4.23.21 Hello Again

Hello dear friends and family. It has been quite some time since I have updated Aimee’s blog. Many are the reasons, but as it seems there is a new busy season ahead, it is again the simplest way to make sure that I am giving updates to everyone who loves our girl. 

We found out last fall that Aimee’s growth rods are maxed out/no longer functioning. With the help of her surgeon and her pulmonologists, we have decided that the next best step is a full spinal fusion rather than replacing the growth rods. She is now 12 years old and has only a small amount of spine growth remaining. There are concerns about Aimee’s respiratory future as her chest wall is becoming more flat and her lungs more compressed. We want to proceed with this final spine surgery while she is in the best health possible to maintain as much function as we can. 

This surgery is really big. New rods will placed and secured all along her spine with bone grafts throughout. It has not been an easy choice. We have met with palliative care and processed our goals and fears as best we can. Neither Ed or I want to do this... and yet we both feel that it is the best choice for Aimee’s continued health. 

 

Unfortunately, as we have now scheduled this major surgery and planned the year around this hospital stay and recovery, we have hit a couple of snags. One is that at this same moment, her Gastroenterologist has decided it is time to move to a colostomy. We were hesitant before, but have become increasingly overwhelmed with how difficult this area is. For Aimee’s quality of life and for our own, it must be done. But what to do? We have this major spine surgery looming. Could we possibly squeeze it in ahead of time and majorly reduce our risk of infection after the spine surgery?? Is it safe to do so? We don’t know yet. We will be meeting with the other surgeon soon to decide. If it is not safe or possible, we will still do it, just later in the year. 

Oh and another snag. I met with Aimee’s Neurologist yesterday. She is concerned about recent bloodwork showing a negative trend. White blood cell counts and absolute neutrophil counts are really low. She will not give her okay to anesthesia until we meet with hematology to find the cause. We certainly can’t open up a wound all the way down Aimee’s spine when she is at major risk for a bacterial infection. 

This is where we are at the moment. A bit uncertain. We are currently planning for a full spinal fusion on June 15th, but there are more doctors to be seen and decisions to be made first.

Overall Aimee is well. She spends more of her time asleep on her bipap machine than ever. She is only awake and attentive for about an hour in the morning and about 6 hours in the afternoon/evening. On a good day. 

Yet, when she is awake, she is home participating in school with us, snuggling with her siblings, taking walks outside, and enjoying family movie night on the couch. We sure love Aimee and pray continually for her healing. 

2.12.20 New Wheels

I have great news! Aimee got a new van and we can all fit together again. We even have 2 extra seats! We’ve been able to go to church, on a field trip to the museum of flight, and several appointments. It is really amazing. A bit of a learning curve for me, but I’m getting there.

10.11.19 Baby Louisa

Dearest friends and relations,

Here we sit 6 weeks after the birth of another little DeVoe bud on our family tree. Louisa Annette joined us on August 30th and we have delightedly gotten to know more of her developing personality every day.

 

Aimee’s life has been added to with a new little sister of course. Another fun addition for her this summer was the move into our new house. Ed worked so so hard to make it livable in time for baby’s arrival. I was grateful to deliver here in the home he built. We moved in 12 days before she arrived. Right on time. What little princess has ever been this spoiled by her daddy? He built a whole new house for her arrival. ;) 

Aimee loves her new room. It is so bright and has beautiful windows that look out on the pasture. I am hoping to plant some beautiful shade loving plants around her window that she can see blowing in the breeze or catch the scent of through the open window. 

We love having a simple process for bathing her with a stunning roll-in shower. It is life alteringly good. 

Aimee has continued through her many specialists this summer. The primary changes for her have been related to our decision to once and for all declare her as homeschooled. She is attending our homeschool co-op with the boys now and we are pursuing ongoing therapy options through childrens, which seem like great fits so far. The most difficult to resolve is speech services, but we are hopeful to begin that soon. Aimee has been daily working with her new iPad and communication buttons. I need to tell you more details about our hopes there soon. 

I do have one big prayer request right now. We have added another baby and another car seat, so our family no longer all fits in the one vehicle. We have been attempting to get Aimee a car seat so that we could at least get her out in the winter or when Ed and I take them together. The big problem is her regular, ongoing appointments. She generally has one or two appointments a week. All of our kids cannot fit in one vehicle and none are old enough to remain home. Even if we can get her a car seat, it would be an impossible task to get her in and out of it with just the nurse to help me. Let alone getting the wheelchair loaded into the back of the suburban. 

Okay, all of that info to say, would you please pray with me for a solution to this problem. Family members have been helping us this past 6 weeks so that we have made it to all appointments, but this is not a permanent solution. We are praying for a new vehicle that can accommodate 4 car seats, 2 adults, and 1 wheelchair. We are even considering a sprinter or small bus at this point. 

I’ve said that we would not decide about the number of children to have based off of vehicles. I am so glad that we did not decide to limit our family and miss out on this amazing girl who is already adding more love. However, we do still need wisdom to find a solution to this practical life issue. 

6.20.19 Sober Appointments

Facing the facts, the official terminology, the medication decisions, the prognoses this past 2 weeks. I don’t like to focus on these areas on a regular basis, but occasionally a specialist really wants to be sure we understand the gravity, the “thin ice” that we casually walk. 

This conversation happened during our big day in spine clinic last week with spinal x-rays, a spine rod expansion in ultrasound, and then a meeting with both the spine surgeon and the head of pulmology. There was a lot to this appointment, but the Pulmonologist sat with me and really wanted to be sure that I understand that Aimee has Chronic Respiratory Failure due her inability to exhale carbon dioxide. He said that her left lung is not functioning nearly as much as her right lung. There are a few implications particularly to be aware of during illness. Basically he said to pray that if she gets a respiratory infection next winter that it hits her bad lung, not her good one. He said it will take her longer to recover with an infected bad lung, but she will be much more sick if her good lung becomes infected. They consider her stable still, but are really watching the levels in her blood as an indicator of how well the respiratory supports we have in place are doing for her. They are not happy with her current numbers and want to see improvement in the coming months, before next winter especially. 

He was also concerned with how her weight growth curve has changed over the past 2 years. She has gone from steady gain, to maintaining, to losing ground. We are trying to address that with increased food, but it is a slow process with the j-tube feeds. 

In the midst of these things, the process of adolescence is upon us causing some odd behavior, new seizures, and concerns from many practitioners about how puberty is going to shift the situation. Every doctor says that this is a critical time for their area. We don’t really know how hormones and cycles will change life for her, but, like it or not, it is upon us. 

Overall, Aimee is generally happily spending time with her brothers, time in the sunshine, and, of course, lots of time snuggled up in her bed for whole days sleeping. It is hard to believe the dark picture that lurks in the background. Whatever the future holds, however far away that may be, we are enjoying the sunshine, the view, the joy as we walk across what we are cautioned is a thin ice plateau.  

We saw Aimee’s neurologist last week and discussed some episodes we had just started seeing in the past 6 months or so. At first I didn’t think that they were seizures due to the extreme vocalizations that happen during, but the neurologist thought that they were definitely seizures. She recommended doubling the dose of one of Aimee’s seizure meds and we compromised at a 50% increase with the agreement that if we continue to see them that we will increase again. Given Aimee’s struggles with energy, I am always hesitant to up meds that may make her any extra drowsy. It will take about 1 month for us to tritate up her dose to the increased level and we are hoping for no decrease in alertness. 

We took her back down for a routine GJ tube change in Interventional Radiology. She did great with the change and I was grateful to have a nurse to help as I could not be in the room with her. It is pretty complicated to coordinate between having a young child along who cannot go in the room, being pregnant so not being able to stay there, and needing to gown up in the surgery suit in order to take Aimee in and to move her onto the table. Moments I am so grateful to have a nurse! 

This week we also met with the sleep pulmonologist. There was some confusion as one of the settings that they planned to change was not a setting available on our machine. We are also concerned about how high they have turned up her back-up rate. They decided to bump her up to 24 breathes per minute. We’ll see what bloodwork shows, but it seems like she won’t have enough time to exhale between breaths. She has also had some of those big desats she had before with a pressure change, though not nearly as many as before. It seems like they’re not entirely sure what the best course is going forward, so we wait for the next blood results in about a month. 

We are interviewing a second nurse this afternoon and praying that she is a good, right fit for our family. It is so hard to have a stranger come in to take care of your vulnerable child in the midst of family life.  Yet, we need to have that care established before baby arrives at the end of August. With how long it has taken for them to hire a new option, there is some pressure to accept this new person. 

In family life, our cow delivered another heifer this week. There is new life and new experiences all around us. I especially love seeing Walter sit outside in the garden quietly watching new things, listening to birds trill at him, excitedly name the animals he sees, or sit in front of my berry bucket happily eating as I go. 😂 

5.31.19 Sharing Smiles

Aimee and Elliot sharing a hug and a smile. 

We made it through sickness and no nurses. We finally had a new nurse start last week. She seems to be a good fit and is very attentive to Aimee. Ironically, we were approved to have our nursing hours increased from 56 to 70 per week right during this time. This nurse is able to cover 3 straight days, so we do have help, but it is a long stretch without. Especially difficult to make it through appointment days without help. To be honest, I feel like a wrung out rag after yesterday’s run on our own. The kids were such good listeners though that I took them out for doughnuts as a reward afterwards. 

We are praying for another nurse to cover the remaining hours. Praying that they would be available in time for us to get a good schedule and routine before baby girl arrives. So far the nursing company does not seem very motivated to fill our hours. :( 

Anyways, Aimee had another sleep study done. Ed ended up taking a day off of work so that he could stay down at the hospital with her. It was a tough one, not because of the study, but because she had multiple post-cecostomy leaks. What a mess and in a place that has no accommodations. We were praying that everything stayed the same. We did not want any pressure changes and I was really worried about losing our oxygen if she happened to not need it that one night. We would be in the hospital so much without it. Results were supposed to take a good month, but I’ll come back to those. 

We met last week with the occupational therapist to work towards getting Aimee a car seat. $2300?! Yep. 😱 We have had some car trouble with the van come up and have had to cancel appointments. The van also does not run well in extreme weather and it makes me really nervous to not be able to get Aimee out of our place. We want the car seat as a back up option. As an added bonus, once the baby arrives, our whole family would be able to fit in the suburban we picked up. No idea how we will get Aimee in and out of it (especially how to get her wheelchair in and out), but just something else to figure out along the way! 

We also met with Aimee’s GI nutritionist last week. Aimee has been dropping weight this past 6 months. She is down to a BMI of about 12, less than the 1 percentile for her age. She is right about 5 feet tall (approximately), but only 59 lbs. Yes, we don’t expect her to typical, but getting thinner isn’t helpful, especially when she just keeps getting taller. Anyways, we are going to very slowly go up on calories. It isn’t as easy to do now that she is fed intestinally. There is a possibility that with her sicknesses this winter/spring that she has not been absorbing her nutrition as well as she would typically as well. 

Wednesday we had appointments with her Foot Orthopedic doctor and the Orthotics department. I am pretty bummed because they want to increase her time in the AFO foot braces from 10 hours per day to 22 hours. It is such a long time to be in braces, particularly during the summer. We had new molds taken while we were there to get updated braces with some different toe straps. She is having more significant toe contractures and there is a possibility that tendon releases will need to be done in the future. 

Yesterday, Aimee had an appointment with her Respiratory Pulmonologist. After her illnesses, he said she is sounding clear, but as per her usual, she is breathing very shallow. So shallow it is hard to tell if she actually does have clear lungs. 

He was able to go over the results from her sleep study with us as well. Apparently they were able to collect a ton of data from this study, more than they have ever been able to collect for her. They worked hard to go through the data so that this Pulmonologist could review prior to our meeting with him. During the study, they were able to make a lot of tweaks to the settings during that sleep and want to make a lot of changes to her bipap. Her main respiratory issue is carbon dioxide retention. Due to her shallow breathing, all day long it is retained, but during sleep it is even worse. She has significant amounts of obstuctive and central apneas still as well. So, they want to increase her pressure for breathing in, increase her breathes per minute to 24, and adjust the sensitivity of the machine. They are hopeful that these changes will decrease her carbon dioxide levels overall. They did a blood check of her levels yesterday and will repeat it in a month or two to see if these changes have helped. I’m nervous, because everytime she has had her pressure increased at all, she has ended up with terrible/scary desatting. 

In good news, the Pulmonologist really took more ownership of Aimee’s care than he has before. He also told me that if insurance tried to take our oxygen that he would fight them tooth and nail. I’m so glad that I voiced my fears to him in that area. 

We are so grateful for a beautiful, healthy girl as Spring weather is here on our little farm. We are expecting a new calf in the coming weeks and looking forward to the abundance that comes with that birth. :)