5.29.18 ER visit

Aimee has been having some return of digestive issues with bloating, pain, and gas. She has been retching some days and had a couple instances of difficulties with her cecostomy flushes. Last week we took her in to the registered dieticien who wants to substantially increase her calories again and to redo bloodwork. We put off the first recommendation for now, due to the distress she is already experiencing. Increasing calories would mean increasing rate and causing more stress on her intestines. 

I’m glad we waited, cause Sunday it all came concerningly together. She was with her nurse all day and when we came home the nurse was worried. Her farrell bag contents (draining from her stomach) was bloody and she had vomited several times the same dark bloody color. There have been days with some blood flecks, but never near this amount. Her abdomen was bloated and very sloshy. I called the GI doctor on call and they asked that we bring her down to be seen in the Emergency Department. Because we had called ahead, the doctor had ordered several tests, had a nurse assigned, and registration was expecting us. It made the whole process very smooth. In only 4 hours we had an IV placed, panels of blood tests done, ultrasound, x-ray, and video x-ray performed. All appeared consistent with previous examinations, which was reassuring. They concluded that it must be a bleed somewhere in her GI system, but they didn’t know where it originated. They gave us specific instructions of what to keep our eye on and when to bring her back. They prescribed a higher level reflux med, hoping that will resolve the issue. We got to bring her home and now have to troubleshoot her daily routines from here. 

I’m disappointed as this medication is one I had weaned her off of several years ago due to its effect on nutrient absorbtion. She was maxed out on the lower level med though, so we have to at least trial it for a couple weeks to see if it helps. 

5.18.18 Good Reports

A few key updates to share for Aimee. 

First, her bloodwork finally is showing her carbon dioxide levels have decreased back to her own version of normal. This is really good news and a big relief. 

Second, concerns have been expressed during recent appointments about her left hip. Repeat x-rays show that her hip is still partially out, but has not progressed noticeably in the past 6 months thankfully.

Third, not so good news, her right elbow has lost 10 more degrees of extension in the past 6 months. Although her left elbow is worse, her right is the one she uses to touch, explore, ext. Her rehab doctor asked that we at least meet with the orthopedic surgeon to discuss a lengthening procedure. We will see them at the end of the month. I think it may be a difficult decision. We really don’t want her to lose any ability she has to engage. We also really don’t want to put her through any surgery, even minor, if there isn’t a clear benefit. Hopefully talking with the surgeon will make the choice obvious. 

Aimee continues about the same. She has some good moments where she is energetic, even hyper. Most often she is tired and staring, unengaged. We’re fully enjoying all the good moments. 

4.10.18 Last Month

It’s been a month since I posted an update to Aimee’s friends. I keep waiting for things to settle completely before I post, but it feels like every few days it is a different challenge or a new positive. Aimee has made the rounds through a lot of appointments this past month. We had med changes, new equipment, progress and decline. 

Here are the highlights. 

-In positive news, we were able to increase the speed of giving meds from 1ml a minute to 1ml every 45 seconds. Doesn’t sound like a huge change, but with 156 mls of meds each day it reduces us from 2 hours 36 minutes to 1 hour 57 minutes. 

-Her seizures seem to have responded to a medication increase, which seems to have allowed her to be more alert again. 

-About half the time, she seems to have the light in her eyes again. She still spends most of her time in bed now. When she is out of it very long, she gets uncomfortable and distressed. She has returned to school a few hours a week (as much as possible between appt days) and is responsive and engaged there again. 

(Playing at school)

(Classmate reading her a book)

(She loves having snuggle time with her brothers)

-She had her GJ tube replaced in the Interventional Radiology room and that was successful. 

-We were able to do a slight increase of calories and she is regaining the weight she lost over this past 6 months. 

-We also were able to get a decent enough mouth x-ray finally at the dentist. One of her baby teeth still hasn’t come all the way through and I was concerned that there was an issue there. She also has quite a bit of gum overgrowth probably due to medications. All is well for now according to the x-ray, so that is a relief. 

-In negative news, we met with her GI doctor and we are disappointed to see that that her function has declined on both ends. She is still requiring continuous draining of her stomach via farrell bag. She is being fed continuously through the j-tube into her intestines for fluids and nutrition. We had hoped that she would recover the use of her stomach, but since we have seen no progress, the hope of that is slim. We have had to continue her on a low dose of antibiotic 3 times a day to stimulate motility. We have also had to concentrate her cecostomy flush dramatically, as well as, using an adult dose enima. It is working again and she spends 75 minutes every day getting that done. It seems that everytime we increase in this area, she does well for about a month. If this trend continues, it is very likely that we will need to divert and surgically place an ostomy. 

-With her pulmonary appt we did labs to check carbon dioxide levels, which were higher in December than her average. The test results came back even higher still. 

-Aimee has had several respiratory illnesses, but having the nurse here has been a great help in keeping her at home. First, because it gives us a break, but also because the nurse is able to help us monitor risk. She is also really good at getting out mucous plugs! :) 

-We did not continue with the night nurse. It was not comfortable having a stranger take care of Aimee during the night or having her in our home while we slept. We decided to follow our gut on that and discontinued her hours. 

-Aimee’s back is looking good, though she does not seem as straight as she did post surgery. We are noticing her pelvis is tipping more and more, which seems to be causing her whole body to twist. At the moment this means we need to monitor her skin closely to ensure that the extra pressure does not cause sores to develop. 

3.10.18 Happy 9th

We celebrated Aimee’s 9th birthday last week with balloons, icing snowflake cookies, dancing, finger nail painting, presents, and watching a movie together. Aimee LOVED it. She laughed and danced the day away. 

2.23.18 Magec Rods

Aimee spent Wednesday in clinic for wheelchair adjustments and her first magec rod expansion. I wouldn’t have ventured out with her yet as she is still recovering from a cold, but her nurse was with us to help her stay safe. It was a bit chaotic with all the kids and all the suctioning and all the meds we had to pick up, but we did it! Because her appt had been messed up the week before, we weren’t able to have the surgeon do her intial expansion. The nurse practitioner was the same one who had brushed us off with Aimee’s wound stuff. We don’t have much confidence in her and, honestly, this is experience didn’t inspire more. We were told by the surgeon last week that the left side would be expanded 2mm. She ended up expanding it 8.1mm after 15 minutes of head scratching, contradiction, and discussion with the ultrasound tech. It was not inspiring. In the end though, Aimee is okay, if irritable, following the expansion. 

Monday we had our first night with an overnight nurse. It was exhausting as I needed to stay up to train her. Hopefully after a couple more shifts with her, it will mean more sleep instead of less. I never would have believed that I would agree to a night nurse in our home that was a stranger. It is not easy to accept, but we are ready for the help. 

Aimee has been enjoying listening to audiobooks lately. Yesterday she listened to part of the Jesus Storybook Bible and all of Mr Popper’s Penguins. It is pretty sweet to see her brothers (usually Caleb) sneak into her bed to listen along with her. If anyone is looking for a gift to give Aimee for her 9th birthday next week, she would definitely enjoy amazon audible gifts to build her collection of audiobooks. 

2.18.18 This Week

Unfortunately, Aimee is in the middle of another respiratory bug. We’re using all our tricks and so far staying ahead, but the timing isn’t great. Her nurse had vacation scheduled and we got hit with a snowstorm that has our power knocked out. Thankfully, we have been given a generator to run her equipment, but it was a pretty tough night last night as we didn’t have the generator prepared and the power went on and off. We did inhalers, cough assist, deep suctioning and managed to keep her at okay levels. 

Other bits of news from this past week:

Monday Aimee started school for the first time since June. She was so excited when she heard the school bus pull up and enthusiastically participated all day. We were pretty surprised, given that it was a new school and that she has been so lacking in energy lately. She made up for it with some tough days and then this sickness unfortunately. 

She also had 2 appointment days. We met with her neurologist, who decided to double one of Aimee’s seizure med doses to attempt to rid her of these more frequent clusters. We are slowly moving the dose up over this next 4 weeks. 

We also took her in for spine x-rays and her first rod expansion. Unfortunately, this appointment was scheduled incorrectly and after a long delay, we ended up only getting the x-rays done and having to return for the expansion next week. 

This coming week, we will be attempting the addition of a night nurse 2 evenings a week. It seems a bit unreal, but to have 2 nights a week that we don’t have to perform her 90 minute bedtime routine, don’t have to wake up to give two sets of during the night meds, don’t have to respond to o2 alarms or bipap leaks, and don’t have to do repositioning to get Aimee settled back down... it may end up being a big blessing and help us in our quest towards restored health. 

2.9.18 Until We Meet Again

Our hearts are broken for our dear friends right now. Their sweet girl, a special friend of Aimee’s, passed away earlier this week. We used to live close by and drove through a snow storm to meet them when Aimee was nearly 2 years old and Cami was only 4. We had laid the girls together on a cushion of blankets on the floor and took their sweet picture together.

 It is bitter to imagine the pain that they are experiencing, the devestation and torent of emotions. I could hardly bare to drive Aimee in for her appointment days yesterday and today, to take her to the hospital where sweet Cami spent her last days. Our hearts are tied to this family always and we are crying, grieving with them. 

I did manage to make it through the entrance for Aimee’s clinic appointments, though I wanted to turn around. She met yesterday with her Pulmonologist. Her nurse at home has been concerned about decreased lung sounds on her right side caused by some mucous plugs. We are increasing her cough assist frequency and using more inhaler treatments even when she is well. The doctor is wanting us to use her bipap with her high heart rates during the day or when she needs oxygen for periods longer than an hour, as he is concerned about avoiding pulmonary hypertension as much as we can. He had us do a blood draw for electolytes to check her carbon dioxide levels again. Her September count was high for her (her normal is already high). 

Today we met with the GI Nutritionist. Aimee is quite slender, though in the healthy range at 15th percentile BMI. We would like to get her more of a buffer, get ahead of any challenging sicknesses ahead. We are going to attempt a small increase in formula to add some extra weight. Hopefully she will tolerate the increase. We also had her zinc and vitamin c levels rechecked with a blood draw today. 

Aimee starts school again on Monday. For now her nurse is contracted to ride the bus and attend with her. We are praying that this becomes the permanent situation. It would definitely be the ideal.