My Special Spleen: A Sensory Story About Living with “Polycythemia Vera”

Living with polycythemia vera (PV) is like having a body that constantly feels a step ahead of itself — too much energy, too many red blood cells, and too many signals asking the spleen to work overtime. 🩸 This chronic condition doesn’t always announce itself loudly at first, but once it does, it changes the rhythm of daily life in subtle yet powerful ways. This article dives into what it feels like, how it’s managed, and how patients can live fully with this rare blood disorder.

According to the National Organization for Rare Disorders (NORD), polycythemia vera affects about 1 in 100,000 people each year. It’s a myeloproliferative neoplasm (MPN) — meaning the bone marrow makes too many red blood cells, sometimes also increasing white blood cells and platelets. NORD PV Overview highlights that this overproduction thickens the blood, leading to poor flow and increased risk of clots.

Understanding Polycythemia Vera

At its core, polycythemia vera is a blood-making factory gone into overdrive. The JAK2 gene mutation, found in more than 95% of PV patients, tells the bone marrow to keep producing cells — even when the body doesn’t need them. As a result, the blood becomes thicker, the spleen enlarges, and symptoms begin to appear.

Common symptoms include:

• Persistent fatigue 💤
• Dizziness or headache
• Itchy skin, especially after a warm bath (aquagenic pruritus)
• A feeling of fullness or discomfort in the upper left abdomen (due to enlarged spleen)
• Reddened skin on the face or hands
• Shortness of breath or blurred vision

How It Feels to Live with Polycythemia Vera 🧠

Many patients describe PV as a “quiet storm” in the body. The symptoms are sensory and deeply physical — a constant hum of signals from blood that’s too thick and a spleen working double shifts.

1. The Heat and the Itch

A hallmark symptom, aquagenic pruritus, makes bathing a challenge. Even a warm shower can feel like an electric current across the skin. Patients often describe this as pins and needles combined with fireflies dancing on the skin.

2. The Weight of Blood

Imagine your blood as honey instead of water — slow-moving and heavy. That’s how many with PV describe their fatigue and sluggish circulation. Small tasks can suddenly feel monumental because the oxygen delivery system isn’t running smoothly.

3. The Overworked Spleen

An enlarged spleen (splenomegaly) can cause constant discomfort or fullness in the abdomen. It’s not just physical pressure — it affects appetite, digestion, and energy levels. Some even say they can feel their spleen pulsing when lying on their left side.

Diagnosis and Medical Journey 🧬

Diagnosing polycythemia vera requires careful blood analysis and genetic testing. Doctors often look for three key indicators:

Test	What It Measures	Typical Finding in PV
Complete Blood Count (CBC)	Measures red and white blood cells, and platelets	Elevated red cell mass
JAK2 Mutation Test	Detects JAK2 V617F mutation	Positive in 95% of PV cases
Erythropoietin (EPO) Level	Measures hormone controlling red cell production	Lower than normal

According to the Mayo Clinic, these tests confirm PV and help rule out other myeloproliferative disorders. Mayo Clinic Polycythemia Vera Information

The Role of the Spleen in PV

The spleen filters old blood cells and stores platelets, but in PV, it becomes overburdened by excess cells. This can lead to splenomegaly, making it a central character in the PV experience. Some patients even nickname it their “special spleen”, since its behavior often signals how well or poorly the body is managing blood production.

Treatment Options and Management Strategies 💊

While there’s no cure for polycythemia vera, treatments aim to reduce the number of red blood cells and prevent complications like blood clots and strokes.

1. Therapeutic Phlebotomy (Bloodletting)

This is one of the first-line treatments for PV. It involves removing blood from the body, similar to donating blood. By lowering blood volume, viscosity improves and symptoms ease.

2. Medications to Slow Blood Cell Production

Common medications include:

• Hydroxyurea: Suppresses bone marrow activity.
• Interferon-alpha: Encourages normal blood production.
• Ruxolitinib (Jakafi): Targets the JAK2 pathway to control symptoms and spleen size.

The U.S. Food and Drug Administration (FDA) approves Ruxolitinib for patients intolerant to hydroxyurea, marking a significant advancement in PV management.

3. Lifestyle Modifications

To reduce the risk of complications:

• Stay hydrated (thin blood flows more easily)
• Avoid tobacco and alcohol
• Exercise moderately but regularly 🏃‍♀️
• Maintain a healthy weight
• Protect against extreme heat or cold

Emotional and Sensory Experience of PV 💭

Polycythemia vera is not just a physical diagnosis — it’s a sensory and emotional journey. Patients often report emotional fatigue from managing chronic care routines, medication schedules, and frequent blood tests. Support groups and counseling can help in coping with the psychological toll.

How It Affects Daily Life

• Sleep Patterns: Frequent itching and discomfort can disrupt rest.
• Mental Clarity: Brain fog is common due to reduced oxygen flow.
• Social Activities: Some avoid crowded or hot environments due to symptom triggers.

Patient Perspective

Many describe living with PV as learning to “listen to their blood.” Every ache or flush feels like a signal. Over time, patients become attuned to these cues, adjusting habits and treatments accordingly.

Nutrition and Wellness Tips 🍎

Diet plays a supporting role in managing PV. While no specific food cures the condition, a balanced diet helps optimize circulation and overall health.

Recommended Foods	Why They Help
Leafy greens	Contain antioxidants that improve vessel health
Omega-3 fatty acids (fish, flaxseed)	Support blood flow and reduce inflammation
Berries and citrus fruits	High in vitamin C for vascular support
Whole grains	Maintain stable energy levels

Avoid iron supplements unless prescribed, as iron can fuel excess red blood cell production.

Complications and What to Watch For 🚨

If untreated, polycythemia vera can lead to serious complications:

• Blood clots (thrombosis): Increased cell count thickens blood, raising clot risk.
• Heart attack or stroke: Due to restricted blood flow.
• Myelofibrosis: Scarring of the bone marrow over time.
• Acute leukemia: In rare cases, PV can progress to acute myeloid leukemia (AML).

Regular monitoring through blood work and imaging is essential to catch these early.

Coping Strategies and Support 🤝

• Join a patient community: The MPN Research Foundation MPN Support Network offers resources and emotional support.
• Track symptoms daily: Apps and journals help identify triggers and improvements.
• Consult specialists: A hematologist experienced in MPNs ensures precise care.
• Stay positive: Mindfulness and therapy improve emotional resilience.

Technological Advances in PV Research 🔬

New therapies are emerging that target specific molecular pathways in PV. Researchers are exploring drugs that inhibit JAK2 and related mutations with fewer side effects. Gene therapy also shows promise in correcting faulty blood-forming stem cells.

According to American Cancer Society (ACS), survival rates have improved drastically thanks to better treatments and early diagnosis. ACS on Polycythemia Vera notes that with regular care, many patients live decades after diagnosis.

Living Fully with a “Special Spleen” 🌈

Having an enlarged spleen might sound daunting, but with awareness and care, life can still be vibrant and fulfilling. Regular checkups, listening to your body, and staying informed empower you to manage PV with confidence. Many patients find joy in simple pleasures – mindful walks, art, and connection with others who understand their journey.

It’s not just about surviving with polycythemia vera — it’s about thriving while embracing the body’s unique rhythm and resilience.

Final Thoughts 🌟

Living with polycythemia vera means learning to balance awareness, self-care, and resilience. While it requires ongoing management, it doesn’t define who you are. Every patient’s story is unique — a blend of medical science and human strength. By tuning into your body and partnering closely with your healthcare team, you can continue living a full, meaningful life, spleen and all.

5 Frequently Asked Questions (FAQs) ❓

1. Can polycythemia vera be cured?

Currently, there is no complete cure for PV, but treatments like phlebotomy, medications, and lifestyle changes effectively manage symptoms and prevent complications.

2. What causes polycythemia vera?

The primary cause is a mutation in the JAK2 gene, which tells the bone marrow to produce too many blood cells. This mutation is acquired during life, not inherited.

3. How is PV different from secondary polycythemia?

Secondary polycythemia occurs due to external factors like chronic lung disease or living at high altitudes, while polycythemia vera is a bone marrow disorder caused by genetic mutation.

4. Can exercise worsen PV symptoms?

Light to moderate exercise improves circulation and energy levels, but overexertion can trigger fatigue or discomfort. Always consult your doctor for a safe routine.

5. What is the life expectancy with PV?

With modern treatments, people with PV can live 15–20 years or longer after diagnosis. Regular follow-ups and adherence to therapy significantly improve outcomes.