Treatment and Nursing Care

The treatment of clubfoot is typically started shortly after birth; otherwise, the bones and muscles continue to develop abnormally. During infancy, conservative treatment consisting of manipulation and casting to hold the foot in the right position is carried out. Manipulation, known as the Ponseti method, and casting are repeated weekly for the first 6 weeks and then at 1- to 2-week intervals. This is done to allow for rapid growth and to help the foot obtain and retain a more anatomically correct position. If the deformity is corrected, the infant is placed in a brace that maintains the correct position. The brace may be worn for 23 hours a day for an extended length of time, and then only while the child is sleeping. If manipulation or casting does not work, surgery is performed. Although surgery is tailored to the type of deformity, in most cases it involves releasing tight tendons and repositioning and pinning the foot bones. The goal is to complete the treatment by 1 year of age so that the child can use normal shoes when he or she starts walking.

Signs and Symptoms

Subluxation of the hip is commonly discovered at the time of the newborn examination. Ongoing screening for DDH should be done during routine health examinations during the first year of life. One of the most reliable signs is a limitation of abduction of the leg on the affected side. When the infant is placed on the back with knees and hips flexed, the physician can press the femur related to the normal hip back until it almost touches the examining table. On the affected side, however, this can be accomplished only partially. Also, the knee on the side of the dislocation appears to be shorter and the skin folds of the thigh are often asymmetrical. When the infant is prone, one hip is higher than the other (Figure 17-2). In some infants younger than 4 weeks of age, the physician can actually feel and hear the femoral head slip into the acetabulum under gentle pressure. This is called Ortolani’s sign or Ortolani’s click and is considered diagnostic. If the child has begun walking and has had no treatment, he or she will display the characteristic waddling gait and will test positive for the Trendelenburg gait test (pelvic drops on the side of the affected hip joint when standing on one leg). Bilateral dislocation may occur; however, unilateral dislocation is more common. Radiographs and ultrasound scans confirm the diagnosis.

Treatment

Treatment is begun as soon as the dislocation is detected and varies with the age of the child. The goal of treatment is to form a normal joint by keeping the head of the femur within the hip socket. This constant pressure enlarges and deepens the acetabulum, thus correcting the dislocation. The bones of small children are fairly pliable because they contain more cartilage than bones of adults.

From birth to approximately 6 months of age, abduction of the hips is maintained with the use of the Pavlik harness (Figure 17-3). The harness is worn full time until stability is attained plus 2 months, then a decrease in wearing time begins. Weaning time from the harness is gradual until normal hip function is established by ultrasound or x-ray. The Pavlik harness allows the infant to move the legs. If the dislocation is severe or has not been detected until the child has begun to walk, it may be necessary to use traction. This pulls the head of the femur down to the correct position opposite the acetabulum. After the traction has stretched the muscles enough to allow the hip to be placed in the acetabulum, the dislocation is reduced with general anesthesia and a spica cast is applied to hold the abduction. This type of cast is shown in Figure 17-4. The length of time that the child remains in the cast varies according to progress, growth, and the condition of the cast; however, it is usually from 3 to 4 months. During this time, the cast may be changed about every 6 weeks. Sometimes surgery is necessary. In this case, open reduction of the dislocation or repair of the shelf of the hip bone is done. A cast is applied after surgery to keep the femur in the correct position. After removal of the spica cast, some children may require an abduction brace. The brace is worn for 4 to 8 weeks, and then only at nighttime for 1 to 2 years (Canale and Beaty, 2008). For children 6 to 18 months of age, the Pavik harness is less effective and traction is required.

Nursing Care

Parents require instruction regarding skin care while the infant wears the Pavik harness. A T-shirt and long socks can protect the infant’s skin from rubbing as the harness allows the infant to move his or her legs. Parents need assurance that they may hold the infant and provide normal activity experiences.

The body spica cast encircles the waist and extends to the ankles or toes. General cast care should be reviewed (see Cast Care section under Clubfoot in this chapter). Skill 17-1 describes how to turn and position a child in a body cast.

Skill 17-1   Turning and Positioning a Child in a Body Cast

• Method

1. Use two people to turn a child in a body cast.

2. Lift the child and place in the prone position.

3. Do not use crossbars between the legs as handles.

4. With the child in the prone position, place a pillow under the chest and under each leg to prevent pressure on the toes.

5. With a bedpan, support the upper back and legs with pillows so that body alignment is maintained.

Firm, plastic-covered pillows are placed beneath the curvatures of the cast for support. Older children may benefit from an overhead bar and trapeze. Do not elevate the head or shoulders of a child in a body cast with pillows because this thrusts the child’s lower chest against the cast and can cause discomfort or respiratory difficulty. The head of the child’s bed should be slightly elevated so that urine or feces drains away from the body of the cast. A fracture bedpan should be available for appropriate children. To prevent soiling of the cast from urine and feces, plastic wrap or wide cloth tape can be tucked around the edges of the cast at the openings between the legs. A disposable diaper can be tucked under the edges around the buttocks for the same purpose. As these coverings become soiled, they must be changed immediately. Frequent changing of position is important; immobilized children need to be turned often. A covering or bib should be used during feedings to prevent food particles from getting under the cast.

Itching is a particular problem with a child in a body cast. Use of a fan facing toward the opening of the cast or a hair dryer on cool setting may relieve the discomfort. An antihistamine can sometimes help alleviate itching. Any methods that might cause injury to the skin beneath the cast are discouraged because any break in the skin under a cast is difficult to treat. Avoid giving children small objects that could be inserted inside the cast. Be aware of any foul odor from the cast, as this can indicate an infection or skin breakdown. These findings should be reported immediately to the physician.

The child with a long-term disability such as this requires help in meeting the activities of daily living (ADLs). The child is growing and developing rapidly. Dressing and clothing can be a problem. The child also may not be able to use regular furniture or play equipment enjoyed by other children. Transporting children in spica casts can be challenging. Specific equipment is available for a child with a spica cast. Wagons with pillow support, car seats that accommodate a spica cast, and reclining wheelchairs for older children are helpful. A referral to home health agencies can assist parents in obtaining equipment.

Signs and Symptoms

The symptoms of CP vary with each child and may range from mild to severe (see Did You Know?). About two thirds of children who have CP are intellectually impaired (National Institute of Neurological Disorders and Stroke, Cerebral Palsy: Hope Through Research, 2010). CP is suspected during infancy when developmental milestones are not met. Diagnostic tests include MRI, electroencephalography, computed tomography (CT), and screening for metabolic disorders. Brain tumors must also be ruled out. Early recognition is important so that early intervention can begin.

Children may have symptoms of more than one type of CP. Spastic CP is characterized by tension in certain muscle groups. When the child tries to move the voluntary muscles, jerky motions result, and eating, walking, and other coordinated movements are difficult to accomplish. The lower extremities are usually involved. The legs cross and the toes point inward (scissoring). Toe walking can occur from muscle tightness (Figure 17-5). Upper extremities, or upper and lower extremities on only one side of the body, can be affected. With athetoid or dyskinetic CP, the child has uncontrolled, slow, writhing movements that can increase during periods of emotional stress and disappear during sleep. These abnormal movements usually affect the hands, feet, arms, or legs and, in some cases, the muscles of the face and tongue, causing grimacing or drooling. Problems can also occur with the coordination of muscle movements needed for speech (dysarthria). Ataxia, or lack of muscle coordination, can be shown by disturbances of balance and depth perception. A wide-based gait accompanied by unsteadiness is generally present. Intention tremor may also be observed. As the child begins a voluntary movement, such as reaching for a book, there is a resultant trembling that affects the body part being used and worsens as the individual gets nearer to the desired object. Mixed CP is usually a combination of spastic and athetoid movements; however, other combinations of symptoms are possible.

Treatment and Nursing Care

The goal of treatment is to help children make the most of their assets and guide them into becoming happy, well-adjusted adults who perform at their maximum ability. Both short- and long-term goals must be realistic and attainable. Parents work with a multidisciplinary team including a physician, such as a pediatrician or orthopedist; a physical therapist; an occupational therapist; a speech and language pathologist; and a social worker. The long course of this disability is financial burden, and contact with social service agencies can assist families in this regard. Parents need to be informed of community resources available to them.

The specific treatment of CP is highly individualized, depending on the severity of the disease. Treatment often includes physical and occupational therapy to assist with ADLs, special education and communication devices, and recreational activities designed for the child’s developmental level and adapted to functional limitations. Sometimes surgery is necessary to correct deformities, improve function, or reduce spasticity. The speech therapist helps with communication. Many children with CP cannot produce intelligible speech.

Medications may improve overall function in children with CP. Dantrolene reduces calcium release, thereby decreasing excitation-contraction in skeletal muscle. Skeletal muscle relaxants such as baclofen may decrease spasticity. Intrathecal baclofen delivered via a pump reservoir can help with spastic quadriparesis. Botulinum toxin type A (Botox) has also been successfully used to reduce spasticity (Burg et al., 2006). Antianxiety agents may relieve excessive motion and tension, especially in the athetoid child. Anticonvulsants are prescribed for children with seizures.

All precautions are taken to prevent the formation of contractures (degeneration or shortening of the muscles because of lack of use). The damage may be permanent, resulting in loss of function of the part involved, such as a leg, arm, or finger. Children should be encouraged to do as much as they can for themselves. Other measures used to prevent deformities include frequent changes of position, passive range-of-motion (ROM) exercises, stretching exercises, and use of braces or splints. Assessment of the skin is essential for these children.

Feeding problems may occur because of swallowing and sucking difficulties. Aspiration is often a risk that requires monitoring. Feeding time should be adequate to facilitate chewing and swallowing. Older children require improved seating and adaptive eating utensils to enhance their nutritional intake. High-caloric diets are necessary to replace calories used by the constant muscle tension. Gastrostomy feeding devices may be essential to provide adequate calories.

Signs and Symptoms

The onset is generally between 2 and 6 years of age; however, a history of delayed motor development during infancy may be evidenced. A waddling gait, slowness in running or climbing, and enlarged rubbery muscles are indicative of this disorder. The calf muscles, in particular, develop pseudohypertrophy from the accumulation of fat and connective tissue. Other signs include frequent falling, clumsiness, contractures of the ankles and hips, and the Gower maneuver, a characteristic way of rising from the floor (Figure 17-6).

Laboratory findings show marked increases in blood creatine phosphokinase (CK) levels. An electromyogram (EMG), a graphic record of muscle contraction as a result of electrical stimulation, shows decreases in amplitude and duration of motor unit potentials from the destruction of muscle and not nerves. Electrocardiographic (ECG) abnormalities are also common because of progressive cardiomyopathy. A muscle biopsy may be considered. It reveals degeneration of muscle fibers and replacement of these fibers by fat and connective tissue.

The disease becomes progressively worse. With orthopedic bracing, physical therapy, and sometimes surgery, the child may be able to maintain ambulation until 12 years of age. About one third of boys with DMD have some degree of learning disability, although few are seriously mentally impaired. Life expectancy averages between the late teens and mid-thirties. Death usually results from cardiac failure or respiratory infection.

Treatment and Nursing Care

Treatment at this time is mainly supportive. It consists of passive exercises to prevent joint contractures, bracing, weight control, surgery for joint contractures, and referrals to appropriate social service agencies. Corticosteroid treatment has been explored with these children. The steroids improve muscle strength, but weight gain from steroid use may prove to be more of a problem. Infections are treated with antimicrobials. Although cardiac manifestations are usually late events, digoxin and diuretics may be beneficial in the early stages of the disease. Keeping weight down is essential; a low-calorie diet may be encouraged. The psychological impact related to the chronic and progressive nature of the disease and its fatal outcome should be addressed. Family denial of the diagnosis is common early in the disease, when symptoms are fairly benign.

Compared with other children with disabilities, children with muscular dystrophy may appear passive and withdrawn. Early on, they may become depressed because they are unable to compete with their peers. Social and emotional pressures on the child and family are great. Financial pressures become magnified as medical and surgical costs escalate. In addition, expensive alterations to the family lifestyle, home, and vehicles are sometimes necessary.

Nurses function as team members along with personnel from many other disciplines in the care of the child with muscular dystrophy. The child should be encouraged to be as active as possible to delay muscle atrophy. Swimming and other activities that promote ROM and mobility for as long as possible are helpful. Nurses provide support for the many daily issues that occur by referring parents to other parents, camp programs, respite care, the Muscular Dystrophy Association (www.mdausa.org), public health nurses, home health agencies, family therapists, and eventually hospice care. Although there is no cure at present, different drug therapies, genetic engineering, and stem cell research are beginning to show promise and continue to be investigated (Quintero et al., 2009).