2. 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
3. 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
4. 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
HISTOR
Y
Paget’s disease
5. 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
6. 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
7. CLINICAL FEATURES
Most common symptom is pain:-
o Bone pain
◦ Pain may be worse at night
◦ Headache
◦ Arm pain(bilateral)
◦ Leg pain(bilateral)
◦ Neck pain
o Joint Pain
◦ Shoulder pain(bilateral)
◦ Elbow pain
◦ Hip pain(bilateral)
◦ Knee pain(bilateral)
◦ Ankle pain
o Back pain
◦ Low Back pain
8. 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
9. 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
10. 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
11. 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.
12. 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
13. 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: (1000-1500 mg/day)
Vitamin D supplement: (400 units/day)
Biphosphonates- to stabilize rapid bone turnover
14. SURGICAL PROCEDURE
The purposes 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. 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
17. 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.
18. NURSING CONSIDERATION Contd………….
Nursing 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.
19. 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.
• Encourage Client to Stay active – exercise helps to
maintain bone health and joint mobility, as well as
strengthen muscles.
• Aggressive physical activity is not recommended, as
the risk of fracture is high.
20. 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.
21. 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.