Sugar Sugar

Having grown up in the 80s and 90s when there wasn’t as much known about CF and people with it weren’t living as long, the priority was gaining/keeping on weight. My whole childhood my parents were told to get me to eat as many calories as possible. They were told to keep candy in the house at all times so that I could eat it any time. Based on what we know today this sounds pretty terrible, but that’s just how things were done. Fast forward some years and I’ve spent the majority of my life eating anything & everything that I want. Unfortunately, I also happen to have a very strong sweet tooth and very little will power.

Blood tests are a regular part of clinic visits. They check vitamin levels, liver functions, and sugars, among other things. The last year or so, my sugars have been running at the high-normal end, so this summer my doctor had me do a fasting 2-hr blood glucose test. This entails a blood draw, drinking an orange flavored, super sugary drink, then drawing blood again at one hour & two hours to see how the sugar is absorbed into the body. I was able to find out my results during my visit that day as I had clinic scheduled at the same time. Well, my doctor read the results and said that I was officially at the high end of pre-diabetic. Cystic Fibrosis Related Diabetes (CFRD) is different from Type 1 or Type 2, but can be treated with insulin. Since I didn’t have diabetes just yet, I met with the nutritionist and went over how to control my sugar levels with diet. Ugh.

I had to give up all candy & soda and start making sure that every 2-3 hours I ate a balance of protein and carbs. My body still needs sugar, since my levels also tend to get really low, it just has to be the good kind. Eating all of the time wasn’t a problem since I pretty much snack at my desk all day long anyway, but having to cut out the junk food was awful. I craved it so bad. It doesn’t really help that the kitchen at work is stocked with candy & we have a soda fountain. They sat there and stared me down every day at lunch. And I have to say there were a couple of days that I went and stared longingly at the pantry shelves and had to talk myself out of having “just one”. I was counting the days that I’d gone without junk food and congratulating myself every day on a job well done. After about 7 days it started to get easier and I found some substitute things to eat that sort of satisfied my sugar needs.

At about this time I got my test results in the mail and come to find out that my doctor read the 1 hour test as my final instead of the 2 hour test, so my results were that I’m actually high-normal, and not even pre-diabetic at all (yet). Well I tried to convince myself to continue eating healthy, but my aforementioned low will power took over and I ended up saying a giant fuck it and started back on the candy & soda. All of it.

I'm making sure to drink at least 4 liters
of water a day, along with making
good decisions on my snacks.

Now it’s a new year and I’ve decided that I really need to get back on the no junk wagon. I’m pretty sure my body will thank me in the long run, but it’s still not easy. A little easier since it’s my decision and not forced upon me. I really don’t do well with being told that I have to do something, never have. 

I’m drinking a literal gallon of water every day and substituting all of my usual candy with fruits, veggies, nuts, cheese, and popcorn. I still have bad days where I want nothing more than to drink a root beer and stuff handfuls of Cracker Jack in my mouth, but I know that if I give in I’ll end up telling myself that I can have one thing every day and then it will spiral out of control again. So for now I eat with my back to the candy-filled pantry and pretend that my sugar snap peas are of the sour patch variety.

Technically I'm a Mutant

Cystic Fibrosis is caused by genetic mutations, so technically I'm a mutant, but sadlyI don't have any super powers (unless you count turning into a human salt lick after a work out or being able to swallow a handful of pills without choking).

In order to inherit CF a person must get one defective (mutated) gene from each parent. A person with only one mutation is a 'carrier' of the CF gene. When two carriers have a child, there is a 25% chance the child will be born with CF, a 50% chance a child will be a carrier, and a 25% chance the child will not have CF and not be a carrier. Thank you high school biology!

There are roughly 1900 different mutations of the CFTR gene that can cause CF, which means that there are millions of different combinations of mutations. Since every mutation presents differently, you can see why CF is a difficult disease to treat. We're all super special little snowflakes that need individualized care to be at our best health.

My specific mutations are Delta f508 and G542X. Delta f508 is the most common one. About 90% of people with CF have this (or two of this) mutation. G542X is the second most common with about 5%. According to a chart I found published in 2012, there were about 2,100 CF patients with my same mutation combination, out of roughly 70,000 CF patients worldwide. So essentially I'm super duper special!

For more information on CF mutations or more specifics about the science behind CF please visit CFTR.info or cff.org.

The Diagnosis

Last week there was a story on the CF Foundation's facebook page about a woman that was diagnosed later in life, after a decade or so of not knowing what was causing her symptoms. Now days most people with CF are diagnosed either by a newborn screening (I think I heard that all states test for CF at birth now) or shortly into toddler-hood. Well it got me thinking about the story of my diagnosis.

I wasn't diagnosed with CF until I was 5. August 20, 1985 is the official diagnosis date according to my medical records, yep exactly 31 years ago today! Happy CFAnniversary to me ;)
I don't remember a whole lot about that time, so I asked my mom to tell the story:

My family circa 1982/83

"A friend had heard a radio announcement in 1982 about a "kiss your kids" campaign to detect a salty residue on your children to get them tested for cystic fibrosis. That is the first time I had ever heard of this disease. I took my beautiful little girl to the doctor to ask if he could run this test, because I had been detecting a salty taste on her face when giving her kisses. He said I was a neurotic first time mother and there was nothing wrong with my daughter. He was a doctor, I assumed he was right, and never gave in to my gut to force the issue. Jennifer continued to be the light of our lives and be our delightful baby girl, but she was sick frequently, had a hard time gaining weight, and always had"unique" bowel movements. We were blessed with another beautiful baby girl, and we grew as a family, moving a few times in those short years, as oilfield workers often did. I was taking Jennifer to the doctor for her check-up to get ready for kindergarten, and asked about her tummy......she always had a distended stomach on such a little body. He suggested we do a sweat test. I didn't know what that was, but said lets do it. My sweet neighbor, who worked in the hospital lab we went to, asked if I knew what that was for, and since I didn't, she informed me it was to test for cystic fibrosis. Well, I went to the library and looked up everything I could on CF.  Read everything I could get my hands on, and we cried and prayed more than I knew was possible. The tests came back positive, and our doctor sent us to the Children's Hospital in Denver, Colorado to the CF clinic they had there for further testing on her and her little sister.  Well, three years after my initial "neurotic" request, we found out my sweet little girl did have cystic fibrosis. I still kick myself every day that I didn't force the issue with my first doctor.....I let my daughter down, and I will always regret not standing up for her! I am so thankful for all the progress that has been made in the medical field. I'm so proud of my beautiful daughter for taking care of herself, and so thankful her husband is so caring and understanding and loving! I still pray for a cure and a miracle every day!" 

Me, Mom, and my Sister

My younger sister does not have CF, thank goodness. Neither of us can remember if she is a carrier, or if she was ever tested for that after the CF mutations were discovered in 1989.

As far as I know I am actually the only one in my extended family that has (or had) CF.

Pills, Pills, and More Pills (plus a bunch of other stuff)

 This photo represents the typical amount of pills that I take in a day. Included are my vitamins, acid reducer, and pancreatic enzymes. I also threw in something for headaches since I end up taking that on most days :/

My enzymes (specifically Zenpep 15 for any curious fellow CFers) are the bulk of this since I have to take them whenever I eat. How many I take depends on how much fat is in what I'm eating, but on average I do 2-4 with a meal or snack. The enzymes have improved a lot since I was a kid, at one point I was taking 7 with every meal. My acid reducer also helps increase the functionality of the enzymes as well as let me get through the day without crazy heartburn. TUMS were my best friends for several years, now omeprazole is.

Since my pancreas doesn't work properly to pull the nutrients out of my food I need to take additional vitamins so I can make sure that I'm getting enough. Low vitamin levels are pretty common in CF. There are some vitamin brands out there like AquADEK, that combine a bunch of them into one pill, but I've found that those come back to haunt me (and anyone around me) all day long so I prefer to take them individually.

In addition to pills I also have to use a nasal spray to help keep the inflammation in my sinuses down, a sinus rinse to keep them gunk free, and a saline solution that I inhale using a nebulizer to help loosen the mucus in my lungs. Oh and I almost forgot about my bronchodilator inhaler, I use this prior to running/exercise to help open up my airways (or if I'm just having a bad, wheezy, lung day).

I'm no stranger to antibiotics either, as soon as I know a respiratory infection has started I'm on the phone with my doc to get a round to help clear it up. I don't love the idea of taking antibiotics so often, but I do love being able to breathe.

I know this seems like a lot and slightly overwhelming, but keep in mind that I am on very few medications compared to most CFers out there. Maybe one day I'll have to give up an entire closet to my medicine cabinet, but for now a couple of baskets stashed in the kitchen and bathroom will do.


**I have tried using essential oils instead of antibiotics (my last employer sold them, so I got well versed in all that I needed to try), but I found that they didn't quite work like the miracle cure that they were being sold as. I do still use them, but more as a supplement to what I already do.