Adult Medical-Surgical Adult Medical-Surgical NursingNursing

Musculo-skeletal Module: Musculo-skeletal Module:

Pagets DiseasePagets Disease

Bone FormationBone Formation

Bone is a dynamic tissue constantly Bone is a dynamic tissue constantly reforming and resorbingreforming and resorbing

Bone cells (osteocytes) are:Bone cells (osteocytes) are:

Osteoblasts (bone formation)Osteoblasts (bone formation)

Osteoclasts (bone destruction, Osteoclasts (bone destruction, resorption, remolding)resorption, remolding)

Bone FormationBone Formation

Osteoblasts secrete collagen and Osteoblasts secrete collagen and glycoproteins to form a bone matrixglycoproteins to form a bone matrix

Deposit minerals in the matrix Deposit minerals in the matrix (calcium, magnesium, phosphorus, (calcium, magnesium, phosphorus, chloride)chloride)

Osteoclasts resorb calcium and Osteoclasts resorb calcium and minerals from bone causing depletion minerals from bone causing depletion

Hormones Affecting Bone TurnoverHormones Affecting Bone Turnover

Hormones which promote bone Hormones which promote bone formationformation: (inhibit resorption): (inhibit resorption)

Oestrogen and calcitonin Oestrogen and calcitonin Testosterone in males (↓ with age) Testosterone in males (↓ with age)

Hormone which increases bone turnover Hormone which increases bone turnover and and resorptionresorption::

Parathormone (↑ with age)Parathormone (↑ with age) (Also insufficient oestrogen/ calcitonin/ (Also insufficient oestrogen/ calcitonin/

testosterone)testosterone)

Factors Influencing Bone Factors Influencing Bone FormationFormation

Local stress: usage/ weight-bearing Local stress: usage/ weight-bearing and walking aid bone formation and walking aid bone formation

(Immobility leads to increased calcium (Immobility leads to increased calcium resorption from bone and resorption from bone and osteoporosis)osteoporosis)

Adequate calcium and vitamin D Adequate calcium and vitamin D (intestinal absorption of calcium)(intestinal absorption of calcium)

Parathormone/ Calcitonin activityParathormone/ Calcitonin activity Blood supply (inadequate → necrosis)Blood supply (inadequate → necrosis)

Bone PathologyBone Pathology

Bone Pathology: Classification Bone Pathology: Classification

OsteoporosisOsteoporosis OsteomalaciaOsteomalacia Pagets DiseasePagets Disease

Bone tumour:Bone tumour: BenignBenign Malignant primaryMalignant primary MetastasesMetastases

Pagets DiseasePagets Disease

Pagets Disease: DescriptionPagets Disease: Description

Pagets disease (osteitis deformans) is a Pagets disease (osteitis deformans) is a condition of localised rapid bone condition of localised rapid bone turnoverturnover

Affects the skull, femur, tibia, pelvic Affects the skull, femur, tibia, pelvic bones, vertebraebones, vertebrae

Affects 2-3% of population > 50 yearsAffects 2-3% of population > 50 years Affects males more than femalesAffects males more than females Causes deformity and risk of Causes deformity and risk of

pathological fracture pathological fracture

Pagets Disease: AetiologyPagets Disease: Aetiology

Cause unknownCause unknown

Family historyFamily history

Increased risk with ageIncreased risk with age

Pagets Disease: PathophysiologyPagets Disease: Pathophysiology

Localised rapid bone turnover:Localised rapid bone turnover: Primary proliferation of osteoclasts Primary proliferation of osteoclasts

(bone resorption) (bone resorption) Compensatory ↑ osteoblastic activityCompensatory ↑ osteoblastic activity Leads to a disorganised pattern of Leads to a disorganised pattern of

bone in the areas affected (mosaic):bone in the areas affected (mosaic): Highly vascular, structurally weak Highly vascular, structurally weak

areasareas

Pagets Disease: OutcomePagets Disease: Outcome

Bones are prone to:Bones are prone to:

Deformity Deformity

Pathological fracturePathological fracture

Pagets Disease: Pagets Disease: Clinical ManifestationsClinical Manifestations

Insidious onset Insidious onset

May only show on a routine XrayMay only show on a routine Xray

Pagets Disease: Pagets Disease: Clinical ManifestationsClinical Manifestations

Skeletal deformity: Skeletal deformity: Bowing of tibia and fibula, deformed pelvic Bowing of tibia and fibula, deformed pelvic

bones, cortical thickening long bonesbones, cortical thickening long bones Ape-like postureApe-like posture Skull enlargement: face appears small, Skull enlargement: face appears small,

triangular, hearing affected (cranial nerve)triangular, hearing affected (cranial nerve) Pain in back, joints, arthritis, warmth Pain in back, joints, arthritis, warmth

tenderness over bones (↑vascularity)tenderness over bones (↑vascularity)

Pagets Disease: DiagnosisPagets Disease: Diagnosis

Xrays confirmXrays confirm Bone scansBone scans Bone biopsyBone biopsy Serum alkaline phosphatase (↑)Serum alkaline phosphatase (↑) ↑ ↑ urinary hydroxyproline (↑ urinary hydroxyproline (↑

osteoblastic activity)osteoblastic activity) Serum calcium is normalSerum calcium is normal

Pagets Disease: Pagets Disease: Medical ManagementMedical Management

Control weightControl weight Adequate dietary calcium and vitamin D Adequate dietary calcium and vitamin D

and supplementsand supplements Non-steroidal anti-inflammatory drugs Non-steroidal anti-inflammatory drugs

(NSAIDs)(NSAIDs) Drugs to reduce bone resorption/ Drugs to reduce bone resorption/

turnover:turnover: Calcitonin, FosamaxCalcitonin, Fosamax Cytotoxic antibiotic (Mithracin) if severeCytotoxic antibiotic (Mithracin) if severe

Pagets Disease:Pagets Disease:Management of ComplicationsManagement of Complications

May require hearing aidMay require hearing aid Hip replacement surgeryHip replacement surgery Fracture care: closed or open Fracture care: closed or open

reduction and internal fixationreduction and internal fixation

Regular follow-up examination for Regular follow-up examination for early detection treatment early detection treatment

Pagets Disease: Pagets Disease: Nursing ConsiderationsNursing Considerations

Ensure patient education about Ensure patient education about condition and means of preventing condition and means of preventing deteriorationdeterioration

Adequate dietary and calcium/ Adequate dietary and calcium/ vitamin D supplements (note levels) vitamin D supplements (note levels)

Weight controlWeight control Psychological/ emotional supportPsychological/ emotional support Careful gentle handling and ensure Careful gentle handling and ensure

adequate pain relief. Monitor hearing adequate pain relief. Monitor hearing