What is Cystic Fibrosis?

Cystic Fibrosis... or CF.... what really is it?

I have been struggling with people not really knowing what CF is. I had a neighbor stop us to chat the other day as we returned from our family walk. He commented on how big Brandon is getting.. then he said "So he is probably growing outta that thing he has huh?" I told him "No he will always have it and it gets worse as he gets older". It is a comment that has stuck with me for a while now and I cannot get it to leave my brain. I believe its because it was not intended as a hurtful comment nor did I take it that way, but as time goes on I find it sad that people don't get it. I know I have blogged about this and yet it still is heavy on me. How else are you out there gonna get it if I don't show you.

I spend a good part of my days and life being positive for Brandon and my family. To often us Cf families don't like to think about or even talk about CF in a negative light because simplicity is bliss. I guess I think about when I have been in pain or even better getting a tattoo I had to think about other things than the pain to get through it. If I spent the whole time talking about how much the needle hurt and the artist was being a little heavy handed then I would either want to hit the artist or quit in the middle. Most of the time you talk about things that have nothing to do with what is going on at the moment. Other times you talk about the positive things, like how you hope it will turn out in the end. People who actually have CF have to be able to learn to ignore pains often that you may not even know they are struggling. I guess this is why I really study Brandon, I know as of now he cannot tell me and as he gets older he won't tell me every time he is struggling or hurting. Most of the time we only really know is when its gotten bad enough to earn a 14 day stay or longer "in the hole" as many jokingly call it. This is where IV medications are given because oral medications are not enough to kill off the bacteria attacking their lungs. During this time they are also struggling to have an appetite so they must usually force themselves to eat, children often refuse to eat and many time even through daily care at home must have feeding tubes to supplement the nutrients their body needs. Many times even if the person does eat it may not be enough which can be extremely frustrating and they have no other option. (I have recently written a guide for parents to fight through the food issues and hopefully avoid a feeding tube.) Also while in the hospital IVs can only stay on for so long, so they start their stay with an IV to later be given a PICC line:

A PICC is inserted in a peripheral vein, such as the cephalic vein, basilic vein, and then advanced through increasingly larger veins, toward the heart until the tip rests in the distal superior vena cava or cavoatrial junction.

PICCs are usually inserted by radiologists, doctors, physician assistants (in the USA), radiologist assistants (in the USA), or specially trained certified registered nurses using ultrasound, chest radiographs, and fluoroscopy to aid in their insertion and to confirm placement. PICC insertion is a sterile procedure, but does not require the use of an operating room. When done at bedside, (that is, in the patient's room), a suitable sterile field must be established and maintained throughout the procedure. For this reason, visitors are normally requested to leave the room until the insertion is complete.

The insertable portion of a PICC varies from 35 to 60 centimeters in length, that being adequate to reach the desired tip position in most patients. Some lines are designed to be trimmed to the desired length before insertion, others are simply inserted to the needed depth with the excess left outside. As supplied, the line has a guide wire inside. This wire is provided to stiffen the (otherwise very flexible) line so it can be pushed through the veins. The wire is removed and discarded after insertion.

I have heard variations of how this affects people who are frequent fliers so to say on the PICC lines. Brandon's first PICC he was not sedated enough which resulted in 5 tries of insertions and a hamburger resemblance on the failed arm. Also these things are fraqile so B has broken one and even has a scar from receiving a central line in the groin. I have talked to parents of little ones who often have issues of their little ones ripping out PICC lines. I have talked to adults who have received so many that they have many collapsed veins and or numbness and rejection from many veins. Often people consider getting a port a cath, which is a line that is connected to a button so to say that is buried under the skin. this way when it needs accessed it is there and just need to have a needle break through the skin each time. It is often used in cancer patients or others who receive frequent IV's. This to some is still a hard choice and for many can cause them to be selfconcious.

So daily while at home CF still affects them more than you, or even I for that matter can know. I try to often think about the times I have had a horrible sinus infection and I have all that build up of mucus in my body... yeah that is normal and extra thick for someone with CF. I think of the times I have had bronchitis and it felt like it was hard to breathe and that there was junk stuck inside my lungs that no matter how hard I tried it wouldn't go away. How often I coughed and surprisingly it felt good and hurt at the same time when I coughed because It relieved that nagging tickle in my throat and lungs of needing to release the gook, yet when you are always coughing it irritates other areas of your throat and chest.. Yes this to is normal for CF. I think about when I go running and I get that burning sensation and I find it difficult to catch my breath, so I stop and take in some deep breaths and realize someone with CF doesn't get to stop and "catch" their breath. I think of someone drowning and realize that if I run that thought in slow motion minus the water I am sadly looking at CF's destruction it has on the lungs....

How is this so? The easiest way to describe it is the gene that doesn't work regulates the salt and water in our body. When you think of a frog and they have the mucus coating that is sticky, which is how they can shoot their tongues out and catch flies. We all have a mucus type substance in our body that travels through many channels and organs, such as the lungs and pancreas. Our bodies regulate this mucus to make it the right consistency to carry the things the body needs with out holding onto too much. Someone with CF has that regulator broken. The salt which breaks that mucus down is sent to the wrong place and leaves the system through the flesh, which is why they will taste really salty when sweating. At times they loose way to much salt from their bodies, so when they are hot and sweaty they are advised to have a little salt. Because this is broken the mucus in their bodies not only catches the flies, but then becomes too sticky and can't let it go.. any bacteria or dangerous attacker can hang on quite easily. Also because of how heavy and thick it is it doesn't make it out of the body as it is supposed to. Filling every duct and blocking the body from releasing other essential things, such as enzymes in the pancreas to digest food. Which they must take supplemental enzymes to digest their food. Over time and as someone with CF gets older these organs tend to become damaged and scarred. If you can imagine your lungs are two buckets. As time goes on these buckets get filled. Every morning and night you empty these buckets but there is always things left behind, slowly coating more and more of the open space in the buckets. If one is not good about emptying their buckets they will sooner not have room for what they need (air). Every time they have an attack there is possibilities of severe damage and when this happens we will use a rock to represent the space it damaged so between rocks and mucus slowly filling the buckets eventually there is no room left.

Now sometimes people are blessed with new buckets (lung transplants) to start out fresh and begin the process over again, yet they get to hopefully add many, many years on to their lives. Also This lung issue is not to be compared to asthma, even though it may sometimes look like it. When an asthma attack happens the bronchials close and tighten making it hard to breath until the attack is over, although many people with CF do also have an attack on the brochials the area of concern is the lungs themselves that slowly deteriorate over their live. People with CF do massive amounts of therapies to try to force out as much of that sticky mucus as possible. They take antibiotics daily to help the everyday intruders that their bodies are unable to defend against. They take many medications to help with eating and reflux, and SO much more. They have many things that break down and do not work because of all the other things attacking them. Someone with CF fights daily to live half of the projected life the rest of us are said to expect. Most people with CF are used to coughing, shortened breathing, tightness and shooting pains in the chest, gas, bloating, indigestion and stomach pain, and way more than I could ever describe. They are plagued with enough things that many of us would take off work for, and as time goes on forces them to not be able to work with as much ease, whether its because of frequent sickness, long hospitalizations, employers not understanding CF and treating them as if they are liers or lazy. Also many adult CF'ers struggle with medical insurance and often times are not covered for many things. CF is not taking as serious as cancer or other more understood or common medical conditions. People talk alot about Cancer because it is well known, anyone you meet most likely knows one or more people who have struggled with, died from or survived cancer. Cancer is caused from so many things and in so many parts of the body, cancer can hit you at any age and can take you at any age. You can suffer for years or even die before being diagnosed with cancer. It is Horrible, I have lost many people I know to cancer. For someone to come to you and say my child has cancer everyone knows what it is and everyone sympathises and is there to rally and support. When you tell someone my child has CF, even if they stay to listen to what CF is in the short, they say that's to bad and walk on more often than not. Now I have a hard time with this because it is a double edge sword. Do I want more people to know about CF so it is taken serious and understood as the horrible destructive disease that is genetic and incurable...yes, but at what cost is that gonna be? I do not wish there were more people with CF so others can understand it, So what is a mom to do? How do I save my child without wishing more people to suffer? How do I get people to understand and care even though it doesn't affect them in any way?

CF does suck, it sucks everyday from day one from a medical and physical standpoint for those who suffer from it and it sucks for those who love someone with it to not be able to take it away or control the inevitable. So we all just focus on the positive things and carry on, we have all learned things about life and how precious it is. We all know that CF doesn't control your soul, your heart, your faith or your you-i-ness, only you control that. This is why the most common statement is found.."boy I never knew they were really that sick" because it is just a body, a very earthly thing that will not come with them in Heaven. They are not sick, their body is.