Also found in: Dictionary, Thesaurus, Encyclopedia.
Pa·get dis·ease(paj'ĕt), [MIM*602080, MIM*167300]
Pa·get dis·ease(paj'ĕt di-zēz')
Synonym(s): osteitis deformans.
|Mean LOS:||5.6 days|
|Description:||MEDICAL: Bone Disease and Arthropathies With Major CC|
Paget disease, or osteitis deformans, is a slowly progressing condition of bone structure characterized by increased and disorganized bone turnover. It affects approximately 4% of the U.S. population older than 40. The bones affected vary, but those most commonly involved are the femur, tibia, lower spine, pelvis, and skull. Initially, there is an increase in the number of osteoclasts, which leads to excessive bone resorption and a compensatory increase in osteoblastic activity to repair bone matrix. As a result, the bone is enlarged and distorted, with areas of poor mineralization that resemble a mosaic pattern. Consequently, the bone cannot adequately withstand stresses and strains and is weaker.
Paget disease causes bones to fracture easily, often after only a minor trauma; these fractures heal slowly and often incompletely. If the spine is involved, the vertebrae may collapse, causing paraplegia. If the skull is involved, bony impingements on the cranial nerves can lead to blindness, hearing loss, tinnitus, or vertigo. Other complications of Paget disease include osteoarthritis, hypocalcemia, renal calculi, bone sarcoma, hypertension, and gout. The disease is most life-threatening when it is combined with congestive heart failure because of the need for increased cardiac output to supply increased blood flow to the bones.
The cause of Paget disease is not known. One theory suggests that it is the result of a slow viral infection, possibly mumps, with a long dormant period. A familial tendency has also been noted. Regional differences suggest that it may have an environmental cause.
Paget disease appears to have a hereditary component. Family studies have demonstrated that there are multiple predisposition genes, including TNFRSF11A, TNFRSF11B, and SQSTM1, with additional loci at 5q31, 6p21.3, and 18q23. However, families with affected members have been small, making genetic studies difficult.
Gender, ethnic/racial, and life span considerations
Paget disease occurs most frequently in people over age 40 and is more common in men than in women. Prevalence is difficult to determine because many people have no symptoms. Experts estimate that as many as 10% of people over age 80 have the disease.
Global health considerations
For unknown reasons, Paget disease occurs more often in the northern United States than in southern regions. It is also more common in the North America, Western Europe (except for Scandinavia), and Australia than other regions of the globe. Paget disease is more common in African Americans than in Africans, but whether that difference occurs because of genetic or environmental reasons is unknown.
Paget disease is frequently discovered as a result of x-rays taken during a routine physical examination. Ask if the patient has experienced deep bone pain. Establish a history of bone fractures. Ask if the patient’s hat size has changed or if he or she has experienced headaches. Elicit any history of decreased hearing or vision or of vertigo. Find out if the patient has noticed any changes in gait while walking or has experienced any difficulty breathing (possibly caused by kyphosis of the spine).
Although most people with Paget disease are asymptomatic, some present with bone pain. Assess the patient’s skeleton for deformities. Inspect the skull for enlargement, particularly in the frontal and occipital areas. Check for cranial nerve compression by testing for decreased hearing and vision, difficulty swallowing, and problems with balance. Examine legs and arms for bowing and subsequent deformities. Ask the patient to walk across the room and back to observe a characteristic waddling gait.
Observe for signs of kyphosis. Note if the patient is bent forward with the chin resting on the chest or has a barrel chest. Kyphosis can compromise chest excursion and cause dyspnea. Palpate the affected areas for increased warmth. Paget disease causes an increase in vascularity of the affected bones, causing the skin temperature to rise. Elderly patients or those with large, highly vascular lesions may develop congestive heart failure because of the heart’s attempt to pump more blood through bones with increased vascularity. Assess the patient’s apical pulse, respirations, and blood pressure. Note the presence of peripheral edema and neck vein distention from right-sided heart failure and pulmonary congestion.
Because the diagnosis of Paget disease is unexpected, the patient and family may experience increased anxiety levels. There may be fear related to possible falls with fractures and the possibility of self-care deficits. Social isolation may also occur as a result of increasing bone pain and deformities.
|Test||Normal Result||Abnormality With Condition||Explanation|
|X-rays||Normal structure of the skeletal system||Disordered and architecturally unsound||Disease causes increased osteoclastic bone resorption|
|Computed tomography||Normal structure of the skeletal system||Enlarged bones with trabecular (small interconnecting rods of bone making up mass of spongy bone) coarsening and increased cortical thickness||Disease causes increased osteoclastic bone resorption; often used to diagnose complications|
|Bone scans||Normal structure of the skeletal system||Identifies involved areas of the skeleton||Disease causes increased osteoclastic bone resorption|
Other Tests: Magnetic resonance imaging, bone biopsy, serum calcium, complete blood cell count, serum alkaline phosphatase, urinary N-telopeptide, α-C telopeptide
Primary nursing diagnosis
DiagnosisPain (acute) related to fractures and nerve compression
OutcomesComfort level; Pain control behavior; Pain level; Symptom control behavior; Symptom severity; Well-being
InterventionsPain management; Analgesic administration; Cutaneous stimulation; Heat/cold application; Touch; Exercise therapy; Progressive muscle relaxation
Planning and implementation
medical.Generally, patients with Paget disease who are asymptomatic require no specific treatment but need careful ongoing monitoring. Calcitonin and bisphosphonates are used to treat Paget disease. Mild pain is usually controlled successfully with NSAIDs. Nonpharmacologic treatment modalities are also prescribed for patients with Paget disease. Heat therapy and massage can help decrease pain. A physical therapy consultation can provide a protocol of simple strengthening and weight-bearing exercises. Braces and other ambulation aids may help support deformities and improve function.
surgical.Surgery may be needed to reduce or prevent pathological fractures, to correct secondary deformities, and to relieve neurological impairment. Unfortunately, joint replacement is difficult because methyl methacrylate (a gluelike bonding material) does not set properly on bone that is affected by Paget disease.
|Medication or Drug Class||Dosage||Description||Rationale|
|Alendronate (Fosamax)||40 mg/day for 6 mo; may also use etidronate, risedronate, pamidronate, tiludronate||Bisphosphonates; calcium regulator||Slows rate of bone turnover in pagetic lesions; lowers serum alkaline phosphatase; reduces elevated cardiac output by decreasing vascularity of bone, thereby averting high-output cardiac failure|
|Calcitonin||100 IU SC or IM qd initially and then 50 IU SC three times a week||Calcium regulator||Decreases the number and availability of osteoclasts, thereby retarding bone resorption; relieves bone pain; helps the remodeling of pagetoid bone into lamellar bone|
Focus on reducing pain and immobility, preventing injury, and educating the patient about the disease and treatment regimen. Correlate the patient’s pain with her or his activities and modify schedules as needed. Instruct the patient in the use of relaxation techniques, such as guided imagery and music therapy. Evaluate the patient’s level of functioning and encourage the patient to remain as active as possible. Perform range-of-motion exercises to joints, unless contraindicated, progressing from passive to active exercise as tolerated. Encourage the patient to perform self-care activities independently.
For patients on extended bedrest, reposition the patient frequently and use a flotation mattress. Prevent pressure ulcers by providing meticulous skin care.
Institute measures to prevent injury. Instruct the patient to move slowly and avoid sudden movements. Keep the environment free of clutter. Encourage the use of ambulation aids, such as walkers or canes, as needed. Carefully plan exercise protocols and activity regimens to minimize fatigue.
Evidence-Based Practice and Health Policy
Alonso, N., Albagha, O.M.E., Rios-Visconti, M., & Ralston, S.H. (2012). Identification of novel genetic markers that predict disease severity and complications in Paget disease of bone. Bone, 50, S52–S52. doi 10.1016/j.bone.2012.02.141
- A study among 771 patients with Paget disease of the bone found that disease severity score was significantly increased among patients with > 16 risk alleles compared to patients with ≤ 16 risk alleles (mean severity scores, 6.24 versus 5.57; p < 0.0001). In this sample, 33.6% carried > 16 risk alleles.
- Patients with > 16 risk alleles were also more likely than patients with ≤ 16 risk alleles to have a greater number of bones affected (p < 0.009), have received a greater number of treatments for Paget disease (p < 0.001), and have experienced deafness due to skull involvement (10% of patients with > 16 risk alleles versus 5.8% of patients with ≤ 16 risk alleles; p = 0.037).
- Physical findings: Kyphosis, mobility, gait, presence of skeletal deformities
- Description of physical pain, comfort measures used, and response to interventions
- Sensory disturbances: Vision loss, hearing loss
- Level of tolerance of activity
- Laboratory results: Serum calcium and alkaline phosphatase levels
- Reaction to changes in body image
Discharge and home healthcare guidelines
Encourage the patient to follow a recommended moderate exercise program. Suggest the use of a firm mattress or bed board to minimize spinal deformities. Emphasize the importance of attending physical therapy sessions and routine follow-up visits with the physician. Stress the importance of assessing the home environment for safety. Teach the patient to maintain adequate lighting, remove scatter rugs, and keep the home uncluttered. Instruct the patient to avoid abrupt movements and to report any increases in bone pain. Teach the patient to use assistive devices for ambulation.
Be sure the patient understands the dosage, route, action, and side effects of all prescribed medications. Instruct the patient in proper self-injection techniques for calcitonin. Educate the patient about side effects, such as nausea, vomiting, itchy hands, fever, inflammation of the injection site, and facial flushing, as well as the signs and symptoms of hypercalcemia. Teach the patient to take ethane hydroxy diphosphate medication with fruit juice on an empty stomach at least 2 hours before meals. Tell the patient to call the physician if he or she experiences stomach cramps, diarrhea, or new bone pain. Educate the patient who is taking plicamycin about the signs and symptoms of infection. Tell the patient to report any easy bruising, bleeding, nausea, or anorexia. Provide information about the Paget Foundation (165 Cadman Plaza East, Brooklyn, NY 11201).
Paget,Sir James, English surgeon, 1814-1899.
Pa·get dis·ease(paj'ĕt di-zēz') [MIM*602080, 167300]
Synonym(s): osteitis deformans.