pagets disease (imran khan salarzai)

IMRAN KHAN SALRZAI 1

KHYBER MEDICAL UNIVERSITYSubject: AHN II

Disease: Paget’s Disease of bone

Presented by: Imran Khan Salarzai

Class: BScN year II semester IV

Roll no: 39

24/11/2015

IMRAN KHAN SALRZAI 224/11/2015


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.

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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.

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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.

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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.

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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.24/11/2015


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.

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CAUSES OF PAGET'SThe 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.

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CAUSESNoriyoshi 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

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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.

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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).

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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 .

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PATHOPHYSIOLOGYThree 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.

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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.

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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.

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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.

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RISK FACTORSFactors 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)

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RISK FACTORSNational 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.24/11/2015


COMPLICATIONFractures. 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.

DeafnessBlurred vision or vision loss

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COMPLICATIONHeart 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 disease24/11/2015


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)

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DIAGNOSISA 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

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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:

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CONT….ThE American Institutional Policy Rearrange by Zahid khan.

Tiludronate

Etidronate

Alendronate (Fosamax)

Ibandronate (Boniva)Pamidronate (Aredia)

Risedronate (Actonel)

Zoledronic acid (Zometa, Reclast)

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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.

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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.

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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.

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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.

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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

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SURGICAL MANAGEMENT

In rare cases, you may require surgery to:

Help fractures healReplace joints damaged by severe arthritisRealign deformed bonesReduce pressure on nerves

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SURGICAL MANAGEMENTPaget'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.24/11/2015


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.

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EPIDEMIOLOGYThe 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

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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

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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.

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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. 24/11/2015


NURSING INTERVENTIONAdminister 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.24/11/2015


Any question

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Thanks for your attention

pagets disease (imran khan salarzai)

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