of protein/calories. After more than a month of arguing with the insurance and then the supply company we finally received 5 days worth of formula. So the fight continues as obviously this will not help for long. We are already missing the sweet vanilla smell of Elecare as the smell of Vivonex brings back memories of old preemie puke, as my NICU colleagues will understand.
February brought us the Pasadena Rock-n-Roll half marathon supporting CureMito. It was great to be involved with this program and I was able to raise $400! Unfortunately that was not the $1000 needed to participate in the walk/run but I was OK with that. It was great to see the words "mitochondrial disease" out in the community. I am always amazed at how little known this disease is. My friend made t-shirts for us to wear the day of the raise which featured pictures of her son, who is also affected, and Audrey. We went to the race and supported our friends who were able to participate. We were even interviewed by the local newspaper. Hopefully next year we can participate. I also hope they will create a stroller option so we can have our kids who are so affected out in public to raise more awareness.
The month continued on to have me go back to work! By the time I returned, I really needed the "time off" for my own mental sanity. As bad as it makes me feel for saying so, I find I am unable to be the sole caretaker for my daughter 24 hours a day/7 days a week. With all of the feeding, pulse ox, oxygen, glucose monitoring, temperatures, etc. it's like being a nurse on duty 24/7 and as nurses know, we all get to the point where the money isn't worth one more hour of work. Obviously I'm not getting paid for this, but combine the nursing work with the emotional toll of caring for my girl and I was ready to lose my mind. So February 20th everything was set-up for me to return to work. Yay! The first 2 days went great! I was feeling so much better. Even though I carry a pager and the daycare calls me frequently with questions and she is right on campus with me at work, I was able to have a few hours each day when I didn't have to wonder if her oxygen was ok or if she was tolerating her feeding. While it was never far from my mind, I knew the daycare would call at the first sign of trouble...as they did on the third day.
I knew Wednesday (our 3rd day back) was going to be difficult from the moment I went to wake her up. She was curled in a little ball in her bed and cried when I tried to wake her up. The whole way into work she was slumped over so she didn't have to hold her thumb up to suck and drooling. Yep, big flag, no energy to hold her arm up and oral hypotonia. But I willfully ignored it all. I dropped her off at 8am and they called at 9am the her feeding pump was alarming. Uh-oh :( Sure enough her tummy was full. We shut off her pump for 2 hours and let her lay down in the class and read books (which is all she wanted to do). I ran back to my office to finish the few things I had to get done. By 1130 they were calling again. They had tried turning her pump back on with no success and she was looking very pale. Ok, I admitted failure. I returned to put her O2 on and take her home. Thankfully she was rested enough to be back on Thursday which is my most important day of the week at work. Friday was her therapies at home as usual and she did well. Saturday she woke up with a cough which progressed to a mild fever by the afternoon. Great! After only 3.5 days of daycare she was sick!
This is our first big cold since her mito diagnosis. While thought of metabolic acidosis and dysautonomia swirled in my head, we worked to keep her stable and fight off this cold. Thank heavens for the g-tube!! We were able to keep the fluids going that she really needed and when she wasn't tolerating the formula we were able to mix polycose to essentially create D10 and run that in her g-tube. Our room was quickly converted to a hospital room with her pulse ox on continuously, the oxygen bubbling away and her feeding hanging on an IV pole. We took turns staying up with her and checking her temperature as she has a bad history of going from 99.5 to 105.7 in less than 10 minutes. Tuesday she was tested for influenza which was negative. She continued on in the precarious fight against the common cold. Finally by Friday she was feeling better! Wow, if this is how every cold is going to go we are in trouble! We hope that this was a particularly virulent cold and that is why it hit her so hard. We also have a new appreciation for the g-tube, as she would have been hospitalized without it.
So hear we are. About to start week 3 of work. She only tolerated 3 day during week one, missed week 2 entirely, who knows what's in store for week 3?!? We've been able to adapt so far to our new normal. My son is learning and now knows what to do if we ask for a flush or to tell us when his sister says her head hurts. It breaks our heart that this is what they both have to deal with, Audrey with all her medical interventions and Jake needing to understand it all, but for some reason this is what we have. This month we have so many things to do. Happily we get to journey to Las Vegas for my little brother's wedding! We are all looking forward to this brief break for the routine. Audrey is going to have to see a few more specialists, a nephrologist for her kidneys because she has too much protein in her urine and frequently wakes up swollen. She will also see an ophthalmologist to screen her eyes. Kids with mito disease frequently have vision problems so we need to be sure this is not the case with her. We also get to follow up with her pulmonologist, orthopedic doctor and she gets to meet with the school district as she is 2.5 and will leave Regional center at 3. Wow! Such a busy month, I sure hope we can keep up with it all!!