Thursday, September 4, 2014


8-29-2014 Decisions, realizations - letting go of busy-ness!

Today I decided that I'm not going to teach piano lessons anymore and that instead of directing my Youth Show choir (The Centerstage Singers) in the fall and spring, I would only do it once a year, each spring. I realized that I didn't know how long I would have the boys and I can always come back to teaching lessons again later on. BUT I want to be available for them now, while they are here. I want to give them a stable, loving, calm, happy home to be in. If I am stressed and really busy, it is much harder for me to keep up with everything. I want to be able to get them ready for bed each night and take care of them every day. Brad (my husband) has always been supportive of the things I do, but I can tell it is stressful for him when I am teaching lessons all evening. On those busy nights, my daughter will often get lost in the shuffle. She won't get all her homework done and we'll forget to keep track of her allowance and often the house will get messy.

I just want to be able to focus on taking care of my family and home. I want to enjoy the time I have with my children while they are young, but especially knowing that two of my children may pass before they reach adult-hood, makes it all the more important. It was the thing I guess I needed, to realize that I need to let some things go. I know it will get harder as the boys lose more of their abilities. We already have to do pretty much everything for Eli, although he can sometimes entertain himself a bit. And don't get me wrong, I am grateful for the things he can still do! But once we have to take care of both of the boys all the time, especially as their health starts to deteriorate, it will be difficult. Brad will need me available each day and it will have to be a team effort for the whole family.

In the past I've taught up to 20 students at a time as well as directing my youth show choir two sessions a year. I've done a lot of preparation for those show choir sessions, by making practice CDs for them and recording most of the songs myself. When I'm recording those, I of course want them to be good quality, so I have spent hours and hours recording accompaniments, vocals and then polishing them up in the programs. I also spend a good few hours a week in addition to teaching my lessons, planning the lessons and sending out email updates. I decided that I could continue to do Centerstage but only in the spring. That way I will have a lot more time to prepare for it and it won't be such a time crunch for me. Plus that only lasts 2 months and it is over. I wanted to keep that because I feel like there isn't anything quite like it in our area. I feel it is a fun opportunity for the kids to get to learn the music, harmonies and sing solos on stage. Laya is also involved in it and I would want her to be able to continue. Our spring performances have been at the local Pima County Fair. The Fair treats us great. They comp all of the performer's tickets and give them extra free tickets for the performer's family members as well as free parking. The youth have a great time performing and then playing at the fair.

I will also continue my online work through my website, developing my piano teaching workbooks and explanation videos. The good part about that is, I can do it on my own time and there isn't any time restraints or stress involved with those.

I feel pretty much at peace with my decision, although I feel bad letting my students go. I plan on having one last, big, fun Recital at the end of this semester and then getting them set up with other teachers. This decision will be better for our family, I feel confident about that.

8-30-2014 Decisions, realizations- the type of Gm1 I believe my boys have

Today I decided that I need to let go of trying to figure out how long I will have them with me and just be ok with not knowing. This past week, I have been making myself crazy, looking up any information I can find about Gm1 Gangliosidosis. I have been staying up late every night, searching the internet and asking questions to the nice people in the Facebook Gangliosidosis group. Here is some of what I've gathered so far. There are two sub-types of Gm1 type II. (although it is important to note that scientists now think of this disease as more of a spectrum, since there can be a wide variation between types) I was trying to figure out if Eli and Evan have the Late-Infantile or the Juvenile type. From what I can figure out, I believe they have the late-infantile type. Here are the reasons why I think so:

Age of Onset: Late-infantile onset is between ages 1 and 3, and other resources say usually around 18 months. Juvenile seems to be between ages 3-5, but some classify the adult type III as onset between ages 3-30.

Eli's Age of onset - Eli's balance was poor when he started walking at 10 months and continued to be that way for a good 6 months. He might have peaked at 18 months, but he was still behind other kids his age at that time. His speech was very delayed (just said beginning sounds of words). Between age 18 months and 2, he didn't progress at all, and then after age 2, he started to regress. He developed strabismus (his left eye crossed severely), then after eye surgery, his right eye began to cross. After that surgery, both his eyes were still a bit messed up, so they did surgery on both eyes. His eyes look ok, but his left eye now wanders outward (instead of inward, like it used to). He developed kyphosis in his lower lumbar spine. I noticed that around 2 1/2 years old. He didn't progress from 2 on and began to lose skills. He lost most of the little amount of speech skills he had. He lost his fine motor skills he had, he became ataxic and seems very stiff. He now (at age 5) has a difficult time walking, doesn't speak words, but can make sounds, does not have the coordination to feed himself with a spoon or fork, or dress himself. He can walk a little bit, but falls easily. He pushes a walker and it works ok for him. But he is often too tired to walk, so we end up just pushing him in the stroller. He has begun to spit up after eating (reflux) and has started to spit out his water, while drinking. I have noticed him choking on food or water more easily.

Evan's age of onset - Evan has just turned 2 this month and his speech hasn't progressed much between 18 months and age 2. The difference between Evan and Eli, is that Evan hasn't had any of the balance issues that Eli had. Although even at a young age (when Evan was 12 mo) Brad began to become worried about his progression. He has been behind other kids his age speech wise and a little bit cognitively, but not significantly. Now that Evan is 2, he does seem a bit wobbly/clumsy and is falling easily. His mental capacities seem great (We were more worried about Eli's comprehension and receptive language at this age, than we've seen in Evan). Evan's left eye is crossing inward now and sometimes his right eye looks like it is crossing inward as well. He seems so happy and funny right now. But he is behind a bit in his speech and a little bit delayed in his comprehension.

Speech - Another reason why I believe they have the late-infantile is because the examples of people I have found that have the Juvenile type, have been able to talk at some point, but then lost it. Eli never got to the progression of being able to talk with real words instead of approximations. Evan is talking better than Eli did although most of his words are approximations as well, except for probably Uh oh and bye bye, although even those ones sound more like "uh uh" and "bah bah". Lack of speech development is another characteristic of late-infantile Gm1 Gangliosidosis.

The reason why I was trying to figure out if they had late-infantile versus juvenile, is because there seems to be a significant difference between the mortality rate of the two. From what I've read, late-infantile cuties, usually last until mid-childhood (usually defined as ages 6-12) and juvenile last into early adulthood. That is a big difference to me and it could mean 10 less years I will have with them. That's why I felt so much urgency to figure it out. I realize now that things can vary and I need to let go of wanting to know everything.

All I can do is document our experiences, enjoy our lives, and appreciate and be grateful for any time that we get with them. I need to let go of my hopes to control what happens in my life and give up what I wanted to God. What God wants for us is always going to be better than what we had expected or wanted, especially when looking at it through an eternal perspective. He knows what is best and I just hope that Brad and I, as well as Laya and the boys, can get through this beautiful hardship with Faith, Love, Hope and Charity.

Brad shaved the boys' heads ha ha. Now they look like two really big babies. Evan enjoyed cleaning Eli off after the haircut!