Tuesday Hell

Monday Night, we slept like logs. In a king size bed, both us girls used every one of the 6 pillows and every inch to spread out.. The only problem was the alarm that woke us at 630... Daddy was the alarm clock, I answered the phone and mumbled "call back in 15".

We quickly packed our room, checked out, stored our luggage and caught the shuttle by 715a. First appointment of the day was..





Neuromuscular

I've had reservations about this visit and whether or not they would be a good fit for us. Upon checking in, they handed me an iPad and ask that I complete all the surveys on it - with each question, I found myself more intrigued and proud that we have landed here. "Does the child tire easily even when they've gotten enough sleep? Does exercise tire specific areas of their body? Does your child complain of weakness in their legs? Does your child trip or fall more than usual? Have you ever noticed anything unusual about the way they walk?" I answered the survey over 20min and found myself having to expand on about 75% of the questions. That brought relief, maybe Neuromuscular will be a good visit for us after all.



The doctor was amazing, she asked questions and built on them to enforce accuracy in how I answer. It's really hard to answer vague questions with precision, I don't want to mislead but I also don't want to avoid a question that may guide the doctor towards the right train of thought.. Make sense?

For example, "Is she getting better or worse?"
Answer: In comparison to where she was at 2 years old when we didn't think she would survive, she's much better. In comparison to 6 months ago, she's worsened. Her general overall health is better than it was a year ago, but the isolated areas in her body, such as her lower half (hips legs / endurance and fatigue) are considerably worse. She's fallen down our steps three times in a month. She trips over nothing because her knees buckle and she's falling asleep quickly after any amount of exercise. So is she getting better or worse? It's a hard question.

We talked for quite some time.. the longer the conversations, the better assessment i felt she was able to get of Annabelle. Finally, it was time for Annabelle's physical assessment.

They jumped, stood on one foot, ran down the hallway and climbed stairs together. After the 5min of working, Annabelle asked to go lay back down - and she did. She checked her reflexes, muscle strength across her body etc.




The doctor, I believe was able to gain an excellent assessment during this visit. She recognized the concerns we have of Annabelle before I was even able to communicate them. She soothed me by explaining that she sees MANY EoE patients, bc often times their pain and weakness is part of their undiagnosed global diagnosis, just like Annabelle. She also looked up from her writing, mid-thought and smiled, "Mom, I know this is hard, but you are a great advocate for your daughter. Don't let that go". I can still hear her tone and see that smile now. I rarely get a compliment like that from a doctor... those words bring more of a relief than I could ever describe in words.

The first plan of action is to capture a lab draw after fasting. The issue however, is that Annabelle cannot fast without being impatient bc her body doesn't tolerate it and her nutritional needs are extremely strict to avoid seizures, etc. Therefor, I plan to perform this lab draw during our next scheduled scope when we fast for two days before surgery.

These labs will look for:

• Cogenital Myasthenic Syndrome - "The muscle weakness typically begins in early childhood but can also appear in adolescence or adulthood. Facial muscles, including muscles that control the eyelids (Annabelle's left eye only partially opens), muscles that move the eyes, and muscles used for chewing and swallowing, are most commonly affected (slow GI top to bottom). However, any of the muscles used for movement (skeletal muscles) can be affected in this condition. Due to muscle weakness, affected infants may have feeding difficulties. Development of motor skills such as crawling or walking may be delayed (she crawled at 11m and walked at 16m... not too terrible). The severity of the myasthenia varies greatly, with some people experiencing minor weakness and others having such severe weakness that they are unable to walk." 
• Muscle Metabolic Myopathy - Metabolic myopathies are genetic diseases, usually inherited, that affect the body’s muscles. Metabolic refers to chemical reactions that provide energy, nutrients and substances necessary for health and growth. Some people with a metabolic myopathy (muscle disease) develop weakness; others tire easily with exercise or physical activity, suffer muscle pain after physical effort, and/or experience severely swollen and tender muscles. These symptoms occur when muscle cells don’t get enough energy. Without enough energy, the muscle lacks enough fuel to work properly.

If both these tests are negative, we will move forward to performing muscle biopsies.

The doctor seems confident our diagnosis is going to be either Metabolic or Mitochondrial. We've heard both of these for years.. Our genetics doctor agrees, as does our GI. We just need to confirm it.. And confirming either of these is really, really tricky and time consuming. She has all the signs, shows all the same patterns and shares the ailments of both diseases. When I say my prayers though, I pray with everything it's wrong. I don't want either of those labels, not on my Annabelle.

After that appointment, we walked the halls and made our way to Rehab.




Rehab felt a lot like neuromuscular, we discussed her fatigue, weakness, limitations and how all these things impact her day to day life.. and ours!

I thought our conversation was going to center around ways to help build her endurance, tricks to make life easier for belle, improve her muscle strength etc. the moment our therapist began saying words like "push chair, wheelchair etc" I froze.

"No, no no no.. Annabelle walks fine, she can play and does dance class! She isn't a kid that needs a wheelchair, not Annabelle. We just need to find a better way to handle school and physical activities, that's all."

She asked things like "does she tire when you go to the store? How much has she walked since being here? What do you do when you go to the mall or park or zoo?"

My heart sank to the bottom of my stomach and I immediately began scanning the room for both a trash can to vomit and tissues to cry. I felt like I was being cornered .. I felt just like I did when a doctor from Johns Hopkins stood over me and lectured me on how malnourished Belle was and how I was hurting her by avoiding a feeding tube. I remember how badly I fought that tube, but I love it now. It's saved my daughters life and helped our family tremendously. But a wheelchair?! No, that's for different people - not us!

I fought, hard. I debated, tried talking my way out of the appointment.. my head flooded with every excuse to avoid the conversation. And then the rehab doctor said, "Let me confirm something / or get resources (something like that... i was just hoping she would leave). That's when she came back and said the Neuromuscular doctor (WE JUST SAW!) is also recommending a chair. She then advocated for Annabelle in such a way, I knew I was wrong, I needed to stop fighting and allow what is best for Annabelle to happen. I was defeated as I looked over at sweet Belle..... she was laying on the bed and staring at the wall, utterly EXHAUSTED from the 15min of walking around we had done prior.



I bowed my head and answered the doctors questions.

At times when Annabelle's class is playing in the gym, running and hanging fun - Annabelle lays on the bleachers and cries because she cannot keep up or play. She can't physically run or walk that much anymore and it's progressively getting worse.
- A wheelchair will give her back that independence back, that she can keep up with her friends and remain included in the fun instead of constantly sitting on the sidelines, recognizing how vastly different she is (the result then trickles down into her diagnosis of depression..)

When our family goes to the zoo, mall or park - AK and I break our backs by carrying her most the way. I've spent the past two days, literally carrying Annabelle 90% of the time bc she can't walk this much. The reality is that she's only getting bigger and heavier. I don't have a stroller that fits a 40lb 4.5year old.
- A wheelchair will help our family continue to take trips and make memories, and not just break our backs and plead with Annabelle to walk on her own (which then leaves her feeling defeated and a disappointment when we get frustrated with her bc she doesn't walk on her own).

A wheelchair is not, by any means, permanent (maybe to the doctors, but not in my book), or a solution for laziness. Hopefully we won't use it daily, but it will save us on the trips and times she struggles and does need it.

We plan to purchase one that SHE can use on her own and we can also push if needed. It's her lower body (hips, legs, knees and joints) that don't allow her to keep up. It's also extreme fatigue, low endurance/energy etc. therefore we needed something that she can use when she tired so badly that she cannot move, and also something she can use for her own independence when she just cannot walk or keep up.

The conversation still, was not easy. I wanted AK with me. AK warned me this day was coming and I didn't want to hear it from him either. I know he would have received this news better than I was. That being said, I couldn't shop or discuss wheelchair options while we were there, I want to do this with AKs opinion in mind too. Shopping for one of these puppies is like shopping for ......... Fabric? A trillion styles, types, textured and then colors and patterns. Every single chair they showed me, the only thing that ran thru my mind was "nope. Not getting it. Nope, don't want that either. Oh pink! ?! .. Nope, still don't care. No wheelchair." I took a prescription for the chair and will shop for one in Richmond instead. (My pathetic way of winning that battle lol).

I will say, Annabelle is excited about it. They explained to her how wonderful it will be to have a chair she can sit in and still have fun and play when her legs are too sleepy. Once they told her she can have a pink glitter one, she was sold. She calls it her wheely-chair, and wanted it immediately. To the point every time we saw a child in a wheelchair in the hospital for the rest of the day, she asked "Is she sitting in MY wheely-chair?!"

After the appointment wrapped up, I needed to escape. We packed our things and left as quickly as I possibly could. We had lunch and headed back to the hotel to pickup our bags and enjoy a few hours by the fire and not in a frickin hospital. While Annabelle napped on the lobby couch, I collected paperwork and legibly updated my notes so I wouldn't forget everything that had been discussed.



130p we headed back to the hospital for our final appointment appointment of the trip, Neuro-psychological Evaluation follow up.

This appointment was actually gentle. I think all that we had just been thru of the day, I took this one the best. It also helped that I had already read the final report for the eval and knew what to expect from the conversation. She explained the evaluations findings and what that meant for belle overall. She then explained for me, what depression and anxiety are like for a 4 year old and what contributes to them: prematurity, her overall health and medical issues. It's like a Venn Diagram.. when you suffer from all those things like Annabelle and they begin to overlap one another, you begin to psychologically suffer.. Annabelle has never been taught how to process all these things that are happening in her life, and when she does attempt to do something, she fails (ends in pain, confused or bad behavior = timeout).

I asked the Psychologist about ways we can help her work thru them and which things were in my control and which weren't. (Medical issues and impaired brain development from prematurity, malnourishment and years of drugs). The ways I can help her are mostly thru positive encouragement, structure and coaching with very very small expectations, then working our way up. Basically, set every situation up in a way that she will succeed. In life right now, most all situations Annabelle faces, she will fail.. we will work to shift that.

We discussed ADHD and what that means for Annabelle. My main concerns are how shes progressing academically. The fact is, she isn't. The neurologist says that if we place belle in kindergarten. The risk for causing additional anxiety and adding to the depression is between a moderate and high risk. In the wrong setting (not structured but high academic expectations), we would only set her up for failure. That being said, keeping her in the same preschool program also may not help and support moving her forward academically. We're just kinda between a rock and hard place. My approach: shop. I'm going to shop for the best fit of education that encompasses a highly structured schedule (for her ADHD) and lower academic expectations (for anxiety and depression). With this right combo for Annabelle, I trust she'll excel in school. I just need to find the right fit.

This appointment lasted an hour and we discussed SO much. I am able to understand my little girl on a level I never did before. I understand how she thinks, why she frustrates, why she handles things the way she does and also recognize how our parenting is helping/hurting her. Parenting is never a one-size fits for all kids, I know that, but Annabelle seems to be a curve ball we REALLY weren't able to understand until now. What a priceless gift this doctor was able to give us. (No wait, not priceless.. Im sure I just paid an insane amount for that, but still). I left with a lot of resources and homework to better understand and shift our parenting.

After we were finished, Annabelle and I headed downstairs to await our scheduled shuttle pickup for the airport. I purchased Annabelle a gift in the gift shop (tiny plush toy) for being so good during our trip. We took our seat in the lobby and exhaled. Finally, WERE COMING HOME!

  

Flights were a nightmare. Not much else to be said. They were all delayed. The snow was coming down sideways in huge clumps. At one point on the plane, I didn't know if we would even be able to make it out. Our plane was de-iced and the runway was being plowed as fast as they could move. The weather was awful. Annabelle was awful. My anxiety was awful. We just wanted to be home so bad! Our layover in Philadelphia flight was not much better.. equally frustrating, delayed, bad weather, plane de-iced etc. but we were able to fly into Richmond vs Reagan, so that helped tremendously. We made it home shortly before midnight and everyone fell asleep, in our own beds, and again slept like rocks. Whew, the marathon was over!




This trip was hard. Probably the hardest trip we have taken. I don't know if it's because of the sheer amount of running, walking, appointments, shuttles, coordination involved or if it was because of the information we learned was simply so hard to accept. This trip was the easiest for Annabelle. I made every step of the way exciting and fun for her. She has no idea how stressed and upset I was (well, she may a little, but she wasn't phased). All Annabelle kept saying once we made it to the airport to come home was, "5 appointments, FIVE mommy, and NO POKES (needles)" she was so stinking proud and happy. This was a victory for her in the course of our trips to a hospital. Honestly, I can't recall a time when we went to an appointment and there WEREN'T any pokes. She's become conditioned to associate a medical appointment with pain, and this trip allowed her to see that not all hospitals, appointments and doctors are bad. I am so grateful for that.

The trip has emotionally taken a toll on me. I am fighting an internal battle with the acceptance of a wheelchair.

let me pause for a second.. I KNOW this is not the end of the world. I KNOW children and people have it SO much worse than we do. I KNOW it could be worse than something as materialistic as a chair. But it's still hard, as a parent, to hear and also accept. So please, do not roll your eyes that I am being dramatic for no reason. This is hard. 

I fought our feeding tube like it was a war. In my mind, I thought a feeding tube meant the end of life (and at the time for Annabelle, we were there). I thought if we placed a feeding tube, she would never be able to eat on her own again or never have the opportunity to do so. I feared the emotional trauma it would cause her as peers, children and adults, gawked at the tube taped across her face. I feared that if we placed a feeding tube, it would stay forever and ever and never come out. I hated how permanent it was.

That feeding tube saved my daughters life. In the end, I fought an unnecessary battle that only hurt Annabelle and prolonged her malnourished. I will never, ever, forgive myself for that. But it was hard. HARD! In my screwed up, exhausted, defensive, 150mph mind I thought accepting a feeding tube, meant saying goodbye to my baby.

I didn't say goodbye to my baby, but we do use that feeding tube. We rely on it much more than I would have wanted to know before it was placed. Go back 2 years and I would be so upset to see my daughters tube and her feeding needs now.... but it isn't a bad thing! It was a learning curve, yes. It's inconvenient in many ways, yes. It's expensive, painful, constant maintenance and the supplies takes over my house, yes. But I would be LOST without it. I am SO grateful for that tube and what it's given to Annabelle.

I'm in the same place about this wheelchair. I don't think she needs it.. but deep down I know she does. I don't want it.. but I know it's necessary. I am making myself artificially accept this next wave as our newest adventure and necessary blessing. I hope one day that I can sit back and reflect, "oh, remember that time I hated the thought of a wheelchair? What a fool I was, we LOVE our wheelchair!" In the meantime, it's going to be exhausting, expensive, time consuming, space consuming and a learning curve all over again. But if it brings life and joy to Annabelle as much as that tube did .. we will be ok.

I keep reminding myself,

Doctor's do not place feeding tube's in children that don't need them.
Doctor's do not put children in wheelchairs that don't need them.
If I have ever trusted a team of doctor's more, they are the ones in Cincinnati. I trust them. I know they are doing what is best for Annabelle. And we are SO blessed to have the opportunity to be part of their hospital, research, care. Every element of it, this week... sleep clinics, neuromuscular, rehab and neuropsych.. what a blessing they all are. They are giving my daughter the best life she can possibly have.