Monday, November 16, 2009
Good news...I think
I received a notice from Kaiser that their transplant team has approved me for a liver transplant. It will be done at UCSF. They have a better than average success rate for liver transplants, so that is reassuring. I am scared, but I think I'm running out of options. My kidney function is decreasing and will only get worse. I am one of the very few, very fortunate amyloidosis patients. My type has "cure" and "excellent outlook" after it. I should be counting my blessings and exercising faith right now. I have had so many prayers answered. Thanks to everyone. I appreciate the support.
Thursday, November 12, 2009
Lab results
I just got back the results from the tests I had on Wednesday. The good news is that my GFR improved to 29 and my serum creatnine improved to 1.80. My serum potassium, calcium, phosphorus, and albumin were all within normal limits. Unfortunately my hemoglobin and hematocrit were lower than they should be. That means I am slightly anemic and will need to rely on more blusher. (Some problems are so easy to solve.)
Kidney Awareness Class
We've been scheduled to go to this for awhile. It is a two hour class at Kaiser to encourage people that are at risk for kidney disease to help themselves a little. Since my kidney disease is so much beyond my control, the doctor said I could have a pass on this, but Lon wanted to go (wouldn't a real date have been a little better?). We went. There were about 20 people there and we received a half inch stack of information. After the visit with the dietician I was skeptical of its value, but it was actually pretty good. We learned some anatomy stuff and some eating ideas. It seemed very practical. She also passed out copies of our lab values. Mine were the worst (not exactly a good time to be at the head of the class); she wondered why I wasn't in the "Choices" class (to decide what kind of dialysis or transplant). After class I explained what I had and I guess it made a little more sense. I was the only one in there where a liver transplant would fix my kidney disease. Doesn't really make much sense, does it?
Robert comes to visit
This past weekend Robert came to visit his sick Mom. Marie was a champ--she had all five of their children while he was here. I appreciated his visit and her sacrifice so much. It was so nice to see him. I made his favorite raviolis and chocolate chip cookies the day he came. It seemed like old times to see him draped in front of the fridge door. He found the ravs right away. They didn't even last 24 hours.
He wanted to wash cars and pull weeds (probably not quite as much as we wanted him to wash cars and pull weeds), but it rained the whole time he was here. He did give Lon a break from dish duty. We were so impressed with his skills. Lon called me to the kitchen to admire the neatly stacked dishes that were drying.
Alan and Ellen came to dinner on Sunday and Robert helped me make maca before church. When the boys get together it seems like they can just pick up from the last time they were together. On Friday while we were at the temple Alan took Robert to Costco and they got the movie "Driving Miss Daisy." They knew it was the last picture that was rated PG to garner Best Picture at the Academy Awards. How do they know all that random stuff????? If I ever need a lifeline for a game show.........
He wanted to wash cars and pull weeds (probably not quite as much as we wanted him to wash cars and pull weeds), but it rained the whole time he was here. He did give Lon a break from dish duty. We were so impressed with his skills. Lon called me to the kitchen to admire the neatly stacked dishes that were drying.
Alan and Ellen came to dinner on Sunday and Robert helped me make maca before church. When the boys get together it seems like they can just pick up from the last time they were together. On Friday while we were at the temple Alan took Robert to Costco and they got the movie "Driving Miss Daisy." They knew it was the last picture that was rated PG to garner Best Picture at the Academy Awards. How do they know all that random stuff????? If I ever need a lifeline for a game show.........
Off to the doctor...again
I had an appointment with the kidney doctor yesterday. I had jotted some questions on the back of a pizza coupon and he thought the coupon was for him. He should know that I'm the only wise guy in the room.
We had a discussion about liver transplant vs. kidney/liver transplant. Conventional wisdom doesn't exist at all because there is so little experience with this fibrinogen amyloidosis. My kidney function is pretty shaky--I think I'm around 25%. The experts that I spoke with are sticking with a liver only transplant though. They think my kidneys will shape up. That's the way Lon and I are leaning too. There are less rejection issues with liver transplant, too.
We also talked about my blood pressure meds. We're trying to get the most effective combo with the fewest side effects. It's not that easy. He changed one and my hands don't have the shakes/tremors.
I also asked him about the foods I should be eating. I told him I didn't think I could handle all those TV dinners. He agreed. I'm trying for healthy, fresh, and moderation. I could be glowing!!
I asked him if he had ever seen the documentary film, "Super-Size Me." It's the true story of a guy who ate at McDonald's three meals a day for a month (I think). He kept meticulous records and was under the supervision of a doctor. He got very sick with the McShakes and the McBarfs and all sorts of McProblems. It's a very good movie, but you won't be real eager to eat at McDonalds for a while.
We had a discussion about liver transplant vs. kidney/liver transplant. Conventional wisdom doesn't exist at all because there is so little experience with this fibrinogen amyloidosis. My kidney function is pretty shaky--I think I'm around 25%. The experts that I spoke with are sticking with a liver only transplant though. They think my kidneys will shape up. That's the way Lon and I are leaning too. There are less rejection issues with liver transplant, too.
We also talked about my blood pressure meds. We're trying to get the most effective combo with the fewest side effects. It's not that easy. He changed one and my hands don't have the shakes/tremors.
I also asked him about the foods I should be eating. I told him I didn't think I could handle all those TV dinners. He agreed. I'm trying for healthy, fresh, and moderation. I could be glowing!!
I asked him if he had ever seen the documentary film, "Super-Size Me." It's the true story of a guy who ate at McDonald's three meals a day for a month (I think). He kept meticulous records and was under the supervision of a doctor. He got very sick with the McShakes and the McBarfs and all sorts of McProblems. It's a very good movie, but you won't be real eager to eat at McDonalds for a while.
Tuesday, November 10, 2009
The Dietician
We just got back from the dietitian. I am still processing (or reeling from) what she said. My dietary needs include low protein, low sodium, low fat, and the latest, low potassium. It was an interesting visit. According to her I am doing OK with the salt, potassium, and fat. Protein has become an issue. Low protein means almost no meat, since many other foods contain protein. For example, bread, pasta, rice, and most things that fill you up have some protein, so if you factor in that, it doesn't leave any room for salmon, chicken, and especially beef. She recommended the "Healthy Choice" kinds of TV dinners. With the nutritional labeling and portion controlled sizes, it made sense, but they have so much other added junk that the idea of eating them several times a week creeps me out. I kind of like fresh food. The big challenge will be getting enough calories to make sure I don't just waste away (seriously).
I was also concerned with traveling. She had a handy book with nutrition information, but everything just seemed really past the amounts I should be eating. I also asked about eating at other people's houses (this is you, kids). I told her I didn't want anyone making a fuss, but she assured me people really wanted to accommodate my needs. She had a personal anecdote to add to that. She has company coming from India. They don't eat any root vegetables like potatoes or carrots because they might contribute to the death of insects that may be clinging to them. She told us she is happy to adjust her cooking to their needs. Everything that she said after that was kind of a blur. All I could picture were buggy potatoes and carrots.
I was also concerned with traveling. She had a handy book with nutrition information, but everything just seemed really past the amounts I should be eating. I also asked about eating at other people's houses (this is you, kids). I told her I didn't want anyone making a fuss, but she assured me people really wanted to accommodate my needs. She had a personal anecdote to add to that. She has company coming from India. They don't eat any root vegetables like potatoes or carrots because they might contribute to the death of insects that may be clinging to them. She told us she is happy to adjust her cooking to their needs. Everything that she said after that was kind of a blur. All I could picture were buggy potatoes and carrots.
Thursday, November 5, 2009
And today...
As for today I am feeling pretty good. I am jumping through the doctor hoops--it seems they always want one more test, so I am feeling pretty tough, especially after that bone marrow biopsy. I am trying to eat healthy and am working on building up my strength for what's ahead. I have told the doctors that I am going to make them look good--a promise I intend to keep. My energy level is usually pretty good--once in a while I have a bad day, but that's OK. I am walking at least four days a week and feeling good about it. I haven't quite figured out why I should be having this particular trial, but that's OK, too. The more important part is how I will handle it. I am so appreciative of all I have--a husband who is at my side constantly and ready to help, kids and grandkids who are ever solicitous, friends who are ready and willing to step in and help. I am so grateful for prayer, fasting, and Priesthood blessings. I would be a total wreck without them.
Genetics
Lon went to the conference for the genetics, but I listened too!
Familial amyloidosis means just that--it's a family thing! The bummer gene was with me at birth, therefore I had to get it from either my Mom or my Dad. In turn they had to be a carrier and get it from their Mon or Dad. So where is the smoking gun? I have spent hours considering this and still don't know--it's really not necessary to know, but it's so frustrating not knowing. We know it's passed at a rate of 50%, so half of my kids may have the gene. My Mom was one of six and my Dad one of seven, so the odds would be that someone had the actual disease, but I can't think of anyone who might have had it. It is more common in certain kindreds, and based on that I could probably rule out my Czech paternal grandfather. My Dad's Mom was born in Sweden and that could be a possibility. She also died relatively young--48--in the flu epidemic in 1918. So if she had the disease she died before it appeared. The other possibility could be my Mom's Dad. He was born in Scotland, but he lived to be 73--too old I think. Her Mom is Danish and that isn't a kindred that is usually affected.
What does all this mean? It's actually a good thing-- a very good thing. It means that the penetrance, the rate that people actually get the disease, is very low, and that the age of penetrance is relatively late in life--kind of like me! So it means the kids could get tested to know for sure, or just make sure that when they get around 50 to get tested every year for any signs of kidney disease--something that should show up in even the most minimal of a physical.
Do I still feel awful that I probably passed this on--of course! But it is manageable now and in the future it will be even more manageable. Hopefully no one will ever get to the point of kidney failure that I am at right now.
Familial amyloidosis means just that--it's a family thing! The bummer gene was with me at birth, therefore I had to get it from either my Mom or my Dad. In turn they had to be a carrier and get it from their Mon or Dad. So where is the smoking gun? I have spent hours considering this and still don't know--it's really not necessary to know, but it's so frustrating not knowing. We know it's passed at a rate of 50%, so half of my kids may have the gene. My Mom was one of six and my Dad one of seven, so the odds would be that someone had the actual disease, but I can't think of anyone who might have had it. It is more common in certain kindreds, and based on that I could probably rule out my Czech paternal grandfather. My Dad's Mom was born in Sweden and that could be a possibility. She also died relatively young--48--in the flu epidemic in 1918. So if she had the disease she died before it appeared. The other possibility could be my Mom's Dad. He was born in Scotland, but he lived to be 73--too old I think. Her Mom is Danish and that isn't a kindred that is usually affected.
What does all this mean? It's actually a good thing-- a very good thing. It means that the penetrance, the rate that people actually get the disease, is very low, and that the age of penetrance is relatively late in life--kind of like me! So it means the kids could get tested to know for sure, or just make sure that when they get around 50 to get tested every year for any signs of kidney disease--something that should show up in even the most minimal of a physical.
Do I still feel awful that I probably passed this on--of course! But it is manageable now and in the future it will be even more manageable. Hopefully no one will ever get to the point of kidney failure that I am at right now.
First ever familial amyloidosis conference
What an unusual course Lon and I have been on these last six or seven months. It seems that at every turn there has been someone "plop" right in our way to help us along. Two weeks after my diagnosis was this first ever conference. We are so glad we went.
There are three doctors in the US who are experts in this field and they were all there--including the doctor who identified my variation of familial amyloidosis. That's an indication of how new all of this is.
There are two major types of familial amyloidosis--TTR or transthyretin and non-TTR. The TTR is by far the more common of this rare disease. It has many mutations that affect about every organ and system. Although it is extremely rare there were four drugs being investigated to treat it. They involve altering or turning off the specific gene that causes it. It was pretty exciting sitting there listening to some truly cutting edge technology.
I was the only one there with my variation of the non-TTR type--fibrinogen alpha chain. I was also the only one there who had no clue whatsoever who I inherited it from. My amyloid is made exclusively in my liver and only messes up my kidney. Unlike the TTR type that can be "neutralized" because there are other avenues in the body to get the transrethin, my body needs the fibrinogen because that clots my blood. The absolutely great news is that my variation is the only type of amyloidosis that has "cure" and "excellent outlook" after it. Unfortunately it comes with a high price tag--a liver transplant. If I get a liver transplant amyloid will no longer be produced and hopefully my kidney will be able to recover enough to do its job. At this point my fate is in the hands of the Kaiser transplant committee. If they agree that a transplant would be beneficial, then it's on to the UCSF transplant committee. Am I scared? Nope, I'm terrified. Lon thinks that now would be a good time to be a good example--brave and stuff. I've always told the kids that when something is hard they need to reach all the way down to their toes and gather courage--I guess I'll have to put that to the test.
I got to meet the doctor that recomended the transplant. I wanted to make sure she knew I was a real live, kicking and screaming person. I also had some good one on one time with both the other doctors that were experts. It was good to get confirmation that this is probably the course of action I should be taking.
I also met others who had had liver transplants. Of course they were the ones who were doing well. They were happy that they had had the transplant. They had the TTR type and a liver transplant isn't a cure, but more of a bandaid--a good bandaid though. They have amyloid that is produced elsewhere in the body, but the liver is still the major production point and it slows down the production quite a bit. They said the hospital stay is about five days. And yes, my bikini days will be over.
There are three doctors in the US who are experts in this field and they were all there--including the doctor who identified my variation of familial amyloidosis. That's an indication of how new all of this is.
There are two major types of familial amyloidosis--TTR or transthyretin and non-TTR. The TTR is by far the more common of this rare disease. It has many mutations that affect about every organ and system. Although it is extremely rare there were four drugs being investigated to treat it. They involve altering or turning off the specific gene that causes it. It was pretty exciting sitting there listening to some truly cutting edge technology.
I was the only one there with my variation of the non-TTR type--fibrinogen alpha chain. I was also the only one there who had no clue whatsoever who I inherited it from. My amyloid is made exclusively in my liver and only messes up my kidney. Unlike the TTR type that can be "neutralized" because there are other avenues in the body to get the transrethin, my body needs the fibrinogen because that clots my blood. The absolutely great news is that my variation is the only type of amyloidosis that has "cure" and "excellent outlook" after it. Unfortunately it comes with a high price tag--a liver transplant. If I get a liver transplant amyloid will no longer be produced and hopefully my kidney will be able to recover enough to do its job. At this point my fate is in the hands of the Kaiser transplant committee. If they agree that a transplant would be beneficial, then it's on to the UCSF transplant committee. Am I scared? Nope, I'm terrified. Lon thinks that now would be a good time to be a good example--brave and stuff. I've always told the kids that when something is hard they need to reach all the way down to their toes and gather courage--I guess I'll have to put that to the test.
I got to meet the doctor that recomended the transplant. I wanted to make sure she knew I was a real live, kicking and screaming person. I also had some good one on one time with both the other doctors that were experts. It was good to get confirmation that this is probably the course of action I should be taking.
I also met others who had had liver transplants. Of course they were the ones who were doing well. They were happy that they had had the transplant. They had the TTR type and a liver transplant isn't a cure, but more of a bandaid--a good bandaid though. They have amyloid that is produced elsewhere in the body, but the liver is still the major production point and it slows down the production quite a bit. They said the hospital stay is about five days. And yes, my bikini days will be over.
We found the Fountain of Youth!!
Yes, we did! All of the shops in Nauvoo are manned (and womaned?) by Senior missionaries. There are bread bakers, weavers, candle dippers, coopers, blacksmiths, wainwrights, brick makers, rope makers--all kinds of period occupations. In front of each shop there are usually two cars--with license plates from Utah, Arizona, Texas, Colorado--that belong to these missionaries. They spend all day demonstrating and teaching about their particular craft. The kicker is that every night at 7 pm and 8:15 pm these missionaries gather together at the Cultural Hall to present a show, "Rendezvous in Old Nauvoo!" It is an hour of energetic singing and acting with a little bit of almost dancing thrown in. These septuagenarians weren't in rocking chairs watching reruns waiting for visits from there kids--they were so active and happy. We were so impressed with them. When I grow up I think I'd like to be a missionary too!
Nauvoo
Nauvoo is indeed a special place. The season was changing and the majority of tourists gone. The Church has rebuilt many of the shops and houses, but there were plenty of empty lots and open spaces. It gave it a lonely and sad feeling, especially since we were bundled up to stay warm. How hard it must have been to head West in the middle of winter to an unknown destination.
We were privileged to attend a session at the Temple and do sealings afterwards.
We were privileged to attend a session at the Temple and do sealings afterwards.
Wednesday, November 4, 2009
Good eats
This would be the happiest hot dog I have ever seen. The relish is really that day-glo green, and now that I look at it again, the hot dog is an unusually bright shade of red. This might even be a Christmas dog due to the festive colors. I have kind of, sort of been following my diet, but I admit that I did have a couple of bites.
We only had one free day to eat in Chicago, (food was part of the conference) but I would like a few days to try this menu! So many dogs; so little time and the plane leaves in 2 hours. Really if you visit Chicago, you need to visit "Hot Doug's".
Quote of the day " There are no two finer words in the English language than 'encased meats', my friend." --Secret Robbie
Interesting tidbits
We saw some interesting things on our trip. Here are some of them.
We stayed in Hotel Nauvoo. It was a cute, little place. This sign was on the wall in our room. It worked like a charm because we did have sweet dreams.
Randy loaned us his GPS. It took us on a very round about way to Nauvoo. We got there about 9 p.m. It took us down this one lane gravel road for about two miles. We laughed and laughed about it even though we felt like we were totally lost in the dark, in some random cornfield. We had to go back during the day to get this picture and figure out how in the world we ended up there. Technology is good but we sure wished we had a good old-fashioned map to tell us where we were.
This is in Chicago. If we spent enough time driving around I think we could have found hot dog places named for every one of our grandkids. This one's for you Teddy.
I'd never seen such a good-looking and functional clock. On the clock face it says, "It's time for hardware." I don't think I'll ever think of hardware in the same way forever.
What a serendipitous day this was. We were driving around Chicago (yes, Chicago and not Pisa) when we happened upon this leaning tower. It is in the parking lot of a YMCA--really. That's me at the bottom. It was built to honor Galileo and it stores water.
We stayed in Hotel Nauvoo. It was a cute, little place. This sign was on the wall in our room. It worked like a charm because we did have sweet dreams.
Randy loaned us his GPS. It took us on a very round about way to Nauvoo. We got there about 9 p.m. It took us down this one lane gravel road for about two miles. We laughed and laughed about it even though we felt like we were totally lost in the dark, in some random cornfield. We had to go back during the day to get this picture and figure out how in the world we ended up there. Technology is good but we sure wished we had a good old-fashioned map to tell us where we were.
We found this fungus on a tree. We thought it was alien.
This is in Chicago. If we spent enough time driving around I think we could have found hot dog places named for every one of our grandkids. This one's for you Teddy.
I'd never seen such a good-looking and functional clock. On the clock face it says, "It's time for hardware." I don't think I'll ever think of hardware in the same way forever.
What a serendipitous day this was. We were driving around Chicago (yes, Chicago and not Pisa) when we happened upon this leaning tower. It is in the parking lot of a YMCA--really. That's me at the bottom. It was built to honor Galileo and it stores water.
Sunday, November 1, 2009
Cautiously optimistic
There has been so much going on since we got here. I will be adding more blogs when we get back Tuesday night,but I must make a couple of comments.
There are about fifty people here who have familial amyloidosis. I am the only one who has my type--one of the doctor's commented that "only a handful" has it. The doctors are amazing--with this disease they have to be both researchers and doctors. I have been told that there are a couple of options and will be talking (and teaching) my doctors. These doctors are very generous with their time and resources. I have phone numbers and will probably stay in contact until we make some kind of decision.
It's Fast Sunday and we are both really feeling the power of prayers said in our behalf. How grateful we are for friends and family!
We love and appreciate you all so much.
There are about fifty people here who have familial amyloidosis. I am the only one who has my type--one of the doctor's commented that "only a handful" has it. The doctors are amazing--with this disease they have to be both researchers and doctors. I have been told that there are a couple of options and will be talking (and teaching) my doctors. These doctors are very generous with their time and resources. I have phone numbers and will probably stay in contact until we make some kind of decision.
It's Fast Sunday and we are both really feeling the power of prayers said in our behalf. How grateful we are for friends and family!
We love and appreciate you all so much.
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