please explain!

I am dedicating this blog to the glorious FM girls (you know who you are) and all the wonderful people who appreciated the blog I wrote a few days ago about my youngest son’s diagnosis of cystic fibrosis. I thank you from the bottom of my heart for all your support and friendships.

Since that particular blog was published, I have had a few questions. the most popular one is “So what exactly is CF?” I was like this myself only 20 months ago. I had heard of it, but had no idea what it was.

OK, I have poured myself a glass of wine, and here is my explanation of what cystic fibrosis entails…well at least for our son. Every case is different and everyone gets treated individually.

Most organs in the body can be affected my cystic fibrosis. The biggest worry, and pretty much present in every CF patient (to different degrees, of course) is mucus build up in the lungs. The mucus is thicker and more sticky than the average person, which can breed all sorts of nasty bugs. This why every CF person has to have physiotherapy, or percussion. You make a swan’s head with your hand, to make a cupping shape and you thump down the chest in different positions to loosen and move the mucus around. You want to hear a thumping sound, not a slapping one. this is how you can tell that you have your hands in the right shape.

The analogy that I was given at my son’s diagnosis, is the lungs are like a pond or fish tank. If you let it sit, then you get that murky water and growths on the top and sides. However, if you constantly stir the water, it keeps the “grubby” stuff (I know there is a better word for it, but it escapes me right now) from forming/growing. This is why my son has physio, for half an hour, twice a day, every day, in 6 different positions. Coughing is encouraged as it helps move the mucus around during those periods in between physio. Some people go upside-down to help the mucus to be coughed up, but our CF physio said it is unnecessary, its only been known to cause reflux more than anything else.

He is also on daily antibiotics (currently Amoxycillin) to help prevent getting sick. I have been told that it is “procedure” for at least the first year of life, but as we are approaching the end of the second year, he is still on them. Maybe it will be a life time thing??

As we are regional patients, we do every thing we can at home to avoid the long trips to Perth. This means, when our son gets a moist cough, he has nebulizers, filled with what was described as “turbo-charged penicillin” (more commonly known as Tobramycin) twice daily, for at least two weeks. If there are signs of improvement, we can carry on at home for up to 4 weeks. If there is no improvement, then it means a hospitalization for no less than 2 weeks, in Perth. This is where they are pumped full of intravenous antibiotics for at least a fortnight, until they are happy enough to send him home.

As mentioned in a previous blog, my son’s digestive system is affected as well. His pancreas does not secrete enzymes, which break down fats in food. Without his tablets (Creon 5000), food with a fat content will pass straight through his system within about half an hour. He will poop at least a dozen times, it is undigested, stinks to high heaven, loose and you can see fat floating on top (sorry…I know this is too much information!!) We can generally tell from his nappy content if we need to adjust his medications.

Due to this, my son has to have a really high fat diet. This is because his little body uses up so much more energy than our own. He can have chips, crisps, chocolate, burgers….the lot. Even if he has fruit, it should be smothered in cream, full fat yoghurt, or custard!

This has been a total learning curve for me. It is drummed in to you from a young age “LOW FAT, LOW FAT, LOW FAT!” so it did not feel right pouring oil over his meals AND adding a teaspoon of butter. It took me a good three months to stop gagging over his meals to see it basically floating in oil. Basically, you serve up everyone else’s meal, then pour about a teaspoon of oil, teaspoon of butter, maybe some avocado and shaking heaps of salt over his. If he is eating finger foods, then spray it with cooking oil, or better yet, deep fry it. Crazy, isn’t it??? Seriously…and Big Mac is one of the healthiest things we can give him!

As I just mentioned, a high salt content is generally recommended, though I believe not every CF person has to do this. But in my son’s case, he has to have Sodium Chloride twice a day (salty water) and added salt to his diet. His body loses much more salt than the average person through sweat, tears, urine, poop, even breath. However, when we sweat, our bodies reabsorbs about 70% of the salt we lose. A CF-er’s body is a one way street…out. On a hot day, you can see the salt crusting over the top of my son’s bald little head. At any given time, if you kiss the top of his head, then lick your lips…all you will taste is salt. So we have to boost the salt content in his diet too.

I bet you’re sitting here thinking “But what about long-term health effects?” Trust me, that was one of my first questions to the dietician. I could see clogged arteries on top of the CF. But it is not an issue. The body will burn up the energy before it even gets a chance to have that effect!

Ok, so the pancreas may not secrete enzymes (like my son’s case) but remember, the pancreas also produces insulin, so diabetes may be an issue. You have Type 1 diabetes, and you have Type 2 diabetes, but in the last couple of years, I have found out that there is also a “CF Related Diabetes” (just an easy way to say, “CF has screwed up your pancreas!”) We are lucky. My son does not have diabetes. But since its quite obvious his pancreas has been affected, he is under close watch.

One of the biggest symptoms I had massive trouble getting over, was infertility is usually present. In males, semen is so thick, it creates blockages, prevent from the little tadpoles from getting out. They told me at diagnosis, that if my son wants to have children in the future, there is a procedure that involves a large needle to go around the blockage. But he can deal with that later in life. 

I honestly have no idea why this shook me so much! It’s not even sure if my son is infertile, it’s a just a high possibility. It took me months to come to terms with it.

For CF females, I have been told that their cycles will be irregular, thicker/heavier, and pregnancy will be a huge toll on the body, almost all CF women are induced early as stress is put on their lungs.

I have heard that kidneys can be affected. How? I’m not sure, I’ve only heard that a friend of a friend’s daughter has CF related kidney issues. but I would imagine that thick blockages are preventing the kidneys to filter, or dont secrete hormones.

I’m sure there is much more I am missing….but that is the crux of it, especially with my son. I’m sure more will happen in the future, but I try not to think about “what if’s”. I have no idea what adult life may hold for my son. There have been so many developments in the last couple of years that not even the specialists can guess!

Please don’t think that talking about Cystic Fibrosis upsets me! I consider myself a huge advocate for CF awareness. There is a general lack of knowledge and understanding about CF, I encourage questions!!!!

While I hate being so far away from the children’s hospital, but I am so glad to be under their care. Princess Margaret Hospital for Children is apparently world renowned for its research into lung disease, especially cystic fibrosis.  If your child was sick, wouldnt you want the very best? At the time of my son’s diagnosis, the hospital had received a MASSIVE grant from the US to do their research for them.

The last thing I need is people telling me what I should, or should not be doing, unless they are a medical CF specialist. The people who do that are ignorant about CF life and are usually prattling off about something they have no experience in.

But I want to thank those supportive people in my life. You know who you are. Even if it’s just a kind word, or saying “I’m here if you need to talk”, that’s all I need. Seriously, thank you. You are my rock during those turbulent times!

OK, my glass of wine has turned into a few. Sorry for the novel, but I hope this clears up a few questions…like I said though, feel free to ask any more!

Night all

Bella

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One thought on “please explain!

  1. Great post Bella, you sound like a very knowledgeable CF mommy. I found it hard to talk about CF the first couple of months after the diagnosis – but now I can’t stop talking about it. I think there is so much hope for our little CFers. Have you checked out Cystic Life yet? What a great resource for CF parents. http://www.cysticlife.org

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