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REHABILITATION IN A MUSCLE DYSTROPHIES

Autor: Ligia Robănescu Cristina Bojan
Distribuie pe:

Children with muscular dystrophies have a lifelong challenge to maintain function. That challenge can met with the help of a knowledgeable physical therapist: helping them be as independent and confortable as possible, within the limits of their disability. The major goals of management common to all children: Prevent deformity: ROM exercices, stratching, orthotics, serial casting,electrical stimulations,etc. Prolong functional motor capacity, improve pulmonary function, assisted ventilation, control pain, if necessary, orthopedic treatment : spinal fixation, tendon lengthening, muscular transpositions, etcm assistance of family support.

Muscular dystrophies include a wide pathology, based on chronic suffering of the muscle fibres, with the reaction of endomysial collagen and of cellular fat, necrosis lesions, signs of recovery in the form of atrophic and nonspecific hypertrophic hyalinised fibres. (21)
Both congenital muscular dystrophies and the progressive ones should be sustained since the moment of their diagnosis, by a rehabilitation program to prevent complications, to prolong mobility and for a higher life quality.
The role of the physical therapist is very important in all the three stages of the disease, particularly in the case of the progressive forms (15):

  • Early outpatient treatment;
  • Transition phase, when the patient retains ambulation;
  • Wheelchair – dependent stage.

Assessment:

During the stable stage, the patient is checked with the aim to:

  • Transfer from dorsal decubitus (DD) position to an upright position.
  • Walking along different distances (10 m).
  • Transfer from sitting position to upright position.
  • Climbing up four steps in a staircase.

During these tests, the time necessary to perform the movements and the level of fatigue are checked. For example, the interval of time it takes a patient to rise from the floor (Gower’s sign), if the patient uses one or both hands as support at knee level.
If the 10 m walking test is longer than 9 seconds, this is predictive of the loss of within two years. If the 10 m are walked in 12 seconds, the patient will lose ambulation in about one year.
Another test is the 6-minute walking as quickly as possible, allowing halts along the way.
Vignos functional scale (associated with Brooke scale). It is a classification that corresponds to the analysis of the walking capacity using 10 grades (30):

  1. The patient walks and climbs stairs without help.
  2. The patient walks and climbs stairs using handrails.
  3. The patient goes up the stairs slowly, using the railing (in over 12 seconds for four stairs).
  4. The patient walks without technical assistance (e.g. a deambulator), gets up from the chair, but cannot climb stairs.
  5. The patient walks without technical support, does not climb the staircase, does not get up from the chair.
  6. The patient walks with technical support, or walks independently with long orthoses.
  7. The patient walks with long orthoses, but needs technical support to maintain balance.
  8. The patient keeps standing, but does not walk, not even with technical support.
  9. The patient is dependent on a wheelchair.
  10. The patient cannot leave the bed.

The Brooke Upper Extremity Scale (6) is a 6-point scale that allows classification of upper limb function:

Initially the arms are along the body – patient can abduct them in vertical position, touching the head;
Hands on head, with flexed elbows;
Cannot put hands on the head, but takes a glass of water to the mouth, possibly with both hands;
Takes hands to the mouth, but cannot take a glass of water to the mouth;
Cannot bring hands to the mouth, but can hold a pencil or take a coin from the table;
No function of the hand is possible.
Motor function measure scale for neuromuscular diseases (L’Escale Centre – Lyon) – adaptation after Global muscle function assessment.
It is a list of 32 items divided into 3 dimensions, with testing in the supine, sitting and standing positions:

  • D1: standing position and transfers 13 items
  • D2: axial and proximal motor function 12 items
  • D3: distal motor function 7 items (6 of which are for the upper limbs )

Scores: 0: does not initiate the requested posture or task;

  1. the exercise is performed partially.
  2. the exercise is imperfect as duration, position or motion control.
  3. the exercise is performed normally, with constant speed.

I. Muscle testing can reveal the progression of the disease.
The rate of progression is: rapid, with 10% deterioration per year, average, with 5-10% deterioration per year, slow, with 5% deterioration per year.
Goniometry (or the standard assessment of joint motion): especially dorsal flexion of tibial-tarsal joints, knee extension, hip-femoral joint extension, given the retractions at this level.

Physical therapy

The physiotherapist is faced with many problems such as weakening of muscle strength, decrease of active and passive range of motion, dysfunction of ambulation, decrease in functional ability, decrease of pulmonary function, emotional trauma of the patient and of the family, progressive scoliosis, pain.
Therapeutic Issues
Prevention of deformities, extension of muscle functional capacity, improvement of lung function, support to family, control of pain.
Prevention of deformities [25, 30, 33]:

  • Daily stretching: 10-15 rounds of 15 seconds each. Two times a day for psoas, triceps surae, hamstrings, tensor fascia latae.
  • Fixed ankles-foot orthoses for the night – they prolong independent walking!
  • Orthostatism: 2 – 3 hours/day (Verticalizer) or walking.
  • Serial casting to avoid the fixation of the plantar flexion [14]

For the spinal cord: when loss of ambulation takes place, it has been noted that, while positioned in a wheelchair, the child develops a column convexity at the side of the dominant extremity, therefore it is recommended that the chair be conducted by the patient equally from both sides.

  • Furthermore, cushions will be used in the couch to favour a symmetrical position of the column
  • Classic scoliosis orthoses are not used in Duchenne muscular dystrophy – They have not proved a capacity to halt the development of curves. Soft orthoses are preferred instead.

Surgery for spinal stabilization is necessary if important curves have developed, then the therapist will resume the recovery process, taking into account the stiffness of the spine after surgery. [18]

Classical indications for surgery for scoliosis in DMD are a Cobb angle> 30 l and forced vital capacity> 35% with a comprehensive preoperative evaluation on respiratory function, heart check-up, anaesthetics consultation, antibacterial prophylaxis, etc. [29, 33].
Mubarak recommends intervention to curvatures > 20 l with vital capacity > 40%.
This procedure increases the quality of life, because the posture is much better and lung function decline is significantly delayed compared to the situation in non-operated patients [1].
Please note the possible obliquity of the postoperative pelvis, which must be adjusted by means of wheelchair cushions.

Extension of muscle functional capacity:

The child should be encouraged towards activities of his age as much as possible, while avoiding muscle fatigue, respectively, not feeling weakened at 30 minutes after exercising, or not experiencing pain for 24-48 hours after exercise (muscle cramps, drowsiness in the extremities , tachypnea).
Closed chain exercises, running exercises are contraindicated in Duchenne muscular dystrophy (DMD), as well as exercises with resistance, since there is danger of muscle injury [9].
Exercises to increase muscle strength are very controversial.
There are few randomized controlled studies, groups of subjects with various muscular dystrophies with different pathologies, and tracking was not done for a long time. In patients with DMD, after exercise to increase strength, when they were examined after 4 weeks, the results observed were worse than the ones in the other dystrophies, such as the Limb-girdle or the Facioscapulohumeral ones. [2]
Most researchers say that the exercises should begin early, with a minimum strain on the muscle. [33].
There is very little research on exercises in nonambulatory stage. Studies on mice have demonstrated the damage of the muscle cell membrane after sustained running exercise. [7]
The recommendation to ban exercises with resistance and eccentric exercises in DMD in older children and adolescents remains valid.
Resistance exercises with submaximal value, such as swimming or cycling are recommended only in the young child. [17, 22]
In facioscapulohumeral dystrophy, a study on exercises to increase the strength combined with electrostimulation over 5 months found improved muscular testing and 6-minute test without fatigue or pain elements. [10]
In the case of DMD, TENS stimulations increased muscular strength and motility in general [20, 23]Another study on cases of facioscapulohumeral dystrophy for 6 months, with a program of aerobic exercises, demonstrated functional benefits. [24] It seems that a combination of strength exercises with resistance ones is beneficial for this disease.
Therapy in water is recommended for all forms of muscular dystrophy, especially in DMD.
In the case of Becker dystrophy, a non-randomized study demonstrated that resistance training for 50 sessions of 30 minutes caused an increase in muscle strength with no signs of damage to the muscle fibre. [27]
In the cases of limb-girdle dystrophy and Becker dystrophy, low intensity training of a group was compared with the increased intensity of another group over 6 months. For both groups there was an increase in muscle strength and of the resistance, while the exercise had tolerated well. [27]
Of course, all these experiments need to be followed up in the long term in order to study the effects of therapy and of course, through randomized controlled research.

Prolongation of ambulation:

As the DMD and Becker dystrophy progress, walking occurs with broader base, the step is smaller, swaying of the pelvis and lumbar lordosis appear which is accentuated due to the failure of gluteus medius muscle with positive Trendelenburg test.
Stability of hip joint in orthostatism is ensured passively by the anterior structures of the hip, respectively the iliofemoral ligament.
In this situation, a retraction of the hamstrings will make walking impossible.
It has been demonstrated that the treatment which combines stretching with night orthoses reduces the rate of retraction and extends ambulation. (11, 25)
Besides, Achilles tendon lengthening, posterior tibia transposition, transcutaneous tenotomies followed by sustained physical therapy and orthotics, have significantly prolonged ambulation. (30)
Physical therapy after surgery presumes orthostatism and walking exercises 3-5 hours per day.
If the child has not walked yet, he will be placed in orthostatism in a verticalizer for a minimum of an hour a day.
Children treated with corticosteroids can prolong their walking without surgery and there are many centres that do not recommend surgery in all DMD cases. An alternative to surgical correction consists of serial castings to prevent Achilles tendon retractions, with casts changed every 1-2 weeks. [14]
Ambulation in the case of Becker dystrophy and limb-girdle dystrophy is assisted by various modern methods such as robot-assisted training or facilities for taking body weight. [3]

Use of the wheelchair:

It is better to introduce it before the total loss of ambulation when the child will use it only on long distances.
Predicting the loss ambulation (6):
Ambulation ceases in 2.4 years (range 1.2 to 4.1 years) after the patient cannot climb four standard stairs (6 inches) in less than 5 seconds.
Ambulation ceases in 1.5 years (range 0.6 to 2.2 years) if the patient climbs the stairs in more than 12 seconds.
The wheelchair (mechanical or electrical) will be equipped with support in the convexity of the scoliosis, which is usually painful. In case of kyphosis, the painful pressure is felt at the level of the sacrum.
After a possible spinal arthrodesis the wheelchair is readapted, since the pressure zones are modified.
Upper limb support will be adjusted to favour eating, teeth brushing, etc., considering that trunk flexion is more difficult to perform.
Possibility of relaxing the neck and chest muscles will be considered. [19]
Attention should be paid to weight control – it needs to be performed regularly, both for the child who still walks and for the one who is bound to the wheelchair; he will be prevented to achieve the transfer and the respiratory function will be affected.

In order to facilitate sleep:
Special air or foam mattress will be prescribed, especially for children who can no longer change position during the night by themselves.
For daily activities, the occupational therapist will deal both with self-service skills and with personal hygiene, as possible, and with the adaptations in the home.
Improvement of lung function [4, 12, 26, 28]:
Respiratory problems are multiple and progressive, especially for DMD. Inspiratory volume decreases due to the dysfunction of the diaphragm, the muscles of the chest and abdomen, thus the elimination of the secretions becomes very difficult.
If possible, oxyhemoglobin saturation will be monitored with an oximeter (it should be equal to or greater than 95%).
The child will require exercises of bronchial drainage, chest percussion, and assisted cough. The therapist will use these manoeuvres while simultaneously instructing family members.
Heimlich manoeuvre and acceleration of expiratory flow are very important.
There is also a mechanical assistance by the use of the mechanical in-exsufflator device
In case of the severe impairment of respiratory function, a non-invasive mechanical ventilation should be established 8-16 hours a day.
When oxygen saturation falls below 95%, tracheostomy is performed, passing to invasive ventilation.
Training exercises for the inspiratory muscle are mandatory in any stage of DMD. [32]

Support to families (16):

It is extremely important and falls on each member of the team that takes care of patient, especially the physical therapist who sees the patient and his family most often.
Likewise, the psychologist will cater for the cognitive functioning, school integration, psychosocial and behaviour functioning.
Interventions in support of the patient are important in case of manifestations of anxiety, depression, behaviour disorders, and difficulties of adapting to a progressive condition.
Psychological interventions address to family members, too for emotional support or counselling in the event of death.
Control of pain:
Muscle pain due to overwork may occur in case of inadequate exercise or because of the patient’s effort to perform the transfer, to change position in bed, or to push the wheels of the chair over a long distance. Treatment with creatine seems to be salutary. (15)
Attention should be paid to the grips adopted for lifting the patient; there are special handling techniques not to cause pain and to distribute properly the pressure in the joints. In any case, two people must lift a homebound sick patient.
Measures will be taken for an adapted wheelchair or a mattress suitable for proper distribution of pressure points.
Many specialists are involved in the rehabilitation process of these disorders and they must constantly cooperate with each other to contribute to a higher quality of life of their patients.

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