World Haemophilia Day, marking 50 years of advancing treatment for all

Today, 17 April, is World Haemophilia Day, the day shining a spotlight on the global bleeding disorders community. This year, 2013, marks ’50 Years of Advancing Treatment for All’.

To quote Wikipedia, haemophilia “is a group of hereditary genetic disorders that impair the body’s ability to control blood clotting or coagulation, which is used to stop bleeding when a blood vessel is broken.” The most common type, Haemophilia A, occurs in about 1-2 in 10 000 male births, while Haemophilia B is about half as common. Both forms are more likely to occur in males than females. It is a recessive sex-linked, X chromosome disorder, and since females have two X chromosomes while men have only one, the defective gene will manifest itself in every male who carries it, while it may not manifest itself in a female carrier.

Everybody bleeds, but while this is may be a minor issue for most of us, it can be a matter of life and death for those suffering from haemophilia. (© All Rights Reserved)
Everybody bleeds, but while this is may be a minor issue for most of us, it can be a matter of life and death for those suffering from haemophilia.
(© All Rights Reserved)

People suffering from haemophilia do not bleed more vigorously than a healthy person, but they are likely to bleed longer due to the lack of coagulation or blood clotting. Thus even a rather minor injury can result in excessive blood loss. In some injuries, such as injuries to the brain, and injuries to the insides of the joints, this can be fatal or permanently debilitating.

No cure yet exists for haemophilia, but it can be treated with regular infusion into the body of the deficient clotting factor. Sufferers also have to adapt their lifestyles and activities to minimise injury risk. Exercises to strengthen the joints, and to increase flexibility, tone and muscle strength are also recommended. There are indications that hypnosis and self-hypnosis may have some effectiveness at reducing the severity and duration of bleeding, but much investigation is still required in this regard.

As with many diseases and disorders, haemophilia impacts most severely on people living in developing countries, people who do not have access to proper care and/or treatment. It is estimated that globally 75% of people living with bleeding disorders receive very inadequate treatment, or no treatment at all, with the majority of these people living in the developing world.

Through World Haemophilia Day, it is hoped that increased awareness and support can be gained for people living with bleeding disorders, and that this can help inch us closer to the goal of quality treatment for all.

Fifty Shades of Red

Do you know why your blood is red? It’s thanks to the red blood pigment, haemin, which is one of the components of haemoglobin.

And why do I know this? Well, because I’ve been reading up on Hans Fischer, the German biochemist who was born on this day in 1881, and who was awarded the Nobel Prize for Chemistry in 1930, primarily for his work on the structure and synthesis of the blood pigment haemin. In 1929, Fischer succeeded in synthesising haemin, the deep red, oxygen-carrying, non-protein, ferrous component of haemoglobin, that gives blood its red colour.

It’s elementary, my dear Watson – this is definitely not alien blood.
(© All Rights Reserved)

Oxygen-rich blood (such as arterial blood and capillary blood) is bright red, as the oxygen intensifies the colour in the haemin. When oxygen is extracted from the blood it turns a darker shade of red – this can be seen in the veins, and in the blood collected during blood donation. The colour of blood can also be an indicator for certain medical conditions. Both carbon monoxide poisoning and cyanide poisoning result in bright red blood, as it inhibits the body’s ability to extract and utilise the oxygen in the blood. On the other hand, severe deoxygenation (which can be caused by respiratory diseases, cardiac disorders, hypothermia, drug overdose or exposure to high altitude) results in a condition called cyanosis, where the blood darkens to such an extent that it gets an almost purple-blueish hue, resulting in the skin turning a blue colour.

While the blood of humans and all vertebrates is always a shade of red (containing haemin), it’s interesting to note that it is, in a strange way, surprisingly close to being green! In addition to his work on blood pigmentation, Thomas Fischer also studied the components of the pigments in leaves. He found that, like the haemin in blood, the chlorophyll in leaves is a porphyrin, and that haemin and chlorophyll share a very similar structure, with only subtle differences.

All of this talk of blood, and red and green pigmentation, conjure scenes of science fiction in my mind – if haemin (that makes blood red), is so similar to chlorophyll (that makes leaves green), perhaps the idea of green-blooded aliens is not such a stretch. It makes scientific sense, right?

Anyway, let me rather stop before I get too carried away. Enjoy the day, and keep an eye out for those little green men! 🙂

World Blood Donor Day

With today being World Blood Donor Day, I thought what better way to gather info for my blog than to immerse myself in the experience, and register to donate on the day.  So I duly pre-registered, filled in some forms, and my booking for this morning was made.

Arriving at the blood bank, more forms had to be filled in, and having never donated blood in New Zealand before, I was quite surprised at some of the rules for eligibility to become a blood donor.

Most of the limitations (never give blood if you or your sexual partner(s) are HIV+, if you carry the Hepatitis B or C virus, if you’re on drugs, etc) seemed pretty sensible, as were the limitations placed on sexually promiscuous individuals.  The geographic limitations, however, were more of an eye-opener, and this is where my good intentions sadly got derailed. It turns out that anyone who had previously lived in a region considered to be high-risk for HIV infection, were excluded from donating for 5 years.  Excluded regions include the entire sub-Saharan Africa, large parts of Asia, as well as specific South American regions.

This exclusion is irrespective of sexual history, previous HIV test results, or any other ‘proof’ of not being HIV+.  So, given my South African heritage I was greeted with a friendly but firm “No thank you”, putting a premature end to my intentions of becoming a blood donor in my adopted country. I can appreciate the logic of geographic exclusion, but cannot help finding it sad that, despite being married and faithful to my wife for many years, and having ‘passed’ a number of insurance and emigration-related HIV tests in the past, I am still considered to be a higher risk than someone earning a living as a prostitute in New Zealand (who only has to wait 1 year before being accepted as a donor).

What makes this experience more ironic, is that the international launch of World Blood Donor Day took place on 14 June 2004 in Johannesburg, South Africa.

You can become a life-saving superhero, irrespective of your blood type.
(© All Rights Reserved)

Oh well… So unfortunately I cannot yet share with you any first hand donor experiences.  What I can do, however, is to share some interesting facts and figures about blood and blood donation:

  • When “donating blood”, you can actually donate a number of different transfusable blood products – red blood cells, platelets, or plasma.
  • When donating a pint of “whole blood”, two to three of the above products can be produced from the donation, hence a single whole blood donation can save the lives of up to three people.
  • If you donate only specific blood components – red cells, plasma or platelets – the process is called apheresis.  A single apheresis donation can produce one transfusable dose of platelets.
  • Of the blood products that can be donated, only plasma has a reasonably long shelf life – it can be frozen for up to two years and blood products made from plasma (e.g. cryoprecipitate) can be stored for up to two years.
  • Red blood cells must be transfused within 35 days from collection, while platelets have an even shorter shelf life – it has to be transfused within 5 days.  As a result of this, there is a continuous need for fresh blood.
  • Healthy bone marrow is continuously working to produce new red blood cells, platelets and plasma in the body.  Blood lost during a donation is replenished by the body over time – the fluids of the donated blood is replaced in just 24 hours. The red blood cells take a bit longer, and will be replaced within about 8 weeks. Typically a healthy donor can donate every three months.
  • In New Zealand, the treatment of cancer requires the biggest percentage of all donated blood products (22%), while blood needed to treat accident victims make up 18%. Mothers and babies receive about 7% of the blood supply.

The bottom line is that blood is always desperately needed, and it really is one of the easier ways of doing something truly amazing for your fellow man.