.... you go into a Sephora store and leave without testing any products, let alone purchasing anything.
.... you need to be reminded by your best college friend about the time the two of you hired a stripper for your R.A.'s birthday.
.... the 12-year-old working the coffee counter at Border's calls you "m'am".
.... you spend the weekend in New York and are all the way home before you realize that you consumed absolutely NO alcohol.
.... the cute summer sandals you bought on sale have 1.5" heels, not 2.5" heels.
Thursday, July 3, 2008
Wednesday, July 2, 2008
The Rest of It
First, a big wet sloppy kiss to all of you who read yesterday's post and, instead of rolling your eyes in disgust at my obvious play for your sympathy [g!], hopped on the PKD fundraising link and whipped out your credit cards. Talk about getting started with a bang! I'm all verklempt, and thank you from the bottom of my heart, as do Michael and the kids.
A number of you emailed me some very sensible questions about how I developed PKD and what my future holds, and I thought I'd answer them here. (Knowledge is power, baby.)
The form of PKD that I have is genetic and affects one in 500 people. My father was the first in our family tree to develop it (which is a bit of a mystery), and my brothers and I each had a 50% chance of having the disease as well. Luckily, I am the only one of my siblings who has tested positive.
I was diagnosed in my early 20s but did not become symptomatic until a few years ago, when my blood pressure started to climb. There is no way to know how quickly the disease will now progress. I visit my nephrologist every 8 months or so for new blood work, and I keep an eye on my blood pressure from home. Aside from controlling the blood pressure, staying generally healthy and eating a low-protein diet, there is not a lot I can do at this point to keep a rein on things.
When my kidney function decreases to around 20% (maybe 3 years? maybe 5 years?), my older brother -- who is my only family member who has a compatible blood-type -- will go through a battery of tests to determine whether he would be a good prospect to donate me a kidney. The best case scenario is that he passes the screening, I get one of his kidneys and the transplant works (many don't). I'll then spend the rest of my life on immunosuppressants and hoping that my new kidney won't fail (many do). My father's first kidney transplant failed after 20 years, which is a good outcome -- many only last 10 years (or less).
If my brother is not deemed a good match for me, or does not pass the rigorous health screenings, another option is paired donation. This is a relatively new arena, where kidney patients who have willing donors with incompatible blood types "swap" donors to get a better match. Plenty of unknowns, here, but a good potential fall-back position. This would enable my younger brother to get into the act, as well.
If scenarios 1 and 2 fail, the next step is to go on The List. The statistics of The List are pretty horrible. There are currently 76,000 people in the U.S. waiting for kidneys, and fewer than 10,000 of them receive a kidney each year. The List is growing by roughly 40,000 patients each year, and 4,000 die each year before receiving a transplant. Pretty crappy odds.
Dialysis is a life-saving procedure that cleans the blood in much the way the kidneys do. There are several types of dialysis currently available, each with particular drawbacks. None is a cure, and most people feel very sick while on dialysis. Being on dialysis is very restrictive, and is a way to borrow time, not a way to live out one's life. If my first two transplant scenarios fail, I will certainly have to go on dialysis for some period of time, which I dread.
With regard to the liver issues, as long as my liver function doesn't fail (it doesn't, in most PKD patients) and as long as it doesn't get too grossly enlarged, we will likely just leave it alone. (Alas, I fear my bikini days are numbered.) In some instances the docs will aspirate individual cysts if they are very large or infected, but even going in laprascopically creates some scar tissue and is best avoided. I do know some PKD patients who have had liver resections to remove the most grossly affected portion of the liver, but that's only done in extreme cases.
I am one of the lucky ones -- I have private health insurance that will cover the costs of dialysis and/or transplantation, when the time comes. I have group life insurance, disability insurance, and all kinds of financial contingency planning in place to help cover the mortgage when/if I get too sick to work. I have family members willing to donate me a kidney (you'd be surprised at how often that's not the case). I have a husband who loves me for better and for worse. And I have an incredibly supportive network of friends who cheer me on in all of my hairbrained schemes, and yell at me when I'm taking on too much.
It is hard to feel anything other than blessed.
A number of you emailed me some very sensible questions about how I developed PKD and what my future holds, and I thought I'd answer them here. (Knowledge is power, baby.)
The form of PKD that I have is genetic and affects one in 500 people. My father was the first in our family tree to develop it (which is a bit of a mystery), and my brothers and I each had a 50% chance of having the disease as well. Luckily, I am the only one of my siblings who has tested positive.
I was diagnosed in my early 20s but did not become symptomatic until a few years ago, when my blood pressure started to climb. There is no way to know how quickly the disease will now progress. I visit my nephrologist every 8 months or so for new blood work, and I keep an eye on my blood pressure from home. Aside from controlling the blood pressure, staying generally healthy and eating a low-protein diet, there is not a lot I can do at this point to keep a rein on things.
When my kidney function decreases to around 20% (maybe 3 years? maybe 5 years?), my older brother -- who is my only family member who has a compatible blood-type -- will go through a battery of tests to determine whether he would be a good prospect to donate me a kidney. The best case scenario is that he passes the screening, I get one of his kidneys and the transplant works (many don't). I'll then spend the rest of my life on immunosuppressants and hoping that my new kidney won't fail (many do). My father's first kidney transplant failed after 20 years, which is a good outcome -- many only last 10 years (or less).
If my brother is not deemed a good match for me, or does not pass the rigorous health screenings, another option is paired donation. This is a relatively new arena, where kidney patients who have willing donors with incompatible blood types "swap" donors to get a better match. Plenty of unknowns, here, but a good potential fall-back position. This would enable my younger brother to get into the act, as well.
If scenarios 1 and 2 fail, the next step is to go on The List. The statistics of The List are pretty horrible. There are currently 76,000 people in the U.S. waiting for kidneys, and fewer than 10,000 of them receive a kidney each year. The List is growing by roughly 40,000 patients each year, and 4,000 die each year before receiving a transplant. Pretty crappy odds.
Dialysis is a life-saving procedure that cleans the blood in much the way the kidneys do. There are several types of dialysis currently available, each with particular drawbacks. None is a cure, and most people feel very sick while on dialysis. Being on dialysis is very restrictive, and is a way to borrow time, not a way to live out one's life. If my first two transplant scenarios fail, I will certainly have to go on dialysis for some period of time, which I dread.
With regard to the liver issues, as long as my liver function doesn't fail (it doesn't, in most PKD patients) and as long as it doesn't get too grossly enlarged, we will likely just leave it alone. (Alas, I fear my bikini days are numbered.) In some instances the docs will aspirate individual cysts if they are very large or infected, but even going in laprascopically creates some scar tissue and is best avoided. I do know some PKD patients who have had liver resections to remove the most grossly affected portion of the liver, but that's only done in extreme cases.
I am one of the lucky ones -- I have private health insurance that will cover the costs of dialysis and/or transplantation, when the time comes. I have group life insurance, disability insurance, and all kinds of financial contingency planning in place to help cover the mortgage when/if I get too sick to work. I have family members willing to donate me a kidney (you'd be surprised at how often that's not the case). I have a husband who loves me for better and for worse. And I have an incredibly supportive network of friends who cheer me on in all of my hairbrained schemes, and yell at me when I'm taking on too much.
It is hard to feel anything other than blessed.
Tuesday, July 1, 2008
What It's Like
Ironically, while it's the cysts in my kidneys that are going to cause me the worst problems down the road, the cysts in my liver are what cause me the most ongoing discomfort. (One of the quirks of polycystic kidney disease is that it can cause large cysts not only in the kidneys, but in the liver and pancreas as well.) It is rare in PKD for liver function to be impeded by the cysts (though it can happen); unlike the kidneys, the human liver has amazing powers of regeneration and is therefore less vulnerable to the formation of scar tissue that will eventually destroy all my kidney function.
The cysts in my kidneys give me pain occasionally. They twinge, or pinch, or throb for a few moments, or longer, and then stop. By contrast, I am always aware of the cysts in my liver. They cause a distension of my abdomen and my liver presses against my bladder and lungs, giving me indigestion and often making me short of breath. When my liver cysts pinch me, or burst, it feels like a stitch in my side after a hard run and the stitch won't go away. I have dreams of being pregnant, with vivid phantom muscle memories of the little baby heels and elbows kicking and pushing from inside my abdomen, and upon waking, it takes long moments for me to realize that what's growing inside me is not a baby.
I don't know how big my kidneys and liver are right now. My last ultrasound was over a year ago, at which point both livers measured at about 17 cm (normal size for me would be around 10 cm). At that time, the ultrasound tech measured several individual cysts of 6 cm and 7 cm -- each about the size of a lemon. Everything has surely grown since then.
Size isn't really the issue, though. As the cysts in my kidneys grow, they leave scar tissue in their wake, which means there is less and less healthy kidney tissue remaining to do the important work of cleansing the toxins from my blood. More toxins in the blood means more fatigue and less energy -- both physically and mentally.
As of my last blood work, my kidneys are doing about 40% of the job that they would be doing without the cysts and the scar tissue. This isn't too awful -- most people don't have to start on dialysis until they reach about 20%. The question is (has always been), how long do I have before I hit 20%? There is no way to know. Some PKD patients chug along at 40% for a decade or more and then quickly bottom out; some PKD patients follow a slow curve downward through increasing fatigue, nausea, and pain. It remains to be seen.
********
Meanwhile, there is work to be done. Public funding for a cure for PKD is an embarrasment. The NIH's February 2008 report shows that annual research spending per patient is $60. That's right, about the cost of an Olive Garden dinner for four. Compare that with the $2,733 spent on research per cystic fibrosis patient or the $1,880 spent per muscular dystrophy patient and you'll see why there is huge work to be done.
The Polycystic Kidney Foundation is an amazing organization which uses over 80% of its budget to directly fund research and provide patient education regarding PKD. They are the only group in the U.S. dedicated solely to eradicating PKD, and last year they funded $4 million in research -- a full 9% of what the NIH spent on PKD in 2007.
Last year, with the help of many wonderful friends and family members, Michael, the kids and I raised $9,000 for the PKD Foundation. (You can read all about it here.) This year, I'd like to make that number an even $10,000. I hope you'll join us. My fundraising page is up and ready for action (and will remain up on the sidebar to your right), and as always, I will post our progress here on the blog. It really will take a village to eradicate this disease, and I'm determined to do so before Quinlan's kidneys start giving him any trouble.
How You Can Help
The cysts in my kidneys give me pain occasionally. They twinge, or pinch, or throb for a few moments, or longer, and then stop. By contrast, I am always aware of the cysts in my liver. They cause a distension of my abdomen and my liver presses against my bladder and lungs, giving me indigestion and often making me short of breath. When my liver cysts pinch me, or burst, it feels like a stitch in my side after a hard run and the stitch won't go away. I have dreams of being pregnant, with vivid phantom muscle memories of the little baby heels and elbows kicking and pushing from inside my abdomen, and upon waking, it takes long moments for me to realize that what's growing inside me is not a baby.
I don't know how big my kidneys and liver are right now. My last ultrasound was over a year ago, at which point both livers measured at about 17 cm (normal size for me would be around 10 cm). At that time, the ultrasound tech measured several individual cysts of 6 cm and 7 cm -- each about the size of a lemon. Everything has surely grown since then.
Size isn't really the issue, though. As the cysts in my kidneys grow, they leave scar tissue in their wake, which means there is less and less healthy kidney tissue remaining to do the important work of cleansing the toxins from my blood. More toxins in the blood means more fatigue and less energy -- both physically and mentally.
As of my last blood work, my kidneys are doing about 40% of the job that they would be doing without the cysts and the scar tissue. This isn't too awful -- most people don't have to start on dialysis until they reach about 20%. The question is (has always been), how long do I have before I hit 20%? There is no way to know. Some PKD patients chug along at 40% for a decade or more and then quickly bottom out; some PKD patients follow a slow curve downward through increasing fatigue, nausea, and pain. It remains to be seen.
********
Meanwhile, there is work to be done. Public funding for a cure for PKD is an embarrasment. The NIH's February 2008 report shows that annual research spending per patient is $60. That's right, about the cost of an Olive Garden dinner for four. Compare that with the $2,733 spent on research per cystic fibrosis patient or the $1,880 spent per muscular dystrophy patient and you'll see why there is huge work to be done.
The Polycystic Kidney Foundation is an amazing organization which uses over 80% of its budget to directly fund research and provide patient education regarding PKD. They are the only group in the U.S. dedicated solely to eradicating PKD, and last year they funded $4 million in research -- a full 9% of what the NIH spent on PKD in 2007.
Last year, with the help of many wonderful friends and family members, Michael, the kids and I raised $9,000 for the PKD Foundation. (You can read all about it here.) This year, I'd like to make that number an even $10,000. I hope you'll join us. My fundraising page is up and ready for action (and will remain up on the sidebar to your right), and as always, I will post our progress here on the blog. It really will take a village to eradicate this disease, and I'm determined to do so before Quinlan's kidneys start giving him any trouble.
How You Can Help
Monday, June 30, 2008
My Ten Favorite Things about New York City
10. My sister-in-law's loft on the upper East side
9. La Maison du Chocolat
8. Walking everywhere
7. Manhattan Theater Club
6. Seven floors of women's clothing at Macy's midtown
5. Bouchon Bakery
4. Losing 82 lbs of water weight*
3. Corner bodegas that are open all night
2. Chelsea Market
And my most favorite thing about New York City:
1. Going braless in a tank top with no shred of self-consciousness!
* This figure is an estimate
9. La Maison du Chocolat
8. Walking everywhere
7. Manhattan Theater Club
6. Seven floors of women's clothing at Macy's midtown
5. Bouchon Bakery
4. Losing 82 lbs of water weight*
3. Corner bodegas that are open all night
2. Chelsea Market
And my most favorite thing about New York City:
1. Going braless in a tank top with no shred of self-consciousness!
* This figure is an estimate
Friday, June 27, 2008
Spitfire Designs
I was at a fundraiser this weekend for our venerable neighborhood community theatre and was smitten by some pieces donated by a local jeweler for auction. Unfortunately, I lost the piece I was bidding on; fortunately, she has a website.
I'd love to post a few pictures of her work here but can't figure out how to get around the flash animation on her site. Just click through -- you won't be sorry.
(Michael, you have my permission to bookmark her site. Just sayin'.)
I'd love to post a few pictures of her work here but can't figure out how to get around the flash animation on her site. Just click through -- you won't be sorry.
(Michael, you have my permission to bookmark her site. Just sayin'.)
Public Service Announcement the Third
Don't drive your car when it is leaking gasoline from the gas tank. Have it towed. Apparently, a spark from the engine could cause an explosion.
I was too busy cursing at the $53 worth of gas that was dripping out onto the pavement to worry about such things, and my mechanic was only about 100 yards away, so I drove. Luckily, I did not combust.
No verdict on the car yet this morning......
I was too busy cursing at the $53 worth of gas that was dripping out onto the pavement to worry about such things, and my mechanic was only about 100 yards away, so I drove. Luckily, I did not combust.
No verdict on the car yet this morning......
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