Published on April 21, 2020
I was diagnosed with essential thrombocytosis, or essential thrombocythemia, over 30 years ago, when I was in my early 30s. As my platelet count slowly dropped, I thought I was getting better, until my doctor explained that my new diagnosis was myelofibrosis. Today, with a regimen of ruxolitinib (Jakafi) and bi-weekly darbepoetin alfa (Aranesp) shots, I remain relatively healthy. My only real complaint is fatigue, but I manage a light workout several times a week, and I walk my dog about two miles each day. This slow progression is characteristic of those of us with the CALR mutation.
Having lived with a myeloproliferative disorder for half my life, I have learned quite a bit about managing uncertainty and navigating complex medical systems. Here are a few hard-won lessons.
- Uncertainty is a fact of life. As an educator, I believe in the power of knowledge, and I’ve read widely about my disorder. On a day-to-day basis, though, we all live with a level of uncertainty. Was my extreme fatigue last week a symptom of advancing disease, or a normal reaction to a busy few months? No one can say. What is that funny pain in my diaphragm that comes and goes so quickly? How long will I continue to respond to Jakafi? And why did I get this disease in the first place? I wish I knew the answers to these questions, but I don’t and neither does anyone else. Part of proactively managing our health is accepting that sometimes mysteries remain no matter how much information we collect or how much consultation we undertake.
- Know your lab results. I receive excellent care at a major university medical center, but I feel responsible for monitoring my lab test results and general health so long as I am able to do so. When I don’t understand something in a lab report, I first try reading about it on a reputable website and then if necessary, ask a nurse or doctor. Being an informed patient means that I can ask better questions, discuss my treatment options with my doctor in more depth, and feel more control over my own well-being.
- Pay attention to the trend lines. My bi-weekly blood test results are never exactly the same, and I’ve learned not to overreact to any single number. Instead, I pay attention to change over time. Especially for those of us with slow disease progression, the trends reveal what I should be talking with my doctor about and how my disease is progressing. I’ve also learned that there’s a difference between a lab result that is abnormal versus one that is dangerous. My white cell count has been low for many years, but my immune system continues to do its job. I know that once it drops below a certain threshold, however, the risks will become substantially higher.
- Recognize the expertise of the nurses. At UCLA’s Bowyer Oncology Clinic, nurse practitioners are an integral part of the medical team. These highly trained professionals can order and review lab tests, write prescriptions, and perform examinations and various procedures including bone marrow biopsies. The nurse practitioner I see on a regular basis is knowledgeable, competent and kind. She regularly consults with my doctor, including alerting him to any emerging issues or concerns. If you have the opportunity to build a relationship with a nurse practitioner, do so.
- Appreciate the staff. Whether you are seen at a busy medical center or a smaller office, the staff are the people who keep the wheels turning. The vast majority of staff have chosen to work in a health care setting, received special training, and are diligent and caring. They are also likely to be extremely busy and subject to all kinds of interruptions and demands over which they have no control. When you feel up to it, show them the love. Bring some treats, remember their names, send a thank you card, or simply thank them with a smile. It’s not their fault that insurance is balking at some charge, that you’ve been waiting long past your appointment time, or that the doctor isn’t answering her page. They would prefer for everything to run smoothly too. So do your best to be considerate. At minimum, try not to direct your anger toward them. You can ask to talk with the clinic supervisor (or send him or her an email) or hospital ombuds.
- Waiting room etiquette matters. There are always people waiting to be seen at UCLA’s Bowyer Clinic—many are very ill, and everyone is anxious. I feel sad when I see that a husband and wife can’t find two seats together, because no one moves to accommodate them. I feel angry when people talk loudly on their cell phones, play video games without headphones,or carry in snacks that have a strong odor. It’s simple courtesy to keep the noise down, deal with phone calls in the hallway or a quiet corner, and toss water bottles or energy bar wrappers in the recycling container. Often, it’s friends and family members rather than us patients who don’t understand these basics, and it can be difficult to correct someone who is taking the time to support you. You can model appropriate behavior or simply explain that there are unwritten rules (sometimes there are written rules too).
- What kind of relationship do you want with your MPN? I don’t like the language of “fighting” disease. Myelofibrosis is not my enemy. It’s part of me, and I don’t want to be at war with my body or myself. Instead, I strive for a respectful relationship. Myelofibrosis has taught me so much—about the intricate workings of my body, the amazing advances of science and medicine, and the precious beauty of life. I don’t know what the next weeks or months will bring, so I do my best to stay focused on the relationships and activities that matter most to me. I can thank myelofibrosis for that.
~Maryann Jacobi Gray
Please remember the opinions expressed on Patient Power are not necessarily the views of our sponsors, contributors, partners or Patient Power. Our discussions are not a substitute for seeking medical advice or care from your own doctor. That’s how you’ll get care that’s most appropriate for you.
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