If you’ve ever read my posts on ER Safety, Hospital Safety, or the Hidden Corn- Medical Supplies post, you probably know that blood transfusions contain corn derivatives. I’ve known this a while and made plans for eventually having to have an emergency treatment that would require corn exposure in order to save my life. But I had always hoped it just wouldn’t happen to me. But of course it did, last July.
I’ve had blood tests showing mild anemia here and there for a lot of years. It was sporadic. Sometimes my red blood cell count would be fine but my hematocrit was slightly low. Sometimes rbc & hematocrit would be low. Other times my rbc and hematocrit would be fine, but my MCV would be high. My iron and b12 levels were always fine though, and my doctors and I always felt we had bigger problems to worry about, so not much was done to look into it.
I’ve also had many years of difficulty with what seemed like POTS symptoms (tachycardia when shifting position from laying down to sitting, or sitting to standing) that came and went seemingly with MCAS flares. Over the last couple years these episodes have been coming more and more often, but were still episodic. I kept chasing a POTS diagnosis but we could never manage to do testing while it was happening.
Last July, I finally had a severe “dysautonomia” episode. Except it wasn’t.
What I experienced was a few days of feeling like I had “the flu” except my fever was never above 99°F. I slept constantly and felt too weak and tired to even write an email from my phone to let work know I would be out. After a day or two of this fatigue I began to have dark, tea colored urine which I perceived as “bloody” urine and thought I had a UTI.
After I was feeling less tired, I began to have tachycardia (rapid heart rate) whenever I changed position from laying down to sitting or sitting to standing up. This tachycardia would fade /normalize within a minute whereas in POTS it should continue for some time after changing position The tachycardia would also be accompanied by throbbing head pain that would pulse in time with my heart beat. This pain would happen if I even tilted my head without changing position. At times I would feel the throbbing pulsing in my lower back.
I also had some pretty bad cognitive deficiencies. I left a burner on and almost burned the house down, forgot names and common words, couldn’t remember things that had happened earlier in the same day. I also was so confused that I didn’t realize this was an emergency so I waited to see my regular doctor. I should have gone to the ER
My doctor did a complete blood count “just in case” and saw that I was pretty severely anemic. It turns out that the symptoms of an inability to regulate your own blood pressure and heart rate appropriately are really similar to the symptoms of just literally not having enough blood in your body. We had never done blood testing before during one of these episodes because I had so many other reasons to be dizzy and have irregular blood pressure.
She assumed I had some kind of internal bleeding, and called me back in to her office the following day to do some physical exams to see if she could determine the source of the bleed (she couldn’t) before sending me to the emergency room with my lab results and ER packet in hand. I knew I was likely to need inpatient care, so I also brought my hospital suitcase and made sure my freezer was stocked with frozen prepped food for someone to grab and bring in as needed. More details on my hospital prep in my Hospital Safety Post.
When we arrived at the ER, they triaged me in with 0 minutes wait time, and were actually pretty great about my MCAS instructions. They still managed to cause me a moderate reaction with a pre-filled saline flush for the IV, which it turns out contain preservatives even though the night nurse insisted it only had saline and nothing else. And the hospital itself was pretty bad for me airborne wise due to the cleaners they use, as it always is. I was wearing my Vogmask with the behind-the-head strap to create a tighter seal and make it more comfortable, but I wish I had had my respirator instead, so I have now put it in my hospital kit. You can see what respirator and masks I use on my product list page.
The ER concluded (as I had suspected) that I needed blood transfusions. Normally they would do these in the ER, but since I was a reaction risk, they admitted me before starting them. I was pretty darn scared because of how badly I react to the dextrose and citric acid when I ingest them, and these same derivatives are the ones used as anticoagulants in blood bags. To try to prevent a very serious reaction, we used a modified version of the TMS protocol for pre-medication when a reaction is likely:
- Solu-Medrol (Generic: methylprednisolone) 125 mg IV 2-3 hours before transfusions begin. Must be preservative free.
- Benadryl (Generic: diphenhydramine) 25-50 mg intravenously or intra-muscular 1 hour prior to surgery/procedure. Preferred brand for IV Hospira, NDC 0409-2290-03. Follow with 25-50mg every 6 hours as needed until transfusions/procedure is complete.
- Zantac (Generic: ranitidine) 150 mg or famotidine 20mg orally or intravenously 1 hour prior to surgery/procedure. Follow with same dose every 6 hours as needed.
- Administer 5-day steroid taper after transfusions are compelte. I did a compounded corn-free version of the medrol dose-pak.
We couldn’t find an IV h2 that was without preservatives so I took my own compounded. The iv benadryl was very important if possible because it’s far more effective than oral route. If you are worried about a reaction intramuscular benadryl will be almost as effective as intravenous but less likely to cause as severe a reaction as intravenous.
When we did the transfusions, I definitely reacted to them while receiving them, even with all the steroids going in my system. If we had somehow been able to hold off for several days it would have been better to start oral steroids 2 days ahead of the procedure, rather than a high dose right before, to give it time to build up.
I itched, flushed, and was tachy the entire time I was getting the transfusions, and also had bone pain and joint pain, but no asthma or rash and blood pressure was okay. They released me late into my second day at the hospital. The attending physician said I had hemolytic anemia, meaning I was making red blood cells just fine, but they were rupturing and dying early. He said he’d normally keep me an extra day for observation but that I was clearly constantly reacting to the hospital and he thought I would do better at home. He recommended a hematologist follow up ASAP and discharged me with care instructions to watch for reactions to the transfusions for up to 5 days after the procedure.
For the 5 days after, I would begin itching, flushing, and having tachycardia any time my round the clock benadryl, ranitidine, and steroids would wear off. By the beginning of day 5 I began to be able to tell that I would be okay off the steroids with just the benadryl, and by day 7 I was able to stop taking benadryl every 6 hours and just go with my daily meds.
Something else was going on, even while I was fighting all these reactions though: Other than the constant histamine-related inflammation, I was feeling freaking GREAT. All of the blood pressure problems, heart rate that fluctuated with body position/posture, and general feeling of weakness was GONE. I didn’t realize until that moment how weak and fatigued I’d been for years even during my “good” times. Even though my face was puffy and I felt someone had put itching powder in all my clothes, every time someone asked how I was doing, I would say, “AMAZING, now that I’m plump with donor blood!”
Unraveling the Mystery
Once I was a bit more stable, it was time to figure out what the heck happened to me. I went to a hematologist, and after running some tests to make sure I wasn’t still hemolysing, she formally diagnosed me with autoimmune hemolytic anemia. This sounded likely given that I had faced a lot of MCAS inflammation from traveling right before the episode, and had been running a fever during them time when I was feeling too sick to get out of bed. However I didn’t *like* the diagnosis because the treatment for it, as stated by her, was to basically try real hard not to get sick or end up in any state that would encourage my body to destroy blood, and if I did, to treat with steroids. If the hemolysis continued to be an issue, the next courses of treatment were a splenectomy, and possibly chemotherapy.
That didn’t make me feel too great already. Considering how much better I felt post-transfusion, I had probably been hemolysing frequently for years leading up to the crisis. I didn’t like the idea of just walking around wondering if I was going to destroy blood with no particular way to control it.
If that’s how it was, I was going to have to be okay with that and do what I could to calm my body down and stop it destroying blood. But I just had the feeling it was worthwhile to dig in to other possible causes of hemolytic anemia, especially if there might be a kind where I could have slightly more control than just hoping very hard. Someone from one of my MCAS support groups told me that she had learned about a condition called G6PD Deficiency, commonly called Favism, that causes hemolytic anemia when certain foods (legumes, *especially* Fava beans) and medications. This condition primarily affects people of asian, african, and mediterranean descent, although it can affect other populations as well.
Well, it so happens that I am southeast asian, and that I was eating Fava beans about 3-5 days before this crisis. I also was eating fava beans 1 year before when I had a similarly severe episode that we didn’t do any blood testing around. Before that, I had never eaten fava beans before in my life. It was just a delicacy at the farmers market and I took some home when doing my regular shopping there because they are delicious.
I requested my PCP to help me get tested for g6pd deficiency. It turns out testing isn’t always very accurate for women, especially right after a hemolytic crisis (more on this later), but we figured why not run it once and see what came up.
I was deficient. Just barely, because it was so soon after my hemolytic crisis, but still deficient, and false positives are not a thing with this test really.
We had a diagnosis. I did get a second opinion from a hematologist to confirm it, but she agreed, and so did my original hematologist that had diagnosed me with autoimmune hemolytic anemia, once she saw the charts.
More About G6PD Deficiency
G6PD Deficiency is a genetic deficiency of an enyzme caled glucose-6-phosphate-dehydrogenase, or G6PD. This deficiency causes all of my cells to be vulnerable to damage from oxidative stress. In particular red blood cells, since they are so simple and have no mitochondria, will literally be destroyed by this stress.
This blood destruction is actually triggered by a number of foods and meds, the most severe food being, of all things, fava beans. Which is why the common name for this condition is “favism.”
The list of foods and meds that you have to avoid are as deep as corn, and have a HUGE amount of overlap with corn. The question of whether it’s safe to drink tea or not when you have this deficiency is answered with discussion of how the tea leaves are processed, whether there are an artificial flavors added, and even the tea bags or packaging the tea comes in. When someone asks what is a safe toothpaste for g6pd deficiency (g6pdd for short), the answer is that glycerin, sorbitol, and xylitol are all to be avoided for g6pdd. Yeah, really. Does this sound familiar, corn allergy people???
I won’t go fully into what needs to be avoided for g6pdd, but I will give a rundown of the condition and risk groups, both for the sake of trivia and in case it helps someone else out. One thing that I’ve noticed as I’ve read into this is that a number of my weirder non-corn MCAS reactions are actually to items that are also G6PDD triggers. Apparently there are a number of antifungal/antimicrobial coatings used on things like pipes and faucets that are quite stable but would definitely cause oxidative stress for someone who is g6pd deficient. I think this may be the cause of my weirder reactions to water. G6PDD causes hemolysis, not allergy, however I feel my mast cells may have attuned to hemolysis triggers.
G6PD Deficiency is NOT rare.
What 6PD Deficiency Is
- G6PD Deficiency is a mutation on the X chromosome that affects creation of the enzyme glucose 6 phosphate dehydrogenase, also known as G6PD.
- Red blood cells deficient in this enzyme become unstable and are destroyed in the presence of certain foods and medications.
- The worst of these foods are fava beans, soy, and most legumes.
- The worst non-foods are NSAIDs, tylenol, fluroquinolone antibiotics, anti-malarial drugs, sulfa drugs, and any petrochemically derived substance including most artificial dyes, flavors, and synthesized vitamins.
- The list of foods and meds that trigger this blood destruction are broad and deep. Basically anything that triggers “oxidation” is a problem. You know all those supplements and foods that have anti-oxidants? Well some foods, meds, and supplements have pro-oxidants.
- NOTE that anything that creates oxidative stress on red blood cells can destroy them when they are deficient in g6pd. So I am still at risk to lyse blood cells when I have an infection or a mast cell reaction.
Who It Affects
- The origins of this deficiency are from mediterranean, asian, south asian, african populations. But the deficiency is now found in all populations.
- Primarily diagnosed in men but NOT only present in men. Just harder to diagnose in women when they have it.
Three things I want to point out about inheritance of g6pdd deficiency:
- Contrary to what some outdated resources say, it is NOT a recessive gene. If you have it, it is expressed. This means that if you are a woman, you are not a “carrier” of the deficiency, you have it.
- If you have even one copy of it you will be partially deficient. I am only partially deficient yet had this severe crisis and many issues over my life with anemia and the results of low level hemolysis that did not show up on tests.
- This deficiency is x chromosome linked. That means that if you are a male with g6pdd all your daughters will be at least partially deficient. If you are a woman with g6pdd your children have a 50% chance of being deficient. If you are a woman whose son is deficient, *you are also deficient*. He could only have inherited it from you. More info on g6pdd inheritance.
- In severe cases, hemolytic crisis, such as my recent hospitalization and blood transfusions.
- High ferritin- The inflammation from hemolysis can cause your body to hang on to ferritin. As inflammation decreases this should go down, but if you are low level hemolysing frequently you may see high ferritin stores somewhat often.
- G6PD causes a reduced glutathione deficiency, which is an antioxidant that has a lot of interplay with allergic and mast cell reactivity. I don’t really see from the g6pd support groups that there is a known connection between g6pd and allergy/mast cell disease, but I think it’s possible.
- Methylation defects/MTHFR: G6PD involved in the pentose phosphate pathway, one important pathway for making NADPH. NADPH is used in the MTHFR pathway. There is another pathway by which NADPH is created, so you will still have it, but less.
- Having reduced g6pd activity impairs your ability to deal with oxidative stress by reducing your amount of available glutathione. Glutathione is not directly part of the MTHFR pathway, but it comes up a lot in discussion of related mutations. This is essentially restating the entire definition of g6pdd, however I felt it was important to frame it as part of the methylation stuff for people who are looking into that for themselves.
- Low-level hemolysis can have all kinds of long term health effects that basically look like chronic illness and an inability to detox. This is due to an overtaxed system due to both the high level of creation of new blood cells and the low tolerance for oxidative stress.
Testing & Diagnosis
The definitive diagnosis is a molecular test that checks the total quantity of g6pd in your blood sample. There are a couple of problems with this:
- This deficiency is only carried on the X Chromosome. In women, you likely only have 1 X chromosome with the defect, so the other X chromosome will correct for that and only some of your blood cells will be defective. This means your total g6pd count will be closer to normal, especially if you recently destroyed your more g6pd deficient cells.
- If you just finished with a hemolytic event, you can have a false normal because you lysed all the older, deficient blood cells. In men, this is true because the younger blood cells will have less of an overall deficiency than the older ones. In women, this is true for the same reason AND because of point #1.
It so happens that my quantitative test was deficient/positive for g6pd deficiency. This surprises me because of point 1. It makes me wonder if I either have two deficient x chromosomes, or a large number of my cells underwent lyonization.
If you wanted to get tested, I would start with the quantitative test. It’s very easy and labcorp does it. Quest probably does too.
HOWEVER if your test is negative, it doesn’t necessarily mean you don’t have it because of what I wrote above. If you are eating lots of beans, using NSAIDs, or even taking benadryl daily (yes benadryl can cause some blood destruction for g6pd), you may be destroying all your deficient cells. In this case, the way to make sure would be to avoid g6pd triggers for a few months and test again, or look for genetic testing, which is expensive and difficult.
23 and me won’t cut it. They only test a couple dozen of the SNP’s and there are literally hundreds of kinds of g6pd. I didn’t have any of the major SNP’s on 23 and me and only one of the minor ones. None of the interpreter services considered my results indicative of even a chance of g6pdd deficiency yet I am deficient. Mayo has a genetic test for it available, but I don’t know how much it costs.
In my opinion, the best resource to start with is favism.net. This is a lay website put together by someone who has been a patient and advocate and worked with the g6pdd community for a lifetime. Much like corn allergy, doctors don’t give a lot of guidance on how to actually live a healthy life with the condition and prevent flares. They don’t even give a complete list of medications to avoid although they at least know of the worst medication triggers. (Well, some doctors know anyway.) favism.net is the most conservative list of avoidance advice out there, and IMHO that’s the best advice to follow for the sake of safety.
What This Means For Me
Well, my ER packet is 3x longer than it used to be and has a table of contents now, so that’s fun. I made sure to keep the hematologist who diagnosed me with the condition on my physician consult list because if I end up in an emergent or surgical type situation we’re really going to need her. I’ve already run into some complexities trying to get medical care for other conditions- I have arthritis symptoms as part of my MCAS reactions and my rheumatologist wanted to prescribe Plaquenil which is a big nono for G6PDD. That sucks because it sounded like it could help out a lot. I’m also seeing some concerningly blase attitudes about the condition from doctors, and am not sure how seriously they’ll take it in a more urgent situation like a hospital setting. That worries me some.
If I didn’t already have the corn allergy, the dietary and lifestyle changes would be a much bigger deal. All vitamin fortification is an issue for g6pdd. Cross contamination with soy like pre-seasoned cast iron pans using soybean oil as a seasoning , or ingredients that are commonly soy derivatives such as glycerin, xylitol, etc, or any and all added vitamins are problems for g6pdd and are overwhelming for newbies. On the other hand, I was already avoiding these things because they are also frequently corny.
I’m not able to eat the corn free legumes I had anymore, but that’s fine tbh. It’s a little bit of a pivot and I’m worried about having a lack of protein source variety. If I have too few options and have an interruption in food supply I could end up going hungry. I ran out of beef for a month once and lost like 15 lbs that I didn’t actually have to lose. But the amount of fatigue I had when I was eating them was so bad that it feels well worth it.
I’m really glad overall to have a certain amount of control over whether I destroy blood again. However it’s not 100% control. If I get sick or have a big reaction, or if I have an airborne exposure to chemicals (or fava bean pollen, apparently!) I could still hemolyse, and there would be no preventing that. But I can do a lot to maintain my health and lessen the chance of hemolysis.