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.
Tuesday, August 16, 2011
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!
Tuesday, June 7, 2011
*yawn*
I yawn because I'm tired. I'm tired because this learning curve is getting the best of me. Learning curve? Yes. I'm learning all kinds of things right now. What exactly Brian's disease means, and what forms of treatment are there in other parts of the world, what kinds of natural remedies treat symptoms he has and what are the best DIRECT forms of getting the nutrition he needs, how to cook these "different" foods (and how to afford them on said limited budget!), how to be the "man of the house" when Brian is sick (for example, I tried to learn how to run our rickety old lawn mower last week. Tried and failed, I might add). It's frustrating and overwhelming some days. Days like today I'm just completely tired out.
So the blog has kinda been one of the balls that has been dropped, obviously. A LOT has been dropped. I'm no longer a good sister, a good friend, or a good daughter. Not that I ever claimed to be perfect in the first place. I've forgotten birthdays. I even (almost) forgot Koby's Well, I was a day late. ON his birthday it took a school friend to remind me that it wasn't the NEXT day, it was TODAY! Well, I threw something together last minute and I don't think he noticed. Poor little neglected man...
Well. There you have it. A post!
So the blog has kinda been one of the balls that has been dropped, obviously. A LOT has been dropped. I'm no longer a good sister, a good friend, or a good daughter. Not that I ever claimed to be perfect in the first place. I've forgotten birthdays. I even (almost) forgot Koby's Well, I was a day late. ON his birthday it took a school friend to remind me that it wasn't the NEXT day, it was TODAY! Well, I threw something together last minute and I don't think he noticed. Poor little neglected man...
Well. There you have it. A post!
Wednesday, May 4, 2011
Whoa
Okay. It's been awhile. Has it really been almost a month since I posted? Honestly, I haven't run out of things to say or share. I have just been busy doing LIFE lately. The new-new-NEW normal. It's an adjustment.
Brian ended up struggling again with his platelet count. Now it's stable. Not a HIGH stable, but it's stable. And that's good enough for us, I suppose.
On Monday we got the news that the genetic testing came back. Nothing prepared us for the news that it IS genetic and my kids have a 50% chance of having the same genetic mutation. What that means exactly? We don't quite know. But 50% just sounds frightening. I've had questions swimming around my head since the news, and according to Brian we'll be having a conference call with some specialists who can answer all our questions. It would've been nice to have questions answered the very next day, but I am getting used to playing the medical waiting game. Everything takes time.
For those of you that may like medical mumbo jumbo, and are research buffs and/or curious... The gene affected by the abnormality is in the C3 gene sequencing: the Exon 14 gene. Most sufferers of HUS/TTP have issues with their Adams 13 gene, but Brians case is (of course) different. Of the 6 categories under Atypical HUS, Brian's genetic mutation doesn't fit in with any of them. So the very rare disease he has is even more rare.
If that went over your head, I don't blame you!
Brian ended up struggling again with his platelet count. Now it's stable. Not a HIGH stable, but it's stable. And that's good enough for us, I suppose.
On Monday we got the news that the genetic testing came back. Nothing prepared us for the news that it IS genetic and my kids have a 50% chance of having the same genetic mutation. What that means exactly? We don't quite know. But 50% just sounds frightening. I've had questions swimming around my head since the news, and according to Brian we'll be having a conference call with some specialists who can answer all our questions. It would've been nice to have questions answered the very next day, but I am getting used to playing the medical waiting game. Everything takes time.
For those of you that may like medical mumbo jumbo, and are research buffs and/or curious... The gene affected by the abnormality is in the C3 gene sequencing: the Exon 14 gene. Most sufferers of HUS/TTP have issues with their Adams 13 gene, but Brians case is (of course) different. Of the 6 categories under Atypical HUS, Brian's genetic mutation doesn't fit in with any of them. So the very rare disease he has is even more rare.
If that went over your head, I don't blame you!
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