CF Awareness Post 2 - What is CF?

My second question comes from a fellow CFer as well.  Her question was meant more as a joke but I decided to take it and answer it!

What is CF?

CF means Cystic Fibrosis.  CF is much easier to say and write especially in long conversations and posts.

But what IS CF?

CF is a genetic disease that affects many aspects of the body.  It varies from person to person but it affects mainly the lungs, the pancreas, the digestive system, and the reproductive organs in both sexes.  Some patients have issues with their kidneys, liver, joints, bone density, and heart.  These are secondary issues caused by medications and low oxygen respectively.

The low down dirty reason for CF is the inability of cells to transfer sodium chloride across its membrane.  The result is an abundance of salt in the body.  We CFers tend to be a very salty bunch.  If you look at my legs on a hot summer day they are dry as a bone but covered in a white film....lick me and drink a Margarita!  

This leads to a build up of thick sticky mucus in the lungs which harbor bacteria and cause lung infections.  These lung infections, over time cause a decrease in lung function because of scarring.  Eventually the lungs don't function properly and can not get enough oxygen to the rest of the body, requiring supplemental O2 to be worn.  The decrease in lung function ultimately causes a CFer to either pass away or require a double lung transplant.  These lung infections can be treated with oral antibiotics, IV antibiotics, and inhaled antibiotics.  As well as the use of the airway clearance device the Vest, accapella, the flutter and manual chest physiotherapy (CPT).  Bronchodialators (sp?) are also used to keep the airways open so the medicines can get in there and do their work.  As the diease progresses though, orals and inhaled meds may not work.  As is the case with me.  Orals work sometimes, and inhaled antibiotics are out of the question because I have reactive airways.  Meaning when I inhale something my lungs don't like, they close up and cause severe bronchospasms.  NOT FUN.  Basically I have a massive asthma attack that only Prednisone can get rid of.

In a good portion of CFers, but not all, the pancreas does not work properly.  The pancreas produces enzymes which break down food put in the body to get nutrients.  In some CF patients this does not happen so we must take enzymes WITH EVERYTHING.  If we want the nutrition from the food we must take the pills.  There are a few different enzymes on the market and it takes time to figure out which works for you.  Me personally, I don't take pills with every meal because I have a tendency to get backed up fast.  I take them with my big meals but my 2 small meals a day I skip.  I also have to take a ducolax and Miralax every day.  If not, I risk getting a blockage from my slow moving digestive system.  We learn that the digestive system works in about 6 hours or so.  For me, and many of my CF friends, we might not "see" that meal on Monday until Wednesday.  Bloating and the CF belly are quite common with CF patients.

CFRD is also caused by a reduction in the function of the pancreas.  CF related diabetes.  Kind of like regular diabetes, but not quite.  Sugars need to be monitored, insulin may have to be taken.  I am new to the whole CFRD thing and only monitor with diet so I am not as up to speed on this aspect as I am the rest.  Many CFers don't reach CFRD until after transplant because of the use of Prednisone to reduce rejection, but many do get it prior.

The reproductive organs are also affected.  For many CF men the vas deferens is missing so sperm is not able to make it out when ejaculated.  I do have a few CF male friends who have fathered children the natural way.  But this is rare.  Most need to see a fertility specialist to have the sperm taken out and artificially inseminate the woman.  For women, getting pregnant can be easier than the man getting a woman pregnant.  Though it varies from person to person.  Some women just never get pregnant without help and others find they have "opps'" easily.  There is a extra amount of mucus lining the cervix which can block the sperm from getting through.  This is normal, to see mucus there, but because of the excess mucus in a CFer's body, there is way more here than is normal.  Once pregnant there are risks to carrying the baby and all that, but it is done, and has been done by many many CF women.  Please talk to your CF doctor about it.

The secondary issues such as the kidneys, liver and heart are caused by medications and lack of O2.  All of the medications taken on a daily basis are processed through the kidneys or liver and over time they can wear out causing the patient to need a transplant.  This may happen before the lungs give out or after.  And does not always happen.  Heart issues can also occur because of its need to make up for the lungs declining. Tachycardia is a common issue among CFers.  This is a fast heart rate, I believe above 110 BPM resting.

Check out this chart for more issues we CFers can encounter.

(source)

For more information visit the Cystic Fibrosis Foundation's website.