Wednesday, April 13, 2016

Upper and Lower Endoscopy, biopsies and 23rd round of anesthesia

Honestly, considering where we started and how far we've come, I cannot complain about an ounce on how Annabelle is doing on this trip. She's handled painful procedures like a pro, the hospital has been very keen on addressing any anxieties she has and allowing us to make a lot of decisions for her. All things considered, things have run pretty smooth.
(For the record, I LOVE this kinda blog update :) )

Since our OR time wasn't scheduled until 1:55p, we spent most the morning being lazy, painting, doing arts and crafts, watching movies (we watched Matilda, because I got to pick the movie this time hehe).

The doctors and GI team made their rounds at 9am and we joined them for Annabelle's briefing on her case. I love how they do this. All the doctors on the floor, research team, specialists, and our nurse stand in the hallway outside of our room with their computers and we join in a circle. Our nurse then briefs the team on why we are here and how Annabelle is doing. The main doctor explains to the team what our plans are for Annabelle while she's on the floor and during surgery. They ask if we have any follow up questions or concerns and then they wrap up our case.

(Here's a random internet picture of what this looks like)

It's all open, we are intimately part of every detail of Annabelle's care, just the way it should be. After were finished, we go back into our room and shut our door for them to move onto the next patient. 

The OR called for us shortly after 1p and transportation rolled us to pre-op. Annabelle began to get scared for the first time, she was breathing terribly heavy and had silent tears falling from her eyes. 

"What's wrong sweetly?"
"I'm scared mommy, I don't want surgery anymore. I just want to go to our hotel, please."

She has been so brave until this point. And she still was, but she was nervous. Our room was no longer decorated beautifully with a Disney movie on the TV, instead, we were now in a holding cell with lots of monitors, beeping machines and teams of doctors, anesthesia and research specialists talking to us and surrounding Annabelle's bed. She had every right to be intimidated. We both gave her our cell phones, she played games, took selfies and even snapped pictures of mommy and daddy lol (excuse us, we look like hell)

Finally, Dr. Putnam made his way to see us, this was a relief to Annabelle, she loves Dr. Putnam and was so happy to see him. We quickly discussed our game plan and he noted that we were next in line for the OR and he would see us soon. 

We moved into the OR where about 15 people were standing around and waiting to begin. I feel like such a fish out of water when we go in here.. they're all staring and watching us silently as they work to organize their last things. I eye each and every one of them, trying to smile, but in my mind screaming "keep my baby safe. keep her alive. Take care of my baby. You. And you. And you. And you.." I can't help it, each and every one of them are accountable for my daughter from the time I am escorted out of that room. I don't know what their roles are in the OR, nor do I care, but my expectation is that they all keep her safe. each and every one of them. 

The anesthesia team (initially) followed my request to repeat the same procedure as we did last time for keeping her comfortable and asleep. They administered Versed and within 20-30 seconds, she was drunk.. Just the way I like to see :) this relaxes her so she isn't as anxious when they put her under and she wakes easier without panic. Next they gave her Propofol which usually does the trick without needing the gas to knock her out. Anytime I see Propofol though, I have flashbacks to when we attempted to use it for an EEG and she fought it with every dosage until it was too dangerous to continue so the anesthesia team ended the test bc she wasn't responding appropriately. It was terrifying and I wish I never witnessed it, it's scarred me. She wasn't even 2 yet and was so, so sick ...

This time though, when they gave her propofol, I watched and her eyes bolted open in a panic just like they did three years ago and my heart stopped. I didn't want her to fight or be scared, I immediately crouched down next to her and told her we were going to take a nap together, and she laid her head back down. I told her I love her so much and sweet dreams, she gave a half smile and closed her eyes. That's when the anesthesiologist put the gas mask over her face and we were escorted out. 

This video shows right after she had versed and when they administered Propofol, I cut it off when I noticed they were giving the Propofol and she was trying to sit up.. 

We made our way to the waiting room and found our usual seats, right in front of the monitor that relays how Annabelle is progressing thru the OR. This was AKs first time being inside the OR when they put her under, we talked about it for a bit.. He didn't like what he saw, as a daddy, there are just a lot of parts of this medical process with Annabelle that he would prefer he never sees or knows about. The OR is intimidating, without a doubt, for parents as much as it is for the kids. I'm just grateful Annabelle has us both to balance the emotions of what she's going thru. I am much more medically aware and engaged, a fighting advocate for each and every decision and daddy is her hero that brings a bit of home to the hospital and makes her laugh and forget everything she's going thru. We wear two very different hats in how we provide her medical care and how we parent our kiddos, and I couldn't ask for a better co-pilot on this adventure.
....enough of that sappy stuff..

About 45m later, we were called for a consultation, where they take you into this tiny room between the OR and waiting room and our nurse (Eosinophilic Esophaghitis specialist/ Putnams right hand man, err, lady) briefs you on what went on, updates her notes from us and we wait in silence until Putnam joins with pictures and an update on what he saw visually. She looks good from a visual standpoint, we need to wait for biopsies and he stressed that we need to have some conversations during our clinic visit on Thursday. We agreed, took our paperwork and went back to the waiting room where we wait until she's stable in the PACU and they call us to go see her. 

.. .. .. Almost an hour later, I was covered in hives and wanted to vomit from nerves. She has never ever taken this long to wake up and them call us. Every worst case scenario was running thru my head and I just knew she was screaming hysterically in disoriented fear and I wasn't there (that's how she comes out of anesthesia 80% of the time). I demanded the rep at the front desk let me see her and/or speak to the nurse that's back there with her. I couldn't take it any longer. The nurse on the phone said to send us back, I bolted to the PACU, leaving AK in my dust. When I found her, she was still snoozing away, still had a breathing tube and on oxygen, she just didn't want to wake up yet. After about 5min she wiggled a bit so the nurse removed her tubes and set the oxygen to the side as we watched her for another 30min. She seemed to be slowly, very slowly, coming out of it.. But she was scaring the hell out of me. I couldn't touch her, hold her and I can hardly speak to her bc it's essential she wakes on her own and doesn't panic when her body can't wake as quickly as she wants to. We swallowed over our lack of patience and anxiety and watched our baby snooze... For what felt like a year! Finally, they called transportation and allowed her to finish coming out of anesthesia in our own room on the floor. I was relieved, at least if we're leaving PACU, that's a good sign.. 

When we got back to our room, daddy picked Annabelle from her stretcher and carried her back to her bed where we made her comfortable again. This little bit of movement did wake her a little more and soon she was opening her eyes for us. We both exhaled, loudly and smiled at one another. Whew, she's back! 

I've mentioned it before, but anesthesia is one of my biggest fears, for myself, for my husband, for my babies. It isn't often people are put under, but in Annabelle's case, it is often. This was #23, and although I wish it wasn't in the back of my mind, I always think and say "how many times will we be lucky for everything to run smoothly? This may be the last time I see my baby awake and smiling at me, this may be the last time I hear her sweet little voice." Anesthesia is dangerous, there's no doubt about it and all our doctors remind us. We have to get to a point where she isn't going under all the time bc eventually there WILL be consequences to the repeated bouts of anesthesia.. I just wish we were already there. :( 

We were discharged around 530-6pm, just in time to play in downtown Cincinnati traffic during a rainstorm. Annabelle was too tired to notice how long we were in the car.. She was still so groggy, which was probably a good thing. We arrived at Great Wolf Lodge around 7p and revealed her big surprise :) she was pleased, but couldn't grasp the amount of fun these few days would bring. The last time we bright Annabelle to Great Wolf, she was so sick and weak, we thought we were losing her.. Hospice was called a month later. She doesn't remember that trip at all, she never really got in the water, she just cried occasionally and slept 90% of the time. Here are some pics from that trip:

Mady remembers the trip though and remembers how much fun it was.. When she finds out where we took Annabelle, she's going to be FURIOUS! Lol

We didn't do anything fun open arrival, we had dinner where Annabelle couldn't eat much and then sat for story time, but she asked to leave within 5 min of the story beginning because she wanted to go to bed. 

Tuesday she did play for a bit, but she is still incredibly weak. I can't gauge whether it's all because she couldn't eat for two days and then underwent another surgery or if she's tired because of all the running and swimming thru the water park. Nonetheless, she could only play for about an hour and a half and then couldn't walk or run much any longer and needed rest and some sleep. Her cough is getting pretty out of hand too so we're keeping a close eye on that.

No appointments Tuesday. Wednesday we report at a satellite location for a sleep study.. and based on how much we are wearing Annabelle out playing, I don't foresee any issues sleeping on the only night she DOESN'T need to sleep like a log. lol 

Sunday, April 10, 2016

Divide and conquer VA to Ohio

Yesterday was nothing less than exhausting for the Bishop home. We had opening day at the baseball field, tball pictures, tball game, soccer pictures and a soccer game. All the while it decided to snow in VA making the day miserable for all of us outside all day. Between our games and pictures, we managed to finish packing the bags and our car so we could hit the road as soon as Annabelle's first tball game was over! 

I rode in the backseat with belle for the first couple hours, we watched The Sandlot and ate snacks while daddy drove. She asked often why we HAD to go to Cincinnati and we answered her honestly. She wasn't confused on this trip, shes remembering our hospital visits much better each and every visit, but she still has anxiety about them. Her biggest fear are getting pokes. She pleaded for us to not have to make her get any pokes, or perhaps for us to ask the doctor for some sleepy medicine so she could go to sleep while they do the pokes (how clever! That sure would be nice, heck I would sign up for that myself!)

Annabelle fell asleep in the car around 9p and by 1030p we decided it wasn't safe to drive much longer. The temperatures were below freezing and we were driving in and out of snow storms as we wrapped around the mountains of West Virginia and Kentucky. We were both exhausted so we stopped shortly past Charleston, WV to get some sleep. 

We got back on the road by 7a and were Cincinnati bound. Annabelle continued to ask and negotiate about going to the hospital, and we continued to answer her questions honestly but provide excitement about the trip, "we also get to pick out your wheely chair while we're here this time! AND mommy and daddy have a big surprise for you after your surgery tomorrow!" She was pleased, but still so anxious and disappointed. Watching a broken 4 year old process the fact she's being forced to a hospital for painful procedures is something a parent should never watch across their child's face. It kills me...... I always have to look away..... There's nothing I can do about it either...

We arrived at the hospital on time and were admitted quickly. Our room was personalized for Annabelle and we met so many familiar faces on the GI Surgical Floor. It's like we're 'home' again. 

Before we knew it, it was time for the dreaded poke. Everything leading up to it ignites her anxiety.. Getting undressed, taking off her own clothes, putting on a gown, sitting in the hospital bed, child life and all their distractions - every level of detail is another element that brings her closer to that needle and she knew it. 

I asked for an IV team to help with the stick. Not because we didn't trust our nurse, but because Annabelle's arms are building so much scar tissue that were having to stick her 2-3x each time we attempt labs or an IV. If I can subside just an ounce of her anxiety and fears from the pokes, I will take it. They brought in the queen of IVs and she is accompanied by her ultrasound machine that helps her place the IV. It was super cool. They scrubbed Annabelle's arm, and while child life distracted her with pictures and stickers, the nurses watched her veins on ultrasound and incognito made the stick. The moment Annabelle moaned ouch, we pointed her attention to the ultrasound machine and she smiled Bc she could see what was happening. 

She didn't have one tear. Not a drop. She didn't cry or once complain. In fact, when the nurses left, she told us it was the best day EVER Bc it was the first time the poke didn't hurt! I could kiss the IV nurse for what she did for Annabelle. Relieving her from this stress and anxiety was every bit of my prayer for today and she made it happen. 

Once the IV was placed, it was time to begin running the meds to flush her GI system. Unfortunately though, the nurses couldn't get their feeding pumps to work. We tried using our own extension sets (tubing) and finally after two hours and them trying all her equipment and supplies, I offered for us to just use our own equipment and supply. They agreed. (I really feel like I should be getting a credit on our bill for this) 

The evening has been smooth. Annabelle made a trip to the playroom to paint a few pictures, we did a puzzle, the second was a different puzzle than the box it came in and frustrated the ever-living out of her so we gave up ;) we watched Inside Out a few times and before we knew it bedtime was here. 

At present, we've had two blowouts equivalent to that of an atomic explosion. I look forward to the rest of the night following suit.. If she continues to move stool at this rate, fingers crossed that we won't spend the entire night doing painful rectal irrigations. 

Sweet dreams world

Monday, April 4, 2016

Richmond preparations for Cincinnati

We're finally in the process of crossing T's and dotting I's for our upcoming trip. Annabelle is aware that were traveling soon and Madelynne knows that she will be staying behind because she has school. The anticipation is growing, but so are our frustrations with Annabelle's health, so we are all very much looking forward to this visit.

I mentioned before that scheduling this time, was extremely difficult. There was a lot of strategic planning that took place to get the appointments down that we needed. I am still waiting on a date confirmation for the Wheelchair Clinic (they're accommodating us individually after-hours because we couldn't get in while they're available). The only other appointment I couldn't manage to figure out was the Gastric Emptying Study.

This procedure needed to be performed enough in advance to have the results before we scope and do surgery. We know something is terribly wrong with Annabelle's GI system, but we don't know where the problem lies, the Gastric Emptying Study will allow us to see how the food she eats, moves thru her GI system, how quickly, efficiently and if there are any pauses/issues/obstructions/blockages etc along the way. If we can pinpoint where an issue is in her GI system, we can take a closer look around the area when the doctor performs the surgery and scope in Cincinnati. With the help of our team in Cincinnati, we were able to schedule the scope in Richmond last Friday at 9am.

Gastric Emptying Study
We arrived at the hospital around 845a, cranky but excited. She needed her formula but wasn't allowed to have anything before the test, she was hungry and nervous but hid it well. On the way to the hospital, we sang songs and I asked if she was alright or wanted to talk about anything, her only question was "Will there be any pokes today?" I couldn't guarantee there wouldn't, but told her that I was almost sure we would not have any pokes.. she seemed satisfied with that answer.

Once we were checked in and bracelets on, they escorted us to our room in Nuclear Medicine where they explained the procedure. Annabelle would have 10min to eat the scrambled eggs that are impregnated with radioactive dye, after that they will do a scan of her stomach. From there, we would do another 2 1/2 min scan every hour on the hour, for 4 hours. Once everything was explained, our nurse asked the routine questions, "Why are you having the study? What concerns do you have? How do you spell that? Can you spell it slower? E-O-S-I-N-O-P-H-I.......... Who ordered this test? And finally, do you have any questions? I did. #1: I need the final report, in hand, when I leave today to take with me to Cincinnati. #2: Annabelle has been allergic to eggs all her life and just recently tested negative to them. She's never had eggs before, so do you have a bottle of benadryl near by? This is the moment his eyes turned into quarters and he darted out of the room.

Over the next 30min, every physician and nurse was consulted and every conversation to talk us out of having the test was discussed. I repeated, "Her doctors know her allergy history, he knows she's never had eggs and he's ordered the test!" They would ask over and over, "Alright mom, what do you want to do today?" Me: "GIVE HER THE DARN EGGS! Don't you have an action plan for an emergency?!" They explained, "We only have Benadryl in our crash cart, we don't have it on hand. If something were to happen we would have to use the crash cart and call a code.." "Well Ok! That's fine. That's your plan. If she begins having a reaction, we'll just call a code. Now bring us the dang eggs so we can get started." They handed me the panic call button and everyone watched her cautiously as she ate. She did a great job and devoured them within our 10min.

Once the eggs were finished, she washed her hands and we were off for our first scan. Everything was painless, no pokes, the machine wasn't loud and nothing was truly intimidating.


Annabelle did such a wonderful job. The hardest part of the day was finding distractions between each hours exam. We toured the new VCU Children's Hospital (not impressed), spent some time outside, walked the halls, visited nenaw (my mom), and watched movies on my phone. The last couple hours of the 6 we were there were the hardest, but we survived. Her pain started around hour 3 after eating the eggs, they documented everything and continued the test. By the time we were finished, I could tell Annabelle was getting increasingly more weak and started scaring me. She hadn't been fed in almost 15 hours, and that is entirely too long for her body. During the chaos of the morning, I forgot to bring her feeding supplies and formula with me so I could hook her up after the last scan, so as soon as we were finished I had to leave. I signed a waiver for my mom to pickup the final report that afternoon, and Annabelle jetted home for food and a nap!

She did well that evening, but late that night she began retching (trying to vomit), Saturday did the same and by Saturday night the diarrhea began. If you ask Annabelle, she isn't pleased with the egg experience. She's quite miserable really.

Auto-Immune Skin Rash
Another curve ball Annabelle has thrown our way, are these tiny bumps that she has all over her body. It started out as one bump, on her knee, that honestly just looked like a wart.. and then it spread.. across her knee, up her legs, and is now on her chest, neck, face and eye lids. Ive shown this to our doctors in Cincinnati before (at the time it wasn't nearly as bad as it is now, bc it's spreading so quickly). The more it spread, the less I wanted to wait until we get back to Ohio, especially after seeing it was moving towards her eyes etc. I took her to the doctor and was told its an auto-immune virus, and typically never spreads this rapidly, but because she has an auto-immune disease and pretty crappy immune system in general - were left with a case of bumps spreading like wild-fire. I picked up the prescription last night, it comes in a few tiny packets, smaller than a sugar packet, and it cost $78. Yay for great healthcare (insert sarcasm). So let's hope this magic cream works, and quickly!

SO! Besides getting everything scheduled and on the books for Cincinnati, we are focusing on just keeping a close eye on how Belle is doing and praying time moves quickly so we can get her comfort soon. Besides the eggs, she's complaining of pain on a daily basis, she isn't resting well at all any more and is becoming so clingy because she doesn't want anyone to leave her. She does still run and play when she's feeling well.. so by all means, we are not at our worst, we just know that she's heading downhill and we want to move quickly.

Annabelle's a tough cookie and is looking forward to this next trip. I think she's secretly looking forward to the time alone with mommy and daddy ;) She will miss her sister dearly, but the time alone is always exciting! We plan to leave Saturday afternoon. AK is head coach for Annabelle's T-Ball team and they have their first game at 3pm Saturday. As soon as the game is over, we are hitting the road. The goal is to make it to Kentucky late Saturday night, stop for a late dinner and hotel and then hit the road Sunday morning in time for admitting to the hospital at 10am.

Let's get the prayers started now!

Tuesday, March 15, 2016

Full-Time Mom, FT Employee, FT Medical Advocate & Scheduling Coordinator

I have to say, the amount of time, exhaustion and sheer dedicated attention to detail you must have to survive the medical world, is incredible.

Our issues lie mostly with the fact that we are limited to trips to Cincinnati Children's Hospital only every few months, so the time we are there is absolutely slammed packed. We are at the mercy of the hospital and pray all the stars align, every time. Before we ever leave our visit, we discuss our follow-up game plan for our next trip, we discuss the work we will do while at home and plan a strategy to maximize our visits.

And then it comes down to actually getting those visits on the books.

Before we can even get an appointment confirmation, we must first jump thru all the hoops known to man to make sure, 'Said department has all the information they need before we can speak to someone in scheduling'.
  • Referrals
  • Physician Orders
  • Insurance Approvals
  • Prior Authorizations
  • "New Patient Questionnaire" completed and submitted
  • Preliminary labwork/tests
For the most part, working with doctors and nurses in Cincinnati Children's isn't too bad. The fact that they have a SYSTEM and process for everything, makes life much easier. But still, the leg work that's required to make it all actually happen, is exhausting.

Our next trip is slowly making it on the books. Here's our agenda while we're there April 9th-14th:
  • Surgery. Upper and Lower Endoscopy and Biopsy's
  • Sleep Study
  • Gastric Emptying Study
  • Dermatology
  • Wheelchair Clinic
  • Sleep Medicine, clinic visit to discuss Sleep Study findings
  • Gastroenterology, clinic visit to discuss Biopsy Results and Gastric Emptying findings
The problem this time, lies less with the leg work of mercy of nurses submitting the right paperwork, but more with the coordination on how to make these specific tests happen.

Surgery must be performed on Monday, April 10th. It's the only day our physician performs surgeries and they're always done on Monday's so we can have biopsy's back by Thursday in time to review the findings.

The Gastric Emptying Study must be performed prior to the surgery. Although it can't be done the DAY BEFORE the surgery because during that time, we are in the process of cleaning out her GI System in-patient. It needs to be done two days before, but they don't perform them on Saturday's. We are waiting now for approval to do the study two days *after* the surgery and hope there isn't an issue.

Sleep Study is performed overnight (6pm-6am), but we can't do it during the night we're doing the GI clean-out. (Obviously waking a toddler every hour on the hour to perform painful rectal irrigations isn't conducive to determining why she doesn't sleep and rest well at night). She needs a peaceful night of sleep during the sleep study. The only day they have available to do the Sleep Study though is on Sunday night, into Monday = The day were already booked for GI Cleanse / Surgery.

Dermatology and Wheelchair Clinic are both wild cards. Both essential (ESPECIALLY the wheelchair clinic), but both are very tricky to schedule with. Wheelchair Clinic only books on Thursday afternoons, we have that time slot already booked for a follow-up with the Sleep Clinic.. and Dermatology needs a referral but we don't have a doctor in Cincinnati that's addressed this issue specifically.

So, as you can see, it's a mess. It's just as exhausting to read as it is to coordinate, but I know things will work out as they should. I just hope we can maximize our time and secure all our appointments while we're there.

On the Annabelle side of things, she's worrying us. She is persistently complaining of her tummy hurting.. regardless who she's around. Some times I take the complaint with a grain of salt and offer her a distraction, and sometimes, the look in her eye validates her pain and no distractions can make her forget about her tummy. She's having a hard time sleeping at night and the only coping method she seems to know is to drink her 'milky' (medical food / formula that we serve in a cup and also via gtube). She's waking 4-6x night crying for someone to help her. Last night she slept in our bed and it took her over an hour to fall asleep, after we entertained all her requests (milky, venting her gtube with an extension and 60cc syringe, rubbing her back, holding her hand, etc). She swore something was inside her tummy making her sick but there's nothing we could do to resolve it.

She cannot have a BM without an enema any longer. She just simply cannot go. This battle has been ongoing for years and I am at the point that I'm demanding an answer come hell or high water. That's the reason for this next scope. Dr. Putnam promised us in October that if her BM issues hadn't resolved over the next few months, we would move forward with treatment. In order to do so, he needs her scoped and the Gastric Emptying Study performed. I hope these reveal some sort of answer as to why she's having such a hard time. Because we can't stand to see her suffer in this pain and I am tired of the trauma it's doing to her every couple days (I won't go into details... just know this poor kids bottom is a mess :( 

When we left Cincinnati the last trip, I came home with a heavy, heavy heart over the wheelchair conversation. I continued to fight it for a few days until I accepted it. Now that it's been a little over a month, I am recognizing all the times and opportunities that Annabelle does need a wheelchair. At school, she only lasts about 2 hours until she's pleading to come home, and when she does, she goes to sleep. At home she plays well, she runs and jumps, but when the height of fun is over.. she crashes harder than anything you've ever seen. Annabelle is 4.5yo and she wants nothing to slow her down.. but this disease is doing it. I am watching the works of how this disease destroys lives, in slow motion, I am watching it take down my baby girl. It's paralyzing to witness something slowly evolve into something you hate, and you can't do anything about it. I am just grateful that she's young, she's resilient, she's a bull when it comes to being weak, but she's adaptable and will take pride in her 'wheely-chair'. The stigma of being in a chair isn't part of her mindset, yet. She has an opportunity to embrace and fall in love with all the things her wheely-chair can do for her, and how much independence it can give back to her, well before she learns that being in a chair isn't ideal.

On that note, the reason we are going back to Cincinnati to the Wheelchair Clinic, is that I've hit roadblocks in Richmond. They want her assessed by all their own physicians, tests re-run and performed over again, and their doctors to determine whether or not she needs a wheelchair. After that, I will be assigned OT/PT and we can move forward to scheduling a date to discuss chair options. And since we're talking about Richmond, that means were talking months and months down the road. First and foremost, I am NOT putting Annabelle back thru any more testing. She's done all the leg work she's needed to do. We put her thru tests every several weeks in Ohio for other various reasons, I won't force Annabelle to be subjected to another doctors series of tests. I'm also not paying or making my insurance pay for it. Not happening. We have the BEST physicians in Ohio that have made their decisions, collaboratively together, for Annabelle and we support their opinion. I'm not jumping thru Richmond's hoops so they can get their money out of us and traumatize Annabelle any more than she already has. Plus, I trust Cincinnati Children's more than ANYONE in Richmond anyway. So there's my rant on why, once again, I hate Richmond and we're carrying her right back to Ohio to pick out her wheely-chair.

Life is wild. It's hectic, busy, frustrating and painful, but it's a blessing. Our family has been thru many things over the years with Annabelle, and we recently climbed yet another hurdle - thru all the hurdles and climbs though, one thing remains. We are blessed, we are loved and we are strong. Together, our family can challenge and overcome anything in our path.. even when we don't see the path ahead, somehow we find it. Madelynne reminds us every single day, how blessed we are. Annabelle reminds us every single day how valuable life is. Her little body has fought more battles than you can imagine, she has disproved doctors over and over and over. She continues to wear a smile and the best laugh, even when her little body is hurting and aching all over. She is wise beyond her years and speaks a medical language that most children should never understand. She is growing to become an advocate for herself and stand proud despite the world pulling her down.

She is so brave. She is so smart. She is so sweet. Annabelle is such a blessing.

I told her a few days ago, "Annabelle sweety, I made our next appointments for Cincinnati and we will be leaving in a few weeks." Her response, for the first time ever, was: "Okay mommy. I can't wait to see the bridges over the water, see Dr. Putnam 'oh! maybe he can change my tubby when he gives me the sleepy medicine so it won't hurt!' and maybe, mommy maybe they will tell me what is wrong with my body and why it's broken with so many boo-boo's inside..". I told her, "Gosh hunny, that would be really nice, huh?! I hope they can give us some answers this year, probably around Halloween time, we will do our big tests and they can tell us.... Are you scared of this next trip, do you have any questions?"

"No mommy. I'm not scared anymore. I know they are going to poke me and it will hurt, it always hurts. I know we're going to have to see a lot of doctors so we should bring my kindle so I don't get bored. But I know you and daddy have found the best doctors for me and one day it won't hurt anymore. I can't wait until I can be like sissy and my friends, and play, and go to school every day. My doctors are going to fix my booboos and I am going to be just like everyone else.

Don't be scared mommy, I'm not scared."

The conversation while driving down 295 on the way home, made the world stop. Everything was silent around us and I couldn't speak, I couldn't breathe. I pray a miracle heals her entire body, but the reality is that this disease will continue to manifest, I pray she finds peace and happiness and that her body aligns with whatever she defines as 'normal', I pray her heart and the love and happiness that fulfills it, lives forever!! She is heeling and saving us more than we are for her some days. Count your blessings every single day.

Sunday, February 14, 2016

Tuesday Hell

Monday Night, we slept like logs. In a king size bed, both us girls used every one of the 6 pillows and every inch to spread out.. The only problem was the alarm that woke us at 630... Daddy was the alarm clock, I answered the phone and mumbled "call back in 15".

We quickly packed our room, checked out, stored our luggage and caught the shuttle by 715a. First appointment of the day was..


I've had reservations about this visit and whether or not they would be a good fit for us. Upon checking in, they handed me an iPad and ask that I complete all the surveys on it - with each question, I found myself more intrigued and proud that we have landed here. "Does the child tire easily even when they've gotten enough sleep? Does exercise tire specific areas of their body? Does your child complain of weakness in their legs? Does your child trip or fall more than usual? Have you ever noticed anything unusual about the way they walk?" I answered the survey over 20min and found myself having to expand on about 75% of the questions. That brought relief, maybe Neuromuscular will be a good visit for us after all.

The doctor was amazing, she asked questions and built on them to enforce accuracy in how I answer. It's really hard to answer vague questions with precision, I don't want to mislead but I also don't want to avoid a question that may guide the doctor towards the right train of thought.. Make sense?

For example, "Is she getting better or worse?"
Answer: In comparison to where she was at 2 years old when we didn't think she would survive, she's much better. In comparison to 6 months ago, she's worsened. Her general overall health is better than it was a year ago, but the isolated areas in her body, such as her lower half (hips legs / endurance and fatigue) are considerably worse. She's fallen down our steps three times in a month. She trips over nothing because her knees buckle and she's falling asleep quickly after any amount of exercise. So is she getting better or worse? It's a hard question.

We talked for quite some time.. the longer the conversations, the better assessment i felt she was able to get of Annabelle. Finally, it was time for Annabelle's physical assessment.

They jumped, stood on one foot, ran down the hallway and climbed stairs together. After the 5min of working, Annabelle asked to go lay back down - and she did. She checked her reflexes, muscle strength across her body etc.

The doctor, I believe was able to gain an excellent assessment during this visit. She recognized the concerns we have of Annabelle before I was even able to communicate them. She soothed me by explaining that she sees MANY EoE patients, bc often times their pain and weakness is part of their undiagnosed global diagnosis, just like Annabelle. She also looked up from her writing, mid-thought and smiled, "Mom, I know this is hard, but you are a great advocate for your daughter. Don't let that go". I can still hear her tone and see that smile now. I rarely get a compliment like that from a doctor... those words bring more of a relief than I could ever describe in words.

The first plan of action is to capture a lab draw after fasting. The issue however, is that Annabelle cannot fast without being impatient bc her body doesn't tolerate it and her nutritional needs are extremely strict to avoid seizures, etc. Therefor, I plan to perform this lab draw during our next scheduled scope when we fast for two days before surgery.

These labs will look for:
  • Cogenital Myasthenic Syndrome - "The muscle weakness typically begins in early childhood but can also appear in adolescence or adulthood. Facial muscles, including muscles that control the eyelids (Annabelle's left eye only partially opens), muscles that move the eyes, and muscles used for chewing and swallowing, are most commonly affected (slow GI top to bottom). However, any of the muscles used for movement (skeletal muscles) can be affected in this condition. Due to muscle weakness, affected infants may have feeding difficulties. Development of motor skills such as crawling or walking may be delayed (she crawled at 11m and walked at 16m... not too terrible). The severity of the myasthenia varies greatly, with some people experiencing minor weakness and others having such severe weakness that they are unable to walk." 
  • Muscle Metabolic Myopathy - Metabolic myopathies are genetic diseases, usually inherited, that affect the body’s muscles. Metabolic refers to chemical reactions that provide energy, nutrients and substances necessary for health and growth. Some people with a metabolic myopathy (muscle disease) develop weakness; others tire easily with exercise or physical activity, suffer muscle pain after physical effort, and/or experience severely swollen and tender muscles. These symptoms occur when muscle cells don’t get enough energy. Without enough energy, the muscle lacks enough fuel to work properly.
If both these tests are negative, we will move forward to performing muscle biopsies.

The doctor seems confident our diagnosis is going to be either Metabolic or Mitochondrial. We've heard both of these for years.. Our genetics doctor agrees, as does our GI. We just need to confirm it.. And confirming either of these is really, really tricky and time consuming. She has all the signs, shows all the same patterns and shares the ailments of both diseases. When I say my prayers though, I pray with everything it's wrong. I don't want either of those labels, not on my Annabelle.

After that appointment, we walked the halls and made our way to Rehab.

Rehab felt a lot like neuromuscular, we discussed her fatigue, weakness, limitations and how all these things impact her day to day life.. and ours!

I thought our conversation was going to center around ways to help build her endurance, tricks to make life easier for belle, improve her muscle strength etc. the moment our therapist began saying words like "push chair, wheelchair etc" I froze.

"No, no no no.. Annabelle walks fine, she can play and does dance class! She isn't a kid that needs a wheelchair, not Annabelle. We just need to find a better way to handle school and physical activities, that's all."

She asked things like "does she tire when you go to the store? How much has she walked since being here? What do you do when you go to the mall or park or zoo?"

My heart sank to the bottom of my stomach and I immediately began scanning the room for both a trash can to vomit and tissues to cry. I felt like I was being cornered .. I felt just like I did when a doctor from Johns Hopkins stood over me and lectured me on how malnourished Belle was and how I was hurting her by avoiding a feeding tube. I remember how badly I fought that tube, but I love it now. It's saved my daughters life and helped our family tremendously. But a wheelchair?! No, that's for different people - not us!

I fought, hard. I debated, tried talking my way out of the appointment.. my head flooded with every excuse to avoid the conversation. And then the rehab doctor said, "Let me confirm something / or get resources (something like that... i was just hoping she would leave). That's when she came back and said the Neuromuscular doctor (WE JUST SAW!) is also recommending a chair. She then advocated for Annabelle in such a way, I knew I was wrong, I needed to stop fighting and allow what is best for Annabelle to happen. I was defeated as I looked over at sweet Belle..... she was laying on the bed and staring at the wall, utterly EXHAUSTED from the 15min of walking around we had done prior.

I bowed my head and answered the doctors questions.

At times when Annabelle's class is playing in the gym, running and hanging fun - Annabelle lays on the bleachers and cries because she cannot keep up or play. She can't physically run or walk that much anymore and it's progressively getting worse.
- A wheelchair will give her back that independence back, that she can keep up with her friends and remain included in the fun instead of constantly sitting on the sidelines, recognizing how vastly different she is (the result then trickles down into her diagnosis of depression..)

When our family goes to the zoo, mall or park - AK and I break our backs by carrying her most the way. I've spent the past two days, literally carrying Annabelle 90% of the time bc she can't walk this much. The reality is that she's only getting bigger and heavier. I don't have a stroller that fits a 40lb 4.5year old.
- A wheelchair will help our family continue to take trips and make memories, and not just break our backs and plead with Annabelle to walk on her own (which then leaves her feeling defeated and a disappointment when we get frustrated with her bc she doesn't walk on her own).

A wheelchair is not, by any means, permanent (maybe to the doctors, but not in my book), or a solution for laziness. Hopefully we won't use it daily, but it will save us on the trips and times she struggles and does need it.

We plan to purchase one that SHE can use on her own and we can also push if needed. It's her lower body (hips, legs, knees and joints) that don't allow her to keep up. It's also extreme fatigue, low endurance/energy etc. therefore we needed something that she can use when she tired so badly that she cannot move, and also something she can use for her own independence when she just cannot walk or keep up.

The conversation still, was not easy. I wanted AK with me. AK warned me this day was coming and I didn't want to hear it from him either. I know he would have received this news better than I was. That being said, I couldn't shop or discuss wheelchair options while we were there, I want to do this with AKs opinion in mind too. Shopping for one of these puppies is like shopping for ......... Fabric? A trillion styles, types, textured and then colors and patterns. Every single chair they showed me, the only thing that ran thru my mind was "nope. Not getting it. Nope, don't want that either. Oh pink! ?! .. Nope, still don't care. No wheelchair." I took a prescription for the chair and will shop for one in Richmond instead. (My pathetic way of winning that battle lol).

I will say, Annabelle is excited about it. They explained to her how wonderful it will be to have a chair she can sit in and still have fun and play when her legs are too sleepy. Once they told her she can have a pink glitter one, she was sold. She calls it her wheely-chair, and wanted it immediately. To the point every time we saw a child in a wheelchair in the hospital for the rest of the day, she asked "Is she sitting in MY wheely-chair?!"

After the appointment wrapped up, I needed to escape. We packed our things and left as quickly as I possibly could. We had lunch and headed back to the hotel to pickup our bags and enjoy a few hours by the fire and not in a frickin hospital. While Annabelle napped on the lobby couch, I collected paperwork and legibly updated my notes so I wouldn't forget everything that had been discussed.

130p we headed back to the hospital for our final appointment appointment of the trip, Neuro-psychological Evaluation follow up.

This appointment was actually gentle. I think all that we had just been thru of the day, I took this one the best. It also helped that I had already read the final report for the eval and knew what to expect from the conversation. She explained the evaluations findings and what that meant for belle overall. She then explained for me, what depression and anxiety are like for a 4 year old and what contributes to them: prematurity, her overall health and medical issues. It's like a Venn Diagram.. when you suffer from all those things like Annabelle and they begin to overlap one another, you begin to psychologically suffer.. Annabelle has never been taught how to process all these things that are happening in her life, and when she does attempt to do something, she fails (ends in pain, confused or bad behavior = timeout).

I asked the Psychologist about ways we can help her work thru them and which things were in my control and which weren't. (Medical issues and impaired brain development from prematurity, malnourishment and years of drugs). The ways I can help her are mostly thru positive encouragement, structure and coaching with very very small expectations, then working our way up. Basically, set every situation up in a way that she will succeed. In life right now, most all situations Annabelle faces, she will fail.. we will work to shift that.

We discussed ADHD and what that means for Annabelle. My main concerns are how shes progressing academically. The fact is, she isn't. The neurologist says that if we place belle in kindergarten. The risk for causing additional anxiety and adding to the depression is between a moderate and high risk. In the wrong setting (not structured but high academic expectations), we would only set her up for failure. That being said, keeping her in the same preschool program also may not help and support moving her forward academically. We're just kinda between a rock and hard place. My approach: shop. I'm going to shop for the best fit of education that encompasses a highly structured schedule (for her ADHD) and lower academic expectations (for anxiety and depression). With this right combo for Annabelle, I trust she'll excel in school. I just need to find the right fit.

This appointment lasted an hour and we discussed SO much. I am able to understand my little girl on a level I never did before. I understand how she thinks, why she frustrates, why she handles things the way she does and also recognize how our parenting is helping/hurting her. Parenting is never a one-size fits for all kids, I know that, but Annabelle seems to be a curve ball we REALLY weren't able to understand until now. What a priceless gift this doctor was able to give us. (No wait, not priceless.. Im sure I just paid an insane amount for that, but still). I left with a lot of resources and homework to better understand and shift our parenting.

After we were finished, Annabelle and I headed downstairs to await our scheduled shuttle pickup for the airport. I purchased Annabelle a gift in the gift shop (tiny plush toy) for being so good during our trip. We took our seat in the lobby and exhaled. Finally, WERE COMING HOME!


Flights were a nightmare. Not much else to be said. They were all delayed. The snow was coming down sideways in huge clumps. At one point on the plane, I didn't know if we would even be able to make it out. Our plane was de-iced and the runway was being plowed as fast as they could move. The weather was awful. Annabelle was awful. My anxiety was awful. We just wanted to be home so bad! Our layover in Philadelphia flight was not much better.. equally frustrating, delayed, bad weather, plane de-iced etc. but we were able to fly into Richmond vs Reagan, so that helped tremendously. We made it home shortly before midnight and everyone fell asleep, in our own beds, and again slept like rocks. Whew, the marathon was over!

This trip was hard. Probably the hardest trip we have taken. I don't know if it's because of the sheer amount of running, walking, appointments, shuttles, coordination involved or if it was because of the information we learned was simply so hard to accept. This trip was the easiest for Annabelle. I made every step of the way exciting and fun for her. She has no idea how stressed and upset I was (well, she may a little, but she wasn't phased). All Annabelle kept saying once we made it to the airport to come home was, "5 appointments, FIVE mommy, and NO POKES (needles)" she was so stinking proud and happy. This was a victory for her in the course of our trips to a hospital. Honestly, I can't recall a time when we went to an appointment and there WEREN'T any pokes. She's become conditioned to associate a medical appointment with pain, and this trip allowed her to see that not all hospitals, appointments and doctors are bad. I am so grateful for that.

The trip has emotionally taken a toll on me. I am fighting an internal battle with the acceptance of a wheelchair.

let me pause for a second.. I KNOW this is not the end of the world. I KNOW children and people have it SO much worse than we do. I KNOW it could be worse than something as materialistic as a chair. But it's still hard, as a parent, to hear and also accept. So please, do not roll your eyes that I am being dramatic for no reason. This is hard. 

I fought our feeding tube like it was a war. In my mind, I thought a feeding tube meant the end of life (and at the time for Annabelle, we were there). I thought if we placed a feeding tube, she would never be able to eat on her own again or never have the opportunity to do so. I feared the emotional trauma it would cause her as peers, children and adults, gawked at the tube taped across her face. I feared that if we placed a feeding tube, it would stay forever and ever and never come out. I hated how permanent it was.

That feeding tube saved my daughters life. In the end, I fought an unnecessary battle that only hurt Annabelle and prolonged her malnourished. I will never, ever, forgive myself for that. But it was hard. HARD! In my screwed up, exhausted, defensive, 150mph mind I thought accepting a feeding tube, meant saying goodbye to my baby.

I didn't say goodbye to my baby, but we do use that feeding tube. We rely on it much more than I would have wanted to know before it was placed. Go back 2 years and I would be so upset to see my daughters tube and her feeding needs now.... but it isn't a bad thing! It was a learning curve, yes. It's inconvenient in many ways, yes. It's expensive, painful, constant maintenance and the supplies takes over my house, yes. But I would be LOST without it. I am SO grateful for that tube and what it's given to Annabelle.

I'm in the same place about this wheelchair. I don't think she needs it.. but deep down I know she does. I don't want it.. but I know it's necessary. I am making myself artificially accept this next wave as our newest adventure and necessary blessing. I hope one day that I can sit back and reflect, "oh, remember that time I hated the thought of a wheelchair? What a fool I was, we LOVE our wheelchair!" In the meantime, it's going to be exhausting, expensive, time consuming, space consuming and a learning curve all over again. But if it brings life and joy to Annabelle as much as that tube did .. we will be ok.

I keep reminding myself,

Doctor's do not place feeding tube's in children that don't need them.
Doctor's do not put children in wheelchairs that don't need them.

If I have ever trusted a team of doctor's more, they are the ones in Cincinnati. I trust them. I know they are doing what is best for Annabelle. And we are SO blessed to have the opportunity to be part of their hospital, research, care. Every element of it, this week... sleep clinics, neuromuscular, rehab and neuropsych.. what a blessing they all are. They are giving my daughter the best life she can possibly have.