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Living With Wolfram Syndrome – Adam Zwan

Running Out of Senses

Image of 5 SensesWolfram Syndrome patients, like me, suffer from optic atrophy and deafness. It’s a belief that deaf and blind individuals rely on their other senses to experience life. A great example is Helen Keller because she had to depend on taste, smell, and touch to accomplish all of her lifetime endeavors. Unfortunately, vision and hearing are not the only senses that Wolfram patients struggle with.

In my case, I have lost most of my sense of taste and smell so those are two areas I cannot rely on to live a daily routine. As far as food and drinks go, it’s not really the taste that helps me distinguish what’s for dinner but the texture. When I cook a meal I use seasonings not to change the taste of my food but to create a crispy coating that satisfies my mouth with a crunchy texture. When it comes to drinks, I’m not able to acknowledge how sweet, salty, or spicy a beverage is but how silky, smooth, or grainy it feels on the tongue.

My sense of smell is even weaker than my sense of taste but I try to think of it as an advantage in some cases. Sometimes when I am preparing some food for myself there is an aroma that everyone except for me can sense. Whether I am burning my food to give it a crispy texture or I am gathering roses for Valentine’s Day my sense of smell makes no difference. On a lighter note, I am thankful I can’t smell after my father eats Mexican food and or a person smells like he or she works in a perfume factory. If I live past the life expectancy of 28-31 years-old there is a chance that Wolfram nerve damage will spread to my brain stem and result in paralysis, which would end my sense of touch. Again, it is unfortunate that I cannot rely on any of my senses except for touch but I make it through the day by reminding myself of every positive factor associated with Wolfram Syndrome, like not being able to smell rotten eggs on Easter.

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Living With Wolfram Syndrome – Adam Zwan

All Gassed Up

wolfram syndrome and gas issuesNerve damage caused by Wolfram Syndrome results in many lifelong complications, some minor and others very severe. Eating and drinking has been one of the never ending issues I have struggled with. Regarding food and drink, issues experienced by Wolfram patients consist of glucose fluctuations, mood swings, low tolerance for specific food groups, as well as metabolic problems.

In the past few years, my metabolism has suffered from nerve damage making eating and drinking feel like a chore. My diagnosis is called Gastro Paresis and it is caused by pancreatic and metabolic nerve damage. After the pancreas has experienced nerve damage, the body runs into a deficiency of metoclopramide, which is a pancreatic enzyme that promotes proper contraction in the metabolism. The symptoms I have experienced are gas, bloating, nausea, acid reflux, heart burn, and indigestion. I have been prescribed a metoclopramide pill but unfortunately the symptoms still exist. The purpose of the medication is not to defeat my symptoms but to prevent my metabolism from falling asleep and resulting in a hospital visit due to constipation.

I experience gas, bloating, acid reflux, heart bun, and indigestion each time I have something to eat or drink so eating and drinking is more painful than pleasurable. I know that my circumstances could be better but they could always be worst. I just remind myself to count my blessing and be thankful that I can still chew and swallow because a feeding tube is the next stage.

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Living With Wolfram Syndrome – Adam Zwan

The 2014 holiday season was a great one; lots of food, laughter, and stories of the past. Starting in October and lasting until January celebrating holidays is the thought on everyone’s mind. Celebration events do not occur without some withdrawals, especially for a Wolfram patient.

Beginning with Halloween and finishing with a New Years Day meal the eating is something that always brings my family together. With diabetes, gastro paresis, and low tolerance for fiber eating well takes on a whole new meaning. As a Wolfram patient, times of eating seem more like a chore than an enjoyable event. A Wolfram patient, like me, has to put a lot of time and effort into measuring, avoiding, and denying foods because eating the wrong thing could mean a visit to the hospital. Also, due to my increased sensitivity to insulin, gaining ten or so pounds results in glucose fluctuations. However, thinking about research efforts and searches for a cure help health struggles seem like just an event on the timeline.

Furthermore, my holiday season was filled with stories of life experiences that brought smiles, inspiration, and or jealousy. In my case, jealousy was a common feeling while family members spoke of vacations, relationships, and living independently. I’m 27 years old and listening to stories from family members makes me ask myself whether I have really lived yet. Reason being is that almost all of my stories pertain to health issues, hospital visits, and longing for changes in life. Happily, if research continues and success is achieved then I will be able to tell stories of my vacations, relationships, and independent living. All in all, the 2014 holiday season, minus a few health worries, was a memorable one.

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Living With Wolfram Syndrome – Adam Zwan

Wolfram syndrome patient Adam Zwan and family at Christmas time

The Zwan family celebrating Christmas with their festive holiday sweaters.

A Little Worried?

I feel lucky to have a family that loves me as much as they do, but like everything in life, there are some consequences. Before being diagnosed with diabetes and then Wolfram Syndrome, I could visit family members and make it an enjoyable event. I still enjoy seeing family, but my presence has changed from a pleasant visit to a worrisome task.

In previous years, I would pay visits to parts of the family and there would be lots of food, fun, and laughter. In the past few years I have felt guilty for creating so much fear and worry that I think twice before packing a bag to stay the weekend at someones house. The fear and worry that I speak of is due to my health and its complications, creating this mindset of “I hope nothing goes wrong while he’s here.”   My visiting experiences are filled with “Can Adam eat this?  Has Adam eaten enough?  Is Adam in pain?  Does Adam need to go home early?  Lord I hope nothing happens while Adam is here.” I have had one or two issues with glucose levels during the evening that no longer occur, but each time I stay somewhere, my family is adamant about using a baby monitor and/or sleeping on the sofa next to me just to make sure nothing occurs while I’m asleep.

I am very thankful to have a family love me as much as I do, but pleasant experiences have certainly changed due to Wolfram Syndrome. When I get the feeling that I am a fly in the ointment and not a pleasant visitor, I just grit my teeth and say that it could be worse and accept it and count my blessings. I try to stay optimistic by hoping and praying that something will change for the better in the near future.

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Living With Wolfram Syndrome – Adam Zwan

Health Glitches

Thanksgiving 2014 was a fun filled day of food, family and laughter. The meal felt like a Golden CorralWhat a person with Wolfram syndrome cannot eat buffet without the Golden Corral. Thanksgiving used to be a favorite holiday of mine but due to Wolfram Syndrome it has become my least favorite. The family getting together and enjoying one another is a great event but the food is another story.

Before Wolframs and its complications took a toll, I could enjoy splurging a little and try each dish that was served. However, this year was low carbohydrate and low sugar for diabetes as well as low fat and low fiber for gastroparesis. When the feast was prepared and served buffet style, family members piled up plates of turkey, ham, pork tenderloin, casseroles, dressings, and stuffing. Finally it was my turn to say turkey breast please and……ok, turkey again please. The feast ended with cakes, pies, and puddings. I went against my morals and had a sugar free cheesecake cupcake. The smiling, laughing, moaning, and groaning made the holiday even more memorable. Two hours later did not occur as pleasant for me.

Later in the day, while family members were sprawled out in the den, my stomach decided to empty its contents and spike my sugar. With the little bit of food I ate my metabolism slowed down and held on to everything I consumed. Gastroparesis is a fancy term for delayed stomach emptying, so after my insulin did it’s, work my stomach empties without any insulin to combat the contents. This glucose spike lasted through the night and was finished by Black Friday. On a lighter note, anytime glitches in health occur, I just read successful research blog entries by Dr. Urano to give myself hope for the future.

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Living With Wolfram Syndrome – Adam Zwan

Eye see you…no I don’t! Vision Loss and Wolfram Syndrome

Photo of Adam ZwanOne of the many struggles a Wolfram Syndrome patient will experience, is vision loss. As a young man going through grade school, I noticed my vision quickly getting worse. In a classroom amongst individuals my age, I stood out from the crowd by always sitting in the front row, squinting to see the chalkboard, and having to get close and personal with any reading material. Before being diagnosed with Wolfram Syndrome, doctors were unable to properly treat and diagnosed my visual difficulty.

After grade school, I made it to college where I learned that there are avenues of help; large font textbooks, note takers, and CCTVs for magnifying materials at home. I was grateful for all the help provided by Vocational Rehab and disability resources at school. However, a few months after graduation, my vision continued to deteriorate and resulted in me having to hang up my car keys. I have been told that a driver assist technology is under way and will be marketed in a few years. Let’s hope.

At age 27 I feel a little amputated because I cannot transport myself independently to work, the grocery store, social events, or doctor appointments. Although visual struggles are a part of my life, I know things could be worse. I just tell myself that vision loss is nothing to be ashamed of; it’s just not something worth bragging about on the first date.

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Living With Wolfram Syndrome – Adam Zwan

Chasing the Numbers

Adam Zwan struggles with his glucose levels.Wolfram syndrome makes it more difficult to treat and control all other more common illnesses. A Wolfram patient has to deal with diabetes, kidney failure, optic atrophy, and deafness but all these conditions are further complicated due to Wolfram syndrome. Currently, one of the most troubling tasks in my life is trying my best to control diabetes.

In simplest terms it is called chasing the numbers; these numbers represent glucose readings. Wolfram syndrome has increased my sensitivity to insulin and as a result, causes me to require less insulin to combat food and high glucose readings. On the other side, I have developed gastro paresis, which is a fancy term for delayed stomach emptying. There have been many cases where regardless of what I eat my glucose level is uncontrollable.

With all the complications due to Wolframs, any time I swallow something it is an uphill battle. My increased sensitivity to insulin causes an immediate drop in glucose level after taking insulin to combat carbohydrates. Hours after eating, a jump in glucose level occurs because my stomach has finally emptied the food I ate sometimes 15 hours earlier. These glucose fluctuations may be difficult and frustrating to deal with but I’m just thankful that I can still eat food and not depend on a feeding tube.

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Living With Wolfram Syndrome – Adam Zwan

Tired But Can’t Sleep

Image of sleeping manHave you ever experienced full body exhaustion but cannot fall asleep?  Fellow Wolfram syndrome patients may know exactly what I am talking about.  A snapshot of this sleepless exhaustion is lying in bed with a severe case of yawning while closing the eyes as tight as possible wishing for at least a few minutes of sleep. 

It has been suggested that I participate in a sleep study so that some solutions to restless nights of sleep can be developed.  However, part of my problem is not only the sleep patterns but the health issues I struggle with.  Due to Wolfram syndrome, I have to wake up each night to catheterize relieving discomfort, wake up every two hours to check my glucose, and try to relax a cold body that sometimes deals with shivering.  As always, it is not simply solved with one thing like sleep medication but bigger helping hands like an accurate continuous glucose monitor (CGM).  Something like a Dexcom CGM would relieve my body from having to wake up every two hours and relieve my alarm clock from mandatory overtime.  The only thing allowing me to continue daily routines is a 20 minute power nap every day.  It may be possible to obtain a Dexcom sensor in the future but until that day arrives I will continue going to bed each night eagerly awaiting the 5:00 A.M. alarm signaling that it is gym time.

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Living With Wolfram Syndrome – Adam Zwan

Counting My Blessings

AdamHave you ever seen the movie Jack?  In case you have not, Robin Williams (Jack) is the lead character who has a rare condition causing him to age three times as fast on the exterior.  This is a good comparison when speaking of Wolfram Syndrome because as a 27 year old Wolfram patient I am, quote unquote, about 90 years old internally.  Thus, it is like living the life of Jack but inside out. 

The rapid aging and deterioration of my central nervous system has resulted in many health complications that usually occur later in life.  These health complications I am troubled with are prevalent in the lives of almost all Wolfram patients beginning at birth.  In my own lifetime, I have experienced Diabetes, chronic kidney failure, decreased vision, decreased hearing, loss of taste, loss of smell, and a weakened immune system making me more vulnerable to the everyday free radicals.  My aging central nervous system is probably why I can relate so well to elderly individuals; conversations always contain “yes sir, been there, done that”, and “yes, I have that problem too.” 

Wolfram may cut my life short but it just reminds me to be thankful for what I do have and count my blessings.  Not being bed ridden, still having brain activity and still being able to use my legs and arms makes me realize that Wolfram syndrome may be a tough bullet to chew but things could always be worse.  So long as the Wolfram research and the Snow Foundation are doing well, I choose to keep fighting and hoping for new developments. 

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Experience Life and Enjoy the Now! – Adam Zwan

Statistics are only numbers but for me they are motivation to stay physically active and nutritionally sound.

Photo of Adam ZwanAfter turning 27-years old I thought about the Wolfram Syndrome statistics of 28-31 years of age as the life expectancy. I do not believe it will happen next year but at the same time I will not deny the possibility. All the while, I go to the gym everyday and tell myself that if an early life checkout finds me, I want to be able to say, legitimately, that I never gave up on fitness and nutrition. I did everything in my control to stay healthy.

Wolfram statistics are also motivation for me to live every moment to the fullest. I do not want to regret not taking chances or missing once in a lifetime experiences because numbers show that dying next year is a possibility. Each day I am faced with hearing difficulties, visual difficulties, spontaneous kidney infections, and glucose fluctuations due to metabolic problems. All the while, I’m facing each moment head strong and beating back all problems with Greek yogurt and dumbbells.

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