Cognitive Behavioral Therapy and Exercise can help with Myotonic Dystrophy

There is no treatment for myotonic dystrophy…yet. In the interim period a new study shows that behaorial therapy and exercise can help to stem the huge impact this disease has on patients. Here is the conclusion : 

Interpretation Cognitive behavioural therapy increased the capacity for activity and social participation in patients with myotonic dystrophy type 1 at 10 months. With no curative treatment and few symptomatic treatments, cognitive behavioural therapy could be considered for use in severely fatigued patients with myotonic dystrophy type 1.

However, in reviewing the study this approach requires huge amount of medical resources that may not be available. Hours of analysis of the issues with patients and then tailoring the approach to each patient and tweaking it periodically. Working with a medical professionals for hours is very expensive and most health systems ahve no way to accomplish this.

Cognitive behavioural therapy with optional graded
exercise therapy in patients with severe fatigue with myotonic
dystrophy type 1: a multicentre, single-blind, randomised trial

Kees Okkersen, Cecilia Jimenez-Moreno, Stephan Wenninger, Ferroudja Daidj, Jeffrey Glennon, Sarah Cumming, Roberta Littleford,
Darren G Monckton, Hanns Lochmüller, Michael Catt, Catharina G Faber, Adrian Hapca, Peter T Donnan, Gráinne Gorman, Guillaume Bassez,
Benedikt Schoser, Hans Knoop, Shaun Treweek, Baziel G M van Engelen, for the OPTIMISTIC consortium†

Background Myotonic dystrophy type 1 is the most common form of muscular dystrophy in adults and leads to severe fatigue, substantial physical functional impairment, and restricted social participation. In this study, we aimed to
determine whether cognitive behavioural therapy optionally combined with graded exercise compared with standard care alone improved the health status of patients with myotonic dystrophy type 1.

Methods We did a multicentre, single-blind, randomised trial, at four neuromuscular referral centres with experience in treating patients with myotonic dystrophy type 1 located in Paris (France), Munich (Germany), Nijmegen (Netherlands), and Newcastle (UK). Eligible participants were patients aged 18 years and older with a confirmed
genetic diagnosis of myotonic dystrophy type 1, who were severely fatigued (ie, a score of ≥35 on the checklistindividual strength, subscale fatigue). We randomly assigned participants (1:1) to either cognitive behavioural therapy plus standard care and optional graded exercise or standard care alone. Randomisation was done via a central webbased system, stratified by study site. Cognitive behavioural therapy focused on addressing reduced patient initiative, increasing physical activity, optimising social interaction, regulating sleep–wake patterns, coping with pain, and
addressing beliefs about fatigue and myotonic dystrophy type 1. Cognitive behavioural therapy was delivered over a 10-month period in 10–14 sessions. A graded exercise module could be added to cognitive behavioural therapy in Nijmegen and Newcastle. The primary outcome was the 10-month change from baseline in scores on the DM1-Activ-c scale, a measure of capacity for activity and social participation (score range 0–100). Statistical analysis of the primary outcome included all participants for whom data were available, using mixed-effects linear regression models with
baseline scores as a covariate. Safety data were presented as descriptives

This trial is registered with ClinicalTrials. gov, number NCT02118779.

Findings Between April 2, 2014, and May 29, 2015, we randomly assigned 255 patients to treatment: 128 to cognitive behavioural therapy plus standard care and 127 to standard care alone. 33 (26%) of 128 assigned to cognitive behavioural therapy also received the graded exercise module. Follow-up continued until Oct 17, 2016. The DM1- Activ-c score increased from a mean (SD) of 61·22 (17·35) points at baseline to 63·92 (17·41) at month 10 in the cognitive behavioural therapy group (adjusted mean difference 1·53, 95% CI –0·14 to 3·20), and decreased from 63·00 (17·35) to 60·79 (18·49) in the standard care group (–2·02, –4·02 to –0·01), with a mean difference between groups of 3·27 points (95% CI 0·93 to 5·62, p=0·007). 244 adverse events occurred in 65 (51%) patients in the cognitive behavioural therapy group and 155 in 63 (50%) patients in the standard care alone group, the most common
of which were falls (155 events in 40 [31%] patients in the cognitive behavioural therapy group and 71 in 33 [26%] patients in the standard care alone group). 24 serious adverse events were recorded in 19 (15%) patients in the cognitive
behavioural therapy group and 23 in 15 (12%) patients in the standard care alone group, the most common of which were gastrointestinal and cardiac. Interpretation Cognitive behavioural therapy increased the capacity for activity and social participation in patients with myotonic dystrophy type 1 at 10 months. With no curative treatment and few symptomatic treatments, cognitive behavioural therapy could be considered for use in severely fatigued patients with myotonic dystrophy type 1.

Print Friendly, PDF & Email

The Effect of Myotonic Dystrophy on Daily Life

This post refers to a study done by Cynthia Gagnon one of the preeminent researchers in myotonic dystrophy. In 2007 a study was published and this post is based on that article. Click here for a full copy of the scientific study ==>Life Habits with Myotonic Dystrophy

Continue reading

Print Friendly, PDF & Email

Job Accomodation


According to the Muscular Dystrophy Association, the standard Myotonic Dystrophy is among the most common of Muscular Dystrophies with an incident of 1 in 7000 or 1 in 8000. Today, many individuals are living and working with MD, and employers are seeing more incidents of MD among their employees. This coupled with the requirements of the Americans with Disabilities Act (ADA) show why knowing about workplace accommodations for people with MD is important.

When considering accommodations for people with MD, the accommodation process must be conducted on a case-by-case basis. Symptoms caused by MD vary so when determining effective accommodations, the person’s individual abilities and limitations should be considered and problematic job tasks must be identified. Therefore, the person with MD should be involved in the accommodation process.

Not all people with MD will need accommodations to perform their jobs and many others may need only a few accommodations. For those who need accommodation, the following pages provide basic information about common limitations, symptoms, useful questions to consider, and accommodation possibilities. The following is only a sample of possibilities to consider; numerous other solutions and considerations may exist.

Also included in this publication is a list of resources for additional information.

This publication was written by Beth A. Loy, Ph.D., and Linda C. Batiste, MS, Human Factors Consultants with the Job Accommodation Network. If further information is needed, please call JAN at 1-800-526-7234 (V/TTY).


Muscular Dystrophy

The following information regarding MD was edited from several sources, including many of the resources listed in the resource section of this publication and especially the Muscular Dystrophy Association (MDA). The information is not intended to be medical advice. If medical advice is needed, appropriate medical professionals should be consulted.

What is muscular dystrophy?

MD is the common name for several progressive hereditary diseases that cause muscles to weaken and degenerate. MD is caused by defects in genes for muscle proteins. Most of these proteins appear to play a role in supporting the structure of muscle fibers. The term “muscular dystrophy” refers to a group of genetic diseases marked by progressive weakness and degeneration of the skeletal, or voluntary, muscles, which control movement. The muscles of the heart and other involuntary muscles are also affected in some forms of MD, and a few forms involve other organs as well. There are forty different neuromuscular diseases; the major forms of muscular dystrophy are myotonic, Duchenne, Becker, limb-girdle, facioscapulohumeral, congenital, oculopharyngeal, distal, and Emery-Dreifuss. Some of these names are based on the locations of the affected muscles or the doctors who first described the diseases. For additional information on the major characteristics of the nine MDs, see Facts About Muscular Dystrophy (MD) from the Muscular Dystrophy Association at: <>.

What causes muscular dystrophy?

Flaws in muscle protein genes cause MD, which prevents the body from manufacturing essential substances in adequate amounts to maintain and fuel the muscles. All genes are inherited, half coming from a child’s mother, the other half from the father. Flaws in genes can be inherited along with the parents’ genes, although they can also occur for the first time in a child. Scientists use the term spontaneous mutation when this happens. The different forms of MD can be passed from parent to child by one of three inheritance patterns: 1) autosomal dominant (when only one parent passes on one defective gene), 2) autosomal recessive (when both parents pass on the same or similar gene defects), and 3) X-linked recessive (when gene defects on the X chromosome occur).

What are the symptoms of muscular dystrophy?

Individuals with MD usually exhibit contractures, a condition often associated with shortened muscles around the joints. Due to the abnormal and sometimes painful positioning of the joints, most individuals with MD have extreme fatigue and weakness as well as speech, mobility, and fine motor limitations. In addition, scoliosis, or curvature of the spine, is common.

Who gets muscular dystrophy?

MD is generally inherited but in some cases no family history of the disease may exist. MD can affect people of all ages. While some forms first become apparent in infancy or childhood, others may not appear until middle age or later. Duchenne and Becker are the most common types of MD and they occur in the male sex. X-linked recessive MD usually affect males since males do not have a “backup” copy of a normal gene on a second X chromosome. Females have two X chromosomes, so a normal gene on the female’s other X chromosome serves as a “backup” copy. A woman with an X-linked gene defect usually does not show any signs of an X-linked disease. However, she is a carrier of the disease and has a 50 percent chance of passing it to her sons. Her daughters each have a 50 percent chance of inheriting the gene and being carriers of the disorder.

How is muscular dystrophy treated?

Moderate exercise programs and physical therapy can minimize contractures, and certain exercises may prevent or delay scoliosis. Surgery can sometimes be helpful in relieving muscle shortening. In addition, respiratory care for some individuals with MD may also help.

Medications known as corticosteroids have been found to slow muscle destruction in Duchenne MD. However, these potent anti-inflammatory drugs, used for many conditions besides MD, have serious side effects, such as weight gain, bone loss, cataracts, skin problems, high blood pressure, susceptibility to infection, and psychological difficulties. Researchers are testing new corticosteroids that may have fewer side effects.

Researchers have developed accurate protein and DNA-based carrier detection tests for families affected by Duchenne and Becker MDs. Carrier testing for other types of MD is being developed.

The prognosis of MD varies according to the type of MD and the progression of the disorder. Some cases may be mild and very slowly progressive, with normal lifespan, while other cases may have more marked progression of muscle weakness, functional disability and loss of ambulation. Life expectancy may depend on the degree of progression and late respiratory deficit. In Duchenne MD, death usually occurs in the late teens to early 20s.


What symptoms or limitations is the individual with MD experiencing?

How do these symptoms or limitations affect the person and the person’s job performance?

What specific job tasks are problematic as a result of these symptoms and limitations?

What accommodations are available to reduce or eliminate these problems? Are all possible resources being used to determine possible accommodations?

Has the employee with MD been consulted regarding possible accommodations?

Once accommodations are in place, would it be useful to meet with the person with MD to evaluate the effectiveness of the accommodations and to determine whether additional accommodations are needed?

Do supervisory personnel and employees need training regarding MD, other disability areas, or the Americans with Disabilities Act?


(Note: People with MD will develop some of these limitations/symptoms, but seldom develop all of them. Limitations will vary among individuals. Also note that not all people who have MD will need accommodations to perform their jobs and many others may need only a few accommodations. The following is only a sample of the possibilities available. Numerous other accommodation solutions exist as well.)

Activities of Daily Living:
  • Allow use of a personal attendant at work
  • Allow use of a service animal at work
  • Make sure the facility is accessible
  • Move workstation closer to the restroom
  • Allow longer breaks
  • Refer to appropriate community services
  • Reduce or eliminate physical exertion and workplace stress
  • Schedule periodic rest breaks away from the workstation
  • Allow a flexible work schedule and flexible use of leave time
  • Allow work from home
  • Implement ergonomic workstation design
Gross Motor:
  • Provide a scooter or other mobility aid if walking cannot be reduced
  • Provide parking close to the work-site
  • Provide an accessible entrance
  • Install automatic door openers
  • Provide an accessible route of travel to other work areas used by the employee
  • Make sure materials and equipment are within reach range
  • Move workstation close to other work areas, office equipment, and break rooms
Fine Motor Impairment:
  • Provide alternative computer access
  • Provide alternative telephone access
  • Provide arm supports
  • Provide writing and grip aids
  • Provide a page turner and a book holder
  • Provide a note taker
Medical Treatment Allowances:
  • Provide flexible schedules
  • Allow a self-paced workload with flexible hours
  • Allow employee to work from home
  • Provide part-time work schedules
Speech Impairment:
  • Provide speech amplification, speech enhancement, or other communication device
  • Use written communication, such as email or fax
  • Transfer to a position that does not require a lot of communication
  • Allow periodic rest breaks
  • Develop strategies to deal with work problems before they arise
  • Provide sensitivity training to coworkers
  • Allow telephone calls during work hours to doctors and others for support
  • Provide information on counseling and employee assistance programs


An engineer with MD had difficulty grasping frequently used files. He was accommodated with a desktop carousel.

A student with MD was limited in her use of the computer. She was accommodated with the Magic Wand Keyboard, a miniature computer keyboard and mouse. The keyboard worked with the slightest touch of a wand and no force was needed to activate the keys.

A staff employee with MD who operated a power chair with a joystick was having difficulty opening doors. The individual could not grasp door handles and was accommodated with automatic door openers.

A physician with MD was having problems getting up from a seated position after consulting with patients. The individual was accommodated with a lift cushion for his chair.

A counselor was having difficulty performing psychological evaluations due to cognitive limitations. Her manager agreed to provide written job instructions when possible, and make memory aids such as schedulers or organizers readily available.

A service worker was having difficulty maintaining a full workday due to fatigue. The individual was accommodated with periodic rest breaks away from the workstation, a flexible work schedule, flexible use of leave time, parking close to the work-site, and periodic work from home.

A technical writer with MD was having difficulty reaching her workstation. The individual was accommodated with a flat screen monitor, monitor arm, keyboard tray, footrest, headset, and strategically placed filing racks.

A secretary with MD was restricted from typing information into her computer due to fine motor limitations. She was accommodated with speech recognition.

A manager with MD was having difficulty with daily living needs. The individual was allowed to bring her service animal to work and provided an accessible restroom.

A lawyer with MD was having difficulty climbing stairs. He was accommodated with a stair lift.

An office worker with MD who distributed mail was having difficulty carrying mail to workstations. He was accommodated with a cart.

A writer with MD had severe hand weakness and was limited in her ability to use the keyboard. The individual was accommodated with a miniature keyboard with light touch features.

(This is a non-inclusive list)

Job Accommodation Network (JAN)
A Service of the U.S. DOL Office of Disability Employment Policy
West Virginia University
P.O. Box 6080
Morgantown, WV 26506-6080
800-526-7234 (Voice & TTY) & 800-ADA-WORK (Voice & TTY)

Office of Disability Employment Policy
1331 F Street, NW
Washington DC 20004-1107
202-376-6200/202-376-6205 (TTY)

Center for Disease Control and Prevention (CDC)
1600 Clifton Rd.
Atlanta, GA 3033

The CDC promotes health and quality of life by preventing and controlling disease, injury, and disability.

Facioscapulohumeral Dystrophy (FSHD) Society
3 Westwood Road
Lexington MA 02173-1833

The FSH Society is a nonprofit and tax-exempt U.S. corporation, organized in 1991, that address issues and needs related to facioscapulohumeral muscular Dystrophy (FSHD).

International Myotonic Dystrophy Organization
PO Box 1121
Sunland, CA 91041-1121
(866) 679-7954    (this number is toll free in the USA)
(815) 477-0047

This site is devoted to information about Myotonic Dystrophy (MD) and Congenital Myotonic Dystrophy (CMyD), both forms of muscular dystrophy.  Also contained in this site is information on Congenital Muscular Dystrophy (Thompsen’s disease), which is a separate condition but in some cases having similar symptoms.

Muscular Dystrophy Association (MDA)
3300 E. Sunrise Drive
Tucson, AZ 85718

MDA is the source for news and about neuromuscular diseases, research, and services for adults and children with neuromuscular diseases and their families.

Muscular Dystrophy Association of Canada
2345 Yonge St, Suite 900
Toronto, ON M4P 2E5

MDA of Canada is a national voluntary agency that is committed to funding leading research to find the causes, treatments, preventions, and cures for MD.

The Muscular Dystrophy Family Foundation, Inc.
615 North Alabama Street, Suite 330
Indianapolis, Indiana 46204

The Muscular Dystrophy Family Foundation, Inc. provides services, resources, home medical equipment, and adaptive devices to help people with MD.

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
National Institutes of Health (NIH)
1 AMS Circle
Bethesda, Maryland 20892-3675

NIAMS is one component of NIH that conducts research and disseminates information on diseases of the musculoskeletal system and the skin.

National Organization for Rare Disorders (NORD)
PO Box 8923
New Fairfield, CT 06812-8923

NORD is a federation of voluntary health organizations that is committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and service.

Parent Project for Muscular Dystrophy Research
125 Marymount Court
Middletown, OH 45042
800-714-KIDS (-5437)/513-424-7452

The Parent Project for Muscular Dystrophy Research, Inc. is committed to expedite a treatment and cure for Duchenne and Becker Muscular Dystrophy.

Print Friendly, PDF & Email