everyone has a story, here is mine:

I have Cystic Fibrosis, diagnosed at birth by Meconium Ileus (an intestinal blockage requiring surgery). Although considered healthy most of my life (with no lung involvement), I've always dealt with fatigue, digestion complications and sinus issues.

As a child it was thought I was miraculously spared the devasting effects of CF - it was believed I had a mild case of CF, and wouldn't develop the typical complications of a person with Cystic Fibrosis. Respectively, my health was taken for granted, and I wasn't taught the importance of lung maintenance.

So, when I woke up sick one January morning in 2002,
I simply thought I had the flu, and didn't feel the necessity to go the doctor. Two weeks later after no improvement, I found myself in the hospital, on I.V. antibiotics and oxygen for no less than 14 days.

Not until just recently have I come to understand that what I experienced was my first CF exacerbation, which, sadly, caused irreversible lung damage by waiting for treatment.
So much for being "healthy".

Although I tried to return to work, it proved to be too stressful, too demanding, and too exhausting.
So, that October I retired and started my "life with CF".
And here we are today....

Sunday, February 17, 2008

What is a Tune-Up

What is a CF Exacerbation?
From Dictionary.com:
ex.ac.er.ba.tion
  • to increase the severity, bitterness, or violence of (disease, ill feeling, etc); aggravate
  • to embitter the feeling of (a person); irritate; exasperate
- Synonyms: intensify, inflame, worsen.
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  • An exacerbation is when the CF kicks it up a notch. You feel lousier, you have a harder time doing things, you cough more, you cough up more, the bacteria starts fighting harder/growing more, maybe you lose more weight than usual. Any combination of these would compromise an exacerbation.
  • An exacerbation is a worsening of symptoms that leads to needing a tune-up. Sometimes we call them exasperations because they're so frustrating and each one makes it more difficult to make a come-back.
What is a Tune-Up?

Just as our cars need regular maintenance to run efficiently and effectively, so do the bodies of those with cf. They refer to this maintenance as a "tune-up"A Tune-Up is recommended based on a few factors:
  • increase in cough/"lung junk" and/or a decline in spirometry (pulmonary function test)
  • weight loss
  • elevated blood sugars (for those with cf related diabetes)
  • increased fatique
  • increase in fevers

As you can see, increase is the key here. Due to the chronic infection in the lungs of those with cf, it's possible to experience all of these symptoms on a daily basis, depending on the progression of their lung disease, and worsening with lung damage. However, when a bug is growing and multiplying, all symptoms pick up speed.

What is involved in a Tune-Up?

  • minimum of 14 days on i.v. antibiotics (ab's), and sometimes longer depending on how well the body responds. Typically a combination of ab's are used to fight the different "bugs."
  • aggressive respiratory treatments (rt), a minimum of 4 times a day, and some require more. (see Treatment of CF to learn what's involved in this process.)
  • physical therapy - anything from treadmill walking to stretching exercises
  • all the food they can eat - there is a proven connection between weight and lung function in those with cf. Tests show that a drop in weight results in a drop in pft's. When the body is fighting infection, it burns more calories and so not only is it important to keep the weight up, it needs extra for just those times of infection

Most begin their I.V. antibiotic therapy in the hospital. Some remain there for their entire 14 day course, while others choose to finish their therapy at home after an initial stabilization period. Most certified cf centers would rather see their patients remain in the hospital for several obvious reasons:

  • They do all the work while allowing the patient's body to rest. As stated previously, a combination of ab's are typically used, which can mean 'round-the-clock dosing schedules. Some antibiotics are dosed every 4 hours, other every 6, or 8 or 24 hours. While hospitalized, nurses can begin the next ab w/o waking the patient, allowing them to get better rest. If the patient is at home, their sleep (or a family member's sleep) is disrupted.
    *It's important for someone who is ill to get plenty of quality rest. While in the hospital, the patients aren't disturbed if sleeping, for vitals or miscellanous tasks.
  • At home, responsibilities call which makes it easier to put off caring for oneself. On the contrary, while in the hospital, they do all the work - the cooking, the cleaning, gathering all the medicines, and scheduling the breathing treatments - everything is brought to the patient. The patient is able to concentrate on getting well, rather than preparing dinner or seeing that the laundry is done.

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