An IPF Patient Perspective on What It Means to Be Rare
For most of my adult life I was blissfully unaware of rare diseases. This lasted until 2016, when I was diagnosed with idiopathic pulmonary fibrosis (IPF), a life-threatening and progressive lung disease that affects about 200,000 Americans, with 50,000 new cases diagnosed annually in the U.S.
Like many others, I had heard of more common illnesses that typically affect older generations, and as a 20-something, I just associated health issues with aging. Before my own diagnosis, I had no idea that patient advocacy organizations existed or that nationally coordinated movements, such as Rare Disease Day (RDD), focused on raising awareness for the 300 million people living with rare diseases worldwide.
Now that I have a rare disease, I am aware of these efforts and try to advocate for awareness as well. As an IPF patient, I’m particularly passionate about two months: September, which is Pulmonary Fibrosis Awareness Month, and February, which is when RDD occurs (this year on Feb. 28).
Each February I spend time reflecting on what it means to be rare for me. More specifically, I think about which aspects of my life differ from my healthy peers. Even if we’re doing the same task, such as going to the emergency room (ER), inevitably the process will be different for a rare disease patient.
Following are some of the roles, responsibilities, and largely unknown aspects of being rare.
Being an educator and an advocate
While I believe everyone should be an advocate for their own medical care, rare disease patients often go beyond the scope of regular advocacy. We also spend time educating doctors and nurses on our disease and subsequent medical needs, including what is considered normal for us but might be alarming for others who are healthy.
I have had several ER visits where nurses took my medical history while triaging me and made note of my IPF diagnosis, but then were alarmed to see a low oxygen saturation reading when checking my vitals. I often have to explain that due to IPF, my normal oxygen levels are lower than those of other young adults.
Likewise, when an ER physician examines my lungs and listens to my chest, they’re often alarmed at the crackling sounds they hear. I have to explain that Velcro-like crackles often accompany IPF and are not due to an acute issue, such as a virus.
Suppressing our needs
This is hard to admit because I try to be a proud rare disease patient. However, aside from the use of supplemental oxygen, IPF is an invisible illness, and as a young adult, I am aware of how it is perceived when I choose to take the elevator instead of the stairs or am short of breath after walking a short distance.
As a result, I try to suppress my needs and do everything my peers do, which never bodes well for me. I try to shovel my laneway after a snowfall, work full time, and attend social gatherings because other young adults can do these tasks. Being rare means I am different from my peers, but suppressing my needs reduces that differential gap.
Dealing with medication side effects
Most rare disease patients require medications to manage their condition. For those like me who are on long-term steroids, you’ve likely experienced the “moon face,” caused by swelling and fluid retention, or other side effects, such as thinning hair, gastrointestinal issues, and mouth sores.
Regularly feeling unwell due to medication side effects can prohibit normal activities, such as maintaining a job. Being a rare disease patient means we have to figure out strategies to manage the side effects because medications are necessary for most of us.
Navigating insurance and extra medical costs
Regardless of where you live and the healthcare coverage you have, there are financial implications of having a rare disease. Extra time, energy, and effort go into learning how to budget for these expenses, be it navigating insurance companies or researching grants to help pay for medications.
Being rare means figuring out creative solutions to the extra costs associated with ongoing medical care.
What does being rare mean to you? Please share your thoughts in the comments below.
Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Fibrosis News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to pulmonary fibrosis.