Hemophilia B

For someone with hemophilia, injections of clotting factors are a normal part of life

Inheritance of Hemophilia B

Hemophilia B goes by many names: the Royal Disease, factor IX hemophilia, and also Christmas disease. It is the second most common cause of hemophilia, a bleeding disorder caused by a decreased ability for the body to clot. In the case of hemophilia B, the body lacks one of the molecules involved in the natural clotting reaction: factor IX. It is an X-linked condition, which is why one of the risk factors of the disease is the sheer matter of being born male. Of course, a family history is another risk, especially if it is seen on the mother's side. Women who have a family history of hemophilia may be carriers, and a carrier has a fifty percent chance of producing a hemophiliac son. It is possible for a girl to be born with hemophilia, but with all X-linked recessive conditions it is very unusual as the father would have to be affected and the mother would have to be a carrier.

The reason one of the common names for this disease is "the Royal Disease" is due to the fact that it once ran in the lines of European royalty that could be traced back to Queen Victoria. Perhaps one of the best-known of her hemophiliac descendents was Alexei, the son of Tsar Nicholas of Russia, whose bleeding was able to be helped by the monk Rasputin. He would hypnotize Alexei, and this is thought to have helped slow bleeding by calming him down. Rasputin's influences are just part of what led to the Russian revolution. It is believed all current descendents of Queen Victoria lack the gene that causes hemophilia B.

As would be expected, the main symptom of hemophilia is heavy bleeding. However, that bleeding can lead to other issues, such as joint problems and possible malformation. Other than the obvious risks that excessive bleeding can cause, there are other serious risks that can come from hemophilia. One of the common treatments in transfusions, which in the past place patients at serious risk of infectious diseases such as hepatitis and HIV. Though screening of donated blood today is very efficient and affective, there is still a risk that something may be missed. In people with a family history of the disease, genetic counseling is suggested. This is partly a preventative measure as a child born with hemophilia will have to lead a rather unusual, potentially very uncomfortable life.

Recently research into gene therapy for those with hemophilia B has produced some positive results. The current treatment of injections containing the missing clotting factor could potentially be a thing of the past. However, more research is needed before the treatment becomes widely used.

Sources are PubMed Health from the U.S. National Library of Medicine, the National Hemophilia Foundation, and the Speaking of Research Blog. Images are from Wikimedia Commons and are copyright free or under a Creative Commons license: one, two. 

Why you should Vaccinate your Kids

A young girl being vaccinated for typhoid in 1944.

Giving an oral polio vaccine

Today, doctors advise that children are vaccinated against numerous diseases. According to the CDC, in 2011 by the age of six years all children should have been vaccinated against Hepatitis B (HepB) and A (HepA), Rotavirus (RV), Diptheria, Tetanus, Pertussis (together as DTaP), Haemophilus influenzae type b (Hib), Pneumococcal disease (PCV), Polio (IPV), Influenza (strain changes yearly), Measles, Mumps, Rubella (together as MMR), and Vericella. Many of these are series vaccinations, requiring a series of shots over a specified period of time. Others should be done annually, such as influenza. That's not all, either, as vaccinations continue on even into adulthood. Also, catch-up vaccines are suggested for many diseases if someone had not previously received them. People in certain lines of work may be required to have extra vaccines, such as a rabies vaccine for people working with animals. Vaccinations are also often a requirement when traveling abroad, where other diseases are more common or where health standards may be quite different from here.

A smallpox vaccination kit, including the famous forked needle. The smallpox vaccine is derived from the far milder virus that shares many of the same characteristics: cowpox.

Through the use of vaccination, some amazing feats have been achieved. Smallpox has been eradicated and now only exists in laboratory samples kept under lock and key. The vaccination is no longer given to everyone as it once was, but is only given as a precaution to members of the military and people who hold certain other positions. Polio, which once lead to the paralysis of numerous children, is basically a thing of the past. The incidence of many more diseases, such a measles, have dropped significantly and may only be rarely seen. People who have been vaccinated will rarely, if ever, contract the disease they have been vaccinated against.

One of the common trends in modern society has been to not vaccinate, mainly due to the assumption that vaccinations have lead to complications, up to and including autism. It is true that some vaccinations carry certain risks, such as some of the potential problems associated with the vaccine for chicken pox (Vericella). Minor symptoms such as redness, swelling, soreness, itching, mild to moderate fever, and others can and will occur rather commonly. However, vaccines are overall incredible safe with serious reactions only happening at frequencies of one in every one hundred thousand (that's 100,000) people or even rarer. For most vaccinations, incidence of severe reactions are one in a million or rarer. To put that in perspective, there are over three hundred million (300,000,000) people in the United States. Of those, only about three thousand (3,000) will have a severe side affect to a vaccine where severe reactions happen once in ever hundred thousand people vaccinated. That's really very good odds, considering the alternative of placing your child at risk of contracting diseases that may potentially kill them, such as whooping cough (Pertussis).

When compared to the risk of severe reaction of a vaccine, the risk of very serious disease or complications are much higher. For example, rubella (also known as German measles) causes dangerous brain infection in one out of every six thousand adults infected, and pregnant women who develop the disease will often loose their baby, or have it be born with serious birth defects. In contrast, severe reactions to the MMR vaccine only occur at most in four of every one million people vaccinated.

An autism sign: obsessiveness

Lately, one of the largest arguments against vaccination is autism. There are many people out there, including some celebrities, who believe vaccinations (usually MMR) are responsible for autism. Many of these people are parents of an autistic child. In fact, that theory simply does not hold water. Only a single study was every published that implied such a link (Wakefield et al, 1998), but the theory on a autism-MMR link is based on flawed methods and has since been retracted. It has also since been disproved on numerous occasions by further scientific reviews. It is true that often a child will begin to show signs of autism about the same time that they receive their MMR vaccine. Children in the United States today usually receive their MMR between the ages of one and two. Autistic children are usually diagnosed before the age of three, and signs can appear as early as six months. Also, it is not unusual for an autistic child to appear normal for quite some time and then appear to regress. Since there is a correlation between the age at which autism is identified and the age at which a child will receive certain vaccines, it can be seen why an assumption can be made that autism was caused by the vaccine. However, as any scientist or statistician will tell you, correlation does not imply causation. So, even though a link may appear to be present, that doesn't necessarily mean that the link exists.

So, let's look at these facts again: vaccinations prevent children and adults from contracting very serious illnesses. Severe reactions to the vaccines are rare, and these rates are better than the risks of the disease that is being vaccinated against. If you think about it, it really is a much better idea to vaccinate than to go without. If you refuse, you kid could get polio, hepatitis, or so many other serious diseases if they come in contact with an infected person and then you'll be regretting the decision for the rest of your life.


Main source is the Center for Disease Control and Prevention's Vaccines and Immunizations portal. Other sources are the New York Times, Children's Hospital of Philadelphia, Brian Deer, Exceptional Learners: An Introduction to Special Education, and Vaccinate your Baby. Images are from Wikimedia Commons and are under a Creative Commons license or are copyright free: one, two, three, four.

The Common Cold

I've managed to get sick, so I thought it would be appropriate to talk about the common cold.

A model of the rhinovirus, one of the viruses that can cause the common cold

The common cold is an incredibly common ailment, causing more than one billion cases in the United States alone each year. It is the number one reason that people miss work or school. Though you can get a cold at any time, they are more common in the winter, thus "cold and flu season." The common cold is easily transmissible, through miniscule droplets released when someone who has a cold coughs, blows their nose, or sneezes. Generally, someone will only be contagious for a few days, and symptoms will appear within three days of contact.

An uncovered sneeze can spread disease

Symptoms include sneezing, nasal congestion, a scratchy throat, and a runny nose, but can also include a sore throat, cough, muscle aches, decreased appetite, postnasal drip, and headaches. Treatment is basically riding the virus out and treating symptoms. Over the counter medication can do wonders to make a person feel better, including cough suppressants. Antivirals have so far proven to be completely useless in treating the common cold.  Antibiotics are only suggested if a secondary infection occurs. Complications of the common cold can include bronchitis, sinusitis, pneumonia, and ear infections.

Chicken soup is good for the cold

Taking vitamin C regularly can prevent your cold from being very long, and taking zinc when you first show symptoms can also shorten the length. Echinacea is sometimes suggested to help prevent you from contracting the virus and also for easing symptoms, but studies have shown that it really doesn't make any difference. Chicken soup is also a common home remedy, and it is believed that the combination of heat, fluids, and salt may be fairly effective. It is unusual for someone to not make a full recovery. If symptoms last more than a week a visit to the doctor is suggested.

Preventive measures are pretty straightforward, including regular hand washing, disinfection, and use of hand sanitizer. A decreased immune symptom can put you at risk for this virus, and ways to improve your immune system include avoiding cigarette smoke, using antibiotics only when necessary, drinking plenty of fluids, eating yogurt, getting enough sleep, and breastfeeding your children. Kids who are not breastfed are, on average, far more sickly than those who are breastfed.

Source is PubMed Health from the U.S. National Library of Medicine. Images are from Wikimedia Commons and are under a creative common license or are copyright free: one, two. three. 

Name That Disease #5

This one should be easy!

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Name That Disease #4

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Name That Disease #3

Well, technically this is not necessarily a disease. Can you guess it?

Read more for the answer:

Name That Disease #2

This should be an easy one.

Read more for the answer!

Name That Disease #1

 I saw a man with this same condition today. Can you guess what it is?

Read more to see the answer!