Calm before the storm

We are watching Annabelle closely right now. We are beyond thrilled to have an appointment scheduled for the immediate future, but so worried that 'immediate future' just isn't soon enough. We are praying and holding our breath as we watch her begin showing signs approaching another episode of hyperammonemia. Her ammonia levels are rising and we have no choice but to watch it happen.. .. and wait. We're waiting for the moment of crisis where we react, we're praying the damage again is minimal and her sweet little systems and brain are unharmed during the 'storm'. 

Annabelle has recently begun changing the presentation of her rising ammonia levels. Her body used to remain extremely lethargic, unable to walk, stand, or even stay awake for long periods of time. Her little body now demonstrates hyperammonia by 'agitation'. She appears to be on a high. It's terrifying to watch.. I would even say it's beginning to become more difficult to watch this reaction vs the lethargy. I don't know the little girl that is spastic, running in circles or figure eights around our home. The child that will willingly crawl in your lap to play and jump, but then melt down screaming in the floor within an instant for no reason. At first I embraced this change in behavior. It was the first time I really began to see my little girl smile, laugh and 'play'. But learning what is happening, recognizing why she acts the way she does during these times.. and realizing within seconds, hours or days she will slip into a coma or become unable to wake - there is no giggle or smile that is welcomed when it's followed by that behavior. A laugh and Ring-Around-The-Rosy game that ends with the potential of losing my daughter is no longer fun. The laughter become a noise worse than fingernails on a chalkboard. This illness is ruining every ounce of happiness in our home. Our little girl is fighting with everything she has and our family is searching for every cherished moment and memory but somehow, it always becomes fogged with the dark cloud that remains over our family. I hate this. I HATE THIS FOR OUR FAMILY! 

So right now we watch. We're waiting to react. We're praying we are able to scoop Annabelle and deliver her to hospital safety when the time comes. We wait. We hurry up and wait.

I absolutely hate waiting.

Some of our dearest friends live in Florida; we have dinner and 'skype party' with them and their two little girls often. All the girls love seeing one another between our yearly vacations together and all us adults enjoy sharing a meal and glass of wine via technology & 600 miles away.  

We love you Matt & Kristy! 

During this time... we don't sleep. How could we? How can you rest and count sheep when your little girl could be in the next room slipping away from you? 

9 Days ago Annabelle's decline began. It started with a rising heart rate & blood pressure. We keep an eye on her vitals daily and log everything. Each day its continued to climb. Her normal blood pressure should remain in the 90/60s and her's is hovering around 140-150/90s. I generally only see her blood pressure rise this high when she is in an extreme amount of pain. Her gtube site is still bothering her, but not nearly to the point I would consider it 'extreme pain', I would call it 'very sore/uncomfortable/sensitive'.   

She's lost over 3 pounds in the last week and is moving all her food quickly thru her GI system - she's having several BMs x day that contain whatever she's eaten in its whole form.  

Her energy is spiraling out of control, but reached a plateau by the weekend. 

Today, both AK and myself were off work and wanted to take the time to enjoy outdoors while it wasn't too chilly and before the snow arrives. We took the girls to the local park and Annabelle had a wonderful time. She out-climbed Mady on the jungle gym until she spyed the swings, her favorite! Both girls enjoyed playing, but within 10min of being at the park, Annabelle began heading downhill. She usually enjoys being pushed on the swings for ENTIRELY too long, but today she asked to get down. When I put her on the ground she stumbled. My heart skipped a beat and I cautiously smiled at my little girl. The other parents judging as I helped my daughter and her backpack, holding all her tube feeding supplies, walk the 6 steps to the next toy. She fell. My heart started to beat faster and my smiled disappeared. As I helped Belle onto the toy she wanted to ride, my eyes started scanning for AK and Mady on the other side of the playground. I panicked when I couldn't find him. Again, she was quickly uninterested and wanted to get off. She wanted to be held when I noticed her little arms were shaking.. I picked her up quickly to prepare for a seizure. The moment I got her into my arms she screamed to get down. She fell twice and couldn't stand. I scooped her up and started walking, AK was directly in front of me and knew something wasn't ok. I told him we needed to go. He collected Mady and we loaded the van in a casual manner without anyone ever able to grasp a hint that AK and I were both having full parenting panic attacks. I carried Annabelle with her head lifeless on my shoulder & AK raced Mady to the car while playing tag and I cheered Madelynne on. We were silent on the car ride home and both watching Belle like a hawk in the rear-mirror. . . . It's starting. 

Annabelle is home and resting, she will not sleep, though she will not run around either. She is quite monotone since this morning at the park. Her feeds are going well. Her appetite is the same, though not nutritionally important, she just enjoys eating foods. Her gtube site started to look much worse at the end of last week but by yesterday and today, I have hope we may be able to to avoid the surgeons office soon. I am praying my hardest to not have to pay them a visit.. I just know it will be ugly. 

For now, we hold our breath. AK leaves early tomorrow morning to go out of town so the girls are helping him pack. I'm taking advantage of being as lazy as I can while I still have the extra hands!

Stay tuned.