People who need people.

7 04 2014

There’s something about a rainy Monday morning that turns me into a soppy, contemplative, ugly-crying, junk food-craving mess. Especially a rainy Monday morning following a hot mess of a weekend. (You know.)

We all acknowledge that I’m headed toward break-down, right? I’m in my Coming-To-Jesus moment. So close to the tipping point.

I have a very best friend who’s also having her Moment, and maybe it’s because of her and her pain and her need for real, true help, that I’m looking so hard at my own face in the mirror this morning. Her crisis is fairly monumental. Mine is not yet. But her wake-up call should be fair warning to me. I should accept this gift of someone else’s alarm and get out of bed before my own alarm starts screaming in my ears.

I had a panic attack yesterday. My first in years and years. It’s no real surprise. I’ve been in a retrograde as of late , revisiting aspects of my past life, both good and bad. My late teens and early twenties were such a tangled rat’s nest of coming into my own and losing parts of my soul. I was so full of promise, full of motivation, a bright shining star of drive and curiosity. People thought I was going places. People believed in me and took chances on me. I dutifully did my best to make them proud. I worked hard, lusted after learning and set myself up to gain as many new experiences and branch out into as many networks as I could.

Unfortunately, I was also in a whole lot of physical pain, a whole lot of the time (and didn’t know why of course,) and physical pain can easily fade into emotional and psychological pain, until they are one and the same. I started having panic attacks, and I figured out that drinking alcohol would temporarily stop a panic attack, so I did a bunch of drinking. I needed it. Couldn’t make it through without it. I went to some terrible psychiatrists, went on all sorts of pharmaceuticals that made me fat and zitty and emotionless and sleepless and numb, but nothing did the trick like beer. Lots and lots of beer. (Good, fancy beer of course. Then you can call it a hobby and say you like learning about how beers are brewed or some bullshit like that. I’ve now given up that facade and drink wine from a box. Because I’m poor.)

Between the immense pain I was almost always feeling, and staying either drunk or hungover, I spent my early twenties inching further and further away from all that promise of great things to come, and found myself falling into irrelevance and obscurity and without the network of support I worked so hard to create. Depression took over everything.

I’ve been close to putting the fire out over the years since, but I’m still dancing around, stomping out flares, never quite able to stop the smoldering. I’ve had some wonderful moments, and beautiful children, and a supportive partner, lovely sunshiny days with sand in my toes, big smiles on my face, happy moments with laughter and joy. But underneath it all has been that annoying depression pit blowing smoke in my face, no matter which side of it I stand on.

I’ve lost control (as I have over the years from time to time) and too many flames have popped up in too many places and I’m over my head. It’s either get some help dowsing the flames, or burn.

For whatever reason this happens to people, my way of coping has been to rebel or something? Have a mid-life (third-life?) crisis kind of thing? I’m so overwhelmed with scary thoughts and anger, that I’ve wanted to disconnect from Right Now. And be in another place and another time. Like my back-thens. When things were hopeful and fun and there was still a good enough amount of serotonin pumping from my brain. I’ve started eating meat again, digging out dusty old CDs, contemplating crop-tops for this summer. Putting myself back in my 21s. But I forgot about the ugly flip side: The panic attack, I’m floating off the face of the earth and can’t breath and will surely die or stay permanently out of my mind side of my 21s.

It sucks.

I tried to eat brunch with one of my most favorite trusted people yesterday. As we sat on our bar stools and decided which fancy DC version of snobby eggs and meat we wanted, the panic hit me. Boom. Out of nowhere. Just like that.

Hello, old friend. It’s been years! Where have you been? Mucking around the darkest recesses of my psyche? Oh? What’s it like down there? Why don’t you tell me all about it while I sit in this brunchy place-to-be-seen and pretend to be interested in turkey hash and lox on a salad! How about we hold hands, spin around and get dizzy together! What’s that? You want me to hold my breath and see how long I last before passing out? Ok! Uh-oh! There you went and took my perception of reality again, you tricky panic you! I better find a way to make it into the bathroom and stare in the mirror for a few minutes to remind myself of who I am and what I’m really doing right now! But then, how will I ever manage to walk all the way back to my seat ALL BY MYSELF? Maybe all these people in the restaurant know you’re here. Maybe they can see you. Yep. Definitely they can see you. And hear you. You’re not staying invisible enough! We should leave. It’ll be better if we’re alone. Except then you might kill me! Ugh. What do I do? The thought of us staying here together seems impossible! But the thought of us being somewhere else together seems awful too! You’re so high-maintenance, panic. I hate you.

I had to make my friend leave brunch early because I really, honestly thought I might just fall right off the bar stool and make a fool of myself.

I also was having a really, really hard time not losing my fucking mind and screaming and crying and begging to be taken to a hospital and knocked out so I could just wake up on the other side of it, in a psych ward, with gallons of Xanax. Can you imagine!?! The lady with perfectly smoothed hair and pretty coral lipstick, in her boyfriend jeans and booties and latest thrift score of a gorgeous Banana Republic silk trench, just dropping then and there, grabbing the server by his shirt and screaming “Take me to a hospital! I’m going insane! The monsters are eating me!!!!” foaming at the mouth, black mascara making blobby raccoon circles around wide, crazy eyes… I almost wish it had gone down Iike that. It seems so dramatic and interesting.But no, when panicking, I must always maintain the picture of calm and happy on the outside, and somehow I do it well. (The picture below is actually me at that brunch. While I spun out of control. Can’t you tell? The other picture is the night before. If only I knew what was coming.)

Eventually the panic caused some nausea, I did some throwing up, saw some blood of course, and the physical pain took over and drowned out the panic.

So that’s one way to cope.

What are we all going to do with me?

So yeah. Before this shit gets out of control again… I gotta find something to grip onto and pull myself together. I have importance, and my brain knows to tell myself that, but my heart is having a hard time believing it. It seems easier on me and everyone else to just fall apart and be done with it, because I forget that I matter to people. People, like my kids. My husband. My parents. My friends. There are actually people and things in this world who need me, like my dear friend who’s in the midst of her own falling apart and deserves my shoulder to lean on. Plus, I have some plans for what’s to come next, and they’re fairly important plans.

So could someone get on over here with a fire hose? Sheesh. I’m exhausted.

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Next?!?

28 03 2014

Here I go, blogging on a stupid iPhone. But I’ll do it, this once. I’m feeling a strong pull to get some things out of my heart and into the world. (Maybe one of you blessed readers will send me a pretty computer in the mail to replace the one that a toddler decided to explore and destroy, thus allowing me to share with you in a manner that doesn’t drive me crazy from typing on a phone screen. A phone, people!)

I’m at a pinnacle of sorts. On an edge. A razor’s sharp, shiny edge. One wrong move and I fall hard. Or slice myself right down the middle, from crotch to head? Maybe that.

I came home from the hospital again seven days ago from yet another week-long stay. I’ve stopped writing about my attacks because I doubt they’re interesting to anyone anymore, yeah? But for those of you keeping track, it was a grand total of something like 7 visits (totaling about 48 days or so?) in 2013, and 4 so far for 2014. Each incident brings a new complication, becomes a little harder to treat, requires more intervention. I lose energy, momentum, will, dignity, modesty each time. My body doesn’t feel like mine anymore. There are no more private parts. After as many catheters, monitors, enemas, scopes, and cameras as I’ve had stuck in and on my body, there is just no room for propriety any longer.

I’ve gone through being unresponsive with only 4-6 breaths per minute, prompting the rapid response team to race to my bedside. I’ve laid perfectly still and remained calm while they told me they were about to administer a drug to stop my heart, in the hopes of resetting it and breaking it from the pattern of supra-ventricular tachycardia it was stubbornly racing through. (Feeling your heart straight-up stop is terrifying and exhilarating and I’m almost inclined to recommend it.) I’ve experienced moments of searing nerve pain so excruciating that all I can do is beg the nurse to rip all the clothes off my body, so I squat on the bed naked, minimizing anything coming into contact with my skin, and cry, because no medications stop nerve pain. I’ve watched blood splatter out of my mouth during moments of violent hurling, from the gashes burned into my esophageal track after such constant illness. I’ve had two ports yanked out, and three put in, because after several months of hemetin and dextrose they gunk up and stop working, essentially threatening my dripped-in promise of life.

And these attacks have, for the most, fallen into a neat little pattern. About four days before my period is to start, I usually feel the first twinges of pain. Usually less than 48 hours from that point, I’m hooked up to my machines and pumps and I’m struggling to post squinty smiley pictures on social media to assure everyone I’m fine.

Seeing as the attacks are following such a tidy calendar, the obvious (to many) conclusion is to eliminate that calendar. Take away my cycle. Treat me with hormones. I started talks with one of my doctors, who happens to be a smart, quiet, funny researcher from NIH for whom I have a great deal of respect and fondness, about just this a couple of months ago. In my mind, we were talking about birth control pills (or a shot or IUD or something,) to stop Lady Flo in her tracks. But In his mind, we were not discussing birth control at all.

We were discussing menopause.

Dr. NIH wants to give me a hormonal therapy that will send me into post-menopausal hormone levels. He dropped a few printed pages of a study done on the treatment for AIP, and said “This is what we’ll do. Find a gynecologist to work with and we’ll get stsrted.”

Just like that.

As though facing menopause at 33 is something I shouldn’t take issue with. Sure! Let’s shut the ovaries down! Dry that silly uterus up! Gain weight! Get hot flashes and mood swings! Kill what libido remains after 5 years of chronic illness! Make sex painful and difficult! Get wrinkles and saggy parts! Turn old, now!

Of course it may not be quite that extreme. And I don’t mean to be offensive to women who are menopausal! (I’m being hyperbolic here because I’m sort of panicking, obvi.) I’m not one to say that I’m dreading menopause, WHENEVER THAT MAY NATURALLY HAPPEN.

But y’all. I’m 33. I’m not 100% sure I’m done with my baby making! I’m feeling (other than sick and exhausted and beat-down,) pretty bangin and confident, actually. I’d like to sit in this third-life space for a while, experience my thirties as a 30-something year old. Not having to worry about significantly increased risks of lady-cancers (I already have a greatly increased risk of liver cancer to work with,) with crazy-times mood swings and even less energy that I have now.

Maybe I’m making too big a deal of it .

Maybe I should do it and shut up .

Maybe I can’t.

This has literally stopped me in my tracks. Reading through the study has not encouraged me, with only about a 25% rate of good results, out of only FOURTEEN WOMEN , one of whom died… But if I don’t, then I am selfishly robbing my family of the opportunity to not lose their mother/wife/teacher/housekeeper/meal-maker/laundry-doer monthly to serious illness and recovery.

I don’t know what to do. Obviously I have more research to sift through, but it’s hard to come by, and no clear answers are going to glare at me from the pages of study summaries . I have a strong feeling that my decision will ultimately be made from my gut. The one that bleeds when it’s upset with me. My heart. My silly heart that beats too fast and too hard and needs round- the- clock monitoring at times.

This is the beginning of the end of living in denial for me.

You see, I truly have been in denial since I was diagnosed. You may not think I have, but I have. Each time I experience an attack , I think it’s the last (you’d really think I’d have caught on after the 12th or so hospitalization…) And I have a deeply buried belief that I am responsible for being ill, that my symptoms are not some disease that I innocently inherited through no fault of my own, but that are manifestations of my own poor decisions and sins. And that porphyria wouldn’t rule my life if only I didn’t let it.

It’s time to face my life head-on now. Either find a way to live peacefully with the disease , or find the fortitude (and energy and money) to fight the disease. I think there are merits in both. Validity in wanting rest and surrender and passivity, and validity in not wanting to roll over and settle for this as a way of life.

Which way will I go?

It’s time to make some choices.

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Shrinking… shrinking…shrunk.

12 08 2013

This post has been sitting, unpublished, for quite some time. It’s been tricky for me to hit the post button on this one. But, here I am, in bed, on dilaudid, facing the possibility that I may be checking in for my fourth (FOURTH!) hospital stay of 2013, and it seemed a good time to let this one out there…

I have a really good dishwasher now. I’ve never had one before. This one has all sorts of settings, like how hot you want the water to be, how sanitary the dishes should be when they’re done (which, by the way, does this mean that dishes typically emerge un-sanitary?) and how long you want it to wash for, when you want it to start, and what you’d like for breakfast in the morning. Before, my dishwashers were just on and off. And they just picked up the grime from the dirty dishes, swirled the grime around a bunch to mix it into a brown grime batter, and redistributed it on all the dishes, so I could have the pleasure of standing at the sink for another hour chipping dry grime batter off all the dishes as they came out of the dishwasher.

But this one is different. This is a DISHWASHER. If I set it on super hot, sanitary rinse, heated dry, extra forceful water-guzzle-put-a-hole-in-the-ozone setting, I can drop completely full bowls of cereal in that puppy and have squeaky clean dishes an hour and a half later. It’s awesome.

Except for one thing.

After running it like that for the first time, I was unloading the dishes and putting them away. I have an über-organized container cabinet. It’s the only thing in my life that’s über-organized, and I’m crazy about it. Lids go on the containers, and they are stacked by type and size. None of this nesting the containers inside each other, letting the lids go all willy-nilly mess. And if by some act of God I lose a lid or a container, and I end up with a floater, it stays tucked away to the side for about a week, giving its partner a little time to show up under the kids’ bed, before it goes to the trash. I will not tolerate uncoupled containers or lids.

So, I’m unloading dishes. I pick up a glass container and its respective plastic lid. I try to pop the lid on as I spin around to place it in the cabinet. But the lid won’t go on. I look in the dishwasher, study the lid, look at the brand of the container, double checking I have the right pair. I do. I try again, and it won’t go on. I put it down on the counter and push on one side. It pops into place. I push on the other side, and it pops into place, but the first side pops up. So I push it down again, but this just sends the other side back up. I stood there, like a fool, pushing on this lid, going around in circles, for a solid five minutes, with little tantrum breaks. I was being pranked, I felt sure.

After it sunk in that if someone really wanted to Ashton Kutcher me, Tupperware wouldn’t have been at the top of their list, the reality of the situation came into focus. My crazy dishwasher and it’s boiling water had shrunk the lid of the container, just a tad. Enough to send me into crazy-times in my kitchen that morning.

The pieces went into the recycling bin.

This is a post that’s been weighing on my mind for quite some time. It’s not a post about dishwashers. It’s a post about money. Specifically, what role money plays in the lives of those with long-term serious illness.

It’s a subject that rarely gets mentioned in any sort of a relevant way, I feel. Sure, you hear about people on NPR whose lives have been ruined by hundreds of thousands of dollars of devestating medical bills, who’ve gone through bankruptcy again and again and lost their homes and jobs and insurance won’t cover their life-saving treatment. It happens to good people. People who don’t deserve it. It’s a horrific situation.

You also see people all over social media raising money for the kid down the street with cancer, or the mom who got hit by a car and is in a coma, or the soldier who came back maimed and can’t work. And those people have thousands of dollars raised for them by caring individuals in their communities and annonymous well-doers touched by their facebook page and businesses who host fundraisers (and get a shitload of awesome publicity, ahem.) If you’re one of the people who’s given to families in need, I commend you. We need more people like you in the world, who are sensitive to the suffering of others and want to help fellow mankind have an easier walk through the rocky roads of life’s hardships. And if you’re a person who’s been sick, and your friends and families have rallied around you and helped with your medical bills and living expenses and maybe even given you gifts and sent you on vacations, that is awesome and I am happy for you. I really am. Because I know first hand what a stress money issues become on sick people and their families. It can take over your life. Fast.

But most of us are in the middle of these. Most of us either have some insurance, which leaves us with giant co-pays and deductibles and procedures not covered, or don’t have insurance and get hospitals to write off some of the bills and work out payment plans for what’s left over. Either way, we’re drowning in debt that follows us around the rest of our lives, knowing full well we’ll just keep wracking up more debt for as long as we live, and we probably have less income than we used to, because we miss work and miss pay, or our spouses have to take time off to care for us, and we’re trying so, so hard to squeeze the lid on the container. It used to fit! It IS the lid that came with the container! But now there just isn’t enough lid to squeeze around the rim. What happened?

This is my monthly budget: Every single paycheck, I write down what expenses we have, then I subtract them all from the paycheck amount, and then I use the negative number I end up with at the end to revisit the expenses and determine what’s not going to get paid this time.

If we have an emergency, and we always do, forget it. We either can pay for it and sacrifice another bill, or we can not pay for it. We most often opt to not pay for it.

Many of us with diseases live like this. Many of us get hospital and doctor and lab bills every day in the mail that we throw into a pile on the kitchen table. When that pile gets too big, we move it to the BIG pile in the office. When that pile gets too big, we box it up and throw the box in the basement. Which we periodically dump out and frantically search through when we need a copy of a birth certificate or car title or something because we can never find those stupid things. (Or, that’s just me ?)

Many of us don’t answer 800 numbers, or any number we don’t recognize, because we know it’s a bill collector. Many of us write and re-write and re-re-write the numbers in different orders, hoping to somehow make it all add up. Many of us ponder bankruptcy, but feel like we maybe aren’t really there yet(?). Many of us grapple with the decision of whether or not we should try to work and risk our health for the sake of paying bills, and risk losing our tiny disability payments and Medicare that we spent 3 years fighting to get. Many of us are seized by guilt every moment of our lives for the burden we’ve placed on our families.

Money is so paradoxically personal, and yet on display, in American culture. Polite people don’t really talk about it. You’re not supposed to tell people how much you make, or how much you spend. You’re just supposed to look like you make and spend an appropriate amount, whatever that is. Eric and I have been struggling with money for years. We’ve spent a lot of our lives trying to hide that fact from people we know, making excuses for why we can’t go to Bermuda for a wedding or rent a beach cottage with friends or why we just “needed some more time to try to find the right place” when we moved in with his parents. Fact is, we’re broke. Some of it has to do with bad money management habits, not being frugal enough, not saving the right amounts.

But mostly, these days, it’s because of my disease.

If I was well:

-I could work at least part time and make good money. Enough to fill in the gaps anyway, and even save a little. I’m educated! I could earn a few bucks. I did, before I got sick.

-I may have been in a position to upgrade my busted computer and software so I could continue to do some freelance graphic design, and my kids would have a better tool for educational purposes. Instead we struggle with an old, broken down machine that’s beyond software upgrades and is useless to half of the internet, and can’t run Adobe programs without checking out to race its engines every five minutes before ultimately just giving up all together.

-I would never have gotten to the point of having a credit score in the double digits that feels beyond repair with loads of medical bills. And, by the way, if anyone ever tells you that medical debt doesn’t matter on your credit report, they’re lying! They don’t know what they’re talking about! Come talk to me and I’ll let you ride in my 23% APR car that I had to buy with my awesome medical bill credit history.

-I wouldn’t be so desensitized to medical bills that I just made no effort anymore with them. When you have ONE, and they ask you to set up a payment plan, ok. That’s doable. When you have 25, and they all want you to set up a payment plan of $20 or $50 or $100 a month, not so doable. So you just ignore them all. I’ve devoted way more energy and time into correcting insurance mistakes, billing errors, applying for aid, reapplying for aid, etc. than I have to just trying to be as healthy as I can. Seems like a bad deal for the insurance companies to me, when the stress they create literally makes me sicker, but what do I know about the big bad insurance industry?

-We probably wouldn’t have been in such a pinch when Eric had a dental emergency a few years back, and left us with a huge bill we couldn’t pay. And dentists, unlike hospitals, are under no obligation to accept your payment-plan plans, and can insist on nothing less than two payments of half over two months, or nothing at all, which is what ours did, so we paid nothing and three years later without warning they started garnishing it from his wages, so our paychecks have been hundreds of dollars less than they should be the last couple of months.

-And maybe we’d have some savings so that when Eric went to a good accountant to have his taxes done and found mistakes from years past that meant we owed a bunch of money, we could have paid it then and there. Instead, we offered payment plans (are you seeing a payment plan theme in my life yet?) and while the federal government accepted it and now deducts a relatively small amount from our bank account every month for the rest of forever, the state government just didn’t, and again, started garnishing wages, so that our already smaller paychecks lost another couple of hundreds of bucks for a few months.

So let me do that vague math for you: Our income is almost $1000 short per month these days.

I don’t care how much you make, that’s a pretty devastating shortage. Trying to hide that sort of financial crisis just doesn’t work. It’s rent that can’t get paid, kids that don’t get to go to summer camp, internet that gets turned off, gas that can’t get put in the tank, groceries that don’t get bought. It’s not hide-able.

But I’ve been (almost literally) killing myself to pretend like everything is ok. We have a lovely (rental) home and a car in the driveway, kids who do the summer reading program at the library, a guy who goes to work in a suit and tie everyday, a mama who goes to yoga twice a week (which she gets for free in exchange for doing some work for the studio) so everything looks perfect from the outside.

But we are so -not- perfect. I am crumbling. I am fatigued from trying to squeeze this lid on my life. I’m sweating, out of breath, trembling, with bleeding blisters on my fingers, trying to make this lid bigger than it is.

And so I’ve started telling my friends. I just couldn’t pretend anymore that things were fine. And you know what I started finding out? Many of them aren’t fine either. They’re struggling to pay off student loans and car payments and and it gives them nightmares, or they’ve ruined their credit scores by ignoring their Target card statements, or they’ve got a mortgage bigger than they can handle and they’re living off their 401K.

But we all put on our lipstick, fill our wine glasses, and smile and chat about lawn mowing services.

For REAL y’all?

Look, if we all suck with money, then we need to acknowledge it, stop judging each other, and stop comparing our own insides to other people’s outsides. ESPECIALLY those of us who are struggling with illness. Money is a source of stress, and stress is poison to a sick person’s body. Lying is also a source of stress. Money and lying together are enough to shut you down.

And if you’re one of the ones who DOESN’T suck with money, help a sick sister out! And I don’t mean by giving your money away. (Fundraisers are nice, but I wonder how much they help people in the long run?) I mean by supporting your friends who aren’t doing so well in other ways. Like, first off, don’t invite us to the Bahamas. We can’t go. Instead, come over to our houses, bring us booze, sit down at the table with us, and help us work out our budget. Tell us what you’ve done to get your finances in order. Talk to your rich friends to find out if anyone is, or uses, a good fee-only financial adviser who will donate their services for a short time to help get things on track. Offer to babysit for free if we need our spouses to get a second job to cover expenses but the thought of being home alone with the kids for 20 more hours a week is terrifying. Help us sort through medical bills and call hospitals to make sure everything is being billed correctly, because that alone can be a full time job. Cook us dinner once a week, because time with friends is like medicine, nights out on the town are just not an option for those of us who are financially challenged, and saving the cost of even one meal can be helpful to us.

It sucks putting this out there. It does. It’s degrading and embarrassing. I feel like I am too smart for this to be my life. But it is my life. And a few weeks ago, when I was feeling particularly hopeless and I was sobbing to my parents after we had to tell the landlord he was only getting half the rent payment and the next half would come with the next paycheck (hopefully,) I heard myself saying “I am just so terribly disappointed in life. This is not how I thought it was going to be.”

And it got me thinking that I bet most folks who’ve been handed a serious diagnosis are feeling the same thing. Not only are we less able than we’d like to be, less energetic, less clear-headed, more restricted, more isolated, but we’re probably all pretty fucking broke, and angry and sad and stressed out and ashamed of that.

Well, I’m ready to at least stop being ashamed. I may be angry and sad and stressed out still, but I can at least stop being ashamed. How about you? Ready to slap it on your sleeve? “POOR AND PROUD!” Let’s start a movement, y’all.

There’s just absolutely no reason we should pretend to keep up with the Jones’. The Jones’ might be underwater with their upside-down mortgage, paying for their automatic sprinkler service with the credit card that’s almost maxed out, the balance of which they’ll just transfer to the next lowest interest rate offer they get so they can max that one out too, strapped with huge car payments and if anyone loses their job they’re ruined next week. Maybe not, but my point is, you’d never know.

For those of us who are sick, we simply can’t afford to worry about what we can’t afford. If you are the supporter of someone who is sick, please remember that in almost every circumstance, the financial side of disease is just as eroding to our well-being as any physical symptom. Ask us of we’re ok, and listen to us when we say we’re not. That’s all. Listen. Without judgment. Without political agenda. If we can all just admit that the damn lid shrank, toss it out and move on, there’d be a lot less crazy in the kitchen.





New AIP treatment in the works?!?

22 05 2013

I got an interesting email yesterday. It’s really technical, but here’s the exact text, straight from the American Porphyria Foundation:

Alnylams Pharmaceutical presented data on their new AIP treatment at the recent International Porphyria Congress in Lucerne Switzerland.

In pre-clinical models of the human disease, they demonstrated RNAi therapeutics targeting ALAS-1 can completely block the abnormal production of toxic intermediates of the heme biosynthesis pathway that cause the symptoms and disease pathology of AIP.

They expect to identify a final development candidate by late 2013 and advance ALN-AS1 into the clinic in 2014. ALN-AS1 is a subcutaneous RNAi therapeutic targeting aminolevulinate synthase-1 (ALAS-1) for the treatment of acute intermittent porphyria (AIP), an ultra-rare genetic disease. ALN-AS1 has the potential to be a therapy for the treatment of acute porphyria attacks, as well as a prophylactic approach for the prevention of recurrent attacks.

To view the presentations from Alnylam and Dr. Robert Desnick’s Mount Sinai team, see below: Note that Protect the Future doctor, Dr. Makiko Yasuda, made the following presentation on the Mount Sinai research.

http://www.alnylam.com/capella/wp-content/uploads/2013/01/Desnick-AS1-PorphyriaCongress-May2013.pdf

Alnylams presentation:

http://www.alnylam.com/capella/wp-content/uploads/2013/01/ALNY-Porphyria-AS1-Pres-May17-2013.pdf

Visit the Alnylam site and read details of this exciting news:

http://www.alnylam.com

Now, what I think this means, is that a new treatment, that happens to be some sort of subcutaneous device (?) can treat an attack more effectively than hemetin and glucose, and when used as a preventative, can provide two weeks or more of protection against triggers. I come to this conclusion after reading through the PowerPoint presentations and, while I’m no doctor, I’ve been around a lot of them lately. And I think I’ve picked up some of the foreign language they speak, known as medicalese. It’s a difficult, awkward, overly-verbose and nonsensical language, often scribbled illegibly. If you have more experience with this highly coded and secretive language, and better understand what the hell those PowerPoints are saying, please do share.
But, if I’m correct, and I think I am (wink wink) then maybe this means an end to weekly infusions in the future? Maybe it means every few weeks, I can just go get a little injection or something? That would be… Life-changing. Huge.
I’ll keep you updated on any more news about the study as I get it.





A girl at every port…

17 05 2013

So I’m home and re-ported now! The surgery went well. I was at least not awake this time. (I’ll have to share the story of getting the infected port out with no sedation next post…)
I know the staff of IR (interventional radiology) well now, thanks to a cranky old port that required quite a bit of… intervention. So I felt pretty comfortable going into it this time. After some back-and-forth with the anesthesiologist about which drugs we could and couldn’t use, we landed on a pretty simple plan.
In the freezing cold theater of IR (I came prepared this time with wool socks,) he started me with a hefty dose of fentanyl to take the edge off. It made me relaxed and friendly. We were all besties there for a few minutes, me and my anesthesiologist and nurses. I shared my hopes and dreams, funny stories, embarrassing stories, dark secrets, and promised to name my next child after them all. Then he gave me the actual anesthetic and that was that.
I woke up asking for more of it.
I remember them telling me it’s what killed Michael Jackson, see why he liked it so much, hahaha… Geez. Glad they didn’t mention that little nugget of trivia before sending it into my bloodstream.
I felt good and woozy for a whole, then the pain came, so they generously dosed me up with some toredol and morphine. After an hour or so of laying around and nibbling gluten free pretzels, I decided it was time to go. I stood up and promptly decided it was in fact not time to go.
There it was… The sea legs and double vision and nausea and floppy muscles.
I’m home now and still a bit woozy, but I’ve managed to eat something decent, but the morphine has worn off and my chest and neck hurt like a bitch. I forgot how bad this hurt the first time! It absolutely sucks folks! Good thing I have a cabinet full of narcotics, which I’ll be dipping into momentarily.
Before I go, I leave you with a treat. They removed the picc line after surgery was over (they used it during surgery instead of starting an IV.) And pulling a picc seems so closely related to extracting a giant blackhead, so Eric couldn’t resist taping it for your viewing pleasure :) Click here to watch it.
Enjoy!

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Well, then.

13 05 2013

It seems I’ve committed the worst of blogging sins. I’ve started and stopped and started and teased and stopped.

Here’s the thing, folks. Two kids, a crappy computer, lots of weird moves and shuffles in life, and the blog hit the back burner. I’ve thought of it often, and I’ve been asked about it and I get comments every now and then with questions from readers, and I’m always like “one day, I’ll get back to that!”

And a year later…

My last post was a year ago.

My next-to-last post was a year before that.

Oops.

If you’ll have me, I’d like to tip-toe back into this space. But maybe in a different way?

I like to write. A lot. But your time is precious, and so is mine. And you don’t have time to read epic-length blog posts, and frankly, I don’t have time to write ‘em.

But I’ve had so many, many relevant experiences and thoughts and ideas in the last year, and I’d really like to be sharing them again. It’s cathartic to me at the very least. And it’s potentially helpful to some of you.

So let’s try again, K?

Here’s the quick rundown since my last post: That illness I spoke of last? Yeah. Resulted in 4 nights in the hospital, with an attack, step throat, and a staph infection in my bladder. It was SWELL. But the hospital let little 5 month old Esther Pearl stay in the hospital with me, sleeping in my bed, with no interruption to her nursing schedule, Eric in the pull-out chair thingy next to us, so it all worked out ok.

Then life went all roller-coaster on us, and we ended up moving to Alexandria. For Eric’s job. That told him he HAD to move or he’d lose his job. So we lost our house to not lose his job. Then he lost his job anyway. And we were stuck in Alexandria, in what felt like The Most Expensive City In The World in The Most Expensive Apartment Ever.

So that was fun.

(He found a new job after a few months, and he’s doing great now, and we moved out of that dumb expensive apartment and to a more rural area of western Northern Virginia in a great big old stone house with swings and a big yard and a marsh and a creek. It’s all good.)

So, I was ok-ish until spring of last year. Specifically Mother’s Day 2012. The day porphyria came to visit. It apparently decided I needed a Mother’s Day vacay, so we checked into the hospital, porphyria and me. We stayed for 4 days or so. In the end, it was all worth it: The hospital gave me a lovely little Mother’s Day gift of some decorative ceramic measuring spoons from Pier One. Win!

A few months later, in August, another attack slammed me out of nowhere. (OK… Not exactly out of nowhere, but more on that later.) This time, it was my birthday! Weeee! Four nights that time. But again, as with the Mother’s Day gift, the hospital pulled through with gluten free doughnuts stacked up and topped with whipped cream as a special “cake” for me. Even though I was too busy puking to eat it, I was pleased as punch, as it was the first time in YEARS I didn’t have to make my own birthday cake.

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My special birthday cake concoction on the left. A regular, glutenous chocolate cake on the right, that they threw in for my visitors. I was told it was the worst cake ever in the history of cakes.

So then on New Year’s Eve, which was also our 5th wedding anniversary, porphyria was all “Oh? Another special day we can spend together? Let’s hold hands!” I woke up New Year’s Eve morning thinking I was going out on the town with my groom-of-five-years. I ended New Year’s Eve in the ER with yet another FALL RISK bracelet on my wrist, pumped full of fun mind-altering drugs, aiming for the little bean-shaped vomit caddy the hospital so generously provides, coming up for air to tell my date (Eric, of course,) stories about the orange children’s chairs floating in the corner of the room. Yep.

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Eric took this in between vomits. It was a very fleeting, drug-induced, shit-eating grin. In my head, in that moment, I was probably telling off the imps who kept shaking their little butts in my face. It was a weird night. Pain+Delirium+Narcotics+Fever+Dick Clark’s NYE Special on TV= Weird Freaking Night.

That one was almost a week in l’Hotel l’Hopital, thanks to a very special strain of the Flu From Hell. Not only was a pain pump not keeping up with the ninjas punching me in my stomach, but I couldn’t breathe without some O2 assistance to boot. It was, by far, the scariest, most horrible hospital stay yet, thanks to some serious incompetence on the part of the hospital staff (to be discussed later) and some serious asthmatic behavior on the part of my lungs.

And last, but not least, to round out this last year of getting to know porphyria all the more intimately, I was yet again invited to be a guest of the hospital for 5 days, thanks to an infected port. That was a couple of weeks ago. The port was yanked (without sedation and with very little pain medication, by the way; A really fun story for another post!) IV antibiotics were administered in the hospital for five long, boring days, a picc line was put in, IV antibiotics were continued at home (mostly by my six year old daughter) for another 2 weeks, and now that I have absolutely no sign of flora or fauna or bacteria or fungus or elves in my system whatsoever (thanks to 2+ weeks of shooting powerful antibiotics straight into my veins,) I’m allowed to have a new port put in, which happens this coming Friday.

That’s where I am with this. Two years in a nutshell.

I’d love to share more with you about each of these ordeals, and what I’ve learned (and not learned,) but not here, not today. I’ll feed you little bites, yes? We can do this snippet by snippet.

And when the bullshit explodes like it does, I’ll give you play-by-plays in realtime! No more playing catch up. How does that sound? (Friday should be good, y’all. Watch out! Should I live-Tweet the surgery???)

Don’t be mad at me for disappearing. Let’s just pick up where we left off. Pretend that year didn’t go by. And get ready for some more me!





Moving right along…

12 02 2012

Hiya.

I’m thinking of coming back.

I took a year off from blogging, in case you didn’t notice. I had a baby, and a million obligations, and I’m no Amanda Soule.

But maybe I’ll come back here now.

A WHOLE LOT has happened. Life-wise and porphyria-wise. And as I’ve been searching for my own answers and solutions lately, I’ve been thinking that maybe, just maybe, some of my questions could be someone else’s answers, because things tend to work that way. And maybe it’s not fair for me to be withholding my experiences, since there is so very little out there about my disease.

So shall I go way back to a year ago, and start fresh? And leave you with a cliffhanger to entice you to tune back in?

Yes, I think I shall.

The last post I entered was announcing Esther Pearl’s birth. She’s 13 months now. A little stinker, she is. Crawling at 6 months, climbing onto the kitchen counters at 8 months, walking at 10 months, and trading stocks at 12 months.

Esther Pearl, the Stinker.

After she was born, I stayed home for a couple of weeks, snuggling, sleeping, loving the new little pumpkin. And recovering from my vagina being completely ripped to pieces by a human being who was a little bit stuck in me for a lot too long. Recovery seemed slower this time than with Adelaide. Maybe because this time my age started with a 3? But for whatever reason, I dragged. I crept back to life, one hesitant little step at a time (being careful to never spread my legs too far lest I, ahem, rip things back open. Sorry.) I was really, really happy though. I can remember standing at the kitchen sink, wearing the baby on my chest, hearing Eric and Adelaide giggling in the next room, and weeping with joy because I was now a Mother Of Two, hand-washing dishes and scraping poop out of cloth diapers!

Yeah, that didn’t last long.

Eventually I got back into the swing of things, taking Adelaide to co-op, teaching flute, trying to make it to the Unitarian Church now and then, dragging Adelaide around the neighborhood on a sled whenever we got an inch of snow. And the winter blues caught up with me, though  not as severely as in years past. I had a few days of feeling stuck and sad and I’d cry for no reason, but those days would pass quickly and I made it through relatively depression-free.

I’m thinking somewhere along there, in late Feb or early March, I got a little sick,  and started feeling a bit porphy. So I resumed my weekly hematin infusions. Remember, I’d stopped them while pregnant because of a lack of information about the effects of hematin on a fetus. But I went as long as I possibly could after having her, until I was finally feeling the effects of not getting it. I remember being afraid that it would make my milk taste bad, and that she wouldn’t want to nurse (which would be a problem, since she’s never, ever accepted a bottle. By the way, as a side note, this means that to this day, Eric and I have yet to get a proper date night. Just so ya know.) But my worries were in vain. Even if the stuff did make my milk taste off, after spending 3 or 4 hours away from her food source, which wasn’t something she was used to, she was happy to have her boobs back and nursing was never an issue.

I think I kind of coasted along for the next couple of months, but stayed kind of sick-ish. Like, virusy and infectiony. Then, one night in mid-may, after spending a few days flying solo since Eric was out of town for work, I had a really, really, long day. I was asked to perform in an alumni flute choir performance for my former flute teacher from the Governor’s School for the Arts, who was retiring. I’d spent 2 days in a practicing frenzy. The alum pulling it together, who had actually graduated, like, the year before, who was currently studying flute somewhere fancy, picked the piece and sent me the music, via email, literally 2 days  before the performance. This wouldn’t usually be a problem for me, as I’m generally good at sight-reading and flubbing through, except that in this case, she picked something “simple”, and by “simple” she meant Senior Recital for Julliard simple. So, I did what I could to practice it, hoping that 180 tempo would manifest in something like a, I don’t know, 60 tempo, and figured there’d be plenty of alum there to play over me and I could just “pretend” on the fast, fancy runs.

She’d asked me to show up at the big theater hall at 2pm to rehearse, so I arranged for my parents to watch Adelaide, and I strapped EP on and walked in to the practice room, to find that the alum group consisted of me and about 4 other people. All of whom were exactly 19 years old. They stared at me with my baby carrier and raised their little teenage eyebrows, saying um? that’s, like, cute? um? is it, like, a boy?

Awesome.

I was told I’d be taking the second flute part by myself. I felt the sweat immediately run down my legs, Christina Aguilera style. Whatever. Only the most important musicians and teachers from my high school years would be gathered there that evening to take in the performance. No pressure.

We practiced for all of about 10 minutes, when the non-human prodigy college freshmen decided they’d rather be texting than practicing. So I walked across the street to  find something to eat, which resulted in a wilted bowl of lettuce with dry slivers of carrots and a quarter of hard tomato from Wendys because thats what happens when youre gluten-free and vegetarian. Knowing my stress level was up, and I’d been plagued with what felt like a UTI for weeks, I knew I needed to carb-load to stave off the porphy monsters, but I think all I could manage to find carb-wise was soda or something.

Anyway, the performance was supposed to take place after the GSA Orchestra concert at a special surprise reception. So, the concert stared at 7, and I had my parents bring Adelaide and meet me at 8 (she loves to watch me perform,) thinking the concert would be over around 8:30, the reception would start, I’d play, and be out the door by 9:15.

Well.

The concert lasted until 9:30. The reception didn’t start until 10. And then the 19 year olds told me we were playing at the end of the reception. My poor little girls lasted and lasted and lasted, until 10:30 or so, and, just as I was told we’d be going on in 5, EP melted. the heck. down. In a panic, I stepped out, nursed her,  begging her to pleeeeeease just fall asleep, and she conked out JUST as someone opened the door to say I was on.

I strapped her into the Ergo, her little sleepy head flopping back, and walked onstage. I played. Everyone thought it was the cutest thing ever. I smiled. I bowed. I grabbed my stuff and got the hell out of there.

Can you see the little bump on the front of me, second from left? That's a sleeping EP.

On the way home, I felt that ickiness that happens when a fever’s starting in. The heebie jeebies in my hips. The shivers. By the time I walked through my front door, my throat was scratchy. I kept telling myself I was just exhausted. I fought two little over-tired, cranky girls whose sleep schedules were completely off, into bed somehow. Eric was actually due to arrive in the middle of the night. I tried to sit up in bed and wait for him. By the time he got home at 2am, I was a shivering, sweating, lump of feverish mess with a blazing throat and delerium.

He spent the next day trying to nurse me back to health. We were both thinking it, but neither of us were saying it. We knew it was the perfect formula for an attack.

By noon, the popsicle he’d talked me into ingesting was making its  second appearance.

Then the back and abdominal pain hit, and between that and my throat, it was too much for me to handle. By the way, ever throw up violently when you had a sore- no, fire-breathing-needles-in-your-tonsils throat? It sucks, is what it does.

Eric didn’t like where it was going. He wanted me on pain meds and in the ER asap. I wanted to stay home with my 5 month old and avoid pain meds because of her refusal to accept any other form of nourishment than my actual boobs.

So, did I stay or did I go?….

And there’s that cliffhanger!

{Thanks for reading again. I think I’m going to be glad I came back ;)}








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