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Meet Fatboy’s CF Coordinator: Sue

Not SueI went out on a limb one day a while back and asked my totally awesome coordinator if she would be willing to answer some interview questions so we, as CF patients could get her perspective on what she does and what we do/do not do as patients. I was threatened with life and limb or something about never getting another Rx again if the photo we took at Great Strides ended up on the Internet, so this post is sans-Sue photo. Instead, I’ve used a photo of a person pretending to be Sue. Just so we’re clear – this is not Sue (of course, she knows that!), so I can continue to receive treatment at my CF center.

She warned me of her rambling answers, but after reading through them, I have editorially decided to leave them be, and de-labeled them as rambling. It’s rather a stream of consciousness that makes perfect sense. I think they tell an interesting story that I’d never heard, and I’m sure most of you haven’t either.

Fatboy: Ok, so I know you’re an RN, but how did you get so darn good at being a coordinator with all of the administrative things that you do? You’re undoubtedly one of the reasons I’ve been able to get and remain compliant because of easy e-mail access to the doctors through you. Before it was like jumping through hoops to get meds or an appointment. Did you set out to change that or was that why you were selected as the new coordinator. Mad props to your administrative abilities. I couldn’t do your job.

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Cystic Fibrosis Patients Need More Salt

This entry is part 1 of 3 in the series Meet My Little Friend...

@NanosmakemepukeIn recent months, I’ve made a new CF friend on Twitter, @nanosmakemepuke, who just so happens to be a chemist with a Tumblr blog with a name that immediately gave me a source to go to for an expert answer to my ongoing query. I got the bright idea this week, “Who better to ask for an explanation of why cystic fibrosis patients need more salt than normal people than Amanda? She’s got a PhD in chemistry!” So I did, and she obliged with a pretty detailed reason in layman’s terms that I think you’ll find as fantastic as I did. Many, many thanks for such a great piece that will live on for a very long time to help educate others looking for the answer to the same question that has been bugging me for years.

Amanda: Salt is the combination of sodium and chloride. The biochemical processes that go on in our bodies are such that only a very narrow range of salinity (concentration of salt in our body’s fluids) is acceptable. Too little or too much is literally a matter of life and death. Fortunately, our bodies are really good at ensuring that it contains the ideal concentration of salt by adjusting the volume of fluid in our blood and tissue so that the salinity is always in the ideal range. If you have too much salt you become thirsty, causing you to drink water, which dilutes the too-high concentration of salt in your body. If you have too little, you usually crave and eat something salty.

In cystic fibrosis, improper transport of chloride (a component of salt) causes all the horrible symptoms and manifestations of this disease. CF bodies don’t fully regulate the concentration of salt within the cells that line our airways, digestive tract, and skin, causing the concentrations to be a bit out of whack—either too low or too high, depending on which side of the cell you’re looking at. In the case of the cells that create sweat in a CF patient’s sweat glands, they leave too much chloride inside the “sweat reservoir,” which must in turn be balanced with an equivalent amount of sodium (wherever chloride goes, sodium follows), making the super-salty sweat characteristic of cystic fibrosis.

Every time a CF person sweats, their body loses up to 10 times the amount of salt a normal healthy person would in the same amount of sweat. That means excessive sweating (as might happen in the summer or when exercising) puts people with CF at a much higher risk of dehydration than other people. Because of this salt loss, people with CF must be very diligent to replace lost salt whenever they sweat, otherwise there is a risk of suffering from dehydration.

Generally speaking, people with CF need to include salt as a regular part of their diet to compensate for abnormal amounts of salt lost through sweat. The usual reason doctors recommend a low-salt diet for non-CF people is so that they don’t develop high blood pressure, which causes your body to retain more fluid in your blood to compensate for the excessively high concentration. If you have CF and have high blood pressure, obviously the amount of salt in your diet is something you need to discuss with your doctor.

Bottom line: it’s important for people with CF to add a little extra salt into their diet in order to avoid dehydration in warm weather and when exercising. Also, always make sure you consume something salty with plenty of fluids so your body can process it properly.

2010 Great Strides Walk – I won

Great Strides Tampa 2010I made my Great Strides walk a race. I didn’t want anyone walking faster than me; after all, I am a superhero who goes by the name of Fatboy. The walk started at the entrance of the Lowry Park Zoo and led us around a mile or so loop around the exhibits. It was pretty easy stuff, but I had a hard time getting my team to keep up with the poor guy with 58% lung function. Can you tell I’ve got a competitive streak a mile wide and won’t take pity from anyone? I’ll cream you before you give me pity.

When we finished our first lap, Doogie Howser was standing at the Entrance/Exit thanking us for coming. We were done in 17 minutes, so there was no way we were going after waiting an hour for the walk to start… after a 40 minute drive to the zoo… after 5 months of raising funds. It’s supposed to be a 5k, after all… so we told him we were going around again until we hit 3.1 miles as he got on his radio/phone to find out why the heck I wanted to keep dragging my team around the zoo at 6:47 in the evening.

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