During our Self-Advocacy Special Education Week, multiple members of the PF NOW! community volunteered to share their advocacy stories. Our first two advocacy stories are available here and here.
This month, our advocacy series continues with an interview featuring our community member Amy. To see highlights from the interview, click here. For the whole transcript, continue reading below.
PF NOW!: When did you get diagnosed with PF or IPF, and what caused it (if you know)?
Amy: I was diagnosed with an autoimmune disease called systemic scleroderma in 2004, and right away I had PF in my lower lobes. So I was diagnosed with Interstitial Lung Disease/Pulmonary Fibrosis right away, and it stemmed from my scleroderma. It goes hand-in-hand with that disease. My lungs stayed okay for a couple of years, and then they started to take a dive… and that’s when I had to start looking at alternatives, and ways to treat that.
PF NOW!: What are the symptoms that you struggled with at the time of diagnosis, or that you’re handling right now?
Amy: A lot of coughing. Waking up in the morning, I don’t much anymore – I think because of the medications I’m on – but I would cough for an hour every morning. Then throughout the day, I would be coughing. Sometimes it would be random. The coughing spells would last for half an hour to an hour. My whole family is very adept at being able to hit my back to try to bring stuff up and help clear the airways.
Laughing and crying will cause me to cough. Any kind of fake smells (perfumes, and any synthetic fragrance), I can tolerate some natural fragrances, but anything that’s not natural will immediately give me a headache. Then within about 10 minutes I start coughing. The wind, breathing cold air, anything that gets my heart rate up will make me start coughing. I can’t eat or drink anything very cold (I can’t have ice cream, malts, or smoothies), because that will instantly make me start coughing. It’s just exhausting.
And then shortness of breath. That’s been slow over the years, so I think I don’t realize the change as much. I used to be a really fast walker, and now I notice that I’m no longer the person that people are trying to keep up with. It’s the other way around: “Slow down, slow down!”
PF NOW!: With the PF and the scleroderma symptoms, have you been able to find the support that you need?
Amy: Yes. I am fortunate that I have a husband, and (most of) my friends and family are incredibly supportive and accepting of my limitations.
I was on some scleroderma forum, where someone said, “My husband laughs at me; he thinks I’m a lazy piece of shit and tells me I don’t do anything…” That’s just so odd to me. I don’t have that in my life. I don’t have anyone that doesn’t believe I don’t have the disease. It’s hard when you have an invisible disease/disability because you appear healthy, therefore people don’t understand. It’s painful when it’s those who are “close” to you.
I did help start a local scleroderma support group shortly after I was diagnosed, but nobody really wanted to look at ways to improve life, everyone just wanted to come and complain. Which I get – you need space to do that. But we were trying to bring meditation, self care and positive thinking into the group, and nobody wanted that. Unfortunately, it became something that was dragging me down and I really couldn’t be a part of it anymore.
It’s really helpful to have groups like PF Now because people can vent and feel heard, and there’s such a wealth of information being shared. There’s also a great sense of community and understanding.
I did some online support groups for a while, and then life got in the way and I was just living. I never had Facebook, but since starting the OFEV, I started wanting to know more about that, and to just see what people are doing for the disease that I may not know of.
PF NOW!: If you ever want to try rebooting meditation, positive thinking, or other similar programs, we’d be happy to help! We really want to offer all the support that people need.
PF NOW!: Can you think of a time when you had to stand up for yourself or speak up about your needs? What was that like for you?
Amy: Yeah, absolutely. It has more to do with scleroderma than PF. After my lungs started declining around 2006, my local rheumatologist (who was like the person to go to if you had scleroderma in the city I live in) was answering my question, “What can I do? What are all the treatments that are being offered right now?” She said, “Well, I’m leading a stem cell transplant trial right here. You’d be the first patient. It’s really your only option. There’s nothing else.” So I started going through the pre-screening and testing to see if I would qualify and I did.
Part of the protocol in that transplant trial included full body radiation and there was a delay in getting some shield and I was having to wait to start. I was full of doubt and trepidation.
In the meantime, I was on a scleroderma forum, and this person privately messaged me and said, “I know that you’re doing this stem cell transplant, but I just want you to know that there’s this doctor in Chicago who’s doing a different stem cell transplant trial and he’s not using full-body radiation.” Radiation, you know, is very caustic and it can cause further hardening, (that’s what scleroderma is – everything becomes hard) so she said, “You may want to look into it. I’m sorry if this is going to complicate things for you, but I feel that I need to tell you that there is this other option out there. Since you live so close, you may want to check it out.”
The next day in the mail, I got a postcard telling me that the Chicago chapter of The Scleroderma Foundation was hosting a talk about the stem cell transplant trial without radiation the following weekend. I said to my husband, “Let’s go.” Literally, within a couple minutes of the talk starting, I looked at my husband and said, “This is where I want to be.” I just had this feeling, this intuition, and I thought, “There is where I’m supposed to be.”
I ran up to the doctor afterwards and talked to him, and he was so kind. His name is Dr. Richard Burt. He was just the kindest man, and he said to call him on Monday, and he gave me his phone number and said, “I will help you.”
So I came back home and nervously called my doctor and said to her, “I went to Chicago and heard Dr. Burt talk,” and she said, “Ah… he’s pretty good, isn’t he?” And I said, “Yeah, well he convinced me, and I do not want to do the full body radiation.” She said, “Well he does a really good sales job, but I don’t think that’s a really good move for you. The full body radiation is part of the necessary protocol. His won’t work.”
I didn’t even address the fact that she’d failed to tell me there was another study out there. She’d told me that hers was the only option. I said, “I’m going to go with Northwestern, and I’m going to drop out of your study.” That was around Christmas time.
On New Year’s Eve, I was working an evening shift (as a nurse at a hospital). As I went to leave, the security guard said, “Someone dropped off a letter for you.” I was just like… well that’s weird… and it was a letter from my local doctor, the one running the study I dropped out of. The letter said that I was letting down the entire scleroderma community because I was choosing a non-NIH funded study, and if everybody chose to do that then the NIH wouldn’t fund those studies anymore. That I was making a big mistake, and that I really needed to think twice about what I was doing.
I made an appointment with her, sat down with her, and said, “I’m not okay with you doing this. I’m not okay with you dropping a letter off at my place of employment, or telling me that I’m letting down the scleroderma community. This is my life, and I’m going to do what I think is best for my life.” And I said, “I’m no longer going to be your patient.” I’d just lost all confidence and trust in her. As a patient, it was very awkward – even being a nurse – it was difficult for me to let her know how I felt about what she did. In hindsight, I wish I would’ve taken the letter and reported her, because I feel like she probably did other patients a disservice with her “care.”
I did have the stem cell transplant at Northwestern Memorial Hospital in Chicago with Dr. Richard Burt.
After I got my stem cell transplant, I found a local rheumatologist willing to work with me and collaborate with the doctors at Northwestern. When I told him this was a must for me and asked if he was ok with that he said, “Absolutely, I would love the opportunity to learn more about scleroderma.” Still to this day, I see him. He’s always like, “I should be paying you, because you help educate me!” He’s now seen a lot more scleroderma patients and referred some to Northwestern. He was just completely able to leave his ego at the door for the betterment of his patients, where my other doctor was just trying to make a name for herself.
The whole situation really taught me to speak up for myself with doctors, and not just take what they say as gospel. It also taught me to do more research myself.
I feel like the biggest thing is: you gotta rely on your intuition. I think if people don’t have a good relationship with their intuition, they need to develop that. There are apps and books and online classes that help you connect with your intuition. I definitely use that a lot in my disease process – sensing what’s right or wrong for me.
I’m going to have to start advocating for myself with the OFEV a little bit, because I’m not happy on the OFEV at all. It’s just miserable. Nonstop diarrhea, nausea and lack of appetite. I’m not a fan of Imodium, and I take the smallest amount but it goes the other way…. I don’t really feel like I’ll be staying on the OFEV for very long. It’s a scary thing to consider, because my lungs have taken a big hit in the last year. It’s a difficult place to be. On one hand, I’m like, “Well if the OFEV’s gonna keep me alive, I guess I can tolerate the side effects… But it’s such a crappy existence – no pun intended! So I think I’ll have another big advocacy experience about that.”
I said something to my pulmonologist about systemic enzymes at my last appointment, and she was like, “I’ve never heard of that.” I highly respect her – she’s the woman who runs my whole show, but I talked to her about breathing exercises and she was like, “Well… it’s not going to hurt you, but it’s not gonna help you.” She’s just very old-school medical I guess and not very open about “alternative” treatments.
I did talk to that pulmonologist last time and said, “The quality of life is very important to me, and the OFEV has definitely hit my quality of life.” And she said that’s absolutely something I have to take into consideration. But I just know with things like that, you have to stand your ground with your doctors if you don’t have one that’s understanding or open to collaborating with you.
PF NOW!: Since you’re already in touch with your intuition, do you have any advice for yourself while you’re trying to advocate or speak up about this?
Amy: Good question. As much as I try not to be, I do still get intimidated by doctors. I think that’s just something I have to overcome.
Remember, the doctors are working for you and ultimately you’re the one making the decisions. I think you have to be firm, be brave, and be clear about your goal where your disease is concerned. Ultimately, it is 100% up to you to decide what direction your treatment goes.
I’ve already said if I’m faced with a lung transplant, I don’t know that I will do that. I’m only 54, and the mother of two, but I just don’t think that’s a path I’d want to walk down. Do I want quantity or quality? I must decide and then advocate for that.
The interview concluded with a conversation about PF NOW!’s List of Patient-Recommended Specialists, the challenges of finding the right care during COVID-19, and NSIP. We discussed what it’s like to have family or friends not understand the realities of PF, and assured Amy that we are here to support people like her. If you want help educating the people in your life, contact us.
Please comment below to thank Amy for sharing her story and wisdom!
One comment
Lee
Posted on October 27, 2022 at 4:55 amI appreciate hearing your story. I’m not sure I would choose to have a lung transplant either. There’s so much to go through. But It seems that you don’t associate transplant with improving quality of life. I don’t understand that, can you talk about that?