A Family Affair

A Family Affair

When my nurse suddenly called out sick last night, the nursing agency had no one to send instead. It was extremely last minute. Plus, it was Christmas Eve and few nurses were available to work in the first place.

That meant that my mother had to stay up while I slept, in case my ventilator malfunctioned or its tubes came loose. Because a humidifier is connected to ventilator tubing, water droplets collect in the tubing. So the water must be emptied every hour. I also need medications at the end of the shift. They need to be mixed up and put into my feeding tube.

The most difficult part for anyone taking care of me overnight is obviously staying awake. Fortunately, my two sisters volunteered to keep my mother company throughout the night. I've never wanted my disease to affect my sisters any more than it had to, but I was really touched by their willingness to help.

I obviously prefer to have my nurses take care of me. They're more familiar with my routine, from medications to how I like to be positioned in my bed. But I appreciated how my family pulled together at a time like this.

Out with the Old, In with the New (Part II)

Out with the Old, In with the New (Part II)

I had my trach (tracheostomy) tube changed last night. Unlike my g-tube changes (see post from 10/5/06), trach changes can be done at home, as I lay in bed. Typically, I have it changed every other week. It is easiest to do with two people, usually my mother and a nurse. One person pulls out the old trach and the other person inserts the new one. I have had my tracheostomy (hole in my trachea) for four years, so there is less of a rush to get the new tube in before the hole starts to close, but the tube should be inserted quickly.

Once the new tube is in, the velcro ties are attached and a gauze pad is placed around the trach. (above, right). An adapter (the blue piece seen in the picture) is also connected; my ventilator tubing is hooked to the bottom, while the top (clear part)can be opened for suctioning.

The curved tube (above, left)is 6.5 mm in diameter and about 9 cm long and has a "cuff" or balloon that can be inflated with water by connecting a syringe to the piece with the thin tube attached to the trach, to ensure the maximum amount of air reaches my lungs without escaping through my nose and mouth.

However, I never fully inflate the cuff because if I do, air cannot reach my vocal cords and I am unable to speak. (Imagine me speechless!)

During the day, I keep the cuff fully deflated. At night, I inflate it partially so I am still able to call my nurse when I need assistance.

Changing the trach doesn't hurt as long as the new tube has enough lubricant on it (a little K-Y jelly does the trick). However, changing it tends to stimulate the trachea to produce more secretions (mucus), so I usually need more suctioning afterward.

The Interrogation

The Interrogation

Don't worry, I'm not suspected of a federal crime or anything like that. But I did have my pacemaker "interrogated" at a routine visit today to the electrophysiologist (cardiologist specializing in heart rhythm problems), meaning that information was downloaded from the memory of the device implanted in my chest.

My pacemaker, made by Guidant, is similar to the one pictured to the right and is about 1 centimeter thick and about the size of a doorknob, so it's really quite small. However, the left side of my chest, where it is implanted, does look bigger than the right side (in case any of you ladies out there were wondering why I only have half of a studly chest!)

Actually called an ICD (implantable cardioverter defibrillator), the device is not only capable of "pacing" my heart back into a normal rhythm, but can deliver a shock if pacing doesn't work. Pacing doesn't hurt, but I've been told a shock feels like a swift kick in the chest (hasn't happened to me so far). The ICD also constantly monitors my heart and records any unusual rhythms, even if it takes no action.

To download that information, a magnetic "wand" (left) is placed on my chest, directly over the device. The wand is connected to a laptop computer-like machine that prints a lengthy report. The process is completely painless, although I hate one of the tests that the machine performs because it briefly increases my heart rate. It feels like being in an elevator that is going down.

The report downloaded today was a good one. Over the previous three months, the ICD recorded one dangerous rhythm that lasted about a second, but it resolved on its own.

While people with Duchenne's are susceptible to fatal arrhythmias (irregular heart rhythms), I don't know anyone else with an ICD. It's a relatively new phenomenon. My ICD cannot stop the breakdown of cardiac muscle due to my disease, but it gives me a shot at surviving a sudden dangerous abnormal rhythm.

It's a chance I'll take, and that should come as no "shock" to anyone who knows me.

The Doctors of Tomorrow

The Doctors of Tomorrow

In what has become an annual tradition, I addressed the second-year medical students at the University of Pennsylvania today. After sharing my medical history, with a particular focus on the nutritional and pulmonary issues that arose while I was in college, I answered questions from the students.

Interestingly enough, most of the questions I received did not pertain not to the specifics of my disease. I was asked, for example, whether I had any siblings (yes, two younger sisters) and how my disease has impacted them. Another interesting question was about my relationship with my friends.

My favorite question was about the technology I use on a daily basis. I nearly brought the house down when I described the arguments I have with my voice-activated environmental control system (see my entry from 10/12/06). Cursing under my breath, I explained, is the only way to blow off steam at the system without eliciting a response from it.

For me, the most touching moment occurred when all of students in one of the classrooms (I addressed three separate groups) got out of their seats and gathered around my wheelchair as my pulmonologist explained the settings on my ventilator.

It was obvious that they truly cared. Translate that into a hospital setting and you have some pretty good doctors.

Just Hangin' Around

Just Hangin' Around

This post is part of a Winheld's World series on the equipment on which I depend on a daily basis.

No, that's not a medieval torture device pictured above. It's the Hoyer lift I use to transfer me between my bed and my wheelchair and between my wheelchair and the toilet. The lift is a major back-saver for my caregivers, but it is also the safest and most comfortable way for me to be moved.

As you can see above, the lift's metal chains connect at four points to the nylon sling on which I sit. The sling, pictured below, has a cut-out for toileting purposes. Thus, it is of the utmost importance that the white sling be properly centered under me!

Funny story: When the hydraulic pump on the lift started to wear out a few years ago, using the toilet proved to be quite the adventure. Every two minutes, my attendant had to come in and raise me.

"That's fastest you've ever gone to the bathroom," she remarked afterward.

Damn right!

Anyway, several companies beside Hoyer make patient lifts. There are various styles, including some whose slings can be removed once the person is sitting in his or her wheelchair. My style of lift, actually called the Hoyer Classic, is considered a dinosaur by many in the healthcare field.

Forgive me, but they aren't the ones whose asses are hanging in the air. Call me old-fashioned, but I'll stick with what's familiar when I'm the one up there!

Back to School

Back to School

It was my privilege to speak to Professor Ruth Ost's Honors American Lives class about my newly completed manuscript today at my beloved alma mater, Temple University (Journalism, 2000).

Afterwards I traveled across campus, visiting with a former professor and several friends. I also made sure to stop for a cheesesteak at Ernie's lunch truck (the red one on 13th St., between Cecil B. Moore and Montgomery). It's the best on campus!

I had a wonderful time today, but I still had some mixed emotions about my visit to Temple. My time as a college student was the greatest period of independence in my life. Since then, I've become much weaker and far more dependent. On the other hand, at least I'm still here and able to relive those great times.

Though I fondly recall my college days, it sure was nice to be delivering today's lecture as opposed to listening to it!

THE BOOK IS DONE!

THE BOOK IS DONE!

I'm pleased to announce that after just over a year, I have finally completed the first draft of my memoir.

It has been an interesting ride, to say the least, and one which has challenged me intellectually, emotionally and even physically. I have learned more about myself and about others and their perceptions of me than I had ever imagined. I've revisited countless memories, both good and bad. And I have reconnected with so many friends with whom I had lost touch over the years.

Many thanks go out to all of my family, friends, doctors, and nurses (and anyone else I have forgotten) for your patience, support, and assistance. I could not have reached this point without you!

As I move forward in my quest to get this thing published, I will post any exciting developments right here, at Winheld's World...

And the Beat Goes On...

And the Beat Goes On...

Paid a visit to my cardiologist today. In heart failure when I started seeing him four years ago, I'm doing a whole lot better today. My condition remains serious -- that's why I have a pacemaker/defibrillator implanted in my chest -- but I consider myself fortunate to be in the position I am.

My checkup went so well that we were able to shift the focus to the doctor and his wizardry (or lack thereof) with the new computer equipment in the office. It's a good thing he has a better understanding of how the heart works, because I certainly wouldn't ask him to show me how to navigate the internet!

Thanks, doctor, for making everything possible in my life...

Coming Down the Homestretch

Coming Down the Homestretch

I completed the next to the last chapter of my book late last night. It covers the period from about ages 5 to 10. Events described include standing up to a school bully, serving as a local poster child for the Muscular Dystrophy Association (MDA), and going to MDA overnight camp for the first time. I could tell you more, but then you wouldn't read the book!

As for the final chapter, I have a few ideas knocking around my head, so hopefully it will happen quickly. But after making and breaking several deadlines, I'm making no bold predictions this time.

Stay tuned...

Fire and Rain (A Lot of Rain)

Fire and Rain (A Lot of Rain)

I saw James Taylor perform last night at the Tower Theater in Upper Darby. My father introduced me to Taylor's music at a young age, and I've been a fan ever since. In 2005, I often listened to his soothing music while recovering from pacemaker surgery, a very emotional time for me.

But with the monsoon-like weather outside, it could not have been a worse night to venture out. When my parents and I arrived, I desperately needed to have my trach suctioned. However, we first had to figure out how to assemble my new suction machine. (If you're wondering why I didn't think of that beforehand, don't worry; I've already heard that one!) Then, I nearly got killed by a car as I crossed 69th Street!

Fortunately, James Taylor didn't disappoint, performing favorites like the aforementioned "Fire and Rain", "Carolina In My Mind ", and "Sweet Baby James". So while it was a bit of an adventure getting there, the concert was well worth the effort.

Like a Fish out of Water

Like a Fish out of Water

With the headset for my environmental control unit (ECU) in the shop for repairs the past two days, I have felt helpless. The voice-activated ECU -- which makes it possible for me to answer and dial the telephone, control my stereo, TV, VCR and DVD player, operate my hospital-style bed, and even page my nurses when they are not in the same room with me -- has become a vital part of my life.

While it is true that I have a nurse with me all day who could certainly help me answer the telephone, for example, there is nothing like being able to do things for myself. Before purchasing the ECU, I never realized how great that felt. But after owning the system for more than a year, I had come to take it for granted.

Having lost much of my independence as my disease has progressed over the years, it's important for me to maintain as much of it as possible. That's what makes the ECU so wonderful.

I should have the repaired headset back by Saturday (overnight shipping is a godsend) and then all will be well. That is, until the first time I tell the system to change the channel on the TV and it answers the phone instead. Then, things could get nasty!

Out with the Old, In with the New

Out with the Old, In with the New

I had the my feeding tube changed today in the Interventional Radiology (IR) department at Children's Hospital of Philadelphia (CHOP)*. Through the feeding tube, also called a g-tube -- "g" is short for gastrostomy -- I receive medicines and liquid nutrition similar to Ensure. Normally, the 4 cm long tube (pictured above by itself on the left and in my abdomen on the right with a gauze pad against my skin) could be changed at home, but because I have what is known as a "false tract", the new tube tends to end up in that tract instead of in the stomach (not good). So every three months, I go the hospital, where a wire is inserted through the old tube while still in my stomach. The balloon holding the tube in place is deflated using a syringe and the tube is pulled out. The new tube is then slid over the wire and its balloon inflated. To confirm placement, an x-ray of the stomach is taken while a contrast dye is injected through the g-tube.

Piece of cake? You try having someone yank something out of your stomach sometime! Not only that, but getting on the table is complicated by the fact that my knees are contracted and I cannot keep my hips from flopping out to the side. I need restraint ties and several towels and pillows to safely position me. I bring my Hoyer lift from home because it's the safest way to move me. But it does not reach the level of the table, so some careful maneuvering is required!

It's not exactly my idea of fun, but with the help of my nurse and the considerate IR staff (thanks guys -- you're the best!), I get through everything just fine. As for the discomfort from the tube change, it's relatively mild and is usually gone within a day or so.

*You may be wondering why I still go to a pediatric hospital at age 28. The reality is that until fairly recently, few Duchenne's patients reached adulthood. That's obviously changed, but the best clinic remains at CHOP, so doctors, nurses, and other staff are familiar with the disease.

May the Force Be with Him

May the Force Be with Him

I'd like to take a moment to wish the best of luck to Frank, my nurse for the past three years (pictured with me today, outside the Philadelphia Museum of Art), as he moves on to a new job. Winheld's World won't be the same without the big guy with a penchant for fly-fishing, football, and twisted humor. Dedicated and reliable as a nurse, he was (and still is!) the kind of guy to go with to ballgames, movies, and on roadtrips.

Filling his shoes won't be easy, but a couple of promising candidates will give it a shot. There will be a necessary period of adjustment for me, but it's nothing I haven't been through before. As Frank himself reminded me, it even took me a while to "break" him in!

Thanks for everything, man!

Defying the Odds

Defying the Odds

I attended the 30th birthday celebration of a good friend with Duchenne's today. As recently as 10 years ago, few people with the disease reached 30. While there remains no treatment, advances in medical technology such as spinal fusion surgery, portable ventilators, effective cardiac medications and tiny pacemakers have made it possible for people with Duchenne's to have longer, healthier, and more productive lives.

At 28, I feel fortunate to be here. No two people are affected the same by Duchenne's, to be sure. Yet in celebrating with my friend, I couldn't help but feel more hopeful for my own prospects.

Now, if only I felt as good about the prospects of my beloved Phillies!

The (Virtual) Check is in the Mail

The (Virtual) Check is in the Mail

Several years ago, I wrote a column in a local newspaper praising online shopping as a convenient alternative for people with physical disabilities. Last week, I became a new fan of another internet innovation -- paying bills online. A few minutes after registering on my bank's website, I paid my very first bill online (to the telephone company, in case anyone is curious).

Paying bills online is convenient in general, but especially for someone like me. Unable to physically sign a check, I have a joint account with a relative, so that someone else can sign my checks. Trusting that person isn't an issue, but at 28, it's about time that I take full responsibility for my own money. By paying my bills online (thanks to my computer's accessibility features) , I can do just that.

Just another way the internet has made my life more accessible.

A New Attendant in Town

A New Attendant in Town

I recently hired a new attendant -- to get me out of bed in the morning, brush my teeth, shave my face, and get me set up for the day. It is usually hard to find someone who is reliable, pleasant, motivated, and not too heavy-handed (I could tell you some stories, but you'll have to buy my book!), but I seem to have lucked out this time.

That doesn't mean that training a new attendant -- even a good one -- is a walk in the park. Trust me when I say it's not very easy to direct someone where to move a toothbrush in your mouth with a mouthful of toothpaste. Or that it is scary teaching someone how to operate the Hoyer lift that moves me from my bed to my wheelchair because I could be crushed, dropped or whacked with a metal bar. Or that I get nervous the first few times someone moves my legs to attach the legrests to my wheelchair, as my knees cannot be extended more than 90 degrees without hurting them.

Mistakes can always happen, but if I find an attendant who is willing to listen and really seems to care, I'll take my chances. So far, so good...

Feeling Invincble

Feeling Invincble

In the midst of learning how to use Blogger, I forgot to write that on Friday, I saw "Invincible", the story of former Philadelphia Eagle Vince Papale (played by Mark Wahlberg), a 30-year-old teacher and bartender who made the team when coach Dick Vermeil held an open tryout.

With the NFL season looming, I was so inspired by the film that I wanted to get out of my wheelchair and tackle someone! Actually, it made me want to hurry up and finish writing my book. I really identified with Papale's drive to make something of his life. In a way, I suppose that's why I'm writing a book. Having muscular dystrophy doesn't make it easy, but it doesn't mean I can't do something positive with my life. I truly believe my book has the potential to help people dealing with medical challenges like mine.

Is It Safe?

Is It Safe?

I spent yesterday morning at the dentist -- no cavities to report! When you have a disability like mine, finding a dentist can be a challenge. Many offices are not wheelchair accessible. Once inside, many dentists won't work on patients unless they transfer out of their wheelchairs. I cannot sit in a chair other than my wheelchair, which is designed to support me. Fortunately, my childhood dentist, whose practice is normally limited to children, still sees me. I position my wheelchair near the dental equipment and use its tilt mode to make it possible for the dentist to reach me.

(By the way, if "Is it safe?" doesn't mean anything to you, get yourself to the video store and rent "Marathon Man", starring Dustin Hoffman and Laurence Olivier. Well, maybe wait until after your next dental visit!)

The Maiden Voyage

The Maiden Voyage

Welcome to Winheld's World!

I never saw myself writing a blog (blogging seemed like reality TV, which I despise), but here I am. Someone suggested a blog would be a good forum to talk about the book I'm writing (see my profile) and that's how the idea was hatched.

Similar to my book, this blog serves a dual purpose: helping people/families going through diseases like Duchenne's (again, see my profile) and educating the medical community and other professionals who interact with us on a daily basis.

I hope you will find Winheld's World informative, insightful, and perhaps even humorous at times. So welcome aboard! And please check back often...