Avleigh's Journey

We live our days to the fullest, cherishing every memory possible. Avleigh not only has a very rare disease, but she also has rare symptoms and unknown progression of the disease. We have most of these symptoms under control. We know that she has to be vented at least once a day to avoid vomiting. We know she has to be on seizure medicine to prevent those nasty episodes from showing their ugly faces. We know we have to give anti-reflux meds and feed her only to her J-port to limit reflux. We know reflux, vomit and even excess saliva can cause deathly aspiration. But with all this, it is under control, for the most part. We try not to think about it or let it change the happy, adventurous life that we are striving so hard to give her.

We were recently on a wonderful, winter land vacation in Canada where everything was lining up just perfectly. It felt like we had won the lottery! Avleigh enjoyed her very first time in a sit ski! She was like a pro and absolutely LOVED SPEED! It was like she was made for this. Her guides took her all over the mountain and gave her an experience of a lifetime. The sky was clear, there was fresh snow and all the skiers expressed how those two days were what they call “a perfect ski day”. I know most of you reading this are thinking how cold it must have been. Really, it wasn’t so bad and even so, Avleigh loved being in the cool fresh air. On her second day in the sit ski a photographer followed her around taking pictures and video for the adaptive program to update their website! How cool and perfect timing was that! The odds were definitely in our favor.

Avleigh enjoyed walking around the village, visiting some shops and watching her big sister, Alyssa, playing in the snow. The next adventure we took her on was snowmobiles! I’m telling you, this girl has a need for speed. She sat on that snowmobile with her dad and didn’t say a peep for the entire tour. The few times we stopped to see the beautiful views she was glowing through her eyes and beautiful face.

All was going so well. Everyone was enjoying themselves like a real vacation should be. We returned to our room and I began to fix us a pot of coffee as Jacob went to shower. Just a few short minutes later I see her little body convulsing on the bed with her lips blue and foam coming from her mouth. I immediately started asking Alyssa to find the suction and did my best to get her upright and to get the saliva out from in her mouth. She then started vomiting bile. I knew she had too much pressure in her tummy. Even with nothing going into her stomach, her body has been producing too much bile and it backs up into her stomach. With her little to no motility her stomach can’t handle any volume. So we hooked her up to a Farrell bag to relieve the pressure and try to get her to stop gagging and better breathing. After 100 ml of bile drained from her tummy she was feeling better again.

In those few minutes to put a pot of coffee to brew our world got turned upside down. It’s terrifying as a parent. The look in her eyes as she stared at me for help. The feeling that I may not be able to get her breathing again. It put our reality back into the picture. It’s an awful reminder of what our day to day is really like. I was able to save her this time. But what would’ve happened if I didn’t have such a close eye on her? She had no way to call out. No way to indicate that she was in trouble except to convulse her little body in fear when she was unable to breathe. My sweet little angel has been struggling so much lately. She’s finally having some good days but these types of “challenges” always seem to creep in and put the fear right back into us.