Step Up

This Mother’s Day we wanted to do something really special for Elsie’s grandmas because they have been two of the most amazingly supportive, understanding and loving women not only throughout Dave and I’s lives but also through this period of transition as we adjust to life with cystinosis. Although a gift can never really honour and thank our mother’s the way they deserve I think we came pretty close, and as you can see in the picture above Elsie approves!

Step Up For a Cure are these adorable, personalized, wooden stools made by a family in Ontario. A perfect stepping stool for little ones, they’re sturdy and can be decorated anyway you like. We had the phrase “Grandmas hold our tiny hands for just a little while but our hearts forever” and a picture of the backs of two teddy bears hugging painted on our stools. The price per stool ranges from $40-$50 and the best part is that all of the proceeds go to the Cystinosis Research Foundation! The family started this project in 2012 shortly after their beautiful daughter was diagnosed with cystinosis, so if you’re in need of a stepping stool (or a time out stool) in your house or the homes of your relatives I hope you will consider these one of a kind stools. Elsie’s grandmas loved theirs!

For an Elsie update she has been doing really well lately! We just received the results of her white blood cell (WBC) cystine test and it’s 0.82! The WBC cystine test is the test that officially confirms if someone has cystinosis. A person without cystinosis will have a level right around 0 but when Elsie was diagnosed her WBC cystine was 2.26. After starting cystagon at 400mg per day her WBC was tested again a couple months later and it came back at 1.13, which was a definite improvement but the aim is to get the level below 1, although between 2 and 1 is still beneficial. So her cystagon was upped to 500mg per day and tested again 3 months later, which came back at 0.82. We’re super happy that her cystagon doesn’t need to be increased any further and that her cystine level is once again safe.

We’re also getting close to starting growth hormones. We’ve been registered with the Saizen program (Momentum in Canada) and received their Easypod in the mail which is a sort of high tech injection system. The pharmacy has already filled her prescription for growth hormones but we are just waiting to hear about coverage from our insurance company before going to pick them up. This is because the cost is over $300 just for two weeks! And if all goes well for Elsie on growth hormones she could potentially be using them for years. Once we get the coverage figured out, hopefully in the next week or so, a nurse will be coming to our house to show us how to use the Easypod and we’ll be good to go. Fingers crossed she doesn’t mind the nightly injections.

We’ve also just enrolled in a local infant development program. We were referred to an occupational therapist for feeding therapy to get some tips on how to increase the amount Elsie eats but the wait list is quite long. So they offered to put her in this development program as we wait. Initially I didn’t want to do it because we don’t have any developmental concerns with Elsie and I didn’t want to put her under even more of a microscope. But while at the CRF Day of Hope Conference Dr Angela Ballantyne did a talk on Quality of Life Issues in Nephropathic Cystinosis which looked at the the potential academic and motor problems that can occur with cystinosis. In it she talked about the little things that we can do to encourage development in our children which could help make their futures easier. During this talk I realized that my resistance to the infant development program was more because I didn’t want to have another thing to worry about as opposed to wanting to protect Elsie from more tests. So we will be visited by a counselor in a couple weeks to discuss our goals and learn how we can encourage Elsie to continue learning and developing at a healthy pace.

As always Elsie continues to be her happy, smiling self which is all we can ask for! Now go buy a stool 😛