Laura Sos was born in June 1969 in Castellón, Spain. At age 4, she was diagnosed as having slow progression degenerative muscular dystrophy (MD). From approximately age 11, she began land-based physiotherapy-assisted active and passive stretching treatments to increase range of motion, and she still maintains this treatment.
Laura functioned independently until the age of 21, at which time, she developed pronounced lumbar lordosis when walking. It was at this time that she began to have difficulty moving and required assistance when transitioning from a seated to a standing position.
Walking distances became shorter, and progressive fatigue kept Laura, at 27, wheelchair bound when she left the house. Her condition continued to worsen so much so that by age 31, she had lost so much strength that she needed to use a walker to move around her house. She learned how to lean on other people, the furniture, or the wall to stand up from a seated position and grab the walker.
Two years later, at 33, she used her walker and began to wear an ankle-foot orthotic to minimize the effect of pes equines. Pes equinius, also known as "clubfoot," is a deformity in which the toes are extremely flexed. Walking is performed on the dorsal surface of the toes, and the heel does not touch the ground.
A year later, at 34, she could no longer get up or sit down by herself. Laura lost strength in her upper body and stopped using the walker, relying more on the wheelchair for mobilization around the house. Pain in her lumbosacral region increased, leading to even greater movement limitations. Her morale began to decline as she became less autonomous and realized that her illness was progressing with more bad days than good. By age 35, Laura's generalized loss of muscular strength and chronic debilitating pain in her lumbosacral region meant that she could not bear to sit for more than 1.5 or 2 hours at a time and spent most of the day lying down.
It was then that Laura began aquatic exercises. During the first few months, we observed that she was a very withdrawn young woman, who was pessimistic. She hardly ever smiled, was suspicious of the challenges we put before her, and was afraid to try any new exercise. At home, she spent a lot of time alone in her room lying down because of back pain. Fortunately, she enjoyed being in the water quite a lot, and gradually, we were able to get her to relax and trust the aquatic program, which we designed especially for her.
WHAT IS MUSCULAR DYSTROPHY?
Muscular dystrophy (MD) is a term used to describe a group of more than 30 inherited disorders that cause progressive muscle weakness and degeneration of skeletal muscles that control movement. Some forms of the disease also may affect cardiac muscle function (6,7).
Congenital MD, the kind Laura lives with, is a rare form of the disease that is present from birth and can affect men or women. The cause of the disease is thought to be a genetic mutation affecting some proteins used by the muscles and sometimes the eyes and brain (4). Symptoms usually progress slowly and include general muscle weakness, flaccid tone, bent joints that may be either stiff or loose, spinal curvature, respiratory insufficiency, and slow motor development (4,7). In some cases, people also may present with mental retardation or learning disabilities, eye defects, or seizures (4). Children with congenital MD can usually learn to walk (assisted or unassisted) and can live into young adulthood or beyond (7).
There are no cures for any of the dystrophies, and few drugs have any effect. Treatments target quality of life by reducing muscle weakness and muscle degeneration, increasing mobility and balance skills, maintaining a healthy body composition and respiratory function, and strengthening the immunity systems. Surgery may be needed to implant a pacemaker, correct severe contractures, and compensate for shoulder weakness. Occupational and physical therapy have been found to be beneficial for maintaining and improving activities of daily living (ADL), yet studies in this area are lacking (4-7).
BENEFITS OF EXERCISE
During ACSM's 2010 Annual Meeting, Dawn A. Lowe, Ph.D., University of Minnesota, chaired a session on exercise considerations for MD. The discussion focused on what was known and unknown about exercise recommendations. A lack of evidence, however, did not dampen the optimism of presenters and attendees to jump in and examine the potential benefits of exercise. More research is being conducted, but rather than waiting, practitioners were encouraged to start working with this population, tracking and sharing outcomes, so specific activity strategies are developed. MD patients are a varied group, where individual symptoms and responses to exercise vary widely. Limitations in study designs may make generalizations to this group difficult. Outcomes based on statistical significance may be difficult to attain and could mask the lessons that are clinically important. Case studies and program evaluation of data may help tease out important clues to treatments.
A review of studies examined programs that measured the safety and efficacy of strength and aerobic exercise training in people with muscle disease. Authors found that in myotonic and facioscapulohumeral MD, moderate-intensity strength training appears to do no harm, but there is insufficient evidence to conclude it offers a benefit. Participants with mitochondrial myopathies, which include a group of neuromuscular diseases caused by damage to the mitochondria (4), safely increased submaximal endurance capacity with a combined aerobic and strength training program (12).
Vignos and Watkins (11) found that 24 patients with a mild level of three different MD increased muscle function after 4 months of graduated maximum resistance strength training that was maintained for 1 year with continued training. The initial resistance was determined by a trial that measured the load each patient could lift 10 times through a full range of motion. Sometimes, weights were used to assist exercises that could not be performed against gravity. During the first 6 months, patients spent about 30 minutes a day performing 10 repetitions of each exercise (hip abduction/adduction, hip and knee extension, forward flexion of the arm, and abdominal sit-ups). During the next 6 months, they exercised 30 minutes a day, 3 to 5 times per week.
Within 2 to 4 months, the average increase in the amount of weight lifted was 11 lb. Authors noted that stronger muscles tended to improve the most. Functional skill outcomes including timed tests such as sit-to-stand and stair climbing were considered significant if there was a 20% change. After 4 months, the results were mixed, with some patients improving, others maintaining, and some declining in function. A total of 7 of 10 patients reported functional improvements at 6 months that were maintained at 12 months. They reported improvements in gait stability, bike riding ability, ability to rise from the floor, and less fatigue. Two women no longer required assistance cleaning the house, and one man reported a consistent improvement in his golf scores and physical endurance while playing. Authors comment that exercise should begin early in the course of the disease to maximize benefits.
LAURA'S PROGRAM OBJECTIVES: GENERAL AND SPECIFIC
Laura's program was designed generally to decrease pain, improve fitness, and to enhance her independence for ADL.
Specific objectives were tailored for Laura and modified as her condition changed to provide an individualized approach. Some specific objectives are to:
- increase spinal stabilization
- strengthen the lower body, including quadriceps, tibialis anterior and peroneus, hamstrings, and gluteals
- improve balance
- progressively increase hip extension range of motion
- strengthen core muscles of the back, abdominals, and trunk
- increase cardiovascular endurance
- improve coordination of arms and legs
- increase flexibility and functional range of motion of the hip and shoulders
- improve functional skills, such as sitting, walking, and transferring from wheelchair to her bed or chair; increase time she can sit comfortably in a chair
- improve mood, decrease depression, and increase motivation to become more socially involved with family, friends, and the community
AN INTEGRATED TEAM
Laura's team includes the authors of this article, a clinical exercise physiologist, who serves as the water program developer, and a physician who monitors Laura's medical condition by tracking physical, psychological, and medical outcomes while providing guidance to the trainer. Additional members include Diane Schofield, our translator and generous program sponsor, and Laura's mother, Ana Torres, who encourages and transports Laura to the pool. By far, the most important member of the team is her daily trainer, Charo Belenguer Benítez. Charo skillfully applies the program methods to meet Laura's physical and psychological needs through excellent teaching and coaching skills that are grounded in positive reinforcement with an abundance of compassion. Charo knows when to refer to other team members for help and is sensitive to the challenges that Laura faces each day. (To understand the critical role of Charo, read Laura's letters, http://links.lww.com/FIT/A5.)
AQUATIC PROGRAM DESCRIPTION
Laura's trainer applied the WaterFit® and Golden Waves®, Functional Water Fitness for Older Adults program methods (9,10). The programs are a water-specific evidence-based system that teaches participants skills so they can tailor each exercise to their individual needs. (For specifics about this water exercise program design, visit http://links.lww.com/FIT/A6) (1). Simple skills allow participants and trainers to balance work and rest by changing how buoyancy and resistance act on the body. Simple basic moves such as walking, jogging, rocking, kicking, jumping, and scissors (cross-country skiing) serve as the foundation for all exercises. Variations in speed, surface area, impact (jumping or suspended by floating), range of motion, planes of movement, and stationary or traveling through water provide a blueprint for exercise and class designs.
This systematic method provides the participant with individualized progressions that quickly allow for variations in exercise intensity and complexity. Trainers can be responsive, moment by moment, as they meet the needs of participants and target general conditioning and specific functional training objectives.
LAURA'S WATER EXERCISE PROGRAM
Webbed gloves were used to increase the surface area of the hands for stabilization and to assist travel by "gripping" the water. The time of each session varied based on Laura's daily condition and progressed as the program continued. Water depth varied between 1.2 and 5 m.
The following are examples of exercises included in Laura's program.
Months 1 to 2
- ○ To achieve adaptation to the water because throughout her life, all of Laura's therapies had been conducted only on land (see sidebar on Range of Motion Land Exercise).
- ○ Walking forward, sideways, and backward in water supported by a buoyancy belt, using a "sitting," and progressing to an assisted standing position. Hands were used to push the water to assist travel. Speed was gradually increased.
Months 3 to 7
- ○ To increase joint mobility in shoulders and hips
- ○ To improve muscular endurance of the shoulders and upper back (paravertebral musculature, trapezius, and latissimus dorsi), together with strengthening the deltoids and pectorals
- ○ To decrease back pain with posture practice
- ○ Pushing and kicking for upper body muscular strength/endurance: Pushing, pulling, and kicking a 1-kg medicine ball back and forth as she stabilized in water with a belt. Upper body exercises targeting biceps, triceps, deltoids and trapezius, pectorals
- ○ Progression: Increase speed, surface area (buoyancy belts and webbed gloves), and travel to create currents for more resistance
- ○ Walking forward for body strengthening and range of motion: Walking forward and backward, kicking and scissors basic moves, working the legs around the body in different planes.
- ○ Progression: Enlarge the move and then increase speed of movement and travel
Months 8 to 10: Laura exercised in a very shallow pool at a depth of 1.20 m.
- ○ To stand independently in the pool, increase hip extension, improve posture and walking coordination
- ○ To increase cardiorespiratory endurance
- ○ Walking forward and backward, assisting with her hands, progressed to no hand assistance (hands behind the back)
- ○ Kicking in shallow and deep water
- ○ Deep-water jogging, bicycling, and scissor exercises in a suspended position; abduction and adduction of the hip, traveling forward, backward, and sideways in suspension.
- ○ Progression: Increase speed and change surface area; change the working positions from extended to suspended (feet not touching the bottom), enlarge the moves, work arms and legs around the body in different planes, and travel.
Months 11 to 18
- ○ To improve autonomy in walking
- ○ To increase her cardiorespiratory endurance
- ○ To improve coordination and balance
- ○ To increase lower and upper body strength and improve posture for back pain reduction
- Exercises conducted in shallow and deep water
- ○ Walking in shallow water assisting with arms to travel in different directions and to change directions
- ○ Muscular endurance: Biceps/triceps push and pull with buoyancy barbell
- ○ Catching and throwing a 1-kg medicine ball
- ○ Jogging while resisting with upper body
- ○ Static and dynamic kicking with 1-kg medicine ball
- ○ Static balance, unsupported
- ○ Deep-water jogging, cycling, scissors, kicking
- ○ Progression: Increase speed, change the working positions, extended to suspended, enlarge the move, work around the body in different planes, and travel
Month 19 to current
- ○ To target previous objectives, increasing intensity of exercises and by increasing time and volume of exercise performed, to increase land-based function. As Laura began to meet her objectives, new and more complex goals were set.
- ○ In shallow water, all exercises were performed in the extended (standing) position using good posture with coordinated arms and legs. All the basic moves were worked through various planes of movement to target multidimensional muscular and cardio endurance.
- ○ Walking, jogging, kicking, scissors (and jax), jumping with arms used to assist with lift forward and sideways assisting with arms
- ○ In the deep end, a buoyancy belt was used for support, and exercises were performed with special attention to good body alignment with an emphasis on hip extension when possible.
- ○ Jogging, scissors, bicycling, kicking
Progression: Variations in surface area (hand or no hands), speed, working positions (rebound, neutral extended and suspended), enlarging the move, working around the body in planes, and traveling were all applied to increase intensity and complexity.
Equipment was added to increase challenge. Standing and running using a tether created challenge to balance and agility. Laura ran against the tether that pulled against her and then used the tether ride in a seated suspended position to train core and posture using arms and her own body to use isometric contractions to resist the power of the current forces.
Equipment also was removed to increase challenge: Exercises were now performed without the aid of a buoyancy belt. Basic moves plus exercises such as the sit to stand were practiced without buoyancy support.
Laura practiced transferring in and out of her pool lift without help at each session.
FOLLOW-UP OBSERVATIONS AND ASSESSMENTS
Follow-up 1: Postexercise, 2-8 Months
Two months after beginning the aquatic exercise program, Laura told us that her back pain had decreased. Four months after beginning the program, she reported continued reduction in back pain and was happy to feel greater strength in her arms and legs. Two months later, she felt motivated to try to walk greater distances at home with the walker. During her pool session, in hip-deep water, which provided little support, she was able to walk 10 steps with only partial assistance by her instructor Charo Belenguer Benitez. About 2 weeks later, her distance increased to 20 steps. It was at this time that Laura said that she felt very happy because she had a lot less pain and felt more strength in her legs. After 8 months, Laura walked 80 steps with limited assistance in hip-deep water.
Follow-up 2: Postexercise, 1 Year
Clinical assessments indicated decreased body fat at circumference measured at the arm, chest, waist, hip, and thigh. When measured as skin fold assessments, she decreased body fat at all sites (triceps, suprailiac, abdominals, and quadriceps). Assessment revealed lower resting heart rate (76 beats per minute [BPM] to 66 BPM) and an increase in joint mobility for both active and passive flexion for both knees, hip, with legs flexed and extended.
After 1 year, Laura was able to move short distances about her house, leaning on the wall or on another person (from her room to the bathroom, from the bathroom to the kitchen, etc.). She gained hours of independence, sitting in her chair in good posture, without pain for 6 to 7 hours before needing to lie down. She continued the same physiotherapy range-of-motion sessions in her home, three times per week, 30 minutes per session.
The most important aspect was that since she began to do the exercises in the water, she has noticed a progressive decrease in pain, even when a week goes by without her doing exercises in the swimming pool; the pain in her back takes longer to recur. Because of these improvements, Laura's character has changed. She's much more pleasant to be with and has become an extrovert, smiling more often. She has a greater interest in meeting new challenges with positive energy.
Laura continued the exercise program for years with greater increase in her functional abilities and general health, with a decrease in pain.
Follow-up 3: Postexercise 5 Years Interrupted. No Water Exercise Participation during the Last 6 Months of This Period
Laura's situation changed, and she had to discontinue her water program caused by a number of factors not related to her health status. For approximately 6 months, she performed only her land-based stretching exercise with the physiotherapist, 3 days per week. After 5 years, we took follow-up measurements, 6 months after water training ended and compared her results with the baseline and/or 1-year postexercise training outcomes.
Follow-up assessments in body fat measured as circumference revealed an increase in all areas, chest, waist, hips, thigh, and calf. The follow-up scores were still lower than preexercise baseline measures. Body composition measured as skin fold assessments at the suprailiac, abdominals, and thigh all showed increases at the sites.
Follow-up assessments indicated higher resting heart rate, 15 to 20 BPM (86 BPM to 91 BPM), but it was her joint mobility and strength that were the most affected. She decreased joint mobility in all four limbs. Decreased mobility was noted in her arms and knees (although still better than baseline, pretraining scores). She decreased mobility in her hips (hip flexion), measuring worse than pretraining baseline. This change is thought to be caused by one or all of the following: prolonged sitting, lack of muscle activation from the water exercise program, a degenerative effect of the iliopsoas and muscles of the pelvic girdle.
She has maintained a reduction in pain, which allows her to remain seated comfortably in her chair the same number of hours that she could after 1 year of training. Perhaps the water program provided a protective long-term effect and positively affected the neurological pathways that transmit pain.
At the time she discontinued her water program, Laura was able to support herself without assistance during walking in shallow water with no loss in stability. About 6 months later, Laura's mother noticed that Laura seemed to be weaker, moving more slowly, with slower reaction to movements that required muscle reflex responses.
Her mood and social life have remained positive, although she has not been in the pool for 6 months. She has the same optimism she had while she attended the water program for 5.5 years. Despite having lost some physical gains made previously, her mood is much more positive than when she began the program.
SUMMARY AND UPDATE
The water program seems to have a long-term protective effect. Even after 6 months away from the program, which could occur as a result of surgery, medical instability, or an acute condition, the decline in training was noted for some physical abilities, but her psychological and social skills remained positive. This could result in a greater encouragement to return to physical activity leading to a faster recovery. Charo and Laura have returned to the pool. After working together for only 2 weeks, Laura was able to stabilize her body and move skillfully through the ranges of motion at the level she was performing when she stopped the water program. However, her strength and muscular and cardiorespiratory endurance are lower than when she was participating in the program. At no time, not even during the 6 months when she didn't attend the water sessions, has her pain increased to baseline levels. This is an especially important outcome that should encourage other MD participants to integrate water exercise into a lifetime of physical activity.
It is our hope that this story helps guide others to try water exercise as an activity that improves the quality of life for MD patients and helps provide some clues about safe and effective exercise choices.
Permission was granted to print Laura and Ana's full names. We would like to thank all those who contributed to this program, especially Laura, who has inspired us all to believe more fully in the healing power of water and the strength of a human heart to try his or her best.
All photos courtesy of Wellness Samaruc, Castellón Spain, http://infoplus.qdq.com/wellnesssamaruc/es/.
2. Masumoto K, Shono T, Takasugi S, Hotta N, Fujishima K, Iwamoto Y. Muscle activity and heart rate response during backward walking in water and on dry land. Eur J Appl Physiol
3. Masumoto K, Takasugi S, Hotta N, Fujishima K, Iwamoto Y. Electromyographic analysis of walking in water in healthy humans. J Physiol Anthropol Appl Human Sci
4. Muscular Dystrophy Association Web site [Internet]. Muscular Dystrophy Association [cited 2010 July 30]. Available from: http://www.mdausa.org
5. National Center on Physical Activity and Disability. Muscular dystrophy [cited 2010 July 30]. Available from: www.ncpad.org
8. Sanders ME. Golden Waves, Functional Water Exercise Leadership Program
, Distance Education Course. Available from: www.dswfitness.com
, updated 2005.
9. Sanders ME, editor and co-author. YMCA Water Fitness for Health
. Champaign: Human Kinetics; 2000.
10. Sanders ME, Maloney-Hills C. Aquatic exercise for better living on land. ACSM Health Fitness J
11. Vignos PJ, Watkins MP. The effect of exercise in muscular dystrophy. JAMA
12. Voet NB, van der Kooi EL, Riphagen II, Lindeman E, van Engelen BG, Geurts ACH. Strength training and aerobic exercise training for muscle disease. Cochrane Database Syst Rev. 2010;Jan 20(1):CD003907.
Supplemental Digital Content
© 2010 American College of Sports Medicine.