Living with a rare disease can present a unique set of challenges, as people may experience symptoms for years before receiving an accurate diagnosis. Once diagnosed, it may be challenging to locate a physician familiar with this rare disease, or to even connect with other patients facing the same diagnosis. For patients with polycythemia vera, or PV, a rare, chronic and progressive blood cancer that affects approximately 100,000 people in the United States, recognizing the symptoms of the disease can be even more challenging because they can vary over time and from patient to patient.
“Patients living with rare diseases don’t always know what to ask their doctor as they work to manage their disease, where to turn for resources, or what signs and symptoms to look for,” says Ellen Ritchie, MD, Associate Professor of Clinical Medicine at Weill Cornell Medical College. “PV can develop slowly and get worse over time. Its symptoms can be hard to spot, so it’s critical that those diagnosed with PV understand the symptoms and discuss any changes with their physician, as these symptoms may be a sign that their disease is not under control.”
The symptoms of PV can sometimes be difficult for patients and healthcare providers alike to recognize. Some people living with PV may be asymptomatic, having no symptoms at all. Others could have symptoms for years before receiving an accurate diagnosis. While not a comprehensive list, PV symptoms may include:
* Itching (especially after a warm shower)
* Abdominal pain or discomfort
* Sweating (at night or during the day)
* Feeling of fullness, even when you haven’t eaten
Approaches to Tracking Symptoms
There are many approaches to tracking PV symptoms. Keeping a regular diary to record symptoms or changes in symptoms is one approach. There are also online trackers such as the PV Tracker Tool that can help patients monitor their PV symptoms. This tool can record previous entries and compile those results for sharing with your healthcare professional. Beyond tracking symptoms, it’s important to talk with your physician about your symptoms and to be knowledgeable about your disease.
What to Ask Your Physician
To ensure you have an informed conversation with your healthcare professional, come to all appointments prepared with a list of questions, as well as a rolling log of symptoms and any changes in their severity experienced since the last checkup. Possible questions you may wish to ask your physician include:
1. What are my target blood counts, and what are my actual blood counts?
* Hematocrit (volume of red blood cells)
* White blood cell count
* Platelet count
2. What is my treatment plan to keep my PV under control?
“All people diagnosed with rare diseases should advocate for their care and take an active role in managing their disease with their healthcare provider,” says Dr. Ritchie. “It is important to create a routine that not only includes monitoring blood levels, but also recognizing, tracking, and talking about symptoms with a healthcare professional.”
For tools to help track PV symptoms and additional tips on managing PV and other MPNs, visit VoicesofMPN.com.