I have attempted to write this update for almost a week now, and each time I sit down and reflect on what is happening, my heart shatters and I slam my phone down. Please bare with me as I very quickly update you guys on what's going on with our sweet Annabelle.
Essentially, all we know right now is that her GI system shut down for 6 days and is not working properly any longer. It is not moving contents from her stomach to empty, nor is it emptying from the bottom. Everything in between is largely swollen on xray and her tummy is severely distended to the sight and touch. They are labeling this condition as an ileus.. which gives absolutely zero indication as to why this began or how to fix it, it just explains what's happening.. "GI shut down".
Monday Annabelle woke with a high fever. She had complained of feeling yucky around 3:30am, so when she woke with a fever I decided we would take her to the pediatrician. We landed an appointment at 10:15a and tested positive for strep throat, although her throat wasn't red and she didn't have any complaints, so hopefully we caught it early. I filled her prescription and she spent the afternoon snuggling in bed and resting. Each time she would try to eat, she would begin retching (dry-heaving bc she cannot physically vomit).
Tuesday was a much better morning. She woke for the day without a fever, happy as a clam and bouncing around the house. With barely 24hrs of an antibiotic, she was already feeling much better. By noon she was ready to try to attempt eating something and I gave her a few edamame .. she slowed down. She laid down quickly, complained of tummy pain and then began retching again. I thought perhaps her fever was returning and she was feeling crummy from the strep.. so I gave her a dose of Motrin and encouraged her to relax a bit.
Within the hour, everything changed and began to spiral downhill. She became extremely weak, each time she would try to walk her legs would buckle. Her body was retching every 10-15 minutes and then her legs began to hurt. At first they were just crampy, but then she started to cry as she would squeeze her thighs and calfs in her tiny hands and look at me desperately for help. They started to tremble and I told her to stand up, perhaps the circulation was making them ache, growing pains(?)
She continued to cry that they were hurting and they continued to tremble harder.
She continued to cry that they were hurting and they continued to tremble harder.
My mind started to race in two different directions. Initially, I wanted to chalk everything to strep / lack of eating / antibiotics and keep seeking rationale as to what was happening. And then the other side of me went into doctor-mom mode and I found myself watching her from a distance so I could collect every bit of information as to what was happening....
I hate when I turn into this woman, I think I scare myself as much as I scare her while she watches me assess her from a distance. When you've been told for years and years that no one can figure out what is wrong with your child, and doctors have even accused you of making things up, it's hard to rationalize what is happening. I never want to hurt Annabelle and over react, but I also don't want to miss something and not seek help at the right time... our journey together has made this timing and decision making quite muddy. So I watch, I assess and I collect all the information and I (literally) write it down so I can process what is going on:
I hate when I turn into this woman, I think I scare myself as much as I scare her while she watches me assess her from a distance. When you've been told for years and years that no one can figure out what is wrong with your child, and doctors have even accused you of making things up, it's hard to rationalize what is happening. I never want to hurt Annabelle and over react, but I also don't want to miss something and not seek help at the right time... our journey together has made this timing and decision making quite muddy. So I watch, I assess and I collect all the information and I (literally) write it down so I can process what is going on:
- retching
- trembling
- fatigue that is getting worse by the minute
- extreme tummy pain
- leg pain and involuntary quivering muscles
- pleading Annabelle "Mommy help me, please!"
By the time I navigated our back roads and made it to the interstate, Annabelle was 'asleep'. I called her name a few times and reached behind me to pat her leg and she wouldn't wake. Perhaps she was just exhausted from the two hour marathon of retching and pain? I parked in the Emergency Parking Lot at St. Mary's and picked her up from the backseat. She was asleep....... and she was covered in stool and urine. She was clean prior to leaving our house bc I had changed all her clothing. My heart sank and I quickly loaded her into her wheelchair where she flopped from one side to the other as we entered the ER.
From here things moved quickly. Annabelle was now conscious but not very coherent. She had a hard time following you with her eyes around the room and she appeared utterly exhausted. We concluded she had likely suffered a seizure in route to the hospital which explains the involuntary loss of stool/urine. Another thing I learned is that, similar to migraines people will see stars prior to the migraine hitting, experiencing leg and joint pain is a premonition your body gives prior to having a seizure. Interesting, huh?
We took her to xray almost immediately and scans showed an ileus. Her intestines and guts were visibly swollen. After she began to wake a bit, we encouraged her to eat a little something.. we started with a popsicle, two licks and she wouldn't touch it. We tried apple juice, she had drank enough to wet her mouth.
The doctors joined us and informed our family that Annabelle wouldn't be going home any time soon. They wanted to admit her until they could resolve what was wrong and allow the ileus time to recover. We were taken upstairs to the pediatric floor, met our new doctors around 1030-11pm and began our hospital routine.
From here things moved quickly. Annabelle was now conscious but not very coherent. She had a hard time following you with her eyes around the room and she appeared utterly exhausted. We concluded she had likely suffered a seizure in route to the hospital which explains the involuntary loss of stool/urine. Another thing I learned is that, similar to migraines people will see stars prior to the migraine hitting, experiencing leg and joint pain is a premonition your body gives prior to having a seizure. Interesting, huh?
We took her to xray almost immediately and scans showed an ileus. Her intestines and guts were visibly swollen. After she began to wake a bit, we encouraged her to eat a little something.. we started with a popsicle, two licks and she wouldn't touch it. We tried apple juice, she had drank enough to wet her mouth.
The doctors joined us and informed our family that Annabelle wouldn't be going home any time soon. They wanted to admit her until they could resolve what was wrong and allow the ileus time to recover. We were taken upstairs to the pediatric floor, met our new doctors around 1030-11pm and began our hospital routine.
Wednesday was tough. Annabelle was very weak and the doctors were hesitant to allow her to have anything orally, as no one quite knew what was going on. While I continued contacting Cincinnati Children's Hospital and our team there, Annabelle's nurse cared for Belle in our room and brought comfort and normalcy to such a chaotic couple days.
The first agenda Wednesday morning was to determine why she had endured a seizure and if so, what it was caused by. They did an EEG and Annabelle handled it beautifully. I give a lot of credit to the tech that performed the EEG and also the extreme fatigue Annabelle was under.. had she wanted to fight, I don't think she would have even had the energy to do it anyway.
That evening, AK and I felt it was best to allow the girls to see one another. Madelynne carries so much anxiety and worry about her sister on a daily basis and situations like these really manifest in her poor mind and heart. Annabelle wanted her sissy for comfort, and our entire family honestly just wanted some time together. After AK and Mady had dinner, they visited us in the hospital. Mady brought Belle multiple goodies from the house to play with and she cautiously climbed in the bed with her as they watched a movie. While those two girls can fight like cats and dogs some days, the love and understanding they have for one another is nothing short of unconditional. They need each other, just as I need my husband and our family needs one another. Daddy and Mady stayed until well past bedtime and we all snuggled watching TV until we finally said our prayers together and hugged Goodnight.
Thursday we met with the Neurologist that discussed the results of the EEG. Fortunately the test did not show signs of a seizure nor could they initiate more seizure activity during testing. None of us knew why Annabelle suffered another seizure out of nowhere, but we did all decide that we would not move further with seizure medications, as we have no clue what provokes them to start with. (meanwhile I am internally SCREAMING "This is why we need a diagnosis to explain these things!!")
Thursday also marked the 5th day since Annabelle last ate any food / tolerated a formula feeding. We were growing more and more nervous by the hour that her system wasn't recovering. And while the nutrition side is important, the lack of nutrients also started to drop her blood pressure. By that afternoon her BP was 83/52.
We encouraged her to eat. We ordered chicken broth, applesauce, ice cream (sorbet), popsicles, apple juice, jello . . . she wouldn't take more than a half taste of anything. By late morning, we spoke with the doctors and discussed our plan bc I needed some clarity for what they were doing, "She needs to start eating. Once she can begin to eat and prove that she can keep enough food and formula down, we can go home." Belle was becoming restless, she wasn't feeling well but she wanted to be home. The hospital's plan was only to treat the acute reason she was admitted and send her home, so my focus became "let's do what we need to get out of the hospital so that we can get to Cincinnati and determine what's happened and why so we can fix it."
I asked if we could begin tube feedings. I didn't like the pressure that everyone was putting on Annabelle to put food in her mouth when subconsciously she was terrified of what the food may do to her. She was scared and the threats of "If you don't eat, you can't go home" just didn't seem fair. She was upset as we were asking her to do something that she knowingly *may* cause her severe pain. I say 'may' because we didn't know now, 4 days later, if she would still be in pain.. perhaps the food would digest and she's just too scared to try? We've all been sick with a tummy bug, I get it.That afternoon, we started her on a diluted 3/4 pedialyte 1/4 formula feed via pump at a rate of 30mL / hour. (Translation - ridiculously slow. On a good day, Annabelle can tolerate a feed at a rate of 150/hr or better). Within about 20min of beginning the tube feeding, she began to feel nauseous so we asked for Zofran. That hardly touched anything, Annabelle went from being giggly and silly, sitting up in bed to rolled on her side, white as a ghost and silent in pain. We called our nurse who paged the rest of the doctors and they came in to see her. Everyone agreed that something was wrong for a little girl to spiral this quickly downhill within 45min of beginning a very delicate feed. Our GI doctor performed a few very uncomfortable tests on Annabelle as we held her down and then concluded that we needed to stop all feeds and prep for the next morning. He was convinced she had some type of abdominal obstruction. In order to perform the dye/barium test, she would have not been allowed anything on her stomach for 6hrs. In addition, if we did find the obstruction, we would need an OR team available to perform the work of fixing it. Therefore we all decided to let Annabelle rest for the evening. We would snuggle and spend time together and first thing in the morning they would send her for the test and we would move from there.
The night was difficult. After AK left, my heart and mind raced as I laid in the bed beside that sweet baby girl. She was getting so big. When this began she was a tiny baby that was helpless and couldn't talk but would watch me with the biggest eyes and plead for me to help her. It was heartbreaking. Now I lay beside a growing almost 6 year old little girl that can verbalize what is going on. I always thought it would be easier when she could "tell me" what's wrong, but it isn't. She still lays beside me helpless and she still watches me with the most beautiful big eyes but when she asks "Mommy, why am I sick? Why can't the doctors fix my boo-boo's? Mommy help, please!" . . . I am speechless and find myself more heartbroken then ever. I've come to realize that no matter what age or stage, no matter what circumstance whether it be the day before a surgery or a sprained ankle, this parenting business is always full of an aching heart that we can't do more for our babies. So that was my prayer Thursday evening, for understanding, patience for myself, grace in all that we have positive surrounding us and peace that I am doing the best I can for my daughter with all that I have.
Friday morning began early for me as nurses were in and out of Annabelle's room like a revolving door. Fortunately, Belle remained asleep for most the chaos. I didn't want her to wake until daddy arrived and they were ready to take her for testing. Shortly before 9am, we moved downstairs to Radiology where Annabelle began the contrast test.
She did a wonderful job. For as much barium they put thru her tube into her tummy, her complaints and discomfort were minimal (thank heavens to all the drugs we gave her prior!). The test should have taken just over an hour.. Annabelle's took approx 4 hours.
Later that afternoon, the GI doctor joined to tell us our prayers were answered and they did not find any signs of an obstruction. However, her GI system was still moving at a snails pace, but at least it was starting to move. We agreed to give the slow feeds a try once more and see what happens during the night. Once Annabelle went to sleep, we gave her more nausea meds and began a very slow, diluted/formula feed again. I was eager to start getting nutrients into her body as her blood pressure was still continuing to drop by the hour. Friday afternoon she was down to BP 67/38. She couldn't sit or stand much without becoming symptomatic. This was Day 6 of no foods and hardly sitting up or moving.. something needed to change quickly. I prayed harder.
Saturday was the day Annabelle finally woke and when the nurse arrived she said on her own, "I am going home today and Mommy's taking me to Jumpology!" (cue my shocked face! "Um, What?!
haha). For the first time she was very perky and happy. She was sitting up in bed and eager to pick out new movies and do a craft together. We had friends visit us Saturday and we were so, SO happy to show off how well we were feeling. Our doctors still were not comfortable letting Annabelle leave until she could prove she can eat a bit on her own. She drank and ate a very, very little but I assured the staff that I was comfortable with the amount she was eating, as long as she was tolerating her tube feeds. They trusted my knowledge of my daughter and our plan of care for her while she was home.. before we knew it, they were preparing the discharge paperwork.
By late afternoon, Annabelle and I broke free from the hospital. We came home and took the hottest, best shower of our life (first shower in 5 days, gross). We put on jammies, and the entire family crawled into bed together to watch a movie. Boy had we missed being home.
Annabelle is stable at this point but not well by any means. Her BP is still low but is improving daily and her diet is still very low but she is tolerating slow feeds. We have no idea why her GI system mysteriously stopped working for 6 days, but we are headed to Cincinnati to our team to hopefully find some answers.
While inpatient at St. Mary's in Richmond, nearly every doctor we met explained their concerns for Annabelle being so globally undiagnosed. The words stab me in the heart as I understand the urgency for these answers in an effort to help my daughter.. but we simply can't get them. I explained that I have appealed the insurance denials in every avenue I have found and still, they will not be part of testing. The doctors in Richmond were quite honest in their speculation of Mitochondrial Disease, which is one of our biggest fears. I recognize and support their thought process on why they believe Annabelle may be suffering from Mito. We had many long conversations with the doctors and I asked most everyone "Do you have any advice for me on how to keep fighting for these answers? Perhaps a different avenue? A trick or secret that I haven't explored?" We did get some great advice:
- Instead of continuing the Genetic Testing thru our Geneticist, perhaps have her Neuromuscular Doctor request the genetic testing? Sometimes it looks like a geneticist is simply nosy whereas a different doctor is requesting the test for a specific diagnosis. (Of course we have everything documented from every single doctor on her team, but maybe the top name being "Neuromuscular" could help?)
- In response to insurances denial reasoning "Medical studies do not show that this test improves health. For this reason, it is investigational and not medically necessary". The doctor in Richmond said that our physicians need to respond by "it may not improve her health, but it will provide clarification and explanation to her health's progression. Without this diagnosis, the patient will continue to undergo testing, prolonging the need for multiple insurance claims etc. (half of me fears this is the goal of an insurance company.. they know they can make the most money off Annabelle if they keep her sick vs helping her get well)
