Monday, August 12, 2013
Today we met with our local MLA to ask him for his support. He got SO fired up about the subject when we told him our story and the meeting went much longer than we'd anticipated! It was so encouraging. I walked away with a few more ideas about how we can reach the public with our message and better communicate the value of funding for Soliris for each and every aHUS patient. I have so much more work to do since meeting with him but I'm excited to do it for all the patients I met at the conference, and for all the patients I know are out there struggling!
Saturday, August 3, 2013
After Soliris
Remember these pictures?
We'll call them the Before Soliris pictures.
We'll call them the Before Soliris pictures.
Well, now his treatment looks like this:
That's our couch. In our home. He sits there for about an hour and watches his TV or plays on his phone. The nurse arrives and administers Soliris through an IV and then she packs up and leaves. And he goes on about his business, whatever it may be on that day. He doesn't have the hour and a half drive into Vancouver. He doesn't have to take enough Benedryl to take down a horse, knocking him unconscious for hours and causing the foggy brain he used to have for days afterwards. He doesn't have life-threatening allergic reactions (yes, despite the Benedryl!) or come home and go to bed because he's feeling so miserable, missing whatever family event we'd planned or time spent with his kids.
And here it is. His life-saving medicine. We are so grateful to the drug company for making sure our lives are so much easier and that his access to Soliris won't be cut off. They do this for people all around the world, you know. Give them their million dollar medicine. For free. It kinda makes it hard to demonize the drug company when you think about all the millions of dollars they've donated to the average joe every single year.
On Monday Brian and I have a meeting with our local MLA. It should be interesting! We'll tell our story and try to see if we can get a commitment from him to support us in a practical way. The rest of the week we'll try to carry on as normal, but we'll also be expecting more media interest. Interviewing is my least favorite thing to do but it's so necessary to raise public awareness. I just hope that this time I feel REALLY good about the interview and that the key points come across loud and clear. I may have to practice on my kids. They'll love that.
Friday, August 2, 2013
aHUS Q & A
With the recent articles and interviews now out, I'm interested to see the public comments on each of the stories. And I realize that there are so many misconceptions floating around out there. So it's time to address some of them here!
Q: Why $750,000 per year per patient for Soliris?
A: $750,000 is a figure thrown out by the media recently. I'm not really sure where they got that number from but it sounds astronomical, doesn't it? (To be fair, a few newspapers have reported it as $450,000 a year. Not much better.) Well, the number is wrong. Basically the dosage is different for every patient. It depends on weight, age, and severity of the disease.
Q: Why is it so expensive?
A: If you consider the millions of dollars the drug company spent in research, pinpointing exactly what they want the drug to do, paying for clinical trials and marketing the drug, and paying to have the drug developed every month to give it to the consumer, the cost doesn't seem so high. Soliris' producer, Alexion, invested nearly $1 billion in research and development over 15 years before approval of Soliris, its first product, says Jennifer Devine, a spokeswoman for Alexion.1 In the case of colorectal cancer, just as an example, you have approximately 140,000 new cases a year in the US. Each of those 140,000 people will cost the medical system a certain amount of money in pharmaceuticals. It adds up. The dollar amount the government spends on treatment for them is divided up by the amount of patients. In Canada we have so FEW aHUS patients to cover the cost of the drug, so effectively the cost per person will be higher. But the total cost? No. And yes, they DO want to make some money on top. After all that time and effort, wouldn't you?
Q: How on earth do you pronounce your last name??
A: Thanks. An easy one. Phonetically it's pronounced "Chep-Ke-Ma".
Q: We need to think about the needs of the many, not the few. Shouldn't you just accept that this is life and stop expecting the Canadian government to foot the bill for your medical expenses?
A: (Yes, this question has been asked and personally I think it's a good one) Brian is 39. Yes he'd love to see his children grow up but, for the most part, he's okay with what is happening to his body. It's not fair, but it's life. We're not fighting for funding for HIS sake, though. He is one of the lucky ones. He gets Soliris on a compassionate basis from the drug company, free of charge. We have been told that they will not cut him off. But in April we met children that have aHUS and they aren't so lucky. One of these children is an adorable little blonde girl that captured our hearts. Does she deserve this? Who in their right minds could tell her "Sorry, but it's just not worth our while to keep you alive." We're doing this for her. And for the energetic 11 year old boy we met. And the beautiful preteen girl that is just starting to dream about life outside of her medical issues.
Q: Seeing as this disease is genetic, we think you should consider making sure you don't procreate and when your children become adults they should think about being tested before procreating.
A: Ha ha ha! Oh wait. You're serious. We have very serious discussions with our children about this disease, as you can imagine. One thing we've decided to do is give our children the gift of choice. When THEY choose, they will get tested. Until then we are doing everything in our power to avoid triggers that will cause the activation of the disease in them. This may even be unnecessary seeing as they only have a 50% chance of the gene mutation, and WITH the gene mutation present they have a 50% chance of developing aHUS. Which is why it's so rare. As far as people who have this disease continuing to procreate? We all have our opinions but, ultimately, it's a personal decision.
1. http://www.ottawacitizen.com/health/Rare+condition+could+leave+year+drug+orphan/8738395/story.html
Q: Why $750,000 per year per patient for Soliris?
A: $750,000 is a figure thrown out by the media recently. I'm not really sure where they got that number from but it sounds astronomical, doesn't it? (To be fair, a few newspapers have reported it as $450,000 a year. Not much better.) Well, the number is wrong. Basically the dosage is different for every patient. It depends on weight, age, and severity of the disease.
Q: Why is it so expensive?
A: If you consider the millions of dollars the drug company spent in research, pinpointing exactly what they want the drug to do, paying for clinical trials and marketing the drug, and paying to have the drug developed every month to give it to the consumer, the cost doesn't seem so high. Soliris' producer, Alexion, invested nearly $1 billion in research and development over 15 years before approval of Soliris, its first product, says Jennifer Devine, a spokeswoman for Alexion.1 In the case of colorectal cancer, just as an example, you have approximately 140,000 new cases a year in the US. Each of those 140,000 people will cost the medical system a certain amount of money in pharmaceuticals. It adds up. The dollar amount the government spends on treatment for them is divided up by the amount of patients. In Canada we have so FEW aHUS patients to cover the cost of the drug, so effectively the cost per person will be higher. But the total cost? No. And yes, they DO want to make some money on top. After all that time and effort, wouldn't you?
Q: How on earth do you pronounce your last name??
A: Thanks. An easy one. Phonetically it's pronounced "Chep-Ke-Ma".
Q: We need to think about the needs of the many, not the few. Shouldn't you just accept that this is life and stop expecting the Canadian government to foot the bill for your medical expenses?
A: (Yes, this question has been asked and personally I think it's a good one) Brian is 39. Yes he'd love to see his children grow up but, for the most part, he's okay with what is happening to his body. It's not fair, but it's life. We're not fighting for funding for HIS sake, though. He is one of the lucky ones. He gets Soliris on a compassionate basis from the drug company, free of charge. We have been told that they will not cut him off. But in April we met children that have aHUS and they aren't so lucky. One of these children is an adorable little blonde girl that captured our hearts. Does she deserve this? Who in their right minds could tell her "Sorry, but it's just not worth our while to keep you alive." We're doing this for her. And for the energetic 11 year old boy we met. And the beautiful preteen girl that is just starting to dream about life outside of her medical issues.
Q: Seeing as this disease is genetic, we think you should consider making sure you don't procreate and when your children become adults they should think about being tested before procreating.
A: Ha ha ha! Oh wait. You're serious. We have very serious discussions with our children about this disease, as you can imagine. One thing we've decided to do is give our children the gift of choice. When THEY choose, they will get tested. Until then we are doing everything in our power to avoid triggers that will cause the activation of the disease in them. This may even be unnecessary seeing as they only have a 50% chance of the gene mutation, and WITH the gene mutation present they have a 50% chance of developing aHUS. Which is why it's so rare. As far as people who have this disease continuing to procreate? We all have our opinions but, ultimately, it's a personal decision.
1. http://www.ottawacitizen.com/health/Rare+condition+could+leave+year+drug+orphan/8738395/story.html
Welcome
Welcome to our blog! This blog is about our journey through Brian's Atypical Hemolytic Uremic Syndrome diagnosis and our plea for public funding for his only medical source of hope, a drug called Soliris. A year ago he started on Soliris on a compassionate basis by the drug company, Alexion. We're so thankful to them for changing the course of our lives. What once was a dark, bleak future is now one filled with the hope and promise any young family could wish for. Thank you for your interest in our lives!
Thursday, August 1, 2013
Brian
Tjepkema holds a package of Soliris, a drug he needs to treat a rare
genetic disorder known as atypical Hemolytic Uremic Syndrome at his
Abbotsford home on Thursday. Photograph by: Ric Ernst
, PNG photo
It’s one of the most expensive drugs in the world, but without it, an Abbotsford father says he wouldn’t be able to live. Brian Tjepkema has atypical hemolytic uremic syndrome, also known as
aHUS. He was diagnosed in 2010 with the ultra-rare disorder in which the
immune system attacks the body, damaging vital organs and often
resulting in kidney failure.
“I couldn’t function,” he told The Province. “I couldn’t be the dad I should be to my kids.”
Thanks to an innovative new drug, Soliris, the father-of-two no longer has to deal with the extreme fatigue, headaches, weakness and debilitating nausea. Before Tjepkema started the treatment, he underwent four-hour weekly plasma exchange treatments— where his blood was removed from his body, the plasma separated and replaced with donor plasma— which caused severe allergic reactions. When Tjepkema’s doctor heard about Soliris, he contacted the drug maker. Soliris has put many aHUS patients in remission and improved their kidney function to the point that they can stop other treatments like dialysis and plasma exchange. But now he fears others won’t have access to the drug he gets for free from the drug maker on a compassionate basis.
While it varies for each patient, the drug costs about $450,000 per patient per year, making it one of the most expensive treatments in the world, according to the Canadian Organization for Rare Disorders.
Health Canada approved Soliris for the treatment of aHUS in March, but earlier this week, the Canadian Agency for Drugs and Technologies in Health released a common drug review recommending the provinces and territories not fund the drug.
The review stated the benefits of Soliris were not adequately established as tests did not include a randomized control group. Common drug reviews aims to compare drugs with competitors, determine whether a drug provides value for money, and give advice on whether it is cost-effective for provincial plans to cover.
But Durhane Wong-Rieger, president of the Canadian Organization for Rare Disorders, said ultra-rare diseases, by their very nature, cannot be subjected to the same review framework as other types of drugs.
The disease affects an estimated five in every one million people, according to Dr. Christoph Licht.
As a lead investigator in the international clinical trial for Soliris, Licht said he felt it was unethical to only give the drug to some patients. Control groups are often not included in drug trials for such rare and aggressive diseases, he said.
“I don’t see a way to have done it differently or to ever do it differently,” he said.
While the recommendation to not fund Soliris for aHUS was disappointing, Wong-Rieger said it was not surprising.
In 2009, the review also recommended Soliris not be funded for the treatment of paroxysmal nocturnal hemoglobinuria, or PNH, another ultra-rare and life-threatening disorder. In 2011, B.C. Pharmacare eventually agreed to fund Soliris for the treatment of that disorder despite the negative review.
That gives hope to Tjepkema for others, like his cousin, who haven’t been able to get Soliris for free from the drug maker. Tjepkema said his cousin, who has the same genetic disorder, is in kidney failure and on dialysis. His cousin was previously misdiagnosed, a common problem with the incredibly rare syndrome, and underwent a kidney transplant.Because he isn’t on Soliris, his new kidney is also failing, but he can’t go on the transplant wait list again until he gets the drug.
“I couldn’t function,” he told The Province. “I couldn’t be the dad I should be to my kids.”
Thanks to an innovative new drug, Soliris, the father-of-two no longer has to deal with the extreme fatigue, headaches, weakness and debilitating nausea. Before Tjepkema started the treatment, he underwent four-hour weekly plasma exchange treatments— where his blood was removed from his body, the plasma separated and replaced with donor plasma— which caused severe allergic reactions. When Tjepkema’s doctor heard about Soliris, he contacted the drug maker. Soliris has put many aHUS patients in remission and improved their kidney function to the point that they can stop other treatments like dialysis and plasma exchange. But now he fears others won’t have access to the drug he gets for free from the drug maker on a compassionate basis.
While it varies for each patient, the drug costs about $450,000 per patient per year, making it one of the most expensive treatments in the world, according to the Canadian Organization for Rare Disorders.
Health Canada approved Soliris for the treatment of aHUS in March, but earlier this week, the Canadian Agency for Drugs and Technologies in Health released a common drug review recommending the provinces and territories not fund the drug.
The review stated the benefits of Soliris were not adequately established as tests did not include a randomized control group. Common drug reviews aims to compare drugs with competitors, determine whether a drug provides value for money, and give advice on whether it is cost-effective for provincial plans to cover.
But Durhane Wong-Rieger, president of the Canadian Organization for Rare Disorders, said ultra-rare diseases, by their very nature, cannot be subjected to the same review framework as other types of drugs.
The disease affects an estimated five in every one million people, according to Dr. Christoph Licht.
As a lead investigator in the international clinical trial for Soliris, Licht said he felt it was unethical to only give the drug to some patients. Control groups are often not included in drug trials for such rare and aggressive diseases, he said.
“I don’t see a way to have done it differently or to ever do it differently,” he said.
While the recommendation to not fund Soliris for aHUS was disappointing, Wong-Rieger said it was not surprising.
In 2009, the review also recommended Soliris not be funded for the treatment of paroxysmal nocturnal hemoglobinuria, or PNH, another ultra-rare and life-threatening disorder. In 2011, B.C. Pharmacare eventually agreed to fund Soliris for the treatment of that disorder despite the negative review.
That gives hope to Tjepkema for others, like his cousin, who haven’t been able to get Soliris for free from the drug maker. Tjepkema said his cousin, who has the same genetic disorder, is in kidney failure and on dialysis. His cousin was previously misdiagnosed, a common problem with the incredibly rare syndrome, and underwent a kidney transplant.Because he isn’t on Soliris, his new kidney is also failing, but he can’t go on the transplant wait list again until he gets the drug.
With the relief Soliris has provided him, Tjepkema said he owes it to others in need to ensure they can get the drug, too.
“It’s so critical that we get the government to start funding Soliris,” he said. “However long it takes, we’ll keep at it.”
The B.C. Ministry of Health was unable to respond to a request for comment before going to press.
tfletcher@theprovince.com
twitter.com/thandifletcher
“It’s so critical that we get the government to start funding Soliris,” he said. “However long it takes, we’ll keep at it.”
The B.C. Ministry of Health was unable to respond to a request for comment before going to press.
tfletcher@theprovince.com
twitter.com/thandifletcher
© Copyright (c) The Province
Tuesday, May 14, 2013
A Long Time Coming
Brian is still on Soliris and he's doing better and better. Much fewer headaches, and the nausea is almost completely diminished. We went to the inaugural Atypical HUS conference in Toronto recently and learned a bunch of new information about the drug and about the disease. It was amazing. It also made me realize how well off Brian is compared to most patients. He's lucky to be given Soliris, and it was nice to have the reminder.
That's all I can say for now!
Tuesday, October 23, 2012
Break
I'm taking a break from this blog, if you hadn't noticed. So many times I would wander back over here with blog entries in my mind, but the idea of forming a post just tired me out. I've got so much on my plate! (And so little time!) I'll be back at it soon though...
Wednesday, April 18, 2012
Busted
I know, I know. It's been way too long since I put up a blog post. I didn't MEAN to take a break, but one was needed. So much going on, and so much complaining I wanted to do. And really, who wants to go to a blog to hear nothing but complaints? I needed an attitude adjustment.
So what's new in our world? Hmm... Let me try to figure out where I left off!
Brian's health. He's doing okay. Right now there are some adjustments being made to his medication(s) and I hope that means he'll feel much, MUCH better. Soon. He had his line pulled (yay!) so that means he only has one appointment every two weeks. It's freed up a lot of his time. But he's had a few more diagnosis (diagnoses?). One being "Pancreatitis". Look it up. It's not fun. But it just made the symptoms he'd been dealing with already worse. The good news is that his blood work has been very positive. All of his numbers (even his kidney function) are in the normal range. All of them! It's amazing. It doesn't mean he feels better, unfortunately, but it means he's as healthy as he's going to get! One of the new medications prescribed to him should help him with the constant nausea he battles, and that should make him more comfortable.
So what's new in our world? Hmm... Let me try to figure out where I left off!
Brian's health. He's doing okay. Right now there are some adjustments being made to his medication(s) and I hope that means he'll feel much, MUCH better. Soon. He had his line pulled (yay!) so that means he only has one appointment every two weeks. It's freed up a lot of his time. But he's had a few more diagnosis (diagnoses?). One being "Pancreatitis". Look it up. It's not fun. But it just made the symptoms he'd been dealing with already worse. The good news is that his blood work has been very positive. All of his numbers (even his kidney function) are in the normal range. All of them! It's amazing. It doesn't mean he feels better, unfortunately, but it means he's as healthy as he's going to get! One of the new medications prescribed to him should help him with the constant nausea he battles, and that should make him more comfortable.
Monday, January 30, 2012
Forgotten Fotos
Every once in awhile I like looking through recent pictures, and usually I have a few surprise "daily life" pictures that I've forgotten about. Here is one from the last few months:
Sunday, January 29, 2012
A Little of This, A Little of That
I'll start with the questions that are frequently asked!
"How is Brian?" He's doing pretty good these days. Still some uncomfortable side effects on a daily basis, but they're diminishing enough that he is living life much easier lately. The headaches and nausea are still frequent, but he's not REALLY sick like he has been. He even looks better and, I think I can safely say, I believe he's finally stopped losing weight! Eating has been hard for him with the constant nausea, but I think it's getting better.
"Did you finally get disability?" Yes. We are breathing a very huge sigh of relief. It is so nice to have a bit of income again. I quite literally jumped for joy when we got the call saying our first cheque was waiting for us. And then I went grocery shopping ;)
"Is Brian continuing to get Soliris?" That was more good news we recently got. The drug company that makes Soliris tried to get the government to fund it, but quickly realized they weren't going to make much progress. So they decided to approve Brian for the drug three months at a time again.
"How are Brian's 'numbers'?" His creatinine continues to decline (which is good), his platelet count is stable, his red blood cells finally normalized for the first time since all of this began. Generally, he is STABLE. Stable enough that we got some MORE good news recently. His main doctor decided to pull the line! In just over a week, he'll have day surgery and they will remove the line that goes to his heart. They don't believe he'll need the line for further PLEX treatments. That also means he'll have one less appointment at the hospital a week because he doesn't have tubes that need to get cleaned. And in an effort to keep his veins "clean", they have requested that he only get bloodwork done at the hospital when he gets his Soliris dose. That means he's going from about 3 appointments a week to 1 every second week! That is HUGE for us and frees up a lot more time.
"How are you and the kids?" We're good! I've been working and trying to come up with ways to support my family. I'm getting creative in my solutions to all the financial issues we've had. It's hard work. The kids are doing great and that means I'm doing SOMETHING right in giving them as stable a family life as I can manage!
Thanks for sticking with me through all of this!
Wednesday, December 21, 2011
No News is NOT Good News
We still have not heard about possibly being on disability. We DID have an appointment today regarding our (nonexistant) income that left us reeling. We're in trouble. BIG trouble.
Decisions have to be made. I'm trying to stop my panicking and just concentrate on getting through Christmas with my sanity intact.
The house is quiet tonight. Lately Brian goes to bed at about 8:00 - 9:00pm. He wakes up STILL tired the next morning. It doesn't make sense. His hemoglobin is at an all-time high. Tonight he put Koby to bed in our bed (with a steamer, in an attempt to have Koby sleep through the night without a hacking cough that's been pestering him for a week now!) Brian fell asleep. He woke up just a bit ago, started to do dishes, and declared it bedtime again.
I'm still trying to pull together a few Christmas events despite the illness going on at our house. Friends came over and decorated cookies today, and tomorrow some more friends are coming to do the same. We look forward to visiting. We look forward to a bit of laughter and joy. And I REALLY look forward to having my mind on something else for a few short hours.
Decisions have to be made. I'm trying to stop my panicking and just concentrate on getting through Christmas with my sanity intact.
The house is quiet tonight. Lately Brian goes to bed at about 8:00 - 9:00pm. He wakes up STILL tired the next morning. It doesn't make sense. His hemoglobin is at an all-time high. Tonight he put Koby to bed in our bed (with a steamer, in an attempt to have Koby sleep through the night without a hacking cough that's been pestering him for a week now!) Brian fell asleep. He woke up just a bit ago, started to do dishes, and declared it bedtime again.
I'm still trying to pull together a few Christmas events despite the illness going on at our house. Friends came over and decorated cookies today, and tomorrow some more friends are coming to do the same. We look forward to visiting. We look forward to a bit of laughter and joy. And I REALLY look forward to having my mind on something else for a few short hours.
Monday, December 12, 2011
I feel like I have very little to say so I don't post. Life continues to be a struggle for us in more ways than one. Brian has been really sick lately and very rarely has "good days", which makes it hard on all of us. I've been spending way too much time out of the house trying to bury myself in other things but it's burning me out much too quickly. I haven't had enough work, and I hope to change that in the new year. But right now I just need to figure out how to handle all of this!
Brian's numbers have been excellent, so he should be better. Right? But with the daily nausea, migraine-like headaches (also almost a daily thing), fatigue, weakness, dizziness, (etc, etc)... he is unable to function in a work environment. Probably not even a desk job, which is what we thought he might have to resort to. So for Christmas we are waiting on the news from the government as to whether or not he is "disabled", in their standards.
Apart from all of that, I have been learning to deal with my new role as breadwinner. I never saw myself being the one going out and "bringing home the bacon". Can I admit that it freaks me out? The only thing I know how to do, since being a stay at home mom, is clean and cook. I don't feel like I know anything else. I think I need some training somewhere, but I don't know what direction to go. And I still feel like I'm needed at home (if Brian has a day where he's too sick to care for kids, do I just put a movie on for them to babysit them and leave them? What if almost EVERY day ends up being a sick day - which has been the case lately?) I'm trying to keep things as normal as possible for my kids, and I'm failing miserably. Actually, I'm failing miserably at everything right now. With so many balls in the air, how can I not?
Well, this is sounding much more depressing than I thought it would! Which is why I've stayed away from posting! Sorry about that!
Brian's numbers have been excellent, so he should be better. Right? But with the daily nausea, migraine-like headaches (also almost a daily thing), fatigue, weakness, dizziness, (etc, etc)... he is unable to function in a work environment. Probably not even a desk job, which is what we thought he might have to resort to. So for Christmas we are waiting on the news from the government as to whether or not he is "disabled", in their standards.
Apart from all of that, I have been learning to deal with my new role as breadwinner. I never saw myself being the one going out and "bringing home the bacon". Can I admit that it freaks me out? The only thing I know how to do, since being a stay at home mom, is clean and cook. I don't feel like I know anything else. I think I need some training somewhere, but I don't know what direction to go. And I still feel like I'm needed at home (if Brian has a day where he's too sick to care for kids, do I just put a movie on for them to babysit them and leave them? What if almost EVERY day ends up being a sick day - which has been the case lately?) I'm trying to keep things as normal as possible for my kids, and I'm failing miserably. Actually, I'm failing miserably at everything right now. With so many balls in the air, how can I not?
Well, this is sounding much more depressing than I thought it would! Which is why I've stayed away from posting! Sorry about that!
Monday, September 19, 2011
Anxious
I could go on and on and on about all the things spinning around in my head, but do any of you really want to read it? And could my fingers even keep up?! Nope, probably not... Brian just came back from his PLEX/Soliris treatment. The doctors decided to do a PLEX even though his numbers were still stable. And they are thinking it might be his LAST PLEX!! Amazing. We'll see what happens when he goes back to the hospital in two weeks. We're hoping the numbers stay stable.
Well, I'm going to go sip a cup of tea and see if I can stop my brain from going into overload. I am physically exhausted from all that's going on in my head. Sitting on the couch and watching some TV sounds like a really good plan...
Tuesday, August 16, 2011
Yay Soliris!
Brian started Soliris last Monday. To date he's had two doses. We hold high hopes that this drug will be the answer, and so far it is working well! I have to remind my blog readers, though, that this drug is not a CURE, it is a lifelong treatment. His numbers yesterday were as follows (I'll put yesterdays numbers first, and the approximate number's he's had this year in brackets just to put it in perspective!)
Platelets: 211 (anywhere from 60-180 during non blitz weeks)
Creatinine: 126 (it's been as low as 130, and as high as 160. Lower is better. Normal, for Brian, is 120ish)
Hemoglobin: 113 (It's been stuck in the 90-100 range, normal is 135+)
So... I'm trying not to get my hopes up. But this is looking good! He actually has color in his cheeks for the first time in ages, and he is no longer dealing with constant nausea and fatigue. I've even seen him chase his kids around the playground without getting winded. I don't remember the last time I saw that! It's very encouraging.
Platelets: 211 (anywhere from 60-180 during non blitz weeks)
Creatinine: 126 (it's been as low as 130, and as high as 160. Lower is better. Normal, for Brian, is 120ish)
Hemoglobin: 113 (It's been stuck in the 90-100 range, normal is 135+)
So... I'm trying not to get my hopes up. But this is looking good! He actually has color in his cheeks for the first time in ages, and he is no longer dealing with constant nausea and fatigue. I've even seen him chase his kids around the playground without getting winded. I don't remember the last time I saw that! It's very encouraging.
Tuesday, July 26, 2011
Big News
I've been looking into clinical trials for Soliris worldwide, and hadn't come up with anything quite yet. I wanted to be armed with information and then present it to Brian's team of doctors and start to "fight" for him to get the drug. At least to try it out!
So imagine my surprise when Brian phoned me from the hospital yesterday to tell me that his favorite Hemotologist told him he was going to start on Soliris in the very near future! With the complications Brian's been having at PLEX treatments (allergic reactions to human plasma, lack of a good response to synthetic plasma, numbers being very low despite blitz treatments, etc) the doctors have been running out of options. And Brian, I would think, would be a good candidate for a "trial run" of Soliris based on the fact that his kidneys are still functioning at 80%. In terms of aHUS, 80% kidney function is huge! Most often, the disease isn't caught until kidney function is almost at zero.
They hope to start treatment at the end of August. In the meantime they have to get a few things sorted out. For starters, they have to get IN WRITING that the drug company has agreed to put Brian on Soliris. So far it's only been a verbal agreement. Today Brian is getting the meningitis vaccination that is required for treatment. Soliris lowers resistance to infection, especially meningitis.
Brian has only been approved for three months of Soliris, being that it is such an expensive drug. When the three months are up, they expect the disease to return. Soliris is not a cure, it is a treatment option. But while he is on Soliris, here's what he can expect: Normal platelet count, without PLEX treatments. Lowered blood pressure, without medication. A return to normal hemoglobin, and creatinine levels. Woohoo!
Life will look VERY different for those three months. And at the end of those three months, his doctors can always re-apply, especially if Brian does really well! A few other things are going to have to change while Brian is being treated. Being that he is prone to infections, we will have to stay VERY far away from sickness
So imagine my surprise when Brian phoned me from the hospital yesterday to tell me that his favorite Hemotologist told him he was going to start on Soliris in the very near future! With the complications Brian's been having at PLEX treatments (allergic reactions to human plasma, lack of a good response to synthetic plasma, numbers being very low despite blitz treatments, etc) the doctors have been running out of options. And Brian, I would think, would be a good candidate for a "trial run" of Soliris based on the fact that his kidneys are still functioning at 80%. In terms of aHUS, 80% kidney function is huge! Most often, the disease isn't caught until kidney function is almost at zero.
They hope to start treatment at the end of August. In the meantime they have to get a few things sorted out. For starters, they have to get IN WRITING that the drug company has agreed to put Brian on Soliris. So far it's only been a verbal agreement. Today Brian is getting the meningitis vaccination that is required for treatment. Soliris lowers resistance to infection, especially meningitis.
Brian has only been approved for three months of Soliris, being that it is such an expensive drug. When the three months are up, they expect the disease to return. Soliris is not a cure, it is a treatment option. But while he is on Soliris, here's what he can expect: Normal platelet count, without PLEX treatments. Lowered blood pressure, without medication. A return to normal hemoglobin, and creatinine levels. Woohoo!
Life will look VERY different for those three months. And at the end of those three months, his doctors can always re-apply, especially if Brian does really well! A few other things are going to have to change while Brian is being treated. Being that he is prone to infections, we will have to stay VERY far away from sickness
Saturday, July 23, 2011
A Year Ago
A year ago today I was walking around a really fun fair with my two kids, my good friend and her two kids. We'd just entered the fair grounds and were looking around at all the displays near the entrance.
A year ago today I got a phone call on my cell phone. Brian was in the hospital in Langley because he was having what I thought would be short lived issues with his blood pressure and kidneys. On the phone he told me that they were going to put him in an ambulance and rush him - "code blue" - to VGH where he would require emergency medical attention. He told me that they'd just discovered that his platelet count was dangerously low.
A year ago today I was trying to figure out how not to crumple to my knees in a public park and start sobbing in front of my children. I was trying to keep my voice calm and I was wondering if keeping their Daddy's hospital stay a secret. I was thankful when my friend almost read my mind and distracted my kids by pointing out the newly hatched chicks nearby.
A year ago today I didn't know if I'd ever see my husband again. I knew how serious it was, and I could hear my doctor's words echoing in my head. "If you don't go to the hospital right now, you'll likely die within 24 hours" I wandered around the fair grounds for awhile just letting it sink in, not knowing if I was becoming a widow while Brian was riding in an ambulance, alone. I didn't have any family available to me that day, so I had no where to drop my kids off. I had a long drive into VGH and I didn't know what I'd find when I got there.
A year ago today I knew nothing about platelet counts, or plasma treatments, or the link between blood pressure and kidney function. I'd never heard of hemolytic uremic syndrome and I didn't know anything about rare blood diseases.
A year ago today my entire world changed...
A year ago today I got a phone call on my cell phone. Brian was in the hospital in Langley because he was having what I thought would be short lived issues with his blood pressure and kidneys. On the phone he told me that they were going to put him in an ambulance and rush him - "code blue" - to VGH where he would require emergency medical attention. He told me that they'd just discovered that his platelet count was dangerously low.
A year ago today I was trying to figure out how not to crumple to my knees in a public park and start sobbing in front of my children. I was trying to keep my voice calm and I was wondering if keeping their Daddy's hospital stay a secret. I was thankful when my friend almost read my mind and distracted my kids by pointing out the newly hatched chicks nearby.
A year ago today I didn't know if I'd ever see my husband again. I knew how serious it was, and I could hear my doctor's words echoing in my head. "If you don't go to the hospital right now, you'll likely die within 24 hours" I wandered around the fair grounds for awhile just letting it sink in, not knowing if I was becoming a widow while Brian was riding in an ambulance, alone. I didn't have any family available to me that day, so I had no where to drop my kids off. I had a long drive into VGH and I didn't know what I'd find when I got there.
A year ago today I knew nothing about platelet counts, or plasma treatments, or the link between blood pressure and kidney function. I'd never heard of hemolytic uremic syndrome and I didn't know anything about rare blood diseases.
A year ago today my entire world changed...
Wednesday, July 20, 2011
An Update
On Monday I went with Brian to the hospital to get some answers to the questions that plague my mind. In the last few weeks I've done more and more research (Have I mentioned that I'm a research buff?) into his condition and I'm not finding much in the way of help. But I HAVE found out a few interesting things (at the hospital, and before I even went)
- Brian's diagnosis is now an even longer title. Familial atypical hemolytic uremic syndrome. If you look it up, you won't find it. The most important part is the "atypical hemolytic uremic syndrome" part. The rest of it just signifies what TYPE of atypical HUS he has. Familial is in the title because his disease is hereditary, and it can either be a recessive or a dominant gene passed down. Recessive ALWAYS shows up in infancy or childhood, which naturally means that Brian's case is dominant.
- There has been a treatment plan introduced for such a rare case of HUS. It's been tried on a few patients with outstanding results. It's a drug called "Soliris". You may have heard the name before. Apparently it recently made the Forbes list of the most expensive drugs in the world. And, you guessed it, Soliris topped the list. It can only treat two different kinds of blood disorders so, naturally, it is not a widely used drug. That means the drug company can charge whatever they want for it. And they charge $20,000 per unit. If Brian were to receive this treatment, he would start off having a few units of Soliris a day until the doctors start to see results. In one week, Brian's treatment could easily top a million! And, um... yeah. We don't have that kind of money.
- Soliris was approved for use in the US in 2009. That means there could possibly be clinical trials elsewhere in the world. Maybe even Canada. We have to find out where clinical trials could be taking place and see if we can get Brian on "the list".
- The doctor I spoke with at the hospital said that, from his standpoint, the disease is "managed". He didn't say it was managed WELL, he just said he was satisfied with where numbers are. This was explained to me as a response to my concerned questions about Brian's well-being here at home. There has been a noticeable decline in his health. Last week was hard. He spent so much time either being nauseated to the point that it affected his daily life, or throwing up. He is fatigued much of the time and I am so glad he doesn't have to go to work every morning right now. He is losing weight, pale, and generally feels unwell the majority of the time. I tried explaining that to his doctor, but he said the only concern the hematology team has with Brians numbers is his consistently high blood pressure. He's on a handful of drugs to try to bring his blood pressure down, but they don't seem to be doing their job. Next on my "research agenda": finding some way to lower his blood pressure!!
Well, this is just a snippet of information I've received recently and I'm overwhelmed just typing it out! More to come as I continue searching out answers!
- Brian's diagnosis is now an even longer title. Familial atypical hemolytic uremic syndrome. If you look it up, you won't find it. The most important part is the "atypical hemolytic uremic syndrome" part. The rest of it just signifies what TYPE of atypical HUS he has. Familial is in the title because his disease is hereditary, and it can either be a recessive or a dominant gene passed down. Recessive ALWAYS shows up in infancy or childhood, which naturally means that Brian's case is dominant.
- There has been a treatment plan introduced for such a rare case of HUS. It's been tried on a few patients with outstanding results. It's a drug called "Soliris". You may have heard the name before. Apparently it recently made the Forbes list of the most expensive drugs in the world. And, you guessed it, Soliris topped the list. It can only treat two different kinds of blood disorders so, naturally, it is not a widely used drug. That means the drug company can charge whatever they want for it. And they charge $20,000 per unit. If Brian were to receive this treatment, he would start off having a few units of Soliris a day until the doctors start to see results. In one week, Brian's treatment could easily top a million! And, um... yeah. We don't have that kind of money.
- Soliris was approved for use in the US in 2009. That means there could possibly be clinical trials elsewhere in the world. Maybe even Canada. We have to find out where clinical trials could be taking place and see if we can get Brian on "the list".
- The doctor I spoke with at the hospital said that, from his standpoint, the disease is "managed". He didn't say it was managed WELL, he just said he was satisfied with where numbers are. This was explained to me as a response to my concerned questions about Brian's well-being here at home. There has been a noticeable decline in his health. Last week was hard. He spent so much time either being nauseated to the point that it affected his daily life, or throwing up. He is fatigued much of the time and I am so glad he doesn't have to go to work every morning right now. He is losing weight, pale, and generally feels unwell the majority of the time. I tried explaining that to his doctor, but he said the only concern the hematology team has with Brians numbers is his consistently high blood pressure. He's on a handful of drugs to try to bring his blood pressure down, but they don't seem to be doing their job. Next on my "research agenda": finding some way to lower his blood pressure!!
Well, this is just a snippet of information I've received recently and I'm overwhelmed just typing it out! More to come as I continue searching out answers!
Sunday, June 19, 2011
End of the Year
The end of the school year is sneaking up on me. I was forewarned, when Mercy entered Kindergarten, that the end of the year tends to be a very busy time. The term "busy" is an understatement! There have been assemblies to attend, parties, field trips, sports day and countless little girls and boys birthday parties to attend.
Don't get me wrong, I am not complaining. Far from it! I enjoy these little activities that remind me that life goes on. Sometimes it feels as if life drums along to a different beat for my family. Depending on whether or not Brian is having a "sick week". The possibility of a blitz week always looms over my head like a dark cloud. So when we have a week filled with normal activities, I celebrate!
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