Pagets disease (Imran Khan Salarzai)

KHYBER MEDICAL UNIVERSITY
Subject: AHN II
Disease: Paget’s Disease of bone
Presented by: ImranKhanSalarzai
Class: BScN year II semester IV
Roll no: 39
24/11/2015 IMRAN KHAN SALRZAI 1

OBJECTIVES
At the end of my presentation the students will be able:
• To define Paget's disease of bone.
• To enlist the signs and symptoms of Paget's disease.
• To discus the causes of Paget's disease.
• To discus pathophysiology of the paget’s disease.
• To know about the risk factor of Paget's disease.
• To know about the complication of Paget's disease.

OBJECTIVES
• To know about the diagnosis and investigation of
Paget's disease.
• To know about the pharmacological, non
pharmacological and surgical management of Paget's
disease.
• To know about the epidemiology of the disease.
• To formulate nursing diagnosis and nursing
intervention of the disease.

WHAT IS PAGET'S DISEASE?
 Paget's disease of bone was first described by Sir
James Paget in 1877.
 In Paget's disease, the bone-making process (bone
turnover) becomes faster and out of control. Affected
areas of bone form new bone material in an abnormal
way. The newly formed bone is thicker than normal and
the bone may become wider.

CONT….
 However, the bone is not made properly and it is
weaker than normal bone. This can cause deformity of
the affected bone, particularly in weight-bearing bones
such as the leg bones, which may bend. Affected bone is
also more likely to break (fracture).
Principally affected areas: pelvis, tibia,
lumber spine, skull and scapula.

SIGNS AND SYMPTOMS
 Most people who have Paget's disease of bone
experience no symptoms. The first manifestation of
parget's disease is elevated level of alkaline phosphatase
(an enzyme produce by the liver, bile duct, and some
bone disease) in the blood and the second most common
complaint is bone pain. Other signs and symptoms are
given below.
 Pelvis. Paget's disease of bone in the pelvis can cause
hip pain.

SIGN AND SYMPTOMS
 Skull. An overgrowth of bone in the skull can cause hearing loss or
headaches.
 Spine. If your 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 you to become
bowlegged. Enlarged and misshapen bones in your legs can put extra
stress on nearby joints, which may cause wear-and-tear arthritis in your
knee or hip.
 Warmth at the site of affected area.

CAUSES OF PAGET'S
 The cause of Paget's disease is unknown. It may be due to genetic
factors or a viral infection early in life.
Viral:
 Paget's disease may be caused by a slow virus infection (i.e.,
paramyxoviridae) present for many years before symptoms appear.
Associated viral infections include respiratory syncytial virus and the
measles virus. However, recent studies show that measles virus have
close association with paget disease.

CAUSES
 Noriyoshi Kurihara, Ph.D., of the University of Pittsburgh, and a
team of investigators led by David Rodman, M.D., Ph.D., used genetic
engineering techniques to create a strain of mice in which the measles
viral protein is produced in osteoclasts. These transgenic mice
developed osteoclasts that were larger and formed more readily, as in
people with Paget's disease. Additionally, the mice acquired skeletal
malformations in specific sites that resembled pagetic bone. Also of
interest is the fact that pagetic changes were only observed in the oldest
mice; in humans, Paget's disease is more common in older age groups

CAUSES
Genetic:
 There is a hereditary factor in the development of
Paget's disease of bone. Two genes, SQSTM1(
Sequestosome 1)and RANK, and specific regions of
chromosome 5 and 6 are associated with Paget's disease
of bone.

CAUSES
 About 40-50% of people with the inherited version of
Paget's disease have a mutation in the gene SQSTM1,
which encodes a protein, called p62 ( Nucleoporin p62)
that is involved in regulating the function of osteoclasts
(bone cells).

CAUSES
 Paget's disease of bone is associated with mutations in
RANK. Receptor Activator of Nuclear Factor κ B
(RANK), which is a type I membrane protein that is
expressed on the surface of osteoclasts and is involved in
their activation upon ligand binding .

PATHOPHYSIOLOGY
 Three phases of Paget disease have been described:
lytic, mixed lytic and plastic, and sclerotic.
Lytic phase: Paget disease begins with the lytic phase, in
which normal bone is resorbed by osteoclasts that are
more numerous, are larger, and have many more nuclei
(up to 100) than normal osteoclasts (5-10 nuclei). Bone
turnover rates increase to as much as 20 times normal.

PATHO….
Mixed phase: It is characterized by rapid increases in bone
formation from numerous osteoblasts. Although increased in
number, the osteoblasts remain morphologically normal. The
newly made bone is abnormal, however, with collagen fibers
deposited in a disorganized fashion rather than linearly, as with
normal bone formation. As the osteoclastic and osteoblastic
activities of bone destruction and formation repeat, a high
degree of bone turnover occurs.

PATHO,,,,
 Sclerotic phase: In the final phase of Paget disease, the
sclerotic phase, bone formation dominates and the bone that is
formed has a disorganized pattern (woven bone) and is weaker
than normal adult bone. This woven bone pattern allows the
bone marrow to be infiltrated by excessive fibrous connective
tissue and blood vessels, leading to a hyper vascular bone state.

PATHO,,,,
 After a variable amount of time, osteoclastic activity may
decrease, but abnormal bone formation continues. Some
pockets of normal-appearing lamellar bone may replace
immature woven bone, but mostly woven bone is may still
formed and therefore it is more prone to fracture and bending
or twisting.

RISK FACTORS
 Factors that can increase your risk of Paget's disease
of bone include:
Age. People older than 40 are the most likely to
develop Paget's disease of bone.
Sex. Men are more commonly affected than are
women. (3:2)

RISK FACTORS
National origin. Paget's disease of bone is more
common in England, Scotland and central Europe as well
as countries settled by European immigrants. It's
uncommon in Scandinavia (Norway, Denmark )
and Asia.
Family history. If you have a close relative who has
Paget's disease of bone, you are much more likely to
develop the condition yourself.

COMPLICATION
 Fractures. Bones affected by Paget's disease break more
easily. Extra blood vessels are created in these deformed bones,
so they bleed more during repair surgeries.
 Osteoarthritis. Misshapen bones can increase the amount
of stress on nearby joints, which can cause osteoarthritis.
 Deafness
 Blurred vision or vision loss

COMPLICATION
Heart failure. Unusually extensive Paget's disease
may force your heart to work harder to pump blood to the
affected areas of your body. In people with pre-existing
heart disease, this increased workload can lead to heart
failure.
Bone cancer. Bone cancer occurs in less than 1
percent of people with Paget's disease

DIAGNOSIS
• X-rays: the involved bone is expanded and denser than normal. Multiple
fractures may be present in long bones.
• Serum calcium and phosphate are normal but serum alkaline
phosphatase is markedly high.
• Urinary hydroxyproline (hydroxylation of prolin in the presence of prolyl
hydroxylase enzyme. Major component of the collagen protien) is also
elevated in active disease.
• Marker of bone turnover in urine. eg: pyridinoline and deoxypyridonoline
(main component of the collagen protien and may release during bone
degradation)

DIAGNOSIS
 A bone scan is a nuclear scanning test to find certain
abnormalities in bone. It is primarily used to help diagnose a
number of conditions relating to bones, including: cancer of
the bone or cancers that have spread (metastasized) to the
bone, locating some sources of bone inflammation (e.g. bone
pain such as lower back pain due to a fracture), the diagnosis of
fractures that may not be visible in traditional X-ray images,
and the detection of damage to bones due to certain infections
and other problems



PHARMACOLOGICAL MANAGEMENT
 Osteoporosis drugs (bisphosphonates) are the most
common treatment for Paget's disease of bone. Some
bisphosphonates are given as oral medications, while
others are given by injection. Oral bisphosphonates are
generally well tolerated, but may irritate your
gastrointestinal tract. Examples include:

CONT….
ThE American Institutional Policy Rearrange by Zahid khan.
 Tiludronate
 Etidronate
 Alendronate (Fosamax)
 Ibandronate (Boniva)
 Pamidronate (Aredia)
 Risedronate (Actonel)
 Zoledronic acid (Zometa, Reclast)

CONT…
 Long-term bisphosphonate therapy has been linked to
a rare problem in which the upper thighbone cracks, but
doesn't usually break completely. Bisphosphonates also
may increase the risk of osteonecrosis of the jawbone —
a rare condition in which a section of jawbone dies and
deteriorates after a tooth extraction.

CONT….
 If you can't tolerate bisphosphonates, your doctor may
prescribe calcitonin (Miacalcin), a naturally occurring
hormone involved in calcium regulation and bone
metabolism. Calcitonin is a drug that you administer to
yourself by injection or nasal spray. Side effects may include
nausea, facial flushing and irritation at the injection site.

NON PHARMACOLOGICAL
MANAGEMENT
 To reduce your risk of complications associated with Paget's
disease of bone, try these tips:
 Prevent falls. Paget's disease puts you at high risk of bone
fractures. Ask your doctor for advice on preventing falls. He or
she may recommend that you use a cane or a walker. Take
measures to fall-proof your home. Remove slippery floor
coverings, tuck away cords, and install handrails on stairways.

N P M
 Eat well. Be sure your diet includes adequate levels of
calcium and vitamin D, which facilitates the absorption of
calcium. This is especially important if you're being treated
with bisphosphonates. Review your diet with your doctor and
ask if you should begin taking vitamin and calcium
supplements.

N P M
 Exercise regularly. Exercising on a regular basis is essential
for maintaining joint mobility and bone strength. Talk to your
doctor before beginning an exercise program to determine the
right type, duration and intensity of exercise for you. Some
activities may place too much stress on your affected bones

SURGICAL MANAGEMENT
 In rare cases, you may require surgery to:
 Help fractures heal
 Replace joints damaged by severe arthritis
 Realign deformed bones
 Reduce pressure on nerves

SURGICAL MANAGEMENT
 Paget's disease often causes the body to produce an
excessive number of blood vessels in the affected bones.
This change increases the risk of serious blood loss
during an operation. If you're scheduled for surgery that
involves bones affected by Paget's disease, your doctor
may prescribe medications to reduce the activity of the
disease, a step that tends to reduce blood loss during
surgery.

SURGICAL MANAGEMENT
 Orthopaedic surgical procedures to address symptoms
include long bone osteotomies, fracture fixation, spinal
decompression, joint arthroplasty, and tumour resection.
The anatomical aberrations, abnormal bone architecture.

EPIDEMIOLOGY
 The overall prevalence and severity of Paget's disease
are decreasing; the cause for these changes is unclear.
Paget's disease is rare in people less than 55 years of age,
and the prevalence increases with age. Evidence from
studies of autopsy results have demonstrated Paget's
disease in about 3 percent of people older than 40 years
of age. Paget's disease is more common in males than
females. Rates of Paget's disease are about 50 percent
higher in men than in women

CONT..
 About 15 percent of people with Paget's disease also
have a family member with the disease. In cases where
the disease is familial, it is inherited in an autosomal
dominant fashion, although not all people that inherit the
affected version of the genes will express the disease

CONT..
 The incidence of Paget's disease varies considerably with
geographic location. Paget's predominantly affects people of
European descent, whereas people of African, Asian, or Indian
ancestry are less commonly affected. Paget's disease is less
common in Switzerland and Scandinavia than in the rest of
Western Europe. Paget's disease is uncommon in the native
populations of North and South America, Africa, Asia, and the
Middle East. When an individual from these regions does
develop Paget's disease, there is typically some European
ancestry present.

NURSING DIAGNOSIS
• Acute pain r/t bone fracture s/t Paget's disease.
• Impaired physical mobility r/t disease process.
• Anxiety r/t disease process
• Risk for infection r/t surgical procedure.
• Risk for GIT ulcer r/t effect of the prescribe
medication.
• Body image depict r/t disease process.

NURSING INTERVENTION
 Administer the prescribed painkiller . Eg: diclopenaic
 apply proper movement technique to avoid fall.
 Mind diversion therapy.
 Use proper sterilization technique.
 administer the prescribed OLPER D drugs and
misoprostol.
 give proper hygiene care.

Thanks for
your attention

Pagets disease (Imran Khan Salarzai)

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