One of the side effects of cystinosis in adulthood can be muscle wasting. This is something that is so important to face head-on, and not just in the cystinosis community - the reality is that with age, muscle deterioration can affect almost anyone who isn’t diligent with a maintenance routine. This is just more true with cystinosis (often). The best defense against muscle wasting in cystinosis may be treating cystine buildup with a cystine-depleting medication such as Cystagon or Procysbi, but I don’t believe this is enough.
Here is a trio of products. As with anything, please check with your doctor if you plan on changing your regimen.
Pulmonary Strength: Elevation Training Mask 2.0 This may sound extremely gimmicky, but according to a lot of people it isn’t - to many it is totally legitimate, used by professional athletes and coaches. The mask emulates high altitude conditions by creating pulmonary resistance and thereby forcing you to use available oxygen more efficiently.
There are two main muscle groups that assist with breathing, the diaphragm and external intercostal muscles. This mask seeks to strengthen these muscles, which also work with core muscles due to their positioning. (According the the Journal of Applied Physiology, for example, the diaphragm musculature can be thickened with sit-ups and bicep exercises.) So it stands to reason that if you forced the breathing muscles to work harder (by limiting oxygen) while also doing core muscle exercises, you'd get an extra benefit.
I'll be honest. The first time I put it on (set to 9,000 feet), I didn't feel like I was breathing at 9,000 feet (an elevation I am familiar with) - I felt like someone was suffocating me at 9,000 feet. I initially called it my "panic attack training tool" - how long can I calmly wear this thing before I rip it off due to hyperventilation in the face of ridiculous (and unfounded) fears regarding the fate of my life resting firmly in its grasp? But because it comes with different settings, you can easily start at a lower elevation and work your way up. And honestly, I got used to the 9,000-foot setting fairly quickly and feel comfortable doing exercise at that level (with plans to increase the resistance when I feel ready).
Grip Strength: DynaFlex Pro Gyro Powerball I first saw this hand strengthener being used by trainers at the rock climbing gym I frequent. It is designed to build wrist, grip, and forearm strength by way of a gyroscope mechanism that can spin up to 150,000 rotations per minute.
The concept is simple: keep the gyroscope moving by rotating your wrist while maintaining a firm grip on the ball at the same time. Simple concept, difficult exercise. Because my hands are fairly weak, I feel like the primary benefit to this product is its strengthening of my forearms to compensate. A lot of our grip strength is actually found in our forearms and upper arms.
Here's a short video of me using the product.
[video width="640" height="360" mp4="http://rollerskatingwithrickets.com/blog/wp-content/uploads/2015/01/powerball.mp4"][/video]
Overall Strength (via science or placebo): ZMA So here's the thing. I'm not sure I believe that this supplement does anything in terms of helping with muscle growth. Many claim it does, but (I hope) I am not that naive. At the same time, it includes zinc, magnesium, and vitamin B6, all found naturally in foods. I take two capsules at bedtime in hopes that it lives up to some of its performance-enhancing claims, but even if it doesn't, I'm okay with its contents. Note that women should take less than men.
Final Thoughts Different things will work for different people, and any sort of exercise enhancement that you don't use is a useless one, no matter how many people sing its praises. The most effective tools are the ones you will use. This is something I must daily remind myself of, especially in light of the fact that I believe that these products - especially the first two - can help, if only I stick with them.
Finally - and I hate to be a downer, but - it's important to remember that you cannot "spot strengthen" any isolated areas of your body. You really need to be working out your entire body in order to positively impact any one area. If gaining muscle were easy, there would be a pill for it and the media would be all over it. Sorry!
Have experience with any of these products? Drop me a line in the comments!