Di Has Stories…

(and they’re all true)

Other Things I Learned At The HAE Conference October 20, 2008

Filed under: being a sickie — Diana @ 9:38 pm

(1)  Because I am Type III, there is only a 30% chance that the new treatment, Cinryze, will work for me.

(2)  I am a “mutant”, meaning that I have no family history of HAE.  Twenty-five percent of people with HAE are also mutants.  I am not as alone as I thought I was.

(3)  A lot of people die of this disease.  A. Lot.  There have been three deaths this year of our patient community (estimates are that there are about 7,500 people in the US with HAE – the Association knows of more than 5,000 of them).   That’s a 1 in 2,500 annual rate of mortality.  One of the researchers showed us a few family trees and almost all of the people had died of HAE.  Granted, there was no treatment, even rudimentary, before about the 1970s.  But there are still a lot of people dying.

(4)  That’s not how I want to go.

(5)   I am both in better shape than I thought, and in worse shape than I thought.  While other folks seem to have more “severe” attacks (face, throat) more often than I do, I have more overall attacks than normal.

(6)  All attacks are supposed to be treated, but I’ve only ever treated for throat.  Not sure I’m going to change that – unlike some of the folks, my attacks tend to stay where they start.  Others have spreading attacks – it might start in the eyes, but will move down the face into the throat.

(7)  The quality of life for people with HAE, statistically, is less than that of people with diseases such as Chron’s Disease.  I’d once heard that our quality of life is comparable to that of a cancer patient.

(8)  Half of all HAE patients have lost a job because of HAE.

After having this disease for 13 years, and been diagnosed for the last four, I was surprised at my surprise at some of these facts.  For fuck sake, I’ve been hospitalized eight times.  I’m on disability.  I was on a therapy that could have killed me to save me from a disease that could kill me.

It has never been so clear to me that what I have is so….severe.  So….life threatening.  So….so….intense.  This is the first time that I’d ever actually considered tahat this might kill me.  That I really might pass this on to my kids, and that it might kill them.  Or I might not live to see them get old.

Maybe I’ve been thinking about this too much.

 

Disappointment: The 2008 HAEA Conference

Filed under: being a sickie,bitching,shit i believe in — Diana @ 9:17 pm

This weekend I attended the annual conference of the US Hereditary Angioedema Association conference.  It was the first conference I’d attended – I’d actually registered for the last one in St. Louis, but that was right when we closed on the house, and I didn’t think that I could move house and drive to St. Louis all in a couple of days.

So I was really excited to go this year.  As a mutant (someone with no family history of HAE), I’d never actually met someone with HAE, and I was so excited to do so.  I’d get to meet other people that swell up like I do, and ask them questions about what’s normal and not normal, and find out how many “mutants” there are, and, and and….I was just about bursting all last week.

Because money is so tight in our house right now, I’d gone back and forth as to whether to go.  But after last week’s news that the drug we’ve all been waiting for is approved, Jeff and I decided that the money would come from somewhere, and that I was going to go.  It was too important for me to get the information to not go.

I got to Chicago at about 3:00 p.m. on Friday, and checked into my hotel with one of my roommates, Patty.  She is also a mutant, and 57 years old.  We talked for a few hours before it was time for the cocktail hour that kicked the conference off, and then went downstairs for free booze and appetizers.  We met a mother and 19 year old daughter – the daughter is also a mutant, and an older couple where the woman has HAE.  We also got to talk to one of the scientists from the drug companies where I learned a little something about my own treatment options.

(Background:  Hereditary Angioedema is caused by the absence or malfunction of the blood protein C1 esterace inhibitor.  There are several types of HAE:  Type I’s blood tests show an absence of the C1 protein.  Type II’s have the protein, but it doesn’t function properly.  Type IIIs have “normal” blood results, but show all of the symptoms and may react positively to FFP (fresh frozen plasma) treatment.  I have Type III.  The scientist that I talked to said that there is a 30% chance that the newly approved C1 treatment will work for me.)

The next morning we all met for a very nice breakfast (you know, more than doughnuts!!), and settled in for the meetings  First there was a reenactment of the presentation before the FDA Blood Products Division, which was very effective – I can now see where there was an almost unheard of unanimous vote to approve the C1 product.  This was followed by an address by Dr. Marcel Levi, who walked us through the use, effectiveness, and safety of C1 in Europe (it’s been legal there for 30 years) and the studies that back up that use, safety and effectiveness.

After that, the drug companies started talking.  First was LevPharma, whose product, Cinryze, is the newly approved product.  They talked about the clinical studies (an oddity of the US drug approval process – even drugs in use for 30 years elsewhere have to go through all the studies and back up that a brand new drug does), the safety, efficacy, and presumed time to market.

During the question and answer session is when i started getting nervous.  It turns out that LevPharma is only five years old, and was started for one purpose – to bring Cinryze to the market.  Also, LevPharma has been bought out by ViroPharma (after which shares in ViroPharma fell 20% according to SmallCapInvestors.Com)  for $443 million dollars.  ViroPharma itself only has one drug on the market.

The folks from Lev/Viro were answering questions about insurance coverage and how to get the drug. They have set up a call center – Cinryze Solutions – to help people deal with their insurance companies, and were trying to get all of us to sign up for the program, which included all sorts of personal information that I was not terribly interested in giving out.  The women running the table were running people down and using their names to guilt them into signing up for the program. I’m pretty sure it was less about “helping” the patient and more about knowing who the captive market is.  The entire way it was approached made me uncomfortable.  And it’s not a service that I need – I’m a professional insurance agent, and have worked with appeals for years.  If I need additional documentation, I will turn to my doctor, not to a drug company.

After LevPharma was done, CSL Behring started talking.  They are the ones that actually have the drug approved in Europe.  They have been in business for more than 100 years, and only work with orphan diseases and biologics.  Their presentation spewed the same research statistics and findings, which isn’t surprising, as there is only ONE way to treat this disease that is effective (replacing the C1 that the patient doesn’t have).

This is where I started looking at the schedule.  Out of an eight hour day of meetings, five hours were taken up by pharmaceutical companies, an hour and a half was for us to “interact” with the pharmaceutical companies, and only an hour and a half was an open forum for patients to talk to doctors and pharmaceutical company scientists.

This is not what I was going for.  I wanted interaction with other patients – there was no time for that.  I wanted some interaction with doctors – that only happened with the pharmaceutical company people.  The HAE people were totally kissing the asses of the pharmaceutical companies.  They were branding their own website over any other information source, but trying to say that they were “drug-neutral.”  (It’s true – it seemed to be that they’d be ok with any of the drug companies.)  I found out that not only were the pharmaceutical companies underwriting the entire conference, but they were also providing financial assistance to the patients.

They were trying to bribe us into using their product….when their product is the only thing that can save our lives.

I felt dirty.  I thought that this was a time for patients to get together, build a network, and learn from each other.  Instead, it was a three day opportunity – that I couldn’t really afford to go to – to be marketed to by Big Pharma.  All I learned was that I was a big source of money to these people*, and they wanted to make sure that I was buying into it.

*(It’s estimated that every dose of the C1 will cost $1,000 – $1,500.  Patients must take one to two doses every three to five days.  This means that I will give them $73,000 – $243,000 per year, every year, for the rest of my life.  At least until I hit my insurance lifetime max, which I will in 12 – 41 years.)

I’m pissed.  I’m pissed that pharmaceutical companies behave this way – I knew about these sorts of things, but had never seen it in action.  And I’m also irritated that so many of my co-patients (and that’s how we are referred to – as patients) lapped this shit up and expressed such gratitude for being ripped off and marketed to.

I’d had enough.  I went home.  I now have to think about whether to continue my relationship with an organization that is really just facilitating marketing to Big Pharma.  I am once again disappointed in an organization that I thought was only looking out for me and others like me.  As usual, we are just consumers.

 

Damn Right She’ll Rise Again October 10, 2008

Filed under: being a sickie — Diana @ 4:42 pm

US clears Lev Pharma drug for rare swelling disease

Cinryze(TM) Receives FDA Approval for Prophylaxis Against Hereditary Angioedema Attacks

 

Oh, noes September 13, 2008

Filed under: being a fattie,being a sickie,bitching — Diana @ 2:46 pm

A few months ago, having had an ultrasound for suspected gallbladder issues, it was found that I have Non-Alcoholic Fatty Liver Disease, which is a result of unchecked metabolic syndrome.  (I wrote more about this in my sickie blog.)  The treatment for this is pretty easy – Metformin, baby, Metformin.

Let me just take a minute here to tell you how very much I heart the Metformin.  So far, it has made my skin clear up, my depression abate a bit (during a time in which it should have been flaring pretty good), my hair grow, my blood pressure go down (140/90 to 104/80), my cholesterol go down (230 to 177), and I’m starting to melt off some of the fat that is stored when your body doesn’t know what to do with sugar.

And that’s the deal with Metformin.  Basically, left to its own devices, my body doesn’t know what to do with sugar, so it wants me to eat lots and lots of it, and then it turns it all into fat (have I mentioned lately that I gained 100 pounds in about five years with no change in diet?)  Metformin makes my body figure out what to do with sugar in a more appropriate way, and tells my body that to have too much of it is poison, and will immediately expel said excess in ways that are unpleasant to me, the experiencer, and you, the passer-by.  Just trust me on this one.

For the most part, that’s ok.  Now that I don’t crave sugar every moment of the day, I’m not also eating every moment of the day, and I’ve been able to get rid of a lot of my food obsessions, which is only good.  However, the amount of sugar that I can tolerate on an average day is pretty low.  Very, very low.

For the last few weeks, since my dose got increased again, I’ve been able to have a daily Coke – sometimes two – and maybe one other sweet thing if it was around and I wanted it.  I know that I should give up the Coke, cause it’s loaded with high-fructose corn syrup, which is really Death Made By Corn.  But, I say to myself, I’ve gotten rid of it in the rest of my diet, and lots of other horrible things, so one Coke isn’t going to hurt me – all things in moderation, right? (Let’s not talk about the awful fucking campaign the makes of HFCS are putting on….read about it here.)

And, frankly, I don’t fucking want to.  Part of my journey on body acceptance and Health At Every Size is learning to not deprive myself.  I like to eat, damn it, and although I generally make good choices, I do make some less than great choices, and that’s ok.  It’s all about the big picture, and not buying into my old habits of disordered eating.  So the Coke stays.

The last few days, I haven’t been feeling s’good.  Not anything terribly definable, just a general malaise and some fatigue and not quite feeling like myself.  I especially noticed it at the Tribute to the Classy Broad on Saturday  last, and figured it was just because I wasn’t drunk.  But it’s been going on all week, and my excuses have run out (I’m tired, I’m hungry, it’s raining,  fucking Republicans….)

Until this morning.  And by this morning, I mean when I finally got around to eating “breakfast” (summer sausage, crackers and cheese) at about 2:00 p.m.)  I had my tasty, tasty meal along with my daily Coke….

And an hour later felt shaky, like I needed a nap, and a bit depressed.  Oh, and I’m not going to talk about what’s going on in the bowel region, because I have too, too much love for you, dear reader.

Et tu, Coke?

Yes, it seems that my friend, my love, my partner in life since I was 15, has turned against me.

Bastardo.

So, I guess I am going to give up the Coke.  Not because of the million reasons that it’s not good for me, but because my body has decided for me that the bubbly combination of HCFS and awesome is no longer good for me.

Damn it, liver – you better be worth this.

 

Blog Dump – Long Past Due July 28, 2008

In Which We See Cute Things and Receive Spiritual Guidance from I Can Has Cheezburger

In Which We See Awesome Things That I Or Someone I Love Need To Purchase Post Haste

Pickle Jar With Fork from Ooh-Shiny.Net  (Sarah, I’m looking at you)

The Agreeable Sheep from Ooh-Shiny.Net  (Heather?)

Puppet Hoodie from Ooh-Shiny.Net (This one is for me, but it’s not in my size….do these things stretch in the wash?)

The Urban Homestead: Your Guide to Self-sufficient Living in the Heart of the City on Boing Boing.  (Sarah, you again)

Roku: $100 gadget for watching Netflix movies on your TV (Freaking EVERYONE!  This looks awesome!!)

Portable cardboard toilet on BoingBoing.  Yes, the Shit Box.  My beloved has decided that, because we only have one bathroom, he needs a bucket with a toilet seat in the garage in case we both have the flu or something at the same time.  Last time we were at Home Depot he bought the seat for it.  For the record, I have been lobbying for the installation of a random basement toilet, but so far he’s not going for it.  As a resonable second, I think that we should at least get a shit box.  If I’m going to be emptying my bowels in the garage, I want a proper receptacle.  Those of you that Twitter will find that this is my new icon.  This is why I should never, ever show Christy funny stuff while I am drunk.  When she says, “that would make a great icon”, I’m on it like white on rice.  However, even when I sobered up I still thought it was pretty awesome.

In Which We Have A Commentary On My Job, Which I Am Not Currently Working

From GraphJam:

In Which The Holiest Part of Christianity is Blasphemed for Our Personal Pleasure

From Loltheist:

In Which We Listen In On Conversations:

So I’m Sorry I Did That, Amber  (from Overheard Everywhere)

English teacher: Calling someone a douche is not a constructive comment, even if it’s true.
Flora High School
Columbia, South Carolina

The Pen Had It Comin’! (from Overheard in Minneapolis)

Metro Transit phone employee (on speakerphone): Take the sixteen bus towards…
18 year old girl trying to get directions: Wait, wait you’re talking too fast and my pen died.
Metro Transit phone employee: You killed your pen? You heartless BITCH.

Univeristy/ sixteen bus line
Overheard by Death to ink.

Relax, I’m Just Horngry (from Overheard in the Office)

Financial specialist, in front of his pregnant wife/coworker: God, I would nail someone if they had some bacon right now!
Wife/coworker: What the hell is wrong with you?
Dallas, Texas
Overheard by: God Help Me

Then How Come It Got Me More Time on My SATs? (from Overheard in the Office)

Boss on phone: That is not clinical! Being an asshole is not a clinical condition.
Ginko Industrial Park
Warminster, Pennsylvania

Is It Still a Place Where You Wash Your Hands? (from Overheard in the Office)

Kitchen grunt: You ever poop and suddenly find the world a better place?
Main Street
Greenwood, Indiana

Overheard by: RDC

In Which We Are Surprised.  Wait, No We Aren’t

U.S. still flunks healthcare test, group says

Absolutely fucking OUTRAGEOUS! by Paulius

Fox’s “Anti-Aging Fix” for McCain by Technology Expert

Larry Craig, Daniel Vitter Co-Sponsors of “Marriage Protection Amendment” by Technology Expert

Vampire babies on the attack! by Jen

In Which I Have A Whole Other List of Books To Check Out

List of every book read by Art Garfunkel since 1968.  I’d like to point out that Art started this list the very month and year that my beloved was born.  And that while Art is referenced in this article as a “voracious” reader, he averages about two books per month.  This year, I am averaging two per year.  And I really need to get going on cataloging them.

Speaking of books, some of my favorite books when I was a kid (and even now, I’ll admit it) were the Anne of Green Gables series by L.M. Montgomery. 100 Candles Anne of Green Gables grows old and gets her due. When my mom introduced me to Anne, the books were actually out of print in the states, and I read the same copies that she had read as a child…the hardbound editions with all of the dust covers missing.  For years I thought I was named after Diana Barry, Anne’s best friend, my dad recently told me he picked the name from the Paul Anka song.  When my oldest sister was born, when I was 11, I was allowed to pick her middle name.  I choose Anne.  Although my sister didn’t grow up to be a sassy redhead (wait, yeah she did), she can at least say – for sure! – that she was named after Anne of Green Gables.

In Which I Find New Websites to Waste My Time With

Things I Should Throw Out: Clippings From The Eighties

In Which I’m Just….Amazed.  And A Bit Disturbed

Man electrocutes pickle to demonstrate power of Christianity So, because I don’t love Jebus, I will not glow in the dark and I can’t make any difference in the world?  What?

In Which I’m Just Amazed

The Pregnant Man.  I cried recently when I learned that Thomas Beatie and his wife had safely delivered their little girl.  While I might just be a little sensitive about the whole having-babies thing right now, it makes me so happy when people who desperately want children are able to have them, in whatever way it happens.  Birth, adoption, whatever – these people wanted an expression of their love for each other in the form of a child, and they were able to do it in an unconventional way.  It’s beautiful.  It would have been beautiful if they’d been able to do it in a “normal” way, too, but then the rest of us wouldn’t have been able to share in the joyous birth of a child who was so, so wanted.

In Which There Is a Gratuitous Post About Bacon

In Which Women Smarter Than I Talk About Life and Other Big Things

Is HAES Unhealthy? by Well-Rounded Mama

Yeah, whatever, Kate Moss by peggynature

The cult of dieting by attrice

Possibly, The Most Tasteless CUSS Post to Date by Suzanne

ChronicBit: Lab tests demystified Via Lab Tests Online, where you can search for just about any test and learn what it means. Excellent tool for us Babes… thanks for the top, ChronicPal Shannon!

Family Pride by Happy Villain

Eye-Opener by Happy Villain

 

Blogging Against Disablism May 2, 2008

Filed under: being a sickie — Diana @ 2:39 pm

Yesterday, May 1, was Blooging Against Disablism day, and in true ME fashion, I didn’t even really know about it. I’m now catching up on all of the wonderful things that were written, and wanted to share some of them with you:

The Gorilla In Your House by This Is My Blog (I think that this might be the best How It Is To Be Disabled Post since The Spoon Theory came out on But You Don’t Look Sick)

I’m still not comfortable using the word “disabled” to refer to myself.  A good portion of the time (currently), I am able to function, and when I am not, HAE is a fairly “invisible” illness.  When my symptoms are apparent, however, most people jump to the conclusion that I am a victim of domestic violence, and not a sickie.  (Cause it’s waaay better to think the woman with the fat lip just got thrown down the stairs by the fantastic man next to her than to think that there might – just might – be an alternative explanation!)

Anywho, whether I refer to myself as “disabled” or a “sickie” or just “me”, I often worry that I am an unfair burden on my sweetie.  He’s not a big fan of surprises, and I am one big bundle of surprises, and often they are not good ones.  I know that my illness stresses him out.  In fact, it probably bothers him more than it does me sometimes…at least I have the knowledge that I have The Best Husband Ever who will take care of me, and he has the burden of being that caretaker.  Although, if you ask him (as I have) if he considers it a burden, he’ll tell you no.  It’s just part of who I am.

Did I mention The  Best Husband Ever?

So, posts like this one by the partner of a disabled person make me feel a whole lot better.  Thanks Willendorf5761!  Oh, and I just found this one from Kate…uncensored.  I am all a-glow from reading these wonderful people and how much they care about their partners.

You know it’s good when I snort at work.  (Not that I blog at work, of course.  That’s unprofessional.  And I am, indeed, the very model of professionalism.)  Sad, Pathetic Charity Case by Unreliable Witness.

Who Are These Freaks by Yet Another Never Updated Blog made me giggle.  I have been known to tell people “Don’t sit on that – your ass will swell up!” because I forget that not everyone is going to puff at the slightest pressure.

 

Long Overdue Links April 25, 2008

Filed under: being a sickie,blog dump,fun stuff — Diana @ 12:56 pm
Tags:

Bear hedgehog and coat of arms on Flickr – Photo Sharing!
This is at Christ Church in Dublin – I can’t believe that Jeff and I missed it!

I have so been waiting to use this – from ICanHasCheezburger?

Fat Girl Rants and Links:

Why is the US in a recession?  Because of all the fat people, that’s why! by Cthullhu

Ever had one of those days? by Body Impolitic

A Few Points That Bear Repeating by Fat Lot of Good

Sickie Links:

Review of StickyJ from But You Don’t Look Sick

Random Stuff from Random Bloggers I Like

Bringing up baby by Everywhere Man

The Blasphemy of Creationism by Greta Christina

Horny Teenage Killers On A Rampage To Hell Day! from Girls Are Pretty

From Quotes of The Day

Bill Watterson – “Careful. We don’t want to learn from this.”

Stephen Jay Gould – “In science, ‘fact’ can only mean ‘confirmed to such a degree that it would be perverse to withhold provisional assent.’ I suppose that apples might start to rise tomorrow, but the possibility does not merit equal time in physics classrooms.”

E. B. White -“I get up every morning determined to both change the world and have one hell of a good time. Sometimes this makes planning my day difficult.”

For Good Measure, some Blasphemy from LolTheist:

Why Don’t I Every Overhear this Awesome Stuff?

Father Mike Continues His Downward Spiral (from Overheard Everywhere)

Satisfied customer: They had bacon I would drop-kick a nun for.
Maggie’s
Toronto, Ontario
Canadia

Tipping The Scales Back In Humanity’s Favor (from Overheard in Minneapolis)

College Age Guy to Suit: Hey, Sir, do you work here?
Suit: No.
College Age Guy: Oh. You look nice.
Random Third Guy: You do.

Walgreens, Nicollet Mall
Overheard by Inflatigirl (ok, so I DID overhear this one!)

This Should Be In A Hallmark Card by OIM

Guy yelling into cell phone: You know there ain’t no one else. All them other bitches, I don’t talk to them any more. I don’t want no other bitches, just you. I’m with you all the motherfuckin’ time. I ain’t got time to be with no other bitches. Why we gotta fight? Why can’t we just be cool? Come on, baby.
Light rail train all the way from the 46th Street station to the Warehouse District.
Overheard by someone who’s glad to see that romance isn’t dead.

Nature Will Take Care Of It For You by OIM

Pregnant woman talking to friend and pointing to small initial charms in a jewelry store: Wouldn’t this look cute on my clit ring? (later…) My doctor wanted me to take the ring out ’cause I’m pregnant and all but hell naw, I done spent $150 on this sh*t, I ain’t bout to take it out now!
Mall of America
Overheard by Amused Employee.

Epistemology at the Monsanto Broccoli Plant – from Overheard in the Office

Employee #1: So we don’t sell insurance! What’s hard to understand about that?
Employee #2: I don’t know. It’s like, ‘Don’t yell at the broccoli plant for not growing carrots.’
2145 Riverside Drive
Macon, Georgia

Overheard by: not an insurance salesman