Breathing Easier
Cystic fibrosis patients could soon benefit from breakthrough small-molecule drugs that treat their underlying disease instead of just their symptoms,

ON A TYPICAL school morning, Jeanmarie Youngblood wakes up at 4:45 AM to start her daily routine, an hour and 20-minute ordeal that doesn’t even include a bowl of cereal. If this was a case of typical teenage-girl vanity, she might be spending the time showering, blow-drying her straw-colored hair, and picking out the right pair of skinny jeans. But for Youngblood, setting her alarm for that bleary-eyed hour isn’t about looking good in homeroom. It’s about getting her lungs in shape for the day.

Youngblood has cystic fibrosis, a disease that causes thick, sticky mucus to clog her lungs and disrupt her digestive tract. It’s a trait she shares with some 30,000 other Americans who inherited a copy of an errant gene from both their mother and father.

The first order of business when Youngblood stumbles down the stairs of her Brooklyn, N.Y., home is to attach a hose to her nebulizer, a device that aerosolizes a drug, and then load it with albuterol, which opens the airways in her lungs. Next, she puts on a specially designed vest that compresses and releases her chest wall. Looking like a life jacket, it gives a vigorous massage that helps loosen the mucus in her chest and keeps an otherwise persistent cough at bay. For the 30 minutes she wears the vest, she inhales hypertonic saline, a recent addition to the routine that helps to thin and clear mucus.

After that, Youngblood changes the hose on her nebulizer yet again to inhale Tobi, an aerosolized version of the antibiotic tobramycin. Another hose change, and she breathes in Pulmozyme, an enzyme that snips the extracellular DNA in the lungs’ mucus, an additional method of thinning everything out.

She repeats the entire routine, without the Pulmozyme, around dinnertime. Keeping up with the nearly three-hour-per-day program is no small feat. “It’s a lot of time I spend sitting, not being able to do things,” Youngblood concedes. Yet it is also a fact of her teenage life, something that she has to do to feel okay.

Read more at cystic fibrosis

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