Tuesday, 11 September 2012

What is this cystic fibrosis thing?

Susie meets Johnny.

They fall in love. They make a baby. Everything is perfect!  

Or is it?

They notice the baby's skin is very salty. Salty kisses...very salty. They become concerned as their baby doesn't seem to be digesting her food. She spits up a lot more than they think babies should and they are going through gazillions of diapers. They also notice that their baby is not thriving...not growing or gaining weight like babies should.

They become terrified when she won't stop coughing and is gasping for air...

Finally a diagnosis.

Cystic Fibrosis.

So what exactly is this cystic fibrosis thing?

Cystic Fibrosis is a genetic disease. It is not something you catch or develop...you are born with it.

About 1 in 25 Canadians are CF carriers. If you create a baby with another carrier, that baby has a
  • 25% chance of being born with cystic fibrosis
  • 50% chance of not having CF, but being a carrier
  • 25% chance of not having CF and not being a carrier

CF is diagnosed by an easy sweat test - measures the amount of salt in the sweat. If this is inconclusive then a genetic test is done.

How does CF affect the body you ask? 
  • Lungs - Inflammation and infection damage the lungs (hence me waiting for new ones), difficulty breathing (hence the Darth Vader effect), coughing (hence me being a pro)
  • Pancreas - pancreatic insufficiency means we don't digest our food properly and we need to take enzymes to do the job for us (cause we're lazy) 
  • Liver & Gallbladder- Liver disease is the third most common cause of death associated with CF. Secretions can clog up the bile ducts of the liver and gallbladder. My gallbladder left me in 2008.
  • Intestine - let's leave that one alone, k? 
CF brings other complications to the party including sinus problems, arthritis, liver disease, osteoporosis, pancreatitis, diabetes and more. Nothing but good times.

For those of you who want to know even more, you can consult a good friend of mine: wikipedia. Or, of course, Cystic Fibrosis Canada.

***CF affects each person in differing degrees of severity. Not all patients have all complications. Each patient goes to a CF clinic and has their own personal plan for treatment.***

My CF treatment includes, but is not limited to, the following:

Aerosols that I suck back...
...and the junk that goes inside
What I puff on
1 day supply of meds
Due to having low lung function, plus burning a crap ton of calories by simply breathing, I also have a tendency to lose weight quickly and it's nearly impossible for me to gain.

I'm sorry, I can't bottle that and sell it.

Because of this, I had a g-tube (feeding tube that goes into my stomach) installed over 2 years ago. It provides me with an extra 1175 calories per night. Yes, I still eat with my mouth...I get that question a lot. I'm actually much better at eating than I once was; my parents used to threaten me with a bib...then I turned 30 and learned how to eat like a lady.

G-tube "feed" materials

Sleeps with me 5 nights a week...because for 2 nights I like to pretend I'm normal
As I mentioned above, CF does not like to live in solitary confinement. Because of this, I am the lucky host of cystic fibrosis related diabetes (CFRD), which affects 40-60% of adult cystics.

2 separate insulins - the bottom one resembles a highlighter...kids think I'm cool

The soldiers needles

Go here for statistics (you don't have to be a nerd, it's actually quite interesting).

Please note that I am not a doctor (although I do look like one with my pager) and that this post is certainly not all inclusive. CF is such a complicated beast, therefore it would be impossible for me to cover everything and get it all correct.

There is currently NO cure. This is why fundraising is so utterly important. Funds are put toward research for effective treatments and ultimately a cure.

The hope is that someday no child will have to grow up a cystic.

Thanks for reading...and if you think I'm brilliant, feel free to let me know.


  1. oh my gosh! look at all those pills. Oh damn. I hate the g-tube. I am so traumatize from it... I had one for 2 years in high school.

    This is a great post. A lot of people do not know what CF patients have to go through. It is tough shit!

  2. I feel mildly inadequate now with my plain-Jane navy blue and burgundy pen syringes for my insulin. Where do you get your hands on the rad ones?
    Megan xo

    1. I have connections...something about a back alley...

  3. Brilliant post, brighter than a halogen!
    Well done Gurlfren!

  4. I think you're brilliant... Love, Steve

  5. You're boyfriend is brilliant!

  6. Brilliant post! Thanks for the info.