When it rains, it pours

The last few weeks have been a whirlwind for Annabelle and our family. As always, when we are having a tough time or get bad news, it takes me a while to absorb everything before I can collect words to share them to the world. Thank you sincerely for being patient and keeping Annabelle in your prayers, even without prompt updates, it means the world to us.

During the past few weeks, Annabelle's pain and appetite has gotten increasingly worse. We were scheduled to meet with our doctor and get clearance for an upper endoscopy and biopsy's, while there our doctor was fortunate/unfortunate enough to hear how miserable Annabelle felt and her gross coughing and choking. She listened for quite a while and reassured us that the coughing was not from any respiratory or her lungs, it has to be GI related. This isn't surprising to any of us since Annabelle has been on straight prednisone, twice a day for over 5 months now. Long term steroid use poses many complications,one of which is steroid induced reflux, which is likely what were seeing right now. Annabelle does take a compounded acid blocker twice x day to alleviate reflux, in addition to having a nissen (stomach / esophagus tied snug to prevent reflux from emerging from her stomach to throat). Still, the coughing has been lingering for over 4 weeks and is quite persistent. She's uncomfortable, exhausted and overall just frustrated at the constant battle to get rest without barking all night. Hopefully our next scope will show us something.


After our appointment, we performed a few labs. We have been so fortunate to not have had to draw lab work for a couple months now! Unbelievable!! Entering the lab to have her blood drawn however, was not a distant memory. Annabelle knew immediately what that small white room meant and she melted down. I sat in the chair and the lab tech did an incredible job drawing the labs. She got Annabelle on the first stick (!!!!) but it still didn't mean there weren't a flood of tears and PLEADING to go home, "mommy stop! please! go home, mommy, please!" :( It was over before we knew it and I left Annabelle with our nurse at the doctors office to head home while I flew to work myself.

That evening on the way home, I got a call from the doctors office. The labwork they performed wasn't complete. They accidentally used the wrong tubes when collecting some of her tests and needed us to return to have the labs repeated. My heart sank. There isn't much worse than having to hold your sweet baby down for needles, but to unnecessarily have to do it all over again because of someone's mistake, that really bites. I know the sweet lab tech felt equally horrible - I'm certain she didn't want to stick a baby anymore than I didn't want my baby in pain again, but the situation itself was frustrating. While the labs were important, I knew there wasn't any way I would be able to return the next day for the labs, and I didn't want Annabelle stuck in the same place just a day later - I know that hurts. I agreed to bring her back, but it wouldn't be until the following week. I felt terrible for Annabelle and looked at her pitiful eyes in the rear-view mirror, 'Belle, do you want your toes painted pretty?' She piped up quickly and said 'Yes!!! Yes please!'. She's been pointing at her chipped nail polish for days saying her toes have boo-boo's and I haven
't gotten around to painting them. AK was out with Mady for the evening so I decided Belle and I would take our own girls night and have our nails painted. She picked the color for both of us..

A few evenings later, the labwork we were able to get, began to come in... to our surprise (or no surprise at all, really), multiple tests came back quite alarming. The labs kept coming in over the next day and our doctor and I spoke numerous times about what these labs all mean. For one, her Eosinophil count was as high, if not higher now than it's ever been. Which is beyond frustrating considering we've had this kid on prednisone, little food and kept her in a bubble for 5 months, only to find out we haven't made any progress in regaining control of this disease at all. I think deep down I knew Annabelle wasn't well and we haven't been very successful in bringing the last EoE relapse into remission - but I couldn't have imagined even her labwork would look this discouraging.

Other tests came back alarming high for multiple auto-immune indicators, not associated with Eosinophilic Esophaghitis. Our doctor then mentioned a plausible cause: Lupus. My mind remained confused as she talked me thru it and we began reading and researching together. Lupus is a connective tissue disease and is often times overlooked/undiagnosed because of Eosinophilic Esophaghitis! I continued my research and heavily believe this has the potential to be our root, global diagnosis. With the remaining of the labs filtering in day by day, each and every one of them continued to validate Lupus as a likely explanation. http://emedicine.medscape.com/article/1008066-overview
We are working ferociously to move to a specialty hospital that has a team specifically  for children with multiple auto immune symptoms and diagnosis but are living with little quality of life due to the absence of their global-root diagnosis. That's where we need to be. And we are doing everything we possibly can to get there!

This week, while school shopping for Annabelle's sissy, we decided to grab dinner before going home. Annabelle had been in a great mood all evening. The time was late, it was around 730p and suddenly, at the dinner table in a restaurant, everything changed.

Annabelle began saying her ear hurts, she then began crying and throwing a fit. Within minutes she wanted to lay down and then minutes later crying again. An ear infection is something every parent has dealt with 239016036 times and can pretty much diagnose immediately without the use of any doctor tools or payment of a co-pay. But something about the escalation of how quickly Annabelle went from smiling and playing, to laying in the dinner booth and bawling crying, made my mind race. I got up from the table and kissed my family goodbye before dinner was finished.. I curled Annabelle in my arms and power-walked her to the car. We live just minutes from a KidMed and a hospital. I decided we would visit the KidMed to at least be in a safe place if something took a turn for the worse. I drove, in complete silence and gently singing to Annabelle between promises "We will be there soon sweety! We're almost there! I'm so sorry your ear hurts, just another few minutes!" Annabelle screamed. She screamed and screamed and cried her eyes out. We got to KidMed where I carried a limp and moaning little girl thru the doors and was greeted with more tedious paperwork and insurance questions than I see at DMV! I was heartbroken for my daughter as she waited in pain with tears streaming down her face while I filled out paperwork... bad policy there kidmed - if a child is in pain, let's address that first and paperwork later - or at least allow us to go to our room and begin the doctor process while I fill out the paperwork!

 Once we were finally in our room, the questions began. "Alright, so how is ms. annabelle, does she have any allergies, unusual medical history or any past surgeries?" - ughhhhhhhhhhhhh, where is my binder when I need it?! I have all those questions answered on pre-printed copies that I can just hand the nurse when we see a new doctor/specialist/hospital. I smiled politely, as I always do and asked sincerely "her medical history is miles long, it would be easiest if I tell you her drug allergies and only tell you what foods she CAN eat. Here are her surgeries and confirmed diagnosis.. I know you're going to want details on all this, but my daughter is in pain - can we just start with that until the doctor comes in and we can begin some pain meds and I promise I'll answer the rest of the questions again when I repeat all of this for the doctor." She smiled and thanked me for my honesty and said "thats a great plan - lets get this down in 30 seconds and I'll grab our doctor mom". whew.

She definitely had an ear infection - but that became the least of our concerns when Annabelle began shivering and instantly stopped crying while staring at the wall. Her temperature just moments before was 97.3, she was now drenched in sweat and silent. The doctor felt her all over and asked if this was common. Annabelle hasn't been in acute pain like this for quite some time, nor has she ever had an ear infection - but when she is in pain or her immune system is required to fight something, any and all symptoms and her little body's reaction is in the air as to what it's going to do. The only thing we can do to calm it is to relieve the pain and calm whatever is challenging her immune system. Without a doubt it isn't an immediate fix, but it will at least usually calm the insane rapid things her body is and will continue to do until she's out of pain. I asked for Motrin, dye-free and they didn't have any. The doctor rushed the nurse to end the questionnaire and she handed me my paperwork - the antibiotic was already called in to our pharmacy but I needed to get home to get our safe pain reliever. We live only a few miles away so I rushed to leave.

The moment we pulled out of the parking lot, Annabelle began crying and pleading to make the boo-boo go away. She would stop between cries and go silent.. my heart stopped every time. Finally, about a mile or so from the house, she let out the worst pain scream I've heard in a long time. I have no idea what caused her so much pain to scream the way she did but it didn't stop. She began retching and vomiting, between every moment she could breathe from vomiting, she would scream to the top of her lungs. I couldn't get in our driveway fast enough. AK met me in the garage when we pulled up and I yelled for him to get her extension and a syringe FAST! She couldn't stop retching and I just knew it was her nissen that was tearing or causing her the pain. The pain screams correlated when her body would begin to heave to attempt to vomit. I carried her into the house where Mady was somehow still awake and scared to death when she saw her sissy in the condition she was... We hooked her extension and a 60cc syringe to her gtube and it immediately begin to relieve of pressure and pouring gastric juices out of her stomach in a violent matter. Soon, she was calmed again. Sitting on my kitchen counter covered in tears, saliva, stomach bile and the most pale grey face I've ever seen. I flushed her tube and gave her Motrin, carried her upstairs to wipe her down and put some jammies on. (a bath during times when her temperature is fluctuating, is the worst thing to do ever. Her body cannot regulate it being warm from the bath water to cold when she gets out - that's playing with fire. So during times like this, we just wipe down with some warmed soapy wash clothes and try to look past the smell of stomach bile until a bath is safe, ugh).

Annabelle laid in my bed with me while AK went to the pharmacy to pickup the prescription. She was silent and so still. Her temperature was moving rapidly, up and down. A couple minutes laying down on her side, facing the wall away from me, she began having tremors. They weren't nearly as violent as the seizures/tremors she had last year, but they were present and equally as scary. It stopped and Annabelle exhaled as if it just took every bit of strength out of her. She wouldn't talk or cry, she wouldn't hold my hand - she just stared at the wall with a terrifying blank stare. Then it happened again, and once again. I watched the clock and my heart raced - suddenly 'I' was scared and didn't want to be home with her alone. I watched the minutes tick by impatiently and finally he was home. With the meds and wore an equally worried frown when he saw Annabelle. Both our minds were racing I know, but we remained completely silent. She fell asleep and we exhaled. Her temperatures calmed down and the tremors stopped. Around 330am she woke in extreme pain again but it was short lived once we were able to get her more medicine. Her feeds weren't run at night because I was so worried she would aspirate and more importantly, she would try vomiting and not be able to retain the pain meds that she desperately needed.

The next day, our nurse spoiled Annabelle rotten, as she does most days :) She's a god-send to our family. I cannot take anymore time off from work but I knew we needed to get the rest of our labs done. I asked our nurse if she felt comfortable taking Annabelle alone without me. Of course that wasn't a problem for a nurse, but it absolutely crushed my mama heart. Annabelle has never had any labs, tests or procedures performed without me there. Im certain this is probably more about me than Annabelle so I decided it would reasonable to miss this round of labs and have our nurse do them so I wouldn't miss work. As soon as they were over, I called our nurse and asked how it went - with a low tone she said, "She cried and screamed for you the entire time Ashley. She just kept telling me that she only wanted her mommy.. she didn't do so good, but we did get all the bloodwork we needed and she's resting comfortably now." My heart was crushed. The rest of the day was difficult and I found myself buried at my desk with tears pouring in my lap. The mommy pain to know you need to be with your baby but need to be at work is the absolute worst pull of any feeling in this world. Absolutely no decision is right and everyone loses. These are the times when being a special needs parent can / will and DOES break you. I just hope this spiral is short lived and I can get back to my typical 'fake-the-smile' self soon.. but right now, my heart is shattered.

Annabelle still has very, very little appetite. Her fevers are completely gone and the pain is over of course. The coughing hasn't gotten any better, but our labwork is all back and we have everything we need to move forward, cleared for surgery. We are scheduled for September 16th at 7am. Four days before Annabelle's birthday :( Happy 3rd Birthday kiddo!

Belle visits our Capitol!

Sight-seeing, new adventures, laughter and smiles, that's what family vacations are supposed to be made of.

62884825827 stuffed bags, ruined nap schedules, soiled laundry and expensive restaurant meals, that's what a real family vacation is made of.

Then add the curve ball of an OCD parent in a city of germs carrying a child with an auto-immune disease, tube feedings, potty-training, medical supply, equipment and toddler-roid-rage, that's called The Bishop's in DC! 

The girls were beyond excited to take our overnight trip to DC. An anonymous angel donated our family a Marriott stay for whenever we choose to use, we decided our family needed to get away before things get crazy with Annabelle. I can foresee things getting so shaky, I don't like many of the things I am seeing going on with Annabelle right now and my gut is telling me to prepare quickly. AK and I know what life is like for all in our little family when Annabelle is going downhill, so we wanted to proactively do something enjoyable - mostly for Mady before she begins Kindergarten in a few short weeks. So we planned a spontaneous trip to our capitol! The zoo, museums and train ride will be fun, right?
Kinda.


Annabelle was happy when she woke that morning. We packed our bags and she was sure to pack some of her favorite things in her own bag like her sissy. We boarded the train and before we knew it, we were moving along between train stations. AK and I were giddy with excitement for the both of them, the girls loved sitting in their own seats beside one another.

An hour into the train ride, and everything turned around. Annabelle began to get extremely clingy, she wanted to be held, but then wanted down, and then wanted to lay on her belly - side - back - belly, etc. She began begging to go home. I told her we were going on vacation and she cried, she begged repeatedly to go home... see that's the thing with these amazingly strong kids - they adore an adventure, but they tightly hold onto their security of their comfort place. Annabelle's is her home. If she's uncomfortable or nervous, she wants to go home, and most often she requests to go back home to lay in mommy's bed. She wants to snuggle tight over my shoulder into my neck and just lay still in my bed. It's her safe place, and I respect that. Riding on a train towards Washington, DC however, made things quite frustrating for Belle when she realized we weren't headed home. My heart began to sink.


The Zoo was fun, but not convenient for a special needs child as I hoped. Most the animals or sights were behind a glass, or against a window that you needed to be near to see anything. This involved unbuckling Belle, unhooking the feed tubing from it's connections, loosening tubing from her backpack and putting it on my own back, picking up belle as limp as she was, carrying her bag and ensuring i wasnt going to snag tubing, making sure her face mask was still on and she not touching anything... then surrendering the stroller bc it won't fit around the billion people, then fighting towards the window where the animal should be... only to find "Ooh, ok that's it! The monkey is going back inside to eat lunch now, let's see another exhibit kids!" ... thats basically how our morning went, along with a cranky 5yo that moves as fast as an energetic 5yo does but constantly being told "Mady, you need to wait for your sissy hunny.. i need to hook her back up.. your sissy needs to go potty.. Annabelle is choking Mady, I need to help her". Before noon - I looked at AK with a worried face and said "I think we messed up.. maybe we shouldn't have tried this". Then Annabelle asked to go home, again. My heart sank deeper.

We went directly back to our hotel and as a family, we all took a long afternoon nap in the dark quiet. Annabelle snuggled over my shoulder with her soft breaths tickling my neck, Mady cuddled up with daddy on the other bed and soon, we were all snoozing.

The entire trip wasn't bad. The girls absolutely enjoyed themselves and got to see many important monuments and things that we only discuss in conversation. Annabelle's favorite thing, hands down, was the Washington Monument. I have no idea why, but she was mesmerized by it. She couldn't stop looking at how tall it was and asked if it was a tower, she also wanted to know if 'Punzle' lived there (Repunzle).
When we showed the girls the White House, Annabelle was quite disappointed to learn it wasn't a GINGERBREAD House. I have no idea what gave her that thought, but she was certain we were going to see a Gingerbread House and not the President's house. Mady pipped up with an insane amount of excitement when we showed her the White House, I asked if she liked that and she responded, "Oh, absolutely mom! My very favorite president lives there, do you think he's in there right now? President, George Washington is so cool, I hope we can meet him later today"  :)

The weekend was eventful. I ended up carrying Annabelle most the entire time while AK pushed a half-empty stroller around our 100mi of DC walking we did. Why she only wants me to carry her, I will never know. I secretly think she's just trying to break me down ;) after our days of me carrying her, she only wanted to sleep in the bed with daddy - and at that point I had to tell her just how unbalanced this love-spoiled rotten-picking favorites thing is she has going on lol. She is a daddy's girl, thru and thru!

The sight-seeing was great and the weather couldn't have been any better for our trip. Madelynne had a wonderful time and I am so happy we were able to deliver that for her. Annabelle did enjoy herself, but she couldn't wait to get home. She laughed and smiled when we showed her another big monument, but before we could catch our breaths, she asked to go home... again. I know she couldn't have waited much longer so we decided to catch an earlier train home and soon we were all back in our happy place, our own bathtub, jammies and bed. It was a great escape from Richmond, VA and a perfect break before life takes off on the fast lane.

After speaking with our GI, she's agreed we need to head back to the O.R. and see what's going on. We need to scope and biopsy more things and gain an updated Eosinophil count of her esophagus. Let's all begin to pray now that our counts are at zero.

The problem though is a catch 22.. Annabelle needs to be cleared for surgery and anesthesia - her cough though is horrible. It's getting progressively worse and clearly more uncomfortable for belle the longer it persists. 

Because she cannot be vaccinated, our doctors have concerns about Pertussis (whooping cough). We're scheduled to be tested soon. 

Her bowels are another concern. Because of the tube feedings and her diet, Annabelle typically has a BM daily, if not more. Over the last two weeks however, they're becoming more around 4-5 days apart and causing multiple problems, not to mention terrible pain to Annabelle. Eosinophil associated disorders do not only attack the esophagus, they can also attack and damage the stomach, intestines and colon. While I'm hopeful this is simply a change in her GI system and possibly just a side effect from all her medications, it may be necessary to scope and assess the lower GI as well :/ I'm praying with everything that these issues work our bc prepping and the procedure for an upper AND lower scope and biopsy is terrible on Annabelle. My anxiety begins to rise already :(

Otherwise, beside the coughing and no pooping, belle is slow and still but in high spirits. She played outside a bit last night,  it was good to see her acting silly with her sissy on their little bikes. The night before, she wouldn't leave my lap for hours and just wanted to look thru photo books over and over and over and over. I had laundry, floors and dishes to do... Instead, I found myself looking at our family shutterfly books, over and over and over! Lol 

If we can make it to the weekend, AK and I have special plans for the girls. Any time we plan and/or invest to do something, we end up in the hospital - this week isn't a good start but we're hopeful we make it to the weekend with high spirits and can enjoy some family time before school begins for Mady :)

When a simple cough knock you on your butt...

It’s time to consult the specialist again.. Something isn’t right – our nurse, AK and I are all becoming genuinely worried. About two weeks ago, Annabelle began showing signs of a cold/cough. It’s onset was fast but we prayed it would be short-lived. It’s clear your feisty two-year old isn’t feeling well when you take her to a water-slide, pool party and she refuses to get out of your lap the entire time. She never got in the water.. we did however, manage to pull a few smiles from her with the help of bubbles (guaranteed trick, never fails). She was slowing down though.. she wouldn’t eat much and began to need pump feeds constantly.

After a few days it began to get worse and to the point our nurse no longer felt comfortable even taking her much out of the house. She now barely has energy to play outside in the afternoons – her mornings are the only time of day that she has much energy, and even then, she depletes quickly. Her coughing has become a high-pitch chocking / wheezing / gasping. The nights are worse, but not substantially that I would relate it to anything like croup. Her eating habits haven’t gotten significantly worse, but they’re still not where they need to be. She’s undoubtedly lost weight. The high-pitch cough is what scares me the most. Coughing and retching (gagging) is what terrifies me. When she begins to vomit / needs to empty her stomach via tube, that is when we begin playing with fire. When we have to drain her stomachs contents, she loses her counted nutrients that she needs to stay balanced to ensure her ammonia levels don’t spike and begin causing worlds of problems. That’s where we are now. 

We still haven’t managed to get the last EoE relapse under control, even with diet eliminations and protection while playing outside. Annabelle wears a mask most all the time while she is outside. She cannot dare go out if anyone is/has recently mowed the grass. We don’t even cook foods that could set off her EoE inside our home anymore. Our entire family has lost meal items in hopes we can bring Annabelle’s EoE under control by eating and being around only the safest foods possible for her. It isn’t working. We’ve ramped up the medication without luck and are back to putting her in a bubble 99% of the days. Again, no luck. 

Yardsale & Lemonade Stand this weekend to raise money for Annabelle 8) 

These precious little girls did a great time advertising, marketing, bribing and pouring lemonade for a great cause. I am so humbly proud of these two young ladies, that are mature well beyond their years and worked all day to support other kids that aren't quite as blessed as you and I.
Rock on beautiful girls! You're doing bigger things that you could ever imagine!

So we hold our breath and pray.. .. .. .. and drink wine; because fruit is good for you ;)

This last spell of whatever has set her frail immune system into attack is truly doing her in. I have no idea how to stop it and so I’ve called and am hopeful to hear from our specialist soon. We do have an appointment on the books for next week to do more labwork and follow-up with some concerns, but I don’t think we can wait that long. I have no idea what to expect when I hear from the doctor today – my wish for Annabelle is for the least amount of pain as possible, but fast relief from this horrid, horrid disease that’s attacking her little body. I don’t know what other treatment options are even out there for kids like Belle. We get lost in this huge world of thousands of doctors, millions of medications and hundreds of tests… but no effective treatment options. Eosinophilic Esophaghitis is not a one size fits all treatment type of disease… Annabelle’s case involves mystery components that make diagnosis, treatment and management nearly impossible. I just pray, with everything that I have, that one day soon we will have the answers we need for our baby girl. Annabelle deserves a break in this fight, and deserves to know what its like to live and love life without pain. God hear our prayers for this sweet little girl!

Moving along

Moving along
No big updates to share, just the usual (which is oh so very much welcomed!!!).

Annabelle is pretty tired but we're using it to our advantage. She's too exhausted to put up much of a fight about most things, and I enjoy it.
Me: "Annabelle, mommy is sleepy, do you want to come cuddle with me?"
Belle: "No - I want to go play with my puzzles..."
goes to play with puzzles.
...3 minutes later.
Belle: "Mommy, can I cuddle with you? I want to watch Mickey Mouse"
voila!

While she isn't doing back handsprings around the house anymore, she is still full of her silly self and conversations. She loves singing lately and makes up new songs everyday. She loves Katy Perry Firework & Roar. I bought her a new 101 Dalmatian puppy and it's apparently the best thing since buying her Lambie. She's obsessed with the water, any way she can get it. She loves going swimming and is completely fearless. She loves bathtime, washing her hands, playing with the water hose, or just sitting outside splashing in a bowl of water. She loves water. She also loves giving out kisses and hugs lately. When she is wild and on baby roid-rage, the hugs are few and far between.. but when she is sleepy and worn out like right now, she becomes quite cuddly and reminds you multiple times x day how much she loves you.

She doesn't stop talking. Ever. She's learned to communicate and her speech is absolutely EXPLODING. She tells you stories, counts on her fingers the things she has to say, sings, chants, marches to a beat and hums. She loves to talk. . . . . too darn much.

AK and I left town this past weekend, and on the first day of my mom watching our girls, Annabelle decided to make that the time to begin the weekend of heart attacks for nenaw. (grandma). Mom called me worried, "She hasn't eaten anything. She's had all of her medications and isn't touching anything. I drove her a few minutes down the road in YOUR car and her carseat. She never got out the car and all I did was put some tomatoes in the back. I turned around and she's covered in hives on her face, arms, chest, legs etc. What is this from?!"
It became the problem solving that never revealed itself. We have no clue what initiated her immune system to set off. It could be absolutely anything - it could be something airborne, something she touched, ate or smelled, or could also be stress. Yes, stress itself can do the exact same harm and damage as her eating a handful of peanuts. That's the hell of having an auto-immune disorder. Anything can set it off, and it's root cause may never be determined.


 We have no idea what happened Saturday morning. She was dosed with the safest form of benedryl (bc the standard store-bought kind is unsafe for Belle), took a nap and woke without hives. The rest of the weekend was less eventful and at 1am Tuesday morning, our flight landed and we were finally able to see our little girls for the first time in 4 days - the longest we've EVER gone without seeing our girls!

Belle was so happy to see us in the morning, and we were so heartbroken to have to give her kisses and immediately say goodbye as we headed to work. The week has been well - AK and I are dragging from all the travel, but the girls are good. Annabelle is dragging as much as we are so for that, I am actually counting our luck.

Next week will be another eventful week, we have more tests and labs to perform. Hopefully we will have made contact with a specialist to see Belle by then and get some of the work started that needs to begin to get her there. Traveling out of state and insurance coverages are not an easy task to accomplish - they're a nightmare that feels as if the system is built AGAINST traveling to seek better medical care. It will be fun, Im certain.