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 someone’s 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 ate 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 when using a baby monitor and or sleeping on the sofa next to me just to make sure nothing occurs while sleeping.

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 so 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.

Living With Wolfram Syndrome – Adam Zwan

In January 2013, Adam Zwan was featured by the NBC affiliate in his hometown of Wilmington, North Carolina, WCET News. The story was to show what it is like for Adam to live  with Wolfram syndrome. That night, Adam began to experience severe abdominal pain and was rushed to the hospital where he remained for a week. Despite the delay from Adam’s hospital stay, the news team came back to finish their story.  Here is the five minute video on our good friend, Adam Zwan, where you can see for yourself the courage and spirit Adam exudes every day.

http://youtu.be/ElJXKyf1gf4

 

Living With Wolfram Syndrome – Adam Zwan

How to slow Wolfram Syndrome progression?

Steps toward a cure for diabetes and Wolfram Syndrome are being made and successful results are occurring in scientific research.  The treatments are not yet available for Wolfram patients so it’s wise to attempt slowing Wolfram progression until treatments are available.  In addition to being a Wolfram patient I am also a huge advocate for correcting one’s health on one’s own without the use of prescriptions.

From the age of 7 years old to 16 years old I was placed on nine different prescription drugs to treat my various ailments, ranging from diabetes to kidney failure to elevated cholesterol.  Upon my own research, I discovered that each prescription drug will eventually require an additional prescription for resulting side effects. I took it to heart and disciplined myself to edit lifestyle and correct health issues without the use of medications; today I am down to three prescriptions and doing very well.

Diabetics without Wolfram can experience nerve damage simply due to uncontrolled glucose levels.  Thus, a Wolfram patient, whose central nervous system is genetically experiencing nerve damage, along with uncontrolled diabetes will be faced with hurried progression of kidney failure, vision loss, and hearing damage.  Therefore, I have spent a great deal of my life in becoming physically active and nutritionally sound so that I may more closely take control of my diabetes, my health, and my life.  Through a daily exercise routine and a low sugar/carbohydrate diet I have successfully kept my blood glucose levels, cholesterol, blood pressure, kidney function, vision, and hearing at a stable and satisfactory quality.

In my visits to Washington University of St. Louis during the annual Wolfram research event doctors have told me that my lifestyle choices have proven to slow the progression of nerve damage caused by diabetes and Wolfram Syndrome.  In short, I was told to keep doing what I have been doing.

Great Changes Are Coming

I have always had great intuition (good or bad), ask my friends and family! The Snow Foundation has hired a new President and CEO, Dr. Saad Naseer, who is driven and ready to make our dreams become reality. The foundation is finally receiving the full time attention and direction it deserves, which means Wolfram syndrome patients are going to get the help and their prayers answered.   I am excited to spread the word and I mean really spread the word and I am more excited that Snow Foundation has the leadership to make it happen. My prayers have been answered and I am grateful. This year is going to be revolutionary, great changes are coming.

 

A Mom on a Mission!

prescription drug imageA quick description of my job; work 24/7, deal with scientists, researchers, doctors, philanthropists, sports figures, political figures, full time advocate for rare disease, counselor for parents and patients, breakfast, lunch, dinner meetings, travel around the world, public speaker, accountant, secretary, marketing and most important Fundraiser.

​​Combine all the above and they do not equal what it is like to fundraise. For anyone who has ever had to go out and ask for money, it is not fun. I hated it at first but now to be honest, I ​am learning to like it​. Because each morning that I wake up and see my beautiful 10 year old daughter Raquel slip farther and farther away from her independence, it ​makes me very mad​. Mad, because ​we are sitting around waiting. ​Our research is on hold, our clinical trials are on hold, drug therapies are on hold. The only thing holding us back is MONEY​. We need a minimum of $4M to do the necessary research on the eyes, ears, brain and bladder and begin clinical trials on drugs that could possibly stop the progression of this disease and may even restore vision.​ As per experts, as we get into trials we may need to adjust this amount, but we cannot wait till we have perfect knowledge. We need to act NOW to save our children. MONEY does make ​the world go round, without it, ​these patients​ do​n’t have a chance​.

​Please visit our ​crowdfunding campaign at Snowfunds.org. We are asking you to contribute and spread the word and support our humble cause. Thank you.

Living With Wolfram Syndrome – Lauren Gibilisco

It’s been 10 years since I graduated from High School. We had our 10th year reunion this last weekend. I had to find someone who could help lead me around. I love my mom but going to a reunion with your mom is just not cool, so I asked my friend Meredith to take me.

The first festivity was a float in a parade. The class reunions are always during our town’s celebration weekend. As much as I wanted to sit on the float with my classmates, I knew it would be impossible. The weather was 85 degrees which is way too hot for me to be outside long and then there was the problem of needing a restroom. We had to line up on our float an hour before the parade and then the parade was an hour long. I can’t go that long without needing the restroom. It really disappointed me but I’ve learned there are things I just can’t partake in.

The next festivity was out at a cabin.   I talked with my mom and decided it would just be too hard for me to walk across an uneven surface, be outside in the heat, and again needing the restroom. So we decided to just skip that also. Then my friend Meredith said she was only going to stay out there for an hour and would help guide me around. I was very excited about that. I really did want to go but didn’t want to be a burden to anyone. But Meredith insisted that I wouldn’t be a burden and she would be glad to take me there. I was really happy I went. I got to see a lot of my classmates. Well I couldn’t SEE them but I was able to talk with them and say hello. Meredith took me around and let me know who was there so I could say hello to everyone.

Then that evening there was a dinner banquet. Meredith again took me there and helped me to the table. Unfortunately not many of my classmates came. So I was really glad I was able to go to the cabin because I saw many more classmates then I would have been able to see at the banquet.

After the banquet everyone was heading to the bar to celebrate. There was a live band playing and it was less than ½ block to my house. Unfortunately by this time I was getting very tired. That darn fatigue was getting in the way of my continuing the celebration. I told everyone that I would be the designated driver to drive them home but no one took me up on that one. I wonder why? But overall it was a great experience and I’m glad I went and I’m glad that my friend Meredith took the time to help me enjoy the celebration. I am very grateful to her.

Image of Lauren Gibilisco's class reunion.

Living With Wolfram Syndrome

Maybe or Maybe Not

image of a golf cartOriginally, I was told that Vocational Rehabilitation could provide financial assistance towards a street legal golf cart. When I first heard these words I was exhilarated to think that independent transportation is a possibility. I am still not full time at the wellness and rehabilitation center I work for so financial assistance is needed to acquire a street legal golf cart. It was explained that I must obtain a quote for a golf cart, add it to my file, Vocational Rehabilitation would purchase the golf cart, and then Vocational Rehabilitation would receive a tax rebate. It was all planned out and I was feeling optimistic that a Wolfram Patient, like me, could increase level of independence. Although, I try my best to be cautiously optimistic by saying that it may happen or it may not.

The next stop I was making was at the Vocational Rehabilitation office to add the golf cart quote to my existing file. While in the office a close review of the vehicle purchase policy took place and it was discovered that only 15% of the total vehicle value (down payment) could be offered. This information seemed logical but was and still is a slight stretch for me financially. Secondly, it was discovered that a street legal golf cart requires registration, tags, auto insurance, and a valid license. Obviously, the golf cart scenario has put on hold. It is times like these when I tell myself to “count my blessing, cope with the hand I was dealt, and keep pressing on.”

On a lighter note, my next eye appointment is on July 10th and it will provide me with details as to where my vision stands. I know that rapid aging and detonation of my central nervous system (Wolfram Syndrome) is a tough hand to me dealt in life but I do my best to stay optimistic and keep a realistic outlook in life. It’s one of my favorite clichés: “It may happen and it may not.”

Living With Wolfram Syndrome – Adam Zwan

New Beginnings

a group of children in the sunset with a sign that says HOPE above them.Wolfram Syndrome is a rapid aging and deterioration of the central nervous system. The damages consist of diabetes, chronic kidney failure, deafness, and blindness; it’s not anything to be ashamed of, it’s just not something worth bragging about on the first date.

Throughout life I have experienced my fair share of health issues, some resulting in hospitalization, some ending with a seizure, and others finalized with constipation. I now look at all my past complications and can comfortably say it is water under the bridge because there is hope in the future for improved living. Things started improving when I first decided to move out on my own. Next is the golf cart I will acquire through vocational rehabilitation that will provide independent transportation to and from work. The next thing I hope will happen is a clinical trial for Wolfram syndrome. I understand that there are multiple hurdles before the trial starts. If trials are successful (first in the U.S. since 1938) then my mind will be put at ease from thinking that health struggles will continue getting worse. Thus, I will be forever grateful to The Snow Foundation and Dr. Urano for making such a life-altering path.Road sign that says the future is now.

All in all, I have proven to myself as well as people I come in contact with that patience is a virtue and perseverance can pay back in full. I will maintain hope and keep on being patient until the trial starts. It is amazing to imagine what the future may hold and that I may now start setting long term goals to be accomplished decades from now.

 

High sugar levels in the brain and neurodegeneration

My top priorities are to: 1) repurpose a FDA-approved drug and 2) develop novel drugs for Wolfram syndrome. I am also looking into environmental factors, diets, and life-styles that can potentially delay the progression of Wolfram syndrome. One of the possibilities I am looking into is to keep our blood sugar steady (i.e., maintain normoglycemia).

I have an impression that patients who maintain normoglycemia may have milder symptoms and delayed progression. I was reading an article introduced in the Wall Street Journal very carefully. This study was led by David Holzman, MD, a renowned Alzheimer’s disease scientist. His team’s study strongly suggests that high sugar levels in the brain accelerate amyloid deposition in the brain, which increases the risk for Alzheimer’s disease. Based on their study, neuronal functions and activities are altered by high sugar levels. We should look into this in our animal models of Wolfram syndrome.

Dr. Holzman’s study might explain the link between Type 2 diabetes and Alzheimer’s disease. Please note that Wolfram syndrome 1 gene variations are associated with the risk of Type 2 diabetes, the most prevalent form of diabetes.

Thank you for reading this blog. I am thinking of one of our patients and sincerely hope that her condition gets better. We really need a treatment for this devastating disorder.

Take care,

Fumi Urano

Various kinds of sugar in wooden bowls

Various kinds of sugar in wooden bowls

Living With Wolfram Syndrome – Adam Zwan

Independence

wolfram syndrome independenceI recently moved out of my parents’ home to live on my own in an apartment all in an effort to gain some independence and self-sufficiency. It has been a start to the next chapter in my life with both pros and cons. It feels good to depend on myself to fulfill everyday tasks but it will take some time for my family to relax and feel confident that I can fend for myself.

The whole scenario was made possible after acquiring the Dexcom continuous glucose monitoring system with Share. This device accurately monitors my glucose levels and through Bluetooth communicates with five chosen followers who can also see what my glucose levels are. For the first time in my life other people can see part of what my body is experiencing. As some may have guessed, when my glucose level is below 150 mg/dL all my following family members believe that my glucose is plummeting and I need assistance. The drama has settled somewhat and they now see that an assistant is not needed to feed me orange juice and peanut butter but I am fully capable of serving myself.

Next in line is the cell phone chaos. When my glucose falls a little bit I receive phone calls from three or four of my followers, regardless of the time of day. If I do not have my phone by my side, I am in the shower, using the restroom, or in the gym and someone is trying to call me but I am unable to talk it is all perceived that I am in trouble. Thus, a family member is dispatched to my apartment or 911 is called and then canceled when I become available. I know I should just be thankful that I have a family who cares for me as much as I do but it make everyday living a bit difficult. Although, the yo-yo effect my glucose readings go through is mostly my fault. It seems the harder I try to control my glucose levels the harder it becomes. The exercise I participate in six times a week, my Gastro Paresis, and my limited diet all play a part in my frequent glucose fluctuations.

Everyone has a story and everyone has something he or she struggles with, my struggle in life is health (Wolfram Syndrome). I remind myself everyday that challenges in life are a given but coping and pushing forward are choices. My prayers rest with the Snow Foundation to fund research and Dr. Urano in finding a treatment for Wolfram Syndrome.