Dates Matter: World Sickle Cell Day, June 19 and Juneteenth

I don’t know how the United Nations came to decide that June 19 should be World Sickle Cell Day; the ways of international organizations are mysterious. But it was a good decision.  One suspects that America must have had a hand in the choice of the date, since sickle cell continues to be a significant public health problem in that country.

Dates matter. We can be sure that had it not been for an event that occurred on a certain date in America’s history, sickle cell disease would not have become the burden there that it is today. The event that I am thinking about took place in August 1619 and possibly marked the beginning of an infamous trade that lasted two centuries. The event was believed to be the first ever landing of Africans on North American soil at a small place in Virginia to which disgruntled Englishmen had fled away from the religious intolerance that blighted their native land. In the event, after the wasting of ten, or may be, twenty million black lives and the passage of two centuries, the worst ever example of human trafficking came to an end. Sort of. But it took a civil war for the institution of slavery itself to end in the American republic. Again, sort of. It is an irony impossible to ignore because that republic had been founded ostensibly in response to what the descendants of those refugees escaping religious persecution deemed tyranny, rooted in the actions of a mentally unstable monarch in far-off England. As it happens, that war is still being waged today, to the extent that, many in the republic feel bound to own assault weapons as their own personal guarantee against oppression.

Dates matter. The American civil war had been fought over the southern states’ refusal to abandon slavery, and it was not until May 1865, when the pro-slavery gang had been utterly defeated, that their resistance to the notion that all men are created equal was broken.  And on June 19, 1865, Major-General Gordon Granger, commander of  the military District of Texas at Galveston, TX, issued  his General Orders, Number 3  that declared, The people of Texas are informed that, in accordance with a proclamation from the Executive of the United States, all slaves are free and that… this involves an absolute equality of personal rights and rights of property between former masters and slaves, and the connection heretofore existing between them becomes that between employer and hired labour. Yes. There was, naturally, jubilation among those who believed that they had been made free by the order, and the day, nicknamed “Juneteenth”, (“June” plus “teenth”) became a holiday.

 Dates matter. You see where I’m going?  June 19^th is a significant date in the history of blacks in America, marking emancipation from the burden of slavery. So, perhaps it is not a coincidence that Juneteenth, the 19^th of June, has become the day of observance for sickle cell disease and the date to note the work that remains to be done to emancipate, worldwide, the 30 million suffering under the public and personal burden that is sickle cell disease. So, we must mark this day in 2016 in whatever way we can, but, best of all, by donating towards the struggle. If you want to donate, go to the website of The Sierra Leone Sickle Cell Disease Society at www.sleonesickle.org. and follow the links. This could very well the most important thing you do for the rest of the year.

Tell Fren Tru

Fantastical Science

When the Sierra Leone Sickle Cell Disease Society released its 2012 annual report recently (http://www.sleonesickle.org/2012AnnualReport.html) it reminded me that there has been little progress in the treatment of sickle cell disease in recent times, even though affected children in rich countries have been surviving well into adulthood. But survival into adulthood does not necessarily guarantee relief from the continuing anxiety induced by the threat of early mortality. Besides, other severe, but not necessarily lethal complications of the disease, such as acute severe pain and strokes and infection remain a constant blight on lives of patients, whether they live in a rich country or in a poor one.

Doctors and researchers, worldwide, are doing their damnedest to discover new and effective treatments for the disease, but the promise of a magic bullet that cures all the ills of sickle cell remains unfulfilled. That is perhaps not surprising because, the deeper we dig, the more complex the sickle cell story becomes. When sickle cell became the first “molecular” disease to be linked to the inheritance of an abnormal hemoglobin, the blood pigment that carries oxygen and gives it its red colour, it was hoped that the cure would not be long in coming. Those were simple times: All one had to do, it was thought, was to discover or design a chemical that would engage the wayward hemoglobin and force it to behave itself.

Now, it has become clear that the out-of-control hemoglobin is just one part of the problem. The inheritance package consists of not just hemoglobin, normal or abnormal, but, as well, of all the other characteristics that make us who or what we are. Teasing out all the factors that contribute to the bad act that sickle cell disease is has, since, become a complicated business. And I am not even talking about the social aspects. I am not going there. You can wrestle with that one yourself, considering that two parents are required to produce the individual affected with sickle cell. I am  talking biology here.

That brings me to the complexity and anxieties surrounding big research. I was listening to a radio programme the other day in which four experts were discussing the relevance and cost of current research not only in the biological sciences, but also in the physical sciences as well. The problem for physics, it was said, is that it has become too theoretical to the point of being almost nonsensically abstract, whilst biological science has become too big for its boots, claiming that it can cure just about everything, from “making the blind see, the crippled walk and the deaf hear” (I quote here), especially invoking the use of stem cells. Meanwhile, Big Pharma is going completely out of control. 

The remedy? Theoretical physicists have to stop “stringing” us along a path to weird universes. And biology should stop twittering on about stem cells and the marvels they can accomplish. As for Big Pharma, it should stop holding the public to ransom with fabulously expensive nostrums which even rich countries can ill afford.

What relevance do string theory, stem cells and Big Pharma have with sickle cell disease? Well, at least some of the money spent on research in these areas can be applied to research in sickle cell disease. Some of this money can, hopefully, find its way to researchers in Africa where the burden of the disease is greatest, and perhaps, combined with the vast riches in the African eco-system, productive connections can be made for effective therapies for the disease. That would be fantastic. After all, sickle cell is the human genome’s response to the gauntlet thrown down at it by another forest-dweller, the malaria parasite.

Too bad Bill Gates does not read this blog.

Tell Fren Tru

Post Script: June 19 is World Sickle Cell Day