Slow decline

Each day closer to Cincinnati, is another day further from the day we removed Annabelle from all her medications. Every day she is off the "bandaids" for the disease, she deteriorates even more. Annabelle is so strong and full of life, but right now - she's scaring us. She's in pain and she's so tired.

The stoma around her gtube is in terrible pain. Her little body is deteriorating on the outside just as quickly as it is on the inside. Any and all tissue is a target for this disease. We watched the stoma for a couple days until she couldn't take it any longer and we started pain medication. I also removed some fluid from the balloon of the gtube in hopes that it will lessen the irritation and pressure on her stomach wall.

Her body is becoming so very weak. Annabelle sways between sleeping long periods, laying down everywhere she goes, trying to have fun as a 3yo and play, crying and telling us her tummy and body hurts, or then... My least favorite, she lays motionless and stares into space with wide eyes. I fear what is running thru that little babies mind.
"Why is this happening to me?
Why can't I play like my friends?
Why can't I eat what my family does?
Why does my body hurt so badly?
What did I do wrong?
Why won't mommy and daddy make it better?
Why can't I just be like my sissy and friends?"

She lays silently with the saddest eyes and it leaves us speechless. All we know to do is lay with her. Running our fingers thru her curly hair and telling her just how much she is loved and how proud of her we are for being so strong.

Neurologically, Annabelle is showing us signs that she's declining. We are praying with all our might that she isn't. If so, there is a great chance that it is a result of the protein intake she's getting from her diet. Annabelle's body does not filter protein properly. Her body uses protein for energy and to grow, but the biproduct of protein in anyone's body is ammonia. Annabelle's liver does not filter the ammonia out the way ours does (thru urine), Annabelle's liver filters the ammonia directly into her blood stream.. poisoning her body. First stop, Neurologically (brain). Second stop, her heart and it continues to move on. It took us a long time to learn this was happening, at the time, Annabelle was having repeated seizures, not growing, thriving or talking, she stopped walking and had little balance. We immediately removed protein from her diet and the problems stopped. We have since added protein back into her diet because she wasn't growing, gaining weight or thriving - since adding protein, she has grown hair, finger nails and gained weight... unfortunately though, since coming off her medications, we are not able to balance that protein / ammonia balance as well and we are seeing a direct impact. Cincinnati and time will tell what is happening, our prayers are so strong that what we are seeing is not an impact from the protein (if so, there is a good chance she will loose it from her diet all over again).



She is stumbling and having a harder time with balance and control.
She tires very easily and is sleeping/resting for longer and longer periods of time.
She complains of headaches (her hair hurting while she hits her head).
Fortunately, it's been a few months since her last seizure.
Her left eye is beginning to droop along with her smile.

CINCINNATI UPDATE

Since last week's change of plans in our itinerary, we've exhausted ourselves trying to find the best way to get Annabelle to Cincinnati.She does not do well in a car for a long period of time. Her nausea during feeds at night and in a vehicle is miserable for Annabelle.. and 8hrs does NOT seem like a fun time for any of us to be in the car with her, Annabelle included ;)

The Answers for Annabelle Event is from 12-5p Saturday June 27th (I HOPE TO SEE YOU THERE!), we live a few minutes from the event so we will be able to get Belle back home to rest and snuggle Madelynne. However, we have to be in Cincinnati Ohio, and checked into the hospital by 10am the next morning. Our two options are to either fly first thing Sunday morning or drive Saturday evening after the event. I have reached out to Angel Flights and with enough prayers and fingers crossed, it looks like they are moving the process along in our favor. I got paperwork in the mail to sign over the weekend and I am hoping that is a good indication they are accepting Annabelle and will be able to help fly her to Ohio so we can avoid the drive.

Also - flyers are out and bracelets are available. The bracelets and event tshirts will both be available for sale at the event but if you want a bracelet sooner, let myself or guiltyonesrichmond@gmail.com know and we can find a good place to meet. They are 1/$3 or 2/$5 and come in various sizes. We do still need sponsors and vendors! If you choose to sponsor the event for Annabelle, submit your logo and we will make sure it'll be printed on the event tshirts!! Check out the Answers for Annabelle page here on the blog. I will continue to try to keep any information updated on that page for you all.