PAGET'S DISEASE

Paget disease is a chronic bone disorder that typically results in enlarged, deformed bones due to excessive breakdown and formation of bone tissue that can cause bones to weaken and may result in bone pain, arthritis deformities or fractures.

1. METABOLIC BONE DISORDER (PAGET’S DISEASE) Prepare by: Arpana Bhusal BNS

2. INTRODUCTION The disease is named for the great English surgeon and pathologist, Sir James Paget (1814-1899). Paget’s disease of bone was first described by the English surgeon, Sir James Paget, in 1876. Recent research indicates linkage of the disease in some families to genes on chromosomes 6 and 18, although other genes may also be involved (Van Hul , 1999) Based on these observations, it is thought that the disease occurs as a consequence of a viral infection in genetically predisposed individuals. Sir James Paget

3. DEFINITION Paget disease is a chronic bone disorder that typically results in enlarged, deformed bones due to excessive breakdown and formation of bone tissue that can cause bones to weaken and may result in bone pain, arthritis deformities or fractures. Paget's disease of bone is a condition that affects the way your bone breaks down and rebuilds (metabolizes). Healthy bone metabolism allows for old bone to be recycled into new bone throughout the course of your life. In Paget's disease of bone, the rate at which old bone is broken down and new bone is formed becomes distorted. Over time, the affected bones may become fragile and misshapen

4. AETIOLOGY The cause of Paget’s disease is unknown. Up to 15% of affected individuals report a family history in a first-degree relative, suggesting that genetic factors are important in pathogenesis. Genetics infectious viral cause Calcium & Vit . D Deficiency

5. RISK FACTORS There are no known risk factors for Paget’s disease. Older than 40 year Men more than Female Close relative AGE SEX FAMILY HISTORY Paget’s disease

6. BONE INVOLVED IN PAGET’S DISEASE Most common bones in Paget’s disease are: The pelvis Lumbar spine Femur Thoracic spine Sacrum Skull Tibia and Humerus Internal structure of bone, chest Lower extremity Hands and wrists Feet

7. PATHOGENESIS Age, Sex, Family History Infectious Virus, Genetics & Environment   Proliferation of Osteoclast Lead to Osteoclastosis activities than normal As a result Bone resorption then held Compensatory mechanism cause Osteoblastic activities Lead to Bone formation As a bone turnover continues rapidity of new bone formation develop Classis mosaic pattern of bone( in size, structurally weaker, change in shape cause Pathological Fracture, Structural Bowing of the leg Lead to Malalignment of the hip, knee & ankle joint As a result Arthritis, Back & Joint pain Arthritis, Back & Joint pain

8. CLINICAL FEATURES Most common symptom is pain:- Bone pain Pain may be worse at night Headache Arm pain(bilateral) Leg pain(bilateral) Neck pain Joint Pain Shoulder pain(bilateral) Elbow pain Hip pain(bilateral) Knee pain(bilateral) Ankle pain Back pain Low Back pain

9. CLINICAL FEATURES Contd …………… Bowed legs Bone tenderness Loss of height Fatigue Headaches and hearing loss may occur when an overgrowth of bone in the skull. Tingling and numbness in an arm or leg If spine is affected, nerve roots can become compressed. Waddling Gait Hip pain Arthritis- Damage to cartilage of joints

10. DIAGNOSIS History Physical Examination Physical findings in someone with Paget's disease may include: Bowing of long bones Decreased motion Difficulty in walking Enlarged skull Kyphosis of the spine Loss of hearing Muscle weakness

11. DIAGNOSIS Contd ……… Radiology Bone X-Ray- show areas of bone reabsorption, enlargement of the bone and deformities, bowing of your long bones. Bone Mass Density Laboratory test Blood - Alkaline phosphatase - Elevated Urine- Hydroxyproline - Increased Bone Biopsy

12. MANAGEMENT General measures give information and education about it. Treat the patient with the multidisciplinary team i.e surgeon, physiotherapist and occupational therapist. Patients with lower limb deformities may develop secondary foot problems and should be referred for podiatric assessment. Advice about suitable shoe-wear or the provision of simple foot orthosis which can often result in reduced pain and improved mobility.

13. MANAGEMENT Contd ………… General measures Encourage patient with lower limb for the degree of activity and rest that they can undertake, including the importance of pacing and planning activities. They should receive counseling about lifestyle measures to reduce risk of falls. patients should have audiometry and provision of a hearing aid if necessary. Cane or Walker

14. PHARMACOLOGICAL MANAGEMENT The goal of drug treatment is to control Paget's disease activity for as long a period of time as possible. If you don't have symptoms, you may not need treatment. NSAIDs for pain- Naproxen, Ibubrufen , Ketoprofen Calcium supplement Vitamin D supplement Biphosphonates - to stabilize rapid bone turnover

15. SURGICAL PROCEDURE The purpose of surgery to Help fractures heal Replace joints damaged by severe arthritis Realign deformed bones Reduce pressure on nerves Surgical procedure Total Hip replacement Spine surgery to correct spinal cord compression

16. COMPLICATIONS Fractures: Osteoarthritis: Heart failure: Bone cancer: Loss of Vision Paraplegia Spinal stenosis Deafness

17. WARNING SIGN OF PAGET’S DISEASE Notify the doctor if you have Paget's disease and any of the following: Difficulty in walking Inability to take prescribed medications Kyphosis Abnormal curvature to the upper spine Loss of height Joint pain and Joint swelling Worsening back pain, hip pain and joint pain

18. NURSING CONSIDERATION Nursing Assessment Asses the pain and function ability of the affected part. Observe for bowing leg or waddling gait. Assess for cardiovascular complications. Assess for auditory symptoms- tinnitus, vertigo & hearing loss. Nursing Diagnosis Pain R/T pathophysiologic process. Risk for injury R/T fall , fragile and dense bone structure bow, weak leg. Nursing Goal Patient’s pain will be reduced as evidenced by providing facial expression,verbalization .

19. NURSING CONSIDERATION Contd …………. Nursing Planning & Action Reducing pain - administer and teach self administration of analgesics. -position the patient as ordered or frequently if there is no restriction. Dietary Therapy - Encourage patient to take more calcium diet. - Ask patient to have regular Vitamin D.

20. NURSING CONSIDERATION Contd …………. Preventing injury Establish exercise protocols through a physiotherapist to maintain physical abilities and prevent from fall. Assist the patient with activities as necessary. Provide heel lift, walking aids as needed. Surgery Intervention Do perioperative care to the patient as needed.

21. HOME BASED CARE Ask Family to remove slippery floor coverings, use nonskid mats in your bathtub or shower, tuck away cords, and install handrails on stairways and grab bars in your bathroom, use a cane or a walker to prevent from fall. Maintain good lighting to prevent from fall injury. Provide education about the disease process and medication. Educate the patient about the use of mobility aids. Ask patient to give rest to the painful joint with use of walker, crutches and cane .

22. HOME BASED CARE Contd …….. Follow an exercise plan developed with your doctor. Perform gentle stretching after exercise. Encourage patient to loose weight if she/he is overweight. Apply warm compresses for stiffness for 20-30 minutes, every 1-2 hours. Use splints when the joints are swollen .

23. PREVENTION PAGET’S DISEASE Avoid Smoking Avoid Alcohol Calcium & Vitamin D Diet Avoid prolong standing Avoid Smoke

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