Avleigh's Journey

We all have goals for our little ones. For some it’s what they will be when they grow up or what sports they will master. For us it’s quality of life vs quantity of life. This was our main subject at Avleigh’s appointments today. 

It all started out with the gastrologist. Although her blotted tummy and constipation has gotten better, he made it very clear that it will be something that we will always battle given her disorder and her limited fluid intake. Although I was prepared for this suggestion, hearing it about her just really makes reality set in. This decision could come with a few minor complications but the benefits out weigh the bad by a long shot. She will still be able to eat and drink by mouth as she loves doing, but we’ll have the ability to administer fluids and medicine with no fight. No more mornings and nights of her screaming to take medicine and me getting frustrated after all the work to get it in her mouth and she spits it out. Those times that she’s sick and refuses to eat or drink will now come with less stress. Those gas pains that she often suffers from will then be managible by venting. And unless we have her on a feed, no one will even know she has the port. Choosing to proceed with the tube will be improving her quality of life. Surgery is scheduled for January 6th. 

We then moved on to her Genetist. There wasn’t much change in this area. Just confirmation to continue her on her mitochondrial cocktail that is helping support good organ health. That cocktail is giving her the right kind of energy to keep her organs functioning properly. She also made the recommendation to add in a sleep aid on a regular basis. Her sleep is very important for her development and our sanity! This one could improve our whole family’s quality of life (haha). As long as we continue visiting with Neurology regularly we can now go down to seeing her once a year. 

Neurology was our final appointment of the day. And I must say he’s one of my favorite! He will be communicating with the Neurologist in Baltimore on the labs and plan of action to treat. He didn’t want to do labs without speaking to him first since we may have what he is looking for from other labs already drawn. We discussed options for seizure meds since the keppra has been keeping her up at night. We decided to give the keppra a chance with the sleepaid since this is the drug that has the least amount of effects on other things. He referred us to a Neuro surgeon mostly to establish the relationship. The referral is for a Baclofen pump in place of the pill she takes 3 times a day and VNS Therapy for seizures. The VNS is something that will not happen for some time but the Baclofen pump can be done as soon as she’s big enough for it to fit in her little body. It’s like a hockey puck that is placed right under the skin on the abdomen. This has Baclofen that is administered directly to the spinal cord. This option would regulate her dose, avoid side effects since it bypasses the stomach and make it much easier to adjust the dose. Not only that but she would no longer have to swallow that pill 3 times a day! There’s only so many ways you can trick a (almost) 3 year old to swallow a pill. 

Then the serious subject came up. We have been asked several times if we had a do not ressatate order. My response was always no, I’m not ready for that. Now I’m still not ready to put that on paper, but it was presented in a completely different way today that I can now somewhat relate to. It was more of a discussion on what we want for Avleigh. If we get a monitor to know if her vitals decrease is that enough to save her life. If we do get to her and able to save her life would we have done it fast enough to prevent more brain damage. If there is more brain damage and she is limited more than she already is then how is that improving her quality of life? I also often question myself on giving her a “normal” life when I know how sensitive her immune system is. I wonder if I’m doing the right thing by keeping her in a public daycare. The illnesses and risks for infection are so large for her. But the smile that is on her face when we walk through those doors is what keeps us going. I don’t want her getting sick and I will keep her home if something goes viral, but keeping her in a bubble to keep the germs away would make her miserable. Having this conversation helped me to know that making these risky decision are ok if it is what makes her happy. 

So overall today was very successful. We have a plan for more research and possible treatment. We are on the path to improving her quality of life through the G tube and possibly a Baclofen pump. And we will continue to make decisions based on what makes her happy! Anything for our Avleigh girl!