Friday, May 19, 2017

GI has completely shut down

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.
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:
  • 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!"
... something wasn't adding up right and it sure wasn't in my head. All I knew is that when Annabelle spirals out of control at rapid speed, something is wrong. That was about the ONLY thing I knew for certain, that this was getting worse by the minute. As unpredictable as she was behaving, I didn't want to see what was next. From the first signs around noon, it was about 2hours before I threw in the towel and loaded her into the car to head to the hospital.
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.

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:

  1. 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?)
  2. 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)
The team in Cincinnati is currently reviewing the medical records and notes for Annabelle that took place during this admission. With those they'll help us develop the best team of resources when we arrive in a few short weeks. We don't know what to expect yet beyond a fleet of testing.. but I will prioritize finding a different route for genetic testing while we are there. This trip has the potential to be quite comprehensive, so well take any and all well wishes you can share!

Thursday, March 30, 2017

Our Journey for Answers ...

There are truly no other words to describe what happened the beginning on January. It's been years since we've seen Annabelle crash that hard and fast before, I was scared to death we were losing her as each and every system started coming down with one another.. her team panicked, her nurse was scared, and mommy and daddy were motionless and terrified.

Annabelle had relapsed, hard.

In November, we introduced her to pork (bacon, sausage, tenderloin and ham). When her body began to respond to the new protein, it started attacking itself. We didn't know what was happening until it was too late. Annabelle has lived a life of pain, so for her to complain of hurting is nothing unusual.

Around Christmas she got worse.. shortly after Christmas she began crashing and we started watching her body slow down. She couldn't run or play, she could hardly stand without falling or being dizzy and her blood pressure began to show signs of distress. Annabelle stopped eating.. we bumped up her tube feedings. Then her pain became so violent, I was calling Cincinnati almost every day pleading for an appointment or some type of guidance to help her. Minutes felt like hours and days felt like an eternity. Once it got to the point we couldn't even send her to school as she could hardly stand, move or cry - her team began to weigh whether or not we needed to admit her to a local hospital or flea towards Cincinnati Children's. We decided late that evening that we would find the earliest flight and by the next morning, Annabelle and I were in route to getting her the help she needed.

January is hard to reflect back on. I've wanted for months to share with you all what happened during those weeks in the hospital and following during her recovery but the words are hard to find.. and sometimes, just not expressing them is easier to mourn what's happening to our daughter. So I thank you for understanding. It isn't often that I throw in a towel for complete privacy on Annabelle's Journey (although I don't share nearly as much as it seems sometimes), but during this time - I haven't been able to process what happened enough myself as mommy, let alone find the appropriate words to share.

That being said and my heart full of gratitude for your understanding - I do want to share where we are now.

Annabelle is still recovering from the relapse. It took months for her immune system to improve. During that time she was left susceptible to every germ and virus in the county.... and she caught it. With every illness, we held our breath. Knock on wood, she has been healthy for a few weeks now finally and we are counting our blessings.

While we navigated the waters of illness after illness, we've also had some meetings with her school and phone conversations with her medical team.

Unfortunately, the physical demands of a relapse take a huge toll on how Annabelle performs academically. When she's in pain, when she's fatigued etc she cannot and does not concentrate or focus. When your body works so hard fighting for your health, developing new skills, concepts and learning a world of new things becomes very secondary as a priority for the brain. Our Neuropsychologist explained in great detail the direct correlation between chronic illness and learning. As a result, we are seeing the affects in how Belle performs academically. While her health is a top priority, normalcy and being a child has also always been a priority for us too.. we want our daughter to excel in school, at least to a point that she doesn't struggle, frustrate and begin to dislike school. I don't need to go on as to the importance of prioritizing school / structure / learning / friends etc, but I did want to share that at this point, AK and I were left with some big decisions in how we will balance her health vs education.

Annabelle needs as much classroom focus and attention as we can give her. For that reason - we decided to cancel all our food trials and any testing or doctors appointments until school is over. She also needed the medical break and we didn't think her weak immune system should be pushed much more right now.

Because we've decided to cancel food trials, that leaves Annabelle with very little protein in her diet. Protein is critical and our next food trial was going to be trialing another form of protein (she choose fish! ) So now, that option is gone and were left continuing to balance a weak diet. At the same time, her system has been having a harder and harder time digesting Elecare (liquid medical food we tube feed her with). We're at the point that we dilute the Elecare each night before bed, otherwise she will spend most the night crying in pain from a full belly. We're not doctors so clearly we have no idea where the pain is coming from, but diluting the formula seems to be providing some relief so we're going with it.

She is also having a more difficult time getting rid of gas in her GI system. We haven't had to "make bubbles" (we do this by connecting her g-tube to a long 'straw' like contraption called an extension and then a 60cc syringe. Once we remove the plunger from the syringe, there is an open passage for any air to escape her stomach thru the tubing.) Annabelle has a Nissen, where her stomach is sewn snug around her esophagus, preventing her from being able to burp or vomit. For a while she was doing better with her body filtering the gas but recently a lot of gas is getting stuck in her tummy and were required to extract it by tubing multiple times x day.

We have a few more things going on.. such as the weakness in her legs is getting worse and we've noticed her struggling in new ways that we haven't seen before. Her depression is getting harder to  manage (hurry up summertime and pool weather!!) and her anxiety is skyrocketing but all in all.. it's just another day with our Annabelle.... and not to mention a 5yo old going on 15 ;)

... so that brings us here.. to the purpose of today's post.. the update that literally has tears dropping from my chin as I type..

Insurance has again denied all of Annabelle's Genetic Testing. We received our denial letter a few months ago and I immediately appealed. I have appealed thru my employer with HR, I have appealed directly with the insurance company and we have had doctors in the hospital contact and appeal the decisions as well. Despite our frantic efforts at fighting this decisions, we are at a dead end in Annabelle's "Answers for Annabelle" Journey and simply don't know what to do.

As parents, we are grieving.

- We are grieving the absence of a diagnosis that we prayed would give us some answers for our daughter.
- We are grieving the heartless decisions made by a corporate health system that looks at our child by a dollar sign and not a human needing help.
- We are grieving the loss of words when our children ask "Will the boo-boo's go away?" "What will make Annabelle better?" "Will she ALWAYS be in a wheelchair?" "Is Jesus going to let Annabelle die?" "Why can't the doctors fix it?"

We don't have answers for family.. friends.. doctors.. the school during an IEP / 504 meeting. We don't have answers for our children, we don't have answers for recreational sport teams, we don't have answers for one another as we look into our spouses dark eyes of confusion and terror.

We are mourning as a family.

Genetic testing is astronomically expensive and is not affordable for us. The purpose of this blog is to share Annabelle's Journey with those that love, support and pray for her as she moves mountains and overcomes some of the most heroic things a child never should have to. I won't share finances here, but I will share this: supporting, caring for and fighting for a chronically ill child has to be the most financially destructive path a family can endure. We make the best decisions to align our children's lives in a good school, good home, healthy food, kind friends and warm church - those are the foundations that will hold us together regardless what lies ahead. Everything else is to support Annabelle, and let me just say, she has drown us. She is worth every single penny... bc heavens knows she's taken it, but there becomes a balance in which we still have to preserve what is best for our family. Every trip to Cincinnati costs our family no less than $1,000.. last year we traveled to Ohio eight times. EIGHT. That does not include the out-of-pocket wheelchair, medical supply, endless prescriptions, etc. I don't care how much money you make, I don't know anyone that can afford this financial burden.

That being said, we cannot afford to pay cash for Genetic Testing on our daughter. Without this test, our Journey to finding our official diagnosis has ended. . . and we are broken. Numb. Hurt. Utterly terrified. Confused. Nervous and just.. broken.

Our fight for keeping our daughter healthy, comfortable and stable is not over. We will continue to do that each and every day as her parents. We will continue all our care in Cincinnati Children's Hospital as they are truly the best of the best when it comes to managing her Eosinophilic Disease. We will continue to blindly navigate the muddy waters when she relapses, even when we don't know why. We will continue to react and fight when her body mysteriously crashes. And with every step in the future, we will pray that God leads us to safety with our daughter, even if we don't have answers or a 'user manual' on how to keep her healthy. Our faith is being tested but it's getting stronger. Our family is broken but our snuggles are tighter. Our worries are heavier but our priorities are more clear.

I will continue to update and I hope those that have always loved our sweet Annabelle will continue to pray for her and our family. We are blessed to have you walk this journey with us.

Saturday, January 7, 2017

Emergency Trip to Ohio.

"Hope is not pretending that troubles don't exist. It is the hope that they won't last forever. That hurts will be healed and difficulties overcome. That we will be led out of the darkness and into the sunshine."

We're in the midst of our first food trial in years. Annabelle chose to trial Pig (pork, ham, bacon), we are thrilled to have the opportunity for another protein in her diet. When we agreed to do a food trial, we made a compromise and promise to Annabelle that this would be our last trial thru her disease until she is much older and she can make these decisions for herself.

In a perfect world, we would want so much more for our daughter. We would want her to be able to enjoy a birthday cake, or eggnog, or a cookie and trick-or-treating, we would want her to be able to run, skip and swim all day without pain, we would love to allow her to play in the chick-fil-a jungle gym at Christmas or attend an inflatable bounce-house birthday party without wearing a face mask. Then again, in a perfect world we would also wish for Madelynne to be more flexible so she could do the splits like her sister or have a dash more common sense so she doesn't walk into walls or trip over air (laughing, but that's so my kid lol) - reality is that we have and love our children just the way they are. We wouldn't change their personality, abilities or limitations for a second but we do pray for a happy, healthy and pain-free childhood. Our only goal in raising our girls are to keep them as healthy, safe and loved as we possibly can and let God do the rest.

That being said, I do want to share that our family is coming to such a peaceful place with who and where we are. I am so happy to say that - wow - I literally just exhaled. For years it's felt like we have swam against the current in fighting for our daughter. And we have! I have stood toe to toe with Physicians in more hospitals across more states than I care to count. Together as a family, we've spent more holidays in a hospital bed than we should, I've wiped more tears and held down a baby like no one ever expects to. AK and I have spent so many sleepless nights, ridden with fear and worry for our family and it's all finally taken us to the place we are today. Those fights, tears, sleepless nights, money, heartache and brokenness was all to carry us here... I wouldn't change a single thing to be where we are. In peace with the brokenness, finding grace thru the pain and seeing all the blessings that God grants yes.

The amount of clarity in our lives that I parent with now is priceless.

I wanted to share that insight of our lives to bring you to where we are with Annabelle and in our family today.... let's start with the least painful:

Insurance has denied all our appeals for Genetic Testing.
The denial letter states, "Based on the review of the information provided to us, the service you have requested (Exome Genetic Testing) is considered investigational/experimental and is not covered. Your doctor has requested genetic testing for you and your mother due to your medical conditions. Medical studies do not show that this test improves health. For this reason, it is investigational and not medically necessary. This decision does not mean you cannot or should not receive this service. But, this decision means that if you do receive the service, it will not be covered on your medical plan."

My heart drops for our daughter. True, Genetic Testing IS experimental, but it is the only way to know what is genetically wrong with Annabelle. We have spent years, tests and so much money in preparing for this Genetic Testing, we were so close and now insurance will not participate :( Basically, they don't want to play along because we cannot guarantee the test will find anything (we're 99% certain it will, but we have to look first!), and if we cannot guarantee it will find something, then we cannot guarantee it will change Annabelle's Plan of Care.

I CAN guarantee though, that having this information, and the ability to finally label and understand what is wrong with our baby girl, we WILL be able to improve her quality of life, care, we will be able to recognize what helps/harms her system. We will have a label for doctors that opens up so many doors to providing her the best treatment possible. We will know how to advocate for her, find her the best doctors that specialize in her case, and not walk blind into the unknown world ahead!
  • Tiny example: Insurance denies our wheelchair for Annabelle. Why? Because her only diagnosis of Eosinophilic Esophaghitis, a GI Auto-Immune Disease, does not impact her ability to walk. Absolutely true(!), but the undiagnosed side of Annabelle DOES impact her ability to walk, muscle strength, endurance, fatigue etc. None of those things are associated with EoE, but Annabelle experiences all of them. And until we have a diagnosis that reflects ALL of Annabelle, she will never get the services she needs to live the best life she can - aka: wheelchair. So for now, we pay cash.... bc we don't have a diagnosis...  clear as mud?
We are still fighting hard for Genetic Testing though. Without this test, we are practically at a dead-end in her care and progress moving forward. On the other side of this door is an answer that has the potential of saving our little girl... I just have to find another key to getting it.

Now for the unpleasant news that I didn't want to have to share.. Annabelle's body is crashing. 

Everything had been going so well! She had been enjoying life so much in fact, that I've remained terribly secretive in sharing the good news for hopes of not jinxing it (haha, a little superstitious, I am). Until week 7-8 of her food trial, we had hopes that she would be gaining pork into her diet without issue.. Unfortunately, right before Christmas, the progress came to a screeching halt.

The night it all began, Annabelle was making Christmas cookies with her cousin, sissy and friends.. in the midst of the fun, she came to my side with eyes the size of quarters and told me something was wrong and her boo-boo's were hurting again - I assumed her belly was bothering her from dinner or extra sugar, but she wouldn't leave my side. She curled in my lap and I could feel her exhales and moans - with friends and neighbors surrounding me, my mind raced and instinctively I knew "this is it.... she's turning a corner.. hold on mama, it's begun" - But that's the worst part of this disease and the rest of Annabelle's body. When she begins to spiral away from us, we have absolutely no clue how to rescue her and stop the painful manifestation. I gave her meds and started removing pork from her diet in hopes that the pain would start to subside. Days before Christmas, I called her team in Cincinnati and reported what was going on. They scheduled us for a scope at the end of January and we all prayed she would improve so that we could complete the 12wk food trial and have a clear answer if we can keep pork in her diet (we so desperately wanted this source of protein).

Christmas service at our church, Atlee Community. 

Christmas was a struggle for Annabelle. She wore a smile everywhere we went, but she sobbed in pain every evening. After the Christmas activities were over, she curled on the couch with mommy, daddy and sissy, and we didn't leave the house. For nearly a week straight it was Bathtime.Jammies.Bedtime.Snacks.Bathtime.Naptime.Bedtime.Snuggles. I cautiously watched my daughter from a distance and began to die inside... there is nothing I can do to help her, I have prayed so hard, but damnit this disease and whatever else plagues her body has reared it's ugly head and again torments our family! I am so helpless in this fight, and it kills me.

Around New Year's, Annabelle was again laying on the couch watching TV. I went to her side to check on her and noticed piles of hair around the couch. I immediately snapped, "Annabelle Grace! Did you cut your hair?! Where did you get scissors? Where did this hair come from?!" She was so surprised and disappointed as she cried back "I don't know mommy! I didn't cut my hair! I promise!" Her words sounded innocent so I cleaned the mess and went back to household chores.. sometime later, while standing in the kitchen, she began to have another bout of pain where she started ripping clothes from her body, kicking the blanket off and then . . . . ripping her hair out while crying. My stomach sank so fast I thought I would vomit. Immediately after consoling her, I phoned Cincinnati and put in a mayday call to get something started about getting her help asap.

Within days (this past week) her body began to shift again and her cardiovascular system began to show signs of dehydration beside being overly hydrated. Between her heart rate continuing to rise (Pulse: 103) and her blood pressure dropping ( BP: 81/55 ) more and more with each day - we knew something was happening to her little system. By Thursday, her doctor instructed us to stop giving her pork and take her to the Emergency Room immediately. Annabelle's nurse and I have been watching her like a hawk. I spent most the week at home with Annabelle where we could keep a close eye on her changing little body.. her nurse and I agreed to continue working hard at home to keep her hydrated and try to get her BP up on our own to avoid the hospital. Meanwhile, Annabelle went onto three Physicians stand-by lists for the next available time slot for an Upper and Lower Endoscopy. We waited, we prayed like crazy and waited....

By Friday, things continued to get worse. Her blood pressure lying down was 100/58, pulse 78 - pretty good for Annabelle :) However, while sitting her BP was 94/60, pulse 89 and once she stood up it dropped to 85/44 and pulse 92. Each time Belle stands up, she tries to vomit and will seldom fall back down. She's screaming in pain, refusing a lot of foods, coughing and choking on everything and generally declining. By noon on Friday, I spoke with our team again and they called back within the hour - We needed to get her Cincinnati as soon as we possibly can so they can treat her right away if she crashes any more. They're admitting her first thing Monday morning and her doctor gained privileges to the OR on Tuesday (not his allotted OR / Surgery day) and we will investigate then what is happening. At this point, sadly we've sacrificed our food trial :( I know, in the grand scheme, it's the least of a concern but in the back on our mind, we really did want this for Annabelle. On the surface, something terrible is happening to our daughter, and we have absolutely no idea where to begin.

Tomorrow morning, Sunday January 8th, AK is taking us to Baltimore Airport where Annabelle and I will take a last minute flight to Cincinnati, Ohio. The weather in VA has not been friendly this weekend and I don't want to risk the drive thru the mountains, especially in the condition Belle is in right now. Unfortunately AK cannot come with us :( He has to work and stay home with Madelynne, so I recruited my Grammy for the adventure. An extra set of hands sometimes is helpful when I'm alone, so I am looking forward to the help on this trip.

We do not have a return flight because our doctor couldn't tell us how long he plans on keeping Annabelle... he said she won't be able to come home until we learn, resolve and improve whatever is happening to her little body. My prayers are that we won't be gone more than a week, but heavens knows how long we will be living in the hospital during this time :( As eager as I already am to get back home to my family, I do know that the best for Annabelle is to stay in Cincinnati until she is comfortable and no longer in pain, regardless how long it takes.

From the bottom of my heart, I offer my apologies for the delay in an update.. I truly wanted to only share good news with our prayer warriors and family for Annabelle. My heart shatters to share this news, my heart sinks with every word of sadness in such a wonderful time of the year. Half our family celebrates the joy of the season as one of our daughters spends the weekend building snowmen with friends and Annabelle returns to a hospital to face her reality .. the reality of a 5 year old, Kindergarten little girl suffering, struggling in pain, unable to balance her body and flying an emergency flight to leave her family behind and head towards more pokes, surgeries, hospitals and procedures where they will inevitably break her spirit and remind her exposed and gentle soul that the world is cruel and unfair.
My . Heart . Shatters .
For all of us, me myself as her mommy, for her daddy that has to say goodbye to his favorite little 5yo, to her sissy that has to spend every night with a soul full of worry, my heart hurts for you that has to read these words and learn once again that all your prayers for this sweet little girl have again gone unanswered.

I trust God hears them, all our prayers; I do not believe he makes bad things happen, but he allows them to happen in order to teach us and guide us and shape us. I trust he loves my daughter more than I, but it's so hard to remember sometimes. I guess all we have left is to trust that everything will be ok in the end, in the meantime we love her as much as we can, we pray for her comfort and healing, I will advocate for her the best I know how and hope our medical team can quickly and swiftly begin to comfort her body and rid it of pain. I also pray you will continue to keep her in your prayers, keep my little family in your prayers for a safe flight, safe travels and protected hearts as we navigate very difficult waters ahead, while hundreds of miles apart. Thank you all, thank you for loving my little girl as much as we do..

Annabelle and her cousin snuggles

Wednesday, October 26, 2016

Biopsy Results are Back

I can't believe we had to wait so long for our results.. we've never had to wait longer than 3 days and this time we waited 8 days for the final result. Dr. Putnam wanted to make sure they were correct before sharing the findings with us.

Before we left Ohio, we met with the GI team. That Thursday we intended to discuss the biopsy's but they weren't back yet.. we stalled.. we talked, discussed hypothetical game plans, reviewed how Annabelle was doing overall and stalled some more with the Doctor. After a while, we decided that we probably were not going to be able to wait for the results and discussed two plans with Dr. Putnam.

Plan 1 - Good Report from Biopsy's
If by a miracle, the biopsy's are clear and she is in remission - we agreed Annabelle deserves a new food and could use more protein in her diet. We haven't been able to consider this for years because we weren't allowed to alter her diet or aggravate her system while we were exploring the genetic testing. Now that the muscle biopsy and DNA extraction is done, we are free to begin life again in managing her Eosinophilic Disease.

We talked about foods and which would be a good idea to trial. I asked questions like, "If she can have chicken, why can she not have eggs?" The answer, "They are very different types of protein and it's that protein that triggers the disease to activate. Eggs are a top offender of EoE and cannot be considered to be part of her diet for a long, long time, if ever." The more I asked about new food recommendations, the more Dr. Putnam would remind me, "I think you need to let Annabelle decide what she wants to eat". So, so very true. The thought and idea is so fun that she may be able to have a new food! I know she wants one so badly and I would love that for her. If we do choose to trial a new food, she will have to eat at least 4oz of that food, every single day, and then we scope again in 90 days to determine if it is a pass/safe food or a failed trial. So yes, Annabelle will get to choose but it does have to be a smart decision because whatever we add, she will have to consume it daily.

Plan 2 - Bad Report from Biopsy's
We didn't talk heavy about this scenario. If the biopsy's are still bad, Annabelle will likely loose the opportunity to eat and take anything orally again and we would go back to using drugs to help the disease heal. The idea of telling my daughter that she cannot eat anymore is devastating. We have been thru this once already and didn't feed her for 6 months.. it was torture on our family. It uprooted our entire lifestyle and changed our entire family routine. Holidays, celebrations, dinner and saying grace, movie theatres, birthday parties, EVERYTHING changed. While we know that it may come down to it again as Annabelle battles this disease for the rest of her life, we prayed with everything that it wasn't today.

We left Cincinnati last Thursday with a lot of information to think about. We left not knowing if we should be thinking and planning for the positive or negative. My mind was so happy at the idea of being able to tell Annabelle her boo-boo's were better and she can eat a new food.. I started thinking about new recipes to add to our short dinner list. I imagined Thanksgiving with a new item on her plate. I pictured her little face lighting up as she tasted something different for the first time. Then my mind would shift... it's equally likely that we will have to sit our confused, broken and faithful daughter down and tell her that she cannot eat any longer. She cannot partake in snack time at school, she'll be in the cafeteria at lunchtime without a meal. I decided in my head that we will not be celebrating Thanksgiving and we would do something fun instead. I listed all the things I need to rearrange, I will remove her snack bucket in the pantry and hide her personalized plates in the cabinet. How will we handle meals in a house of 7 people? How can I distract her for every single meal of the day? How do I explain to her broken heart that she isn't being punished but food just isn't safe for her. She doesn't know ANYONE that can't eat food.. how do I make her believe that she is just like everyone else when she globally isn't?! The anxiety ate us alive. The conversations were so hard.

Tuesday, October 25th at 12:34pm my phone rang, "Cincinnati Children's CCED" and my heart sank. I knew this was the call and I panicked. In fact, I hesitated for so long that I almost missed the call.

The words this nurse would speak will change our family monumentally... was I ready to hear it? No. I wasn't. Did I want to hear it? No. For an instant I panicked and my mind raced "I don't want to know... we will just keep doing what were doing.. the idea of good news isn't worth the risk of hearing bad news. We are happy with our limited diet and Annabelle isn't in too much pain. I don't want to hear anything ... I just want to pretend this conversation isn't happening..

Me: Hello...
Nurse: Hi Annabelle's mom! Dr. Putnam just called me and asked that I call you immediately. He's reviewed her results from pathology multiple times and we have news, are you ready?"
Me: (in my mind, NO! DIDNT YOU JUST HEAR ME?! NOOOOO!) ..... Yes, I'm ready. But I only want to hear good news.
Nurse: :laughing: well I can do that for you. Mrs. Bishop, Annabelle is in remission. The biopsy was perfect and showed zero signs of the active disease. It hasn't spread, it hasn't manifested. Your daughter is healthy from an Eosinophilic Gastrointestinal perspective.

I cried over the phone and then we went back to our previously discussed game plan.. a food trial. I asked what she recommend and she told me what Dr. Putnam has asked.
(side note, Annabelle's 'food' of choice is to have pepperoni pizza. that's her choice when you ask her.... and that's not possible, that would be a trial of beef, pork, milk, wheat and multiple spices... we can only choose ONE thing to trial and a pizza is just not logical lol).

I asked if she could try milk like cheese, yogurt, sour cream, ice cream etc. Unfortunately milk has such a high failure rate and isn't recommended.. even more, Dr. Putnam won't allow her to try cheese/ice cream etc until she trials 90 days of white cows milk ONLY and can pass via biopsy. Afterwards, she can try the rest of milk based products but again, he doesn't want us to consider it at all.

His recommendation is to trial pork, and for logical reasons.
  1. It's another form of protein, in which we currently only have one (chicken) and she does need another.
  2. It's another meat... I am so tired of chicken
  3. It has a high success rate
  4. We get a lot of food options out of pork:
    1. Tenderloin
    2. BACON
    3. Ham
    4. Sausage
    5. Pork chops
We could also trial peanuts because she does not have an allergy. Peanuts will give her a source of protein.

Through conversation with the nurse, we decided the best idea is truly to consider pork as a trial. I love the idea that it provides a high success rate for passing the trial and also that it opens up a LOT of meal options, breakfast, lunch and dinner for Annabelle. Our meal list of choices are quite limited in our house and I am eager to be able to cook more; pork will provide that opportunity.

After I hung up the phone, I walked to the bathroom and dried my eyes.. when I got back to my desk, I texted AK and told him the good news.. my mind was racing and I just couldn't wait for the day to hurry and finish so I could go home and tell Annabelle! I went to the store and purchased a card for her and wrote her a letter to tell her how proud we were of her and congratulate her on gaining remission of this disease.

As soon as we got home, I made the girls sit down and told them that we had news to share from Dr. Putnam.

Our daughter is in remission. Remission. Oh my God, her little body is healed of the damage brought on by this awful disease. She is not cured, she is still very much suffering from an auto-immune disorder and this moment of happiness could be very short lived. But for our family, this is the best we could have ever hoped for. Our prayers are for comfort, less pain and zero progression of the disease in her body. Our prayers were answered. This celebration is purely a gain of quality of life for our daughter. I am so proud of her, without her commitment to an extremely limited strict diet and persistent prayers, we could have never gotten here. Annabelle is the one that deserves all the credit to this victory. She wears a face mask when germs are around.. without a fight. She washes her hands until they're raw.. without question. She showers daily after school before she's allowed to play.. willingly. She never sneaks food anymore. She rarely cries when she learns she cannot eat what others are.. She doesn't expect birthday cake or shared celebrations with her friends. She embraces a unique bland meal at the dinner table daily. She understands she cannot 'buy lunch' at school like her friends. She doesn't put anything in her mouth she isn't supposed to. She takes her medicine without fight and she never gives up on her faith. Annabelle earned this victory.

Please know, we are so happy to celebrate this wonderful news but Annabelle still has a long, long way to go before she is healthy. This news is completely different from her genetic testing and global diagnosis that will ultimately help us save her. This news is only 1/10 of her battles, but it's 1/10 of her battle that we are winning!

Mommy, do we have any ham here at home? Can I have some now???
Yes, baby. Absolutely you can.

Thursday, October 20, 2016

We handed our baby to an anesthesiologist for the 27th time..

We never, in a million years thought we would be here. At most, we imagined our marriage would suffer the typical strains.. finances, bickering disagreements about what to eat for dinner, not seeing eye to eye on disciplining our children, perhaps job loss etc. But never, did we ever imagine that each and every one of our 'family vacations' would be held in Cincinnati Ohio as we await the moment where we hand our daughter over to an anesthesiologist for the 27th time in her life. Never could I imagine the debt our family would carry, the heavy, grieving, frustrated and broken heart I would wear on my sleeve and the strength this nightmare would offer our family as we stick together.

But as life has it, and as funny as God thinks he is in his ways... here we are.

I didn't sleep a wink. At most I may have caught an hour-ish, but it wasn't restful. Every single worst case scenario of the OR would run thru my mind, and as I watched them play out in my head, my prayers would scream louder, "PLEASE GOD! PLEASE PROTECT MY BABY!" I prayed that the sun would finally appear so we could end the loneliness and quiet of the night.

The girls were awake at usual time, 6ish and I quickly did their hair and began to get them dressed. Mady was hungry and I kept having to remind her to stay quiet as Annabelle couldn't eat so no one would be eating that morning (not in front of her at least). Annabelle knew what was happening, she knew this was surgery day and she was so nervous. She randomly pleaded out "Mommy, can we go home, do we have to have surgery today"...she knew the answer but bless her heart, she wanted to try. When I would explain what's happening, she would cry or pout, but Mady was quick to jump in line to offer a distraction.

Traffic in Cincinnati is horrible. And since we switched hotels, we are about 30min outside of the city, NOT including Monday morning rush-hour. Needless to say, there wasn't enough coffee to keep my blood pressure from skyrocketing after the day we were already beginning to have.

AK parked the car with Mady and met us upstairs (they also took advantage of this time to eat their breakfast away from Belle.) Once upstairs, we did our standard vitals and Belle clung tight to this trips favorite stuffed animal - "Tubey Puppy", he's a doggie that we put a G-tube / Feeding Tube into his belly just like Annabelle's. She loves him.

As we waited for daddy, sissy and/or the doctors to join us in our room for our Genetics consult, Belle hit her fight or flight moment, she pleaded to go home, she begged 'I will be a good girl mommy and use alllll my manners. I promise. I won't have accidents in my undies anymore, please mommy, please can we go home, I don't want to be in the hospital anymore". Her words are still ringing in my head and stabbing me in the chest. I tried so hard to explain to her that she was NOT being punished, that her accidents are at absolutely zero fault of her and if I didn't think this would make her boo-boo's better, than I would surely get us home super fast. But I know these procedures are going to make her feel so much better...... convincing a nervous 5 year old that the long-term benefit is worth the sacrifice of her short-term/now fears = impossible.
I held her tight and fought back the tears...

Daddy and sissy met us in our room before Dr. Burrows, our Human Geneticist, joined us. When he did... things suddenly took a turn for the worst.

"I am so very sorry Mr. and Mrs. Bishop, but insurance is repeatedly denying any testing for Annabelle... we can't do the testing today"
And just like that, our #1 goal for this entire trip was stripped from us and shattered.

Dr. Burrows could see the pain tearing across my face and he tried to talk thru multiple other options that we may have. We spent the next solid hour trying to figure out how to make the genetic testing happen... we came up with two plans:

  1. If Annabelle is admitted after surgery, we will do the DNA extraction at that time and order the testing thru the hospital, coding that it's necessary for her medical situation.
  2. If she isn't admitted, we would draw labs and hold the bloods until we can get a preapproval from insurance, and we would then send the blood to an outside vendor that does genetic testing.
Neither of those options are ideal but they were the best options available to us at the time. I agreed to call Dr. Burrows once Belle was out of surgery and we would determine then what we would do. (remember, we were not sure if she was going to be admitted afterwards or not.. it was all based on how well she did in recovery. Our goal was to be discharged, but now with the genetic testing issue, we were ok with having to stay if it were necessary.)

The rest of our conversations were around the genetic testing itself and the decisions we needed to make for it. There was such a great deal of information, so here are the highlights that we spent a lot of time discussing:

What the chances are that we will get answers, even if it means we don't get our final, global diagnosis, would we at least learn something? The very unfortunate and sad answer was, no. There's only about a 25-35% chance we will get Annabelle's answer from this testing.. while the statistic is low, its 25% higher than it was years ago. That being said... Exome Genetic Testing is the most extensive option available now to anyone. In another 5 years, we will be able to perform Whole Genome Sequencing which will provide even more answers, but for now the technology is simply not there.

This 25-35% chance does improve though when we compare Annabelle's genetics to her parents. When studying her genes, they are looking for any changes that make her different from other individuals - when they compare that specific change to us, mom and dad, they will be able to determine if that 'change' simply makes her a unique reflection of mom and dad or if that change is actually impacting her health.

By comparing Annabelle's information to ours, we will learn about inheritance patterns, whether or not we passed this condition down to Annabelle, and if so we will learn specifically which parent passed it. It will also tell us whether or not it has the potential to affect any of our other children.

When being part of Exome Genetic Testing, we are also offered the option of the 56 Gene Screening that discloses whether or not you carry the genes associated with cancers, adult onset chronic diseases, cardiac disease etc. This information is not necessarily what we are looking for to diagnose Annabelle, but if the option to know whether or not in the future if she is predisposed to some of these diseases, we would like to know. Unfortunately, if we choose to learn this information about Annabelle, since they are comparing our genetics to hers, we will also in result learn this information about us. The downside to learning this information, is that it has the potential to make us uneligible for Life and Long Term Care or Disability Insurance. We cannot be denied health insurance of course, but because we've now had genetic testing, we can be denied the other.

Because there are so many advantages and disadvantages, Annabelle's a minor, and were essentially playing God in some ways of how much information we may potentially learn, AK and I have to be 100% on the same page in order to perform any testing on Annabelle. We decided we were ready. We are ready to learn everything we need to hear to help our daughter... the good, bad and ugly. In another situation I would be making a very different decision, but where our life is now and the necessity of how urgently Annabelle needs answers, we are ready. No questions asked. We will continue to be coached and will have a team explain the results to us when they come in.... I am nervous about those, I am scared to death to hear the words "Annabelle has x,y,z and it was passed down from the mother.." etc. but who knows. I have several months to get my head where I need it to hear whatever is in store :)

Our biggest challenge is going to be insurance. However if we continue to be denied, we have the option to generate a Letter of Medical Necessity because more than one body system of Annabelle's is being affected. Insurance unfortunately doesn't care and will not pay for the testing just so we can have answers.. they will only pay if we can prove that it will change her medical management (aka more money.. if a diagnosis means she may need a different drug, elaborate wheelchair, etc = medical company and insurances begin to make even more money off Annabelle which in result, they support the idea. It's a sad, corrupt medical world that simply makes me sick).

Bottom Line that we were strongly reminded, multiple times before we left the visit:
  • If we find an answer, we may not have a treatment.
  • We can't change genetics but we can alter the way our body reads them to better her body function.
  • There's a higher chance we learn nothing than learn something; it doesn't mean that what Annabelle has isn't genetic, it's just that were not there yet medically to diagnosis it.

After our genetics appointment, we made our way to admitting and Same Day Surgery. Mady stuck by her sissy's side every step of the way.

Belle was so nervous but the nurses at Cincinnati Children's are priceless. They treated Tubey Puppy as another patient the entire time..

Both Annabelle and Tubey Puppy got their arm bands. Annabelle always has a second red band to indicate all her allergies.. Tubey Puppy had one too that read "Allergic to Chocolate"... bc.. he's a dog.

Once we were settled into our room, the team began to meet us before the procedure.. this is when the doctors, nurses, surgeons, anesthesiologists etc will come to discuss and confirm one last time our game plan. These conversations are critical and typically where I will sit on the opposite side of the room from my family so the doctors can have my full undivided attention and I can assure everything will go according to plan..

1st problem: They had the wrong GI surgeon scheduled for the OR with Annabelle. I immediately stopped our movement and demanded an explanation. I was under the impression that Dr. Kaul would be performing the surgery but apparently, they scheduled one of his colleagues instead. I wasn't happy, mostly because of the sudden change of plans. but I agreed that I would allow him to do it but not until I meet with him personally first and we talk. That's my baby you're operating on, I want a face to face conversation on what you're about to do! And thank goodness for it, because when he came to our room, he was under a completely different understanding about the surgery than what was discussed. He had different intentions in the OR than what we talked about with Dr. Kaul on Friday. I made him call Dr. Kaul in front of me so we could all three be on the same page.  . . . 45min later, everything was settled and we were back where we should have been - same plan as before, we would be doing everything just like we discussed last week and the new surgeon was brought up to speed on our expectations. My heart was in my throat at the potential of missing this - what if I were the type of mom that doesn't question everything? what if I were passive and allowed doctors tell ME what they're going to do instead of asking questions? What if Annabelle didn't have an advocate that would speak on her behalf? I am not, NOT giving myself a pat on the back by ANY means, bc this is my child and goodness I don't need a kudos for being her mom - but I am simply explaining the horror and fear that I do have if Annabelle didn't have someone to speak for her. What about ALL those kids and people that don't have their voice in the medical world and don't have someone to speak for them? My heart aches and stomach twists at the thought of what happens to them.. how quickly you can become lost, mistreated, mistaken, overlooked and accidents could happen. We are all people, but just because doctors and surgeons make over six figures a year doesn't mean they don't make mistakes, they're humans. And it's our job to be a solid second set of eyes, especially when it's your baby under the knife.
:stepping off soap box:

Once the GI surgeon was finished and we were all clear on our game plan, he left the room with a team of other nurses as they had to re-prepare the OR to accommodate all the changes in the plans. That's when we met the general surgeon who would be doing the muscle biopsy. I thought she would be removing only muscle, but actually, she removes skin, tissue AND muscle all the way from the surface into the muscle of her leg. She marked where she would take the sample and discussed what recovery would look like. After the general surgeon was finished and left, it was time our nurses cleans the area :)

Because Annabelle was now going into a specialized portion of the OR for this particular multi-part surgery, mommy had to suit up to be able to walk her to the back. Mady and I wanted to take selfies... but Belle was simply too nervous to care to partake..

Before we knew it, the time was nearing where we would have to say our "See you later's.." Mommy's heart sinks, Daddy wears a brave face and Madelynne tries to stay strong and determine her opinion based on our reactions. There were so many nurses and doctors in and out of our room, the distraction was welcome but it was overwhelming for all of us.

Wheeling down the hallway to the OR.

In the OR, Annabelle made it very clear to her team that Tubey Puppy was the one in their to have surgery, not her. He wanted Strawberry flavored gas, just like her, and he was nervous about his IV so be very gentle with him.

She had the OR in stitches of laughter :)

Strawberry flavored gas.

If you've never been in the OR and seen your child put under anesthesia, I am here to tell you it is one of the most heart wrenching, painful things to ever watch. My stomach turns at the sight and thought of what's happening to her as I see her little weary eyes trace the room and the life within her die away.. AK has only seen her being put under anesthesia once and he told me immediately afterwards that he never wants to see it again. You should never see your baby in that condition.... it leaves a hole inside you fearing whether or not that was the last time you will see them conscious again, last time you'll see their eyes again, and they're glazed over and lifeless. It's awful.

Annabelle however, oh my goodness, she handles it so beautifully at this point, that I am in total awe each time. She used to fight anesthesia and the process and now she holds the mask herself until we lay her little empty body down to her back and her eyes fall back. Sweet, sweet baby..

We waited for 3 hours in the OR waiting room. Fortunately the time passed quickly because as each of our surgeons was complete their portion of the procedure, the would meet with us individually in a consult room to discuss how things went, so every hour or so, we were able to talk with a doctor.

The first was the GI surgeon, he explained everything that he did and how well the procedures went, where he injected botox and the results we should see. I asked about the placement of her G-tube and whether or not that was still aligning correctly, unfortunately as Annabelle is getting older and growing, the location her tube was placed at 2 years old is starting to shift now at 5 years old and her stomach inside her body isn't quite aligning with the hole on the outside of her body. Therefore when we insert the tube, it's leaning quite considerably. With time we will have to move her g-tube location and surgically find another hole that will continue to grow with her body..

The general surgeon was next. The muscle biopsy went well and took about 40min to perform. Mady and I had a bet on how many stitches she would have as a result, I guessed 5 and Madelynne guessed 8.  Mady was right :) 8 stitches.

After hours of waiting, it was finally time that I got to go see Annabelle in the PACU. Daddy couldn't go because Madelynne isn't allowed in the back so they had to wait in the waiting room. When Belle woke initially, she was frantic and thrashing around but fortunately fell right back to sleep. She laid there so peacefully. I try not to touch or wake her because I prefer she wakes up on her own like a good nights sleep, but it's so hard watching her take her time.. my mommy heart is impatient to see her pretty brown eyes again.

Look at Tubey Puppy! Bless his heart, he had an IV and was also in recovery, he must have had a tough surgery back there..

Because Belle did so well, they choose not to admit her afterwards (thank heavens). We spoke with the Geneticist and he put in the orders to draw the blood before Annabelle's IV was removed in the PACU. They called two extra nurses and while my sweet baby was asleep, they did the draw. I can't lie, I dropped a tear or two five watching them extract the blood. I prayed the entire time so heavy that this test would provide my baby with answers and the help she needs. This was the blood that has the potential to decide so much!

The next day, Tuesday, Annabelle woke early and was excited to go see Noah's Ark as promised. We had hesitation on whether or not she would physically be ok to go but she seemed happy and full of energy to do it. We headed that way and unfortunately shortly after noon, I think the drugs from surgery began to wear off and her pain kicked in. Her leg was hurting her so badly. She couldn't eat or drink without her throat hurting her and her stomach was a mess. I gave her all the Zofran I brought from home and never her lapse of Ibuprofen and Tylenol but it wasn't seeming to provide her much relief.. she wanted to go home... so back to the hotel we went. She told us so many times during the day, "This is a really really bad day for me... this is my worstest day EVER". Belle rarely, RARELY ever complains of pain, so to hear her cry and see her physically hurting was torture. We took her back to the hotel and found the Disney Jr channel where she made her bed and we watched TV for the rest of the evening.
Sweet Baby