On the right track

Finally I can rest. Sunday brought the scariest experience I have ever had with Audrey. Just as I was getting to go downstairs for the CureMito event, she woke up crying that her arm hurt. Her IV was out again. The doctors had decided that if she lost her IV they would place the PICC at the bedside with conscious sedation. Everyone was assembled and Audrey thought the hats and masks were kind of funny. The first versed dose makes a drowsy, goofy girl. They give fentanyl and she a little less drowsy and trying to sit up. Three doses of each are given and she is maxed for the amount she can receive on the floor. The doctor says sorry she will have to get her line in IR and everyone leaves. In the meantime Audrey is increasingly restless. She wants me to hold her but when I try to pick her up she starts choking me and pulling my hair. I try to put her down and she grabs on so tight I have bruises. She is shrieking and kicking, something is wrong! The nurse calls the doctor who says she was just disoriented, no way, this is a drug reaction! I hold her in my arms while she screams "Help me!" at the top of her lungs, kicking and thrashing around. We are both crying. I have never been so scared for my girl! Finally after an hour she falls asleep. She ended up sleeping for 12 hours! While that is normal at home, it's impossible in the hospital since they bother you so much. I thought about in January when she had fentanyl in the recovery room after her g-tube was placed. She acted in a similar manner (though not as severe). At the time we thought it was pain but maybe it was the medication! I call a friend who's husband is an anesthesiologist. He says some kids have disinhibition with fentanyl. I quickly google "disinhibiton and fentanyl" extreme irritability, agitation, outbursts, can have hallucinations. That was definitely her! She is scheduled for the morning for her PICC placement and G-J tube and I'm really scared we will go through this again.

Monday morning comes. I am sure to have my husband come because I am not strong enough to go through that again. A mom can only take so much helplessness and I knew I would have to leave the room if that scene played out again. We went down stairs and I made sure she was documented to have a reaction to fentanyl. I spoke with the radiology nurse practitioner, the physician placing the line, his nurse and finally the anesthesiologist. Making sure all of them understood what happened and that she was not to get fentanyl again. Off they took her. I was able to go into to floroscopy with her for the induction. They decided to give her only gas and they would try morphine if she need any pain control. She was such a good girl and so brave with the mask. Very quickly she fell asleep and I left. It's really hard to see your baby go limp and unconscious, even knowing that it is intentional. Though she was scheduled for an hour ant even 40 minutes goes by and our pager goes off. Shes done! We sit with her in recovery and after some initial confusion (they put her pulse ox on her thumb she sucks on!!) she lays quietly in my arms. Though she continues to sleep, at one point she asks for her blanket and then her hair before falling back asleep. This is the best recovery ever!! Her dad and I breath a sigh of relief. I think we have found the reason she has such a hard time with anesthesia and hopefully it is a simple solution.

So now her procedures are done. There is nothing else we need to do to her. She has her new G-J tube and she has a PICC so we don't have to keep poking her for IV's. Now it's time to start feeding her to get her to gain weight! She is on full TPN now that she has her line. Fingers are crossed that she is able to transition to feeds soon and start gaining weight.We are hoping we won't be here too much longer! Here are some pictures of her stay thus far...

this was at admission, not a happy camper!

so skinny!

this is how she spends her days

back from her procedure, doing well

"Cat" her Tubie Friend!

Finally feeling like playing! That is her PICC in her left upper arm and her new G-J is in the same spot as her old g-tube.

So that's where we are. Hoping she is able to get home soon!

Now it's the weekend

Audrey is still in the hospital with no upcoming plans about going home. Yesterday she went down for an upper GI. Many people have these tests, I've had one myself and it is fairly non-invasive. However, my 2 year old is terrified of x-ray. I know this so I asked ahead of time for Child Life to go with us, hoping for better tolerance. She showed up with a basket of distraction tools which made her gurney ride through the hospital lots of fun. However as soon as we got to x-ray the crying and clinging started. The radiologist was very nice and understanding but Audrey doesn't seem to understand "lay still" "keep you legs straight" "no kicking" especially once she gets so worked up. So that took about an hour and then we had a few hours of x-rays in her room which barely went any better.

So, after much miscommunication the fellow finally came in to say she does have delayed gastric emptying, her stomach is shaped funny which may be part of the problem, and she has mild SMA syndrome. Huh? I don't know that one. SMA syndrome or superior mesenteric artery syndrome was described to me as something that happens when you quickly lose weight. the fat pad between the aorta and superior mesenteric artery, which gives blood to your entire gut, is decreased causing compression on the duodenum lead to a bowel obstruction. So the plan was to consult surgery and notify GI to find their recommendations but ultimately she really needs to gain weight. So of course, once the doctor left I hoped onto google. I know it's bad and I always advise my patients to be cautious of what they read but I can't seem to help myself. The first article I read said "this is a very rare but serious and life threatening event with a mortality rate of 1 in 3." What?!?! This seriousness was definitely not relayed to me by the doctor. So I continued my google search and found lots of interesting information, not all of it reassuring, but learned there are varying degrees of seriousness and hers is still mild. That being said it is so important to get her to gain weight to prevent progression.

So here we are on Saturday, and as some people know, nothing happens on the weekend in the hospital. I know my girl has this rare but potentially serious condition on top of her already-difficult-to-treat mitochondrial disease. We can't feed her through the g-tube and they weren't able to do the PICC line so she has an IV in her hand which does not last long. She had 4 IV sticks already and they will continue to add up this weekend. I know eventually they will go to her right hand. They have avoided it so far because she sucks her thumb, but it's bound to happen and she will not be a happy camper. I am obviously still very worried about though maybe I am relieved to find an answer?? At least this new problem has a potential solution where as everything else she has is just hoping and treatment of symptoms. So here's so to at least getting a plan for Monday since nothing will happen on the weekend!

Admission #3

For the third time in 9 months Audrey has been admitted into the hospital. I hope it is just a strange coincidence that she is here every three months and not a sad prediction of our future. This time we are here because she continues to not tolerate her feedings. It has been over 2 weeks since she was on formula. She is losing more weight each day and today she is so weak she cannot even sit up. I am glad that we came in yesterday, I would be much more panicked today at home if I had found her so weak.

I find myself struggling with the inpatient speed. We have been here for almost 24 hours and we've spoken to many different doctors who are going to contact the other doctors we've spoken to to determine a plan. In the meantime the only thing that has happened is she has an IV with fluids running. I know things run differently here and I know nobody has the sense of urgency that I have. It is my little girl laying here too weak to play. I will remind myself to have a little patience, but not too much as someone needs to advocate for her!

Unfairness

I know one of these times I will be able to post how well everything is going. It is probably just that when Audrey is feeling well, I am busy enjoying what she is able to do. When she is not well I need to turn to my blog to spew everything that she is going through.

Audrey continues to get sick every other week or so. While we are waiting to see an immune specialist, we are doing our best to keep her healthy. Since her last illness she has not yet made it back to her regular feeds. As a mom, there is nothing so distressing as not being able to feed your child. If I can't do anything else for her I want to at least be able to feed her. But apparently this is a big, long-standing problem for mito kids. Whenever their little bodies are stressed their GI system just stops. I know it's hard to imagine. She has a feeding tube to her stomach, how hard can it be to feed her? Well I can put the formula in but it will just sit in her stomach until she throws up or I vent her and let it all come back out. It's been almost 2 weeks since she was on full formula feeds. This mommy is getting worried! I'm watching her get more tired and lose more weight, knowing we aren't giving her what she needs to get healthy and beat this vicious cycle she seems to be caught in.

For one afternoon Audrey had fun. It was hot on Saturday and though she wasn't tolerating her feeding she woke up from her nap feeling OK. I let her outside with her brother to play in the sprinklers. Normal, 2 year old fun! She played for an hour and though I knew she was quickly burning through her energy stores, I didn't have the heart to stop her. Finally after an hour I made her come in. She quickly collapsed on the couch and there she stayed until bath time. She was wiped out Sunday and Monday also. How sad is a disease where one hour of play causes 2 days of exhaustion?!? It is so unfair that these children endure so much medical stuff all for one hour of play here and there.

In our quest to keep her healthy, we are looking into alternative care for her. She can't go to daycare any longer. Not only does she become ill if someone even looks at her, she just doesn't have the energy for it. When I picked her up today she was a mess. All of her feeding had backed into her bag, she was crying begging to go home and so exhausted she slumped forward in her carseat to suck her thumb since she couldn't lift it up to her mouth.. I can't watch her go through this. I know I've written about our struggles for services for her but it is so unfair! Every program or waiver I look into I get the same answer..."If she has medi-cal she will qualify." Her pediatrician wrote for home nursing care. The insurance company authorized 400hours per calendar year of nursing care. That works out to one 8 hour shift per week. The nursing company said not to worry, we can just put her on medi-cal and they will cover the rest. The problem is she doesn't qualify for medi-cal! We are doing everything in our power to take care of her and remain working, contributing members to society but the system is working against us. Instead of funding programs that help working families keep working, I'm encouraged to quit my job so my child can get what she needs. Unfair!

And that's where I am right now. It's unfair! It's unfair that my daughter can't eat any food. It's unfair that I am unable to provide her with the services she needs. It's unfair that she has to work so hard for just a little fun. It's unfair that she has to be affected by mitochondrial disease at all! No child should have to go through what she, and thousands of other children like her, go through every day. I know that there are many other children who are sicker than her, who are fighting for their lives or have already lost the battle. But right now, for my daughter, all I can say is it's not fair.

A sick little girl

We are in the midst of her 4th big illness in the past 8 weeks. Each one lasts a little longer and she gets a little sicker with fewer "well" days between bugs. Today we spent 4 hours at the doctors office. We went to be sure she doesn't have pneumonia. It's day 7 of being ill and she's not looking better. Last night her ear started draining so I knew we needed more ear drops for that. In the morining she started vomiting so I hooked up her venting bag. Its much better if that can keep it all contained! By the time we got to the doctor her venting bag had 200 mls of pedialyte and bile! Her rate is only 35ml/hr so this was the whole days worth! Come to think of it, I hadn't changed a diaper all day. Well a couple X-rays, some labs and they think she may have a little bit of an ileus brought on by her nasty cold and ear infections. So after 4 hours of holding her and carting her around my arms are cramping and she is tired and crabby. Now we are home with our fingers crossed that we can stay here! I cringe when the doctor says "oh you're an NP, I'm comfortable sending her home then." I wish doctors would remember that when I'm sitting in the room with my screaming 2 year old I'm a mom way before a medical professional. So now on top of all of her other medications she has eye drops for pink eye, ear drops for an ear infection, antibiotics because the ear infection is really bad, albuterol nebulizer and to top it all off she got mag citrate and an enema to hopefully get her tummy working. I'm crying right along with her :( I really hope and pray she starts feeling better soon!

House of cards

Life with Audrey continues to be a struggle. In the six weeks since we have returned from her surgery she has only been able to go to daycare four days in a row twice. After each of those weeks she ended up missing the next week due to sickness. In fact Audrey has been sick every week since the week of Valentines. What are we going to do?? For the past month we have been working with her pediatrician to brainstorm different ideas to provide care for her. Since I am the major income earner and I carry the benefits, not working is not an option for me. We seem to fall in this middle America gap where we make too much income to qualify for any assistance but we are unable to afford it ourselves. I can only imagine how many other families find themselves in this same predicament. There are so many programs for low income families it is very discouraging that we can't qualify for anything! Don't get me wrong, those families absolutely need the help but so do we! Audrey also falls into this gap with her disability. She is mildly delayed and has some medical needs. She is 2.5 so we are in transition to the school district. They are afraid she may not be delayed enough to get accepted but are concerned with all her medical issues, without any therapies she will quickly fall behind. Regional Center is in the same boat. She does not have epilepsy or MR so while we are going through their evaluations, we don't think she will qualify for that either. It certainly does not look promising.

So now I'm evaluating my job. Though I have to keep working, and I'm terrified of switching employers and risking a change in insurance, she cannot continue to go to daycare. This week she came down with hand-foot-mouth disease. It's such a common daycare illness, that really every child has at sometime or another. With Audrey though she had fevers over 103, required 1L of oxygen continuously and 6 days later is still not better. She's losing weight and is weak and wobbly. When her body is taxed her speech and thought processes really suffer which leads to frustration on all of our parts as we don't understand what she's telling us. The last illness was just a simple URI with the same dramatic response. We have to find a solution that will work for the whole family and be able to keep her healthier. For the time being my husband and I are switching off days staying home with her. He doesn't have sick time at work so everyday off for him is without pay. He is also less comfortable with the medical aspect of her care, though he is becoming more proficient each day. While I have plenty of sick time and am able to do her medical care, I have a job where I have patients with appointments to see me. If I don't go to work, my work continues on and I have to catch up. If I am not there to see my patients I have to get someone else to see them so it's not an easy thing to just call out sick. Add into this whole mix the emotional strain of watching your toddler go through all of this and it's no wonder we are all walking on eggshells waiting for the shoe to drop. I know sometime soon something has got to give as this situation we are in right now will not last long.

What a long month!

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!!