UPDATE:
This blog was initially created following Annabelle's first diagnosis of Eosinophilic Esophaghitis. Since then, her health has deteriorated and led us on the hardest road in search of the true answer for Annabelle's condition. Please keep this amazing little girl in your prayers and thank you for following her story.
The summer for the Bishop family has been full of many highs and lows. We were been flooded with love from all over during our Answers for Annabelle Superhero Fun Run. It was such a blessing to see so many people come out to high five, run, play and spend the day with Annabelle. Undoubtedly one of our best memories as a family.
August 23rd, Annabelle became a Big Sister to Juliette Catherine. Belle is moving into her role with so much excitement. And although Juliette still isn't home with us, we know she will fit right into our family perfectly as soon as she is.
We've had a few summer getaways, we've spent many late summer nights with friends, and inevitably school has come around again and we've begun 1st Grade!!
Though the blessings and distractions have filled our days as a family, we haven't been able to avoid the elephant that lingers among our home.. today marks 94 days since Annabelle ate a meal, enjoyed dinner around the table, celebrated her friends birthday by enjoying her own safe version of a cupcake. Today marks ninety-four awful damn days that our family has fought literally every. single. meal. around. Not one single day has been easier than the last. Not one single day went without tears. Not one single day has passed where it didn't break our little girl down into a depression like we've never seen before. Annabelle's tiny soul has been shattered during the last 94 days, and I have no idea how long it will take to repair.
I usually update and post often, at least I try. But I have found that during our families hardest time, when we don't quite know how to respond or put our next steps in front of the other.. it's hard to update. That's kinda where we've been. I hate sharing bad news, I want to share celebrations with you all, but honestly, this summer has been excruciating for us. We have exhausted ourselves as parents to distract Annabelle any and every way we know how. Poor Madelynne hides to eat her meals, we avoid cooking indoors whenever possible, heck, I hardly even cook anymore! We surely don't go to restaurants if we can help it and now that school is back, Annabelle sits in the classroom alone while her class goes to the cafeteria for lunch - her choice - she said she can't see other people eating. I know it would just crush her.
So here we are.
Tomorrow, Friday September 15th and 6:30am, Annabelle will undergo her 30th surgery to determine whether or not her Eosinophilic Disease is still active or if it's healed. Our doctor will assess the motility in her stomach and how it empties to see if that portion has healed enough that perhaps she can digest foods thru her actual stomach again. For the last 94 days, we have bypassed her failing upper GI, including her esophagus and stomach, and have been feeding Annabelle thru her small intestines via GJ-Tube 20hrs x day. Our prayers are heavy that her body has healed itself enough we can safely re-introduce foods back into her diet.
Because Madelynne is in school, and Juliette is still in the NICU - Daddy took Annabelle alone to Cincinnati, OH for this trip. My momma heart is shattering! This is the very first time I have been away from Annabelle.... ever maybe(?) This is definitely the very first time I am not there for a medical visit, let alone a surgery! I am a nervous wreck. As I packed her last bags and strapped her into the car this morning for her and daddy to drive away, my heart sank... they pulled away and the tears fell. AK knows the drill, he knows what to do and he will communicate all my questions and concerns to Annabelle's medical team (or else!) - I fully trust and know just how luck Annabelle is to have two parents equally involved in all her needs.. but that still doesn't appease the mommy heart, does it?
I promise to update tomorrow night after her procedure. Please say a prayer if you could that Annabelle is able to get food back and we as a family can return to finding a bit of normalcy in the midst of daily chaos.Thank you always for understanding, thank you even more for sticking by us as we have silently weathered a rough storm as a family this summer.. we love you guys immensely and your prayers are felt, I promise.
It simply means you were stronger than whatever tried to hurt you."
Our family is scarred. We have been wounded emotionally, physically, financially, our faith has taken it's toll, our friendships and family have been redefined, our own family dynamic has been forever changed by the beast that is hurting Annabelle.
We are not fearful of these scars, rather learning to adjust and readjust, sometimes by the hour. Our family is and will forever be scarred by what we are going thru, this journey is shaping and building us, and while we are grateful for the lessons, we are scarred. Some people call them blessings, but right now, they're scars from wounds we never could have imagined.
It's been 13 days since she underwent a very unplanned, unscheduled, emergency surgery in Cincinnati, Ohio. A routine visit that was intended to be only a discussion quickly escalated out of hand and landed our entire family on the edge of our seat, praying hour by hour about what would happen next. Daddy was home with Madelynne and had projects going on at work; we considered flying him to Ohio to be with us but for a flight ticket of $500, it wasn't feasible. We were in limbo with hotels as we didn't have reservations to stay longer.. and I was running out of medical supply. We managed though, lots of phone calls and extra prayers, we made it to Tuesday morning and checked in to the Surgery Center at 8am.
Annabelle was very nervous for surgery this time, I believe because she wasn't prepared. She asks a lot of questions about her upcoming surgeries and we always tell her the truth and to the level of detail she will understand. This surgery however, I had to explain they would be giving her a different type of tubey and when she wakes up, it will be sore. She knew her throat and tummy would hurt as it always does after an endoscopy but this procedure added another dimension.. one she couldn't understand and didn't particularly care for. This brought a lot of anxiety... and to be honest, to the both of us.
Surgery #29 was not nearly as smooth as our last several have been. We follow an extremely specific anesthesia plan that we've been using for years, but this time a few things were different: she didn't have an IV to deliver versed, so instead we gave her liquid versed via g-tube which gave a different effect. She was also quite nervous and full of anxiety, so once the drugs kicked in, she became panicked. That's the worst way to go under anesthesia :( it usually means recovery would be hard.. and it was.
While she was being moved from the OR, our doctor met with me in a consult room to discuss what he found during surgery. The Esophaghitis was still very active :( we were both absolutely shocked as we genuinely expected it to be healed by now. It had been 5 months since ending our pork trial and her body still was not healing. He also shared how the GJ-tube placement went and explained Annnabelle had excellent anatomy for the tube and we shouldn't experience many problems with the placement (thank goodness for some good news!)
Shortly after speaking with Dr. Putnam, I was allowed to join Annabelle in the PACU.
That walk is always hard.. I follow the bumble bee's on the floor that lead me to bed #14 and with each passing bumble bee, I could hear a disoriented cry - a mother knows her child's cry but after surgery, anesthesia and intubation, it sounds so different. I pray it isn't my daughter. My pace increases and the sound becomes more and more familiar as I turned the last corner.... it is Annabelle :( My heart dropped and I raced to her bedside.
If you haven't experienced being with your child as they're emerging from anesthesia, it is one of the most heart-wrenching things to watch. They are confused, disoriented, absolutely terrified and hurting - but they don't know who you are, they don't recognize your voice and you can't comfort them. You just pray the minutes tick by faster but then the seconds turn into hours. I sing our song that the girls get sung every morning:
Good Morning, Good Morning
Good Morning to you
I've waited all night
Just to see you
So Good Morning Annabelle
Good Morning to you
It took Annabelle about 40 minutes from the time I got to her bedside until she began to recognize who I was and she calmed down. Fortunately, she did not seem to be in much pain other than being sore, though she was extremely weak. Our doctor released us directly from PACU to go back to the hotel and rest for the day. We checked into a hotel near the hospital and spent the evening watching American Ninja Warrior and napping.
We stayed in Cincinnati until biopsy results were back from Pathology on Thursday. In the meantime, we had a wheelchair appointment to have the chair adjusted and found a few things to do in the city - like visiting the Cincinnati Zoo :)
Annabelle was tolerating her J-tube feeds very well. I didn't know what our goal rates were or much at all about the new tubes so for a couple days, her and I both just winged it together. We flushed the tubing, hooked up and ran at a low rate and slowly increased every several hours until she was no longer hungry. I kept her G-tube (stomach) vented and by day 3, she didn't appear to be bleeding from her stomach any longer.. she was healing and we were so grateful.
Thursday we packed our car to go home, but before hitting the road we made our last stop at the hospital to visit Dr. Putnam where we would hear what was happening, what our plans were and what the future holds. My stomach sank and daddy held his breath from Richmond and watched his phone until I gave him the update. The world froze for the next couple hours and as rocky as the ground was below our feet already, somehow an earthquake hit our family and de-railed us forever.
This is hard to write .. this is the sole reason I have been avoiding this update :'(
...
Secret Revealed: We agreed to move forward and pay for Genetic Testing weeks ago. Our decision to move forward was made when we committed to the Superhero Fund-raiser for July 15th. Our prayers are heavy that this event will help pay
for the testing.. as a mommy and daddy, our conscious and hearts couldn't go
another day without knowing what was happening to our daughter.
Genetic Testing is back and has found two conditions:
MCAD Metabolic Disorder
"Condition that prevents the body from converting certain fats to energy, particularly during periods without food (fasting).
Signs and symptoms of MCAD deficiency
typically appear during infancy or early childhood and can include
vomiting, lack of energy (lethargy), and low blood sugar (hypoglycemia).
People with MCAD deficiency
are at risk of serious complications such as seizures, breathing
difficulties, liver problems, brain damage, coma, and sudden death."
Mitochondrial Disease"Mitochondrial diseases result from failures of the mitochondria,
specialized compartments present in every cell of the body (except red
blood cells).
Mitochondria are responsible for creating more than 90% of the energy
needed by the body to sustain life and support organ function. When
they fail, less and less energy is generated within the cell. Cell
injury and even cell death follow. If this process is repeated
throughout the body, whole organ systems begin to fail. The parts of the body, such as the heart, brain, muscles and lungs,
requiring the greatest amounts of energy are the most affected.
Mitochondrial disease is difficult to diagnose, because it affects each
individual differently. Symptoms can include seizures, strokes,
developmental delays, inability to walk, talk, see, and digest food
combined with a host of other complications. If three or more organ
systems are involved, mitochondrial disease should be suspected."
AK and I have heard both of these disorders in conversation with doctors for years. Although our prayers have always been heavy that we wouldn't hear this news, our prayers have also been "Just give us answers, please God, just tell us what this is so we know how to help our daughter." Nothing though prepares you for when you hear those words.
We did also opt to find out another detailed level of information. When we met with the Geneticist, they asked if we would like to back-up any findings to the parents genetic DNA. Meaning, if they were to find something in Annabelle's Genetic DNA - we would cross reference those findings to both AK and I to ensure we have a clear diagnosis.
Just as hard and fast as the news was to absorb that my little girl was fighting two life-threatening diseases, I also learned that Annabelle had inherited both from her maternal side. Me. Her momma. The loving, life-giving person that has been by her side for twenty-nine surgeries, held her down for hundreds of needle sticks, prayed on my knees for years - I now hold the knowledge that all my daughters pain and suffering was inherited from me. ... I will pause to simply say, there are no words. There are no words that AK can generate, there are no words the doctor offered nor is there anything that I think I am ready to hear that will provide comfort in bearing this news...
Cincinnati Children's has a Human Genetic doctor that specializes in the specific variation of Mitochondrial Disease her Genetic Testing revealed. We will meet with that doctor August 23rd to discuss the disease and it's progression in more detail. At this time, we are aggressively trying to control and slow it's manifestation.
With the symptoms we know Annabelle is presenting already, she appears to be in Stage 2. The progression and her symptoms remained fairly consistent thru infancy but during the last year they have begun to shift and change. Here are the areas that are suffering now (using the chart above as an easy reference):
Nervous System - Seizures. Movement disorders. Developmental Delays. Poor balance/coordination (Annabelle began having seizures around 2yrs old, they stopped for a few years and have now returned. Her Neuro-Psych doctor has diagnosed her since an infant with multiple developmental (both physical and cognitive) delays - she's spent a lot of time in therapy and has improved.)
Skeletal Muscles - Muscle weakness, exercise intolerance, pain, fatigue, low muscle tone. (This was confirmed with our Neuro-Muscular doctor who we have been seeing for years as Annabelle's lower body has succumbed to the exercise intolerance / fatigue / weakness and pain. With a suspected diagnosis of Mitochondrial Disease and supporting muscle biopsy all her symptoms landed us with the wheelchair that we now use daily.)
Liver - Liver failure. She has been in this category since 2 years old when we removed protein from her diet as much as possible in an effort to stop stressing her liver. Her liver does not filter the bi-product of protein (ammonia) out thru her urine like it should. Instead, Annabelle's liver filters it directly back into her blood stream. High amount of protein = high amounts of ammonia = ammonia in her blood stream. First stop: brain = Seizures.
Kidneys& Endocrine - Beside the inability to balance her internal body temperature, these systems are not currently affected.
Digestive Track - I don't even need to list the GI trouble she has, you know that story!
Heart - Beginning January 17', her heart began to show signs of trouble. Each and every time her body or system is stressed, her blood pressure drops and takes days/weeks to improve. We have never seen cardiac problems with Annabelle until recently.
Eyes - Drooping eyelids. At this time only her left eyelid droops. It has since she was a few years old. At first we thought it was the result of a seizure, but it's now better explained by Mito. She's also always had an excellent bill of health with the optometrist until her most recent check-up when she failed her vision tests. We are waiting now to have her assessed again.
Ears - Currently no symptoms
Our Plan
With the understanding of both Metabolic and Mitochondrial Diseases, Annabelle's doctor was able to formulate the best plan of care for Belle moving forward.
In order to keep her body functioning as smooth and effortlessly as
possible - we can never allow her to fast and we need to keep her well
hydrated. Thus resulting in continuous J-Tube Feedings. Ideally we would run Annabelle on a slow feed for 23hrs x day. That limits so much of her quality of life.
I looked down at Annabelle who was playing on my phone (watching YouTube videos of Baby Alive, if I am being honest), and I just couldn't imagine her being hooked to a pump for the rest of her life. Perhaps one day we will be there, but as her mom, I needed to be her advocate right now. I asked if there was any room for negotiation and we agreed to 20hrs x day, absolutely no less. That would give Annabelle 4 hours every day to unhook from her pump and climb trees, play, run, ride her bike, swim in a pool etc. We agreed to split that time, 2 hours in the morning and 2 hours in the evening.
We also needed to allow her body to heal from the Esophaghitis that was still aggressive and active. The bulk of the damage was at the bottom of her esophagus which tells us reflux (despite her stomach being wrapped tightly around her esophagus to AVOID reflux) was still quite active. Her stomach itself was not working properly. It was dumping food heavy and quickly, not allowing her body to absorb nutrients and causing her to loose weight.
We knew the top portion of her GI was failing, and know the bottom portion is failing, but now we've lost her stomach too :( The decision was made to stop feeding Annabelle ANYTHING by mouth, put absolutely nothing into her stomach and only provide feeds thru her intestines from now on.
We will follow back with our team in September to perform Surgery #30, biopsy again, change her GJ-Tube since it has to be done in the OR and with Radiology. The doctors will be able to assess her progression again at that time. Meanwhile, we will meet with the Human Genetics doctor in August and begin to wrap our family and our understanding around these new Genetic findings.
We left the hospital numb. Annabelle kept asking "What did he say mommy? Are my boo-boo's better? Are we going home now?" I was speechless. I was absolutely, 110% broken inside. I picked Annabelle up and while pushing her wheelchair with one hand, I carried her in my arms to be closer to her. She laid her head on my shoulder and said over and over "It's okay mommy, I'm okay!" I silently let tears fall from my cheeks. The walk out of that hospital was silent. The moms and dads I passed down each hallway offered the most compassionate solemn head-nod - every parent in that hospital is walking a path they never intended and cuts deep. Today was the day we stepped foot onto a different path... and they understood. No judgements, no words needed, a simple head nod of acknowledgement from one grieving parent to another that said "I know...... I know..."
We got into our car in the parking lot and I kissed Annabelle over and over and told her how much I loved her and how proud I was of her. We buckled in and I ensured her carseat was harnessed super snug and safe for our trip home. Once we had our movie (Trolls) playing on the TV, I selected "Go Home" on our GPS and exited the parking garage. As soon as I was able to breathe and clear my thoughts, I made sure Annabelle was occupied by the TV with headphones and called AK. That was one of the hardest conversations we have ever shared, and I wish more than anything it didn't have to be over the phone.
We drove almost two hours before I realized I never turned the radio on and the car was silent. Peeked back to Belle and she was happily watching her movies.
Another hour we stopped for gas and potty. I grabbed a water from our cooler, got back on the highway and again considered turning the radio on but couldn't bring myself to decide what type of music I wanted to hear.. so I left it off. Even today, I avoid the radio. Classic Rock is my favorite, but every song has a memory "before we got our diagnosis". Pop music is too happy.. I don't want to be happy, I am furious. Country music will make me even more depressed and Contemporary Christian, which is what we typically listen to, simply felt like a salt in my wounds. I was and am still so broken and bitter, our faith is being tested and while I know and still trust God to care for my little girl - I am heartbroken. I still don't want to listen to the radio.
After driving for several hours, Annabelle was asleep. We passed Charleston WV, our half-way point, Belle was still snoozing so I kept driving. Before we knew it we were in Beckley, WV and I asked Annabelle if she was ready to stop and get a hotel for the night. She begged and pleaded to go home. I've never driven from Cincinnati, Ohio to Richmond, Va straight & on my ownbut that night.. in my numb state and a motivated Belle to see sissy - we did it. We arrived home shortly after 10:30pm and met daddy in the driveway. He immediately picked up his baby girl and loved her far too long as I was next in line waiting for that same embrace. Belle was excited to be able to see her sleeping sissy, so AK and I both fought back our tears together and joined Annabelle to wake sissy and tell her she was home.
We've never slept better to be back with our family. We've also never slept worse knowing what is happening to our family. I think that night was the most solid, non-restful sleep I have ever gotten. By 4am AK and I were both awake and crying.
It's been a few weeks since then. And our days, while they are not easy by any stretch of the imagination, they are etching their way into a routine of the Bishop house. We are equipped again with IV poles both upstairs and downstairs. We have medical supplies from one stretch of the house to the other. Annabelle is becoming a champion at hooking and unhooking her own feeding tubes. We have mastered how to clean around her stoma without pain and it is healing quite nicely. The best news is that Annabelle feels GREAT. I am not kidding. She feels and looks better than I have seen her in years, YEARS. She is rested and sleeping 10hrs every night. She wakes and is not grouchy or exhausted. Beside the daily / sometimes hourly begging and crying to have food, she is cheerful.
We have not told either of the girls her diagnosis, for all they know "we still don't know what is wrong". If you've read this blog before, you know that our philosophy is that we never lie to our girls and we tell them the honest truth about every step. We are just at a loss for our own words right now.
We can't bring ourselves most times to tell family and friends what is happening, we don't know the right way to explain a lot of the unknown future.. when we find the right words, when we find the right timing and when we think the girls are prepared to hear - we will sit them down and explain. But in the meantime, the monumental changes in our home and family dynamic are enough to process. Not allowing our 5 year old to potentially never be allowed to eat food again, forcing our 8 year old to not mention or eat in front of her sister, hiding in closets or eating on the porch after bedtime is enough for us to try to get used to.
We are spending our days finding the blessings in everything. We push Annabelle to explore and see the light of life around her despite the confinement of being in the house most the day and tethered to pumps. We have gotten out of the house and cheered Madelynne for our first Swim Team meet of the season - Annabelle also got a chance to cheer her little friends that were swimming too (we had to pull her off the team weeks ago. This should have also been Annabelle's first swim meet, but instead we watched from the side of the pool together).
Over the weekend we took the girls to Kings Dominion on behalf of Dominion Energy (my employer sends everyone for a 'Family Fun Day'). This was the first time Annabelle had to sit thru a meal and watch us eat but she could not. She handled it as well as can be expected. Beside unhooking/hooking from her pump to ride rides, she was able to be pushed in her chair and keep up with sissy the entire day. The only learning curve that AK and I need to figure out is how to keep her hydrated and cool while outdoors in the heat..
One last thing that I do want to share, is while our family is undergoing a living nightmare, we are also experiencing a miracle in the making. Our family is growing, and after losing three babies, we are happy to announce another little girl will be joining our family this October.
Juliette Bishop
With that being shared, there is a lot of work going on between my doctor and Annabelle's team to determine if Annabelle is a candidate for Juliette's cord blood and tissue. At this time, our GI team does not have a specific plan to use it but the genetic team may and we have been encouraged by all to collect Juliette's cord blood and tissue. Please say a prayer that these two sisters carry the potential to help one another. God works in mysterious ways and nothing is ever by chance, I believe in my heart this sweet girl was sent here to complete our family.
Annabelle has a fierce army behind her. I want each and every one of you to know how deeply we appreciate your love, support, kind words, encouragement and prayers. If you don't pray, we just ask that you love those around you a bit more today. Hug your babies, young or old. Pick your battles more diligently. Stop and breathe in the fresh air. Smile at a fellow parent that you pass, because we never know what another individual is carrying. Those of us that are strong have mastered how to hide our pain behind a smile, even when we are broken into pieces within. All these things I have to remind myself constantly - I have to remember to pick my battles, choose my fights, exhale when things are good, breathe when times are hard and cry in front of my kids when they are crumbling because we are ALL human and nothing in this life is promised. God intricately built us just the way we are and for a purpose, it's our gift to live it out. Meanwhile, we can help one another, love one another, support this little girl and together we can also help her fight a battle no one ever expects to fight.
July 15th, in Hanover VA at Pole Green Park, we are having a 5K Superhero Fun Run for Annabelle. This event is being hosted by a dear friend we met thru church and 100% of the proceeds go directly to the cost of Genetic Testing and Annabelle. The trips to Cincinnati, Ohio for treatment is overwhelming, the price we pay to have our family together when we can isn't always affordable but the cost for Genetic Testing and answers is detrimental to our family. (I am still waiting on the bill... we were quoted $14,000-19,000 depending on how far into detail they needed to explore.)
Please. Please, come out in July to support Annabelle. We talk every single day about the fun run and meeting new friends. She questions every night if God hears her prayers and I would love nothing more than to show her just how loved and supported she is by your attendance. Even if you do not want to run (I'm not running!!!), still come out! There will be plenty vendors, food, drinks, custom Annabelle t-shirts, bounce houses, games, raffles, live music and more. We want this to be a really fun family event and we look forward to seeing you and making it happen.
She's playing with a beach ball and making silly faces to me from in the pool. She's obsessed with mermaids and I think if it were possible, my fish of a daughter would actually grow a mermaid tail from how much time she spends in the pool over the summer.
But my sunglasses are hiding the tears that keep falling from my eyes like a slowly leaking faucet.
Why is this happening to our family? Why is this happening to such a happy, beautiful, strong little girl? Surely she deserves a break, God, surely you wouldn't do this to hurt her - but what can she possibly gain by losing the happiest parts of her life in addition to the hell she is already enduring?
This trip was intended to be informational and an opportunity to discuss Annabelles last admission in Richmond. We planned to see GI Motility, GI EoE and OT.
Points to discusss were:
GI Motility. Continued extremely loose stools that leaves her soiling herself multiple x day. Chronic pain, especially during fast feeds.
GI EoE: Followup to discuss the random ileus last month, why she took so long to recover and also why she isn't back to her baseline since. Our scope in January showed Annabelle had relapsed during the pork trial so we removed all pork from her diet and were scheduled to begin our next trial this spring.. Annabelle hasn't been doing well for months so we decided to cancel the food trial. Add the spontaneous ileus, pain during eating AND bolus feeds, extreme fatigue and weakness, unstable BP etc and we had quite an appointment planned.
OT Wheelchair Clinic: it's been a year since we receiving Annabelles wheelchair so we're due time to make some adjustments according to her height growth over the year.
Annabelle and I hit the road together Saturday afternoon, leaving her sister and daddy behind. Because this trip was intended to be short, sweet and to the point, we didn't find value in daddy taking time off work or sissy missing any school.
To say the girls were upset about the trip is an understatement. They spent the morning inseparable.. playing games, snuggling, climbing trees together and finally locked arms when we told them both it was time to leave. The pain and fear tore across Annabelle's face as she gripped her sissy tighter.
Mady tried so, so very hard to be strong but was quickly also taken over with emotion - she paced between wanting to go inside and be alone and staying locked arms with Annabelle, absolutely pleading with AK and I not to take her away. It was gut wrenching and hands down the hardest separation our family has endured to date. The somber drive down the road as I watched AK and Mady disappear from the rear view mirror and calm Belle in the backseat from hysterically crying was about all the strength I had to not crumble.
The drive wasn't bad, we never hit traffic and only stopped a few times before arriving at our hotel for the night in Charleston WV. It took about 5.5hr which isn't too bad. We were hungry and very sleepy.
Sunday we hit the road by 7am. There was a Starbucks in our hotel parking lot so we made a lap thru the drive-thru and even found Annabelle a new drink! Caramel Frapp with, coconut milk, no caffeine and no whip. She was in heaven!
We stopped in Dayton Ohio for lunch and a break to walk around and then made it to our hotel in Cincinnati around 4pm. The trip wasn't going terrible.. it was smooth, not rushed because we gave ourselves plenty road time. Annabelle had FaceTimed sissy and daddy enough that she wasn't as upset any longer. We counted our blessings and focused forward to our appointments Monday. Dinner was relaxing, although, as typical Annabelle goes.. within minutes of eating, she was in pain :(
Today, Monday, everything took a drastic turn for the worst.
We arrived to the hospital shortly after 8am and first delivered a box of donations to the hospital that was collected by Annabelle's school. There were so many bottles of bubbles and Annabelle was so very proud to donate them for other boys and girls in the hospital like her.
Next, we followed our signs to Concourse C, checked in and as soon as we sat down in the waiting room, we were taken back to meet with GI Motility, Dr. Kaul. As planned, we talked about her last surgery with Motility which included Anorectal dialation and Botox injections. Our conversation included the ileus and extremely lose stools in which he then noted her weight... she's lost weight. I've mentioned a couple times to AK that Belle looks skinny but I just thought she was thinning out and getting taller - I hadn't registered she was losing weight.. in fact, she's lost several pounds since January :( Not gaining weight for a GI pediatric patient is one thing to treat, but losing weight over 5mon is cause for concern. He ordered some scans and X-rays right away, we would hear from him once those are back.
Our next stop was to see Dr. Putnam. By this time, Annabelle was getting very tired and weak (not uncommon), she hadn't walked much at all in the morning beside around our hotel room but her legs were already tired and buckling when I encouraged her to stand. Still though, I flipped thru my notebook to make sure all my thoughts were organized and the conversation with Dr. Putnam would be worth our trip when he comes into our room.
I was blindsided by what unfolded during the next hour...
With Annabelle crawled onto my lap and ignoring Dr. Putnam, she moaned as he assessed her and stayed silent as a mouse while we talked. I think she knew something was wrong.. I watched her body flood with anxiety as the minutes went by.
Dr. Putnam expressed his concern about her weight loss, the feeding intolerance at times and discomfort. I asked repeatedly if we can blame the Esophaghitis for her symptoms and he dismissed each and every one. There was no way EoE could be to blame for all parts of her body showing signs of failure.. Perhaps though, the small percentage of our problems = her refusal to eat much food and pain when eating, perhaps that could be explained by the Esophaghitis being active. The problem with this theory is that it's been almost 5months since we removed Pork from her diet and the inflammation should be resolved by now. That is, if the pork was the ONLY culprit to her last relapse. (Hold on tight, I know this sounds confusing.. and it's going to get worse but I promise to wrap it up in a nutshell that's easier to comprehend).
Eosinophilic Esophaghitis is such an evil, ugly, relentless disease that just when you think you've figured out it's paterns, it can change on you. When Annabelle was first diagnosed, she didn't eat for 6months. Her body was finally in remission and healthy enough for us to begin our journey to finding a food that EoE wouldn't respond to and attack. We started her very first food with sweet potato - it was a fail. So we went back to zero food until she healed. Next, we trailed apples = success! From there, one food at a time, every 12weeks, we would trial another food and pray it was safe to keep. Some were failures, but we knew during her scope that if the disease was suddenly active again, it was 99% likely due to the last food we were just trialing. So we would remove that food and continue with the foods we knew were safe and keep praying and looking forward. We've been doing this for almost 4years straight. I explain all this to tell you - the disease has an ugly curve ball. EoE can decide out of the blue, that even though she's eaten something like apples for years - it can suddenly decide 'apples' is a trigger and set the disease off. Because her diet is so large (in comparison to most EoE kids), we have absolutely no idea how to figure out which food is the culprit. So what do we do? We take ALL her food away. Yes, we stop feeding her completely until her body heals and then one food at a time, we start all over from scratch.
Now, there's a chance this is NOT EoE flared - if that is the case, we have bigger problems and a lot more work to do.
(There's also a third element that were not ready to share quite yet.)
Beside the discussion about how to address a possible EoE flare, Dr. Putnam expressed to me how critical it is that we take our next steps in healing and preserving what we have left of Annabelle's GI system, it's never been more vital than it is now ... her body needs help, and immediately, we can see all the signs: malnourishment, ileus, BP. Most importantly, she needs to be relieved of her pain asap.
He thoroughly explained what would be happening next. I sat there listening, taking very deep breaths and holding Annabelle in my arms tighter and tighter as he spoke.
Emergency surgery. We can't leave until he see's her in the OR. I thought it would be maybe next week... no, they scheduled Annabelle for first thing in the morning, we will arrive for surgery at 8:50am.
He will scope her esophagus and stomach to take biopsies and see if the disease is present
Dr. Putnam will then next build Annabelle a new stoma (hole) in her stomach that will accept a different tube than the one she has now. She will be moving from a G-Tube that feeds her directly into her stomach.. and she'll be gaining a GJ-Tube that allows us to bypass her stomach and feed her directly into her small intestines while we vent her stomach via G-Tube. This process will be quite complex as she's never had a GJ before and we will have to figure out the best placement and sizing of the tube.. not to mention recovery will be excruciating and a new raw stoma will take months to heal.
After surgery, we have to remain in Cincinnati for at least a day to ensure the GJ Tube stays in place and doesn't become dislodged from her intestines (apparently this can happen as recovery is typically painful and involves retching and vomiting when beginning feeds for the first time.. all that stomach muscle movement can cause the J-tube to move into the wrong position).
From there we wait for biopsy results to decide our next steps.. we will move in one of two directions depending on what they reveal:
Biopsies are clear and the Esophaghitis is not active. This means the pain is most certainly coming from other parts of her GI system and we need to determine what's going on. In the meantime, she will be fed 23hr x day by J-feedings and also hooked to her G-tube for venting. She will be allowed very, very little by mouth (for comfort only) as Dr. Putnam does NOT want her stomach being involved in the nutrition process.. her gut needs to heal and the only way to do so is to stop using it. This means, we don't want anything dropping into her stomach = nothing can go into her mouth.. however, for quality of life purposes, we will allow her to comfort enjoy very little but nothing substantial and nothing that takes the place of her daily nutrition needs.
Biopsies show elevated eosinophils and an active disease. This indicates something that has been in her diet for years has suddenly taken a turn and set the disease off. We will be forced to remove everything from her diet and she will not be allowed ANYTHING by mouth. This could be anywhere from 6mon-year before we would be allowed to feed our daughter again. Regardless the outcome of biopsies, she will still be hooked to a feeding pump 23 hours x day.
Our hearts are broken for our little girl. Her favorite thing are her snacks and enjoying a bowl of pasta with us.. how do you tell a 5 year old that she can't eat anymore? We've been thru this before when Annabelle was 18mo old, and while that was painful, it seems a world different than right now. My heart shattered. There goes birthday parties and bringing our own special, safe cupcakes that I keep prepared in our freezer. There goes popsicles by the pool. There goes family dinner nights and cooking in the kitchen together.
She's gaining a new surgical hole in her stomach.. there goes the pool for most the summer. She can't play in any yucky water when she has an open would on her stomach, let alone when she's hooked to a feeding pump for 23hr x day. There goes gymnastics. There goes quality of life as she knows it.
I called AK while we were still in the hospital and told him the news. Together we cried. We knew this day may come.. we KNEW how dangerous and ugly this disease is.. we knew we were blessed to have as much in our diet as Annabelle does, but we definitely didn't expect this news, this week. I wouldn't have come to Cincinnati alone if I had an inclination this would happen. AK and I discussed for hours the thought of him flying to Ohio to be with us so I could have help and Annabelle can have her daddy, but almost $600 for a one-way flight isn't realistic for our family. The best we could offer is numerous FaceTime chats.. .. .. even though they all involve tears.
AK is telling Madelynne tonight what has happened and once she is in bed, together he and I will tell Annabelle about tomorrow. I don't have the heart to tell her, I don't have the words to explain why life is so unfair to this little girl, I don't have the strength to look into her tear-filled eyes when she realizes she is about to have her 29th surgery and won't be able to eat again.
Days when I think I am strong, life sure knows how to knock me right back down. Weeks when I feel like I start to have a grasp on things, the rug is pulled from under my feet.
Moments when I feel like I am losing my world... I pick up the phone and have a sympathetic, humble, incredible husband on the other line.. I have two beautiful daughters that have a magical love for one another and we have friends and family all over the country praying for us. Moments like these are the biggest eye-openers that I may ever experience.
Today, right now, I feel absolutely all alone and unable to do anything to save my little girl. I don't know how to fix it all.. I am momma and I am supposed to fix things and I can't. I don't know how to make the pain go away, I don't know how to make the disease stop, I don't know how to soothe her confused broken heart. I don't know how to comfort my husband from hundreds of miles away. I don't know how to take care of myself and eat while my daughter watches me. I don't know how to think straight... I do know how to pray and plead for you to keep us in your thoughts. I do know how to take a long shower where tears are allowed to fall without judgment and pray for a better day tomorrow.
Surgery is scheduled for 8:50am. Please, I beg you, say a prayer for Annabelle. Say a prayer for the guidance of our doctors as he permanently alters her failing body once again. Pray for relief from the pain that she has been enduring for months and pray for immediately help of the pain she will endure while waking from surgery tomorrow. Pray for AK and I as we figure out how to navigate life's shift once again. Pray for the strength, patience and momma love that it will take from me to drive her home from Ohio when we finally pack up to head East. Pray for sweet Madelynne as her little soul will be consumed with worry for her baby sister. And most of all, pray for Annabelle, pray she finds her smile again soon after we tell her this news, pray she isn't in much pain, pray she understands as best as she can..... and selfishly, I ask, pray she doesn't blame me :(
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:
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)
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!
