Question

Paget's- Diagnosis, pathophysiology related to client problem, health promotion and disease prevention, risk factors, expected findings, Lab tests, diagnostic procedures, nursing care, medications , client education, safety considerations and complications.

Answer 1

#. Pagets disease :-

Chronic metabolic bone disorder that causes an irregular bone formation; with less compact new bone that is more vascular and more fragile

Affects one or several skeletal areas (spine, pelvis, femur, and skull)

Slow and progressive

Causes malignant bone changes in about 5% of patients

Can be fatal, particularly when associated with heart failure, bone sarcoma, or giant cell tumors

Also known as osteitis deformans

#. Pathophysiology :-

In the initial (lytic) phase, excessive bone resorption occurs faster than normal.

The second (mixed) phase involves increased bone formation (with abnormalities in the new bone) to keep up with the rapid bone breakdown.

The third (sclerotic) phase predominantly involves bone formation that's disorganized and weaker than normal bone, allowing excess fibrous connective tissue and blood vessels to infiltrate and causing the bone to become hypervascular.

#. Causes :-

Exact cause unknown; possible familial and genetic links

Thought to be caused by a slow or dormant viral infection (possibly mumps)

#. Incidence :-

Paget's disease typically occurs in persons after age 50, being most often diagnosed in those in their 60s.

For every two males affected, one female is affected.

#. Complications :-

Fractures

Head enlargement

Blindness and hearing loss with tinnitus and vertigo; deafness

Osteoarthritis

Sarcomatous degeneration

Sarcoma

Hypertension

Renal calculi

Hypercalcemia

Gout

Heart failure

Carpal or tarsal tunnel syndromes

Spinal stenosis

Stroke

Vascular steal syndrome

#. Assessment-History :-

Possibly asymptomatic

Severe, persistent pain

Impaired mobility

Pain that worsens with weight bearing

Increased hat size

Headaches

Gradual loss of hearing and vision

#. Physical Findings :-

Cranial enlargement over frontal and occipital areas

Facial deformities, such as loss of facial muscle strength, leading to facial drooping; changes in structure and composition of teeth

Kyphosis

Barrel-shaped chest

Asymmetrical bowing of the tibia and femur

Warmth and tenderness over affected sites

Peripheral neuropathy

Acetabular protrusion

Left ventricular hypertrophy

Aortic stenosis (from calcification)

#. Diagnostic Test Results-Laboratory :-

Serum calcium and phosphate levels are usually normal.

Serum gamma-glutamyl transpeptidase level is normal.

Serum osteocalcin level is usually increased.

Serum alkaline phosphatase level is elevated.

Levels of urinary pyridinoline collagen crosslinks are increased.

Serum uric acid levels may be increased (more commonly found in men).

Urine hydroxyproline levels are elevated.

#. Diagnostic Test Results-Imaging :-

X-ray studies show bone expansion and increased bone density.

Bone scans clearly show early pagetic lesions.

Computed tomography and magnetic resonance imaging show extra bony extension when sarcomatous degeneration is present.

#. Diagnostic Test Results-Diagnostic Procedures :-

Bone biopsy shows a characteristic mosaic pattern of bone tissue (done only in rare cases when other tests aren't definitive).

#. Treatment :-

Oral alendronate, a bisphosphonate, is the first line treatment for Paget disease. 1500 mg of calcium is given as a supplement to reduce the risk for hypocalcemia. When oral drugs are not effective, pamidronate and zoledronic acid are administered intravenously.

NSAIDS

Joint Replacement

#. Nursing interventions :-

- Ensure patient education about condition and means of preventing deterioration .

- Adequate dietary and calcium / vitamin D supplements

- Cessation of smoking and alcohol intake

- Exercise regularly

- Weight control

- Psychological and emotional support

- Careful gentle handling

- Adequate pain relief

- Monitor hearing ability