Best's Disease

So we had gone to a pediatric opthamologists because a few of Brianna's therapists have asked about her vision. On the first visit, the opthamologist, Dr. Young, said her vision appears to be fine. However, he noticed some unusual "cysts" on her retina. He asked about any vision issues that run in the family, at the time I was unaware of any. So we wanted to arrange further exploration of her retinas, which will require sedation. So we wanted to coordinate that with an MRI due and have the pediatric ophtomologist at Tripler take care of it. And he wanted to see us back in a month to see if anything had changed.

Upon further investigation I found that there is a history of vision issues in our family, specifically Best's disease. We went back last week and I mentioned that to Dr. Young. And he said that he believed what he saw resembled Best's, but he's never seen a child Brianna's age present with it. There's a good chance that we may have to go to the mainland (again) to investigate it further, as there are no retina specialists on Hawaii. Typically Best's disease starts between 3-15. Stages one and two aren't visible on the retina, but stage three appears to be like an egg yolk. Stage four, where vision may be effected, appears to look like scrambled eggs. It appears Brianna would be at stage three, which she is incredibly young to have progressed so far - in both eyes.

Best's disease may or may not effect vision. There's no real way to tell if it will. Typically, if it does, vision isn't affected until individuals are in their 40s. And it typically still allows those who have the disease to still read and drive.

Best's disease appears to be unrelated to her PDCD. She seems to have drawn some short sticks with "unrelated" disorders, since this Best's and her craniosynostosis weren't directly related to the PDCD. However, I wonder if maybe the PDCD accelerated the rate of the progression of her disease.

I'm praying hard that it won't effect her vision. It seems so cruel that with all she's suffered, that her ability to see would be yanked away as well. I try so hard not to be angry, I know that she doesn't miss what she's never had, so it's not as hard for me to accept that she doesn't walk or talk, or do things that "normal" kids do. But she sees, and she loves observing the world. And to have it taken away from her, with her having no ability to understand why, truly would break my heart.

I do try to be like Job, and not question what God "allows" to happen in our lives. He knows a greater plan and his brush strokes a greater picture than I can ever imagine. But there are times that, like David, I wonder why God allows things to happen. My faith isn't shaken, but sometimes my resolve is.

So I continue to pray for my Brianna, this precious little light. And I also pray that God will continue to give me strength. To bear me up and give me the tools and the wisdom to know how to help my little girl on her journey of life, and to also provide the love and attention to my incredible little men.

Thank you again for sharing our journey. And please continue to pray for our family. God bless.