Secret Benefits to Thalassemia, Round 2: Email Inquiry

I get emails from people asking for me to diagnose them based on images of maggots that they found on their skin, a peculiar rash they've had for years, or something they found in their poop. I have to say, I love these emails. If anyone is worried about the NSA spying on their email activity, it should probably be me, because my inbox is full of stuff that could be considered private medical information, and I am not an MD.

But sometimes I get legitimate questions, like this one that I featured on my blog forever ago (don't worry, I always ask if they mind if I post their questions). Recently, I got a really interesting and smart question from an anonymous reader. The email said:

Dear Doc,  is there an immunity of reduction of Ebola with people who have beta-thalassemia? Or is it just the opposite, a more susceptible case for those with beta-thalassemia?

If you don't remember what beta-thalassemia is, or why it might be "beneficial" (I say this loosely, as it's not necessarily a good thing to have), feel free to brush up by reading this old post about it and it's relationship with malaria.

Image from the American Society of Hematology

To recap, thalassemia is a disease associated with globin gene mutations (both α- and β-globin genes can be mutated, yet α-globin mutations are most common). Since your α- or β-globin gene is recessively mutated if you have active Thalassemia, your red blood cells develop into malformed, or "sickled", cells, thus inhibiting their affinity to bind and transport oxygen (see image above for a great example). This lower affinity can cause extreme cases of anemia. In major cases, treatments such as chronic blood transfusion therapy, splenectomy, transplantation and iron supplementation are used. Thalassemia would make a great potential candidate for genome editing therapies that are still in the research stage (yes, I know that article is about hemophilia, but it's a good example).

So, why is this anonymous question a great one? Simply put, it doesn't really have an answer. There isn't any research being done to look into populations that aren't as affected by Ebola right now, as far as my searches have illustrated. This might be because most research that's being done is to either understand the physiology of the virus, to identify all possible vectors (yes, most evidence point to bats as the vector, but I'm still not convinced), or to come up with therapies/treatments and vaccines.

But the submitted question addresses an important point: thalassemia, specifically β-thalassemia is really prevalent in Nigeria (25% of the population are carriers for Sickle Cell Disease, while another 25% is hemizygous for the G6PD gene), and other parts of Africa. So, why isn't this being looked into? I can't answer that. I don't know. All I can say is maybe it isn't yet. I'm sure now that Ebola is in the spotlight, there will be a new emphasis on funding down the line for exploratory research.

In my fervent literature search to find an answer for the anonymous email, I did find that similar research has been done to look at the effects of thalassemia on hemorrhagic fevers (specifically Dengue Hemorrhagic Fever), mostly in Thailand and Southeast Asian countries. It turns out that patients with thalassemia don't necessarily have any higher risk, or even less of a risk (as seen with malaria), of getting hemorrhagic fevers, as my anonymous email inquiry asked. But, they do present differently, meaning their symptoms may seem different than infected people without thalassemia. Most normal (non-thalassemia) patients show signs of hemoconcentration (or an increase of solids in the blood), but patients with thalassemia present anemia (which is the opposite). This is the main symptom that presents differently, but its a really important one. Other symptoms also reference changes in AST and ALT levels, which illustrate the concentration of enzyme in the liver, heart and muscle cells, and can indicate liver function.

Hemoconcentration is really important during the diagnostic phase, and to track how the infection is progressing. So, if, instead, your patient is presenting with the exact opposite, it might lead to misdiagnosis. Also, if a patient with thalassemia, who is also infected with dengue hemorrhagic fever, is showing anemia, it needs to be watched. The three papers I linked to above talk about new suggested courses of action for patients with thalassemia, like frequent blood transfusions.

So, anonymous emailer, the answer is probably not. The biggest risk that thalassemia would give you is misrepresenting symptoms, which can lead to improper care. Yes, that can be really dangerous, especially with hemorrhagic fevers. But, luckily, if you have a high fever and other viral (standard) infection symptoms, your doctor will usually run other diagnostic tests (like an ELISA or PCR, etc.). That's why they always ask you questions about where you've been, what you've been in contact with, and other patient history questions.

This is definitely research that should be done, even if it starts with a retroactive epidemiology study.

Thanks for your question!

If any of you have other questions, feel free to email me at microscopic.blog@gmail.com!

Clearing the Mass Miasma

I’ve had a number of inquiries about the Ebola outbreak, whether for clarification (“what is Ebola?” or “should I be scared?”) or desperation (“I don’t know what to believe anymore!”). Let me start by saying that it’s OK to be confused or a little nervous. The media has done a great job at reporting on this terrible and unfortunate situation, but has done an even better job at making people nervous or question their ability to prepare for such a situation in their own town.

It’s always been a little aggravating to me that most science reporters or science writers don’t have a science background. There are a lot of benefits to that, such as their vernacular may not be jargon-heavy, or they might be able to cover a wide range of topics on a broad, elementary level. But, there are also a lot of writers that are feeding into the media-based hysteria to get more clicks. How do you get people to read your stuff? Scare the crap out of them.

You really can’t escape it. I’ve seen posts on Facebook to articles that detail inaccurate methods of Ebola transmission (thanks, “friends”…), and I even heard about it on the radio. Whenever I drive into work, I usually have the radio on so I can get traffic updates. A week ago, one of the personalities on a stupid morning show was talking about the isolated testing for Ebola that was happening in Sacramento (which actually happened for some missionaries, that much is true), and it really made me sad, because it solidified how little people feel they need to research the stuff they read online. Everything the deejay said was inaccurate, and all I could think about was the hundreds (thousands maybe?) of people listening at that moment...and believing everything.

But I’m not writing this to call people stupid, or make anyone feel bad about their news choices. Instead, I’m writing to give you the facts of what I’ve gathered so far. I’m not on the front lines, and I may not be a popular blogger or writer for Jezebel or Huffington Post, so I’ll it up to you whether I’m contributing to they nonsense or not.

So you want to understand Ebola. Where do you start?

If you want to understand the virus itself, you can read some of my previous entries on Ebola.

There are only a few places in the world that are allowed to research and work with any and all strains of the Ebola virus. For a long time, that list was limited to only major governmental agencies (CDC, etc.), especially research agencies that have the capability to work with biosafety level – 4 (BSL-4) pathogens. You can read more about BSLs here, but I’ll break it down for you:

• BSL-1: all microorganisms used are not known to consistently cause disease in healthy adults and present minimal potential hazards to lab members and the environment. A good example of this is non-pathogenic E. coli, which is used in almost every lab that has ever existed. Think everything from entry level biology classes to, well, most other labs.

• BSL-2: In addition to being able to work with BSL-1 organisms, BSL-2s can work with organisms that pose a moderate risk or hazard to lab members and the environment. A good example of this is Staphylococcus aureus.

• BSL-3: This is a restricted environment, as the hazards are increased from BSL-1 and BSL-2. The pathogens or organisms researched in these labs can often cause lethal diseases via respiratory transmission, like Mycobacterium tuberculosis which causes tuberculosis.

• BSL-4: Lab work involves microorganisms that are dangerous and exotic, and may or may not have a defined method of transmission yet. This is where Ebola is researcher. Researchers who work with BSL-4 organisms have to change their clothes before entering the clean room, and must shower and decontaminate all materials before leaving the research space. These researchers have to wear full body, pressurized, and air-supplied suits. This is the solitary confinement of pathogen research.

Image from Frankfurter Rundschau

Get it? It’s incredibly dangerous, and you should thank the researchers that risk their lives to perform the groundbreaking experiments that they do. If you want to read the follow-up report regarding a researcher accidentally becoming exposed to Ebola, you can download that here.

Am I at risk of becoming infected?

Unless you are a health care worker who is working with individuals who are infected and currently experiencing symptoms, your chances of contracting Ebola are slim to none.

With that being said, the only known method of transmission for Ebola is direct contact with the bodily fluids of an infected individual. If you have traveled to Africa recently, and have had contact with someone else’s bodily fluids, you may want to check in with your doctor. Once someone has recovered from the virus, they are typically no longer contagious, but the Ebola virus has been shown to be present in semen for up the 3 months post-infection.

This isn’t like our seasonal flu. Ebola is an incredibly debilitating infection that can have symptoms including high fever, headaches, muscle pain, vomiting and diarrhea, and unexpected bleeding or bruising. There have been some really sad stories about churches, mortuaries, and even entire towns banning traditional funeral and burial practices because it puts otherwise healthy people at risk.

Will the outbreak spread to the US?

The CDC has not indicated any potential risks for the outbreak to spread. Yes, there have been medical professionals and missionaries that have contracted Ebola during this outbreak, and yes, they have been brought back to the US for treatment.

BUT, there is a reason for that. Almost all the major research that has been done to figure out the Ebola virus has been conducted in the US (or in collaboration with the CDC). Experimental treatments are here, better medical facilities with isolated rooms and chambers are here. 

Let me put it this way: As of right now, there are 2,615 cases of Ebola between Guinea, Sierra Leone, Liberia, and Nigeria. There are more than 1 million individuals who are infected with HIV in the US, and 1 in 6 of them are unaware of their infection (typically due to negligence with regular testing). HIV is also spread through direct contact with bodily fluids. But, every time you go into a hospital, you don’t panic about HIV because medical facilities are prepared to handle and minimize these types of risks. Take a deep breath and don’t believe everything Donald Trump says.

What. an. ass.

OK, I believe you about the risk of getting Ebola in the US, but I’m still paranoid and want to track it every day.

That’s fine. Check out this amazing website that will give you a detailed update every time something happens with the outbreak. You can also watch an illustration of the spread of the outbreak since March 14, 2014.

What about treatments? Why aren’t there treatments?

There are currently 3 companies working on developing experimental treatments against the Ebola virus: Tekmira, Biocryst Pharmaceuticals, and Mapp Biopharmaceuticals. All of them are in very early stages of development, meaning none of them have been approved for use in humans. ZMapp is the product that is getting the most media because the experimental monoclonal antibody-based therapy was given to two US patients in Liberia.

Since this experimental therapy has not been approved, it requires each patient to consent, and understand the potential risks associated with experimental, non-approved therapies. Compared to some of the other funded research, Ebola isn't seen as a major threat. Maybe that's because we haven't seen an outbreak this large before, or that it hasn't been a threat to the US yet. Either way, it's getting the attention it needs now to really get some momentum in terms of pushing drug and vaccine development through to (hopefully) a reality.

It is possible for patients to recover and survive an Ebola infection. In order to do this, the patient needs constant monitoring for fluid balance, oxygen and blood pressure, and additional treatments for complicating infections.

This is a lot of information to digest, so I'm going to leave it at that. If you have any other questions that you'd like answered, feel free to let me know!

Ghosts, Goblins & Things That Will Actually Kill You

I love Halloween, which is why I'm sitting at home alone, wearing my festive ghost-print pajama pants, tea in hand, writing this blog entry. As I sit here, eating my unfestive leftovers and unexciting salad, I'm laughing to myself. NPR is posting ghost stories, Buzzfeed posted "10 creepy photos from Halloweens Past", and articles like "13 Real Animals Lifted Directly Out of Your Nightmares" [cracked.com] are actively being read.

So, I'm giggling to myself, pushing my food around in the bowl thinking, "how can I write a blog entry about things that are already halloween-esque?" This is a challenge for me, because I am not a fan of horror films. I know, you'd think someone who dedicates their life to viruses, parasites, and other creepy crawlies would be excited by the thought of paying $12 to go watch two hours of ghouls and scantily-clad ladies screaming... but I'm not.

Then I found this list of the 10 Scariest Horror Movie Characters (also on Buzzfeed, which is oddly addicting if you avoid all the celebrity gossip and pseudo health advice). While I have only seen one of these movies, I think I can match all of these horror movie characters with real horror. So here we go...

1. Exorcist Girl? Ebola

Sure, I haven't seen this movie, but I can recognize all the references from pea soup to spinning heads. There are parasites that take over their hosts' brain functions, but that's mostly seen in smaller animal models or with prion diseases (which are not parasites). Want to read about Ebola? Check out one of my past entry here.

2. Leatherface?  Leprosy.

 Leprosy is a really unfortunate infection that is fortunately curable when discovered early. Read up on it here.

3. Creepy Girl from Quarantine?  Hookworms.

Nothing says sexy like gnashing teeth.

4. Jason Vorheese?  Primary Amoebic Meningoencephalitis

Both Jason and Primary Amoebic Meningoencephalitis are found lurking in lakes and pools of water, and both will make you crazy. 

5. Old Man from The Unborn? Hobo Spiders.

My partner called my attention to these baby hand-sized wonders. Apparently, he used to wake up to them crawling on his chest, and the males also are very territorial and will charge at you and chase you around a room.

6. Pennywise from It the Clown? Tapeworm.

Both Pennywise the Clown and tapeworms lives amongst poop and eat your insides. The similarities are uncanny! Read about fun tapeworms here!

7. Freddy Kruger?  Bedbugs.

 ...because they get you in your sleep! Here's an older post about bedbugs that will make you want to sleep with one eye open.

8. The Girl from the Ring? Onchocerciasis.

 Onchocerciasis is the clinical term for "River Blindness", which is a disease caused by an infectious roundworm. This one is a bit of a stretch, I know, but that girl has creepy eyes.

9. Michael Meyers?  MRSA.

One of the latest things in medical news is drug resistance. Much like Michael Meyers, MRSA just wont die!

10. Old lady from Legion? 

Three Words: Ellen Albertini Dow

Happy Halloween!

The Plague of Inaccurate Articles

I have to admit, I am partially in love and partially disgusted with the internet. On one hand, it's rad to have answers to almost any question right at your fingertips at any moment in time. But how do you know that those answers are correct? Half of the time I spend reading articles, I find myself thinking "who wrote this stuff anyway?"

Everyone I know is constantly worried about where to get the most accurate source of news about politics, social justice issues, business matters, etc., and I commend them for that. But I also want you to know that such an idea completely pertains to science as well.

I'd like to provide you with an example based on an article that was published this evening by NPR. Ok, I admit, I'm an avid listener to NPR, and I follow them on twitter so I don't have to sort through all the repetitive and mundane articles that come up on google news. But sometimes they are wrong. Sometimes they are horribly wrong.

The article is called "Surprise in Your Sewage: Lots of Exotic Viruses", by Richard Knox. According to NPR's bio page, Richard Knox is totally adorable and has a wicked mustache. After reading the article in question, you'll also notice that he ironically won awards in 1995 for articles covering medical errors. Let's talk about your scientific errors, Richard.

The article starts out on an accurate note. Richard bluntly states that, well, being a scientist is harder than any job you've ever had. As someone who has worked a number of strange jobs (autopsy review assistant, event planner, health educator on a college campus, checker at whole foods,  bra slinger at victoria's secret... yea, I was 18, get over it), the time I've spent looking at human poop under a microscope, literally spending 24 hours straight in lab, or falling asleep while reading scientific paper after scientific paper only to wake up with highlighter ink all over my face has been truly hard. I think back to all the dangerous and toxic chemicals I've had to pour into tiny tubes, all the calculations that I had to check 6 times to make sure I didn't accidentally cause an explosion, or all the tubes of moisturizer I've had to purchase for my rough, over-ethanol'd hands, and I know that I love being a scientist.

After that distinction, the article falls apart quickly.

Fault #1: Inaccurate terms coined for viruses.
This is one of my biggest pet-peeves. It's one thing to convert scientific research articles into layman's news articles, but don't use words like "bugs" or "germs". It's wrong.

Richard doesn't stop there, though. He brings in the famous "microbes" term, which is invalid for this article to begin with. Sure, "microbe" is short for microorganism, meaning an organism that is of microscopic size, but it's a term that's typically reserved for bacteria.

 Also, the term "microbe" suggests that the organism is living, whether single-celled or otherwise. No one has determined whether viruses are "living". To say so, one way or another, is inaccurate.


Fault #2: Know your diseases, buddy.

Firstly, what is a "monkey microbe"? Is it a bacterium with a prehensile tail? There is something disturbing about the insinuated phylogeny of that term.

Anyway, Ebola is one of my favorite viruses. I love it. It's deadly and gross, and the amount of information known about it is relatively minimal. So minimal, in fact, that scientists have yet to identify the model system for it. There has been a lot of research done to try to identify monkeys as the model system, yet they have been unsuccessful. While the disease is seen in monkeys, its not necessarily the origin. Another target species is bats. So, to say that Ebola is a "monkey microbe" is wrong.

Once again, I understand the need to dilute such scientific language, but challenge your readers. Say its a "zoonotic virus" instead of "that jumps to humans when given the opportunity". I mean, come on. This isn't FOX news, now.



I have a hard time crediting articles that make such basic mistakes. I'm glad that Richard links to credible websites for further information, but we know that most people are going to stick to whatever is on the page right in front of them.

If you follow me on twitter, you may remember reading about a similar issue I had with an article for DemocracyNOW! that said that someone had been "cured of AIDS". This is wrong, and it just goes to show that the little details can have profound effects on the rest of your article's efficacy.

Want to get accurate information about science news? Here are some sites that I highly recommend:
ScienceDaily
Science News
PubMed

Febrile Summer

I have a confession to make.

Not only am I a horrible blogger (shh, I know!), but...well...

I'm obsessed with Ebola.
Now, let me back up a few steps and tell you why:

Infectious diseases are my "thing". They've always fascinated me, in the ways that they affect their hosts, in their genius, evolutionary design, and in their strength. When I first learned about Ebola, I was in the Stanford morgue with my biology teacher. I was a freshman in high school, and was the only person in my class that could handle the trip. My teacher didn't doubt that, either. Not only was I the only freshman in the class, but I was getting the highest grade. She might have also thought I would have been interested in going since I was a suburban punk with an open fascination for all things morbid and macabre.

My future in science was sprawled out in front of me, sliced open exposing the internal environment of what you could associate that feeling after you accidentally sleep with your mouth open all night: pooling liquids, stressed tissues and an attractive browning in color. These are all minor details in this story, but I'm sure you can forgive me for indulging in such memories.

During the first autopsy review, I asked what the most interesting cause of death was that they had seen to date. Obviously, no one had died from Ebola in suburban-chic Palo Alto, but a laundry list of infectious diseases was recited.

Fast forward to many years later. Not so much outwardly punk rock anymore (forever at heart, though!), I sit in my Virology class in college, amorously listening to lectures about some of the world's deadliest things. Sure, I knew about Ebola by then, through scientific papers and trashy dramatizations of science, but it was only after those lectures that I truly knew I was obsessed.

Since then, my candid crush on Ebola has ruined a number of dates, relationships and any hope that I had (actually never) had to be considered somewhat normal. So what if I am fascinated with something that has killed tons of people!?

Yea, ok. I get it. I probably should not be on the internet, professing my lust and admiration for a virus. But I did. Lets get to the point, shall we?

Ebola, classified as filoviridae, is a really brutal virus that has been in the news a lot lately. Known to cause hemorrhagic fevers and be extremely pathogenic and fatal, Ebola still has a lot of unknowns associated with it. Although the mode of transmission is thought to be through direct contact with fluids or tissues from an infected person or animal, virologists are still not completely sure. The only way to prevent a massive outbreak is to isolate all infected individuals. Scientists are not even sure what the vector could be, although many have researched various bat species that reside in areas surrounding previous outbreaks.

When you have Ebola, the endothelial cells of blood vessles, mainly surrounding orifices, weaken and lose the ability to cause coagulation, which is part of the healing process. Eventually, these tissues break down completely, causing bleeding from any (or all) of your orifices. Another website describes the symptoms in gruesome detail, as if reading the liner notes for a black metal album:

"The first symptoms are a low-grade headache. This quickly progresses to a debilitating fever and muscle pain. Then things get truly bad as the major organs, the digestive tract, the skin, the eyes, the gums, all begin to break down and bleed. The body begins to dissolve. Blood pours out of body orifices while the victim writhes in pain. Death usually comes from systemic shock and blood loss. Researchers were shocked when they first autopsied people who died from these fevers. Their insides had literally melted into a necrotic mess of black fluid."

About a year ago, a research scientist in Germany earned international attention because she carelessly stabbed her finger while injecting mice with the Ebola virus for treatment and vaccination experimentation. Luckily, her team immediately went into action, isolated her and treated her with a vaccine that was still in the process of being developed.



Rumor has it that she is alive and well. Oh goody!

Three weeks ago, Ebola made international headlines again when scientists in Boston published research showing an effective cure for Simian (monkey)  Ebola. Their research achieved 100% protection against death after the monkeys were injected with a seriously lethal dose of the virus. This has nothing to do with infected humans, but will lead the way for future research and vaccine development.

I am always fascinated at how tirelessly research scientists work to solve major problems, such as viruses with 90% mortality rates, cancer or Alzheimer's...yet, there is always some idiot that is working to keep the problem going, despite any efforts to fix it.

A perfect example of this is the bushmeat industry. Just today, reporters uncovered a serious threat to the spread of many deadly viruses in Paris due to the weekly importation of bushmeat from monkeys, crocodiles and porcupines. My favorite part of that article is this:

"Madame Toukine, an African woman in her 50s, said she receives special deliveries of crocodile and other bushmeat on weekends. She declined to give her full name, fearing she could be arrested."

Oh yes. I know I engage in potentially deadly trade that is incredibly illegal, but I'll give you my last name and the location of my quaint little bushmeat-slinging shoppe.
Also, this:

"Of 134 people searched, 9 had bushmeat. Another 83 had livestock or fish. But people with bushmeat had the largest amounts: one passenger had 51 kilograms (112 pounds) of it — and no other luggage. Most of the bushmeat was smoked and arrived as dried-out carcasses. Some animals were identifiable, though scientists boiled the remains of others and reassembled the skeletons to determine the species."

Mmm! Nothing gets my salivary glands excited like unidentifiable jungle carcass!

Also, if you have the time, check out this amazing revamping of a classic: Curious George and the Ebola Virus.