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).
During these months I try to leverage my IPF story and help others understand the social, emotional, mental, financial, and obvious physical implications of my rare disease.
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.
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Comments
Jean Bordu
Good bless you Charlene - It is good to see this rare disease has not broken your spirit but instead has hardened your resolve to defeat it and live a full life.
Charlene Marshall
Hi Jean,
Thanks so much for reading my columns and reaching out via the comments - I really appreciate your kind words. The day a significant breakthrough occurs for this disease will be one to celebrate for all of us. I can't wait for that day!
Char.
Adelaide Campbell
I love how you describe it, Charlene.
I’m in Canada too, and feel myself to be so incredibly fortunate with our health care system.
I find it sort of awkward and embarrassing. I was in a group yesterday and it came up. My acquaintance from years ago said: “You used to smoke, didn’t you?”
Well, yes, 20 years ago, mild cigarettes, and not very many.
I felt so guilty! Since it’s lungs, and since it’s rare, many people associate it with tobacco.
I’ve also had regular pneumonia at least twice, once in Djedda. And walking pneumonia twice, after long flights. Many years as a cabin attendant, breathing that potted air???
None of us know the origin of this disease that we never heard of before.
I’ve never been seriously ill (except TB as a child). Was it that?
Since it’s rare, we generally don’t know other people suffering from it, so we have no one to compare to and commiserate with.
Thank you and others for creating this forum, where we don’t feel alone, and we know others understand.
Some one on here remarked that this disease is defined by exhaustion. How do you explain that?
I find it’s also defined by nasty surprises. Because I had never heard of it before I got it. Yesterday I tried my small POC and found it’s TOO HEAVY for general use. I just cried and cried. I’m going to England soon and will be shopping with my oldest bestest friend.
But I calmed down and called my respiratory therapist, and he’s bringing a smaller lighter version. Being a salesman he could have told me of it before, but they are harder to come by.
I feel so guilty about not being able to do things I could do only a few months ago …. there are other diseases that bring instant care and concern, this brings blank looks.
I’ve bought a lanyard (Amazon) which says Invisible Disability, mainly to quell looks at the airport when this perfectly healthy looking woman is given a wheelchair. I know I’m imagining that, but it’s how I feel.
What do people say when people ask? No one has heard of PF, or ILD. Not in rural Ontario, anyway.
The PFT technician told me she’s been seeing many many more cases of PF in the last few years. Why?
Ah well. Onwards and forwards with the next scheduled tests …. so many of them! But I’ve had a long and very full life. Addie
Charlene Marshall
Hi Addie,
Thank you so much for reading my column and reaching out via the comments. Hello to a fellow Canadian! I resonate with so many of the points you bring up - trying to understand the cause, even though we'll really never know, as my diagnosis is considered 'idiopathic'. I never smoked, nor was I exposed to mold or other household or environmental triggers of lung disease. Is there a connection genetically? I don't think so but perhaps.... I've really given up on trying to understand why; it was taking too much physical and emotional energy away from me. As for the frustration with the POC, I get that too. My original POC was the Respironics Simply GO and while I love that machine, it is heavy!!! I'm glad your respiratory therapist can provide you with something different and please don't feel guilty for what you can/cannot do any longer; it isn't your fault, it's the illness. Focus on quality time with your friend, that is important and enjoy :)
Take care,
Char.
Virginia Currie
I admire you that in spite of this debilitating disease, you are still an inspiration to other people afflicted with this disease. I have a chronic fibrotic hypersensitivity pneumonitis and treatment is similar with IPF. I started with Ofev this month. So far, no side effects. I’m still working as cardiac RN. Stanford Hospital Interstitial Lung Disease is very supportive. I chose not to take the lung transplant and that was my personal choice. Like you, I want to frolic the beach and nature to live peacefully the rest of my life. Thanks to be an inspiration to me. I wish you all the success in your present endeavor. God bless you.
Charlene Marshall
Hi Virginia,
So nice to hear from you, thanks for reading my columns and reaching out via the comments! I've heard incredible things about the ILD team at Standford, I'm glad you have them in your corner and thank you for your career dedication, working as an RN. I have so much respect and admiration for our amazing nurses! I'm so glad the OFEV side effects have been tolerable for you, that is a blessing and I will pray it continues. Wishing you well and thanks so much for your kind words - we're stronger helping one another through this!
Char.
Charlene Marshall
Hi Jean,
Thank you for reading my column and reaching out via the comments. Some days I feel like the disease will break me, other days I keep fighting for myself and fellow patients. There's so much good research and advocacy efforts going on, I just have a good feeling something is coming for us soon.
Take care,
Char.
Librada Martinez
Char I also pray every day that they fine something to help with this ugly disease.
Charlene Marshall
Me too Librada! Me too - won't it be a glorious, worthy of so much celebration?
Char.
Steve Dragoo
Hi Charlene,
Thank you always for being willing to share and for your openness. I can identify with you some days it wants to break us.
A woman a few decades ago had let life's stresses hit her hard physically and mentally in her 60s and she looked like she was on her way out. But she had some insights that helped her change how she looked at stress and she took it head-on as a challenge. Fast forward a couple of decades, her success over stress allowed her to climb Mt Fuji in her 90s - imagine that - internal strength changes the heart and mind for something better.
What keeps me going is I am asked to speak in church about once a month and it is deeply motivating to bring a message of hope and truth as often as possible.
Find out what you are supposed to do and run after it with all of your heart...
Stay well.
Your friend,
Steve
Charlene Marshall
Hi Steve,
Always so nice to hear from you my friend, thank you for reading and writing. I always appreciate your unique lens on things and bringing up inspiring stories such as the 90 year-old who climbed Mt. Fuji. You're absolutely right - internal strength and a positive mindset can do wonders for us! I'm so glad you're asked to speak in church, you would be a fantastic speaker and bring such positive messages to your listeners. Keep up the great work!
Stay well!
Char.
Joseph O'Hanlon
Dear Charlene,
I feel so much for you, and your IPF diagnosis at such a young age. I hope and pray (and that is significant for an atheist) that a treatment for the contol of fibrosis is found in time for you.
I celebrated my 80th last month, so have survived a number of accidents and health hick-ups through the 29,219 days (now 29,237) of my life. WOW what a huge number.
I would like to ask you as an 'insider' to the Pulmonary Fibrosis News, to see if a technical library would be feasible?
From a personal point of view, I would like to investigate the genetic aspects of the disease. Other contributors have asked the question how did I get it? For me I might blame my time in the electricity supply industry in power stations. Not the nice clean hydro plants that I attended in Beautiful B.C., but the dirty rattly diesel powered plants that I trained in during my late teens and early twenty's.
However, my sister 11 years my senior did not have my exposure. She only survived 18 months, I am now over 8 years.
Yes we both did suffer the dirty city smogs of industrial England. Her in London, for a few years, and me the equvalent in Manchester.
IPF wise, I believe that I am in my final year. I'm OK with tnat, but would like to know if I (we) have a mutant gene that gives a disposition to suffer this complaint.
I see that those with Muc 5 are at hightened risk, and other familial gene associations have variously been mensioned.
I would like to have a gene test, but would want to be able to ask the laboratories the correct questions.
Has anyone collected this type of information, and can potentially advise me.
Also, the best labs to approach.
When I was positively diagnosed following a lung biopsy, I signed many forms allowing the NHS to use my information. The info' remains anonymous so one does not get any feedback.
To finish. I note your Canadian nationality. In the late 1960's I worked for B.C.Hydro for 5 years out on the West Coast. A most enjoyable time with projects all over the province.
Last month my supervisor of the time, and a very good friend, David, passed away.
You mensioned your appreciation of fine wines, he was a long standing member of the Vancouver Wine Club. As his girls said, he would not have enough time to drink his collection. On my visit back to Van., in '02, he introduced me to German 'eiswein' (probably mis-spelled). Delectable, a tiny amount needed, just bursting with flavour and sweetness .... you need a sweet tooth!
Thank you for your contibutions.
Kind regards,
Joe
Charlene Marshall
Hi Joe,
Thanks for reading my columns and reaching out via the comments - as always, its lovely to hear from you! I'd be curious to explore the genetic piece with you, and I have a lead on a family that's working really hard on this behind the scenes. Perhaps you could contribute your experience to them? I believe there are several brothers who carry the IPF gene based on other male members of their family having IPF too. It's pretty cool what they're doing! If you want to learn more about their efforts, feel free to email me directly: [email protected] . We can talk more about this there maybe :)
Take care,
Char.