A little more than a year ago four of us with Hermansky-Pudlak Syndrome volunteered to have lung lavages at the National Institutes of Health in Bethesda, Md. Several years ago another group did the same thing. Today, NIH is ready to take the next step and ask more people with HPS to volunteer to have lung lavages, ideally every year to two years. This could mean a lot for the research.
To be honest, a lung lavage isn’t the most fun medical test you could ever have done. Doctors pass a long flexible tube through your mouth, down past the vocal cords into the upper regions of the lung. They then flush saline into the lungs causing you to cough, and thereby slough off cells from the inner parts of the lung. The resulting mucus is then sucked out and collected. This differs from a bronchosopy in that no biopsies are taken. We joked a year ago that it was all sucking, and no plucking. Currently NIH is afraid to try to take biopsies of our lungs, even though it would be immensely helpful to the research, because they are concerned about bleeding. This is partly why I’m willing to volunteer for tests at NIH, even the not so fun ones. Every precaution possible is taken.
Patients must be awake for this procedure so that you can cough on demand; however, the doctors give you some wonderful medication so that you don’t remember how bad it was. It was as if I was asleep for a surgery because I don’t remember anything.
Thanks to the medication they give you, I’d say the procedure isn’t that bad. The worst part was the nasty tasting medicine they spray in the back of your throat to numb it so they can pass the tube down without having you choke on it.
The NIH is interested in lavaging a number of people, but they are particularly interested in younger people with HPS that currently have no lung complications.
Last year they discovered that certain cells in our lungs were “all fired up and foamy” as if they were ready to attack something. They also learned that our T2 cells, cells that release surfactant, seemed to be engorged. We had adequate amounts of surfactant, but apparently these T2 cells don’t give the stuff up easily. Perhaps the “fired up” macrophages were reacting to a distress signal sent out by the T2 cells? Who knows?
The doctors would like to collect lavage fluids from younger people with HPS in hopes of learning whether their fluid looks like that of those of us who are older. Does this process exist throughout our lives, or does something happen to trigger it? Is there more involved somehow? If the scientists could study samples from the same patients over time, it might give them clues as to how the process in the lungs gets started, and thus how it might be controlled or halted all together.
Finding volunteers for this task, however, I know won’t be easy. While it’s vital to better understanding HPS, most people are not going to be excited about volunteering to have this done. To me, however, it’s worth a day of a foggy memory and a sore throat. I’ve volunteered to do it again, if it would be helpful. We’ve had a few other volunteers step forward. I pray that more are willing to take on the task.
To be honest, a lung lavage isn’t the most fun medical test you could ever have done. Doctors pass a long flexible tube through your mouth, down past the vocal cords into the upper regions of the lung. They then flush saline into the lungs causing you to cough, and thereby slough off cells from the inner parts of the lung. The resulting mucus is then sucked out and collected. This differs from a bronchosopy in that no biopsies are taken. We joked a year ago that it was all sucking, and no plucking. Currently NIH is afraid to try to take biopsies of our lungs, even though it would be immensely helpful to the research, because they are concerned about bleeding. This is partly why I’m willing to volunteer for tests at NIH, even the not so fun ones. Every precaution possible is taken.
Patients must be awake for this procedure so that you can cough on demand; however, the doctors give you some wonderful medication so that you don’t remember how bad it was. It was as if I was asleep for a surgery because I don’t remember anything.
Thanks to the medication they give you, I’d say the procedure isn’t that bad. The worst part was the nasty tasting medicine they spray in the back of your throat to numb it so they can pass the tube down without having you choke on it.
The NIH is interested in lavaging a number of people, but they are particularly interested in younger people with HPS that currently have no lung complications.
Last year they discovered that certain cells in our lungs were “all fired up and foamy” as if they were ready to attack something. They also learned that our T2 cells, cells that release surfactant, seemed to be engorged. We had adequate amounts of surfactant, but apparently these T2 cells don’t give the stuff up easily. Perhaps the “fired up” macrophages were reacting to a distress signal sent out by the T2 cells? Who knows?
The doctors would like to collect lavage fluids from younger people with HPS in hopes of learning whether their fluid looks like that of those of us who are older. Does this process exist throughout our lives, or does something happen to trigger it? Is there more involved somehow? If the scientists could study samples from the same patients over time, it might give them clues as to how the process in the lungs gets started, and thus how it might be controlled or halted all together.
Finding volunteers for this task, however, I know won’t be easy. While it’s vital to better understanding HPS, most people are not going to be excited about volunteering to have this done. To me, however, it’s worth a day of a foggy memory and a sore throat. I’ve volunteered to do it again, if it would be helpful. We’ve had a few other volunteers step forward. I pray that more are willing to take on the task.
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