Our world just dropped upside down

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.

1. 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.
2. He will scope her esophagus and stomach to take biopsies and see if the disease is present
3. 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:

1. 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.
2. 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 :(