Pagets Disease

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Pagets Disease

• Also referred to as Osteitis Deformans
• Part of Unit VI

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Etiology and Epidemiology

• Characterized by an excess of bone destruction
  and unorganized bone formation
• Cause is unknown
• Genetic predisposition in 10 of patients
• Slow viral infection may trigger the disease
• Autoimmune dysfunction
• Average age at dx is 50-60 yrs
• Affects the vertebrae, skull, pelvis, femur, and
  tibia
• Men affected more than women

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Pathophysiology

• Increase in osteoclast-mediated bone resorption
• Increase in osteoblast-mediated bone formation
• Result is new bone that is structurally weak and
  enlarged
• Increased vascularity (bleeding may be a problem)
• Mosaic-like mix of abnormal woven bone
• Deformities and bony enlargement occur (bowing of
  the limbs and curvature of spine may occur)
• Long-standing disease can lead to malignant
  transformation (osteosarcoma)

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Manifestations

• Produces no symptoms in the early stages
• Eventually produces severe, persistent pain that
  intensifies with weight bearing and may impair
  movement
• Cranial enlargement occurs
• Headaches occur with skull involvement
• Hearing and vision may be impaired due to
  infringement on cranial nerves
• Other signs kyphosis, barrel-shaped chest, and
  asymmetrical bowing of the tibia and femur
• Pagetic sites may be warm and tender
• Slow and incomplete healing of fractures
• Walks with waddling gait
• Predisposed to pathologic fractures

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Manifestations of Pagets Disease
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Manifestations
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Pagets Disease (humerus)
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Diagnostic Tests

• Xrays
• Bone scan
• Bone biopsy
• Blood tests
• Elevated serum alkaline phosphatase levels
• 24 hour urine test shows elevated hydroxyproline
  level

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TreatmentDrug therapy is primary intervention

• Biphosphonates (inhibits osteoclastic bone
  resorption)
• Fosamax
• Aredia (IV)
• Actonel
• Didronel
• Skelid
• Reclast (IV)

• Calcitonin analogs
• (recommended for tx if biphosphonates
  contra-indicated)
• Miacalcin
• Calcimar injection
• Osteocalcin injection
• Given SQ or IM
• Cytotoxic Antibiotic
• Mithracin

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Treatment (continued)

• Surgery
• May be needed to reduce or prevent pathologic
  fractures
• Correct secondary deformities
• Relieve neurological impairment

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Collaborative Care(Pagets)

• Symptomatic and supportive care
• Correction of secondary deformities by surgical
  intervention or braces
• Medications to slow bone resorption
• Monitoring of serum alkaline phosphatase levels
  (drug effectiveness)
• Administration of Calcium and Vitamin D
• Management of pain (NSAIDS)

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Osteopenia

• What is osteopenia?
• Bone mineral density (BMD) that is lower than
  normal peak BMD, but not low enough to be
  classified as osteoporosis
• Can be a precursor to osteoporosis

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Osteomalacia (adult bone disease)

• Vitamin D deficiency resulting in decalcification
  and softening of the bone
• Same as Ricketts in children

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Etiology (osteomalacia)

• Not enough vitamin D in the diet
• Not enough exposure to sunlight, which produces
  vitamin D in the body
• Malabsorption of vitamin D by the intestines

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Other conditions that can cause Osteomalacia

• Hereditary or acquired disorders of vitamin D
  metabolism
• Kidney failure and acidosis
• Phosphate depletion associated with not enough
  phosphates in the diet
• Cancer
• Side effects of medications used to treat
  seizures (Dilantin)
• Liver disease

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Manifestations of Osteomalacia

• Localized bone pain
• Difficulty walking
• Low back pain
• Fractures are common, and delayed healing occurs
• Muscular weakness
• Weight loss
• Progressive deformities of the spine (kyphosis)

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Kyphosis
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Laboratory findings in Osteomalacia

• Decreased serum calcium levels
• Decreased serum phosphorus levels
• Elevated serum alkaline phosphatase
• Xrays show bone demineralization

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Collaborative Care of Osteomalacia

• Correction of the vitamin D deficiency
• Vitamin D3 and vitamin D2 can be supplemented
  (patient shows dramatic response)
• Calcium salts or phosphorus supplements may also
  be prescribed
• Encourage dietary ingestion of eggs, low-fat
  milk, fish and vegetables
• Exposure to sunlight is valuable
• Encourage weight bearing exercises

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Osteoporosis

• Porous bone (fragile bone disease)
• Low bone mass
• Structural deterioration of bone tissue
• Leads to increased bone fragility
• Known as the silent thief
• Slowly over many years robs the skeleton of its
  banked resources

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Osteoporosis
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Etiology and Pathophysiology

• Risk factors
• Female, increasing age, family hx of
  osteoporosis, white (European descent) or Asian,
  small stature, early menopause, hx of anorexia or
  oophorectomy, sedentary lifestyle and
  insufficient dietary calcium
• Cigarette smoking and alcoholism

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Gender Differences

• Men
• Underdiagnosed and undertested as compared to
  women
• One in eight men over the age of 50 will have an
  osteoporosis-related fracture in their lifetime

• Women
• Osteoporosis is 8 times more common in women than
  in men
• One in two women over the age of 50 will have an
  osteoporosis-related fracture in their lifetime

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Etiology and Pathophysiology (continued)

• Bone resorption exceeds bone deposition
  (normally resorption and deposition should be
  equal, this is known as remodeling)
• Osteoporosis most commonly occurs in the bones of
  the spine, hips, and wrists
• Over time wedging and fracture of the vertebrae
  can result in a loss of height and a humped back
  known as dowagers hump or kyphosis

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Specific diseases associated with osteoporosis

• Intestinal malabsorption
• Kidney disease
• Rheumatoid arthritis
• Hyperthyroidism
• Chronic alcoholism
• Cirrhosis of the liver
• Hypogonadism
• Diabetes mellitus

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Specific Medicaitons associated with osteoporosis

• Corticosteroids
• Anti-seizure medications (depakote, dilantin)
• Aluminum-containing antacids
• Heparin
• Certain cancer treatments
• Excessive thyroid hormones

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Clinical Manifestations

• Back pain or spontaneous fracture
• Fracture from minimal trauma
• Hip, vertebral or wrist fracture
• Collapsed vertebrae resulting in loss of height
  and kyphosis
• Spinal deformities
• Severely stooped posture

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Laboratory Assessment

• Serum calcium
• Vitamin D
• Phosphorus
• Alkaline Phosphatase levels
• Urinary Calcium levels
• Serum protein measurements
• Thyroid Function Tests
• uPYR Crosslinks Assay

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Radiographic Assessment

• X-ray of the spines and long bones
• Dual-energy x-ray absorptiometry (DEXA scan)
• Qualitative ultrasound of the heel or calcaneous

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Common Nursing Diagnoses

• Risk for falls
• Impaired physical mobility
• Acute pain or chronic pain

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Interventions

• Hormone Replacements
• Calcium supplements
• Vitamin D
• Biphosphonates
• Selective Estrogen Receptor Modulators
• Calcitonin
• Androgens

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Diet Therapy

• Calcium and Vitamin D need to be increased
• Select foods high in calcium (sardines, milk, ice
  cream, cottage cheese, yogurt)
• Alcohol and caffeine should be decreased

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Fall prevention

• Hazard free environment at home
• Hip protectors
• Fall risk programs in hospitals and long-term
  care facilities

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Exercise

• Isometric
• Resistive
• Weight bearing
• Range of motion

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Pain Management

• Analgesics (opioid and non-opioid)
• Muscle relaxants for spasms associated with
  fractures
• NSAIDS

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Orthotic Devices

• Dorsolumbar orthoses
• PT or orthotist custom fits the device
• Teach client to inspect skin for irritation and
  report tolerance of device

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Community-Based Care

• Most are managed at home
• Small percentage managed in hospital for care of
  fracture
• Assess for risk factors
• Provide health teaching
• Safe discharge
• May need long-term care for rehab or permanent
  residence if home support is lacking