Monday, November 24, 2014

"So she just has a cough, right?" -NO.

This cough is getting worse.

Annabelle's appetite is depleting, she is hardly getting any rest during the night, she cannot nap during the day and she's utterly exhausted and in pain from the chronic cough. I spoke with our doctor Friday and pleaded for help. She did a lot of studying and reading on the treatment of EoE and agreed with the doctors in Boston that perhaps, our treatment plan as decided from our doctors now, is not the best treatment for Annabelle and her current state.

We decided to immediately switch her from swallowed steroids to an inhaled steroid and also add inhaled albuterol. In addition to those, we reviewed her current PPI and added Singulair Granules to her daily drug cocktail. Saturday progressed similar to the days prior. Persistent cough that became increasingly worse as the evening went on.

Finally, late Saturday night, Annabelle entered another coughing fit that led to painful retching. I couldn't run to her bedroom fast enough before I found her sprawled on her hands and knees in her bed gasping for air and retching. Madelynne followed me asking if her sissy was ok. I ordered Madelynne to run to the Tubey Closet and get be a really big syringe that we use to make bubbles (60cc). She hurried off as I held Annabelle in the best position I could for her state, and continued to calm her down between gasps for a breath and coughing / choking. Mady came back to her room with tear-filled eyes and a 5ml syringe. That wasn't nearly large enough so I told her I would go get one. She was shaking like a leaf and I quickly told her, "Everything is going to be ok Mady, sissy is just having a hard time catching her breath". By the time I stepped back into Belle's bedroom, Annabelle let out a huge scream and began shaking all over her body, she then started vomiting (VOMITING) everything off her stomach and into her bed. Vomit, her last feed, stomach bile, small bits of food, and numerous streaks and traces of blood. I was absolutely terrified. Mady started yelling and pleading for an explanation for why Annabelle was throwing up. Annabelle CANNOT vomit due to her Nissen. She hasn't been able to vomit in almost a year because of her Nissen (where her stomach and esophagus are sewn together). She hasn't even managed a burp in almost a year, let alone a drop of vomit. I knew something wasn't ok. I knew she tore or opened her Nissen, but I didn't know exactly what to do. Annabelle was exhausted. Her stomach was finally relieved from all the pressure that wanted to escape and the coughing spell was finally over. She was in terrible pain, but so so tired. I cleaned her up after about 5min of laying in vomit so she could catch her breath and wipe her tears. Mady asked me repeatedly "If we need help mommy. You should call 911. I can dial the numbers, but can you talk to them because I don't know what to say?" Bless her heart. She was scared to death. Annabelle was scared too, but more in shock than anything. I was terrified and clueless as to what we should do in the middle of the night, alone with two girls. (Daddy wasn't home that night).

Sunday progressed normal but again, as the day went on, Annabelle began to get worse again. With each cough she would wince. Her appetite was nearly gone, I just know it hurt her to eat. Her feeds were fed extremely slow by a trickle rate, if that. No bolus or fast feeds. I spoke to our doctor multiple times again and told her that my gut just isn't allowing this to sit well. Something is telling me that she needs to be seen but I don't know what to do. More than anything, I was scared to death to enter another night when I know the nights are the worst for Annabelle. Laying down is excruciating, and I know it will be even worse now with an opened Nissen. Finally, shortly after dinner, Annabelle's cough and choking began again. Daddy was home by that time and was worried, he finally turned to me and said "we have to do something - this isn't ok". I knew what I needed to do. But everything inside me wants to protect Annabelle from what I know will happen the moment we walk into a hospital.

I told AK that I would let the doctor know that it's time we go in so she can give the hospital a heads up. And then I went upstairs to take a shower. (The things you learn to prioritize when you live in a hospital.. take a shower and pack extra clothes / yoga pants and caffeine, because you have NO IDEA the next time you'll actually be able to shower.. oh, and pee. I've learned my 18hr less from that one too.. when I enter a hospital with my baby, I will never, ever ever ever leave her for a SECOND no matter the need. Unless she's being taken from me for a surgery or procedure, I would never ever leave her to shower or pee. Never.)

Once I was showered and changed, I packed Annabelle's favorite things and blanket (gigi) and picked out the most comfy jammies I could find so she would stay warm. When I made it downstairs, she asked where I was going and AK told me to Shhh... I can't do that. I got down on my knees and told her, "Annabelle hunny, we are going to the hospital so the doctors can help your yucky cough. ok?" Oh, the heartbreak that tore across her face was enough to wound even the strongest of hearts. She begged and pleaded to not go. She ran to the kitchen towards the neubulizer that she FIGHTS and promised to take her medicine. "I do tweatment mommy, i pwomise, I do tweatment and make cough go away, i pwomise!!!" My heart broke and AK saw it in my eyes, she scooped Annabelle up and told her everything would be ok. Mady ran to her sissy and gave her a big hug and kiss goodbye and so did daddy before Annabelle and I headed out the door. The rainy drive to the hospital was full of 2046107636 questions from my backseat. Finally, the doctor called and we discussed mutual concerns and she confirmed she had called the hospital, that they were expecting us.

The hospital on the other hand was terribly uneventful. I explained on as high of a level as the doctor needed to hear about Belle as he stopped me multiple times , "So are you here for her cough?" No. We are here 1) We believe her nissen is torn. 2) she has an auto immune disease that doesn't allow her body to present symptoms as they should when she's sick. 3) Her appetite is depreciating so quickly that her diet is becoming dangerously unsafe based on her history. 4) Her persistent cough is progressing worse as the days go by. 5) I NEED HELP! I can't continue to do this alone at home with this child and watch her crumble and drown, something has to give, we need help.
Doctor: "So I hear and ca see, she has a cough?"

:eyeroll:  Can you at least make sure the nissen isn't causing damage that needs to be addressed immediately? I gave the doctor my doctors personal cell number and asked him to call her, as she was waiting by the phone to answer any/all questions that may arise while we were in the hospital. Maybe she could explain our concerns better than me. And better yet, maybe they'll be received more respectfully than just a mother with a child 'that coughs'.

Finally, he ordered a chest x-ray. Annabelle did WONDERFUL! She's getting quite used to xrays. She kept her hands on her hips and help perfectly still. And then she faced sideways while I held her 'skirt' and she kept her hands on her head with a "Big smile for the picture" :) No tears or hesitation. She did wonderful.

The chest xray didn't show pneumonia (thank god) nor did it show significant pockets of air around her diaphragm where her nissen had torn. We consulted with the surgeon about the nissen needing to be repaired immediately and all agreed that wasn't necessary as long as she didn't have heavy internal bleeding or air pockets from the rupture. (Again, thank god).

Our doctor demanded an answer from labwork. The ER Doctor replied to her and explained to me, "Even if we do labs, it's still not going to change my course of action. Annabelle looks great, she just has a cough and I'm sure it's just a cold mom. Again, she looks great and healthy to me." My eyes could burst from my head. I thanked them for their time and scooped my baby up. Between every word the doctor spoke, he would have to pause and looked at Annabelle as she coughed her little head off and tried vomiting while wincing from pain. How someone can look at a child in that condition and tell me she looks great, is beyond me.

In the hospital's defense. They cannot do any medical procedures or tests on pediatrics as they do not have a peds unit. If they were to need to do anything beyond 'stabilizing her', they would Med-Transport her to another hospital where they could better conduct those tests. I guessing that's why they were so reluctant to help in any sort of way.

When I spoke with our doctor after leaving, she couldn't believe that someone had the nerve to say 'she looks great'. We understand Annabelle is quite a mystery and doctors just simply don't know where to start when it comes to helping her, but for the love of Pete. Annabelle still deserves some mercy and help when she's struggling right in front of you. And yes, she has weight on her - purely bc she's terribly malnourished and has also been on prednisone twice daily for 6 months. Ya darn right she's going to be chunky!" that doesn't mean she 'looks great' though. 

The next morning we took Annabelle to see our doctor and perform labwork. What do you know, with zero fevers and absolutely no pain or complaining, Annabelle has fluid in her ear and an ear infection. Which I am certain contributed to the upper respiratory infection that she's fighting and the ER never caught. Thank God for pediatricians that know, love and advocate for this child better than over-paid doctors do.

After multiple conversations with doctors yesterday, completion of 6 trips to CVS in less than a week, another appointment from the Home Health Staffing company and lots and lots and lots of kisses, bedtime stories and episodes of "Peppa Pig", Annabelle is feeling ok. She is quite groggy, irritable and the cough isn't getting better yet but hopefully it will soon now that she's on antiobiotics. I am waiting to hear from our surgeon as to how they want to proceed when it comes to the Nissen repair. A nissen is a major surgery and a terribly painful, several-day impatient stay for Annabelle. I am not looking forward to it, but I would prefer something to be addressed sooner vs later, as Annabelle is drowning each and every time she lays down or eats/drinks to quickly. Something needs to give soon, very very soon.

Thursday, November 20, 2014

They want me to feed my baby

We’ve gotten our report from the hospital when we traveled. Both the labwork and the written report reflected what the doctors stressed while we were there; Annabelle is extremely malnourished and her body’s internal organs are suffering the consequences. Immediately, we need to figure out how to nourish her, but as anyone that has followed Belle’s story, this isn’t nearly as easy as just a trip to the grocery store… Let’s discuss the painful position of being between a rock and a hard place.

At the end of the day, the words that continue to ring in my mind from the doctors were these, “If your primary focus is finding a diagnosis, and we continue this treatment plan and keeping her malnourished; by the time we finally reach a diagnosis, we will have a ‘vegetable’ on our hands with a destroyed body we can no longer repair.” I never understood the severity of severe malnourishment until Annabelle. I never knew the multiple systems and invisible damage it does to the body. I never respected how dire it is for a toddler to be nourished as their brain and body is rapidly growing. All these things are easy when you have a ‘healthy’ child. You feed them! Sure, sometimes they eat chips for dinner, but they’re fed and their bodies have something to break down and use. We wish to believe that the healthcare system is steering our children in the safest directions when they tell us to stop food and put this Medical Food liquid thru a feeding tube – we would like to assume they know what they’re doing and this is safe for our children. Follow doctors orders, right? Hell no. Ive fought for years that something didn’t feel right with Annabelle and her pathetic ‘diet’, I’ve fought for the last year that this isn’t nearly adequate and my voice falls absent on those that have the knowledge and ability to make changes.

Yesterday I spoke with our doctor again, the doctor I trust the most and has been the biggest advocate for Annabelle. She reviewed the physician notes and labwork and told me, “Ashley, go feed that baby.” I was speechless. When I saw speechless, I honestly mean I was lost for words all the while I had 39236354 questions and back-peddling from what she just said. I looked down at Annabelle who was mesmerized by the choo-choo train, sitting in daddy’s arms as we all stood in Home Depot. I couldn’t breathe. I told our doctor that I was so nervous to feed her. Im not scared of food, or her choking, and I am only slightly intimidated by the guaranteed EoE relapse, but more than anything – I’ve never fed Annabelle! It’s been since she was an infant was the last time she actually ate an array of foods! She won’t know what to do with herself. 

And then the fear really sank in.. Oh God, what will happen if she relapses too hard to control and we have to remove ALL foods from her diet. We will have to put her AND us in a mental hospital. I don’t know how I will be able to do that again. It’s taken us so long to work to our lifestyle where Annabelle doesn’t ask or expect the same foods as anyone else. I am about to ruin all that hard work and tears shed. She’s too young to understand what were doing. Good gracious I am terrified.

This was late that evening at dinner. Mady loves to open and eat peanuts. Annabelle just watches with no questions asked. 

So. We are going to feed her. I don’t know which foods we will begin, but I will decide sometime over the weekend. If you were to ask Annabelle, she would request a chicken nugget ;)

Monday, November 17, 2014

Premature Awareness Day

When you find out you're expecting, your mind floods and heart races. You feel like you could burst from all the love you feel inside. The emotion and joy is indescribable.

When you lay on an operating room table, awaiting to hear the fate of the premature baby being torn into this cruel, cold world, far too early; your mind races and heart shatters. The fear and pain is indescribable.

Today is prematurity awareness day. I never would have imagined this would be a day my family would celebrate, but it is. It's a day that we look back at those first terrifying hours and days living in the Neonatal Intensive Care Unit and learning how to care and fight for our daughter. For me, it's a day I reflect and wish to thank all the nurses and staff that loved and cheered Annabelle on and hugged us as we finally left the hospital with our daughter.

Someone told me while we were in the hospital, "these days will fly by and you'll forget all about it." They were right that they flew by, but I haven't nor will I ever forget the impact those days had on us.
I'll never forget the moment I met Annabelle and she was hooked to so many tubes and monitors that I didn't know how to touch my own child.

I'll never forget the sinking feeling in my gut as the first time I saw her and felt as though my body had failed my child. 

I'll never ever forget the cries I heard come from her tiny voice as they repeatedly stuck her every limb for another IV.

I'll never forget the lonely, empty hospital room.. because after all, people don't visit you when there isn't a baby to see. 

I'll never forget the ride in a wheelchair past the nursery to the elevators as I was leaving and the waiting room was crowded of happy families... And I had empty arms.
I'll never forget the emptiness I felt when I left without my daughter. 

I'll never forget the quiet house, long shower and alarm clock breast pump sessions as my child slept 35miles away from me, alone. 

I will never ever forget the day I brought my daughter home to complete our family. The fear, ohh the absolute terrifying fear of a preemie is nothing like the excitement of a newborn! It's pure fear!

Every single milestone is adjusted and weight charts are meticulously measured. More than anything though, are the nurses in the NICU that are sent directly from heaven above for premature babies. They are a work and practice all on their own for the miracles they do for the smallest of little people. I have no idea how it's possible to find such incredible and loving people in each and every NICU setting, but somehow it seems as though hospitals do it. Ours was no different, Johnston Willis hospital NICU was our home away from home and daughters first set of guardian angels. 

Today is premature awareness day and my Annabelle Grace is one of the strongest fighting preemies I know... I made her myself ;) 

Eyes, Tubey & Medications, oh my


Annabelle’s eye sight looks good, as does her overall eye health which is a huge relief. The appointment lasted a few hours and both girls had their eyes dilated, which was difficult for them both and us as we continuously calmed down their panic because their vision was blurred. Madelynne was upset that she couldn’t color within the lines and Annabelle fell out of her chair and onto the floor twice. We prayed the time would pass quickly and we could leave, before we knew it we were called back to see the doctor. She noted some slow/lazy blood flow within Annabelle’s eyes but nothing that should cause us concern right now. She does have some signs of being farsighted but at this point, we are just going to monitor it unless something changes.  We will continue visiting yearly due to her diagnosis, nutrition and mostly the seizures and neurological components that are associated with her general health. But for now, we are very pleased! 

I love how the doctor was asking her, "What shape is on the screen?" and if Belle didn't know, she would respond by whispering, "something".  funny kid!

Surgeon / G-Tube Check-up

Our appointment was shuffled multiple times, something was going on at the hospital and we kept getting bumped from our appointment time. We arrived  15 minutes early and by the time we were seated in waiting, we were told the doctor was running about an hour and a half late seeing patients. At this point I looked to my left and counted all my blessings that we had our nurse with us. My nerves were fried from the anticipation of this appointment.

I told Annabelle on the way to the hospital, “Sweetheart, we are going to the doctors today.” She asked why, “The doctor is really nice and going to look at your tubey..” this is about the time she started to panic and negotiate with me that her tubey is fine and no one needs to see it. I didn’t want the surprise to come when we got in the hospital room. I don’t like ‘surprising’ Annabelle anymore.. I don’t think it’s fair or helpful to her nor myself. Then again, I’m not a child psychologist either. But I am a mom, and I do know my child like she’s the second part of myself. I have been the only person beside her for each and every single procedure she’s ever endured. And I have come to the realization; Annabelle is smart. She’s wise beyond her years about the medical world. She is beginning to understand far more than I thought possible about her body and disease. She has owned her g-tube. I promise to stay honest with Annabelle when she asks even the most difficult questions, even if it means my heart crumbles to say the words, I will not lie. The conversation though, is still difficult. For both of us it’s hard. I HATE telling her what is about to happen just as much as I hate seeing it happen or her hearing it’s going to happen. But the amount of questions and understanding she takes from our conversation is priceless to the journey Annabelle is on. She knows that no matter the amount of pleading and begging, that I do not have the ability to say no to a planned appointment or procedure, so she doesn’t test me or expect that from me (thank God).

I asked her if she remembers the last time we changed her tube, and she did. She reminded me that mommy hurt her tubey and it got stuck. I told her the doctor today will change her button out and we will get a new one. She can carry our new box and button to the doctors office and give it to the doctor if she would like. I explained that the doctor needs to see her belly and check to make sure everything looks adorable. She asked multiple questions like, “Is she going to hurt me?” I told her, “I don’t know. It may hurt, or it may not hurt at all. But it isn’t scary, I will tell you everything she is doing, ok”. She wanted to know if she was going to get another glow in the dark tubey, and I promised, “Yes mam! You sure are! and then you can show sissy what it looks like in the dark!”

My mom met us outside the hospital when we arrived and walked with us to the appointment. The nurse and I waited with Annabelle and talked about Christmas and all things festive until they called us back.

Annabelle was nervous, clearly, but she was happy to see our doctor. She talked about her Tubey buddy, Ryan and told the doctor what she wanted for Christmas. The doctor and I talked for a long time about the persistent coughing and choking going on – we considered many plausible explanations and she directed me to a clinic in Richmond that is now seeing EoE patients, maybe they can provide some insight to whats going on as well.

Finally it was time to change the tube. It went great. The doctor actually applied numbing cream to her gtube area and we spun the tube a bit to numb the stoma well before removing her tube. Of course Annabelle cried and fought us all holding her down, but I don’t believe it was nearly as painful or traumatizing it could have been. As soon as it was all over, Annabelle sat up and was fine. She was proud of her new button and told us all that it didn’t hurt (despite the shaky breathing and hiccups from crying). We will be ordering a new size though, Annabelle’s tummy has thinned out and isn’t as thick (chunky) as she used to be, therefore her tube is too loose unfortunately. Hopefully that will be shipped soon.

I didn’t leave the hospital until nearly dark and Annabelle fell asleep immediately upon getting in the car. She was exhausted from the day. That evening was beautiful in Richmond and we decided to all have dinner on the patio as a family for (likely) the last meal outside of the winter before the cold comes in. The girls love eating outside and we all love spending as much time outdoors as we can.

While the beginning of the week started off well, the ending of the week began heading downhill for Annabelle. The coughing and choking is getting progressively worse. AK and I are not sleeping much at all anymore because Annabelle cannot rest if laying down. At this point, she cannot even take a tube feeding without choking.. a SLOW feeding at that. I was done by Friday and finally called our pediatrician and pleaded for help over the phone. I didn’t know what else to do because we’re not getting the help or direction that we need from any of our other specialists. After she did some research, she learned that one of the MANY side effects of prolonged use of steroids, is acid-mucus induced reflux. I begged to come off the prednisone but don’t have a clue how to begin to do so. We came up with a great game plan and began transitioning Annabelle off swallowed steroids and onto inhaled steroids Saturday.
She is taking Albuterol and Pulmicort, inhaled via nebulizer around the clock now. 
She doesn't like the nebulizer at all, and considering she is using it at least every 4 hours ..... mind you, medications multiple times x day, G-tube feeding tethered to her IV pole / pump every 3 hours for 1hr duration, and now breathing treatments.. she is NOT interested! :(

Saturday night, she slept for hours for the first time in months without coughing. I was so happy for her (and us too!). The coughing is still moderately present but getting better. Her appetite isn’t picking up much and she has been extremely tired and exhausted, but fights sleep. (The evilness of steroid use). Our fingers are crossed this treatment plan will help address these acute issues and Annabelle can begin getting adequate rest soon.

We see the GI doctor and a Dietician soon to discuss introducing a food into her diet and setup a plan for scoping (endoscopies / biopsy's) after every 2 foods. We are also working fast and furious into getting Belle to a Rare Disease Clinic… everything is a Hurry Up and Wait game, but we are trying to be patient.  

Sunday, November 9, 2014

She's so strong

I want to say this and simultaneously knock on wood. Annabelle is doing very well right now despite the weather changing and everything she’s been exposed to lately. I am so proud of her immune system for toughing it out for two flights, a hospital, hotel etc. I knew for certain we were playing with fire by taking that chance but she did awesome! A few days after we returned, Madelynne unfortunately got sick and caught a tummy bug. Panic set in again as I recollected everything they both touched, possibly shared a sippy cup, etc.. while we kept the girls pretty isolated from one another, again, Annabelle did terrific and never came down with the bug! I cannot believe, I honestly am just blown away at how strong my baby girl is getting. Every winter sets fear, every illness could mean a trip into the hospital, every fever could result in a seizure, every time she falls asleep, I panic if she’ll waken as the same Annabelle. She is giving us so much hope right now. I could never ask for her to respond and fight as hard as her little body is but she’s doing it, she’s doing it with flying colors and a roaring attitude!

Speaking of roaring attitude. Whoever quoted the phrase, “Terrible Two’s” obviously forgot to mention the 3’s. Madelynne was always very well behaved, introverted, soft and kind. Then our Annabelle came along: fierce, ferocious, loud, honorary, defiant, incredibly loving, head-strong, stubborn, did I mention she’s always really loving and sweet? Annabelle is the full package. Every single ounce of emotion and opinion and attitude, all bottled into a strong 3yo that has every reason in the world to keep fighting. I always say, “if this hell were to happen to one of my children, I am sadly grateful it’s Annabelle because she has the most appropriate attitude for it.” She fights with everything she has, night and day. Whether it’s Annabelle fighting her own immune system or Annabelle fighting with her sister over a toy that doesn’t even belong to her, Annabelle knows how to voice her opinion and stand alone in this big, big world!

Our appointments are coming up very soon. This week will be extremely busy for our family but hopefully enlightening for things we need to discuss and address. Tomorrow Annabelle will see an Ophthalmologist to take a look at her vision and eye health. We need to gauge whether or not these fevers, drugs, seizures, malnourishment etc have done damage to her eye and vision. I pray she handles the appointment well. And I cross my fingers and toes we don’t need glasses!