Last week I drove out to my parent’s house, the house that I grew up in, and sat down with them, at the table where we'd had hundreds and hundreds of dinners and conversations as a family, and interviewed them about what it was like to have a child with CF. Even though I had always felt like my family was very open, and not afraid to talk about anything, it was really the first time we'd had a conversation about my CF, as adults.
I expected to learn things I hadn’t known before, all the things that my bad memory had lost along the way, but what followed was an almost two hour conversation about what it was like in those early years when so much was still unknown. After spending hours listening to the interview over and over again, transcribing it, picking it apart, and cutting it down to a reasonable size, I felt like I finally had the missing pieces to the story I'd been telling myself for years.
(To listen to the full interview scroll down)
(Laughter) First can you say a little about yourselves? Mom why don’t you go first.
My name is Debra Maloney-Evans, I’m Erin’s mother. She is one of three children, the youngest of our three, she has two older brothers. And I’m a nurse practitioner by trade, or profession I should say. And Peter, how about you?
I’m Peter Evans, I’m Erin’s fathers, I’m ...62? (Laughter) I’m about 62. I recently retired as a high school principal, and was a Vermont educator for 35 years, and I still do some work in education as a consultant.
E- How old was I when I was diagnosed with Cystic Fibrosis?
D- You were 15 months old, it was November 1984 when you were diagnosed with Cystic Fibrosis (CF).
P- Right before Thanksgiving.
E- Did you know what CF was, had you ever heard of it?
D- I don’t think I had ever heard of it, I can’t remember exactly but I don’t think I’d ever heard of it.
P- I might have because I think we had a student in the school who had it.
D- That’s right I remember you talking about that student.
P- But I didn’t know much about it and had no real familiarity.
D- And I was not yet a nurse so it was just was not on my radar.
E- And where were you when you found out?
D- We were in Northfield, I was sitting at the table we’re at now. They called me on the phone.
P- We'd had the sweat test a few days earlier and we were counting on getting some information back about that. And I think you had someone over here, I think you had a friend over here making cookies or something, Boo Smith.
D- Yeah, and you (Peter) were at school.
P- Right, I was at school, and mom called me. The secretary called me over the intercom to say that mom had called and had wanted me to call her back. So I knew that something was up.
E- What were your reactions (to finding out I had CF)?
D- I was- I can’t even describe my reaction I was… it just felt like I had been kicked in the guts, and couldn’t breathe, and couldn’t talk.
E- So even though I’d had a sweat test you still weren’t sure?
D- That was the definitive test. We were told that you might have an immune deficiency, or it could be Cystic Fibrosis, and the doctor said with absolute confidence ‘I don’t think it’s cystic fibrosis, but under the circumstances, we should test for it.’ And probably in those few days I’d had an opportunity to do a little bit of research to know enough about CF to be very concerned about it being that.
P-The only information we had was from books and I don’t think they were really up-to-date books, they were probably fairly dated, and I think at that time it looked pretty bleak.
D- I was so glad that Boo Smith was here because she was such a great support, she was a nurse.
P- So we went immediately up to the hospital to repeat the sweat test because they didn’t diagnose it with just one, they needed a second.
D- And then we began a bunch of teaching for what kind of treatments and things you would need, you weren’t sick at the time, it was just that was the best place to do it.
D- Yes. First of all you had to take enzymes for the first time. Here you are fifteen months old, and we had to sprinkle them onto your applesauce.
P- Yeah, it was very different... You were too young to swallow the capsules so we had to break them up.
E- And what else?
D- Chest PT, twice a day.
P- Generally, we were just super aware of every little thing that was going on.
D- That was a big adjustment. And at the same time I felt like I was struggling with depression, and just, my world sort of being turned upside down.
P- You were taking, was it Anatomy and Physiology in White River Junction, or some place like that, it was for your nursing program.
D- Right, I had started Anatomy and Physiology when Erin was about 12 months old, and I was at the mid-term, because it was a two semester course, and this all happened and I contacted the professor and said ‘I can’t do the exams, I’m just too busy with this, and I don’t know if I’ll come back to nursing’. And he was really great, he said ‘Take all the time you need, when you’re ready we’ll do the exam, and then you’ll have some time off to think about whether or not you want to come back to nursing school.’ And he said ‘I really hope you’ll come back.’
E- What was the best advice you got from either a doctor, family member, or friend regarding CF?
P- I think Dr. Swartz probably gave us the best advice, and that was when we were in the hospital, we were meeting with him right after your second sweat test and he was very honest and said ‘You know, I can’t tell you what the future holds but don’t wait to do anything, don’t put off doing things as a family, and don’t spoil Erin.’ I remember him saying-
D- ‘Treat her like your other kids.’
P- Yeah, he was great.
D- He said, ‘Once in a while, she may need a spanking.’
P- Which of course we never did. (Laughter)
D- I remember a few other pieces of advice, Chris Beatty Cambria who we went to high school with, was a physical therapist at the time in Boston, and she wrote to us or maybe we talked on the phone about this, but she was actually doing home chest PT on kids in Boston with CF, and she said ‘The ones who are the feistiest do the best,’ and so I said ‘Well, looks like she’s going to be alright then.’
P- I remember when we came back from the hospital, Jack saying to us, how old was he? 5 or 6? I remember him kind of wondering if Erin was going to die. Remember that? And we were like ‘Whoa’. We explained to Jack and to Ross as much as we knew, but not everything, and he was obviously very concerned about that. I remember he asked right there on the stairs. So yeah, he was thinking about it.
D- Another thing Dr. Swartz said, this was probably at another appointment we had, we were crazy about keeping you away from anyone with a cold and anyone who was smoking, and of course most of my family at the time was smoking and I didn’t know what to do about it. We couldn’t go to family things because everybody was smoking. So I asked Dr. Swartz about it, I told him my father smokes and I really don’t like taking her down to his house because of that. And he said ‘She needs to know her grandfather. Ask if maybe the smoking could be outside.’
P- Well, we never said anything and they never smoked in the house when Erin was around. Remember that? I mean your father just never smoked in the house, and I don’t think anybody else did either.
D- Yeah, well that was just a big concern for us, and he just said ‘You know it’s a concern, but don’t keep her away.’
E- What were your friends and families reactions to finding out about my CF?
D- They were supportive.
P- I don’t think any of them, with the exception of maybe our friend Chris (and Debbie) knew anything about it. But our family as far as I know really didn’t have any understanding of what it was, and they probably did what we did which was go to a book and in some cases the books were probably really old books and it was spelled out very dire. I think it was difficult for them because I don’t think they really knew what to do, because it’s an unusual disease, it’s not something you hear about all the time. But the Natvig’s were very supportive, Dwayne and Nancy both, Craig and Cinda, Debbie. We were surrounded by some really knowledgeable people which was really helpful.
P- Well, I think they knew what it looked like, or what it was like from the reading they did which was probably pretty limited, and she didn’t fit that. She was chubby, and happy, and healthy, she had a runny nose all the time but, yeah.
E- Do you remember what the life expectancy was that they gave you?
D- I think at that time it was maybe 12, 14?
P- Yeah, it wasn’t very old, definitely 20 would have been rare.
D- And then the gene was discovered maybe five years after you were diagnosed, which was a huge break-through.
E- Do you remember when I started to take responsibility for my own treatments and medications?
P- There were a lot of little steps in that.
D- Initially when you started school, even in kindergarten, after we talked to the school nurse and got a note from your doctor, you were allowed to carry your enzymes with you, so at lunch and snack you had to take your own enzymes.
P- We set them up in apple sauce, in a little container, so it wasn’t like you pulled a pill bottle out. But I remember we had to fight a little to get that worked out because the policy was that all medications had to go to the school nurse and we felt that that was something you could be responsible for. The thing with you is you were always so healthy, you always sort of managed your own enzymes, there were never antibiotics unless you got a cold that turned serious. At a certain point we stopped doing Chest PT because you weren’t sick, and that was the system.
D- The doctor said that the research had changed and we didn’t have to do chest PT unless you had a cold and were coughing more.
P- I think probably when you went away to college that was when, I mean you had always taken responsibility for CF yourself in my opinion, we felt good about that. We were always there to monitor and suggest, but I think when you went away to college, we hoped we had set the groundwork for you to be able to do everything yourself.
D- I think you stopped taking things didn't you?
E- Yeah for a while my CF doctor said that I didn’t need to take enzymes, so there was a period when I really wasn’t doing anything. I think that was in high school.
D- I think when you went away to college in Maine you had things you had to take, and you had a flutter, which I don’t think you used. And the first time you got sick up there, I felt sick, I just felt sick being so far away.
P- Your roommate, Aliza, she called and we talked to her and she said that you were really sick, she was really worried about you.
D- So the school health person talked to your doctor and got antibiotics started. But I honestly think you were probably about 25 when you really took over.
E- Yeah, I mean I didn’t go into the hospital until I was 25 I think, and it wasn’t really until then that I started doing the vest and everything else.
P- Yeah, we noticed a real change. In all of us, because I think up until that time, before you were hospitalized that first time, we were very happy with the way things were going and felt like we could continue going like this. That first hospitalization was the wake-up call, and clearly it was for you too.
E- What were you thinking that first time I went into the hospital?
P- Ugh. It was awful.
D- Yeah, it was kind of like the diagnosis all over.
P- It was, until we got into the hospital and saw you, and realized you were starting to get better pretty quickly, and it took a couple days, but you got better. And there’s such a positive atmosphere there. But the realization that you had to be hospitalized, I agree it was like the initial diagnosis. We didn’t have any of that hospital experience with you when you were little, because you didn’t go to the hospital, it was very unusual.
E- What do you think the main reason for my being so healthy was when I was younger?
D- I think it was multi-faceted but right from the start, the fact that you were breastfed I feel was a huge advantage. You were never a failure to thrive, which a lot of CF babies are because right from the start they need enzymes. I think breastfeeding gave you an advantage because you didn’t have to digest those kinds of fats and proteins that are difficult for infants, and you were getting immunity from me, so I think that was a huge advantage.
D- And playing the trumpet.
P- Keeping up with the boys. The boys were involved with everything, I think you were involved with everything as a result. And just being outside, running around, swimming. I think the exercise and getting you engaged in activities like that from a very early age was really important. I’m still amazed that when you graduated you were the only girl in your graduating class that played a sport every semester for all four years. That was very amazing.
E- Were there things you wish you had done differently when I was growing up regarding CF?
P- I wish there had been more opportunities to talk to you about CF, like in high school. I just never felt like there were opportunities, and I don’t know if you were interested in sitting around talking with us about it. I think we were thinking a lot of things, but it was hard to find an opportunity to include you in those conversations. I don’t know if it could have been any different, but I wish we had talked more about the realities around CF. I think we shared a lot of that between Debbie and me, but I don’t think we talked to you about those things.
D- I would agree, I think we worried that Erin might need to talk about her feelings about having CF and how it was affecting her life. I mean I tried some to engage you in conversations like that, but you wouldn’t bite.
P- Yeah that’s true, I do remember that. It was painful. (Laughter)
D- There was a point where we thought you were depressed and we had you see a therapist in Montpelier and thinking ‘Well, maybe she can’t talk to us, but maybe she can talk to this woman’, and you went willingly, for quite a few sessions. So I don’t know, I don’t know if that was helpful or if you had no idea what you were doing there. (Laughter)
P- Do you remember that?
E- Yeah, kind of. I remember liking getting out of school to go to Montpelier. (Laughter) I think I remember talking to her about CF, but I think it was more like this woman asking me questions about it, I don’t remember wanting to really talk about it though.
How did you see my CF affect my life? Or were you unsure?
D- Probably unsure, you kept your emotions very tightly held. You know, you were social, you had lots of friends, you played sports. But I guess I thought that there was probably a part of you that wasn’t coming out, that wasn’t being expressed, and I don’t know if you expressed things like that to your closest friends like Essie or Britt, but I always hoped you had some outlet for it because I thought it might be necessary.
P- Looking back now, I imagine you were doing your own research, and reading about it, and maybe finding things out that you didn’t know about the prognosis and the disease.
E- What else has my CF taught you?
D- Lots. Appreciating time together with family. Appreciating life.
D- I feel very fortunate in lots of ways. That I had support, from you (Peter), and from grandparents, that you (Peter) always had a job that had great insurance. We know of families back then before there was a lot of other help, that this was financially so devastating. We never really knew how much medications cost because you don’t see those things when you have insurance. Hearing that it’s like $16,000 a month or something for treatments. So, appreciating that really good health care system and that we managed to have that because we had the right jobs.
P- Right, we were fortunate enough to have that because of our work. Yeah, I think we’ve said this before, but I think the positives have far outweighed the negatives, on our account, on our front. I don’t know how that’s been for you. You’ve only ever known CF. But I think the positive aspects have been fairly heavy in terms of you having CF.
E- What do you think is the most important thing about raising someone with CF? If there was one key thing you would say was important?
D- Treat your child like a normal child with CF, not a CF child. So don’t ignore all of the rest of them, don't exclusively think of them as, you know, a child with CF. We had really good support from Dr. Swartz and Dr. DiNicola. When we saw Dr. DiNicola he would say ‘Now CF aside, tell me all about Erin otherwise. How is she developing?’ He helped us to focus on that and Dr. Swartz was an expert at the CF part, and they worked well together.
P- I would agree. I think with a disease like CF, it can become who you are, and I think one of the things that is really critical is this not become who someone is, the most important thing is it’s an individual, it’s a human who has this as one of the many things that they have to struggle with, but that this isn’t someone’s identity. It’s hard because there has to be such a focus on it so much of the time.
D- Or a reminder, every day.
P- Yeah, but I think the important thing is treating kids with CF as normal as possibly. Not have them take on an identity as someone with this chronic illness.
E- So I’m 31 now, which is how old you were when you found out I had CF, did you ever think that I would live this long, obviously now you realize that, but when I was younger did you?
D- I tried really hard not to think about that. You were proving yourself to be so healthy in so many ways, every year it seemed like the mean survival age for people with CF was going up and up. I had never met any adults with CF and I was working at Gifford in the birthing center, I worked 3-11 and a new nurse was hired for the 11-7 shift. We would cross paths, me going, her coming into work. She had talked to the other nurse who was on her shift and found out that you had CF, and she shared that she had CF. So one time when we crossed shifts she pulled me aside and told me she had CF, and it was like- I couldn’t believe I was looking at someone who was 30-something years old, working. I don’t know if she was married yet or had kids yet. She told me her story which was that she was in nursing school, she was like 19, when she was diagnosed. Dr. Swartz had actually told us about this case, he hadn’t put any names on it, I think it was pretty unusual for him too, to see a 19 year old just being diagnosed, and I just sort of thought anything was possible for you.
P- I think the goal was high school completion, and you did that without a whole lot of struggle, and then college, and you did that, so I mean way back I don’t know, I think we tried not to imagine what your life could be like, we tried to imagine what we were going to make it like. So in some ways having you be 31 is just amazing, and in other ways its like ‘Yeah, why shouldn’t you be?’
D- I mean now we know of people with CF who are in their 70’s. And 80’s! You know one of the best things I think we ever did was go to that CFRI (Cystic Fibrosis Research, Inc.) conference in San Francisco. To me it was so eye-opening, and to hear those physicians from around the world talking about what was going on with research. And to have you see other people with CF, I remember thinking ‘this is good, this is good for her to see people who are in their 40’s’.
E- So much has changed since I was diagnosed with CF, what advice would you give parents today who just had a child diagnosed with CF?
P- There’s so much more information but I don’t know if I would advise people to start looking at the things on the internet about CF.
D- We were advised to always read current information, not to read books that were 3 or 4 years old and more, because things are changing so quickly. I would tell them our story if they wanted to hear that, I would tell them what we said before about just trying to create as normal a life as possible for your child. And to take care of yourself.
P- Yeah forming a really strong relationship with who ever it is at the clinic is really important, make yourself known there, but not be overly aggressive. They’re there to help you. And I think having a kind of pause in terms of the whole family thing, kind of setting some goals as a family, and working toward those goals. And not to be so prohibited or limited by the fact that you have a child with CF.
Listen to the interview here where you get to hear it in it’s entirety, as well as all the outtakes, bloopers, hysterical laughter, inside jokes, and the many, many times my parents finish each other’s sentences.
Erin Evans is the program coordinator for CFLF. She was diagnosed with CF at 15 months old, and is now 31. She was born and raised in Northfield Vermont with her two older brothers, Jackson, and Ross. She now lives fifteen minutes from their parents and the house that she grew up in, and thirty minutes from her brothers. She can be contacted at: erin@cflf.org
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