IV infusion

Easy (well, easier) IVs


Needing an IV infusion every two weeks for life makes the subject of IVs and ports constant chatter in the Pompe world. Like most debates there are those for and against ports. On infusion days many of my Pompe friends brag about a “one stick” day or vent frustration over a “3 or more sticks” day. No one likes getting stuck with a needle, especially over and over again, which is often when the port verses no port debate arises. I come down strongly on the “no port” side as I’ve already had enough slice and dice done on my body for this blasted disease and I really don’t mind the needles – as long as I don’t have to watch. I do everything I can to prepare my body for a “one stick” day. 

If you’re having trouble with getting “stuck,” before succumbing to “port pressure,” why not give Gatorade a try? Cheers!

The best thing anyone can do is to stay hydrated, which includes not only drinking loads of water, but avoiding alcohol and caffeine. Recently I’ve started incorporating Low Calorie Gatorade into my pre-infusion ritual. I use low calorie because it contains less carbohydrates and sugar – which in my head equals less glycogen build up. I drink at least two servings the night before my infusion and two more servings the morning of. It seems to help reduce the amount of sticks because along with the electrolytes for hydration, Gatorade is loaded with sodium which helps plump up the veins.

A Dozen



No more pole dancing - this is my new IV pump. Pump on the left, Lumizyme on the right. It zips up and I can carry it on my shoulder.

Today I had my 12th infusion, my second at the new infusion center. I took a new route today which made the journey so much easier. I did have to hand over $4 in toll fees each way which irks me. I just really despise toll roads.

Again, everyone was really nice. They even made sure the snack drawer had low-carb snacks in it that I could enjoy. A new addition to the infusion suite was a mini fridge stocked with juice, soda, and water. And I think they finally got the thermostat under control as the temperature was consistently comfortable.

I went prepared with my own “tape” which is really called athletic wrap. It is wonderful for infusions, it sticks to itself and not my skin. My nurse said she would request some for my next visit -she really likes that stuff too.

Magic tape for magic meds day. And it's purple - bonus!

The infusion went well, no reaction and once again I feel great. I’m always so energized on infusion days. That Lumizyme is good stuff! I start feeling run down about four days before I’m due another dose, and I often get pain flare ups in my legs. They are nowhere near as bad as they used to be and don’t last as long. Usually one Aleve will calm the pain, at least enough so that I can sleep. I had to take one last night and one two nights ago. A huge improvement from where I was for, well – most of 2009.
I only hit one small snag this time around. For some reason one of the managers wouldn’t let the pharmacist start mixing the Lumizyme until my IV was in. I’ve always been able to call ahead, about an hour before my appointment, to let them know I’m on my way and they would have it ready when I arrived. Hopefully we can get this glitch worked out, otherwise I’m just sitting in the chair for about an hour while they mix my cocktail.
Still going well and still fighting!


To Port, or Not to Port

Having to get an IV infusion every two weeks for the rest of my life has often brought up the subject of having a portacath put in. A portacath (or port) is a small medical device that connects to a vein via a catheter. Drugs can be injected and blood samples can be drawn through the port’s septum. It is surgically inserted under the skin in the upper chest just under the collar bone.

This is what the device looks like and how it works. It will leave a small rise under the skin, about the size of a quarter, where the port is accessed. All this, and you still get stuck with a needle each time – no thanks, not for me. Image from keepwerking.co.uk.

They are most often used with cancer patients for chemotherapy treatments. The chemo is so strong it can cause tissue damage, so a port is the safest way to go. Lumizyme is basically a synthetic enzyme and will not cause tissue damage so inserting a new IV every two weeks is not a problem and a port is not utterly necessary, but it is an option for Pompe patients who have difficult veins or who just don’t want to be “stuck” every other week.

I don’t mind the IV needles, they don’t hurt, even when more than one attempt has to be made to insert one. I have heard from a fair few cancer and Pompe patients who have ports and they unanimously agree that they love their ports. They enjoy not having to worry about problems with the IV and possibly getting stuck more than once. Again, I don’t mind and until (and IF) it becomes absolutely necessary for me to get one, I will continue to go with the new IV stick each time. Here’s why:

Having a port put in requires surgery and I don’t do well AT ALL under anesthesia. When the did my second muscle biopsy they surgeon decided for some reason to put me out – no one knows why he did this for a fairly simple (albeit, painful) procedure. I had so much trouble recovering from that, my pain and headaches got immediately worse following the surgery. My “theory” is that the surgery “angered” the Pompe. I can’t imagine going through all that again, which could set back the little progress I have made, just to avoid some needle sticks.

It is a personal decision, and everyone is different. Now, down the line I may change my mind as we never know what will happen, or what the future holds for Pompe patients. But for now, I will continue my role as a human pin cushion as the risks don‘t seem to outweigh the benefits for me.

If you have questions about having a port put in, talk to your doctor about the benefits and risks. Like I said, I’ve yet to meet a patient who doesn’t absolutely love having a port.

The First Lumizyme Infusion

June 16, 2010. Today I had my 7th infusion. What made this infusion different is that it was the first official Lumizyme infusion since Genzyme received their FDA approval to produce the drug commercially.
Those of you who have been following along and keeping track of my infusions know that today is not my normal infusion day. The University of Florida wanted to be one of or the first to administer the newly approved drug and asked me to be the first recipient. They were in a race with Duke University to be the first to infuse Lumizyme. Duke ended up not administering Lumizyme today, but rumor has it that a small town in New York had an infusion today too. But I’m pretty sure UF is happy to just hold the bragging rights over Duke.
The hospital sent a car to drive me to and from my infusion, which made it nice because it turned out to be a really long infusion day. I arrived at Shands Medical Center at UF and met with my study nurse Linsday Falk and Dr. Barry Byrne. I had new paperwork to go over and sign, Dr. Byrne said it was essentially like starting all over again. Representatives from the University’s Media Department were on hand to record the “historic” event. You can view the article here and video footage should be available on the UF website later.

Paula Graham, Medical Science Director Genzyme, Dr. Barry Byrne, Monique Griffin, Study Coordinator Lindsay Falk BSN, RNC, Ivy Kelly RN, Sarah Kulke, MD, Director, US Medical Affairs Genzyme.

Representatives from Genzyme were also on hand because months earlier they had scheduled a teaching seminar at the University. So it was just a coincidence they were there for the first Lumizyme infusion. They were very gracious and excited to see all Genzyme‘s hardwork in action. It was a real treat to have them there along with, Dr. Byrne, Lindsay, and the Media Department.

It was a little strange to have so many people and cameras in my infusion room, but my life hasn’t been exactly normal these days anyway. Later in the day I spoke with a reporter from the Gainesville Sun and her article can be viewed here.

Everything went well and it was a pretty exciting day for Dr. Byrne and his research team, the University, Genzyme, and of course me. I was happy to be part of it and glad the chose me to receive the first Lumizyme infusion.

They said I may receive further media requests, but the UF Media Department will help field those for me. What a day! If nothing else, I hope the day brings more awareness to Pompe Disease and the amazing work Dr. Byrne and the folks at Genzyme are doing.

Today’s even almost didn’t happen. We all had to work around the clock for the week leading up to today in order to put everything in place. Phone calls, emails, faxes were going non-stop between UF, Genzyme, myself, my insurance company, the pharmaceutical distributor, and the wholesaler. I didn’t receive the final ok that today’s infusion was going to happen until 8:30pm last night.

In less than two weeks I will return to UF for my next Lumizyme infusion. Somehow I think it will be less eventful.

Photo by John Pastor, University of Florida News and Communications

Baby Steps

Last week I noticed I was consistently having an easier time walking short distances than I had just a month ago. I’m still not ready to sign up for a 5k (doubt I ever will), but it is progress. I have only been on ERT since March, but that coupled with my high protein, low carb diet I think has helped. I can’t believe it!
I know it may not sound like much, but it really is huge to me. I was spiraling downhill so fast for so long, that to feel as if the treatments have not only slowed or stopped the progression of the disease, rather it has actually started to improve is, in my book, a miracle. I never expected to feel the benefits of treatment this soon.
There is hope.


Seeking FDA Approval – Again

Genzyme hopes the FDA will approve their drug alglucosidase alfa (which is my ERT drug) on June 17,2010. The odds are mixed to whether or not this will finally happen as they have been seeking FDA approval for commercial use of this drug for years. They have experienced set-backs in the production of this drug at their manufacturing plant in Massachusetts.

Anticipating growing global demand, the company was moving production of Myozyme from Allston and a smaller facility in Framingham, Mass to a larger 4000 liter bioreactor scale in Geel, Belgium. However, upping production from 160 liter bioreactors to 2000L and 4000L scale-vats required that Genzyme submit supplemental Biologics License Application (BLA) to both FDA and European regulators, as the agencies insist that similar biologics produced in dissimilar bioreactors cannot be considered identical products, due to differences in the carbohydrate structures of the molecules in the so-called brews. Confused? That’s OK: the bigger tubs of alglucosidase alfa will continue to be sold under the Myozyme brand in Europe and under the Lumizyme title stateside. — BNET.Com, November 19, 2009, “FDA Delays Genzyme’s Lumizyme, Manufacturing Problems Blamed” *

What this means for Pompe patients is that, if it is approved those not currently receiving the drug through an ATAP study should be able to get it. And for those of us in an ATAP study, the study will end and we’ll join everyone else in the commercial use of the drug – and having to pay for it. Let the insurance battles begin!

To view the full story click here.

Pompe Everyday

Having traveled my entire life, having lived on three continents, and having to add more pages to my last passport means I’m almost always looking for my next vacation get away. Too bad I can’t ever take a vacation from Pompe. Pompe is with me everyday, there is no packing up and leaving it behind like you can do with most problems like work, stress, or those “crazy” family members.
Pompe touches everything I do from the moment I wake up in the morning to the moment I fall asleep – and it doesn’t stop there. Pompe effects how well I sleep too. I thought I’d take some time and highlight how challenging it can be to live moment to moment while fighting Pompe. Notice I say “fighting” and not “suffering from” because that is how I approach everyday life with Pompe. I fight.
Rise and Shine.
When I wake up in the morning, I can’t just pop out of bed like I used to. I have to carefully raise myself out of bed to a standing position. This can be a slight struggle, but it is something I have gotten used to. I then have to gain a sense of balance before I can start to walk and hope that I slept correctly so I can avoid those ever so common Pompe morning headaches.
Stairs and Chairs.
I can struggle and pull myself up a few steps here and there, as long as there is a handrail. I almost never go down stairs because the risk of falling is too great and it would be a huge set back in my treatment plan if I were to injure myself. Chairs present an interesting challenge – like snowflakes, no two ever seem to be alike. While sitting is a nice break from standing, which I can’t do for too terribly long, if there is not a “decent” height chair for me to rest in – I usually just tough it out and stand. I then hope that I have not tired myself out too much when it comes time to walk again. This is one of the reasons I spend a lot of time out in public in my scooter as I never know what the chair situation will be in any given situation. I have however gotten really good at judging the height of chairs and can tell just by looking at them if I’ll be able to get out of them, but sometimes an overly soft chair will fool me. Bad chair.
Time to Dine.
I have to think about everything I eat – I wrote more about this in an earlier blog entry. Because I am on a high protein, low carb, low sugar diet I can’t just grab whatever is convenient. Meals and snacks take some planning. If I am eating in a restaurant, not only do I have to consider the chairs, I have to choose my entrees carefully. When in doubt, I talk to the chef. I try to think of it more of what I can eat, rather than what I can’t eat. And really, there is a lot that I can eat even when dining out and sometimes it gives me an opportunity to try something new.
Beauty Babe.
Because of the weakness in my shoulders and upper arms, I now have trouble washing and styling my hair. I have to bend my head down so my hands can just barely reach my head to shampoo and condition it. I can’t really put my hair up anymore without a struggle, not even in a simple ponytail. If I’m sitting in certain chairs that support my back, but also allow me to lean my head back, I can usually manage a kind of sloppy ponytail, but even that seemingly simple task can take a lot out of me. Luckily I have no problems washing my face and applying make-up, so I’m not likely to leave the house barefaced and scare little kids too much! I’ve taken to using an electric shaver on my legs as trying to balance on one soapy leg while holding a sharp razor next to my skin is not really a good idea given my current strength level. And sitting on the edge of the tub is not really an option as I’d never be able to get back up again.
Fashion Forward.
Shoes and outfits have to be carefully considered and not just for the usual girly fashion diva reasons. No more heels for me as balance is an issue and I don’t need to up my level of difficulty just yet. Also, heels tend to be slick on the bottom, even with those non-slip pads. No matter how much of the that stuff I put on the bottom, just the tiniest bit of heel can slip and down I go. Many of the cute flats present this same problem so I have to go with shoes that have great grips – Converse Chucks and decent flip flops are my shoes of choice right now. I’ve also been avoiding skirts and dresses simply because of my fear of falling and, well, flashing people. Luckily an 80s trend has come to my rescue – leggings. With leggings I can now sport fun and flirty skirts and dresses with my Converse and flip flops no doubt! Please don’t call the fashion police on me just yet – I’m working on it.
These are just the highlights and new situations will bring new challenges. But as you can see, Pompe really does touch everything I do, every moment of every day. I start physical therapy this week, which will help me improve my movement and strength, but I’ll almost always have to use creativity and tenacity to accomplish day to day tasks that I used to take for granted. And physical therapy, like Myozyme will not “fix” me overnight – it will take a great deal of time and effort to see improvement. Whatever happens, somehow, I’ll manage. Pompe may have won the battles, but I will win the freaking war!
What are your day to day struggles, and how do you tackle them?

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.

No Infusion This Week and Other Quick Updates

My second infusion was cancelled. One of the nurses running the study called and told me that Genzyme was having a production problem, and Myozyme was on back order. So they cancelled my March 29 appointment, but I’m still scheduled for April 12.

I head back to Mayo on April 9th to meet with the pulmonary specialist, the physical therapist, and I will stay overnight in the sleep lab.

Genzyme is hosting a local meeting/dinner on April 11 which I plan to attend. Genzyme representatives, doctors and patients have been invited. I am pretty sure my study doctor, Dr. Barry Byrne, is planning on attending.

April 9-12 will be a busy couple of days for me and I’m sure I’ll have more to report after all is said and done.

The First Infusion

My first Myozyme infusion went really, really well. I didn’t even get a bruise from the IV. It was just an extremely long day.I had to arrive early in order to take a pregnancy test as they won’t administer the drug to anyone who is pregnant because they don’t know what the risks are to a fetus. So after we finally got the test results the pharmacy could then prepare the Myozyme. It takes an hour to mix and because of the expense they have to shut down the entire pharmacy during this time. The IV bag of Myozyme arrived to the infusion center around noon where they had already inserted my IV and had kept my vein open with saline while we waited for it to arrive. They hooked me up to the Myozyme and the actual infusion was complete in 4 hours. They took my vitals every 30 minutes and my blood pressure and temperature remained consistent throughout. I didn’t feel a thing. The Myozyme was not cool or hot coming through the IV, it didn’t feel any different than the saline. It looked light blue coming through the tubing, they could have been infusing me with blue Gatorade for all I knew. After the infusion was complete they flushed the tubing with more saline to ensure I received all of the Myozyme and then I was monitored for two hours to make sure I didn’t have any adverse reactions.

Next time it shouldn’t be such a long day as they won’t have do a pregnancy test and they won’t have to monitor me for two hours after the infusion. My next infusion is scheduled for March 29 (and theoretically every other Monday after that).