Paget's Disease NU240

7/29/2019 Paget's Disease NU240

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Running head: Gerontologic Physiologic Disorder

Paget‟s disease

Lucy Igbinoba

Breckinridge School of Nursing

August 20th

, 2012



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What is Paget‟s disease?

Paget‟s disease of the bone or “osteitis deformans” is a disorder of bone remodeling in

which there is excessive bone resorption followed by excessive bone formation that results in

bone that is architecturally unsound. In 1877 Sir James Paget, an eminent British surgeon,

described „osteitis deformans‟ in an elderly man with progressive skeletal deformities, this

condition became known as Paget‟s disease of bone, this disease “is the second most common

bone disorder (after osteoporosis) in elderly persons.” (Lohr, 2011) Paget‟s disease “occurs in

both males and females, affecting 1.5 to 8% of the population over 50 years of age in many

countries. It is much less common in people of Asian, Indian and Scandinavian ancestory.” (A

Nurse's Guide to the Management of Paget's Disease)

Although many people are asymptomatic, Paget‟s disease can result in pain, deformity,

increased risk of fracture and other complications including osteoarthritis and nerve-compression

syndromes. The condition cannot be cured but treatments can control its progression and

minimize complications. While Paget‟s disease is common, initial diagnosis is often missed and

many healthcare professionals, including nurses, fail to recognize early symptoms and are

uncertain about management.

Pathophysiology

In a living tissue, bone is constantly renewed through a remodeling process that provides a

mechanism for self-repair and adaptation to stress. This takes place at different sites on the bone

surface and is largely regulated by two types of bone cells, osteoclasts (bone destruction cells)

http://www.nursingtimes.net/pagets-disease-1-epidemiology-causes-and-clinical-features/1992719.article




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and osteoblasts (bone formation cells). Osteoclasts degrade bone by attaching to bone surface

and secreting acids and enzymes into the bone cavity. The osteoblasts then synthesise and

mineralize new bone matrix within the cavity created (this is how new bones are formed). “In

Paget‟s disease, the osteoclasts are increased in size and number and contain many more nuclei

than normal and, consequently, they resorb bone more quickly. In response, the osteoblasts

deposit new bone at an increasingly rapid rate. This rapid rate of bone turnover leads to the

production of bone that may be larger in size than normal but is characterized by disorganized

architecture and reduced mechanical strength, leaving people at increased risk of developing

fractures and deformities.” (Sutcliffe, 2009)

Paget‟s disease affects older skeletal bone of the older adults, there is also an extremely

rare form of Paget‟s disease in children. The older adult is more susceptible to Paget‟s disease

due to the following;

The quality of our bones starts to deteriorate gradually after the age of thirty due to a

decrease in our bone density (this is the amount of mineral matter found inside our bone).

For women, a few years after menopause, bone loss also starts. By the process called

resorption, old bones are broken down and are reabsorbed by the body; the old bones are

then replaced by new bones in the process. After menopause, the process of resorption

slows down, because of this, there is a reduced amount of new bone formations to

compensate for the loss of old bones.

Due to stress (weight) on the bone.

Due to wear and tear.





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Causes of Paget‟s disease

“The cause of Paget's disease is incompletely understood, but genetic factors clearly have an

important role. Roughly 15% of patients have a positive family history, JS2S and the

risk of developing Paget's disease of bone has been estimated to be 7-10 times greater in first-

degree relatives of patients than in the general population."'" Many families have also been

described in which classic Paget's disease is inherited as an autosomal dominant trait with high

penetrance by the sixth or seventh decade of life…” (Ralston, Langston, & Reid, 2008) Virus

infection may be necessary to trigger Paget's disease in people who have inherited the genetic

tendency to develop the condition by having these genes ( Sequestrosome 1 gene on

chromosome 5). “Several potential environmental triggers for Paget's disease have been

suggested on the basis of epidemiological studies, observational data, or hypotheses, including

low dietary calcium intake or vitamin D deficiency during childhood,51,52 zoonotic infections,

viral infections,54 and occupational exposure to toxins. The only environmental trigger for

this disease that has been studied experimentally is paramyxovirus infection, which was first

suggested to be a cause of Paget's disease of bone when osteoclasts from affected patients were

shown to have inclusion bodies that were thought to resemble viral nucleocapsids…”

Incidences/Epidemiology of Paget‟s disease

Paget's disease of bone becomes more common with age and goes undiagnosed.”…many

older people who experience discomfort in their bones and joints assume that those symptoms

are a natural part of aging, and therefore don't seek treatment.” (Mayo Clinic Staff, 2010)



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Paget‟s disease is estimated to affect 1 to 3 million people in the United States. Epidemiologic

studies are inherently imprecise, however, because many individuals with Paget disease are

asymptomatic. The prevalence of pelvic Paget disease increases with age, with the highest

prevalence in persons older than 65 years. A survey study suggested that the prevalence in the

United States is 2.3% of the population between ages 65 and 74 years. Paget disease is estimated

to occur in 1-3% of individuals older than 45-55 years and in up to 10% of persons older than 80

years. Geographically, pelvic Paget disease was least common in the southern United States and

most common in the northeastern United States. The prevalence of Paget disease varies greatly

in different areas of the world. The highest prevalence is in Europe (predominantly England,

France, and Germany). The United States, Australia, and New Zealand have high prevalence

rates because of significant populations with northern European ancestry and a large population

of British immigrants. The disease is rare in Asian countries, especially China, India, and

Malaysia, and in the Middle East and Africa.

Risk factors associated with Paget‟s Disease

Age: 40 or older.

Family history of Paget‟s disease, especially in a parent, sibling, or child.

In US, residence in northern states.

Race: Anglo-Saxon, European.

Sex: men are more commonly affected than women.



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Nursing Management

Assessment

Signs and symptoms

Swelling or deformity of a limb.

Increased head size.

Bowing of a limb.

Curvature of the spine.

Chronic bone pain.

Frequent fractures.

Problems with walking or gait.

Headaches, facial pain, or hearing loss when the skull bones are affected.

Swollen or painful joints due to damaged joint cartilage.

Numbness, tingling, pain, or weakness from pressure on nerves.

Depending on the bone site, Paget's disease may result in:

Arthritis, usually osteoarthritis.

Carpal tunnel syndrome.

High calcium in blood.

Kidney stones.

Hearing problems (common).

Dental problems.

http://www.munsonhealthcare.org/Taxonomy/RelatedDocuments.aspx?id=1087&sid=1&EBSCOID=11979&lang=English&db=hlt








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Rarely: eye problems, congestive heart failure, bone cancer (osteosarcoma).

Objective data

Display of one or more of the afore-mentioned symptoms.

Medical diagnosis due to diagnostic tests and physical assessment.

Family and medical history.

Vital signs evaluation.

Decreased functionality.

Change in physical structure.

Subjective data

Constant complains of pain – chronic bone or joint muscle pain.

Inability to perform ADLs.

Lack of restful sleep.

Diagnosis

Diagnostic tests and procedures

Paget‟s disease can be diagnosed with one of the following tests;

Serum Alkaline Phosphate (SAP) Blood Test: an elevated level of serum alkaline

phosphate in blood can indicate Paget‟s disease.

X-rays: this disease can also be discovered in x-rays taken to evaluate other conditions or

musculoskeletal complaints.



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Bone Scans (MRI, and CT scans): this is useful in determining the extent and activity of

this disease.

Nursing diagnosis:

Chronic pain r/t potential tissue damage as evidenced by patient verbalization of pain.

Disturbed body image related r/t possible enlarged head as evidenced by nonverbal

response to actual change in body (head size).

Risk for trauma r/t excessive bone destruction as evidenced by result of MRI scan.

Planning, expected outcome and goals of therapy

The short-term goal is to alleviate bone pain, while the long-term goal of treatment is to

minimize or prevent the progression of the disease and improve the patient‟s physical function.

Expected Outcomes Nursing Interventions Rationale

1.Client will use self-report

tool to identify current pain

level and establish a comfort-

function goal within the next

72 hours.

1.Assess pain level using a

reliable and valid self-report

tool.

1.The first step in pain

assessment is to determine if

the client can provide a self-

report. (Ackley, 2011, p. 611)

2. Client will demonstrate

ability to pace activity, taking

rest breaks before they are

2. Encourage the client to plan

activities around periods of

greatest comfort whenever

2.Clients will find it easier to

perform ADLs and enjoy

social activities when they are



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needed in 72 hours‟ time

frame.

possible. Pain impairs

function.

rested and pain is under

control. (Ackley, 2011, p. 615)

3.Client will verbalize

congruence between body

reality and body perception

within the next 3 days.

3.Explore opportunities to

assist the client to develop a

realistic perception of his or

her body image.

3.Actual body size may not be

consistent with the client‟s

perceived body size.

Inaccurate perception by the

client can be unhealthy.

(Ackley, 2011, p. 174)

4.Client will utilize strategies

to enhance appearance (e.g.,

wig, clothing) within the next

3 days.

4.Encourage client to purchase

clothes that are attractive and

that deemphasize their

disability.

4.Individuals with

osteoporosis are not usually

disabled but may perceive

themselves as unattractive and

experience social isolation as a

result of ill-fitting clothes that

accentuate the physical

changes. (Ackley, 2011, p.

174)

5.Client will remain free from

trauma within the next 72

hours.

5.Screen clients with a fall risk

factor assessment tool to

identify those at risk for falls

5. A major strategy of all fall

prevention programs is the

identification of clients at risk

of falling. This is commonly

performed with the aid of a

fall-risk assessment tool.



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(Ackley, 2011, p. 851)

6.Client will explain actions

that can be taken to prevent

trauma within the next 72

hours.

6.Educate and provide client

and family with hip protector

devices.

6.One recent study has shown

that the use of hip protectors

in care homes with clients

identified as high risk for falls

had a slight decrease rate of

hip fractures, which is a great

cause of morbidity and mor-

tality in the geriatric popula-

tion. (Ackley, 2011, p. 851)

There are four main types of treatment of Paget‟s disease:

1.

Pharmacological therapy: this include treatment with Bisphoshonates (such as Zoledronic

Acid – Reclast, Risedronate - Actonel, Alendronate – Fosamax, e. t.c.) which suppress or

reduce bone resorption by osteoclasts, and Calcitonin (Miacalcin – Novartis) which

reduces elevated indices of bone turnover by 50%, decrease symptoms of bone pain,

reduce warmth over affected bones, improve some neurological complications and

promote healing of lytic lesions.

2. Pain management using analgesics such as, Acetaminophen (Tylenol), and NSAIDS.

3. Surgical intervention: surgery maybe required due to bone fracture, severe degenerative

arthritis, and bone deformity.



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4. Non-pharmacological: this focuses on physical therapy as a means of improving muscle

strength to help control some types of pain.

Evaluation including Patient/Family Teaching

1. Ask the client to identify the pain level, on a self-report pain tool, that will allow the

client to perform desired activities and achieve an acceptable quality of life.

2. Discuss the total plan for pharmacological and non-pharmacological treatment, including

the medication plan for ATC (around the clock) administration and supplemental dose,

the maintenance of a pain diary, and the use of supplies and equipment.

3. Inform client of available community support groups, such as internet discussion

boards.

4. Encourage significant other/family to offer support.

5. Encorage use of proper lighting at home, especially at night.

6. Teach the client to keep his/her home cluster-free. Teach client the side-effects of

medication to watch out for.

Prognosis/complications

In conclusion, it should be noted that Paget‟s disease is asymptomatic at the early stage; early

detection and prompt treatment always lead to a good prognosis. Paget‟s disease does not have a

known cure at this time, but effective and prompt management can lead to alleviation of the

chronic pain associated with this disease, stop or prolong further progression of the disease, and

improve quality of life. Patients with functional impairment, usually as a consequence of skeletal

deformity or complications such as osteoarthritis, require assessment and, if appropriate,



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intervention by the multidisciplinary team, which includes a physiotherapist and occupational

therapist. Surgery, such as total hip replacement, may be indicated for advanced joint disease.

However, Paget‟s disease if left untreated can lead to the following complications; bone

deformity, hearing loss, fractures, teeth, vision and heart problems, osteosarcoma and other

tumors.



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References

A Nurse's Guide to the Management of Paget's Disease. (n.d.). Retrieved from Paget.org:

http://www.paget.org

Abelson, A. (2008, March). Current Medical Research and Opinion: A Review of Paget's Disease of Bone

with a Focus on the Efficacy and Safety of Zoledronic acid 5 mg. Retrieved from Proquest:

http://search.proquest.com

Ackley, B. l. (2011). Nursing Diagnosis Handbook: an evidence-based guide to planning care. St. Louis,

Missouri: Mosby Elsevier.

Badash, M. (2011, September). Division of Endocrinology, New York University (Department of

Medicine). Retrieved from nyu.med: http://medicine.med.nyu.edu

Donohue, P. G., & MD. (2010, August 26). Paget's Disease Affects the Bones, Strikes Primarily at Older

Ages - Proquest . Retrieved from Proquest: http://search.proquest.com

Gkouva, L. (2011, January 24). Health & Medicine Week: Understanding of Paget's disease. Retrieved

from Proquest: http://www.proquest.com

Kerr, M. ( 2010, March 16). Irish Times: Paget's Disease. Retrieved from Proquest:

http://search.proquest.com

Lohr, K. M. (2011, September 29). Medcsape Reference: Paget's Disease. Retrieved from Medscape:

http://emedicine.medscape.com

Mayo Clinic Staff. (2010, August 12). Paget's disease of bone. Retrieved from MayoClinic:

http://mayoclinic.com

Ralston, S. H., Langston, A. L., & Reid, I. R. (2008, July 12-18). Pathogenesis and management of Paget's

disease of bone. Retrieved from Proquest: http://proquest.com

Sutcliffe, A. (2009, February 17). Paget's disease 1: Epidemiology, causes and clinical features. Retrieved

from Nursing Times: http://www.nursingtimes.net

The Paget Foundation. (2010, Winter Vol.32 No. 1). Update: Basic Information About Paget's Disease.

Update, pp. 1-4.



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Paget's Disease NU240

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