Hassle-Free Infusions (sorta)

After fearing I would pass out every time they put a new IV in, I am happy to report that my first three infusions have gone well and I have remained conscience each time. I have really small veins for an adult and they’ve even had trouble drawing blood at times. I was concerned when I found out I would need an IV infusion every two weeks for the rest of my life, especially because my most recent passing out experience happened at a blood draw just two weeks before my first infusion. I really didn’t want my little veins to be a problem that would prevent me from receiving enzyme replacement therapy or that I would be forced to have a porta-cath or PICC line.

But I think I have this whole IV thing mastered. First, good nurses are key! At the University of Florida, there are several expert “stickers” on staff. The infusion center is on the same floor as the Peds ward and the nurses there are used to tiny veins. They also have a nurse who they call in for those really tough cases, she is some sort of Vein Whisperer. Surprisingly by just taking a few easy preparatory steps they haven’t had to call in a Peds nurse (except for that first blood draw) or the Vein Whisperer for me.

Hydrate – I make sure that I’m very hydrated before arriving for my infusions, I drink plenty of water. If Gatorade didn’t have so much sugar in it, you can bet I’d be chugging that instead.

Warm – I try to keep my hands and arms warm and ask for a heating pack when I arrive at the infusion center.

Distract – When the nurse arrives to do the actual “stick” I make sure to ignore her. No, this is not rude, it actually makes her job easier because if I’m not focused on the IV going in, my muscles are relaxed and she has an easier time of it. I’ll talk to someone, read, play with my iPod – anything.

By following these simple steps I’ve turned my infusion appointments into hassle free sessions – well as much as one could hope for sitting in an infusion center for hours and hours every two weeks!

I am not completely out of the woods with the whole infusion thing just yet. My next infusion is number 4 and Dr. Byrne said that an adverse reaction, if it was going to happen, would show at the third or fourth infusion. I’m not going to worry too much about it as 1. There is nothing I can do about it and, 2. The other infusions have gone so well, the chances of me having a reaction at this point are pretty low.

I am however experiencing some sort of allergic reaction to something. I’m getting mild dry patches on my face and in my scalp. The doctors are not sure if it is a reaction to the Myozyme or if it is just a regular run-of-the-mill allergy to the pollen in the air. Florida is having an unusually bad allergy season this year, and this is my first season here so I have nothing to compare it to. I’ve never had spring allergies before either, anywhere I’ve lived. The exception is one summer I was in Paris, France and there was something in the air that time of year that caused my eyes to swell up and itch. It was a text book case of hay fever – the only one I’ve had in my life. The reaction I’m having now is a little a different, yes my eyes will swell and get red, but the itching is minimal. I’m armed with eye drops and other allergy medications which give me some relief and honestly the dryness is not that uncomfortable – I just don’t like the way it looks even if I’m the only one who can see it. My study nurse wrote down that I was having some sort of allergic reaction – to something and we’ll just wait and see. If it is a reaction to the Myozyme, it is so mild that it won’t prevent me from receiving future treatments. Hopefully, whatever it is it will just run its course and go away soon.

Sleep(less) Lab and Stuff

Last Friday I spent the day at Mayo meeting with my pulmonary specialist and physical therapist before spending the night in the Sleep Lab.

The pulmonary specialist wrote me a new inhaler prescription because he hopes to be able to take me of Symbicort completely as he feels it is a fairly strong steroid inhaler designed for asthma or COPD that I may not need. Fine by me, I would rather be on a weaker drug that does the trick (and comes in a generic form), so we’re going to “experiment” with this new inhaler and make adjustments if necessary.

The physical therapist prescribed a 10-12 week plan of 2-3 appointments a week to improve my mobility, strength – and well, everything. I am now tasked with finding a physical therapist who can design a plan for me in a facility that offers a therapy/exercise pool as well massage therapy. I have reached out to the local MDA and asked for assistance in recommending a specialist who is familiar with Pompe. I also contacted my insurance company about approving my PT and they were surprisingly helpful. I thought I was in for another battle over coverage, but no – they said I can go to whoever I want and if the provider needs pre-authorization to just have them call. The administrator even told me they could call her directly and she would authorize it right away! Wow!

I couldn’t check into the Sleep Lab until 8pm and because I had been up since 4:30am I figured I would be zonked out by the time the sleep study began. No, not so much. Yes, I was tired, but I didn’t really sleep through the night.

First, they hooked me up to a bunch of wires. Two on each of my legs with the wires running under my pajamas. Two on my chest, one on my neck, five on my face (yuck!), and twenty in my hair (super yuck!). Plus the pulse-ox monitor on my finger and a tube in my nose. This process took over an hour. The wires were attached with this gross medical gel that dried like glue and took days to get out of my hair. Serious hat days followed the sleep lab slumber party.

At 11pm it was “lights out” and they took away my TV remote and told me to go to sleep – or at least sleep until they woke me up a few hours later! They woke me up every few hours to roll over and at one point they put a bi-pap mask on me. I did not like this. The first mask they tried to put on me made me feel very claustrophobic and it had to come off immediately. The second one wasn’t as bad, but it was still annoying and it was very hard to exhale through, plus it kept waking me up. Not fun. I was exhausted the next day – I don’t know why they call it a Sleep Lab. Shouldn’t they let you sleep? Or at least call it a Disturb Your Sleep Lab?

The results from the Sleep Lab indicated that I do not have Sleep Apnea – I already knew that. However, when I am in R.E.M. sleep my oxygen levels drop into the 80s which is lower than normal. Not scary lower than normal, but enough to want to keep an eye on. They said I could benefit from using a bi-pap machine at night, but agreed to see if my Myozyme treatments would help my overnight breathing improve first. Come on Myozyme! I will be rechecked in 3 months but I’ll only have to wear the pulse-ox on my finger overnight and at home.

I had my second Myozyme infusion on Monday, April 12 and that went well – no adverse reaction and the IV went in smoothly and left no bruise. They only had to monitor me for one hour after the infusion instead of two, but it was still an all day ordeal. Dr. Byrne said that the third and fourth infusions are the ones to watch because if I’m going to have a reaction it would likely happen at that time. My third infusion is scheduled for April 26 and then every other Monday after that – barring any further production problems at Genzyme.