My Medical Equipment and
SMA Drug Trial
When I was younger, I use to be able to walk around the blocks and walk uptown with my family just with a walker. I also used to use my walker at school to help me get around on my own. This year I got my first power wheelchair, so I don't use my walker as much any more.
Recently I got my first Power Wheelchair, I use it in school and lots of other places. Whenever I have to go somewhere that I can't take my powerchair , I use my manual Wheelchair.
Because SMA weakens my lungs, at night I use a BiPAP ( bi-level positive air pressure) machine to help me take deep even breathes. The BiPAP machine also has a back-up rate which means that I get at least a certain number of breaths per minute, and if I don't take them on my own, the BiPAP will give it to me. Before I started using the BiPAP I used to set off the alarm on my Pulse Ox alot, because throughout the night I would stop breathing ( sleep apnea ). Now the BiPAP makes sure that I get enough breathes and I sleep much better.
At night I sleep with a Pulse Ox. This machine measures my heart rate and Oxygen saturation ( the amount of Oxygen in my blood ). This helps my mom makes sure that I'm doing ok, and that I'm healthy. It has an alarm on it which will tell my mom if something's wrong.
Recently I've started using a machine called The Vest. This helps loosen mucus secretions and other junk in my lungs so it's easier for me to clear them with the cough machine. I use this along with all my other machines to help stay healthy.
One of the things I need help with is coughing , because SMA weakens my muscles including my chest and lung muscles, I can't produce an effective cough. The cough assist mask goes over my nose and mouth , then the machine forces a specific amount of air into my lungs, and pulls it back out again, this simulates a cough , and helps me to clear mucus and secretions and expand my lung all of which helps keep me healthy !
Drug Trials for SMA ( written by my mom )
Ian is participating in a clinical trial at the Stanford Hospital in CA. This trial is using the medicine Hydroxyurea, which is already FDA approved for some cancers, and some sickle cell diseases. Dr. Ching Wang is hoping that it will help SMA patients to keep their strength they have now, and hopefully gain some.
Ian is now in the first part of the trial which is the blind study. We have no idea right now if he is on the real medicine or not. We are just patiently waiting for July to come so he will for sure be on Hydroxyurea. Then we hope it will do for Ian as it has done for some other children. Right now even if it does stop the progression and only that, we will be happy. Ian has progressed quite a bit off and on, and we would like to see it stopped!
We get asked a lot if we think Ian is on the Hydroxyurea and not the placebo. I don't know. Its hard to tell. There are some days Ian really surprises me and does things I never expected to see him do, or walk a longer distance without falling. Then the next day comes and he is back to how he usually is. I don't know if he is on the real med, and can't say for sure.
One thing though also about the trial is he has the med/placebo's dosage upped every month when we fly out there. SO maybe if he is on the real med we see more improvement when he gets the amount that is needed for him? I really don't know what one he is on, but believe me if Ian does something that is just amazing with his type of SMA I will be sure to announce it to everyone!
I am going to quote something that Ian said to me when we first flew out to CA for the first visit. "Mom if this medicine works then it will also help all the other kids with SMA get it right?" I said "yes, hopefully." Ian said "WOW I can help all the other SMA kids get better, maybe." Ian isn't just wanting to get the med for himself he is hoping to help all his SMA friends.
I had to share this with you all because that shows you what kind of person my son is.
We will update in the Journal every month on the trial and how things are going with Ian. So please check that out for new updates on the trial, and anything that comes up!
To read more about some of the SMA drug trials please go to http://sma.stanford.edu.