Pompe Disease

Subscription Box Therapy: Stitch Fix #4

Since I was only able to keep two items from my last Stitch Fix, I ordered another box straight away. Note: I did receive the red top from Fix #2 in a size that fits me on the third try. Yay! 

I opened the box and the first thing I spotted was the shoe bag. Yay shoes! I then peeked at my fashion burrito and spied some fun spring colors. Hooray! Spring has arrived… well, to my closet at least. Here in Colorado it can’t really decide – we had snow again this past week. Snow! But over the weekend it supposed to be in the 70s. Insert dramatic sigh. Colorado. Shake head. 

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Moving on. I opened my shoe bag first, because shoes. I found these red sandals that I had pinned on Pinterest and they fit perfectly. I only wish they were a little less orange-red and little more red-red. But they will go nicely with what I have in my closet – and hopefully what is yet to be added to my wardrobe. 

 

The next item was the grey knit top. There is nothing special about this top, it is nice neutral basic, the only downside is that it is a bit itchy. I hope that once it is washed, some fabric softener will release this shirt’s inner softness goddess.  

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Acelyn V-Neck Linen Blend Knit Top by Market & Spruce

The next item was the navy blue and white stripped top. The cuffs are bright, neon orange. I love the pop of color and this shirt is so super soft. My Stylist saw that I had pinned some stripped tops on Pinterest and decided to send me this one. I love it. 

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Lucas Cuff Sleeve Knit Top by Papermoon

The next thing I pulled out was the orchid cardigan. Yes, it is a beautiful shade of orchid, like a dusty orchid the photo does not do it justice. The invoice calls the color light purple, but it really isn’t – my aunt and I pulled out my crayon box and, according to Crayola, it is orchid. The fit and cut are perfect. Unfortunately, I just discovered it needs to be dry cleaned. Boo! 

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Claudine Cardigan by Pink Clover

The last item was the pair of white jeans. This is an item I had requested from my Stylist. She couldn’t find anything in my size for my last Fix, but this time around she managed to snag this pair for me. My heart sank when I saw that they were skinny jeans. Ahhhh, scary! I’ve seen many people try on white skinny jeans on their Stitch Fix unboxing videos and most are in agreement that white skinny jeans are pretty unforgiving – as in they show everything, every bump and imperfection. As well as about half of them seem to be more than a tad see through. But again, being the good little Stitch Fixer that I am I needed to try them on. I needed to get a workout in anyway, why not burn some calories trying to pull on some skinny jeans right? Once again, Stitch Fix proves they are the masters of finding the perfect fitting jeans. My stylist sent a larger size than I needed, so they are not skin tight, nor are they too baggy. They do not fit me like skinny jeans = yay! They are also not see through = yay! They are the right length for my short little legs = yay! They are mine = yay! I cannot believe I own and am happy to wear white skinny jeans.  

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Kay Skinny Jean by Liverpool

I kept every single item in this box and therefore I received the 25% discount. I wasn’t all that keen on the grey top, but it was cheaper for me to keep all 5 items with the discount rather than paying for just the other 4 at full price. 

Once again, Stitch Fix has saved me the time, energy, and frustration of having to go out shopping for clothes. In the world of Pompe Disease, energy saving items and services are gold. 

If you are interested in trying Stitch Fix risk free – as in no styling fee for your first box: You can use my link here and your first $20 styling fee will be waived. Full disclosure: I will receive a $25 credit if you sign up using my link. Once you sign up and order a box from Stitch Fix you will receive a referral link of your very own!

Subscription Box Therapy: Stitch Fix

So, technically Stitch Fix isn’t a subscription box in the traditional sense, it is more of an online styling service. There is a $20 styling fee and of course the cost of the clothes you decide to keep. I hate shopping. I especially hate clothes shopping. I hate trying on clothes. It is just too much time, too many people, too many florescent lights, and too many spoons.* 

In the past I’ve picked out clothes and then brought them home to try on. If they didn’t fit or if I just didn’t like them on, I would take them back to the store. After I broke my leg a few years ago the only pants I could stand to wear because of where my surgical site was located were leggings. Jeans were especially bothersome, so I found Lu La Roe which kept me in comfortable, cute clothing that was delivered to my home. I knew what sizes and what styles worked for me, so that was more or less what I wore. Plus these items are easy to pack and easy to mix and match. Well, I mixed and matched myself into a Lu La Roe rut. I needed help. It was time to bring in professional help. Enter Stitch Fix

 

Stitch Fix uses a combination of algorithms and professional stylists to create your box. The algorithm takes many things into consideration, including your location. It then sends your stylist (a real live person) suggestions. Your stylist will look over your Style Profile (we’ll get to that in a bit), he or she will even look at your social media sites if you provide the links. Pinterest is probably their favorite. Here is a link to my board if you’re curious as to how I’ve set mine up.

Now, on to the Style Profile I mentioned… when you sign up for Stitch Fix, you’ll be asked to fill out your style profile. This shouldn’t take more than 10 or 15 minutes, but do take your time and make sure you are providing honest and specific information. This is not the time to shave off a few extra pounds or add a few inches to your height. Give them as many details as possible, in addition to the “multiple click to answer” choices, you’ll also be given places to type in whatever you feel your stylist should know about your, let’s say fashion quirks. I certainly have more than a few quirks myself. 

You decide what types of items you’d like to receive and how often (as in often, sometimes, or never or only when I ask), as well as how often you’d like your Stich Fix to arrive. You can even pick your delivery date. You set your own price points, for each type of item. So if you like to spend less on tops and more on shoes you can make those choices in your Style Profile. You may update and change your Style Profile as often as you wish.

Stitch Fix will send you 5 items. Remember that $20 styling fee I mentioned? That $20 will go towards anything you decide to purchase. And…. if you purchase all 5 items in the box, you’ll receive a 25% discount on the total. Plus you still get the $20 styling fee applied to your purchase. If you decide you don’t like any of the items and ship all 5 back, you will not get your styling fee back. Do you see why it is so important to be honest and detailed when filling out your Style Profile? 

Since I was on this subscription box binge and in a fashion rut, I decided to give Stitch Fix a try. I told my stylist that I spend most of the day seated in my scooter, so I needed clothes that look and feel good while sitting down. When I do physical therapy or exercise I change into fitness clothes – or just stay in my Lu La Roe because LLR is so comfortable. But again, I was in a rut and wanted to look a little more polished, especially when I went out on speaking engagements. I expected to like maybe one or two tops and that I would send the rest back, that maybe I would give Stitch Fix a second chance once I provided feedback on why I sent the items back. By the way, you have three days from the day you receive your box to get your returns in the mail. They provide a shipping label and shipping bag, all you have to do pack it all up and put in the mail. 

I opened the box and saw that they had sent me a pair of shoes. I had watched enough unboxing videos to know what the shoe bags looked like. “Uh-Oh’” I thought. I could have sworn I didn’t ask for shoes because I am so picky with them. They need to have excellent grips to keep me from slipping and sliding when I am up on my feet. I did go back and look at my Style Profile and I had requested that they sometimes send me shoes – so that was totally on me. 

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The shoe bag! Which is great for packing.

My Stylist sent me a pair of Toms. I tried them on and immediately loved them, until I tried to get up on my feet. Sigh. They were way too slick, especially on my bathroom floor. Bummer. 

I moved on to my clothing bundle. Where I found two tops, a cardigan, and a pair of jeans. A pair of jeans?! I haven’t worn jeans in years due to the lack of comfort from my scar tissue and sitting most of the day. Plus the last time I tried to buy jeans I felt like I had to try on dozens and dozens of pairs at multiple stores which ended in failure. Sigh. Well, I knew I would be a challenge to style and fit, but I was hoping the tops and cardigan would work out, so I started with those. 

Low and behold they all fit and I loved them. I then decided I needed to at least try the jeans on so I could provide feedback on why they didn’t fit. I knew they were going to be too long – the curse of being vertically challenged. Imagine my surprise when they actually fit me – everywhere! And they had some stretch to them so I was super comfortable sitting down, plus the seam on the side didn’t bother my leg with all the scar tissue! What was this sorcery? I decided to give the shoes another try to complete the whole outfit and I was blown away at how well everything looked and felt. Wow! My aunt and uncle were blown away too because we all tend to buy the same things over and over again once we know they fit so we don’t have to actually go shopping. They are both considering giving Stitch Fix a try – yes, they style men too! 

Here are photos of my Stitch Fix haul:

 

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They send you a personalized note from your Stylist and styling suggestions to go with each piece. I decided to keep the slippery Toms because they were so comfortable and went so well with everything else. I purchased some non-slip grips for the soles and they are much safer for me now. Since I purchased all the items in the box I received a 25% discount and after deducting my $20 styling fee I only paid $187 for everything in box! I hope Stitch Fix continues to hit the mark with me, it will save me so much time, effort, frustration, and most importantly spoons! 

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I love that they include a personal note from your stylist as well as outfit suggestions for the items they’ve sent you.

Wanna give Stitch Fix without having to gamble a $20 styling fee?

Use my any of my Stitch Fix links and they will waive your first $20 styling fee, and I will earn a $25 credit! Yay – we both win! Basically you get try Stitch Fix risk free. If your box arrives and you don’t like any of the items, simply ship it back. They pay for all the shipping! Plus, you may even get a referral link of your own to pass on to friends and family. What do you have to lose? Except maybe the fashion rut you might be stuck in like I was? 

*Too many spoons refers to the limited amount of spoons a person with a chronic condition has to spend in a day. Based on The Spoon Theory Theory by Christine Miserandino.

Subscription Box Therapy: Singles Swag – updated

I made a rookie mistake. I passed judgement on an item from my subscription box before having tried it. I mentioned that the socks included in my Singles Swag subscription box  seemed a bit pricey at $14 a pair. Well, yes, $7 a foot does seem like a lot for a pair of thin ankle height socks. However… wearing them made me a believer. I love these socks! I think they were designed to do yoga in, but I believe they were destined to be embraced by the feet of people like me. People like my Pompe Peeps! 

These socks are super comfortable, and while they are thin, they seem to keep my feet warm – inside the house. Have you ever seen or had to wear those so-called grip/safety socks they make you wear in the hospital? They are supposed to keep your feet warm and they have grippy stuff on the bottom. They are not comfortable, and that grippy stuff is kind of a joke for patients like me. I still slip and slide like Bambi on ice with those on. 

The Tavi Noir Savvy Grip Socks from my sub box have grip that rivals some of my shoes! My bathroom tile is super slick. And I swear these socks grip as well, if not slightly better than, my sneakers. It is nice being able to casually hang around the house in a pair of socks again. That is something that Pompe had taken away from me. 

For those of us who struggle with balance and slick surfaces – these socks are our new best friends. So much so, I had to dig the discount code out of the trash that I casually tossed out. 

So, I apologize to both Singles Swag and Tavi Noir for being so quick to judge. Pompe Peeps – check out the Tavi Noir site, you and your feet will love these socks. 

Thank you Singles Swag and Tavi Noir for giving me back sock days at home!

You can sign up for Singles Swag and other great subscription boxes at Cratejoy.

Accessible Escaping

Escape Room SWAG

Escape Room SWAG

 

What? Accessible Escaping? What does that mean?

This is a bit like my reviews of accessible travel, but here I will review the accessibly of escape rooms.

“What? Now I’m even more confused?” you say. “What the heck is an escape room and what does it have to do with living with a disability?” you ask.

Ok, first let me try to explain what an escape game is for those of you who have been living under a rock for the last few years. Escape room games have been prevalent on the internet for several years. They are mostly point and click games where you have to decipher clues and riddles to gain your freedom. Virtually, of course.

Real life escape games appeared on the scene 2007 (or so rumor, meaning the internet, has it) and since then have taken the world by storm. They are opening up all over the world and are evolving quickly as the market becomes more and more competitive. It’s a real life adventure where you find yourself locked in a room with friends, family, strangers, or coworkers. Most escape room locations will offer several game scenarios to choose from. Most very well themed and differ in levels of difficulty. You must work together while racing against the clock to solve several puzzles and escape the room. Sometimes there are other objectives, like finding a cure for a pandemic, or finding a kill code for a nuclear attack and you’re never really locked in. At least here in America you’re never really locked in, I think fire codes might have something to do with that. Some rooms have “panic buttons” that will override the lock and allow you to exit should you need to. Other doors aren’t really locked and finding that final code or key to unlock the door really only serves to stop the clock.

It is an activity that, for the most part a disabled person should be able to fully enjoy – with a little help from your co-escapees. Which, is part of the fun as you really need to work together able-bodied or not. According to my friend Leanna’s spreadsheet (yes, she did that) we have participated in more than a dozen escape rooms in the Orlando area.

What I have discovered is that escape room websites almost always fail to properly communicate whether or not their experiences are accessible or not. I have always had to call or email for clarification. Most of the time the employees are very helpful and respond within a reasonable amount of time. I just don’t understand why they can’t include this information on their website – in the FAQ section for example.  As anyone who is mobility challenged can tell you, the definition of accessibility can vary from place to place and person to person, but is it too much to ask for a little information to be placed on these websites?

I’ve found some places to be 100% accessible, the building, the lobby, the restrooms, the entire escape room. There may be some elements of the game that are difficult if not impossible for someone in a wheelchair to complete, but that is where your able bodied co-escapees come into play. I would say if an escape room game is 90% wheelchair accessible, I would go for it.

Playing escape games from a wheelchair does offer a unique advantage to you and your teammates. My sightline is at a lower level. I have spotted clues much faster than my standing pals would have. So any disadvantage my chair might have is counterbalanced with this unique and often time saving perspective.

One place said all their rooms were accessible, but to get to their location you had to be able to climb a flight of stairs because there was no elevator. So, um no, The Great Escape Room that does not make your location accessible if a wheelchair user cannot access your business – levitation having yet to be mastered.

America’s Escape Game also offers mostly accessible rooms and when I called to inquire they were extremely helpful. They spoke with their game designers and recommended what they felt was their most accessible game (The Lost Tomb of Monthu) and it was roomy enough. The problem however, with this company is that their gameplay is not necessarily a private experience. Meaning that unless you have a enough players to fill all the spots or are willing to buy up all the spots you run the risk of being placed in the room with strangers and they really try to pack those rooms. While making new friends and bonding over puzzles can be fun, being locked in a room with up to 12 people who are not used to moving out of your way can be frustrating. When my friends and I played we had a team of 6 in a room that will hold up to 10 players and it was not bad, nor was it as roomy as some of the other rooms we’ve played. I can’t imagine being in there with 9 other people.

The Escape Game Orlando has some games that are accessible and some that are not. This company also offers what is called “shared experiences” which means you may end up playing with strangers, but unlike America’s Escape Game, their room capacity is smaller (7 – 8 players) so you won’t have to maneuver around (run over, whatever) as many people. We’ve played the two games at this location and had no issues with my mobility scooter. That being said, there were only four of us in the room each time. Had we been playing with a larger group I don’t think it would have been as enjoyable. The two rooms we played, Classified and The Heist, were amazing and offered enough space for our team of 4 play comfortably. We chose a time slots that we hoped would increase our chances of playing alone and it paid off. We booked times in the morning and in between two other start times of the same the game.

MindQuest Live’s location is also accessible as are their escape rooms – mostly. One room requires you to go up an over a 1 inch threshold. That required my teammates to lift me and my chair over the obstacle – but MindQuest Live was very upfront about it and even offered to switch us to a different game if we didn’t think we could do it. MindQuest offers both shared and private experiences. So you can choose to play with just people you know or join up with others. The only game I have not played is Cyber Crash, so I cannot give a first hand account on the accessibility of that room, but all the others were just fine.

Escapology is one of our favorite locations, it was the first location we tried and therefore it is where the addiction began. They can’t add new games fast enough for us. Pretty much everything about this location and their games are accessible. There are some tight corners in some of their rooms that might be challenging for larger chairs, but they are still very accessible as a whole. Their newest room, The Lost City, is pretty snug due to the immersive theming. It is recommended for 2 – 6 players, but with a wheelchair or scooter I wouldn’t recommend trying to play with the maximum amount of players.

Escape Goat offers three different rooms, all of which are fully accessible. There is one room that may pose a challenge to those in wider chairs. If you are in a wheelchair and are planning on trying the Area 51 room, just call for clarification/measurements. Depending on the size of your chair Area 51 will either be 100% accessible or 95% accessible with an easy work around so you don’t miss any of the action. This a lovely family run business and when I visited they told me their goal is to make their experience enjoyable and doable for everyone. They even stayed open later one evening so my friends and I could play a second room.

Escape rooms are a great attraction for wheelchair users, when the business is truly accessible. Unless their website is very, very specific (Escape Goat’s comes the closest), always call or email for specifics before booking a spot as pretty much every escape room requires you to pay in advance.

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Down for the Count

It’s been over a year since I conducted a most epic gravity check. Just over a year ago I fell and broke my leg. I have still not fully recovered. After the surgeon put me back together again, he warned me it would take a least a year to completely heal. I didn’t believe him. I have trust issues with doctors (and television executives – but that is another story. Hint: Firefly). The doctor was right. Here it is, over a year later and I am still not back to the condition I was before my accident. 

So what about this epic gravity check you ask? It was late at night, I was tired from being a little late on my infusion, and I fell backwards off a step stool into a confined space and landed on my hip. In the world of Pompe, falling is not uncommon, minor bumps and bruises, minor (and major) humiliation, and sometimes serious injuries happen in our little awkward community. My previous falls had resulted in a concussion, mild whiplash, and a twisted knee. Those were nothing compared to this fall. I had no idea what I was in for as I sat there on the floor waiting for the aching to subside. I thought I could simply scoot my way to a nearby chair and get up. Nope. I struggled for hours and couldn’t move more than inch without excruciating pain. I had no choice but to call for the paramedics. Even then I thought maybe they could just help me up off the floor and into my bed. I wasn’t in pain, unless I tried to move. When the paramedics got there and examined me, they thought I might have just dislocated my leg. They felt that I would be in much more pain if something had broken. Ok, I thought. They’ll just put my leg back, it will be painful, and I’ll recover in a few weeks. I had dislocated my knee in dance years ago and it healed fairly quickly. My dad is the master of dislocating his shoulders and he always heals up fine. No problem, I can handle this I thought. 

When the paramedics moved me from the floor to their gurney I experienced the worst pain I had ever felt in my life. I would have to endure that same pain of being transferred from place to place over and over again over the course of the night. From the paramedic’s gurney to the ER bed. From the ER bed to the X-ray table – and then they had to move my leg all around to get good images of it. They did give me a mild pain reliever before they sent me to the X-ray lab. After the had me do what felt like yoga on the X-ray table, they transferred me back to the ER bed. And then I waited for them to tell me I had severely dislocated my leg. That was not the verdict. I had in fact broken my femur bone and it would require surgery. Not awesome.

A million thoughts ran through my head:

Like: I don’t have time for this!

I have a ComicCon to attend this weekend. Yes, that was my first concern. The entire cast of Torchwood was going to be there! I am still ticked about this. To add insult to injury, literally, just about all of my doctors attended the event. Making them cool doctors, but cruel people to not have taken me. With a little make up I could have been a zombie apocalypse victim. They could have just rolled me into convention the hall in my hospital bed. HMOs cover that right? 

Worst of all, I had never really had surgery before. For one of my muscle biopsies they put me completely under and I was sick for days afterwards. And now, with my official Pompe diagnosis, I knew how dangerous general anesthesia was to me. 

Luckily, I have one the world’s leading experts in Pompe Disease on speed dial. I got in touch with Dr. Byrne right away and expressed my concerns to my medical team at the hospital. Thankfully my medical team was more than happy to consult with Dr. Byrne about the special precautions they needed to take. Honestly, I couldn’t have asked for better care. They brought a respiratory specialist in to join my team to make sure they were extra careful not to put more stress on my diaphragm than absolutely necessary. 

Their plan was to do the surgery as quickly and as safely as possible. Then to get the tube out of mouth and sit me up as soon as possible. The surgery involved two lovely incisions, to place a rod and some screws into my leg to patch up the break. They stapled me back up and sent me to recovery. I woke up in my hospital room sitting up and without a tube down my throat. Good job docs! Apparently there was a recovery room I was in, but I don’t remember that part nor do I remember acting all goofy while coming off the drugs as a certain father of mine claims to have witnessed. There is no documented or photographic proof of this alleged behavior. I only remember waking up in my hospital room feeling a bit groggy with my leg bandaged up. 

The rods and screws that will reside in my leg forever.

The rods and screws that will reside in my leg forever. Which is why I am still experiencing pain. 

I spent two weeks in the hospital recovering. They brought in a physical therapist to work with me, I was able to stand on my leg the day after surgery. I couldn’t (and still can’t) pull all my weight on it, but I could stand on it. Nice going doc! 

The reason I stayed in the hospital so long was partially due to needing an infusion. The doctor on my case was reluctant send me to inpatient rehab, my next stop, before I had my infusion because they were not equipped to give it to me. Well, neither was the hospital really. They had not been trained on how to mix or administer Lumizyme, but they stepped up to the plate. Once again, my personal care team at Genzyme helped save the day too. They worked with my insurance company and got the staff at the hospital trained so I could receive my infusion. 

Two weeks in the hospital wasn’t too bad. The food was ok , I had a room to myself, free WiFi, a decent amount of TV and movie channels, and I had visitors just about every day. 

After my infusion it was time for me to be moved to an inpatient rehab center. This would allow me to work on recovering enough to be self sufficient. I needed to go from completely reliant on nurses to being able to move myself from place to place on my own. I had a lot of work to do, but the facility provided the perfect motivation in the form of sleep deprivation and inedible food. 

The physical therapists and nurses on this facility were top notch. My care was amazing. However, the living conditions just didn’t work for me. I was basically in a nursing home for lack of a better term. I was the youngest patient by decades. Decades. I shared a room with a patient who had to sleep with a bed alarm because she kept trying to get out of bed and she would fall or try to make a break for it. The alarm went off all night. Night after night after night. The food was almost comical. No, it was bad. I would take photos of it and send it to my friends and ask them guess what it was. A couple of friends would smuggle food in to me when they could. I would have killed for something from Del Taco. Killed. 

Guess the mystery meat - is it even meat?

Guess the mystery meat – is it even meat?

How many times has then been recycled?

How many times has then been recycled?

I think this was chicken, carrots, and some sort of mystery side dish made from 3 days worth of leftovers.

I think this was chicken, carrots, and some sort of mystery side dish made from 3 days worth of leftovers.

I worked hard to get an early release. To reach all my goals as quickly and as safely as possible. In the meantime I was still plotting my escape in case my captors were not prepared to let me go. I had friends at the ready with an unmarked van waiting to snatch me under the cover of nightfall. Luckily it never came to that. It might have led to prison time – where the food might have been better. I was released after one week. The director came to talk to me before she would sign the release papers. She had never had a patient make that quick of a recovery. I wasn’t looking to set any records, I just needed my own bed, and some food that I could identify by sight.  

I made it home and then went for outpatient physical therapy. This helped a lot, but I am still not back to where I was. It is frustrating to say the least. I am glad my surgery and recovery have gone as well as they have, but I am ready to be able to fully walk on my leg again. I am ready to take back the small bit of independence I still don’t have. It is just too painful to walk on that leg to this day. 

Where it really holds me back is with driving. Not with the actual driving, but the loading and unloading of my scooter. Since my leg still causes me so much pain, I cannot walk the length of my car to load and unload my scooter from the lift. Someone has to do that for me. Sometimes it is a friend, sometimes it is a valet. Yes, it has slowed me down. I can’t just get in my car and drive someplace without having someone either with me or people at both ends of my journey to help me. However, it has not slowed me down entirely. With help, I can still enjoy the theme parks and travel and cruising. I am still able to get out there and give presentations about Pompe Disease to the patient and medical community. 

My battle with gravity will never end, but hopefully soon my battle with my new metal infused leg will end and I will emerge victorious! I will Keep Fighting! 

Why do I do this?

I had a patient meeting yesterday in Knoxville, Tennessee. It should have been a a fairly easy journey – famous last words. They loaded about 10 of us on the plane and the Captain makes the following announcement: “Well folks, I don’t have good news. (At least we were still on the ground – geez!). The mechanics have inspected the plane and have found a problem with our right engine. That is all I know, but it was fine all the way here. We have stopped boarding and if you feel like you want to get off the plane you are welcome to do so. I will know more in about 10 minutes, but please expect at least an hour delay.” 

Waiting.

“Folks, it is not looking good. This plane is grounded until at least 3pm.” (Which is about 6 hours after the scheduled departure.) I found out later that the engine had to be totally overhauled and that plane was expected to be grounded for 24-48 hours. Delta did live up to their name “Don’t Ever Leave The Airport.” Seriously, they did a good job in a stressful situation. 

I was supposed to arrive in Knoxville at 1:30pm for a 6pm meeting. Delta tried to reschedule me for a 6pm arrival. When I told them why that didn’t work for me they found room for me on a flight that arrived at 4pm. The meeting was not close to the airport so the driver took me from the airport, to hotel to pick up the folks from Genzyme, and we went directly to the meeting. I changed clothes at the conference center.

The flight home was much less eventful. Except for the guy next to me who kept dropping his phone on my foot. Ouch! And the lady behind me who kept digging around in the seat pocket behind me. Yes, lady, that is my butt. I was already groped by TSA, so I’m all set.

And oh yes, I had to change planes in Atlanta – which I also hate. Not so much changing planes, but changing planes in Atlanta. Ugh! 

All that travel drama turned out to be worth the trouble. There was a patient who was diagnosed in the 60’s with some sort of “glycogen storage disease.” This patient now has a Pompe blood spot test kit and is making an appointment with their doctor. There were other key moments of this meeting, but this was the highlight. I would not wish this disease on anyone, but if it is between Pompe and another cruel disease for which there is no treatment, I think Pompe is the lesser evil. 

I would have been gutted if the plane delays had caused me to miss this meeting. This could change the course of a person’s life. This is why I put up with air travel for one night away from home in 30 degree weather. 

I am now going to curl up in my own bed and sleep until I have to get up for my next presentation. Tomorrow. Gainesville. 

Extreme Makeover: Stateroom Edition

 

Leanna and I in the Bahamas with a classic (Magic) and new (Dream) ship. May 2012.

Leanna and I in the Bahamas with a classic (Magic) and new (Dream) ship. May 2012.

Sailing on the Disney cruise ships in non-accessible rooms used to be a cause of concern for me. Used to. Being the clever person you have come to know and love, I of course figured out a way to make (most) non-accessible staterooms on both the classic and new ships work for me. Of course, a true accessible room is always best, but they are not always available. Disney used to do a really good job of  holding accessible rooms back for passengers who really need them, but now it seems anyone can easily book one. Whether they need one or not. I’ve seen people who claimed to need a walk/roll in shower because they can’t climb in and out of a bathtub, but seem to have no trouble climbing in and out of the hot tubs on the pool deck. I know people request the rooms simply because they are larger, so I had to get creative with non-accessible rooms when none are available.

 

Roll in shower in an accessible cabin. There is a fold down bench, grab bars, a hand held shower head, and best of all nothing to climb over.

Roll in shower in an accessible cabin. There is a fold down bench, grab bars, a hand held shower head, and best of all nothing to climb over.

The Classic Ships: The Magic and The Wonder

I spent two weeks on the Disney Wonder in a non-accessible room, so I figure I can make just about anything work – one way or another. There is always the option of using the facilities in the Fitness Center should I not be able to create an environment suitable to my needs. The Fitness Centers on all the ships have  very nice walk/roll in showers.

 

On the Magic and Wonder, the main bed splits into two twin beds upon request. Brilliant.  This offers more flexibility for anyone, special needs or not. We had our stateroom host remove one of the twin beds and slide the remaining bed against the wall – lengthwise. This gave me plenty of room to bring my mobility scooter in, turn it around, and of course charge it at night. I brought along my own portable grab bar. These can be found in just about any mobility shop. I’ve also seen them in Target. I used the grab bar in the shower, and not only did I use it, but everyone in my travel party used it. We felt it offered more stability than the permanent grab bars. Crossing the Pacific Ocean was a bit bouncy and the grab bar worked great for all of us. Disney Cruise Line provided a raised toilet seat to complete our transformation into an accessible stateroom.

 

The New Ships: The Dream and The Fantasy

The first time I sailed on one of the newer ships was last year on the Fantasy. We  managed to obtain an accessible verandah room. On the classic ships, the accessible verandah rooms are all located at the back of the boat and have a white wall verandah. This doesn’t bother me, but some people prefer the plexi-glass verandah walls so they can see the water without having to stand up and peer over the verandah wall. On the newer ships, accessible verandah rooms are located all over the ship, the front, middle, and back. Our room was located midship and had a plexi-glass verandah. I’ll admit the plexi-glass is nice, but not having is certainly not a deal breaker for me. In my opinion, the biggest advantage the accessible cabins on the new ships are the automatic doors. To enter your cabin you simply tap your card against the RFID reader and your door opens, stays open long enough for a wheelchair user to enter and get out of the way, and then closes. There is a button on the inside of the cabin that you press when you are ready to leave. Non-wheelchair users have complained about the “nuisance” of having to wait for the slow moving to door to completely close before they can leave.

 

This is what I used to create "steps" to use in the non-accessible cabin.

This is what I used to create “steps” to use in the non-accessible cabin.

Recently, I stayed in a non-accessible verandah room on the Dream. There were only two of us in the room, so again, we made it work. Since the beds on the new ships do not split apart it was a bit more challenging than when I was on the Wonder. Our room was a “Family” stateroom, meaning it had a round bathtub instead of a rectangle one. This geometric difference made it possible for me to bring my mobility scooter into the cabin. The round shape of the tub, which was reflected in the wall next to the bed offered just enough room for me to squeeze in. This would not be possible in the rooms without a round tub. Speaking of the round tub, while it offered an advantage regarding bringing my scooter into the room, it simultaneously presented a new challenge. The round tub was higher and much more difficult to climb in and out of than the rectangle tubs. We solved this problem by utilizing my portable grab bar again, and bringing my own “adjustable steps.” Not knowing exactly how high I would need my “steps” to be, I needed something that could be adjusted and wouldn’t become a slipping hazard. I scanned the aisles of the hardware store for something and what I ended up with was floor mats designed for children. They are ABC/123 interlocking floor mats and they worked really well. I ended up stacking 7 of them together and secured them with a luggage strap. They are light and easy to transport, but they can take up a bit of luggage space. Disney provided a raised toilet seat again and the transformation into a semi-accessible cabin was complete. The biggest obstacle in this room was getting out. Since the beds on new ships don’t split apart, I didn’t have enough room to turn my scooter around. I had to CAREFULLY back up – there was little room for error in the narrow hallway leading from the bed to the door (the regular stateroom doorways are 25.5 inches wide). My friend had to open the door and then help me navigate backing out, with practice we got better and faster at exiting the room. I could not leave the room unless my friend was with me, thankfully she was always there to help me. I’m sure in a pinch I could call Guest Services and they would deploy someone, probably my room steward, to open the door and help guide me out.

 

Location, location, location.

Some people believe the best location is mid-ship, the cruise lines fuel these fairy tales by charging more for what they deem more desirable locations. The “theory” they peddle is the midship rooms will experience less movement. I’ve been on different decks and different locations and have not noticed any difference in the rocking. I believe it is merely a ploy to extort more money. For wheelchair users, the best location is going to be near an elevator. The hallways are narrow, filled with housekeeping carts, and other guests going to and from their rooms. Additionally, if your scooter is too large to fit through your stateroom door, you’ll probably need to park it in the elevator lobby. There are electrical outlets in the lobbies, so you can charge your scooter.

 

Accessible rooms are indeed best for me, but if none are available I’m not going to let that keep me from cruising with Mickey and Captain Jack.

 

My friend Lonnie and I on Pirate Night. December 2012 on the Disney Dream. Oh, and that's our friend Donald Duck in the center.

My friend Lonnie and I on Pirate Night. December 2012 on the Disney Dream. Oh, and that’s our friend Donald Duck in the center.

Lumizyme “on the go”

In October I took a 3 week vacation, one my friend Leanna and I dubbed our Epic Disney Vacation. We spent a week in Anaheim, California exploring the Disneyland Resort, and then took a two week Disney cruise to Hawaii. My Lumizyme infusions are scheduled for every two weeks, so obviously some arrangements would need to be made. I become terribly fatigued and my muscles start to ache if I miss a treatment. It is a huge set back in my crusade against Pompe when my treatment schedule is interrupted.

I started working with my Case Manager at Genzyme months in advance to arrange a Lumizyme infusion while I was away from home. My infusion needed to be scheduled for the first week of my vacation because that was the only time I would be “on land” long enough for it to take place. Dr. Byrne asked where I was staying in California and found a colleague who worked close to Disneyland. Dr. Kimonis at the University of California Irvine agreed to take me on as a patient and supervise my infusion.

My Case Manager worked with the Case Manager for the Irvine area and lined up all the approvals with the hospital and my insurance company. I filled out my paperwork a few weeks before we left on our trip and confirmed my appointment. My first “on the road” infusion couldn’t have gone smoother. Everyone at UC Irvine was so helpful and their infusion chairs are the best I’ve sat in. Everything is push button, to recline, or put your feet up, watch TV, or call your nurse, you just push a button on a single remote. The chair even had heat and massage features.

I do not think this is something that could have been pulled off on short notice. It took a lot of people working together to make it happen. For that, I am very grateful. Had this California infusion not been arranged I would have gone about 4 weeks without a treatment. That would have left me feeling pretty cruddy during the cruise.

With careful planning it is possible to make this sort of arrangement for your infusion. Of course it depends on your insurance, where you are going to be, and probably a few other factors, but I believe it is worth trying if you want to travel and not miss an infusion. Be sure to give your Case Manager plenty of notice to help make arrangements for an infusion “on the go.”

Disneyland, October 2012 with my friends Robyn, Elysse, and Leanna. I’m full of energy because of my Lumizyme on the go!

Status Quo

 

I’m dusting off the blog. I have neglected it. Does that mean there has been nothing to report, or that I have been too busy to sit down and write about it? Well, a little bit of both.

 

I’ve been busy with speaking engagements, but that has more or less become a regular event in my life. In fact, I’m heading to New York this week for another one. I always look forward to meeting new people in the patient community.

 

So what else have I been up to and where have I been? I spoke at a United Pompe Foundation  (UPF) meeting. This time the meeting was held near Duke University, which is one of the few places that has experts who not only know about Pompe Disease, but who are researching new and better ways to fight it. The meeting coincided with clinic hours – meaning patients could be seen by Dr. Kishnani and other specialists at Duke who really understand Pompe. This was especially beneficial to patients who don’t have access to knowledgeable doctors at home. The meeting and clinic appointments were very successful, even the “veteran” patients admitted to learning new things. The dates for next year’s meeting have been scheduled for April 18, 2013. Keep checking the UPF site for more information, or visit this Facebook  page.

 

Genzyme sent me to Nashville, Tennessee to speak to their travel agency about booking travel for patient speakers. I repeated the presentation via video conference about a month later. There really is an “art” that goes into planning and booking travel for patients. I also did a few presentations here in the Orlando area for both Genzyme and the Muscular Dystrophy Association.

 

Sprinkled between my commitment to Pompe education and awareness I of course had to include some personal travel. I’ve been on a few more Disney cruises and am boarding the Disney Dream again in about a week. I went up to Atlanta to visit with my friend Lonnie – who I’m cruising with next week. In October, my friends and I embarked on what we deemed our Epic Disney Vacation. We spent a week at Disneyland in California. I even arranged to have one of my infusions at UC Irvine under the watchful eye of Dr. Kimonis. We went up to Hollywood, visited my friend Matt at the Walt Disney Studios in Burbank, and played tourists at Universal Studios. After a week of California fun we boarded the Disney Wonder for a two week cruise to Hawaii and back.  The trip took a LOT of planning, but it was well worth it because the trip couldn’t have been better.

 

So, as you can see…it has really been fairly routine….for me.

 

Leanna and I with my favorite puppy at Disney California Adventure.

The Lab Rat Chronicles

Over the summer I participated in the Amicus Therapeutics AT2220 Trial. The clinical trial is a safety study of a chaperone drug. The study drug basically does what the name suggests, it works alongside Lumizyme and “chaperones” it, making sure more if it gets delivered to where it needs to go. It is an oral (liquid) medication that is taken about an hour before starting regular enzyme replacement therapy (ERT).

I participated in the study at the University of Florida (UF) – where else? The team up there asked me to participate over a year ago – which should give you an indication about how long it takes to set something like this up and running. Additionally, it is a pretty big commitment on the part of the patient (lab rat) as well as the medical team. I had to commit to 6 separate, carefully timed appointments just to receive a single dose of the trial drug. I underwent several tests, including a couple of punch needled muscle biopsies, hourly blood draws during my infusion, and a couple of overnight stays in the hospital – all in the name of science.

The team at UF is amazing – but that is nothing new to those in the know about Pompe. Of course, I was a little nervous testing a new drug, but I knew I was in expert hands. After all, this is where I first started my ERT, before it received FDA approval and I’ve yet to grow a second head. In all seriousness, I couldn’t ask for a better place to be or with better people to work with. Not only is everyone so knowledgeable about Pompe and all the emerging research, they are simply a great bunch of people – the kind you just want to spend time with socially. It really makes having a sucky disease like Pompe a little less sucky.

The clinical trial is being conducted in 4 cohorts, or groups, each of which will receive a different amount of the trial drug. Patients in cohort 1 received the smallest dose, while those in cohort 4 received the largest. I was in cohort 3 and the trail should be wrapping up very soon. Preliminary data from cohorts 1 and 2 are showing promising results.

It could be a couple of years before the next phase of the trial begins, meaning more patients and more doses. Clinical trials are very closely regulated by the FDA and the smallest little mishap could set the whole study back. Which is why things can seem slow going, but it is really in everyone’s best interest that everything is done exactly right down the tiniest detail. The smallest error could result in a big set back.

Would I participate in a clinical trial like this again? More than likely, yes. I would have to take all the factors into consideration, it really isn’t something to be entered lightly. As I mentioned, it is a pretty big commitment time wise, which can seriously cut into my travel plans. It would also depend on who was running the trial. If it was Dr. Byrne and his crew up at UF and I had time – then, yes, sign me up.

Sincerely,
Your Loyal Lab Rat