My experience: HAE Hereditary Angioedema

The picture above is an elderly lady whose eyes are swollen because an “attack” of HAE.

As I think I mentioned previously (Freak Show, only in the Gospel of Luke: the Beloved Physician’s Water-Face-Man), I have a rare Type 1 HEREDITARY (by way of my dear mom) bradykinin mediated, not histamine induced nor otherwise “acquired” but, to repeat, certainly out of control production of bradykinin, which itself creates out of control inflammation by way of vasodilation. I need more C1 Esterase Inhibitor, without which my body is an inflammation factory from scalp to toes. This is Hereditary Angioedema.

In my personal experience:

• The lack of C1 Esterase Inhibitor occasions by the by, for me, a somewhat subtle dry-cough. I can’t tell you how many times people have glared at me for the past four years, assuming that I have Covid because of this minimal dry cough. It’s not the cough which is annoying. It’s people’s freakiness with Covid that’s annoying.
• There are signs that an “attack” is going to come soon. So, people will call this hypochondria, you know, like calling Padre Pio a fraud, that the stigmata were a result of hysteria. Padre Pio’s response was that just because someone thinks he is an ox doesn’t mean that two horns are going to grow out of his head. It never happens. Just because someone knows by way of signs provided in one’s body that there is going to be an “attack” of HAE doesn’t mean, cannot mean that this is hypochondria: One thinks, therefore one explodes. I’m sure Jihadis would like that. But, no.
  • I will feel totally worn out, so tired, devastatingly so. That can go on for a day or so, and then I figure it out.
  • More particularly, whatever part of the body is going to be “attacked” will ache or hurt or call attention to itself in a very peculiar manner, and then the swelling will start. Magic! No, it’s just annoying. It’s the almost violent, however quiet, vasodilation.
• The lack of C1 Esterase Inhibitor also occasions massive swelling, albeit episodic. Episodes, which can be overlapping in different parts of the body, for me, usually last 4 days in whatever location, one day swelling, two days maxed out, with the skin about to break open, and then one day of un-swelling. The following have all happened to me uncountable times, unless otherwise noted:
  • It can be a hand or hands, a foot or feet being the size of cantaloupes, utterly unusable.
  • Arms or legs can become like elephant legs. You walk like a zombie.
  • For zero reason, with me living my chastity, for instance, genitalia in men can swell up to gargantuan proportions as will often happen for any guy after even just an abdominal hernia operation. Soccer ball size, but it feels like you’ve been kicked by a donkey. This is rather uncomfortable, requiring super loose jeans and a super-baggy way oversized shirt. I suppose this is humorous. Hahaha. I suppose I should laugh at myself. Hahaha. Sigh. Whatever.
  • The swelling can travel, from a hand up the arm (usually just halfway up the forearm), and a couple of times from the hand all the way to the shoulder, upper-back and upper chest, and then neck. The neck thing can be fatal. See below.
  • Stomach and intestines can swell such that one simply vomits one’s own spit for days, bent over 90 degrees, kneeling for hours and hours at a time in front of the toilet. Not pleasant, especially if one’s knees are also swollen. One’s thought is that one might want to clean the toilet better, but one’s hands are likely the size of melons. You make a mental note to just get a bucket, but you’re still on all fours on the floor, trying not to pass out.
  • There are brain-swells bringing on end-of-the-world headaches, where one is frozen on all fours unable to lie down, sit down, stand up. And if one’s face is swollen as well, being on all fours, one looks to be like a donkey or an ox…
  • One’s lips can swell as big as bananas so stretched to the breaking point that drops of plasma force their way through the surface and bead up, and if you can stretch your tongue out that far you can taste this bloody taste amidst the almost ripping apart of flesh. One infirmarian-seminarian in a seminary over in Italy decades ago, frightened by seeing my lips this massive, fearing that they would explode, wanted to lance my lips. That, of course, would have been catastrophic. One drools, making one look especially like an imbecile.
  • Eyelids can be as big as billiard balls. And now you can’t see. Tear-duct floodgates are opened, and an impossible thick crust glues the eyelids shut. Eyelashes are tightly glued to each other.
  • I have a longer than usual soft palate, which is easily given over to massive swelling, closing off the back of the throat. This came close to killing me off I don’t know how many times by way of suffocation. Really a lot of times.
  • Laryngeal swelling is such that, if one tries to talk, one sounds like a possessed lunatic. Laryngeal swelling causes the suffocation and the death of about a third of sufferers, including my dear mom who died that way, suffocating… terrible… And it doesn’t matter if one makes it to an Emergency Room. Intubation or a life-saving tracheotomy is almost never going to be provided because the attending nurse/physician will say, in looking at one’s throat, “I’m impressed,” and will then add, “We’ll put you under observation. I’ll be back in 15 minutes.” And by that time you’ve been dead for 10 minutes. It happens, a lot. I was telling a doctor about this and he became instantly sad, and told me that a young man just then died for that reason at the local emergency room. I’ve been to emergency rooms I don’t know how many times, but I can put a number on the laryngeal swelling: 25 times in, like, ten years. That was too much; I started taking the meds my mom suffered for (see below). You try intubation first, but that might not be feasible. One doctor wanted to get me a kit by which I could do up my own tracheotomy in an emergency. Can you imagine cutting into your own throat? Another doctor got so angry at that suggestion, telling me to just never be far away from a hospital. He was so very sarcastic with me: Go ahead! Go be a missionary! Go ahead and die! Yeah, well, life goes on, or not. I can’t live in an emergency room.

Med students and doctors alike might benefit from this rather pedantic explanation which, nevertheless, moves right along at break-neck speed, like a last second cramming for an exam; I love it:

There is a medication that encourages the production of C1 Esterase Inhibitor by way of the liver, namely, Danocrine (generic = Danazol), which had been developed in Bethesda. For my sake, my mom was able to volunteer to be a guineapig for the research on this medication at the start of the 1970s. They about killed her so many times, purposely inducing painful symptoms, then bringing her back, then cycling her through hell again. She had many multi-week sessions of this torture. She did that for me… Thanks, mom. She had frequent and severe “attacks” of HAE throughout her life. She didn’t like taking the medicine she wanted to be available for me. Guineapig PTSD? Thanks be to God, Danocrine / Danazol was all set before Roe vs Wade and the subsequent widespread use of hek293. I’ve used this medicine periodically in the 1970s and, after dozens of near death experiences because of severe laryngeal swelling, I started taking it daily, prophylactically, until today. You’re only supposed to take it for a short period as it’s a bit rough on the liver, but after 40 years of regular usage I’m still alive. I still get attacks, regardless, but less severe, less frequently.

There are more recent, insanely expensive, super dangerous human blood plasma alternatives, etc., with lots of practical difficulties and which are also unethical in that hek293 was used for research, development, testing. I’d rather die than use anything that required the murder of kids for pharmaceutical profiteering. And, no, I didn’t get any fake “vaccines” or “boosters” for any form of any Covid rubbish. And I’m much better off for not worshiping Tony Fauci.

Anyway, not all that long ago the Rochester Mayo Clinic was readying itself to do a deep dive into studying this ultra rare condition. No conclusions to that study to this day methinks. It ain’t easy. I was there for something else, but they had asked if I was allergic to anything, and I said that if I’m getting the logic correctly, I’m surely allergic to ACE inhibitors since the presence of angiotensin-converting enzyme inhibitors give free reign to bradykinin all the more, unto my demise. My internist, one of the top long-time doctors at Mayo (who helped me get rid of latent TB from Mother Teresa’s MC’s TB ward in Byculla, Mumbai, India [also not why I was there]) just stared hard at me for, like, five seconds, and then said, “I think you’re right. Yes. How did you know that?” I told him about my mom and the medicine.

Oh, I should add a note here about the first of that set of videos above. Note that at the beginning of the first colored-magic-marker-sensory-overload video, which is quite brilliant, he mentions the old name for hereditary angioedema as hereditary angioneurotic edema. That “neurotic” descriptor was removed after science became a bit more scientific. Thus…

When there is laryngeal swelling, the patient might sound demonically possessed or sound at least like a lunatic. And regardless of whether there is facial swelling, the observer is going to be scared and say stupid things like “It’s all in your mind,” and then that observer is going to call 911 and have the sufferer interred at an insane asylum as if it were 1888. Yep. We’re just so modern!

Does a sufferer get nervous before the onset of an “attack.” Sure, because he might die in the next few hours and he knows that nobody knows about this ultra rare malady, or cares. An attack doesn’t happen because the sufferer gets nervous for any or no reason and that’s what brings on an attack. Yet another doctor just told me that. No. A sufferer might get nervous because he knows it’s going to happen and gets nervous about immediate consequences, like going to the emergency room only to meet up with doctors who don’t know what you’re talking about and just watch you die. It’s happened, a lot. But get this, however strange it is: an “attack” happens for physiological reasons. Duh. We have to get over the “it’s all in your mind” thing. Calling people crazy to cover one’s a** because one doesn’t know everything is… wait for it… crazy, and makes an a** of oneself (an insult to donkeys everywhere). People die because there are doctors who will not admit that they don’t know everything.

One doctor just graduating out of med-school at one of our best teaching hospitals very recently told me that although she vaguely remembers some seconds spent on this super rare hereditary condition, no meds that actually help were discussed. Nothing. She never heard of Danocrine/Danazol. That made her really upset. She knew what it meant not to have heard of that medicine: It meant that these patients are not worth helping to live. But we are. I’m so happy she was angry.

One can say until one is blue in the face (but you’re unable to talk, and you are blue in the face) that this condition is not histamine but rather bradykinin mediated. And lemme tell you, no matter how many times you tell doctors it’s not histamine related and that epinephrine won’t help, they want to give you epinephrine and then watch you die, thinking they’re coming to the rescue. I have epipens at the ready “just in case”, because “they might help.” Grrr. If I say that I’m “allergic” to ACE-inhibitors I mostly get blank stares, but at least they write it down, but then ignore it.

I’ve tried a couple of times (but no longer) to wean myself off the medication for the reason that it’s somewhat destructive of the liver. In reaction, my whole body explodes into truly monstrous swelling, multiple sites, continuously, for weeks, hands, feet, face, stomach, intestines, genitals, throat. I’m sure all that massive swelling puts enormous strain on the heart. I’ve given up on trying to wean myself off the medication, but what’s one to do if the supply chain goes bad, again?

Here are more triggers or repercussions of episodes of HAE:

SPIRITUAL: As I’ve mentioned, I’ve had the laryngeal swelling dozens of times, with the airway reduced to like a pinhole, so that if, lying down for days, should I turn my head just a degree or two, the throat would close off tightly, no respiration possible at all. People dismissively say, “Just go to the emergency room.” Many times in life, no emergency room is available, and should you try to go, because you moved the position of your head and neck just a degree or two, you will die, within a couple of minutes. I’ve asked Jesus the forgiveness of my sins and asked Him to please bring me to heaven so very many times in such circumstances. That does good for the soul.

LAST RITES: Although being on death’s door because of laryngeal swelling, I’ve many times been denied Last Rites because I’m told that what I have isn’t serious because, you know, the priest hasn’t heard of HAE. The feeling of betrayal unto death because of such arrogance is not good for someone as weak as I am in such a situation. I can’t really explain the matter to their face at the time because I’m so weak, on death’s door, and I can’t really talk. And there’s no sense explaining it later because they’ll just laugh and say that they were right to protect the Sacrament of Last Rites from someone who obviously didn’t need it because one is still alive. It’s like the Salem Witch trials. If you died you were innocent. If you survive you are guilty and now they will kill you outright with their mockery. Have mercy, Lord, have mercy. I’d like to punch some of these priests right in the face, but, no, then my hand would swell up. Laughter is better.

EMPLOYMENT / ECONOMIC: If you’re in the military and then you’re diagnosed, it will get you discharged into 40% disability. I would never have made it into the military, not even as a chaplain. You can’t get a job which can involve any traumatically hard labor.

PHYSICAL TRAUMA: So, it’s true that that physical trauma can trigger an attack. Thus, the hands might swell up as triggered by carrying (in the good old days) 85 pounds of books in one piece of luggage through miles of airports while on one’s way to Rome for studies. And then you can’t use your swollen hands. On the other hand, as it were, there may be no discernable cause for any localization of swelling. It just happens.

MEDICAL COMPARISON: It’s useless to compare the HAE type of “dropsy” with other types of “dropsy” that instead come about through congestive heart failure or hepatic or renal failure. Those other “dropsy” conditions can go on for a long time, causing the skin to grow and make room for the swelling. With HAE, such locative swelling is temporary, although extreme. Other types of dropsy are almost always fatal even if treated over periods of time, while HAE can kill you forthwith. I remember one doctor, so very impressed with swelling due to congestive heart failure or hepatic or renal failure, that he thought that what I had (and he had no idea what HAE is), couldn’t possibly be anything serious like those other things: “AND IT’S NOT DROPSY!” he exclaimed. “DROPSY IS SERIOUS!” I told him that his exclamation was an insult to my mom who died from this. No! he insisted. He’s the doctor! He’s the one one who sat through medical school and took exams! I’ll stop there. Just know that it’s not a game of comparison.

PHARMACEUTICAL: With decades of experience in finally taking the medication quite regularly, I noticed that sometimes I’ll get sick anyway as if I wasn’t taking it, but I am taking it, and I nevertheless feel much worse than ever. I then discover that the pharmacy is using super-out-of-date medication, which loses it’s efficacy and becomes poisonous as it goes out of date. This happens because there’s not much demand. They don’t throw it away. They don’t want to lose a few bucks. Thanks a lot. That can lead to death.

STRESS [anxiety?]: Yes, but only if whatever it is, is tearing you down, wearing you down physically, such as not sleeping for days because of being in an end-of-the-world situation. That’s happened to me in war zones wherever in the world. I have a pretty high threshold of stress. “Normal” people will run away screaming from that which I take in stride. That’s not to brag; I’ve just seen a lot. My difficulty is being stressed out by that which doesn’t stress many people at all, such as my having had to live in a seminary with filthy liberal seminarians and professors and faculty and admin, or to live in a rectory with a filthy liberal priest who’s trying to force me to do filthy liberal things. Ain’t gonna happen. So I’m perpetually in trouble. Stress and anxiety are two different things. Jesus was stressed in the agony in the Garden of Gethsemane to the point of sweating blood. Necessary stress can be good. But it only makes one more vulnerable to an “attack” of HAE. But that’s true for everything, right?

CLOTHING: My hands have swelled up to the size of melons so many times, uncountable, that if I buy a long-sleeve shirt or jacket or coat or cassock or an alb (for Holy Mass), I’ll make sure to get that which is oversized, you know, to hide as best I can the hands which are swollen up the size of melons. When a tailor measured me for a soutane, I told him to make the sleeves longer. He didn’t want to do it, but he did it. I wear baggy shirts, baggy pants, sandals with minimal and elastic straps. Gloves… fuhgedabatit. You just want a track suit top that you can pull over your head, and a track suit bottom with an elastic waistband with no zippers, ties or buttons. There are occasions when you have to use the restroom, and zippers, ties and buttons are annoying. I’m happy attacks are not all the time.

CONDESCENDING REMARKS:

• When I was in the seminary, the vice-rector, later a Cardinal, insulted my vocation from Jesus to the Catholic priesthood by saying that surely the reason why I wanted to be a priest was just to do my part for eugenics by not getting married, not having any children who might have and therefore continue this malady unto future generations. As you might imagine, I told him exactly what I thought about his style of eugenics and his style of discernment of a priestly vocation. Those who are disabled in any way are not trash, and do have lives worth living. And the priesthood isn’t a trash-heap vocation. I’ll stop there.

LAUGHING WITH ME: If I have an episode of inflammation (swelling) in the face, people feel repulsed, but make better of this reaction and laugh with me, saying thing like: “Father George got in a fight!”

DIET: I’m gonna have to think about this. If anyone’s a dietician and can figure out the videos above and has advice for me, I’m all ears:

• I’m only right now finding out that ACE Inhibitors (Angiotensin-Converting Enzyme Inhibitors) are increased by certain foods that I now know I have to stay away from. See the link for the fuller list. I include some here that I have to particularly remove from my diet: Garlic, Tuna, Sardine, Hawthorne berry, Hydrolyzed whey protein, Sour milk and milk peptides, Gelatin, Omega-3 fatty acids, Chicken-egg yolks, Zinc.
• I’m only right now finding out that some foods and vitamins are direct vasodilators, and while that’s good for blood pressure, it’s bad for the HAE. So, some things I gotta cut out, some on the same list above: Omega-3 fatty acids, monounsaturated fatty acids (Omega-9 fatty acids), Potassium (K+), Magnesium (Mg++), Calcium (Ca++), Soy, Fiber, Garlic, Flavonoids, Vitamin C, Vitamin E, L-arginine, Taurine, Celery. I mean, I’ll have to look if I’m taking some of those things…
• NOTES:
  • Chicken-egg yolks: When I was a little kid, hardly more than an infant, eggs gave me huge angry hives, a reaction I grew out of pretty quickly. Looking back on this I’m guessing that eggs were a trigger for an attack of HAE. Eggs to this day are my kryptonite. I can tolerate them a bit as an ingredient, but just a bunch of eggs wears down my system until I get super sick and get attacks of HAE.
  • I’ve been doing up my diet all wrong all my life, eating tons of tomatoes and garlic and whey protein and cheese until this day. I even took Zinc when it was all the craze during Covid. How important is this?
  • Potassium: I looked at foods rich in Potassium… so, this is impossible. Potassium is like… air… You can’t cut it out of your diet. Maybe just don’t take artificial tablets of Potassium?

Weirdly, I have super high blood pressure. It happened over night about a year ago and stayed that way. I don’t know why. But diet is important. Can diet really hurt me? Can diet really help me? There’s lots of contradictory things going on here. One food is good for one thing and at the same time bad for another.

To be continued…

But also, just to say, I’ve had other priests be ferociously envious of the playing out of my priesthood, the places I’ve been, the things I was able to accomplish, the people I’ve known…but, lemme tell you, the grass is never greener on the other side of the fence. Everyone goes through stuff. Sometimes we don’t know what other people go through. What I know is that our Lord will have us all carry a cross. He will have us die to ourselves to live for Him.