New Book about a Family with Myotonic Dystrophy

There are not many books about myotonic dystrophy. There is a fictional series about a skater that has myotonic dystrophy. I wrote a short book about the hopes and aspirations of my son “The boy who was President”. Now comes a great biography about a family with Myotonic Dystrophy. A must read for all with the disease. Here’s a short introduction:

As a young girl, my constant goal was to help my brother, Dustin, walk. Dustin’s limits were hard to gauge because he constantly surpassed expectations. He was born with congenital myotonic dystrophy and expected to die, then to live three months, then three years. Instead, he gained strength and capabilities until age 13, when he had a simple cold and just did not wake up from his nap. His body became too much for the largest muscle in his body, his heart.

While Dustin was alive, I threw quarters in wells, prayed every night, and practiced with him every day after he had surgery and got corrective braces. I would stretch my brother’s legs, rotate his ankles, do resistance exercises and help him practice standing. At age 12, I thought willpower was so strong that, through perseverance and dedication, I could will my brother to walk.

Three years older than my brother, I grew up doing adult caretaking tasks. Through the years, I would change thousands of diapers, brush Dustin’s teeth, lift him into bed, administer nebulizer treatments, clean his feeding tube, watch him when both my parents had to work, bathe him, unload his wheelchair from the bus and play with him. Most things I did for my brother were helpful, but with my conceptions about willpower and Dustin walking, I pushed my brother past his comfort level more than once and caused more pain than progress. For me, a healthy sibling, willpower was a tool to push past obstacles. However, the same view I took of my young healthy body proved detrimental to my brother’s and caused him pain.

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Causes of Death Myotonic Dystrophy – Survival to age 65 lower

This is an older study from 1998 but many folks ask about the longevity with this disease. How long will I live with myotonic dystrophy?

Age and causes of death in adult-onset myotonic dystrophy.


(1998) 121 (8): 1557-1563. doi: 10.1093/brain/121.8.1557

  1. C E de Die-Smulders,
  2. C J Höweler,
  3. C Thijs,
  4. J F Mirandolle,
  5. H B Anten,
  6. H J Smeets,
  7. K E Chandler and
  8. J P Geraedts

+ Author Affiliations

  1. Department of Clinical Genetics, Academic Hospital Maastricht, The Netherlands.


Myotonic dystrophy is a relatively common type of muscular dystrophy, associated with a variety of systemic complications. Long term follow-up is difficult because of the slow progression. The objective of this study was to determine survival, age at death and causes of death in patients with the adult-onset type of myotonic dystrophy. A register of myotonic dystrophy patients was set up in Southern Limburg (the Netherlands), using data longitudinally collected over a 47-year period (1950-97). Survival for 180 patients (from the register) with adult-onset type myotonic dystrophy was established by the Kaplan-Meier method. The median survival was 60 years for males and 59 years for females. Survival of the patients was also estimated from the age of 15 years to the ages of 25, 45 and 65 years and compared with the expected survival of age- and sex-matched birth cohorts from the normal Dutch population. The observed survival to the ages of 25, 45 and 65 years was 99%, 88% and 18% compared with an expected survival of 99%, 95% and 78%, respectively. Thus, survival to the age of 65 in patients with adult-onset myotonic dystrophy is markedly reduced. A weak positive correlation between the CTG repeat length and younger age at death was found in the 13 patients studied (r = 0.50, P = 0.08). The cause of death could be determined in 70 of the 83 deceased patients. Pneumonia and cardiac arrhythmias were the most frequent primary causes of death, each occurring in approximately 30%, which was far more than expected for the general Dutch population. In addition, we assessed mobility in the years before death in a subgroup of 18 patients, as a reflection of the long-term physical handicap in myotonic dystrophy patients. Half of the patients studied were either partially or totally wheelchair-bound shortly before their death.

Case Reports of Pregnancy and Myotonic Dystrophy

With the correct diagnosis myotonic dystrophy can be prevented for the next generation. By using pre-implant diagnosis the next generation can be assured not to have myotonic dystrophy. This is an expensive and not practical approach in all countries though.  This blog contains  case reports from Pakistan of two case studies.

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Celebrating the Life of Sheila Hollern

I was just informed of the passing of Sheila Hollern who is a relative of Jeremy and Erica Kelly, both instrumental in the Myotonic Dystrophy Foundation. Its so sad to see these deaths when we fervently hope that a cure is around the corner. Our thoughts and wishes are with the family members and we will continue the fight to find a cure for this disease that slowly and quickly takes loved ones away

Sheila Brooks Hollern

Last modified: January 16. 2013 12:11PM PST

Sheila Brooks Hollern passed away peacefully in San Francisco on January 15 from cancer and post-surgical complications from Myotonic Dystrophy.  She was 48.

Sheila was born in Palo Alto, California and grew up in Bend.

Sheila spent most of her life in Marin County, California where she volunteered her time at the Cedars Development Foundation and Marin General Hospital.

Sheila spent many happy hours surrounded by friends who entertained and were entertained by her and had a loyal following of family, friends, relatives and dear girlfriends who shared her life in San Anselmo.

Sheila took great pride in decorating her apartment, where every inch of her walls were covered with photos and memories of a happy life.  She enjoyed traveling with her family and her boyfriend of 25 years, Brad McKinsey. Sheila was devoted to her family, her many friends and especially her beloved cat, Midnight.

Sheila is survived by her parents, Mike and Sue Hollern; her brother, Chris Hollern; her sisters Erica Kelly (Jeremy Kelly) and Leslie Lynch (Gene Lynch) and six adoring nieces and nephews.

A private, family remembrance is planned.

In lieu of flowers, donations would be gratefully accepted by Lifehouse, a Bay Area organization dedicated to helping people with developmental disabilities. Lifehouse was instrumental in making Sheila’s life happy and productive. Their address is 899 Northgate Drive, Suite 500, San Rafael, CA 94903 or donations can be made online at

Donate Tissue and Body to Science Myotonic Dystrophy

Many people have asked if they can help with myotonic dystrophy research. I recently became aware of a program where at death you can donate your body to science and they will use tissue samples etc. to further research into myotonic dystrophy. The researchers are always in need of tissue samples and some of these can only be obtained after death.

Although the death of a loved one is devastating the gift of life in research can live on and others can benefit from the donation that is made. Think about this and see if it fits your needs and desires. IF desired fill out the pages and leave with your will.

The Paul and Sheila Wellstone Muscular Dystrophy center in Minneaplois, MN is coordinating this. The hot lines to call are (612)626-0822 and (612) 899-5964

Please click here for the forms that are needed.