At least from the pictures on the Sipe blog, www.angelsinvoice.com, it looks like Candice is doing quite well. You’ll also note that the Sipe family sent part of the biopsy taken from Candice’s lung to Dr. Gahl at the National Institutes of Health for research.
I wanted to take a moment to blog about that decision for two reasons. First, I want to publicly thank Candice and let you all know what a special gift that was. Second, I want to draw a little attention to the need for tissue collection so that the HPS’ers reading this blog can file this away for future reference as something they might want to consider, and something we should all be discussing with our families.
HPS types 1 and 4 cause pulmonary fibrosis. How this happens exactly, however, remains a mystery. One way to uncover the truth about this mystery is to study lung tissue. The problem is that because of the bleeding disorder associated with HPS, doctors are extremely leery to try to take tissue samples from HPS patients that still have healthy lungs. (That’s why right now we do lung lavages for research rather than lung biopsies.) They don’t want to do a procedure that could involve some risk just to obtain research samples. Studying donated lungs from patients with HPS after they’ve passed away is helpful, but what we really need to know is what’s happening in an HPS lung before the fibrosis sets in with a vengeance.
Unfortunately, in Candice’s case, taking a biopsy from her lung couldn’t be avoided. Her doctors needed to determine exactly what was happening with her cancer in order to know how to best treat her – thus there was medical necessity to the risk of causing a bleed. (Thankfully, it seems Candice didn’t have any bleeding complications.)
Candice’s type of cancer is very rare, and so far, we know of no other person with HPS that has developed the same type of cancer, so we don’t think there’s a link. But then again, we’re constantly learning about HPS and documenting health developments, thus it’s really important that those of us that are part of the natural history study communicate major developments in our health to NIH and if relevant, provide samples.
Candice’s sample might teach the docs more about her cancer. Depending on the cells contained in the sample, it might also provide much needed insight into what’s happening inside the lungs of those of us with HPS.
Never in a million years would I want Candice, or anyone else, to have to go through what Candice and her family are experiencing. But, I’m profoundly moved that in the midst of this experience they thought of the value the experience could have to help all of the rest of us with HPS.
It’s a perfect example of how those of us with HPS rely so much on one another on this journey. If one of us should, for example, need a biopsy taken like Candice, we can choose to seize that opportunity to help one another.
If, someday, should anything ever happen to me, I hope my family would also think of the research. I know this sounds morbid, and I don’t mean it to sound that way, but when I go to heaven someday I want Dr. Gahl and his research lab to have dibs on the corpse left behind. And whatever he doesn’t want I hope will be donated to science.
I’m so grateful for all the science that has benefited me, and all the doctors who have known how to care for me because someone else long ago made such a gift. (Family, hope you’re paying attention to this post!) I know there are lots of people who wouldn’t want to do that for lots of reasons, and that’s okay, but it’s my personal wish.
I wanted to take a moment to blog about that decision for two reasons. First, I want to publicly thank Candice and let you all know what a special gift that was. Second, I want to draw a little attention to the need for tissue collection so that the HPS’ers reading this blog can file this away for future reference as something they might want to consider, and something we should all be discussing with our families.
HPS types 1 and 4 cause pulmonary fibrosis. How this happens exactly, however, remains a mystery. One way to uncover the truth about this mystery is to study lung tissue. The problem is that because of the bleeding disorder associated with HPS, doctors are extremely leery to try to take tissue samples from HPS patients that still have healthy lungs. (That’s why right now we do lung lavages for research rather than lung biopsies.) They don’t want to do a procedure that could involve some risk just to obtain research samples. Studying donated lungs from patients with HPS after they’ve passed away is helpful, but what we really need to know is what’s happening in an HPS lung before the fibrosis sets in with a vengeance.
Unfortunately, in Candice’s case, taking a biopsy from her lung couldn’t be avoided. Her doctors needed to determine exactly what was happening with her cancer in order to know how to best treat her – thus there was medical necessity to the risk of causing a bleed. (Thankfully, it seems Candice didn’t have any bleeding complications.)
Candice’s type of cancer is very rare, and so far, we know of no other person with HPS that has developed the same type of cancer, so we don’t think there’s a link. But then again, we’re constantly learning about HPS and documenting health developments, thus it’s really important that those of us that are part of the natural history study communicate major developments in our health to NIH and if relevant, provide samples.
Candice’s sample might teach the docs more about her cancer. Depending on the cells contained in the sample, it might also provide much needed insight into what’s happening inside the lungs of those of us with HPS.
Never in a million years would I want Candice, or anyone else, to have to go through what Candice and her family are experiencing. But, I’m profoundly moved that in the midst of this experience they thought of the value the experience could have to help all of the rest of us with HPS.
It’s a perfect example of how those of us with HPS rely so much on one another on this journey. If one of us should, for example, need a biopsy taken like Candice, we can choose to seize that opportunity to help one another.
If, someday, should anything ever happen to me, I hope my family would also think of the research. I know this sounds morbid, and I don’t mean it to sound that way, but when I go to heaven someday I want Dr. Gahl and his research lab to have dibs on the corpse left behind. And whatever he doesn’t want I hope will be donated to science.
I’m so grateful for all the science that has benefited me, and all the doctors who have known how to care for me because someone else long ago made such a gift. (Family, hope you’re paying attention to this post!) I know there are lots of people who wouldn’t want to do that for lots of reasons, and that’s okay, but it’s my personal wish.
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