Muscular dystrophy is a progressive degeneration of skeletal muscles from an as yet unknown biochemical defect within the muscle. Duchenne’s muscular dystrophy has an incidence of about 3 per 100,000 and is inherited as a recessive single gene defect on the X chromosome, which means it is transmitted from the mother to her male offspring. Another form of dystrophy, Becker’s muscular dystrophy, is similarly X-linked but has its onset later in childhood or adolescence and has a slower course. Other heredity disorders include Landouzy-Dejerine dystrophy (fascioscapulohumeral) and Erb’s dystrophy (limb-girdle).
Nursing care plan assessment and physical examination
A complete family and developmental history provides important diagnostic data for the patient with muscular dystrophy. Since it is a genetic disease, determine if anyone in the family has been previously diagnosed with a musculoskeletal or neuromuscular disease. Children with Duchenne’s muscular dystrophy have a history of delayed motor milestones, such as sitting, walking, and standing. Adults may report progressive muscle weakness of the legs, face, and shoulder. The patient may experience difficulty raising the arms over the head or closing the eyes completely. Other early signs include difficulty in puckering the lips, abnormal facial movements, and the inability of facial muscles to change during laughing and crying.
Most dystrophies involve the hip and shoulder girdle musculature, which leads to functional difficulties. Assess the patient’s ability to raise the arms above the head, get up from a chair, or walk. Inspect the patient for scoliosis and contractures. For 2- to 5-year-old boys with Duchenne’s muscular dystrophy, observe for pelvic and shoulder girdle muscles with distal involvement. Note a waddling, stumbling gait or difficulty climbing stairs. A characteristic sign is the Gower’s maneuver: the patient uses his or hands hands to walk up the legs until he or she is standing erect. His or her postures may also be distorted, with a lumbar lordosis and protuberant abdomen. He or she may toe-walk to compensate for quadriceps weakness. Scoliosis occurs after the child is wheelchair-dependent because of weak trunk muscles. Tachycardia occurs as the heart muscle weakens and enlarges. Generally, any cardiac muscle involvement is asymptomatic until late in the course of the disease. Pneumonia develops easily as the child’s cough reflex becomes weak and ineffective.
Since muscular dystrophy is a progressive disease that limits the normal life span, patients and their families will require ongoing emotional support. The genetic nature of the disease frequently creates guilt that often leads to depression. Boys with Duchenne’s muscular dystrophy often have an IQ below 90. Frustration, depression, and other signs of emotional immaturity may be present because of the intellectual limitation. Family functioning is challenged because of the progressive losses and prognosis.
Nursing care plan primary nursing diagnosisL Impaired physical mobility related to muscle destruction.
Nursing care plan intervention and treatment plan
Currently, there is no cure for muscular dystrophy, and therapeutic management is focused on managing the symptoms and maintaining the highest level of functional independence possible. Patients with muscular dystrophy are managed by a multidisciplinary team because of their complex needs. The goals of treatment are to facilitate ambulation and aggressively manage respiratory and cardiac difficulties. With early diagnosis, interventions such as appropriate diet and exercise and social and psychological counseling can begin to prolong the patient’s independence and quality of life.
Physical therapy is directed toward keeping functional muscle strength and preventing contractures by passive stretching. Swimming is frequently recommended as an excellent exercise for keeping limber and for allowing participationin athletic events. Gait training and transfer training are important as the patient loses muscle power. Crutches and the use of a powered wheelchair maintain independent mobility for as long as possible. The occupational therapist may fit the patient with braces or splints to prevent or treat contractures. Long leg braces are needed to provide stability for weakened muscles that can no longer provide support for ambulation.
Once the patient is in a wheelchair, obesity frequently becomes a problem. A low-calorie, high-protein diet is recommended to avoid this complication because the additional weight places a strain on already compromised muscles. Constipation may also be a problem that can be managed with added dietaryfiber, extra fluids, and stool softeners.
The physician may consider surgery to assist the patient in maintaining a higher quality of life. A spinal fusion may correct abnormal spinal curvatures that occur as the trunk muscles weaken to improve comfort, balanced sitting, and body image. Muscle- and tendon-lengthening procedures may be needed to improve decreased function if contractures form. Preoperative and postoperative preparation is essential to prevent complications. Before a surgical procedure is attempted, an electrocardiogram is needed to determine if the cardiac muscle has been affected by the muscle disease. Malignant hyperthermia (a potentially lethal increase in body temperature in response to certain muscle relaxants or anesthetic agents) is a complication that occurs in children with Duchenne’s muscular dystrophy. Early ambulation is necessary to prevent additional weakness, and aggressive respiratory care prevents pneumonia.
Because of the nature of muscular dystrophy, nursing interventions are primarily preventive and supportive. Prevention of complications requires anticipation of problems and systematic monitoring for progression of the disease process. Proper management can increase the length and quality of life. Differences in care can be divided by decreasing mobility into three phases: ambulatory, wheelchair, and bedrest phases.
In the ambulatory phase, when the family first learns of the diagnosis, information about the
disease, prognosis, and treatment plans may be overwhelming. Be available to answer questions, provide clarification, and provide emotional support and encouragement related to the family’s needs for control in care decisions. The family needs opportunities to express feelings about the genetic transmission, progressive nature, and effect of the disease on the family. The status of younger male children in the family may not be certain; uncertainty creates additional anxiety.
Evaluate safety issues in the home. The child’s bedroom should be moved to the first floor if possible, and rugs need to be removed to facilitate mobility. Rubber-soled shoes help prevent slipping. As the disease progresses, home care equipment may be necessary for assistance with mobility and activities of daily living. Nursing interventions are directed toward preventing contractures and encouraging independence and normal development. The patient needs to be monitored for a tendency toward contracture development and range-of-motion exercises instituted as a preventive measure. Stretching exercises and splinting of the arms and legs at night help to slow the progression of contractures. Assist the child and family in developing a plan for both active and passive range-of-motion exercises to do daily; help make reminder sheets so exercises are consistently done.
As the patient becomes dependent in the wheelchair phase, comfort measures become even more critical. Teach the family to make frequent skin assessments to determine evidence of skin breakdown from prolonged sitting. Support stockings, passive exercises, and elevating the lower extremities may decrease pedal edema for patients in wheelchairs. Provide interventions directed toward attaining the child’s maximum growth and development level. In the bedrest phase, eventually the patient will be unable to move without assistance. Frequent position changes and meticulous skin care are essential. Proper body alignment in the bed or chair can be maintained by the use of blanket rolls, sandbags, or pillows. Sheepskin or an alternating pressure mattress may provide comfort. Adequate fluid intake is needed to prevent urinary or bowel complications. Use incentive spirometry and diaphragmatic breathing exercises to maintain gas exchange when respiratory muscles are weak. Percussion and postural drainage are used to facilitate effective airway clearance.
Referral needs to be made to the Muscular Dystrophy Association (MDA) and other community agencies. These organizations can provide information about the disease, management, and emotional and social support during the long period of illness. They can also provide financial assistance for treatment needs.
Nursing care plan discharged and home health care guidelines
The child with muscular dystrophy has multiple admissions and discharges. Because multiple dystrophy is a chronic progressive disease, teaching needs to vary according to the interventions and phase of illness. The muscular dystrophy team and the MDA are the best sources of providing information to the patient and family in a timely manner. Encourage the child and parents to maintain peer relationships and foster intellectual development by keeping the child in school as long as possible. Teach the patient and family ways to avoid respiratory problems. Encourage the parents to report respiratory infections as soon as they occur.