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Inflammation: The Elephant in the Room

Guest post by Bryan Hyde – another patient at Tampa General Hospital. We met out on the steps of the valet area one day waiting for our cars to be brought to us like kings.

Elephant in the RoomI am a 38 year old male with cystic fibrosis. My journey started 12 years ago when my PFTs were in the low 30s and I decided I was not going to go out with a whimper. I also told myself that I wanted to keep the lungs that God had given me for as long as possible. I decided to make researching my disease a second job and was determined take control of my life and its outcome. My research led me to one major conclusion that has directed the way I care for my body.

Controlling inflammation is key

My belief is that I needed to attack the inflammation like the Indians attacked Colonel Custer. I wanted to surround it and attack from all sides leaving no route for escape. In other words, I was searching for an integrated approach to reducing and/or eliminating the causes of inflammation in my lungs. Inflammation causes a negative feedback loop in the lungs of people with cystic fibrosis “inflammation begets inflammation.” [Read more…]

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.

[Read more…]

Feedback Request on Meconium Ileus Scars

I put out a special request tonight for a topic about Fatboy or CF and got this one in from Jessica – a.k.a. @chronicuriosity:

“Fatboy…the Early Years” or more on why your illieus issues @ birth still cause issues (I’m a illeus baby, too – hot dog scar!)

I’ve promised to stop talking about my bellyaching for now, so here is another angle that I didn’t cover in my posts on my scar or any of my blockage posts. I’m turning this into a personal/community request for information and feedback.

Fatboy - Meconium Ileus ScarI’ve always noticed that my scar (shown again for your benefit) seems to be directly attached to the abdominal muscles beneath it. You can try to grab my scar and pull it outward, but there is nothing to grab onto because it’s so tightly pressed to my muscles, which were totally raped by the surgeon. I have a 3 1/2 pack, even though you could roll a car over me with my strong abdominal wall. This photo was taken before I got a nice layer of fat from my additional 15lbs of weight, but all you can still grab is fat.

It has been cosmetically bothering me because my added weight gain has created a crease running across my gut that is visible to all because my shirts are all tight now. I look like a fat slob with two beer guts instead of one. I know it’s petty, but it is also uncomfortable because I know my stomach and intestines could expand more if they weren’t being held back by the scar.

Do any of you have an attached meconium ileus scar or do you have “freedom” between your scar and underlying tissue? If you have “freedom,” do you agree with my issue or do you wish you had my problem because your stomach just keeps getting bigger and having the scar keep things in check would be nice? Let’s get the CFers chiming in here, since only 10% of us are born with meconium ileus.

Cystic Fibrosis Patients Need More Salt

This entry is part [part not set] 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.