Background (written by Renee)
David met Jennifer McKenna on Day 7 of the It’s All I Can Do Run in Coeur d’Alene, ID. He was only 150 miles into the run back then. It is hard to believe how much has happened since, but the first days of the run are very memorable to both of us. Coeur d’Alene holds a very special place in our hearts; the support we found there was overwhelming.
Jen found our blog online and contacted us through it. When David arrived in CdA at the Holiday Inn Express, she met with him, and guided him around so he could understand the layout of the hotel inside and out. Jen has a flexible job, and David was able to call on her when needed, for any kind of assistance. It was a great comfort to me that she was so supportive of David.
In spending time together Jen told David what it is like for her living with cystic fibrosis. He asked her if she would do a write up for the blog. Here is what she had to say about writing her “dissertation”:
Hi, David and Renee! Writing this was weird, in a good way! I felt comedic, then sad, then self-conscious, then amazing, and then totally grateful and inspired by Dave and all the other people I knew who ever spoke out about CF.
In telling her story of what it feels like to be an adult with CF, Jen give it to us straight. She talks about going through denial, and then dealing with the anxiety and depression that comes with knowing that she will continue to suffer the effects of CF and live a shortened life. Her account is brutally honest and compelling.
Jennifer’s Story (May 26, 2014)
Hi, I’m Jennifer McKenna. I am 36 years old, and I have cystic fibrosis. Dave offered to let me write about what it’s like to live with this illness, and it’s my pleasure to do so!
I was diagnosed with cystic fibrosis when I was a few months old. My parents were concerned, because I wasn’t gaining weight, and I wasn’t a very loud crier. Mom said I kinda hissed, while most babies are supposed to wail. So, thankfully, my very loving parents took me to the doctor to figure out what was up, rather than just thinking they got lucky with a low-volume crier!
Let’s jump forward to what I can actually REMEMBER first hand, as a kid growing up. I grew up pretty symptom free! I am one of the lucky ones, who have a mild case of CF. One thing I DO remember, though, is that I had a CF child’s appetite! One of the symptoms of CF is overproduction of mucus in mucus linings, and because our intestines are mucus secreting organs, people with CF don’t absorb their nutrients as well as most people. What that looked like for me, as a 6 year old, was that my dinner servings were the same size as my dad’s! Friends would join our family for dinner, watch me eat, and tell my Mom, “Jennifer is such a good eater!” My mom NEVER had a problem getting food down my gullet –with the exception of enzyme powder. If you have ever had the displeasure of having pork-derivative enzyme supplements mixed into your Nestle Quik, you know what I mean! I would take nearly an hour to drink that thing down, before I could eat my meal. One day, my Dad tried to demonstrate how “good” it was, by taking a sip, and he almost messed it up by spitting it out! In a later conversation, he said the only way he got that mouthful down, was because I was watching him so closely. Mom and Dad didn’t like “forcing” me to drink something that tasted terrible, but they knew it was important for me to drink this stuff. This supplement helped break down the food, while it was still in my stomach. That way, when the foody mush hit my small intestine, some of the work that my small intestine would do was already started, so I could hopefully absorb more nutrients, during the time that the food was in my intestinal track. I am happy to report, though, that I graduated to being able to take capsules of enzymes, and I got to quit drinking that miserable powder!
So, other than the taking a lot of pills, I didn’t have a childhood that was any different from other kids. Well, I burped and farted a lot… and I had a reputation for some powerful bathroom visits. That wasn’t easy, socially –but really, I could play hard with the best of the kids. Thinking back, I can now notice how my lungs were starting to show more signs of CF, in high school. I hadn’t thought much about it, since coughing seemed so normal, by then. I think I got my first nebulizer sometime during high school. But I remember a lot of kids and even a teacher asking me if I smoked. I had a “good smoker’s hack.” Well, I guess being known as that was as good as being known for anything else, back then.
In my first year of college, I was working part time, going to school full time, and playing a lot. I was definitely “burning the candle at both ends.” I was getting pneumonia at least once a year, and pseudomonas infections more frequently than that. That’s when my mom and doctor really started asking me to acknowledge my disease. I fought my mom and doc every part of the way, because I didn’t want to admit my limits. In that year, I learned that I was supposed to be doing nebulized treatments twice a day every day. Of COURSE I didn’t do them! What was I missing, in doing that? I was missing regular treatments of albuterol and mucus-thinning Pulmozyme. These meds would dilate my airways, reduce inflammation, and help move nasty phlegm out of my lungs. What I ignored was that my CF was advancing and my lung tissues were inflaming. I wasn’t doing anything to help myself. I didn’t feel pain or discomfort, and I barely could notice when I was sick. I didn’t really believe my doctors or my mom, when they said I was harming myself through neglect. It wasn’t until my third year of college that I had my first episode of hemoptysis. Hemoptysis, if you don’t know, results in coughing blood. For CFers, lung tissues get inflamed, until a blood vessel breaks. The fastest route for that blood to go out is to cough it out. Fortunately, I had seen a documentary, and I knew this could happen to me one day. So, when I was first greeted with this crimson gore, I was shocked, but thankfully, I was not panicked. I finally had to admit that I was NOT like everyone else.
As poor as my decisions were, I want to explain something, to defend myself from myself a little: CF patients are not allowed to hang out together. It’s not because we’re jerks or our healthcare workers are jerks. It’s because our lungs are highly vulnerable to germs. We’ve got malnutrition, therefore weakened immune systems, PLUS our lungs are like petri dishes for bacteria. CF patients are not allowed to congregate, because we run a very high risk of cross-contaminating each other with our own bugs. We don’t sit in a room together, and talk about what’s going on. Therefore, we can feel ALONE in our sickness. Our families did not get the benefit of talking to other families who had to walk through this experience. In that vein, I didn’t see what was happening to other people, and I didn’t know what the road ahead would look like. Additionally, my family was like me –just trying to navigate with some really vague ideas and guidelines. I would love to hear that there exists a mentorship between families of CF patients, one day.
Let’s skip forward to today. I want to share how I am at 36 years old. I want to tell you my health status, the concerns of a 36 year old person with Cystic Fibrosis, and the hope I have in my future.
At this point in my life, I am hearing the awesome words of doctors saying things like, “You guys [Patients with CF] are getting older, now, so we see more long term effects of CF!” As I have stated, I have a mild case.
I work 40 hours a week in an administrative position. I no longer have the energy to run around the sales floor, like I did 2 years ago. However, I work this job, and I have enough energy left to work out and enjoy activities such as kayaking. I have been married for 9 years to an amazing man, Corey McKenna. We are considering having a baby this year. While I feel that I am not like my high speed low drag athlete friends, I still have a great life!
I don’t remember my numbers as far as lung functions. Honestly, I don’t have good perspective on what’s “good” or “great” in small airway and large airway clearance, peak flow values, or anything like that. From what I know about how percentages equal grades in school, I would earn a D or F in those areas! But I know I would have a C in total lung volume. I know that, because the doctor’s office has a fun game where you try to blow out birthday candles on a cake, and I can blow out 75% of the candles, on my very best days.
I have a permanent infection in my lungs, Mycobacterium chelonae. I contracted it in 1997. This infection became permanent through a series of unfortunate decisions by both me and a non-CF pulmonologist. I learned a valuable lesson – ALL lung issues MUST go through your CF specialist, no matter how great the other guy is. The infection appeared to go away, but it didn’t. Had I taken my problem to my specialist in 1997, he would have known to treat me aggressively right away, because this mycobacterium has a history of messing up CF patients. It came back with full force in 2005. With all the great efforts and help of my health care teams, it can’t be eradicated. I hear, though, that permanent infections are becoming more common, with adult CF patients.
Because of mal absorption, my vitamin D and Calcium levels are insufficient for a good skeleton. I now have osteopenia and osteoporosis in different areas of my body. My current treatment for that are massive doses of calcium and D, and high-impact exercise.
As a result of CF, I have another issue popping up, for which I have some understanding. It’s CFRD – Cystic Fibrosis Related Diabetes. My endocrinologist explained to me that my CF is changing the healthy tissues of my pancreas into fibroidal tissues. This fiboridal stuff is similar to scar tissue –useless. At this point, my pancreas produces about 30% of the amount of insulin as it should. So I can keep my blood glucose levels low, by just minimizing my carb intake. However, so long as I continue living, I will become diabetic, as the effects of CF advance in my pancreas.
Overall, my quality of life is still pretty good. I’ve got issues, but nothing so big at this time, that I can’t enjoy a normal amount of activity.
I mentioned earlier that my husband and I are looking at having a baby. Our decision to wait had absolutely nothing to do with my health. But as soon as we concluded that we wanted a kid, we had a lot to do, to consider how viable that notion was. CF patients used to be regarded as sterile, but then, as CF care advanced, more and more women with CF were becoming mothers –whether they were ready for that or not! Since I am pretty healthy, and because my mom jokes about how fertile she was, I’m gonna place myself in the category of “likely to get pregnant.” So, now that I’ve established that it’s a biological possibility, what does that mean for me? Broadly, my CF specialist explained that the babies usually do really well, but the mom’s health can suffer. If the mother gets sick, depending on the illness, the med she needs may cause risk to the fetus. For me and Corey, we didn’t want to put ourselves in the position to possibly make a VERY difficult decision. So we investigated further. What were the infections I struggled with all the time? What was likely during that 9 month window? What were the treatments that would keep me healthy, and would they harm a baby at any stage of pregnancy? Happily, with my history of infections and treatments, none of the antibiotics I use would cause harm. Also, I have not had a history of hypertension, which was a big concern for my perinatologist. Timing is also key, I learned. I developed asthma sometime after I hit 30. My perinatologist recommended that I time my pregnancy so that I am in my 1st or 2nd trimester during the cold months. December through March can bring temperatures in the 20’s and below, and breathing that cold air is very difficult for me. If I am approaching full term at that time, the baby will be pushing against my diaphragm, and I’ll have a lot of difficulty getting good air! We’re looking at discontinuing birth control methods in July.
Compliance: Prescribed Treatment vs. Reality
My health regimen, as prescribed by my doctors, is best presented as a list:
• High doses of vitamins twice daily
• 5 capsules of enzymes per meal
• doses of antibiotics 3xs a week, to keep permanent infections at bay
• nebulizer treatments twice a day, amounting to 35 mins. Duration of treatment
• 20 mins. Of therapy with my vest twice a day (I pair it with neb treatments)
• Advair inhaler 2xs a day (followed immediately by brushing my teeth)
• Monthly porta cath flushes
• Annual IV antibiotic therapy
• A minimum of quarterly visits with my CF specialist
• Minimum of 20 mins of weight bearing exercises 3xs a week
What I actually practice can be between 50% compliant and 80% compliant, most of the time. I have periods in which I am fully compliant. This is not good, and I know this. This has been a struggle for me, for as long as the regimen was introduced back in high school. As embarrassing as this can be, I am doing a disservice to myself and other people, if I don’t talk about the psychological and emotional battle of dealing with Cystic Fibrosis.
The Mind, Heart, and Spirit
First, I had a long period of denial. Let’s say this lasted… 35 years??? Denial, as we know, is bad and the denier looks like a selfish and reckless fool. Yeah. Let me say, though, what I got out of denial that FELT positive, even though it wasn’t good. I was able to pretend I was limitless and bulletproof. I pushed myself to go and experience good things in life, that maybe I would have reconsidered, had I paid attention to the damage I was doing. Like I said, it FELT good, but it really wasn’t. In retrospect, I could have slowed down some of the effects of my CF, by reworking my plans and allowing my body to rest. To give an example, I went to school full time, worked out 2 hours a day, worked retail part time at night, and then studied. On days off of work, I’d go pack that time with Judo and Fencing classes, get out at 10:00, then head to the restaurant with my friends, and be home at midnight. I would even study a couple hours AFTER that! I was keeping up fantastically with all my “normal” friends! I even wore my infections well. I was so used to being sick, that I could push through the coughing and low grade fevers, and perform with the others. This was bad! I noticed that, after college, my friends were all fine. But I was left with a lot of scars in my lungs. So what could I have done differently? Take the Fencing and Judo classes AFTER college, reduce the workout time, and cut back on socializing would be good. Or I shouldn’t have gone to college right out of high school. That, though, depends upon personal goals. My point is to prioritize, be discerning, and don’t be afraid to postpone some adventures.
When reality of my condition started settling in, I felt a lot of anxiety about my future. How long would I live? How long, until my friends quit including me in their plans, because I have limits that they don’t? What am I holding Corey back from, by being less-able than him? If I have a child, how old will that child be, when I die, and where does that leave Corey? These questions even spiraled down to “how long until I’m useless and my loved ones would be better off without me?” But here’s the great thing: My health condition is serious, but those concerns aren’t valid. Those thoughts were grounded in false beliefs. My friends love me, and they choose to spend quality time with me, whether it’s enjoying a meal together, or going for a walk. They may have to find another friend to go backpacking with, but that’s okay. I’m still their only Jen McKenna, and backpacking trips can never change that. Similarly, Corey chose to marry ME. He knew the ramifications of my illness, when he chose to propose! When I expressed these concerns, my friends and family wrapped around me and confirmed that I was treasured as me, for me, and not for what I could do. I cannot tell you how important it is to continue confirming for your loved one how they are loved and valued, as they experience changes in their lives. This is true for people of every walk of life, including those with CF.
The final piece of the puzzle for me was spiritual. I am the kind of person who does not do things, unless she wants to. I must agree with why I do it, and I must believe the reason is good enough, in order to “want to” do it. For years, I knew in my intellect, that I had to be responsible with my choices in treatment compliance. But deep down, I didn’t believe I deserved it. Why did I get to live this well, when I have behaved so recklessly? The truths are these: 1) I doubt that I deserve to live this well, but God has certainly granted grace in this area 2) I need to give the shame of my recklessness to God. I don’t need to carry that garbage in my head/heart/spirit any longer 3) God knew what He was doing, even before I was born. I was made purposefully, thoughtfully, and perfectly according to His plans. My response to Him, upon this realization, is to make the best choices as I possibly can, in order to care for the body He made for His glory and according to His purpose. Okay… so, what can be more important than to care of a part of God’s plan?! That seems like a “good enough” reason for me to “want to,” now.
Why I Need Champions
After disclosing some of my heart-journey with CF, you may have concluded that I don’t like to look too closely or too long at my illness. Of course I want to be cured. But thinking beyond my daily responsibilities about living with CF and wondering how it’s all gonna play out for me is too depressing. Thankfully, there are bold and compassionate people who are willing to pick up that fight, when I cannot! I call them my champions for the cure for CF.
David Kuhn is definitely a champion. It takes an extraordinarily determined, hopeful, and loving man to look at his granddaughter’s condition, and then take on responsibility to do “all he can do” to brighten her future. During this massive feat of running around the country –and doing it blind, David is very vulnerable. He is thereby taking great risks just for the HOPE that funds are raised, and that Kylie can benefit from the medical advances that follow these contributions. At the very least, Kylie will be proud of her grandpa and witness this act of love for her. She’s also gotta know that he’s got a LOT of admiring fans and we are all watching for her cure!