Erik Markusson: My Story

People living to be 40 years old with the Duchenne muscular dystrophy is rare.  I just turned 40 so I felt compelled to share my life story and philosophy about living with Duchenne’s with you.  I wrote this especially for others who have muscular dystrophy, their caregivers, and the people close to them. I hope it’s helpful and inspirational for you.

I was born in 1965 in Akron, Ohio. As with other Duchenne patients I was born with the mutation. The particular variant of the mutation I inherited was one which doesn’t affect the brain. At that time there weren’t prenatal tests to spot the mutation. The manifestation of symptoms didn’t appear until I was around 4 years old. I had an abnormal gait and poor balance, which were suspicious. At that age an unaffected child should be able to catch their balance, which I was never able to do. Thus my parents took me to University Hospital in Cleveland, where research on muscular dystrophy was being done to determine a cause of the abnormality. Ultimately a biopsy was performed to confirm that I had Duchenne muscular dystrophy. As I got older my scoliosis — a sideways spinal curvature — and lordosis — a “swayback” spinal curvature — became more pronounced. In the ’60s, orthopedic procedures like hooking metal rods to the spine to prevent these conditions weren’t available. As my legs became weaker they were less able to support my weight. When I was 10 I could no longer walk or stand, which is typical for Duchennes. Thus I became confined to a wheelchair.

Even with my disease my early childhood was fairly normal until I needed a wheelchair.  I was able to walk well enough to play outside. At my house in the woods I hiked around by myself. I was also able to visit my friends at their homes. However, I wasn’t able to run very well. So I couldn’t participate in organized sports. In spite of this I was able to participate in many other activities. In the summer I was able to swim a little bit at the local lake. Every summer my grandparents would take our family on the annual outing to Cedar Point, a big amusement park in Ohio. During the winter I built snow forts and made snow angels and snowmen. Occasionally we went down the street to go sledding. Having full use of my hands I was able to build things with Legos and Tinker Toys. I could do other things requiring use of the hands. My dad helped me build a Pinewood Derby type car out of balsam wood for a group called Indian Guides. One year my car won the big race.

When I reached school age of 6 years old my parents insisted that I attend the local public school along with everyone else. This was before mainstreaming was widespread. I think it was one of the most important decisions in my life. I think it was important going to a regular school so I would be challenged intellectually. This probably wouldn’t have happened if I had gone to a special school. It was also important for my social development. My self esteem was boosted by going to public school because I didn’t feel any different from the other kids. Along with the other kids I was allowed to walk home from school, which was about 5 or 6 blocks away. The only thing I couldn’t partake in was gym class. Growing up I just wanted to be like everybody else.

Throughout my school experience there were only a few kids that weren’t nice to me. So I just stayed away from people like that. For the most part kids treated me nicely. Making friends never seemed to be a problem. In fact, I’m still in touch with my friend from second grade. I made many friends that I would not have if I had gone to a special school.

When we moved to Denver in 1975, a major criterion for my parents in their decision of where to live in the metro area was which school districts mainstreamed handicapped students. At that time, only two districts were mainstreaming, Jefferson County and Boulder. Thus we chose to live in Evergreen which is in Jefferson County. So I was able to continue going to the local public school.

Throughout my school career I took the same classes and did the same work as the other students. I was graded with the same criteria as everyone else. The only assistance I required was physical. From seventh grade on I rode the bus to school. The bus had a lift so I could take my power chair with me. That made a huge difference in my independence since I didn’t have to rely on someone to push me everywhere. As my disease progressed I needed more help. In high school I had an aide to help me, primarily with the bathroom, getting lunch and any other time I might need help. Fellow students helped me some as well. Evergreen was on multiple levels, so I received a key to the elevator. I never required any medical assistance because I wasn’t on a ventilator at that time.

I was also able to participate in many of the extracurricular activities. In both Ohio and Colorado I was able to go on all the field trips. Sometime during sixth grade, Jefferson County students attend the Outdoor Lab School. At this school students are mostly taught subjects dealing with the outdoors. Students live there for a week. It was arranged for me to attend this school. During my ninth grade year I took stats for the football team. In high school I participated in the band and French club. With the French club I participated in many activities such as progressive dinner parties and picnics. When I was a sophomore I rode on the French club float in the homecoming parade. I performed with the pep band at every home football game. High School students are eligible to be counselors and teach some of the classes at the Outdoor Lab School. When I was a sophomore I taught a class on soil conservation at the school.

In addition to participating in school activities I was also a member of the Boy Scouts. I was able to participate in many of the camp outs. Though I couldn’t physically do many of the outdoor skills I still learned them. If I went camping today I would know what to do. I was inducted into the Order of the Arrow, an honorary group in Boy Scouts. This required me to sleep outside without a tent. Since I wasn’t able to sleep on the ground at the time I was allowed to sleep on a cot. It took me six years to earn my Eagle award. The required badges needed to earn Eagle that I wasn’t able to do were allowed to be substituted with another one. It also required me to plan and carry out a service project. I ended up doing an erosion control project at the entrance to Roxborough State Park. I felt a real sense of accomplishment being one of the first handicapped Scouts in the Denver area to earn the Eagle award. I hope it paved the way for other handicapped Scouts to do the same thing.

I’ve been fortunate to have a great family that has included me on vacations in spite of it requiring a few more logistics. I believe it’s very important for Duchenne patients to be included in their family’s activities. We’ve been on many different kinds of vacations from car trips to air travel. This even included four separate trips to Europe. The logistics for traveling in Europe were more involved than in the U.S. But Europe was remarkably accessible despite the cobblestones, which can be difficult. The most difficult thing we did in Europe was getting me to the top of the Acropolis in Greece. It involved carrying me and my chair up numerous steps. Fortunately we had hired a guide to help us get around Greece. He helped quite a bit. I took all these trips before I needed a ventilator.

After I had a tracheotomy, I was still able to fly because I could be off the ventilator for long periods of time. However, we stopped going overseas because that was too long for me to be off the ventilator. I was also a little hesitant about leaving the country with my extra medical needs that the tracheotomy had created. So we just flew within this country, primarily to North Carolina.

We’ve been going to the beach in North Carolina since I was 5 or 6. My family owns a beach house there. It is right on the beach so I can go on the back deck and see the ocean. When we lived in Ohio we drove to North Carolina but when we moved to Colorado we had to fly there. In 1995 flying became too difficult. Consequently we started driving there. We rented a van with a lift which allowed me to bring my power chair. We eventually bought the van. When we’re traveling in the van my ventilator is powered by an inverter that plugs into the cigarette lighter. Thus we don’t have to worry about how much charge is in the ventilator batteries. This allows us to travel long distances. Thus we’ve been all around the country in the van. It’s been a great acquisition because I love to travel.

Each summer I also attended the annual week long MDA Summer Camp until I was 21. This camp lets MD patients forget about the disease and have a great time participating in typical camp activities. It’s similar to any other summer camp except that the campers are handicapped. My favorite activities were fishing and arts and crafts. In arts and crafts I got into building model rockets. It provided a great opportunity for the campers to participate in activities and do things that they may not otherwise be able to do. At camp I developed many long times friends with the counselors and fellow campers. It provided a great opportunity to talk with people sharing similar experiences. I still keep in contact with the counselor I had when I was 10 years old. The week is a nice break for the caregivers and patient. For those who aren’t able to go on a vacation, camp can be the vacation. It’s a week I wouldn’t want to miss.

After I graduated from high school I immediately enrolled at the University of Denver the following fall. It was easier to live at home and commute than to live in the dormitory. At that time I wasn’t able to take notes so note takers were arranged for me. When one wasn’t available my mom took them. When classes weren’t accessible they were moved to a building I could get to. At the beginning I was still able to write so I could take my tests in the classroom with the other students. When I lost that ability and had to dictate my tests I took them at the handicapped students’ center. The handicapped students’ center arranged all the necessary accommodations.

Academically I chose to pursue a BA degree in biological sciences, which also required a minor in chemistry. This wasn’t the easiest or most practical choice for a degree but I had always been interested in biology so I pursued the degree. The two departments were very helpful in working with me so I could earn the degree. For example, the building where the chemistry lab was held was inaccessible, so the chemistry professor let me use his lab. A teaching assistant helped me set up and run the experiments in the lab, but I had the responsibility for knowing what to do. Fortunately the biology lab was accessible so I was able to do the lab with the other students. Since we worked in groups of 2 or 3, I didn’t have to worry about something I couldn’t physically do. In biology lab I was still able to use a microscope but it would wear me out. When it was possible the microscope image was projected onto a screen. Working in the laboratory was a great experience I was glad to have partaken in. Though I wasn’t able to physically use the equipment I still knew how it worked.

Though the University of Denver may not have been the most accessible college, the people made it work. The university had the advantages of being small, so in many cases it was easier to make the various arrangements with professors directly. There were two good examples which illustrate this point. When I was taking chemistry I got sick and missed an exam. Instead of having me come down and making up the exam he let me do it at home. One time my Russian history [professor] let me give an oral book report as opposed to written one.

While I was attending college my mom took some classes too. Our agreement was that I would take the classes I wanted and I would take one of hers. This was necessary because I couldn’t be by myself because of my medical needs. This accomplishment wouldn’t have been possible without my mom’s help, and my mom got her master’s degree in the meantime. Due to my needs it took me eight years to graduate from college.

One factor that caused me to take that amount of time was that I got sick from influenza during my second quarter of college.  This caused me to miss two quarters. As a result of missing those quarters I had to re-enroll. But the most important thing was I got the diploma which I proudly have hanging on my wall.

Going to college and graduating was a great example of how I didn’t let Duchenne prevent me from doing something I wanted to do. To me it’s better to try and fail than to not do something and wonder if you could have.

As I mentioned before I got sick from influenza during my second quarter of college. As a result of the illness I had to have a tracheotomy and a gastrostomy tube put in. Getting to the hospital was quite an ordeal. I had been sick for a couple days when my mom took me to the doctor’s office. While we were waiting to be sent home with oxygen, I passed out. This is where I got lucky, because if I’d gotten home and passed out during the night the outcome might not have turned out as well. I ended up having to be transported to Children’s Hospital by helicopter. This is when I got the tracheotomy and a gastric button. I was in the hospital for eight weeks on a ventilator and oxygen recovering from influenza and it complications.

After I got home I had to wean off the oxygen I was using. It took me about a month to do this. At the same time I got to where I used the ventilator only at night. These tasks were made harder because I live in Evergreen at 8,000 feet. Now I don’t normally use oxygen. This ordeal was traumatic but it may have been a blessing. Both the tracheotomy and gastric button were life sustaining procedures which I would have needed soon. Before this episode I hadn’t noticed any difficulties with my breathing. I did have trouble with eating however. For about the previous year I had been having trouble getting enough calories. I would get physically tired trying to consume enough calories, and getting tired required more calories. It was a vicious cycle that was finally catching up with me. When I got sick I was beginning to lose weight. I don’t know how I’d still be alive without those interventions.

My increased medical needs required professional nursing care. For the previous four years we hired companions to stay with me when my mom wanted to go out. At that time I had no medical needs that required nursing care. Whether it’s professional nursing or just a companion, it provides a needed break for both the primary caregivers and the patient. This puts less stress on the relationship between primary caregiver and patient than there otherwise would be. Caring for a person with Duchennes is a little easier in a two parent family. Thus with just a single parent this type of relief became even more important.

I’ve had many nurses over my 21 years of nursing care.  In that time I’ve also had six home-health-care agencies. I became friends with some of them since when they worked with me I saw them almost every day. I even remained friends with some of them after they stopped working with me. Even if we weren’t friends I would be friendly toward them because I try to be the best patient I can possible be. Currently I have nursing care five days a week and an occasional evening or a weekend day. There’s been three occasions where I’ve had 24-hour care. I had it when my parents took my sister to college and when she graduated from college. I also had around the clock nursing when my parents hiked the Grand Canyon. After I turned 21 my nursing care was payed for with my Medicaid. At the current time I also have a private nurse that works with me occasionally, which is paid for out of pocket. I use part of my Social Security for this purpose. I think being able to use home health care has kept me healthier than I would be if I was in a nursing home or similar institution.  It also lets me stay more independent.

Surviving that crisis was very fortunate because I got to experience some things I would have missed. First and foremost I was able to graduate from college in 1992. In 1997 my sister got married and I was asked to be one of the groomsmen. I also am having the opportunities to know my nephews.

For the two years after I graduated from college I took a few graduate courses so I would have something to do. I’m glad I took those courses because it was a great experience. It furthered my knowledge and I got a glimpse of what graduate school might be like. In one particular course we were each assigned a topic, which we then had to present to the rest of the class.

It was about this time that I lost the ability to hold my head up without support. Therefore, I had my wheelchair adapted to have a headrest and recliner. Without it I wouldn’t have been able to take those courses. My adapted chair and the lift on the van have allowed me not to be homebound. I get out about once a month whether it’s to go to a restaurant, see an exhibit at a museum, or visit my sister and her family. I’m an annual Cherry Creek Arts Festival visitor. It also enabled to continue going to the beach, which I really enjoy. At home I was relegated to reading and watching television. Fortunately we had gotten cable so I had a wide range of viewing choices. I’ve kept up with my field by subscriptions to Scientific American and Science News. Reading books as opposed to magazines is harder, so I listen to audio books instead.

In 1998 I purchased a computer with adaptive equipment so I could use one. When I first got the computer I used DragonDictate, a program which allowed the computer to be operated through voice commands. As my lungs weakened my voice got weaker so I had to find some other adaptive equipment to use. Currently I’m using Quadjoy and MagicCursor, which enables me to operate the computer with my tongue. I can also type with a program called ScreenDoors, a virtual keyboard. It’s been very important being able to use a computer. When you lose the ability to use your hands it limits the things you can do. Most of the abilities I lost with my hands I regained with my tongue. I also play cards and chess, which I especially missed. I’ve also created my own website and update it occasionally. Blogging and sending e-mails also occupy my time. Sending e-mail has allowed me to keep in better contact with my friends and family than I could before e-mail. It’s especially valuable since using the phone is difficult. My computer has made my life since I graduated from college more fulfilling than it would have been without it.

Also in 1998 I began using a Pasy Muir valve (PMV). It enables a ventilator patient to speak while on the ventilator. In fact this valve was invented by David Muir, a young man with Muscular Dystrophy. One of my nurses told me about one of her other patients that was using one. After that I decided to try using one. I’m still able to use a shorter tracheostomy tube with allows me to talk, but not easily without the support of an ambu bag. It’s also very exhausting. As a result my annunciation suffers and I tend to mumble. The PMV is by far the best way for me to talk. While talking with the PMV I don’t get as tired and I annunciate my words much better. It also enables me to clear my secretion above the tracheostmy tube so I won’t lose my ability to talk. From experience I know it would be very frustrating not to be able to talk at all.

In 2004 I got a new wheelchair, which I can drive with my tongue. The main reason I got a new chair was that my old chair, the one I had used since high school, was becoming outdated and hard to fix. As long as I was being fitted for a wheelchair I decided to get one I could drive. I love it because I haven’t been able to drive my chair since 1994 when I lost that ability. I prefer to drive myself because I have more sense of control. It’s also the first chair that has been fitted specifically for me. It has a fitted back that makes me sit up straighter than I have in years. I didn’t realize how much I was leaning until I got into the new wheelchair. It’s the only chair that I can sit in.

In August of 2005 I developed some heart rhythm problems which caused me to develop an irregular heart beat, which caused it to become dangerously slow. Needless to say this required hospitalization. During my two-day stay in the hospital it was decided that I needed a pacemaker. In the process of deciding what to do I had an echogram taken of my heart. It showed no signs of muscle deterioration, which I had expected to see. My heart was also the normal size, which was unexpected as well. The pacemaker seemed to correct the problem because my heart rate went back to where it was before. I found out at my three-month checkup at the pacemaker clinic that my heart was doing most of the work with only occasional help from the pacemaker. This was another instance where my life could have ended.

After about six months I developed some complications with the pacemaker. At that time the incision still hadn’t fully healed.  When the lead started to show I had to go see the surgeon who had put the pacemaker in. I turned out to be infected, so I had to go to the hospital to get it replaced. All that went fine, but a week later I bled a bit from the incision where the chest tube had been. It got into my chest, which caused lots of pain and thus affected my breathing. Needless to say this delayed my recovery, but I persevered and I’m now back to where I was with the original pacemaker.

Currently I’m still not totally ventilator-dependent. I can disconnect the ventilator for almost three hours so I can breathe on my own. When I get winded, which is quite often, I use an ambu bag for support. I have no use of my limbs except for my left thumb, which I use to ring a call button. I also have severe scoliosis, lordosis and contractures. My altered posture causes my right leg to cramp when I sit in my chair for a real long time. Otherwise these conditions only cause occasional minor pain. However, the contractures do cause significant pain if my extremities are stretched too far. I still have the full use of my facial muscles and my tongue. This excludes the muscles used for chewing. I’m able to chew by pushing the food against my front teeth with my tongue.  I do have to be deliberate when I eat because my swallowing is somewhat comprised by the disease. I’ve remained continent so I don’t require a catheter. Due to the disease slowing down the bowel muscles I’ve had occasional issues with constipation. I use lactolose, which keeps my bowels functioning properly. Fortunately, with the physical complications I didn’t get the variant of the disease which causes mental problems.

After living with Duchennes for 40 years I have formed a definite philosophy for coping with the disease. The most important traits to have are perseverance, patience and a positive attitude. Self pity and using the disease as an excuse will get you nowhere. With the uncertainties of the disease, don’t worry about what’s going to happen tomorrow, deal with it when it comes. As the disease progresses however, you may want to keep in mind that your life is going to be shorter than the average person. So, it’s still important to set goals. The saying “use it or lost it” is very pertinent to muscular dystrophy. The longer you use your abilities, the longer you’ll have them. As you lose certain abilities however, it’s important to focus on what you can do, not what you can’t. You shouldn’t let the disease prevent you from doing something. You also shouldn’t let someone discourage you from doing something because of your handicap. You might discover that you can do something you didn’t think you could do. Another important thing you have to cope with is how you relate with others. It’s important to be friendly and courteous. However, if someone can’t look past your disability, don’t concern yourself because it’s their problem not yours. As the disease progresses and you become more dependent on caregivers it’s even more important to try and be patient: You’ll get less frustrated and you’ll have a more cordial relationship with the caregivers. The caregivers should also show the same consideration.

Why have I been able to live as long as I have? There are several important factors I can point to and a few intangibles that may be contributing to my life span. There have been three medical interventions that have extended my life, a tracheotomy, gastric button and a pacemaker. Having a tracheotomy tube allows me to be hooked up to a ventilator, which assists my breathing.  This has extended my life by 20 years. The short amount of time that I can disconnect the ventilator and breathe on my own I think has kept my lungs stronger than they otherwise would be. The gastric button allows me to get the amount of nutrition and hydration I need to be healthy. On my own I wouldn’t be getting enough calories and hydration. This past summer I developed some heart rhythm problems which required me to get a pacemaker. Without the pacemaker I would have died. Along with the medical interventions I’ve received excellent care between my parents, nurses and various doctors. Another factor is probably my excellent overall health. Contributing to my excellent health is my annual influenza shot, which is critical for Duchenne patients. When I do get sick, the ventilator makes it easier to fight the illness. An ordinary cold is much less likely to develop into something life threatening. Lastly, I think my positive attitude has helped as well. And of course, I’ve been lucky.

If I were asked if I would change anything about my life I’d probably say no. This isn’t to say that I’d rather not have Muscular Dystrophy, it’s an awful disease Duchenne Muscular Dystrophy presents many obstacles and complications. It often leaves you down and frustrated. The disease does tend to shape who you are. But the disease doesn’t define you.

A diagnosis of Duchenne muscular dystrophy isn’t a death sentence. It will shorten your life and causes complications and obstacles which can be overcome. You can have a fulfilling life with it. Though you’ll be given a prescribed life expectancy, you shouldn’t have any preconceived ideas of how long you’ll live. My life is a prime example of this. With today’s medical technologies like ventilators, Duchenne patients are living longer than ever before.

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