Patients with Polycythemia vera may experience difficult-to-manage symptoms such as headaches, dizziness, vision disturbances, and fatigue. For these reasons, healthcare professionals must understand polycythemia vera management and how they can help provide quality care to those affected by this life-long condition.
In this blog post, we’ll discuss what polycythemia vera is and explore various nursing interventions used to support patients in managing the effects of this chronic illness on their daily lives.
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What is Polycythemia Vera ?
Polycythemia vera is a chronic and progressive blood disorder characterized by increased red blood cell production. It’s usually found in older adults but can affect people of any age. This rare disease carries an increased risk of stroke and heart attack due to the thickening of the red blood cells resulting from this condition.
Polycythemia vera Pathophysiology
A malignant DNA mutation in a single bone marrow cell causes polycythemia vera. This mutation, which can be found in 90% of PV cases, affects the JAK2 gene and results in an overproduction of red blood cells (RBCs), white blood cells (WBCs), and platelets. While some may experience elevated levels of all three components, others may only have increased RBCs.
Polycythemia vera (PV) is a disorder in which the body produces too many red blood cells. This overproduction can lead to thrombosis and, in some cases, Hemorrhage. Platelets are also produced in abundance by those with PV; however, they are often not developed enough to aid standard clotting mechanisms.
Polycythemia vera symptoms
PV often goes unnoticed until a routine blood test reveals abnormal results. Early symptoms may be mild, so much so that they can be missed. However, recognizing and treating the condition early can help to prevent the development of dangerous blood clots and their associated complications. Common symptoms of PV include:
- heavy sweating
- bleeding or bruising
- unintended weight loss
- pain in your abdomen
- feeling full easily
- blurred or double vision
- trouble breathing when you lie down
- trouble concentrating
Polycythemia vera Causes and Risk Factors
Polycythemia vera (PV) is more commonly seen in males above 60, yet it may appear at any age. The underlying cause of this disease is a mutation to the JAK2 gene, which regulates the making of specific blood cells; approximately 95% of people with PV possess such mutation. It can be inherited or arise spontaneously without a family connection.
Scientists are still investigating the precise cause of this genetic mutation. The chance of developing severe complications due to PV will depend on the probability of developing blood clots. Factors that can increase your risk of promoting blood clots in PV include:
- a background of blood clots
- high cholesterol
- being over age 60
- high blood pressure
Thicker than average blood can always enhance your risk of blood clots, no matter the cause.
To diagnose polycythemia vera, your doctor will take a physical test and question about your previous medical record. They may also order blood examinations to check for other disorders that can cause the same symptoms as PV. These tests may include:
- complete blood count (CBC)
- erythrocyte sedimentation rate (ESR)
- JAK2 gene mutation testing
- electrolyte panel
- immunoglobulin levels
They may also order imaging tests such as X-rays, ultrasounds, or magnetic resonance imaging (MRI) scans to check for blood clots in your body.
Polycythemia vera treatment
PV is a persistent medical condition that does not have a remedy. However, treatment can help you control its symptoms and avert complications. Your treating physician will create a personalized treatment plan for you, depending on your risk of developing blood clots.
Treatment for people with a low risk of blood clots
General therapy for those at low risk of blood clots includes Aspirin and a procedure called phlebotomy.
- Low-dose aspirin. Low-dose aspirin is used to thin the blood, reducing the risk of clotting.
- Phlebotomy. Phlebotomy is when blood is removed from your body and replaced with fluids such as saline or glucose solution. This procedure helps reduce the number of red blood cells in circulation and can help reduce symptoms associated with PV.
Treatment for people with a high risk of blood clots
In addition to phlebotomy and aspirin, people at high risk of blood clots may need more specialized treatment, such as other medications. These can include:
- Hydroxyurea (Droxia, Hydrea). This oral medication helps reduce the production of red and white blood cells in the bone marrow.
- Busulfan (Myleran). Busulfan is an intravenous medication that helps reduce the production of red blood cells in the bone marrow.
- Ruxolitinib (Jakafi). It is an oral medication that helps reduce the number of white blood cells in the bone marrow.
- Ropeginterferon alfa-2b-njft (Besremi). This injectable medication helps reduce the number of white blood cells in the bone marrow.
In addition to the treatments mentioned above, people with PV may receive related therapies. These can include:
- selective serotonin reuptake inhibitors (SSRIs)
- phototherapy (treatment with ultraviolet light)
Polycythemia vera Nursing Care Plan
The polycythemia vera nursing care plan should be tailored to the individual patient’s needs and include interventions that address their specific symptom management, education, monitoring, and medication administration. The goals of the care plan should be focused on ensuring that the patient can manage their symptoms and reduce their risk of developing complications from PV.
It is important to note that the care plan should be updated based on the patient’s response to treatment and any changes in their condition. The nurse should also ensure that the patient understands their medications, treatments, and lifestyle recommendations. In addition, nurses need to provide emotional support and help patients find ways to cope with this chronic condition.
Polycythemia Vera Nursing Interventions
Nursing interventions for polycythemia vera involve:
- Providing patient education and support.
- Monitoring the patient’s response to treatment.
- Managing their symptoms.
- Administering medications as prescribed.
This is particularly important to ensure that the patient’s condition does not progress or worsen.
Educational Intervention: Patients should be provided with accurate information about their diagnosis, treatment options, and lifestyle changes to help them manage their condition. This includes dietary recommendations, information about physical activity, and the importance of taking all medications as prescribed.
Monitoring: Nurses should closely monitor patients for signs and symptoms of progression or complications such as blood clots. They should also assess any side effects of treatments, including medications or procedures.
Symptom Management: Nurses should help alleviate any symptoms the patient may be experiencing and provide support and comfort as needed. This can include giving relaxation techniques, assisting with pain management, and helping the patient to remain physically active.
Medication Administration: Nurses must administer medications as prescribed by the doctor to ensure effective treatment. This includes any medications or treatments that are used to reduce the number of red blood cells or help with symptom management.
Polycythemia Nursing Interventions play an essential role in helping to manage the symptoms of this chronic condition. Nurses must provide patient education, monitoring, symptom management, and medication administration as part of their care plan for patients with PV.
It is also essential for nurses to remain supportive and understanding while providing care. This will help ensure that the patient can manage their medical condition best.
It is also essential for patients to remember that PV is a chronic condition, and they may need to make lifestyle changes to manage their symptoms. People with PV can lead healthy and active lives with the proper care and support.
Mrs. Marie Brown has been a registered nurse for over 25 years. She began her nursing career at a Level I Trauma Center in downtown Chicago, Illinois. There she worked in the Emergency Department and on the Surgical Intensive Care Unit. After several years, she moved to the Midwest and continued her nursing career in a critical care setting. For the last 10 years of her nursing career, Mrs. Brown worked as a flight nurse with an air ambulance service. During this time, she cared for patients throughout the United States.