I'm Meg and I have an extremely rare disorder, idiopathic familial methemoglobinemia, it has killed me twice and makes my skin take on a blue color.
I'm Meg and I have an extremely rare disorder, idiopathic familial methemoglobinemia, it has killed me twice and makes my skin take on a blue color.
Comments: 128 • Responses: 53 • Date: 2015-11-11 13:31:49 UTCsource
Nimja_33 karma2015-11-11 14:31:08 UTC
Hi there, can you elaborate on: "killed me twice"?
And of course good luck!
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xxambellina55 karma2015-11-11 14:44:35 UTC
I was adopted when I was an infant and it was never disclosed that methemoglobinemia ran in both sides of my biological family. I went a very long time without having my methemoglobin spike high enough to turn me the blue color, and when it did I went to the ER where they put me on oxygen. The problem is, no matter how much oxygen they give me- my red blood cells can't carry it-- so I destat rapidly. The first two times it happened I did not get methelyene blue in time and it resulted in me clinically dying. The first time I was in a coma for 12 days protected with a plastic sheet like the damn outbreak monkey. They finally called the CDC who sent a representative that finally figured out it was methemoglobinemia.
However, anyone can have a one time episode from exposure to certain substances, so they did not realize I had the genetic disorder (as it is extremely rare). The second time I just waited way longer to get to the hospital than I should have. Luckily the doctors were able to bring me back each time.
Nimja_19 karma2015-11-11 14:55:22 UTC
Yeah, the problem (unfortunately) with any human healthcare is that more likely options are treated first. With a very rare condition it can take VERY long for it to be determined.
So what about the second time?
xxambellina25 karma2015-11-11 15:02:16 UTC
When your pulse oxygen drops very low your brain doesn't work correctly (I don't remember but friends and family tell me I act like I'm WASTED). When I woke up the morning of the second death (also the second bad reaction I'd ever had) my pulse oxygen was already very low so my judgement was impaired. I remember looking in the mirror and deciding that I was blue because I drank too much cranberry juice the day before and laying back down. I ended up calling a friend (this was like 7am) and they thought I was wasted and since I was acting so funny they came over to check on me and saw the blue and took me to the hospital. It just happened because I waited way to long to get help.
Nimja_5 karma2015-11-11 18:21:24 UTC
Indeed, the brain does funny things on low oxygen.
But I'm glad you survived and hope you manage many more years!
xxambellina5 karma2015-11-11 18:23:56 UTC
Thank you very much! I certainly am a mess when my pulse oxygen is low, I was told by my friend that I was like dealing with a belligerent blacked out drunk.
fannypacks4ever2 karma2015-11-12 06:42:51 UTC
Does it help oxygen levels when you are at sea level (vs being at high elevation)?
xxambellina3 karma2015-11-12 15:01:08 UTC
I sincerely have no idea, but I would think it wouldn't. I'll try to explain it as best as I can without getting to doctorish.
So normal people have ferrous iron in their blood with a tiny (less than 1%) amount of ferric iron. I have a large amount of ferric iron (12-19%) in my blood. The ferric iron binds to the red blood cells/ oxygen and even though there is oxygen there the ferric iron "hangs on" to it and won't release it to bind to my tissues. When you get too much you can't breath and oxygen doesn't help- so I don't think the elevation would do anything as my ability to breath doesn't count on taking in oxygen, but in getting the methemoglobin to release oxygen to my tissues.
I hope that makes sense- I honestly suck at science and chemistry in general so it's hard for me to wrap my mind around- there is a whole thing about the ferric iron has a +3 and the ferrous has a +2 so because of the charge.. blah blah blah. I really should learn it and keep it in my head, but again, I'm chemistry challenged.
fannypacks4ever1 karma2015-11-12 21:40:23 UTC
You explained it well! I understand it better now. It's more of a problem with the oxygen cells not being released due to the high amount of ferric iron holding on to them. Thank you for doing this AMA! :)
xxambellina1 karma2015-11-13 15:57:25 UTC
thank you for being interested and participating!
xandra_enaj5 karma2015-11-11 17:36:37 UTC
I had a bad reaction to benzocaine, and after I turned blue and my oxygen rate dropped at an alarming rate, the doctors decided I had methemoglobinemia. Now I can't have certain medications because they might cause the same reaction. They treated me with methylene blue and everything was hunky-dory but I remember how terrified I was watching that oxygen number drop; my heart goes out to you, having to live with it every day. I wish you all the best!
xxambellina7 karma2015-11-11 17:39:59 UTC
If you'd like a more comprehensive list of things that can cause reactions please inbox me.
The thing is, most people never have a methemoglobin reaction if they don't have the genetic disease. It is thought that people who do react naturally carry a little more methemoglobin in their blood and are perfect candidates to have another reaction.
like I said, it's very rare for a non-genetic person to have acquired methemoglobinemia.
RiseAgainstFan1315 karma2015-11-11 14:51:29 UTC
Do you think you can apply to the Blueman Group?
xxambellina21 karma2015-11-11 14:55:43 UTC
It's actually pretty funny, but no. I am not nearly blue enough to fit in with them- and with my disorder one of the things that I should never ever handle (because it will raise my methemoglobin) is anything with blue, green, or purple dyes that isn't food-grade. Essentially applying blue paint like they wear would kill me without quick treatment.
GreenHatSam5 karma2015-11-11 17:57:01 UTC
Why? How does that work?
xxambellina9 karma2015-11-11 18:13:03 UTC
I'm not sure. Basically what I, and the doctors go on, is recorded instances of people having a methemoglobin level high enough to be life threatening and require treatment. If someone has a reaction then the doctors work hard to figure out what factor caused it. Then the doctors write studies that other doctors used.
for example: I had a spray tan and immediately had a reaction. My doctor went to the tanning place and got the chemical makeup of te product then figured out which chemical did what to cause a reaction. Then he wrote a case study and now people with the disorder know not to spray tan.
I would imagine that the blue dye can sometimes have something in it and that causes methemoglobin to rise. Even though I don't know exact and it's probably that every blue dye doesn't cause the rise in methemoglobin I have to stay away from it because it isn't worth taking the chance of dying or spending a month in ICU.
TakeoKuroda3 karma2015-11-11 18:24:24 UTC
wow, that's interesting. Well, I'm glad you survived that AND your Dr got to write a study to help others!
xxambellina2 karma2015-11-12 17:20:51 UTC
lilkimi14 karma2015-11-11 13:44:59 UTC
How does it feel being blue?
xxambellina30 karma2015-11-11 13:49:37 UTC
It's odd because I'm not blue all the time. Normally when my skin is a very blue color it is because the methemoglobin levels in my blood are so high I require hospitalization. When I'm blue and in the hospital I call myself a "sick-lebrity" because all of the hospital staff come to my room to get a peak at me and the doctors and residents all want to write case studies on me.
When I have a blue hue in public people really stare. It's strange because, as I stated before, I'm not always blue-- so I forget I'm blue and I wonder why people are staring..
lilkimi2 karma2015-11-11 14:13:31 UTC
Get well soon man!!
xxambellina21 karma2015-11-11 14:19:31 UTC
Thanks! If you are able please give blood and encourage your friends and family to do so as well! The only way I can stay alive is through the kindness of strangers who donate.
iluvredditalot4 karma2015-11-11 14:50:26 UTC
how much blood you require to survive in a month, week or year?
xxambellina11 karma2015-11-11 14:58:26 UTC
I get blood and IVIG (immunoglobulin). I have blood tests weekly and if my methemoglobin is over a certain point (and I'm usually looking a little blue/grey by this point) then I will have a blood transfusion. This happens every 3-4 weeks during a bad time or every 6-8 weeks during a good time. The IVIG I get regularly every 4-5 weeks depending on scheduling.
iluvredditalot4 karma2015-11-11 15:15:32 UTC
Wish you a good health.
xxambellina2 karma2015-11-11 15:18:38 UTC
Thank you very much!
Dr_Jerkface11 karma2015-11-11 14:49:03 UTC
Was that Eiffel 65 song about you?
xxambellina11 karma2015-11-11 14:53:05 UTC
Unfortunately, no. I was diagnosed after the song came out. However, I quite enjoy playing it on my cell phone when I'm in triage at the ER for an episode. Sometimes the doctors lose their minds because it's a bit scary (my pulse oxygen gets deathly low very rapidly and because they have never treated or usually even heard of someone like me they have to rely on my instructions to save my life) and I try to lighten the mood for everyone.
SilentShitter9 karma2015-11-11 14:44:21 UTC
Were/are you able to attend school or hold a stable job?
BTW: you're still beautiful!
xxambellina16 karma2015-11-11 14:50:21 UTC
Thank you! Though those photos were taken in haste after a long day, haha.
Luckily when the disorder really picked up and started to interfere with my everyday life, I was finish with my college studies. However, I have not worked for about three years because hospitalizations have been so frequent and for such long stretches at a time.
I have recently come into contact with an expert at Duke (even some very amazing hematologists know nothing about it) who has been helping me a great deal. With regular transfusions and daily supplements the disorder is becoming more controlled. In the past year I have only spent 11 days in the hospital as opposed to 54 two years before. I should be able to return to work soon. In the meantime, I am doing independent copy writing online.
xubax8 karma2015-11-11 18:09:51 UTC
Is it possible you're descended from Appalachian people? I remember reading an article years ago about the blue people of Appalachia who were treated with methylene blue.
xxambellina4 karma2015-11-11 18:20:44 UTC
You are referring to "the Blue Fugates." They are Appalachian people. However, I was adopted from Ireland so it is thought that I might be a descendant of "the Blue Men of Lurgan." Right now my genetics are waiting to be checked to see if they can figure out where I came from, but it was likely not related to the Blue Fugates.
Oznog993 karma2015-11-11 20:41:40 UTC
The founding for the Blue Fugate trait was supposed to be a French-born immigrant man, along with a wife of indeterminate nationality and another local clan bearing the gene.
It's possible that the wife or clan came from Irish roots, connecting your issue with them. Genetic testing would show if it's the same genetic defect or just an unrelated defect with coincidentally similar symptoms.
xxambellina3 karma2015-11-11 20:55:53 UTC
That is what they are working on right now. The thing is-- right now there is only one place in the world working on it, and they have other things they have to do as well. Like I said, having come from Ireland I was probably related to the Blue Men of Lurgan-- but we won't know anything for a while.
Oznog992 karma2015-11-11 21:14:41 UTC
Well I'm hypothesizing that the Blue Fugates might simply by the Blue Men of Lurgan gene occurring in a different geographical location, Fugates inhering the Lurgan gene from a common ancestry.
xxambellina2 karma2015-11-12 14:21:04 UTC
I believe that one time when I was hospitalized a doctor who didn't have anything to do with blood but had studied methemoglobinemia because he found it interesting (he was SO EXCITED when he heard I was in his hospital and so happy to talk to me).. anyway, he said something about the connection between the Irish and the Fugates, something like the Irish people were hiding from some King and went to America and "Hid in the hills" and brought it over--they lived so far from civilization because they were afraid of being found, thus the inbreeding.
I'm kind of iffy about exactly what he told me because I was very ill and in renal failure when he was talking to me. I'm not sure how he found all of that out or if it was true, but he knew a ton about the disorder and history so I'm sure there was something to it. Unfortunately for me, I was incredibly drugged and sick at the time.
However, I'm sure I will see him again because he works in the kidney department at the hospital and knows my name. Unfortunately for me I will definitely be back and most likely speak to him again.
headharlot8 karma2015-11-11 14:58:21 UTC
How do you treat your methemoglobinemia? I'm imagining regular blood transfusions or something. I hope it's more simple than that!
xxambellina10 karma2015-11-11 15:10:05 UTC
I get regular blood transfusions (on average about 2 pints every 4-6 weeks). I also have my blood checked 1-2 times a week depending on how I'm feeling. I take ferrous sulfate to put the "good" iron in my blood. People with methemoglobinemia make ferric iron, each one of us is different, but mine requires transfusions of blood that has ferrous iron. Unfortunately, as soon as the blood hits my blood, my crappy blood gets to work converting the good ferrous iron into the bad ferric iron.
I also take high doses of vitamin C. This is for no other purpose than cosmetic. It just makes it so I have a "normal" complexion instead of a pale blue-grey one.
The disorder also contributes to high blood pressure and vitamin d deficiency so I take blood pressure medication and vitamin D as well.
Besides all of that there is a very long list of things that raise methemoglobin levels that I have to avoid, and since I have such great luck-- I'm always finding new things that raise methemoglobin that are not yet documented by modern medicine. The best one they've found through me so far was a spray tan.
dodomontecaz5 karma2015-11-11 14:08:36 UTC
Do you feel like an Avatar character sometimes?
xxambellina14 karma2015-11-11 14:21:08 UTC
I can't really say I've ever felt like an Avatar character, but I have strongly considered discontinuing my vitamin C therapy around Halloween and dressing as Neytiri.
dodomontecaz2 karma2015-11-11 14:22:45 UTC
That's kinda cool. Hope this doesn't really affect your life. May you always be happy. :)
xxambellina1 karma2015-11-11 15:24:19 UTC
Canukistani5 karma2015-11-11 17:46:32 UTC
are you a vampire?
xxambellina16 karma2015-11-11 17:50:38 UTC
Well, I'm a ginger and if I go in the sun without heavy sunscreen I practically light on fire-- and I need other people's blood to survive, so maybe? The thought has crossed my mind.
alllysone3 karma2015-11-11 15:18:28 UTC
What's your favourite band?
All the best to you! Hope you stay healthy and happy :)
xxambellina7 karma2015-11-11 15:23:16 UTC
Thank you so much for the good wishes!
I don't really have a favorite band and I like a lot of different music. Pop punk, hardcore, hip hop- I'm versatile. My favorite band to jam to or to listen to so I cheer up is Fall Out Boy (how lame am I, right?).. but I love: Jay Z, underoath, blood brothers, coheed & cambria, thursday, boys night out, atreyu, norma jean, silverstein, boy sets fire, and a ton more. I love music!
smhdraper094 karma2015-11-11 16:10:59 UTC
Listen. Fall Out Boy is my guilty pleasure band, too. It's alright. What's your favorite track by them? I would have to say mine is Coffee's for Closers (on Folie a Deux)
xxambellina7 karma2015-11-11 16:30:25 UTC
I used to go old school with the original grand theft auto (I'm old as fuck, I was a freshman in college when Take This to your Grave came out so I have a lot of good memories from that album), but I'm also kind of obsessed with Young Volcanoes.
Zykatious3 karma2015-11-11 16:43:28 UTC
Do you ever get compared to Smurfette?
xxambellina7 karma2015-11-11 16:56:20 UTC
ALL THE TIME! I've even had a few guys see me blue on instagram and tell me that they think I'm sexy because they have a thing for Smurfette. I get her, the avatar girl, and the blue man group all the time. I'm tempted to stop my vitamin C before halloween one year so I can go as something blue!
blunt_monger3 karma2015-11-11 20:12:12 UTC
Are you happy in general with your life? Or has this caused you to experience depression/anxiety etc? I can't imagine going through all the stressful things you've described in your post.
Stay strong, and thank you so much for answering our questions so in depth!
xxambellina6 karma2015-11-11 20:53:30 UTC
You're very welcome for answering the questions! I just wanted people to have a little peak into what it's like to be.. blue.
I have obviously always had this disorder, but it was never super bad or caused the crazy attacks that kill you until a few years ago. When it all started happening I was very depressed and anxious. It's totally gotten better since then, but honestly- I live life with incredible anxiety that I hide from everyone- including my husband.. and it certainly takes a toll..
For one thing I'm always looking in mirriors, the mirrors in places I'm at, the mirror in my purse, my selfie camera. I look vain as shit, but I'm actually just inspecting myself for hints of blue or purple. And then when I do have a reaction and go to the hospital I'm super kind (like I'm the favorite patient in the local ICU and doctors and nurses fight over who gets me), I never demand anything and I joke with everyone.. even when I'm on death's door.
Then when I'm alone in my ICU room hooked up to every monitor that ever existed with a bag of blood going into the port that is under my skin on my chest that I'll have to have for the rest of my life... just thinking like "man, 3 hours ago my methemoglobin was 70%" and that's way beyond where I should die.. Then the last time I went into kidney failure.
I get... I don't know, sad, mad... I throw myself a pity party and scream into my pillow and punch it until I feel better. When I sit down and think about it, it really is a lot to take.. and the idiopathic part of it-- it's like I can avoid everything under the sun but sometimes it just happens and they don't know what causes it. So like, I know it's going to happen again, and again, and again. And I know that it will probably eventually kill me.
I just try not to think about that part and be happy. I have an amazing husband who married me even though my health was a shit show and we have a beautiful (not blue) daughter-- even though every doctor told me I would NEVER carry a baby to term. Plus like, sometimes you can put it out of your head and just have amazing days. I also get my blood and IVIG at the cancer center and see people who are literally just dying in front of me.. and I am thankful that all I have is methemoglobinemia. The pity parties are short, well deserved, and help-- but only occasionally.
One time when I almost died and was in ICU my friend started #blueformeg and had people wear blue and take a picture to send me. Over 300 people did it. It was so so so so touching, so when things look extremely bad or something really scary happens, I look at those photos (I saved every single one). Then I see how many people are cheering for me and how lucky I really am.
long winded, but it's pretty complicated.
yes I am happy in general with my life.
aryst0krat3 karma2015-11-11 17:24:52 UTC
So is the only treatment available to you blood transfusions and the supplements you take? I don't know much about genetic disorders, but is there any progress toward a 'cure'?
xxambellina5 karma2015-11-11 17:36:06 UTC
There are only 111 genetically confirmed cases of methemoglobinemia and the instance of it in the population is unknown as it is incredibly low (like where medical documents say a disorder occurs 1 out of every 300,000 births or whatever, we don't have that for methemoglobinemia). Also, the people who have it tend to be in very isolated populations, like the deep mountains of Kentucky and a tiny farming village in rural Ireland- so they don't get a lot of medical care by places that could study them. essentially it means it occurs in populations that have inbreeding (great, I'm inbred), and as the populations of these tiny places become more connected there are less babies born with it..
So basically- there are so few of us it isn't really studied. There is a hematologist at Duke who I see that studies it and there is only one place that does the genetic typing for it, which is located in Amsterdam. We know very little- but I go to Duke a lot and give my blood and go through test to try to help it be studied.
The only known treatment is blood and methylene blue- even some of the supplements are like, "they may help a little- but we don't know." It's also important to stay away from known triggers like certain dyes and photo developing chemicals. I really have to do my homework handling like, glues, paints, cleaners, etc. to prevent my methemoglobin rising to deadly levels.
Nope, no cure. No one really cares because it's so rare. So far just blood and methlyene blue help.
aryst0krat3 karma2015-11-11 18:16:52 UTC
My friend is some sort of fancy blood doctor. Or will be, anyway - he's currently doing his PhD after bypassing his Masters because he's a smartypants. I will tell him he should cure this for his thesis. :P
xxambellina4 karma2015-11-11 18:24:18 UTC
Haha, I totally approve! xoxoxo
grizzlyking3 karma2015-11-11 17:14:40 UTC
What are the best and worst jokes about your blueness you have heard?
xxambellina9 karma2015-11-11 17:26:40 UTC
The worst jokes are definitely:
Singing or referring to the eiffel 65 blue song. (I've played it in triage as a joke to relax everyone- but I feel like I have that right.)
Anything about Willy Wonka, ie: "Violet, you're turning violet!!"
Comparing me to Smurfette.
Asking if I'm in/ going to join The Blue Man Group.
The best was actually pretty bad, but I thought it was creative and I did laugh my ass off..
I was at a high school football game and it was super cold and some guy said "If you're a blue person, we should make purple," because his face was red from the cold.
Jvillacorta33 karma2015-11-11 15:05:04 UTC
Is there any interesting stories that you can share to us?
xxambellina19 karma2015-11-11 15:17:32 UTC
I have a ton of stories but here's one of my favorites:
I got a message through instagram from a girl who was from America studying in India. She was feeling unwell and her lips were blue so she was trying to google what was wrong with her. She found me and sent me a photo and asked if I knew what it was since the hospital by her was REALLY bad she didn't really want to go.
It turns out she was on an antibiotic that was a known cause of methemoglobinemia episodes (anyone can have an episode one time and not have the actual disorder like I do). I was able to tell her I thought she was suffering from methemoglobinemia and that she needed methylene blue.
She was able to go to the hospital and be transferred to another facility that had the medicine she needed. She ended up being okay and thanked me for saving her.
Jvillacorta35 karma2015-11-11 15:29:25 UTC
Correct me if i'm wrong, Does that mean that she had the disorder but it was prevented due to discovering it at an early stage and having the medicines that she needed?
xxambellina11 karma2015-11-11 15:41:44 UTC
Some people have idiopathic familial methemoglobinemia, like me. That means we have a recessive genetic disorder that is present in our bodies at all times.
However, there is something called "aquired methemoglobinmia." This happens to people who DO NOT have the disorder, but have some into something in their environment that raises methemoglobin levels in the blood and causes them to experience the effects of methemoglobinemia the genetics deal with every day. It will generally be a one time thing from an outside factor (like too much of some sulfa-drugs, certain dyes, photo developing chemicals). When this happens they will be treated with methylene blue just like me, but it's a fluke and will probably never happen to them again. The number of cases of acquired methemoglobinemia are extremely low.
zasto2 karma2015-11-11 15:35:10 UTC
Do you have any memories from the two times you were clinically dead, or from coming out of it?
Wishing you good health, by the way. I've never donated blood before, but I always see blood drives at my college. Now I want to do it next time
Take care <3
xxambellina7 karma2015-11-11 15:50:19 UTC
The first time I died I remember going into the hospital and them rushing me right to the back. I was in a huge room with a big light in my face on oxygen and I watched my levels drop from like 74, to 68, to 63, etc. I knew it was serious from that and from how many people were in the room and running around.
I remember telling my friend to call my parents (they were on vacation and I previously told him not to bother them and wreck their good time) and to tell everyone that I love them so much. Then the doctor told me I was going under. I didn't see a white light or hear a God or anything but it was extremely peaceful. I knew what was happening and I pretty calmly explained it, I only shed a few tears when I told my friend goodbye and asked him to tell my family and friends I loved them. I went from panicking about how serious I must be to have so many doctors and nurses and having such a love oxygen level to feeling at peace.
after that I have a lot of weird dreams and experiences from being in the coma. It was medically induced but I do not respond well to anesthetic and I kept coming out of the coma trying to rip out the ventilator and sometimes writing on a white board asking what happened to me (the drugs they give you to keep you in the medical come cause amnesia).
The second time I was so incredibly far gone when I went in I was just yelling gibberish about shooting some arrows into the closet and telling them that I was fine and I shouldn't drink so much juice because it makes you blue. I. was. GONE.
I don't know what happened when my heart stopped and I only started to remember things during the coma. Weird dreams that incorporated things people sitting with me were saying and stuff, and a lot of waking up and having no clue where I was or how I got there. It was a lot less scary than the first time because I wasn't together enough to realize I was going to die- and when I woke up I wasn't in a plastic tent with people in protective gear around like you see in the pandemic movies.
xxambellina7 karma2015-11-11 15:56:52 UTC
and thank you so much for the well-wishes and for donating blood! The IVIG that keeps my immune system working well takes the blood from thousands of donors to make one dose, and the pints of blood I get literally save my life.
I know the needle isn't very much fun.. but the donation you make isn't arbitrary, it actually does save lives. I try to put a human face on what the gift of blood actually does for the people who receive it.
Without donations from people like you I would be dead and I would have never been able to carry my beautiful daughter to term.
RenaKunisaki1 karma2015-11-11 22:04:44 UTC
Will your daughter have the same disorder?
xxambellina2 karma2015-11-12 14:32:35 UTC
My pregnancy was not planning (shout out to the nuva ring :(), I was going to adopt children because I had a lot of fear about my disorder. I was first told that there was NO WAY I had the full blown disorder (by doctors who really didn't understand methemoglobinemia). They said that I would have been a blue baby (which we don't know- my parents got me at around 5 months of age and we know nothing about my birth) and that I would have had problems and been blue my entire life. They also told me that I HAD a half-marker for methemoglobinemia- thus all the reactions.
I was terrified if I had a child, they would have a 50% chance to have the half marker and would be sick like me. Then I finally found an expert (which is honestly a bit ridiculous I had to go find my doctor when she is only 3 hours away from where I live and is the leading expert on my disorder.) who explained to me that people with a half-marker do not experience any symptoms what so ever. They are as likely to have a methemoglobinemic episode as any other member of the public without the genetic disorder. She then went through all of the blood work she could find up to 23 years ago when I had my tonsils out and found that I have always carried WAY more methemoglobin in my blood, but no one ever pointed it out or said anything. The only indication we had of it in my early life was I would be grey sometimes when I felt ill. My mum used to say that instead of going green around the gills when I was sick, I was grey around the gills.
So, they tested me at the one place in the world that tests for the disorder and sure enough, I have two recessive markers for it and the doctors and geneticists saying I had a half-marker were totally uninformed (I was under the impression they had tested my blood and that's how they knew, not that they made a wild guess).
That actually turned out to be good news, because although my daughter does carry the half marker, she will never: have any effects from the disorder, will never need blood, will never be sick like me. The only way this disorder will impact her life is that when she has children she will have to get a genetic consult to make sure it doesn't run in her partner's family. The odds of her meeting another carrier, having a child, and having that child get a marker from both parents is so astronomical it's ridiculous. She is a perfect, healthy little girl and will never have to worry about my genetics hurting her.
Since my husband does not carry the recessive trait for methemoglobinemia, my daughter and any future children will be unaffected carriers of the disorder.
zasto2 karma2015-11-11 16:04:53 UTC
That's all so crazy. Do you know any of the people you were sitting and talking to? Do you remember what.you were talking about?
Do you remember the moments leading up to waking up from anethesia/comas? I read from someone else who was in an 11 year coma that they remember before coming out of it, they were running through this path filled with sky high black, dripping shreds of curtain and she kept running forward and pushing the heavy curtains aside.
xxambellina3 karma2015-11-11 16:27:44 UTC
I don't remember a lot from the first time. Like some people I thought were with me for a couple minutes and then when I was fully awake (which doesn't happen until like two days after you wake up and the super strong drugs are out of your system) I found out they were there for days at a time.
I had one dream where I was in a big cathedral and I was hearing music playing- I later found out that my mom had called me and played music while they held the phone to my ear so i could here her voice, so it was probably that.
The only other thing I really remember was dreaming that I was wandering the hospital in a gown with an IV pole, but there wasn't anyone there. I just kept looking in rooms and there was nothing. I also remember trying to find the doctors and nurses to tell them thank you for saving me, so I must have realized I wasn't dead. Turned out that while I was in the coma I would come out a little, not fully awake, and try to pull out my ventilator. While they were waiting for someone to approve more drugs to put me back under they would settle me down and give me a white board. Apparently every single time they did- I didn't remember doing anything before. I would thank every person I saw by writing on the white board--- like individually, each and every time I woke up. I also used the 1-10 pain chart smiley faces and numbers and drew them for the doctors and nurses (the ventilator hurt.) I still have a little collection of pictures of the semi-conscious smiley faces and numbers.
I guess once the tube was out of my throat and the drugs were leaving my body I talked to a ton of people on the phone and had so many visitors the ICU had to send people to a different waiting room. I talked to them for the entire 20-40 minutes the doctors would allow and I don't remember any of it. Those drugs are no joke!
The second time I just had this weird- like almost vision- of my family and friends sitting around the table eating. The table was covered with a ton of food and I wanted to eat it all, but I never managed to actually eat anything. Turns out that when I woke up I would thank everyone (I guess my parents raised me with some serious manners that come out even in a coma) and then ask for food because I was really hungry.
That's really all I've got. I was getting 3-4 times the does most people get to be under and still pulling out of it, since the drugs cause memory loss at normal levels, my memory was pretty much wiped with the doses I was getting.
Mcfinley1 karma2015-11-11 18:15:57 UTC
What's it like to hang out with Tobias?
xxambellina2 karma2015-11-11 18:22:45 UTC
I quite sincerely wish I could tell you. I would totally love to hang out with Tobias, but alas I am not blue enough for the Blue Man Group, and I can't paint myself blue.
taytermuffin1 karma2015-11-11 17:47:46 UTC
So what causes your disorder? Allergies to things? And what is methylene blue?
xxambellina1 karma2015-11-11 18:06:21 UTC
This is from the NIH, pros can explain it better that I can:
Methemoglobinemia is a blood disorder in which an abnormal amount of methemoglobin -- a form of hemoglobin -- is produced. Hemoglobin is the protein in red blood cells that carries and distributes oxygen to the body.
With methemoglobinemia, the hemoglobin can carry oxygen but is unable to release it effectively to body tissues.
Methemoglobinemia can be treated with supplemental oxygen and methylene blue 1% solution (10 mg/ml) 1 to 2 mg/kg administered intravenously slowly over five minutes. Although the response is usually rapid, the dose may be repeated in one hour if the level of methemoglobin is still high one hour after the initial infusion.
Methylene blue restores the iron in hemoglobin to its normal (reduced) oxygen-carrying state
With me, basically I carry a way higher level of methemoglobin than normal people. When I come in contact with something that raises it a little (like if a normal person comes in contact with someone that raises their methemoglobin 4% they won't notice it) it can kill me.
They treat things that raise my methemoglobin as "allergies" in my medical records to make sure I'm never around or given them in the hospital, and when I'm there they have a list of everything (it's long) that can raise my levels on the door with a warning.
They aren't truly an allergy and they don't actually cause anaphylaxis (like if i get stung by a bee I have anaphylaxis and an epi-pen fixes it), epinephrine doesn't fix it. They just don't have a way to list everything that goes along with the methemoglobinemia at most hospitals. Luckily the large local hospital has seen me so many times they know the deal.
here is the wiki leak that sciences pretty hard:
Canukistani1 karma2015-11-11 17:49:05 UTC
what is methemoglobinemia?
xxambellina1 karma2015-11-11 18:07:35 UTC
It's basically a double recessive genetic disorder that screws with your blood.
Here is the wiki link:
ranchdepressing1 karma2015-11-11 17:51:33 UTC
Did you enjoy Big Fat Liar?
xxambellina3 karma2015-11-11 18:08:20 UTC
I have never seen Big Fat Liar but people frequently refer to it when they talk to me. I should probably watch it sometime so I know what the hell everyone is talking about. I'm assuming someone is or turns blue?
MeNoGivaRatzAzz1 karma2015-11-12 13:07:55 UTC
Yes, Jason (Frankie Muniz) and Kaylee (Amanda Bynes) pour dye into the swimming pool, turning Marty (Paul Giamatti) blue in the process. It has nothing to do with methemoglobinemia. I don't know why anyone would ask this, other than immaturity. In fact, I feel a bit uncomfortable with this reply.
xxambellina1 karma2015-11-12 15:13:41 UTC
Hahahaha, that's funny and I'm glad I finally understand why people always ask me about Big Fat Liar! Thank you!
You don't have to feel uncomfortable. I think that those kind of remarks are like half immaturity, but the other half are maybe nervous or uncomfortable or don't know what to say so they figure if they say something about the Blue Man Group, Smurfette, Big Fat Liar, that Eiffel 65 song- oh whatever else, it might break the ice and we can have a laugh.
I really don't mind. I understand that when I'm blue I'm super unusual, and kind of scary-- usually people who are blue are dead or oxygen deprived and dying, so seeing someone walking around like that (even in a hospital) is like "HOLY SHIT DID YOU SEE THAT?!"
Normally if people make blue jokes or stare at me I just talk to them about it, so they see that I'm a normal person just like them. This is also the reason that when my daughter gets older and starts asking questions about people with visible disabilities, I'm going to take her up to them so she can ask questions and understand that they are just normal people like everyone else.
GregSparks1 karma2015-11-11 16:56:13 UTC
You said that it killed you twice, did you see an afterlife?
xxambellina3 karma2015-11-11 17:05:36 UTC
I did not. The drugs that they gave me after I died to keep me in a coma while I was on the ventilator cause amnesia so I don't remember a lot.
The thing I do remember from the first time it happened was cool though. I knew I was dying because like 20 doctors and nurses were freaking out running around me and the pulse oxygen monitor was right in front of me so I could see the oxygen level dropping. When that happened, I had my friend who was with me up until I died and they had to resuscitate me holding my hand, I just remember stopping freaking out and an overwhelming sense of peace and serenity coming over me. I told him to call my parents and tell them I love them and to tell "everyone" that I love them so so much. Those were my last words. I cried two or three tears but they were like happy thinking about how many great people were in my life-- and I was happy that I got to choose my last words.
It was very strange, no white light or religious experience (that I can remember) but it was like something greater than myself was there walking me through the process and making me feel peaceful and accepting of what was going to happen.
The second time my pulse oxygen was so low I was absolutely out of my mind (kind of like an uncontrollable super wasted drunk person). I was told I was screaming about arrows in the closet and I was going to shoot arrows into the closet. I also thought that I wasn't having methemoglobinemia (even though it was very obvious I was and I'd had it before). I was yelling at the doctors that they didn't need to do anything because I was blue from drinking too much cranberry juice the day before- then everything went black. I think my brain shut off long before I died that time.
stephenchuk1 karma2015-11-11 19:29:55 UTC
Is it uncomfortable going into public because people might give you weird looks or stare?
xxambellina1 karma2015-11-11 20:24:33 UTC
What's really amazing is that with vitamin c therapy I don't have to be blue all the time. I only get blue when my methemglobin gets extremely high or when I've been particularly neglectful of taking my vitamin c.
That being said, my arms are covered in tattoos and I have big plugs in my ears while living in the bible belt, so I'm used to some stares. If it's a little kid or someone who is REALLY staring me down I'll go up to them and ask if they'd like to learn about my disorder. Normally people are receptive and kind. I don't blame people for staring because that's a natural reaction to seeing something like a blue girl.
truthsyoudontlike1 karma2015-11-11 20:21:37 UTC
Will you please provide a picture of yourself when you are blue?
Also, fuck this disease. If I could make it different...I would. :)
xxambellina2 karma2015-11-11 20:53:55 UTC
also, thank you.
sincerely, fuck this disease.
xxambellina1 karma2015-11-11 20:39:43 UTC
There is one up top where I'm slightly blue. I don't get super blue not that I'm on vitamin C therapy. I'll go digging around facebook and instagram tonight to see if I can find some more blue ones. I don't really love getting my photo taken when I'm super blue, I'm sure you can imagine. But yeah, I'll find some of the crazier ones this evening.
truthsyoudontlike1 karma2015-11-11 20:47:39 UTC
Thank you very kindly. :)
xxambellina1 karma2015-11-12 14:16:17 UTC
I uploaded a few to tumblr, but I'm not a regular user, and they just changed the format... I can figure out how to get the links. I'll work on that or see if I can find someone to help me.
xxambellina1 karma2015-11-12 17:13:19 UTC
Here are some that were taken when I am starting to go blue, it starts at my fingernails, toenails, under my eyes, and my lips. Then it proceeds to turn my whole face a faint blue.
while this is happening my legs start to mottle; One also shows how easily bruises show on me because my blood is brown/black.
Then I get a light blue- which is the color I have all the time without vitamin C therapy.
I used to have a PICC line, and it SUCKED.
so I got a power port put in my chest to get blood and methylene blue as quickly as possible in a crisis.
I don't really like the scar, but it's worth it.
Getting the medicine is a little weird because in order to stop being blue the put something bright blue in me.
Because it can flare back up even worse after the methylene blue, I always have to stay in the critical care ICU- where I'm hooked up to every machine known to man and it's impossible to sleep.
and after methlyene blue I pee a crazy color for a few days...
but the medicine works super fast. This photo is only an hour after my methemoglobin was a deadly 62%
truthsyoudontlike2 karma2015-11-12 17:14:56 UTC
Wow, this so generous. Thank you. :)
xxambellina2 karma2015-11-12 17:16:37 UTC
it's no problem- I don't have photos of the super blue because at that point I'm worrying about like.. not dying, haha. I was iffy if I should include the pee!
wadaball1 karma2015-11-12 01:16:34 UTC
If you were green, would you die?
xxambellina1 karma2015-11-12 14:35:33 UTC
I can't tell you how many people ask me this. I'll tell you the same thing I tell everyone else..
If I were green I would go to the hospital and hope they could save me like the do when I turn very blue.
InTupacWeTrust1 karma2015-11-12 04:00:07 UTC
Wow, that sounds scary! How are you feeling on those blue skin days and have you ever seen the blue guys? We were going to see them, but at 300 a pop we said no thanks
xxambellina1 karma2015-11-12 14:53:51 UTC
I actually have seen the Blue Man Group. I went with my school and we got to meet the blue guys. However, at the time my skin was normal (as normal as super pale, covered in freckles, ginger skin can be) and I didn't know I was a blue person (adoption is a hell of a thing sometimes).
On the blue skin days it really depends. If I have been taking my vitamin C like I should (and I'll admit sometimes I'm really really bad at taking it since it's only cosmetic, doesn't affect my health, and I have to take it four times a day and will just forget) and I am even slightly blue I'm usually very bad off.
I usually notice the change after waking up in the morning. The first thing I do is always go to the bathroom to pee and brush my teeth. I will walk to the bathroom (12-15 feet) and by the time I get there I am super out of breath like I've run a mile and my heart is pounding out of my chest. That's my cue that something isn't right, so I check out my nail beds and look in the mirror. I will usually find a blue color, send my husband or a friend some photos, and get the ball rolling to go to the hospital.
On those days I feel a lot of anxiety about what will happen. There is always a chance the doctors won't take my word for what is going on and I won't get methylene blue and blood soon enough. I also can only walk a few feet before sitting down because I can't catch my breath-- a lot of websites about methemoglobinemia say we need oxygen therapy and that's bullshit-- It doesn't matter how much oxygen (that forced oxygen mask is my mortal enemy) or how many inhalers or breathing treatments I get, I can't absorb the oxygen so I just flat out can barely breath. I'm sure you can imagine how terrifying that is. The other thing that happens is I get a terrible headache. Just monstrously horrible worst migraine in the world. Luckily I am usually at my local hospital, and after freaking out the girl at the emergency desk by being bright blue in color-- the doctors know who I am and rush me in. They call the expert at Duke and she dictates all of my care to them.
On true blue days I feel very anxious, I cannot breath, and I have a massive headache. And yes, I have seen the blue guys.
electrithm1 karma2015-11-12 04:38:31 UTC
Has anyone ever been "racist" to you?
xxambellina1 karma2015-11-12 14:54:41 UTC
Even while I am blue I have that whole white privilege thing going on. I've never experienced anything nearing racism because I'm blue.
BlueLightSpcl1 karma2015-11-12 15:53:49 UTC
What's your favorite type of cake?
xxambellina1 karma2015-11-12 17:17:44 UTC
I'm kind of asking myself how one can choose a favorite kind of cake-- I think I might be cheating with this one, but it's totally cheesecake.
Mcgrathc21 karma2015-11-13 05:52:05 UTC
Have you heard of Vivien Thomas? I read an article about the man who first developed surgery for blue babies at Johns Hopkins. Actually, I taught the article to my 7th grade students today.
xxambellina1 karma2015-11-13 15:57:11 UTC
I actually have not! Once I found out my husband was not a carrier of the defective gene- and therefore our children could not possibly have the condition I didn't do any research on anything involving babies/children with the disorder. I've been in a little bubble of doing research that applied to my particular case. Thank you for letting me know about it, though! I will do some looking around about Vivien Thomas today, I really appreciate the tip!
AlHadeed0 karma2015-11-12 20:47:06 UTC
uhh, wheres the blue skin? all i see is just sickly pale skin.
xxambellina1 karma2015-11-13 16:00:40 UTC
As I said, because of vitamin C therapy I only get a light blue hue. Anytime I have had the dark blue coloring since the vitamin C has been a life-threatening medical emergency and I am too sick/out of my mind from low oxygen to think about photos. Some of those photos you can only see the blue under my eyes and in my lips, but there are definitely one or two where my whole body is a pale blue- so I'm not sure what you're looking at.
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