Medical Nursing (III)
Subtopic:
Paget's disease
Paget’s disease of bone, also known as osteitis deformans, is a long-term condition affecting the skeleton. It is characterized by localized areas of excessive bone remodeling. Globally, it is the second most prevalent bone disease after osteoporosis.
Paget’s disease is defined by an accelerated rate of bone remodeling occurring in specific areas of the skeleton. This process involves both excessive breakdown of old bone followed by the rapid formation of new bone, which can lead to deformities and an increased risk of fractures.
Pathophysiology of Paget’s Disease
Healthy bone undergoes a continuous process of remodeling. Old bone tissue is removed and replaced with new bone tissue in response to the mechanical stresses placed on the skeleton.
Osteoclasts: These specialized cells are responsible for breaking down and absorbing old bone tissue.
Osteoblasts: These cells are responsible for building new bone tissue.
In Paget’s disease, the balance between these two cell types is disrupted, with osteoclasts exhibiting greater activity than osteoblasts. This means bone breakdown occurs at an accelerated rate. While osteoblasts attempt to compensate by forming new bone, their activity is excessive and results in the creation of abnormally large and structurally unsound bone.
The progression of the disease can be described in three stages:
Osteolytic Phase: This initial stage is marked by dominant osteoclast activity. The term “lytic” refers to destruction, and in this phase, osteoclasts actively break down existing bone, leading to the formation of pits or cavities within the bone matrix known as resorption pits. Microscopically, these active osteoclasts appear as large, multinucleated cells.
Mixed Osteoclastic – Osteoblastic Phase: During this transitional phase, both bone destruction and new bone formation occur simultaneously. Osteoclasts continue to break down bone, while osteoblasts attempt to rebuild. However, the rate of bone formation by osteoblasts is insufficient to keep pace with the destruction caused by osteoclasts.
Osteoblastic Phase: In the final stage, bone formation becomes the dominant process. There is excessive production of new bone tissue. This newly formed bone is characterized by a disorganized, mosaic pattern of lamellar bone, often described as having a “jigsaw puzzle” appearance.
Causes of Paget’s Disease
The precise causes of Paget’s disease remain unknown, classifying it as an idiopathic condition. However, it is believed that a combination of environmental and genetic factors plays a significant role in its development.
Risk Factors
Age: The disease is more commonly diagnosed in older adults.
Viral Infections: Some theories suggest that a slow viral infection may be a contributing factor. Viruses such as respiratory syncytial virus (RSV) and measles virus have been implicated in some studies.
Genetics: There is a clear genetic component to Paget’s disease. Studies indicate that a significant proportion of individuals with the condition, ranging from 15% to 40%, have a first-degree relative (parent, sibling, or child) who also has Paget’s disease.
Other Medical Conditions: While not direct causes, some autoimmune, connective tissue, and vascular disorders have been identified as potential contributing risk factors.
Signs and Symptoms of Paget’s Disease
Bone Pain: This is a common symptom, often affecting the pelvis, spine, skull, shoulders, and legs.
The pain is typically described as dull or aching.
It is often felt deep within the affected bone.
The pain tends to be persistent and can be worse at night.
The skin over the affected bone might feel warm to the touch.
Joint pain, stiffness, and swelling: This can occur, especially in the hips, back, and knees, as the abnormal bone can affect nearby joints.
Nerve Problems: These arise when the enlarged or misshapen bone presses on nerves.
This can cause pain that radiates from the spine down the legs.
It can also cause pain traveling from the neck into the arms and chest.
Numbness or tingling sensations may be felt in the affected limbs.
In some cases, there can be a partial loss of movement in the affected limbs.
Balance problems can occur if the disease affects bones involved in balance.
In severe cases, there might be a loss of bladder or bowel control due to nerve compression.
Enlargement and bowing of femurs and lower legs: The excessive bone growth can cause the long bones in the legs to become bowed or curved.
Enlargement of the skull around the forehead: Paget’s disease can cause the skull to thicken, which may be noticeable as an enlargement, particularly around the forehead.
Skull involvement may manifest these symptoms: When Paget’s disease affects the skull, it can lead to specific problems.
Development of hearing loss: The abnormal bone growth can affect the structures involved in hearing.
Loss of vision: If the bone presses on the optic nerve, it can cause visual problems.
Hydrocephalus: A buildup of fluid in the brain can occur due to the altered skull structure.
Somnolence (drowsiness): This can happen due to a “vascular steal syndrome,” where the increased blood flow to the affected skull bone reduces blood flow to the brain.
Diagnosis / Investigations.
X-rays: These are usually the first-line imaging test used to diagnose Paget’s disease. Affected bones may appear larger, denser than normal, and have an altered, deformed shape.
Blood tests: These tests can reveal elevated levels of the alkaline phosphatase enzyme. It’s important to note that calcium, phosphate, and aminotransferase levels are typically normal in Paget’s disease in elderly patients, which can help differentiate it from other bone conditions.
Bone scan: This imaging technique can help determine the extent and severity of the bone abnormalities throughout the skeleton.
Urine tests: An elevated level of Urinary Hydroxyproline, a marker of bone breakdown, can be found in urine samples.
Treatment and Management of Paget’s Disease
Currently, there is no cure for Paget’s disease, and it’s not possible to reverse the changes it causes in the bone.
Aims:
The main goals of treatment are to relieve bone pain and prevent the disease from getting worse (progression).
Treatment strategies primarily focus on alleviating symptoms.
Another important aim is to prevent potential complications from developing in the future.
Non-pharmacological Therapy.
Physical therapy: This can be beneficial for improving muscle strength and providing pain relief for certain types of pain associated with Paget’s disease.
While there are no proven methods to prevent Paget’s disease from occurring in the first place, maintaining good overall health is important for bone health. This includes:
Eating a healthy diet with enough calcium and vitamin D.
Getting regular exercise. These habits help maintain skeletal health and joint mobility.
Pharmacological Treatment. The main types of medications used to manage Paget’s disease include:
Bisphosphonates: These drugs (also used in osteoporosis) work by slowing down or reducing the breakdown of bone by osteoclasts. There are currently six bisphosphonate medications approved for the treatment of Paget’s disease.
Important note before starting bisphosphonates: Patients are typically advised to take calcium and vitamin D supplements daily for two weeks before starting bisphosphonate therapy. This helps reduce the risk of developing low blood calcium after the bisphosphonate infusion.
Bisphosphonates are not recommended for individuals with pre-existing low blood calcium levels, vitamin D deficiency, or significant kidney problems (compromised renal function).
It’s generally recommended to take calcium supplements along with bisphosphonates to further minimize the risk of hypocalcemia (low blood calcium).
Calcitonins: These medications can help reduce bone pain, decrease inflammation around affected bones, improve neurological complications caused by bone pressure, and promote healing of osteolytic lesions (areas of bone breakdown). Calcitonins are often used for patients who cannot tolerate bisphosphonates.
Pain Management: Pain associated with Paget’s disease can arise from bone deformity, arthritis, or neurological issues. Medications used to manage this pain include:
Acetaminophen (paracetamol).
Nonsteroidal anti-inflammatory drugs (NSAIDs).
Surgery. Surgical procedures may be necessary to address specific complications of Paget’s disease, such as fractures, limb malalignment, or arthritis.
Internal fixation: This surgical technique is used to treat fractures in bones affected by Paget’s disease. The broken bone fragments are firstRealignment and then held in place using hardware like screws, wires, pins, or metal plates, which may be attached to the outside of the bone.
Osteotomy: This procedure can help alleviate pain and correct the alignment of weight-bearing joints, particularly the hip and knee. It involves removing a wedge-shaped piece of bone near the damaged joint to shift weight onto a healthier and more stable part of the joint.
Total joint replacement: In this surgery, the damaged parts of an arthritic joint are removed and replaced with an artificial joint made of metal, ceramic, or plastic. This artificial joint (prosthesis) is designed to mimic the function and movement of a healthy joint.
Nursing Diagnosis
Acute pain related to joint inflammation secondary to Paget’s disease, as evidenced by a self-reported pain score of 10 out of 10, guarding behavior on the affected fingers, restlessness, and irritability.
Activity intolerance related to joint inflammation and pain secondary to Paget’s disease, as evidenced by a pain score ranging from 8 to 10 out of 10, fatigue, lack of interest in performing Activities of Daily Living (ADLs) due to pain, verbalization of tiredness, and generalized weakness.
Deficient Knowledge related to a new diagnosis of Paget’s disease, as evidenced by the patient’s statement: “I want to know more about how to manage my illness.”
Nursing Interventions.
Assess the patient’s ability to perform activities of daily living and identify any limitations, both actual and perceived, to their physical activity. Inquire about any forms of exercise the patient previously engaged in or would like to try.
Encourage the patient to gradually increase their activity levels through self-care and exercise, within their tolerance. Explain the importance of reducing sedentary behaviors such as prolonged television watching and social media use. Encourage alternating periods of physical activity with 60-90 minutes of uninterrupted rest.
Administer prescribed analgesics before exercise or physical activity to help manage pain. Teach the patient deep breathing exercises and relaxation techniques to aid pain management. Ensure adequate ventilation in the patient’s room.
Refer the patient to a physiotherapy or occupational therapy team as needed. These specialists can provide more tailored care to help the patient build confidence in increasing their daily physical activity.
Assess the patient’s readiness to learn about their condition, identify any misconceptions they may have, and recognize any barriers to learning (e.g., denial of the diagnosis or unhealthy lifestyle habits).
Provide the patient with detailed information about their prescribed medications, including the drug class, its purpose, benefits, potential side effects, and risks associated with its use for managing acute pain. Ask the patient to verbally explain or demonstrate how they will self-administer the medication to ensure understanding and safety.
If the patient is scheduled for surgery, explain the specific surgical procedure related to Paget’s disease to both the patient and their caregiver. Emphasize that the doctor may recommend surgery to alleviate severe joint pain caused by Paget’s disease.