My HAE Guardian Angels

22 Dec

Meet Kimberly (office superwoman), me (eeewuh, having an HAE attack and no makeup; figures), Dr. Hornberger (medical magician) and Jackie (nursing queen of everything and then some), the three guardian angels who save my life on a daily basis. Without this tiny little office filled with compassionate souls, I would not be alive today, or if I were alive, I’d be so loaded with up on narcotics I’d have the IQ of a jelly bean. I like Jelly Belly Jelly Beans, but their conversation is extremely limited.

I tend to think visually and symbolically, so here’s my explanation of Hereditary Angio Edema: Everyone has these little guys running around in their blood, they’re called C1, and they are the guys are are in charge of the plumbing system in your blood vessels. They carry around cute little wrenches and drink a lot of beer because they’re usually pretty bored. If they all get together and get it in their heads to have a bit of fun, and start using those wrenches to open up the hydrants that force plasma from your blood stream into your actual cells (causing you swell up like a fat hog), there’s always plenty of cops around with huge nightsticks to clobber them over the heads before the fun even gets going. The cops are, oddly enough, call the C1 Inhibitors. Now, as long as the C1 dudes are applying their wrenches when you sprain your ankle or break you leg (swelling is nature’s idea of a cast to immobilize the area so you don’t keep making bad boo boos worse), the C1 Inhibitor cops are cool and let them do their job.

Well, when you have Hereditary Angio Edema (hereditary means you can’t catch it from me unless I give birth to you, so odds are you’re pretty safe; angio means “of the blood,” and edema means swelling), you got short changed on your police force of C1 Inhibitor and their handy night sticks. Anybody with a score under 75 has HAE; my score is 7. So basically I’ve got the C1 Inhibitor police force of a single Barney Fife. And the C1 plumbers are constantly on a binge and using their nasty little wrenches without having received any distress calls at all. Meanwhile, Barney just freaks out and does nada.

The really lovely part of this genetic deficiency is that, if your throat or airway swells up and you don’t make it to an ER on time or you get there and they don’t believe you when you tell them what you have, what’s happening and what needs to be done (there’s not a doctor on this planet who thinks you know your own body better than he will after ordering 16 hours worth of useless and painful tests), you will die of asphyxiation. And if you don’t die from an attack, the pain will make you wish you did.

There is a drug trial underway in which they can actually take donated blood and squeeze out just the itty bitty little cops, the C1 Inhibitor, and give it to someone like me intravenously. After participating in the first trial, which took 12 years, the FDA didn’t approve the treatment because of one doctor’s misconduct. This treatment has been available in Europe for the past 15 years and we still don’t have it in the US. Granted, this is a very rare disorder, but you would not believe the politics that goes on at the FDA and how that drives the cost of research and development (R&D) up to the point that soon the only R&D being done is going to be for recreational drugs (like Viagra).

Give me a break! I so deserve it.
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2 Responses to “My HAE Guardian Angels”

  1. Adrienne February 16, 2008 at 1:30 AM #

    Kat,

    Thanks so much for a great description of HAE. I read it out to my husband; I find it difficult to explain it to him… even when he does see my arm swell. Luckily I have a higher level of the C1 inhibitor cops so I don’t swell as often, but currently I have a stomach swelling which simply sucks… I have to work tomorrow because no one will take over for me (I don’t think I convince them that I’m sick, and they don’t even know I have HAE… I don’t think they would have hired me if I told them). Wish me luck! Thanks again for a great write up!!

    ADRIENNE

  2. Faerie♥Kat February 16, 2008 at 12:24 PM #

    Hi Adrienne

    So sorry to hear about the abdominal attack; that is where mine predominantly occur and I’ve been told by women with HAE who have gone through childbirth and have abdominal attacks that they would rather give birth to a child any day! My daily mantra is: “Be well and don’t swell!”

    For future reference, if your company employs more than 50 people, you are covered by the Family and Medical Leave Act (FMLA):
    Synopsis of Act
    Covered employers* must grant an eligible employee** up to a total of 12 workweeks of unpaid leave during any 12-month period for one or more of the following reasons:

    for the birth and care of the newborn child of the employee;
    for placement with the employee of a son or daughter for adoption or foster care;
    to care for an immediate family member (spouse, child, or parent) with a serious health condition; or
    to take medical leave when the employee is unable to work because of a serious health condition [HAE falls under this “reason”].
    *Covered Employer = Company who employs 50 or more employees for each working day during each of 20 or more calendar workweeks in the current or preceding calendar year.

    **Eligible Employee = Employed by the employer for at least 12 months, and has been employed for at least 1,250 hours of service during the 12-month period immediately preceding the commencement of the leave and employed at a worksite where 50 or more employees are employed by the employer within 75 miles of that worksite.

    To read up on the exact details, visit http://www.dol.gov/esa/whd/fmla/.

    Faerie hugz

    Kat

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