Clinic Visit, New Meds, and a Pity Party

I know they're for everyone's safety, but
I really hate these masks.

I had a clinic visit yesterday and had intended to write a full post about what is involved in them. Turns out my visit was a pretty simple one. I didn't have to do most of the normal tests or see most of the usual people, they didn't even to a blood draw. Way to ruin my intended blog post guys!

However, one test that never gets skipped is the PFT (pulmonary function test). This measures the capacity of my lungs to see disease progression. Growing up my FEV1 was always about 115%, but over the course of the last several years it has gone steadily down to about 83%. My functions have been stable for the last 18 months, which is good since I don't want to see the decrease keep going. But I had my hopes up (probably a little too much) that between running and doing my vest & hypertonic saline nebulizer on a consistent basis I would see an increase. I asked my doctor if it's even possible to see an increase or if this is just going to be my new baseline. We decided to try adding Pulmozyne to my routine to see if that will help bump my numbers up.

Pulmozyme is one of those medicines that most CFers get put on, kind of like digestive enzymes,but I've never needed it since my lungs were so good. I'm pretty bummed that I have to do this and that everything I've been trying isn't helping. I can be pretty competitive and controlling, so I'm feeling a little defeated that I need to start this new med. Not to mention the fact that I now have to get up about half an hour earlier in the morning to do it since it's a nebulized drug. I'm still waiting on insurance/specialty pharmacy to get it, but hopefully it will help and I'll see a rise in my number next time.

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.

jigglyknits

Since my lung functions have started to go down and I spent all winter being sick, I decided that it was time to really take action and start using my vest for airway clearance. Up until this point my doctors have said that my vest is just 'use as needed'. Well as I was gathering up some knitting supplies so I had something to do while using my vest, my husband said "why don't you sell whatever you make and donate the money to CF research, you can call it jigglyknits". (we have always called my vest the jiggly vest, since, it shakes so much while its working)

I kind of brushed him off and went on with my treatment, but as I sat there the idea started to percolate and it sounded like a really good way to raise awareness and help raise some money for the CF Foundation. I started an etsy shop and now I put everything that I make while doing my vest treatments on there. I've sold a few items and I can say that it feels pretty good to send the profits to the CF Foundation!

I created a facebook page & Instagram account to help advertise jigglyknits, which has then led to writing this blog. I never used to really talk about my CF as I was able to hide it pretty well from anyone that I wasn't super close to, but doing all of this over the last 6 months has led me to realized that if I don't talk about it, then I'm really hurting everyone that has CF. There are only so many of us, so the more that it's talked about and the more people are aware of it, the better chance that someday there will be a cure.

To own a jigglyknits creation of your own, you can visit my etsy shop or you can donate directly to the Cystic Fibrosis Foundation to help fund research to cure CF.

It's been a pretty crazy summer with travel and visitors, and I actually haven't really been doing too much of my vest. It's still sort of habit to not have to do it. I was moving all of my equipment around the house depending on which room we had guests in which made it easier to talk myself out of using it, so this weekend I organized the spare bedroom/craftroom/office to have a dedicated jigglyknits corner. I'm pretty excited about it and am ready to be able to add more to my shop!

Getting to Know Other CFers

Cystic Fibrosis can be a very isolating disease. Infections can be passed pretty easily between CFers, causing it to be dangerous to be together, so there is a rule that we aren't supposed to be within 6 feet of each other (the exclusion to this is siblings, since they are around each other all the time and most likely have the same bacteria). Because of this we can't have really support groups or friends that know what you're going through. At clinic visits they hurry us through the corridors and all patient rooms are kept closed. The only way you can even tell that other CFers are there is by the sound of coughing coming through the walls.

Instagram posts - I like to keep it a mix of
day to day life and my jigglyknits items.
You can follow me @jigglyknits

It is a lot better now with the internet and social media, us CFers can chat, commiserate, and offer advice. A group is starting a YouTube channel where they'll post videos about living with CF and another group is putting on BreatheCon this year (all going to be done digitally of course). I share pictures of my everyday life with CF and my jigglyknits creations on Instagram and follow several others with CF. I have to say I get a bit excited with every new follow.

While it's great to be able to meet and get to know other CFers there is a down side. Before, when I had never met anyone else with CF, I only knew my story and my experiences living with CF, which I can't stress enough are not that bad. Now, I see all of these amazing people that are fighting so hard and have low lung functions, and are in and out of the hospital constantly, and it's painful to see what is probably my fate. It's hard to say because I have been so healthy and only hospitalized once, but I do know that it's a real possibility as my lung functions have started to decrease over the last few years. It can be extremely depressing, but it can also be very inspiring. It makes me want to run every day and eat right and take care of myself to stay healthy for as long as possible. It makes me want to live each day to the fullest and not let my CF get in the way. So thank you to all of the other CFers out there that inspire me everyday to be better in every way!

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. 

Let's Talk About Mucus

Boogers. Snot. Phlegm. Mucus. Spit.
Whatever you want to call it, someone with CF has a lot of it. Without getting too sciencey, salt doesn't stay in our bodies like a "normal" person (that's why our skin is super salty, but more on that another time), which causes our mucus to be thicker and stickier.

This thick, sticky mucus is harder to clear out of the lungs and is a great place for bacteria to grow, causing infections, that leads to scar tissue. All of this causes our lung function to decrease and it gets harder and harder to breathe as time goes on.

Mucus is found in many places in the body; pancreas, sinuses, cervix, etc. Anywhere that it's found, it will be worse in someone with CF. That's why many of us have to take supplemental enzymes when we eat. Our pancreas doesn't work properly due to the increased mucus and doesn't digest our food. Woe to us when we forget to, or just don't, take them when we eat something (again, that's a whole story for another time, yay, a poop post to look forward to!). Women with CF tend to have a harder time getting pregnant due to mucus plugs in their cervix, and most men with CF are infertile.
Chronic sinus infections (sinusitis) are also a common occurrence with CF.

Growing up, I didn't have any lung issues at all, but have definitely had my share of sinus issues. I had surgery in 2009 to open up my sinuses and get out all of the gunk that was jammed in them, they were totally blocked. They've stayed pretty clear since, other than major inflammation, but it's not uncommon for a CFer to have a sinus scraping surgery on a regular basis. And I've heard that sometimes the sinus cavities are 'obliterated' to close them off to future infection. I actually didn't even develop the one above my right eye, so lucky me, my sinus headaches are concentrated on my left side for the most part.

With CF, you learn to not be too squeamish about what comes out of your nose or mouth, as a single cough or sneeze can get you a sleeveful of goo. You also learn to inspect it for color as that is an indicator of an infection (yellow = bad, green = good). I can get up close and personal with mine til the cows come home, but if I even see someone else spit a glob out from a distance my gag reflex goes crazy.