Paget's (PAJ-its) disease of bone interferes with your body's normal recycling process, in which new bone tissue gradually replaces old bone tissue. Over time, bones can become fragile and misshapen. The pelvis, skull, spine and legs are most commonly affected.
2. Introduction :-
Paget's disease is a chronic bone condition characterized by disorder of
the normal bone remodeling process.
Normal bone has a synchronized relationship of mechanisms that act to
lay down new bone and take up old bone.
This relationship (referred to as remodeling) is essential for maintaining
the normal calcium levels in our blood.
In bone affected by Paget's disease, the bone remodeling is disturbed
and not synchronized.
As a result, the bone that is formed is abnormal, enlarged, not as dense,
brittle, and prone to breakage (fracture).
3. Paget's disease affects older bone of adults. It's estimated that 1% of
adults in the U.S. have Paget's disease.
There is an extremely rare form of Paget's disease in children,
referred to as juvenile Paget's disease.
Paget's disease is also known as osteitis deformans.
4. Definition
Paget’s disease of the bone is a skeletal disorder resulting from
excessive orthoclastic activity, affecting long bones, pelvis, lumbar
vertebrae & skull.
Paget's disease of bone is a chronic disease of the skeleton.
In healthy bone, a process called remodeling removes old pieces of
bone and replaces them with new, fresh bone.
Paget's disease causes this process to shift out of balance, resulting in
new bone that is abnormally shaped, weak, and brittle
5. 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.
6. Causes
It is not known what causes Paget's disease.
Recently, certain genes have been associated with Paget's disease,
including the Sequestrosome 1 gene on chromosome 5.
Virus infection may be necessary to trigger Paget's disease in people
who have inherited the genetic tendency to develop the condition.
7. Causes ……………. Cont
The cause of Paget's disease of bone is unknown.
Scientists suspect a combination of environmental and genetic factors
contribute to the disease.
Several genes appear to be linked to getting the disease.
Some scientists believe Paget's disease of bone is related to a viral
infection in bone cells, but this theory is controversial.
8. Risk Factors
Factors that can increase the risk of Paget's disease of bone include:
Age. People older than 40 are most likely to develop Paget's disease
of bone.
Sex. Men are more commonly affected than are women.
National origin. Paget's disease of bone is more common in England,
Scotland, central Europe and Greece — as well as countries settled by
European immigrants.
It's uncommon in Asia.
Family history.
9. Symptoms
Bone pain, deformity, fracture, and arthritis.
The most common bones affected by Paget's disease includes:
Spine
Thigh bone (femur)
Pelvis
Skull
Collar bone (clavicle)
Upper arm bone (humerus)
The symptoms of Paget's disease depend on the bones affected and
the severity of the disease.
10. Enlarged bones can pinch adjacent nerves, causing tingling and
numbness.
Bowing of the legs can occur.
Hip or knee involvement can lead to limping, as well as pain and
stiffness of the hip or knee.
Headache, loss of vision, and hearing loss can occur when bones of
the skull are affected.
With very widespread Paget's disease, it is possible to develop
congestive heart failure due to an increased workload on the heart.
11. Symptoms
Bone pain, deformities and fractures.
Signs and symptoms, will depend on the affected part of body.
12. Pelvis. Paget's disease of bone in the pelvis can cause hip pain.
Skull. An overgrowth of bone in the skull can cause hearing loss or
headaches.
Spine. If spine is affected, nerve roots can become compressed. This
can cause pain, tingling and numbness in an arm or leg.
Leg. As the bones weaken, they may bend — causing to become
bowlegged.
Enlarged and misshapen bones in legs can put extra stress on nearby
joints, which may cause osteoarthritis in knee or hip.
13. Assessment & Diagnostic Findings
History and a physical examination.
x-rays.
Bone scans.
Bone biopsy.
Blood and urine tests.
Methods used to diagnose Paget’s disease Three tests – an x-ray,
blood test, and bone scan – are commonly used to discover Paget’s
disease.
14. Blood test (measurement of serum alkaline phosphatase).
When blood contains a higher-than-usual level of a
chemical substance called serum alkaline phosphatase
(SAP), it is a sign that the disease may be present.
15. SAP is a type of enzyme made by bone cells that is overproduced by
pagetic bone.
A mild increase in SAP, up to twice the usual level, may indicate
Paget’s disease or another condition, such as liver disease or a bone
fracture that is in the process of healing.
However, a SAP level greater than twice the usual level strongly
suggests Paget’s disease, especially if the person’s serum calcium
level, phosphorus level, and kidney function are normal.
Bone scan. A bone scan is a test that helps identify which bones have
been affected by Paget’s disease.
16. Management
Medicine
Calcitonin If severe pain and bone loss prescribed a medication called
calcitonin, which is given by injection.
Calcitonin is a hormone that occurs naturally within the body. It helps
increase bone density by affecting the levels of calcium in the blood.
It reduces bone destruction and reduces the formation of new bone
as well. It can also relieve pain.
17. Acetaminophen For mild to moderate pain from Paget’s disease
Acetaminophen is a pain reliever, but has no anti-inflammatory
properties, so it does not actually reduce the swelling and pressure
that may be causing the pain.
Because it is not an anti- inflammatory it can usually be safely taken
along with most prescription medications.
However, there are daily limits of acetaminophen that can be taken,
so caution should be exercised, particularly if other medications that
contain acetaminophen (for example, it’s found in many cold
remedies) are being used.
A serious overdose of acetaminophen can cause liver damage.
18. Diet
Proper intake of calcium.
Advise to drink about two liters (six to eight glasses) of fluid daily to
prevent other complications arising from excess calcium in your body.
Calcium can be obtained by eating a well-balanced diet that includes
foods that are good sources of calcium – for example, milk and milk
products, dark-green leafy vegetables (such as mustard greens and
kale), and canned fish with soft bones (such as sardines and salmon).
Dietary supplements of calcium may be another source.
19. In general, a patient with Paget's disease should receive 1000-1500
mg of calcium, adequate sunshine, and at least 400 units of vitamin D
daily.
This is especially important if treated with bisphosphonates.
If history of kidney stones (calcium deposits in the kidneys) discuss
calcium and vitamin D intake with physician.
Exercise may relieve stiffness and help maintain flexibility.
Always consult doctor before beginning an exercise program.
Depending on the severity of symptoms doctor may advise against
certain exercises. He or she might refer to a therapist, who can show
the exercises that may be helpful and those that could be harmful.
20. Heat/Cold: Heat or cold application can provide temporary relief of
pain.
Heat helps to reduce pain and stiffness by relaxing aching muscles
and increasing circulation to the area.
There is some concern that heat may worsen the symptoms in an
already inflamed joint. Cold helps numb the area by constricting the
blood vessels and blocking nerve impulses in the joint.
Applying ice or cold packs appears to decrease inflammation and
therefore is the method of choice when joints are inflamed.
Protect Joints: Protecting joints means using them in ways that avoid
excess stress. Benefits include less pain and greater ease in doing
tasks.
21. 1. For example, use a shoulder bag instead of a hand-held one. Also,
avoid keeping the same position for a long period of time.
22. Using helpful devices, such as canes, luggage carts, grocery carts and
reaching aids, can help make daily tasks easier.
Small appliances such as microwaves, food processors and bread
makers can be useful in the kitchen.
Using grab bars and shower seats in the bathroom can help to
conserve energy and avoid falls.
Staying at recommended weight can lessen pain by reducing stress on
the bones. If you plan to lose weight, discuss the best program for
you with your doctor and a dietician.
Relaxation Developing good relaxation and coping skills can give you
a greater feeling of control over your arthritis and a more positive
outlook.
23. Surgery Some people with severe, advanced Paget’s disease may
require surgery, though this is rare.
Benefits include less pain and better movement and function.
Some kinds of surgery can repair bone deformity or rebuild part of a
joint.
24. Nursing Process
Assessment
Assess pain & functional abilities.
Observe for bowing (legs) or complaint that has feel tight.
Assess for cardiovascular complication.
Assess for auditory symptom- tinnitus, vertigo & hearing loss.
25. Nursing Diagnosis
Chronic pain related to pathophysiological process.
Goal :- Reduce pain.
Intervention
Advice patient to take rest in bed by giving side lying position or supine
position.
Advice patient to sleep in firm mattress.
Provide local heat compression & back massage.
Instruct the patient to move the trunk not to twist & giving good posture &
teaches body mechanics.
Evaluation :- pain is reduced.
26. Risk for injury related to fall.
Goal :- Prevent injury.
Intervention
Teach safe transferring, & make sure patient can alert & call nurses for help.
Assist patient with activities as necessary.
Provide function & mobility aids such as heel lifts, walking aids as needed.
Evaluation :- Risk for injury is reduced.
27. Deficient knowledge about the diseased condition &
treatment regimen.
Goal :- Provide knowledge to patient.
Intervention
Taught the patient about disease condition like development of paget’s
disease, intervention to arrest or slow the process & measure to relieve
symptom
Advise to patient regarding dietry or supplemental intake of calcium & vit. D,
regular weight bearing exercise, & modification of life style.
Patient teaching related to medication therapy its important & side-effect.
Teach patient to drink adequate fluids.
Evaluation :- Acquires knowledge about osteoporosis & treatment
regimen
29. Gout
Gout is caused by deposition of uric acid crystals in the joint, causing
inflammation.
There is also an uncommon form of gouty arthritis caused by the
formation of rhomboid crystals of calcium pyrophosphate known as
pseudogout.
In the early stages, the gouty arthritis usually occur in one joint, but
with time, it can occur in many joints and be quite crippling.
The joints in gout can often become swollen and lose function.
30. Other Infectious arthritis is another severe form of arthritis.
It presents with sudden onset of chills, fever and joint pain.
The condition is caused by bacteria elsewhere in the body.
Infectious arthritis must be rapidly diagnosed and treated promptly to
prevent irreversible and permanent joint damage.
Psoriasis is another type of arthritis. With psoriasis, most individuals
develop the skin problem first and then the arthritis.
31. The typical features are of continuous joint pains, stiffness and
swelling.
The disease does recur with periods of remission but there is no cure
for the disorder.
A small percentage develop a severe painful and destructive form of
arthritis which destroys the small joints in the hands and can lead to
permanent disability and loss of hand function.
32. Signs and Symptoms
Regardless of the type of arthritis, the common symptoms for all
arthritis disorders include varied levels of pain, swelling, joint stiffness
and sometimes a constant ache around the joint(s).
Inability to use the hand or walk
Malaise and a feeling of tiredness
Fever
Weight loss
Poor sleep
Muscle aches and pains
Tenderness
33. Difficulty moving the joint It is common in advanced arthritis for
significant secondary changes to occur.
For example, in someone who has limited their physical activity:
Muscle weakness.
Loss of flexibility.
Decreased aerobic fitness.
34. Assessment & Diagnostic Findings
History and a physical examination.
x-rays,
Bone scans
Bone biopsy.
Blood and urine tests.
Medications:-
NSAIDS(nonsteroidal anti-inflammatory drugs).
COX-2 Inhibitors.
Pain Medication (analgesics)
Corticosteroids
DMARDs (disease-modifying anti-rheumatic drugs)
35. Treatment :-
physical therapy, splinting, cold-pack application, paraffin wax dips,
anti-inflammatory medications, immune-altering medications, and
surgical operations.
Treatment Goals:- The goals of arthritis treatment are to:
Decrease arthritis symptoms.
Slow progression of the disease.
Prevent or minimize joint damage and deformities.
Maintain joint function.
36. Assessment:-
Assess pain & functional abilities.
Observe for bowing (legs) or complaint that has feel tight.
Assess for cardiovascular complication.
Assess for auditory symptom- tinnitus, vertigo & hearing loss.
37. Nursing diagnosis:-
Chronic pain related to pathophysiological process.
Goal :- Reduce pain.
Intervention
Advice patient to take rest in bed by giving side lying position or
supine position.
Advice patient to sleep in firm mattress.
Provide local heat compression & back massage.
Instruct the patient to move the trunk not to twist & giving good
posture & teaches body mechanics.
Evaluation :- pain is reduced.
38. Impaired physical mobility related to pain, use of immobilization
device & weight bearing limitation.
Goal :- improved physical mobility within therapeutic limitation.
Intervention
Treatment regimen restrict activity.
Encourage to take rest.
Encourage the patient to participate in activity of daily living with
in physical limitation.
Demonstrate safe use of immobilizing & assistive devices. The
bone is weakened by the infective process & must be procted by
immobilization devices & by avoidance of stress on the bone.
Evaluation :- Physical mobility is improved.
39. Deficient knowledge about the diseased condition
& treatment regimen
Goal :- To provide knowledge to patient.
Intervention
Taught the patient about disease condition like development of paget’s
disease, intervention to arrest or slow the process & measure to relieve
symptom
Advise to patient regarding dietary or supplemental intake of calcium & vit. D,
regular weight bearing exercise, & modification of life style.
Patient teaching related to medication therapy its important & side-effect.
Teach patient to drink adequate fluids.
Evaluation :- Acquires knowledge about treatment regimen.
40.
41. Reference
• Brunner, L. S., & Suddarth, D. S. (2004). Text book of Medical-
Surgical nursing (10th e.d). Phi;ladephia: Lippincott.