11.3.18 Test Results

Following Aimee’s Pulmonary Sleep Clinic visit earlier this week, we had labs done to re-check Carbon Dioxide levels and iron levels. Iron supplements have kicked in and her stored iron is now back to a healthy level. Very unfortunately her carbon dioxide came back significantly higher... the highest it has ever been despite the increase to the backup rate on her bipap machine in September. This is really concerning to me and may be the cause of her general low energy levels. I have not yet been able to discuss the results with her Pulmonologist. 

We did get EEG results and there were no seizures during the 2 hour test. Thankful to rule that out as the cause of her fatigue. 

10.17.18 One Year Later

Today marks one year since Aimee’s first spine surgery. A year of recovery and change for all of us. Honestly, I thought by now we would be confident that agreeing to the surgery was the right choice. There have been positives certainly and she has healed fully. We are actually going in on Monday for her 3rd rod expansion. I am hopeful that year 2 will bring more enjoyment and life back to Aimee. 

So, this past few weeks. We have had issues with Aimee’s wheelchair van, which have now thankfully been fixed. The pivotol element, the ramp, was not functioning. Thank you so much to a couple dear people who sent Aimee funds this past month that covered a good portion of this cost. It is now working more smoothly than it ever has for us! 

Aimee had a dentist appt a couple weeks ago at UW. Thankfully there is a new dentist there that seems a lot less concerned with surgically removing teeth. Due to medication, Aimee’s gums have grown over baby teeth. There is still the possibility that she will have to have them messed with in the future, but thankful to put that aside in my mind. 

We met with Otolaryngology a couple weeks ago to get medical clearance for Aimee’s new hearing aids. A funny insurance hoop that must be jumped. It did give us our first chance to utilize the new North Clinic of Seattle Children’s that was built in Everett. It is incredibly close, less than a 30 minute drive. And there ends the positives. I have been fairly disappointed so far to discover that it was not built with disabled children in mind at all. It seems to have been designed for looks over function, as it is very challenging to get the wheelchair into the rooms, through the front door, and there is no changing space anywhere for larger kids/young adults. A big disappointment. 

To swing back to positive, last week we had a 2 hour communication evaluation done by the speech language pathologist at children’s. It was amazing! She is going to help us get Aimee beyond just pushing communication buttons for cause and effect games. She even had us play some interactive games with Aimee in the clinic, similar to Simon Says. It was wonderful, beautiful, and gave me hope. From here we are going to attempt to have DDA funds cover a couple communcation devices. 

We are also hoping to get Aimee an iPad again. The SLP recommended that we get one to use with the communication devices. Plus, Aimee did so well with it in the past. When hers died, we got a kindle for her instead since it was so much cheaper, but it doesn’t have hardly any apps available for her and is much less sensitive to her touch. It also is a dimmer screen and hard for her to see. I am looking around at maybe a refurbished iPad with a larger screen for Christmas. 

Earlier this week, Aimee had a prolonged EEG done. We are just double checking to ensure there are no underlying seizures causing Aimee’s major lack of energy lately. It will be about 5 weeks until we have full results.

In general news, Aimee has been sick this past week or so. She is holding steady, though having some pretty intense choking episodes. She has also begun desatting during the nights again this past month, which had been fairly resolved for the past 6 months previous. 

More updates to come as we navigate our way forward with/without school and communication. Praying that we can see our next steps clearly and can help Aimee wake up to the world around her again. 

9.25.18 The Way Forward

Dear friends, I apologize for my lack of communication over this past few months. It has been difficult to process what the way forward is for Aimee now. She has had a busy summer of clinic visits and exams. 

She had a sedated hearing exam, which has resulted in a set of new hearing aids being ordered. I am excited about these, as they will be blue tooth enabled. 

She has had numerous blood tests, some to show that her carbon dioxide levels have increased much too high. We are attempting some changes in the amount of breaths that her bipap makes her take per minute during sleep, an increase from 18 to 22 breathes. The fact is that all day long she breathes too shallow and does not expel enough carbon dioxide, but we hope to have enough of an improvement during sleep to improve her blood levels to a safe amount. If this is successful, we hope that we can leave the pressure settings alone. 

She has continued to have low iron levels. She has been receiving supplementation for a couple months in the hopes that her energy levels would increase. No improvement noted. 

She has started a medication to allow her stomach to relax and expand with the goal of being able to clamp the farrell bag (stomach drain). So far she is tolerating it being clamped some days for an hour, which is progress. 

She has had multiple rod expansions for her spine, which have gone very well. 

She has started school again, but we are strongly considering pulling her out. She just does not have the energy to stay awake or alert in class. She no longer seems to enjoy being there, so it is not worth the risk of illness and the great amount of paperwork, meetings, ect that it takes to keep her in school. At this point, I’m not sure if I will just include her in our own homeschool activities or if I will pursue home based public school. 

We met yesterday with her neurologist. There is concern that her lack of alertness is due to underlying seizure activity. We had attempted a dose increase earlier in the summer, but it did not affect energy, though we noticed less events. We agreed to have a short EEG performed in a couple weeks to see if we are missing seizures.

Overall, Aimee does have alert, engaged times, but she has them much less often. Generally she is either sleeping or disinterestedly staring. I have so many questions and am so concerned about her lack of participation in life now. Please pray that Ed and I would be given wisdom. I feel that Aimee is hiding away, but wether it is due to pain, seizures, energy levels, fear, coping... Jesus, help us to love Aimee well. 

-We got to see our favorite NP while inpatient earlier this month-

-Aimee’s first day of 4th grade-

9.5.18 Leaving

Our favorite place to leave 

9.4.18 Hospital

Currently inpatient with Aimee. We pulled an all-nighter in the ER and were admitted around 5am. 😴 Aimee is well, but her medications that we gave through her j-tube (intestines), drained right back out of her g-tube (stomach) into her farrell bag last night. Not supposed to happen and not an easy fix. We called the GI doctor on-call and were sent to the hospital. Hoping that we will be able to get her tube replaced in the Interventional Radiology today. Finally got her an IV, some meds, and some fluids around 4am. Finally got her respiratory equipment set-up for her to sleep around 6am. They are watching to make sure she has no problems (seizures, ect) come up from the lack of meds and fluids. 😴 

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. 

2.1.18 New Life

I have so many words of gratitude to express to so many people. We have been carried with many gifts and have not had to worry about any bills being paid. It is incredible that after this long season, we are not holding unpaid bills in our hands. Thank you for walking through this with us and caring for us in such practical ways. 

Aimee is doing well. She is just recovering from a respiratory bug that she has been fighting for a couple weeks. It was a much easier sickness to handle due to the breaks in care that the nurse provided. Also, we did not have to struggle with dehydration and malnourishment due to the j-tube continuously feeding her intestinally. 

Her wound officially closed and we got the all clear from the wound care dept. There is only a simple dressing left and it shouldn’t be needed too much longer. 

She is having more seizure activity again and still only tolerating short periods of activity. She is spending most of her time asleep in bed and needing more middle of the night care. She is just not her happy, comfortable self still. 

I have been dealing with a lot of ramifications of new supplies, new home care issues, new prescriptions, ect. Insurance makes every step complicated. We are also in the middle of getting nurse care set up through the school. This has required a lot of help from the Medically Complex Care doctors at Children’s. We are hopeful to finish up that process in the next week and attempt starting Aimee back into school. She hasn’t been since June of last year. 

For me, the nursing hours has been life changing. There has been so much freedom that I haven’t experienced in years. It is amazing! I have been able to take the boys to the library every week, church events, and errands. I can meet up with another mom or go to free community events. The world has opened up. What I am really most excited for is the ability to focus on the boys and their learning. 

Aimee has multiple appt days during each of the coming weeks. Finally going to get all her providers caught up on her new status post surgeries. Hopefully as the weeks progress, we will see more moments of Aimee returning to her typical self than we have so far. 

1.20.17 Learning

Today is a good day, so today I will write to you. We brought Aimee out to the couch and watched a movie all together as a family. First time we have been able to hold and snuggle Aimee in 3 months. It felt perfect, yet very different with how stiff and tall she is post spine surgeries. 

There have definitely been some tough days. Weird symptoms that don’t add up together and that come and go. Like some blood in her farrell bag (drainage from her stomach). Random fevers. Sometimes needing cathing all day, sometimes not making urine, and sometimes doing it all just fine herself. Crying days where pain meds don’t seem to help. J-tube clogs that have caused us to lose precious hours with fluids. (Thankfully we have been able go get the clogs out ourselves so far. If we ever can’t, she has to go into Interventional Radiology at the hospital for a new tube.) Days with lots of suctioning and some congestion, then days with a dried out tongue. Days with multiple bags full of air drained from her stomach. Days with lots of discharge. We’re doing our best to learn and follow her cues. 

In really good news, the wound continues to heal every day. We are changing the dressing and excitedly seeing positive progress each time. 

We are working hard to get documentation together in the hopes of sending Aimee’s nurse to school with her. It sounds like it may be a battle, but at this point, we don’t feel that it is the right course to send Aimee with an aid/para only. Thankfully the hospital Nurse Care Coordinator and Medically Complex Care doctor are up for writing letters to the district detailing Aimee’s needs. 

Speaking of the nurse, it has been such a blessing on the days she is here. We have been able to go grocery shopping with the boys, take them to the library, catch them up on dentist and doctor’s appts, and attend church! Aside from having days where I can be cleaning or doing activities where Aimee is out of sight range. It is such a strange and beautiful freedom, for which I am beyond grateful.  

1.11.18 Done!

After over 6 weeks, we are officially done with the wound VAC therapy as of today! The wound has filled in nearly all the way and will now just have a dressing change done daily at home by us. And she can finally have a shower again!! Yippee!! :) We will have to continue to watch out for extra granulation tissue and use silver nitrate to burn that back so that the skin can grow. We are hopeful and excited to be moving to this last step towards healing. She will not have to go back into the orthopedics clinic until 2 weeks from now to check over the wound. Then in February will be the first extension of the growth rods. 

-Brenda, ortho nurse practitioner, has been a huge blessing doing weekly wound VAC changes at our home and weekly changes in clinic. Elliot and Walter were there today too, but not pictured ;)- 

In other news, we spent a couple hours in clinic on Tuesday with a PT and the wheelchair company working on her chair. The bigger she grows, the more she twists, the more challenging it is to get the chair to fit her correctly. We’ve been having more pressure spot issues, which could turn into pressure sores when she gets back to typical days spent in her chair. With a new back, seat, and headrest, we are hopeful to see a reduction of these spots and better support. 

Overall, Aimee is making progress. We’ve been having a lot of communication with GI from home to make different tweaks to her routines and meds. It is all so complicated now, but worth it, as she is less distressed and starting to have periods of alertness. We’re kind of figuring out her new routine while she is at home in bed. As she gets more comfortable, we will start to have her spend more time in her chair and start moving towards returning to school. An intimidating, uncomfortable step to consider, but hopefully Aimee will be able to participate in things she enjoys again soon. 

1.4.18 Appt Day

Walter, Aimee, and I went in for some appointments today. We started in Bellevue Sleep Clinic. Aimee has been having big desats 5-6 times each night. She recently had a change in pressure of her bipap and we are hoping another tweek will resolve the issue. Some kids with low tone are not able to tolerate higher pressures apparently. Seems that Aimee is one who has trouble with it causing her oxygen to drop into the 50-60% range. We also adjusted her doses of melatonin and clonidine that she takes nightly. Her blood pressure has been really concerningly low during sleep and this is a side effect of clonidine. Hoping that lowering her dose will not majorly effect her sleep. 

Next we headed over to Seattle for a wound VAC dressing change. Aimee was really worked up and in pain before the appointment. She had a wheelchair remodel done last week and it seems like something about it is not fitting well. I think the headrest is also pinching her ear to her hearing aid. She calmed down when I removed the aid and gave her some valium. 

The dressing change did not go very smoothly, probably cause we didn’t have Ed’s help this time. We had trouble with leak alarms from the machine that took several attempts to fix. Aimee did not tolerate moving in and out of her chair and kept choking/gagging. However, the wound is continuing to make progress. Some more silver nitrate was needed to burn off excess growth, but otherwise it looked healthy. Looks like there is a good possibility that we may be done with the wound VAC after 1 more week. 

We got out to the van to find that someone had parked on the dashed lines. I had to get her wheelchair up onto the ramp without having room to go directly. It is getting harder to this as her chair just keeps getting heavier. 

A tough day, but we made it home in one tired piece.

1.2.18 Nursing

It has been hard to know what to update, since things have been pretty steadily confusing. Aimee is still nauseous and retching in the evenings. She is still most of the time uncomfortable, sometimes with a low fever, high heart rate, and cramping (we think). She is desatting through the night again. She is rarely voiding urine on her own and isn’t producing near as much normal. We have managed to add 6 more mls of water an hour to her intake. 

It all feels very unfamiliar. We feel unsettled and uncertain. Symptoms we would normally assume are sickness or an infection, are maybe related to her digestive system not functioning properly? A new normal, new processes, more care are all fine as long as Aimee is able to be herself, to enjoy her days, to interact and play. At this point, she doesn’t have very much energy and is rarely awake. 

 

She has had multiple dressing changes for the wound VAC since we’ve been home and the wound it continuing to fill in. It may move to a wet/dry dressing in a week or so. We are pretty nervous about that transition as it means multiple dressing changes a day (more skin irritation/breakdown) and a higher chance for infection. But, at some point, it will no longer be an option and we will have to move forward from the sealed wound VAC. For now, we are so grateful for it and the huge difference it has made for Aimee’s wound.

In positive news this week, Aimee had an RN start providing care at home. She was approved for 56 hours a week of nursing care through insurance. We chose to use four 10 hour days for now with one nurse and see how it works for our family. It is definitely different, as there has to be doctor’s orders for everything that the nurse does. It isn’t impossible to get orders, but it takes a couple days. It is also a new person in our home, someone that we are just meeting and trying to get to know. We are hopeful that it will be freeing and will help us provide Aimee with the best, most safe care.