It’s been a rough couple of weeks. Of course, since I’ve been a Bad Girl about not journaling, now I have to remember it all right now. My husband is even sitting right here to make sure that I get this all done. 
Had a lip swell a couple of weeks ago, followed in a couple of days by an eye swell. Neither were too terrible – over by about noon.
In the last few days, since I’ve had to stop taking my Allegra, I’ve been a truely swelly gal. Tuesday night I had a bit of butt and foot swelling, and yesterday my left eye started mid-afternoon, and my hands followed in the evening. They are HUGE – I’m hoping that some typing and later knitting will help move some of the fluid out. Also have a giant hive on my right ankle where my sock was hanging out yesterday.
(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.
Woke up with a very slight bottom lip swelling – didn’t even last long enough to get a picture. Interestingly, I was at the HAE conference and didn’t see a single other person swelling.
Woke up at some ungodly hour with the sensation that my tongue was starting to swell. I tried to go back to sleep for a while, but the swelling made it so that I couldn’t keep my appliance in my mouth that helps me sleep. I was done with sleep at about 6:00 a.m.
I stayed in bed until about 11:00 a.m. trying to get a few winks, but finally got up. I was not yet able to talk or eat/take my meds. I started being able to talk after noon, and everything but the glands under my tongue was pretty well done by 2:00 p.m.
At about 7:00 p.m., my right eye started swelling up. As of right now (12:30 a.m.) the swelling is still on the way up. Also swelling on right hand and right heel.
Fun time lapse of eye swell!
8:19 p.m.
10:18 p.m.
10.16.2008 12:12 a.m.
10.16.2008 7:07 a.m.
Went to bed last night with thumbs and forefingers on both hands starting to swell, and they started to subside late this afternoon. I think that was a pressure swell from me trying to open the back of my laptop. I need to replace the fan. But the damn back won’t come off! And the little screws seem to be stripped, although I’ve never opened the back! And I really, really don’t want to have to pay someone to do this for me!
Anywho, woke up this morning with the residual hands, a bit of feet, and a left eye pretty much swelled shut. It opened by early afternoon but still looks pretty bad. For a day or so after my eye opens, it looks like I have terrible bags above and below my eye. It’s kind of weird looking, but I’m frankly too lazy to take a picture and do all that uploading and stuff right now.
The worst thing about the constant swelling is the fatique. I’m averaging about 12 hours of sleep a night, and am still exhausted during most of the day. I’m not getting anything done (not that there’s anything dire that needs to happen), but I’m feeling like a bit of a lump right now. And my house is a mess. And I have tomatoes that need to be salsa-d, but it’s going to have to wait until next week.
As of right now, 10:00 p.m., my thumb and forefingers are still fat, but not hurting, although they do seem to be a bit more heat senstive than usual. My eye, as mentioned, is still ugly but on the downswing, and my left foot is starting to swell in the heal area, although I have not worn shoes since early yesterday. Boo.
To all of my readers (yep, all two of you), please to be sending non-swelling thoughts my way.
Woke up with upper lip swelling (fairly even through lip) which mostly resolved by about 2:00 p.m.
Update (10:53): left thumb, right pointer finger, right foot, right middle toe (oddly).
Really, this streak can end any time.
Woke up with lower right lip swelling, mostly inside the mouth, which had pretty well resolved by 1:00 p.m.
Had a bit of hand swelling throughout the day with no apparent reason. That lasted all day, and after we helped out at the Smile Network gala, my feet were swollen although I had not walked very much (deliberatly – wasn’t in the mood for more swell!)
I went back to the immunologist this week for my three month checkup. We will all remember that it was about a week after my last check up that I ended up having three swells in five days, and called it quits with employment. I have since been approved for long term disability through at least February, 2009, and will go back to the doc in January to check in again.
I saw Kris, the FNP-C, and we mostly talked about pregnancy and birth things (not because I am pregnant, mind you – don’t get all excited!) and it seems that we are pretty much on the same page (no drugs, no intervention during pregnancy unless absolutely needed, and a shitton of plasma as soon as the cord is cut to keep me from blowing up/minimize the risk to the baby).
As for treatment, there is nothing to report on that front. I am allowed to go back to work for 10-15 hours per week if I want to (that’s still up for debate, and is frankly kind of an issue right now – I just don’t have the intestinal fortitude to write about it right now).
Kris did mention that she thought the C-1 concentrate had been approved by the FDA, but I haven’t heard anything on that front. The last official update I had from the HAEA was that it had gone through the Blood Products Division and recommended for approval, but we haven’t heard anything since then. I’m heading to the HAEA conference in Chicago next month, and I’ll be interested to see what the update is then.
As for swellings: nada. Apparently this no-working thing really agrees with me.
