Role of Physiotherapy in Muscular Dystrophy

Role of Physiotherapy in Muscular Dystrophy

Muscular dystrophy is a disorder of the musculoskeletal system that is characterized by progressive weakening of various muscle groups. This disorder has many genetic presentations giving rise to its different types and forms. In the present times, this disorder is amongst the most challenging hurdles that human science has ever come across.

The role of medication and corrective surgeries is very well known as part of management of muscular dystrophy. Role of physiotherapy, however essential, is very rarely studied and acknowledged. Physiotherapy is a therapeutic science that makes use of manual skills and thermal modalities to correct the deviations from normal health process. Muscular dystrophy being a life disabling condition, that makes a person dependent upon a wheelchair in later stages of the disease, demands maintenance of the bodily functions and reconditioning of affected tissues. This can be primarily brought about by physiotherapy which has no side effects.

An early referral from the hospital for physiotherapy is imperative to prevent unnecessary degradation of the patients´ functioning. This disorder is disabling but consistent efforts on part of the patient and his/her physiotherapist, which is generally ignored, can help make this world a better place to live for the patients having MD.

Causes and Types of Muscular Dystrophy

Muscular dystrophy is not an acquired disorder but primarily a defect of inheritance. Mutations in the genetic structure lead to disruption in the sarcolemmal structure of the muscle fibre. A protein complex, dystrophin-glycoprotein, is responsible for binding together all the muscle fibres in a muscle group. Genetic mutations as a result of inheritance or defective development during embryonic stage disrupts these complexes and henceforth make the musculoskeletal system of human body susceptible to dystrophic/atrophic changes.

Muscular dystrophy is primarily an X-linked disorder which may be either in autosomal recessive or dominant form. Thus the defective gene is carried by a female carrier. Each type of muscular dystrophy internally has many phenotypic variations giving rise to subtypes. For example, calpinopathy is presented in subtype of Limb Girdle Muscular Dystrophy that goes by the name LGMD Type II.

The different types of muscular dystrophies known to date are as follows:

  1. Duchenne Muscular Dystrophy (Onset: 2-5 years)
  2. Becker´s Muscular Dystrophy (Onset: 8-25 years)
  3. Facio-scapulo-humeral Dystrophy (Onset: symptoms develop in early childhood but noticeable in teens)
  4. Limb Girdle Muscular Dystrophy (Onset: 10-30 years)
  5. Myotonic Muscular Dystrophy (Onset: TYPE 1 Steinert Disease: Congenital; TYPE 2 Proximal Myotonic Myopathy: early childhood)
  6. Congenital Muscular Dystrophy (Onset: since birth)
  7. Emery-Dreifus Muscular Dystrophy (Onset: 5-13 years)
  8. Distal Muscular Dystrophy (Onset: late 40´s or 50´s)
  9. Occulopharyngeal Muscular Dystrophy (Onset: Middle age)

Often the three types of Spinal Muscular Atrophy are also studied parallel to the above mentioned types of muscular dystrophy.

Course of the disease, Risk Factors and Symptoms

MD has a gradual onset and a progressive course. With medications and Physiotherapy, the course of the disease can be modified and delayed but not stopped.

The presence of an ancestral history of MD makes the progenies of that pedigree more susceptible to get affected. Environmental variations of the sorts that might cause genetic mutations in the foetus are a risk factor too. Males are prone to be affected by most forms of MD.

Besides the specific symptoms of each type, in general, there is muscular weakness of various muscle groups in the patient. A few sites like the hips and the calves seem to develop greater muscle bulk when actually that is purely fatty tissue deposition. This is called pseudohypertrophy. Clinically, the Gower´s sign is indicative of Duchenne´s MD whereby when a child is asked to stand up from a crouched-on-floor position, he uses his hands in a manner as if trying to climb over oneself in an attempt to stand erect.

Role of Physiotherapy in Muscular Dystrophy
GOWER´S SIGN

Defects of vision and difficulty in swallowing are key representation of the occulopharyngeal type of MD. Involvement of face muscles is primarily seen in facio-scapulo-humeral type of MD. Sensory changes are rarely seen. The involvement of heart and respiratory muscles are the cause of fatality in few of the types of MD.

Lifestyle of a Patient with Muscular Dystrophy

A patient with muscular dystrophy faces many challenges of life. Besides the physical limitations that render the patient dependent upon his/her family members for the basic functional requirements, there are many psycho-social aspects to this disorder as well. Gradually intricate and in the long run even the gross motor functions are lost. Patient becomes dependent for most of the activities of daily living. It is in such situations that a feeling of invalidity develops in the minds and hearts of these patients. Depending on age groups different psychosocial behaviour is seen amongst the patients. The lack of play amongst toddlers, lack of companionship in young adults and family insecurity in all of them are the key features of their psychosocial status. For the many challenges they face, the patients deserve support not sympathy, we need to empathize and re-integrate them in the normal social functioning for their well-being.

Role of Physiotherapy in Muscular Dystrophy

The main goal of physiotherapy in patients with MD is to maintain the available function in their limbs and attain maximum possible improvement in the associated disabilities. This would help the patient attain a socially functional status so that he/she can function in the surroundings, of their own accord, bringing about a sense of independence in the patients.

The therapeutic practices involved and their importance in the life of an MD patient are as follows:

  • Electrotherapy: A patient with MD often complains of musculoskeletal pains at various sites. Electrotherapeutic modalities like paraffin wax bath over fractured limb, TENS, IFT, Ultrasonic therapy for tender points and Contrast Bath can be done to relieve such pains.

  • Passive movement: Passive movements are a technique that involves movement of limbs by the physiotherapist, in all ranges, in a manner so as to maintain joint and muscle integrity. Long term immobilization in patients due to weakness of the muscles can render the muscle fibrotic and the joint may become stiff. To avoid the development of these co morbidities one need to maintain the integrity of structures by passive movements.

  • Active assisted movements: The MD patient has limited muscle power. Therefore, with maximum effort, they can attain only a limited range by active contraction of their muscle. To maintain this power, maximum amount activity upto the fatigue threshold should be encouraged in these patients. While the patient maintains muscle force, the physiotherapist should assist the completion of this movement in the normal biomechanical pattern. This will maintain the joint proprioception i.e. the sense of joint position in space.

  • Stretching manoeuvres: Often again due to immobility and poor power of muscles, the muscles shorten in length. The joints adapt a gravity assisted position and internal muscular forces cannot work against the external gravitational force. As a result of this joint contractures develop. To open up these joints and retain the normal muscle length, stretching is done on the joints. A sustained long duration stretch with crepe bandages or taping can be given initially and this can be toned down a bit in later stages to short duration stretches with greater repetitions given manually.

  • Joint mobilization: Due to imbalance of muscular forces, the joints often get displaced from their normal anatomical position. To guide them back so that proper weight bearing can be done on the joints without causing any harm to the associated structures, the physiotherapist passively mobilizes the bones of each joint to bring them in place.

  • Balance and Gait training: Muscular imbalances are so profound in MD that sitting and standing balance are greatly affected in the patients. So with gradual progression from kneeling to quadruped to high sitting to standing position, balance training should be given. As the balance improves the patient will be able to function better by himself/herself. With gradual degradation of power in lower extremities, the locomotion or gait is affected. So gait training involving proper training in parallel bars progressing from supported to unsupported walking should be done under the supervision of the physiotherapist.

  • Hydrotherapy: Hydrotherapy or aquatic exercises are an innate part of physiotherapy rehabilitation protocol for MD. Activities are performed in water at a warmer temperature than body. This helps in the following manner:

    • Buoyancy of water protects and braces the weak joints.

    • In water a person can feel very little of his/her own weight so this makes activities of partial weight bearing possible.

    • Adding floatation devices can assist the movements while adding high pressure water jet can help in performing mild resistance training in better muscle groups.

    • Warmer water helps in maintaining good thermo-stasis in the body and keeps the active muscle warm and hence at ease.

  • Cardiac Pacing and Breathing Exercises: As the muscles of heart and respiratory system weaken, greater chest and cardiac congestion is seen in the patients with MD. To avoid the deleterious effects of an insufficient cardiopulmonary system, one needs to keep in mind a few points.

    • Clear airway should be maintained by passive chest manipulations given by the physiotherapist.

    • The patient should be taught huffing and coughing to aid him in spitting out the chest secretions.

    • Deep breathing exercises should be taught to condition the general cardiorespiratory performance and endurance.

Each therapy session lasts for as much as 45 to 60 minutes.

Besides the therapy sessions, it is the duty of the physiotherapist to counsel the patient about what his/her condition is and how he/she may expect to change in the course of the disease. The parents/guardians/caretaker should be enlightened about the progressive and degrading condition of the disease and the fatality of the condition if applicable. They should be explained the necessity of the physiotherapy regime that needs to be followed back at home as well after one or two sessions under the physiotherapist´s supervision.

A prime part of counselling involves Genetic Counselling. As MD is an inheritable disorder, patients with MD who are in their youth or adulthood should be explained the risks involved in having their biological progeny. This can increase the risk of developing a similar problem in the child as well. Only by chance can the presentation of this condition skip a few generations but the innate problem of the affected gene remains. Thus if not now, the condition can easily present in one or the other following generations. To avoid this, even if one generation is of adopted children, the risk of affections in the future progenies is greatly reduced.

The physiotherapist´s duty also includes the prescription of orthoses and wheelchairs in conjunction with other rehabilitation professionals.

Role of a Rehabilitation Team

  • Occupational Therapist: An occupational therapist has a very vast role to play. The OT is responsible for training the affected individual in a manner that he/she can attain a good level of independence in the Activities of Daily Living. Besides this, modified workplace and home designs as per the patients´ ergonomic requirements and designing of assistive devices like long handled spoons, scooting chairs etc. are done by the OT.

  • Nutritionist/Dietician: MD patients need to take a balanced diet in a manner that they do not put on weight due to lack of physical activity and that they get adequate proteins to make up for the deficiency of the same in their diet. A nutritionist is responsible for setting up diet plans for the patients.

  • Speech Therapist: In muscular dystrophies like Occulopharyngeal and Facio-scapulo-humeral dystrophy, speech articulation can be affected. Thus under the supervision of a trained speech therapist, the patient can be taught to recruit accessory muscles for speech articulation.

  • Prosthesis and Orthotics Engineer: These rehabilitation professionals are specialised in designing necessary orthoses for the patients according to their specific measurements and requirements. They also give basic lessons upon the usage of those orthoses.

Conclusion

Muscular Dystrophy is a physically limiting condition that can be a source of frustration to the patients. However, with constant counselling and Physiotherapy most of the functions can be retained and maintained. As a whole physiotherapy helps, but while treating the children with Duchenne´s MD one can at most try and maintain the available range of motion and the bodily functions. It is a sad reality but the progression of Duchenne´s MD cannot be delayed any better than any other forms of MD. We can only try and provide a better quality of life. Besides all, one´s own will to lead a happier life is the key to successful therapy.

Article by:

Physiotherapist IAMD: Muthu Ganapathy
Physiotherapy Intern: Ishika Sharma
(Pandit Deendayal Upadhyaya Institute for Physically Handicapped)

 

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