Paget’s disease is a slowly progressive metabolic bone disease. It usually localizes in one or several areas of the skeleton (most commonly the lower torso), although occasionally, widely distributed skeletal deformity occurs. Paget’s disease can be fatal, particularly if associated with heart failure (widespread disease creates a continuous need for high cardiac output), bone sarcoma, or giant cell tumors.


What causes it
The cause remains unknown, but one theory holds that early viral infection (possibly with mumps virus) causes a dormant skeletal infection that erupts many years later as Paget’s disease. The disease also tends to run in families.

In the initial phase of Paget’s disease (osteoclastic phase), excessive bone resorption occurs. The second phase (osteoblastic phase) involves excessive abnormal bone formation. Affected bones enlarge and soften, and the new bone structure is chaotic, fragile, and weak.

What to look for
Although Paget’s disease produces no symptoms in the early stages, it eventually produces severe, persistent pain that intensifies with weight bearing and may impair movement. Characteristic cranial enlargement occurs over frontal and occipital areas (hat size may increase). Headaches also occur with skull involvement. Bony infringement on cranial nerves may impair hearing and visual acuity.

Other signs may include kyphosis, barrel-shaped chest, and asymmetrical bowing of the tibia and femur. The pagetic sites may be warm and tender, with slow and incomplete healing of fractures. The patient may walk with a waddling gait and have increased susceptibility to pathologic fractures.

What tests tell you
• X-rays may show increased bone expansion and density before overt symptoms develop.
• A bone scan (more sensitive than an X-ray) clearly shows early pagetic lesions. Radioisotope concentrates in areas of active disease.
• A bone biopsy reveals the characteristic mosaic pattern.
• Blood tests reveal anemia and elevated serum alkaline phosphatase levels. Routine biochemical screens, which include serum alkaline phosphatase, make early diagnosis more common.
• A 24-hour urine test shows an elevated hydroxyproline level. (Hydroxyproline is an amino acid excreted by the kidneys and is an index of osteoclastic hyperactivity.)

How it’s treated
Drug therapy is the primary intervention. It includes the hormone calcitonin, given subcutaneously or I.M.; etidronate (Didronel), taken by mouth; and plicamycin (Mithracin), a cytotoxic anti biotic.

Retarding resorption and reducing levels


Calcitonin and etidronate retard bone resorption and reduce serum alkaline phosphatase levels and urinary hydroxyproline secretion. Although calcitonin requires long-term maintenance therapy, improvement is noticeable after the first few weeks of treatment. Etidronate produces improvement in 1 to 3 months. Plicamycin decreases calcium, urinary hydroxyproline, and serum alkaline phosphatase levels. This medication produces remission of symptoms within 2 weeks and biochemical improvement in 1 to 2 months. However, it may destroy platelets or compromise renal function.

Surgery still looms


Self-administration of calcitonin and etidronate helps patients with Paget’s disease lead near-normal lives. Nevertheless, these patients may need surgery to reduce or prevent pathologic fractures, correct secondary deformities, and relieve neurologic impairment.

To decrease the risk of excessive bleeding from hypervascular bone, drug therapy with calcitonin and etidronate or plicamycin must precede surgery. Joint replacement is difficult if bonding material (methyl methacrylate) is used because it doesn’t set properly on pagetic bone. Other treatment is supportive and varies according to symptoms.Aspirin, indomethacin, or ibuprofen usually controls pain.

What to do
• To evaluate the effectiveness of analgesics, assess the patient’s level of pain daily. Watch for new areas of pain or restricted movement, which may indicate new fracture sites. Also watch for sensory or motor disturbances, such as difficulty hearing, seeing, or walking.
• Monitor serum calcium and alkaline phosphatase levels.
• Monitor intake and output. Encourage adequate fluid intake to minimize renal calculi formation.
• If the patient is on prolonged bed rest, prevent pressure ulcers by providing good skin care. Reposition the patient frequently and use a flotation mattress. Provide high-topped sneakers to prevent foot drop.
• Evaluate the patient and assess the success of therapy by asking yourself these questions:
– Does the patient avoid activities that increase the risk of fracture, while at the same time maintaining ROM?
– Does the patient have neurologic deficits, such as footdrop, because of progression of the disease or interventions?
– Does the patient demonstrate effective coping skills for dealing with his illness?
– Has adherence to the prescribed medication and dietary regimens prevented progression of the disease?




Rheumatoid arthritis (RA) is a chronic, systemic inflammatory disease that primarily attacks peripheral joints and surrounding muscles, tendons, ligaments, and blood vessels. Spontaneous remissions and unpredictable exacerbations mark the course of the disease. Potentially crippling, RA usually requires lifelong treatment and sometimes surgery.

From intermittent to incessant


In most patients, the disease follows an intermittent course and allows normal activity. However, 10% of patients suffer total disability from severe articular deformity and associated extra- articular symptoms, or both. Prognosis worsens with the development of nodules, vasculitis, and high titers of rheumatoid factor (RF).

What causes it
RA is currently believed to have an autoimmune basis, although the cause remains unknown.

Cartilage damage resulting from inflammation triggers further immune responses, including complement activation. Complement, in turn, attracts polymorphonuclear leukocytes and stimulates the release of inflammatory mediators, which exacerbates joint destruction.

What to look for
Initial symptoms may include fatigue, malaise, anorexia, persistent low-grade fever, weight loss, and lymphadenopathy. The patient may also experience vague articular symptoms.

Sooner and later


Later, the patient may develop joint pain, tenderness, warmth, and swelling. Usually, joint symptoms occur bilaterally and symmetrically. Other symptoms may include morning stiffness; paresthesia in the hands and feet; and stiff, weak, or painful muscles. The patient may also develop rheumatoid nodules — subcutaneous, round or oval, nontender masses, usually on pressure areas such as the elbow. Advanced signs include joint deformities and diminished joint function.

What tests tell you
In early stages, X-rays show bone demineralization and soft-tissue swelling. Later, they help determine the extent of cartilage and bone destruction, erosion, subluxations, and deformities and show the characteristic pattern of these abnormalities. Other tests and findings include:

• Positive RF test occurs in 75% to 80% of patients (as indicated by a titer of 1:160 or higher).
• Synovial fluid analysis usually shows increased volume and turbidity but decreased viscosity and complement (C3 and C4) levels, with WBC count possibly exceeding 10,000/l
• Serum globulins are elevated.
• ESR is elevated.
• Complete blood count shows moderate anemia and slight leukocytosis.

How it’s treated
Salicylates, particularly aspirin, provide the mainstay of RA therapy because they decrease inflammation and relieve joint pain.
Other useful medications include:
• NSAIDs, such as indomethacin, ketorolac, and ibuprofen
• antimalarials, such as chloroquine and hydroxychloroquine (Plaquenil)
• tumor necrosis factor inhibitors, such as etanercept (Embrel)
• penicillamine (Depen)
• corticosteroids, such as prednisone
• immunosuppressants, such as methotrexate (Trexall), cyclophosphamide (Cytoxan), and azathioprine (Imuran).

Sleep well, eat right, and rest often


Supportive measures include 8 to 10 hours of sleep every night, adequate nutrition, frequent rest periods between daily activities, and splinting to rest inflamed joints. A physical therapy program, including ROM exercises and carefully individualized therapeutic exercises, forestalls loss of joint function. Application of heat relaxes muscles and relieves pain. Moist heat, such as hot soaks, paraffin baths, and whirlpools, usually works best for patients with chronic disease. Ice packs are effective during acute episodes.
Advanced disease may require synovectomy, joint reconstruction, or total joint arthroplasty.

What to do
• Assess all joints carefully. Look for deformities, contractures, immobility, and inability to perform ADLs.
• Monitor vital signs and note weight changes, sensory disturbances, and level of pain. Administer analgesics as ordered and watch for adverse effects.

• Give meticulous skin care. Use lotion or cleansing oil, not soap, for dry skin.
• Explain all diagnostic tests and procedures. Tell the patient to expect multiple blood samples to allow firm diagnosis and accurate monitoring of therapy.

The benefits of bubble baths


• Monitor the duration, not the intensity, of morning stiffness because duration more accurately reflects the severity of the disease. Encourage the patient to take hot showers or baths at bedtime or in the morning to reduce the need for pain medication.
• Apply splints carefully. Observe for pressure ulcers if the patient is in traction or wearing splints.
• Explain the nature of RA. Make sure the patient and his family understand that RA is a chronic disease that requires major lifestyle changes.
• Encourage a balanced diet, but make sure the patient understands that special diets won’t cure RA. Stress the need for weight control because obesity adds further stress to joints.
• Urge the patient to perform ADLs, such as dressing and feeding himself. Supply easy-to-open cartons, lightweight cups, and unpackaged silverware.

Can we talk?


• Provide emotional support. Encourage the patient to discuss his fears about dependency, sexuality, body image, and selfesteem. Refer the patient to an appropriate social service agency as needed.
• If appropriate, discuss sexual aids, such as alternative positions, pain medication, and moist heat to increase mobility.
• Before discharge, make sure the patient knows how and when to take his prescribed medication and how to recognize adverse effects such as GI bleeding from salicylate therapy.
• Evaluate the patient. When assessing his response to therapy, note whether compliance with exercise and dietary regimen slows progression of debilitating effects. Has he maintained or improved his ability to perform ADLs? Does he use effective pain control measures? Does he use appropriate assistive devices?



Scoliosis — a lateral curvature of the spine — may occur in the thoracic, lumbar, or thoracolumbar spinal segment. The curve may be convex to the right (more common in thoracic curves) or to the left (more common in lumbar curves). Rotation of the vertebral column around its axis occurs and may cause rib cage deformity.
There are two types of scoliosis: functional (postural) and structural. Both types are commonly associated with kyphosis and lordosis.

What causes it
Functional scoliosis isn’t a fixed deformity of the vertebral column. It results from poor posture or a discrepancy in leg lengths.

Three sorts of structural scoliosis


Structural scoliosis involves deformity of the vertebral bodies. It can be one of three types:
• Congenital scoliosis is usually related to a congenital defect, such as wedge vertebrae, fused ribs or vertebrae, or hemivertebrae.
• Paralytic, or musculoskeletal, scoliosis develops several months after asymmetrical paralysis of the trunk muscles from polio, cerebral palsy, or muscular dystrophy.
• Idiopathic scoliosis, the most common form, may be transmitted as an autosomal dominant or multifactorial trait. It appears in a previously straight spine during the growing years.

In scoliosis, the vertebrae rotate, forming the convex part of the curve. The rotation causes rib prominence along the thoracic spine and waistline asymmetry in the lumbar spine.

What to look for
The most common curve in functional or structural scoliosis arises in the thoracic segment, with convexity to the right. This curve results in compensatory curves (S curves) in the cervical segment above and the lumbar segment below, both with convexity to the left. These curves develop to maintain body balance and mark the deformity. When the disease becomes well established, backache, fatigue, and dyspnea may occur.


Unequal and asymmetrical


Physical examination reveals unequal shoulder heights, elbow levels, and heights of the iliac crests. Muscles on the convex side of the curve may be rounded, whereas those on the concave side are flattened, producing asymmetry of paraspinal muscles.

What tests tell you
• Anterior, posterior, and lateral spinal X-rays, taken with the patient standing upright and bending, confirm scoliosis and determine the degree of curvature and flexibility of the spine.
• Bone growth studies may help determine skeletal maturity.

How it’s treated
The severity of the deformity and potential spine growth determine appropriate treatment. Interventions include close observation, exercise, a brace (for example, a Milwaukee brace), surgery, or a combination of these. To be most effective, treatment should begin early, when spinal deformity is still subtle.

Mild curve


A mild curve (less than 25 degrees) can be monitored by X-rays and an examination every 3 months. An exercise program may strengthen torso muscles and prevent curve progression. A heel lift may help.

Moderate curve


A curve of 25 degrees to 40 degrees requires spinal exercises and a brace. Alternatively, the patient may undergo transcutaneous electrical nerve stimulation. A brace halts progression in most patients but doesn’t reverse established curvature.

Dangerous curve


A curve of 40 degrees or more requires surgery (spinal fusion, usually with instrumentation) because a lateral curve progresses at the rate of 1 degree per year even after skeletal maturity. Preoperative preparation may include Cotrel-Dubousset segmental instrumentation for 7 to 10 days.
Postoperative care commonly requires immobilization in a localizer cast (Risser jacket) for 3 to 6 months. Periodic checkups follow for several months to monitor stability of the correction.

What to do
• If the patient needs traction or a cast before surgery, check the skin around the cast edge daily. Keep the cast clean and dry and the edges of the cast “petaled” (padded).

Under your skin


• Warn the patient not to insert anything or let anything get under the cast and to immediately report cracks in the cast, pain, burning, skin breakdown, numbness, or odor. Watch for skin breakdown and signs of cast syndrome (such as nausea, abdominal pressure, and vague abdominal pain).
• Evaluate the patient. Make sure that she doesn’t develop a neurovascular deficit or loss of skin integrity because of bracing, traction, or surgery. Can she maintain an activity level normal for her age and developmental level?
• Evaluate the results of surgery, if appropriate. Is pain absent or controlled? Are breath sounds, skin color and turgor, elimination patterns, and arterial blood gas levels normal? Are shoulders and hips aligned horizontally?





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