The A to Z of Cystic Fibrosis (letters A to L)

A to Z of Cystic Fibrosis (letters A to L)

I can't take credit for the idea of the A to Z is lead off by another family touched by CF also. It was an emotional journey that I took very serious about raising  awareness for CF so I put a lot of heart and soul and thought into my words. It was these posts on Facebook that lead me to the blog as many people encouraged me to do so. So here are our version of what the A to Z of CF means to me and my family.  I have to also give a shout out to the hubby as he also did some entries for the posts. (O,Q and W)

A

A for us right now is antibiotics. At the moment while Cadel is admitted he is on IV Peptaz to help him clear out the infections. At home he is on oral antibiotics. There has never been a time since Cadel's diagnosis he hasn't been on antibiotics to help keep him healthy. 

The problem with Cadel and antibiotics is that he has become allergic to the most effective ones that are used commonly for CF he has reactions to a list of about 5 and the Peptaz he is on right now has previously made him really unwell and given him a drug fever. So he is closely monitored. 

Also the bugs sometimes become resistant to the antibiotics which is also the case for Cadel and his mycobacterium which is now just maintained so it doesn't take over as it is colonised and set in.

It is like always a fine delicate balance between good and evil. 

B 

B is for Bacteria. Our biggest enemy in the CF community! 

I must confess before Cadel's diagnosis I was a germo phob but boy now I'm worse and Scott is just as bad. But we need to be! 

Bacteria gets trapped in the thick sticky mucous in the lungs and repeated infections cause scarring and ultimately lead to severe lung disease. 

Hand sanitizer is a must and we have it in the pram, the car, my hand bag, numerous spots around the house and back ups too! 

Hand washing is a big deal and were are obsessive about it as a family. ( The kids are notorious for asking people if they have washed their hands). I remember a Dr telling us the cold virus can live on a hard surface for 24 hours. That means the handrail/door knob/lift button you just touched someone who had a cold may have touched it, then you touch your face and bang in a couple of days you are swept up into the world of a cold! Sometimes I feel like I can almost see the germs and bacteria in a cartoon form sitting around waiting for the next in expecting victim. 

Cadel has had the following bacteria ( that I can remember) 

Adnovirus

Pseudomonas 

RSV

Achromobacter

Norovirus

Mycobacterium 

Staph

And plenty more in his short time. 

Some are the common cold and some are more serious and can colonise and be there just causing havoc and become resistant to treatment. Even the common cold can lead to severe damage to a CFer's lungs. So please think twice when you have the sniffles that it could be so much more than 'just the sniffles' to someone we love. 

C

C is for clinic. 

Cadel attends a CF clinic at the RCH at least every two weeks. When first diagnosed Cadel was visiting clinic weekly then fortnightly then we eventually got to 4 weekly. We were told that we would have to visit 3 monthly one day but because Cadel has been so unwell we haven't gone anymore then around 4 weeks between clinics. 

At clinic we see Cadel's 

Consultant, 

Physio, 

Dietician,

Social Worker,

Occupational Therapist and the Nurse Consultant. 

Luckily we are assigned a room and all the above come to us to avoid cross infection from other CF patients. 

They also weigh him and take his height. 

He also may have to have bloods or X-rays before we attend clinic also. We sometimes have to pick up some medications so waiting around for pharmacy can take some tome too. So days when we have clinic take a lot of planning for the whole family.

Sometimes clinic is a quick one and we are out in 40 mins but sometimes it can take hours. 

Eventually Cadel will have to a lung function test before clinic but for the moment he is too young. 

Lung function tests measure:

How much air you can take into your lungs. How much air you can blow out of your lungs and how fast you can do it.

How well your lungs deliver oxygen to your blood. The strength of your breathing muscles. These tests help the Drs to see how much all the infections and scarring has affected the ability to just breathe. 

D

D is for DF508. This is the gene mutation that Scott and I both inherited and that when combined cause CF. As we are both carriers of the CF gene their is a 1 in 4 chance of having a child with CF every pregnancy and a 2 in 3 chance of having a carrier. A carrier like Scott and I has only one copy of the gene error ( prefer error then mutation) and does not have any symptoms of CF. 

There are thousands of different gene errors and some have milder symptoms but DF508 is the most common and has the most side effects so Cadel got the double whammy! 

The main characteristics of DF508 are:

Pancreatic insufficiency. Cadel doesn't produce enzymes to break down his food to help absorb the nutrients, this is why he takes enzymes with all of his food. 

Thicker mucous that clogs up organs all through his body. 

He is also most likely infertile as the Vas Deferens don't form correctly because of the presence of the mucous at fetal development. 

CF is the most common inherited disease and like Scott and I most people don't know they are carriers. The biggest population of carriers is the European Caucasian population with 1 in 25 carriers and they don't even know it. We had no previous family history that we know of. This is why the heel prick test done on newborns is so important as it picks up CF ( most of the time) before anyone even knows that something is wrong. 

Lorelei and Xavier will most likely test their partners when they are ready to have children (when they are much much much older! ) to see if they are carriers. Even if so they can be aware of the chance of having a child with CF.

E 

E is for escape. Or should I say no escape. CF will always be a big part of Cadel's life and our family's life. There is no day off from CF. There will always be medications that need to be taken and physio that needs to be done. Sometimes just for a moment you can forget when you see Cadel playing with his brother and sister but then you are jolted back when you hear a cough. 

I'm guilty of saying I just want a day of from CF and really I can but Cadel never ever will. 

Like Scott and I say CF lives with us and make it fit into our lives not the other way round and that is something we will help Cadel to understand. We will teach him to reach for the stars and never let his CF hold him back. 

F

F is for fighter. Cadel is such a fighter. His personality does not allow him to give up and it is definitely something Scott and I encourage in all our kids. 

Most days Cadel's body fights to just breathe. It takes a lot of energy to breathe when he is unwell and this is why CFers need to eat extra calories and have a high fat and salt diet as they need reserves for when they are unwell just to function. 

Fighter is also the name of the song that Scott and I have choosen as Cadel's song as the words say :

"Give 'em hell, turn their heads

Gonna live life 'til we're dead

Give me scars, give me pain

Then they'll say to me, say to me, say to me

There goes a fighter, there goes a fighter

Here comes a fighter

That's what they'll say to me, say to me, say to me

This one's a fighter"

Give 'em hell Bubba Boo! 

G 

G is for Gastroenterology Team. 

Yes Cadel has CF and his team of Doctors are primarily from the Respiratory Team but he is also under the Gastro Team. 

This is the team that put in Cadel's feeding tubes, did swallow tests on him and help us look after Cadel's CF related liver disease. 

Cadel has had numerous Nasogastric feeding tubes and just over 12 months ago the Gastro team placed a Gastronomy button in his stomach for tube feeding. This is where Cadel gets 99% of his nutrition. He has three bolus feeds a day of 120 mls over 1/2 hour and then has overnight feeds of 700mls over 10 hours. The feed is mixed and formulated specifically for Cadel and is 1.25 calories per ml. He has mouse nibbles on food offered through the day but thanks to the Gastro team we are able to get much needed calories in. 

Now his liver. Cadel was diagnosed with CF related liver disease in around May 2012 after a biopsy was taken. His liver is enlarged and has some scarring. There is only a small percentage of CF patients that will get liver disease and Cadel is in that group. He is unusually young to be diagnosed with the disease and liver disease is very slow and unpredictable when developing. There is not much we can do at the moment only give him medicines that help maintain his liver twice a day. He recently had an ultrasound on his liver and there was nothing new developing so that was good. We were told when diagnosed that because he contracted it so young Cadel is at a higher risk of one day needing a liver transplant. The only problem with that is the fact that his lungs need to be in a good condition for that to happen. It is one big vicious circle. 

H 

H is for Hospital In The Home or HITH. Cadel is currently on the HITH program and technically still admitted while we are away staying on the Gold Coast this week. This great program allows patients like Cadel to spend less time in hospital. 

HITH is a program where in special circumstances IV antibiotics are put into infuser bottles that will give a dose of medication over a 20 - 24hr period. A nurse will come out daily and change the bottles over as well as for CF patients there will be a physio that will attend twice daily for treatments.

We are lucky here in Queensland that we have the HITH program, in South Australia they are losing the funding for the HITH program. ( I have included a link to sign a petition to help out SA, please sign if you haven't already). I cannot understand why with our struggling health system, the program allows many patients to stay at home freeing up hospital beds and I think Cadel gets better quicker in his own environment and not stuck in a hospital. 

I can't say enough fantastic words for this great valuable program that helps our family function a little more easily and still gives Cadel the treatment he needs. 

http://www.gopetition.com/petitions/save-the-wch-hospital-in-the-home-program.html

I 

I is for iPad. The hospital has provided us with an iPad and Cadel's Dr and the CF Nurse Consultant have a matching one so we can Skype if we are worried or just for a check up. 

This saves our family the sometimes 1 hour long drive to the hospital and the crazy expensive parking costs when we visit the RCH. Sometimes we can Skype the hospital 3 or more times a week and sometimes not at all but it is a valuable resource that we know is available. 

It is also handy for when the physio's are doing their home visit and the Dr can Skype getting a visual on Cadel and talk in clinical terms to them about anything that is happening. 

We can only hope that this is available to all patients in the future as this is the way technology is going but for right now we feel pretty special. 

I must admit the iPad has also kept at times the whole family occupied during those looong clinic appointments and during admissions. It has also come in handy during Cadel's 40 min liver ultrasounds. Keeping a two year old occupied for this long is a little easier when there is a game or two to play on the iPad. 

J

J is for JUST being a Mum and Dad to all our children. This is JUST what we do everyday. Even if sometimes that means hooking Cadel up to IV antibiotics, physio, doing all his meds, doing Lorelei's hair for dancing and running Xav around for grading of Taekwondo. Yes we sometimes do a little bit more than the so called typical parent but put anyone else in our shoes and I'm sure you would all JUST step up and do the same. 

We are constantly told by friends, acquaintances, family, hospital staff, Doctors, nurses and complete strangers how strong we are and inspiring we are but I would like to say that we are nothing without our support network of family and friends but especially our 'salty' family of other CF parents who it feels like we have known for a life time who share our journey even if on different paths. 

Two quotes I really like are :

"You never know are strong you are until being strong is the only choice you have. " author unknown 

"When something bad happens you have three choices. You can let it define you, let it destroy you, or you can let it strengthen you. "

P.S They are called our salty family because all our CF children taste salty when you kiss them because of the amount of salt lost through their skin due to the condition.

K

K is for Kindness. We have had so much kindness and compassion come our way especially in the last 6 months. 

We have the great support of our families that always help out when we need them. They give up their time to help us out sitting by Cadel's bedside to give us a break, looking after Lo and Xav and even organising family holidays for us. 

We have some fantastic friends who without even thinking or us asking, help us out looking after the kids or cook us some yummy meals during Cadel's admissions to take the burden off.

Then there was the time when we came home from holiday and our back and front yard had been blitzed and our freezer was full of food thanks to our salty family. 

The biggest kindness of all has come from friends and a big group of complete strangers. Scott the kids and I cannot put into words how grateful we are to all these people for their valuable time and kind heartness that will forever stay with us and I'm not quite sure how to thank them all. I know I'm being a bit secretive but trust me all will be revealed in a couple of weeks and you too will see how big and kind hearted people truly can be. 

All this kindness has given us some tears but plenty of smiles and made our hearts all warm and fuzzy as it makes us realise that there are still great wonderful giving and sharing people in this world. We have had to fight through the weeds but the people in our lives now have made our lives blossom with happiness. 

L

L is for the love of life. CF is a fatal disease and the average life expectancy is only 38 years old. Although many live beyond that age there are also many that don't make it. 

That is why we live life to the max and enjoy every moment that we have with all our children. I find myself locking away all the little moments in my brain so I'll never forget every moment we have with each child but especially Cadel. 

Cadel is an old soul who has been here before and is so wise beyond his years. Like most people with CF he is resilient, brave, strong, fun loving and packs so much into his day. 

We are always doing things to make special family memories. Even just small things like a water fight in the backyard or big things like visiting theme parks. We are surrounded by love and make sure that those important to us know they are loved. 

We will never put a limit on Cadel's life and encourage him to always chase his dreams and enjoy every moment and never ever give up hope that one day they will find a cure for CF. 

K