With the New Year ringing in I had high hopes that this year would be our best year with cystinosis. We’ve been living with it now for just over 2 years, have had lots of time to research, ask questions, learn about what everyone else is doing and coming to terms with it ourselves. So I was almost looking forward to our first visit of the year at the renal clinic (because who doesn’t love trying to contain a 3 year old in a tiny, sterile room for 2 hours) to see how Elsie’s levels were doing and how much more she’s grown.
The good news is that she continues to grow, at least in height. But unfortunately we just can’t get this girl to put on weight. Our doctor would like to see Elsie start to gain much more this year and I would love that too. Obviously her health is the number one priority but secretly I would also love to be able to buy her pants that fit her waist AND leg length. Right now she’s about a 4T in leg length but only a 2T in waist size. I know many people are probably shaking their heads at the shallowness of this but I’m telling you the struggle is real!
Anyway operation fatten Elsie up is officially on and so far I’m finding it difficult to brainstorm healthy ways to get more calories into her. She is so strong willed, I prefer to bang my head against a wall than to attempt talking her into trying something new. So in other words, you can forget about it! Sometimes she will tenderly touch the new food to her tongue, say it tastes good, but refuse to actually put it in her mouth. And our dietician is recommending that Elsie get about 1600 calories a day!
Basically our only option is to trick her into extra calories or work with what she does eat. Right now she loves eating butter, just straight up butter. Perhaps it’s time to just plop a stick of butter in front of her and be done with it? But I can’t imagine there are any long term benefits, or that she would feel very well after that. There is however a product called Duocal which is supposed to be a tasteless powder that you can add to food or beverages for added calories. We haven’t tried it yet but if it is indeed tasteless and doesn’t change the texture too much it would be perfect for adding to her water. We’ll be looking into where we can get it in the next couple days so I will update you guys on how it goes. And if anyone has any other ideas on how to trick a preschooler into more calories I’d love to hear them!
I’m also sad to report that another medication has been added to Elsie’s daily regime. The protein/creatinine ratio in her urine has increased significantly in the past 3 months, from around 300 mg/mmol to over 700 mg/mmol. For reference sake a normal range for someone without kidney disease should be less than 30! The amount of albumin in her urine is also quite high which we’ve been told suggests that not only are the tubules of her kidneys experiencing damage but now also the glomerulus.
To try and combat this additional damage to her kidneys our doctor has prescribed Enalapril, an angiotensin-converting enzyme (ACE) inhibitor that lowers blood pressure. Though she doesn’t have high blood pressure the hope is that Enalapril will lower the pressure in her kidneys so that they won’t have to work as hard and lessen the damage to them. Turns out that this is quite a common treatment to prolong the kidneys in chronic kidney disease patients so we feel good about adding it in with the rest of them. We’ll be testing her levels again in a few days just to make sure all is well so here’s hoping this will lower her urine protein/creatinine ratio.
2016 may not have started out as stellar as I was hoping, but it’s not awful either. The Enalapril will hopefully do it’s thing and getting some meat on Elsie’s bones is a good goal to have throughout this year. Luckily her brother is eating like a champ and then some so now she’s got someone to compete against and my girl loves to win!
Elsinosis: Living with Cystinosis 