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. 

Happy Birthday, Ebon! (lots of pictures)

Ebon was born seven years ago today. He has been my loyal dog for six years and seven months of that, as I brought him home on his five month birthday. My how time flies! Here's a little photo diary of the past few years. Unfortunately, I don't have any photos of him from before five months of age. However, I still remember the tiny creature he was when I first met him at two weeks old. It's amazing how much he has changed. It's been quite an adventure, and I hope to have at least another seven years ahead with my wonderful fella.

Ebon at five months old, the day I brought him home.

Ten months old. One of my favorite pictures of him.

Twenty months old, along with his mother. Here she's about the age he is now. She's still going strong!

Three years old, shortly before he began going gray.

On his fifth birthday, politely begging for his birthday treat.

For his birthday, Ebon also got to have fun and eat good things. We went back to my campus and did a large circuit, which took about two hours. He had a blast, even taking a little dip in the pond. There actually aren't any leash rules on certain parts of campus, so I also spent a little time working with Ebon's off leash manners. Mostly this consisted of practicing recall near distractions, such as a busy road (with a fence between us and the road). He's very good at keeping to a loose heel while off lead, staying within ten feet of me in whatever direction while we walk around.

By the pond right at the beginning of the walk. It was a beautiful day. Notice the difference in the pond from before. It's been warm, so the duckweed has been flourishing.

By the ditch. The path ahead and to the right leads behind the campus greenhouse.

If viewed as a square with the pond as the first corner, this baseball field is at the second corner.

On the campus trail. This is near the soccer field.

Feet on the campus trail. Not much else to say.

At the oldest of the three fountains on campus. This is about halfway along the last side of the square.

Back at the pond right before leaving, I took off the vest and let him off lead to see if he wanted to take a little swim. I think the water was a bit cold because he only waded.

The ingredients.

As for the food, Ebon got a tooble (smoked beef trachea) as a snack and with his dinner he got a mixture of banana, peanut butter, and cream cheese added to his kibble, plus some freeze dried liver. I have actually gone to the lengths to bake him a cake before (using a doggie cook book), but this time I decided to go the simple route and mix him up something with a touch of sweetness that I knew he would like. By the way, two bananas, one small tub of cream cheese and a cup of peanut butter makes plenty for a dog and a few friends, or enough for several treats if you really want to spoil him or her. I did taste it, as I have a tendency to do with anything I hand make, and it just tastes like sweet peanut butter.

The drooling face of anticipation.

Ebon's birthday "cake." He licked every last morsel out of the bowl. He will be getting more tomorrow.

Guess the Genotype #44

Can you guess this dog's genotype? Her breed?

Images are copyright to Jess Booth of Desert Wind Hounds, and are used with permission.