A Day To Remember-- World Sickle Cell Day, June 19th

The calendar is loaded. Just about every day of the year is spoken for for celebrating or marking some event, idea, concept and dare I say, obsession. Some are worthy and even brilliant. The rare one is deeply embedded within the canon and even more rarely have attained the stature of shibboleths that are not to be messed with. I will steer clear of these latter, though some are so entrenched that they are beyond appearing in the international order of world days. Indeed, all of us, in our own way, enjoy, celebrate and revere these rare examples.

The ones that I have no truck with are the obscure and the pointless. Like, for example, Defend The Donot Day (Feb 24) - for crying out loud! - or ADWN, an acronym for a A Day Without News, Feb 22, that Wikipedia gaily assures me exists. And there is World Cat Day (August 8) - for goodness sake - and World Tripe Day (October 24), as if we don’t already have enough of that dished out on a daily basis. You just have to listen to certain world leaders, especially what they say in their early morning rants. And then there is World Pest Day (June 6) - good grief! -which, in the spirit of conservation or parsimony, could be twinned with the day for Trump, sorry, Tripe and, which gives me an idea. Why not have a World Parsimony Day when the entire caboodle could be dispensed with in one massive blowout? But judging by the range of days that already plague us, I wouldn’t be surprised if there is already such a beast in existence somewhere.

So, if one is fed up with all these pointless days, why promote another one? Well, there are some days that are heavy, not only with symbolism, but also with real value. So, it is with some confidence that I present to you, again, this June 19, World Sickle Cell Day. This day reminds us that the battle against sickle cell is still on and that the fight against this debilitating and often fatal disease continues. Even though there are advances every year, sickle cell disease is still responsible for a significant number of deaths in children under five years of age in many countries.

Do your bit to help the fight. Go visit the website: www.sleonesickle.org to see what you can do to help.

Tell Fren Tru

Is This The Cure For Sickle Cell Disease?

Sickled Cells in a patient with Sickle Cell Disease

It’s all over the place. Everywhere you turn there is this buzz. Relief, one is happy to say, from the relentless reports of wars, conflicts and disgraceful tweets. The thing that is causing the buzz is the story about a new cure for sickle cell, a disease that affects hundreds of thousands, if not millions throughout the world.

There is nothing unusual about new cures. Breakthroughs for this or that are regularly announced, both in the regular media and in fringe outlets. And we humans seriously take them on board together with their hope and their hype, especially for diseases that stubbornly resist the best efforts of our medicine men and women, scientific or shamanistic. The cure for cancer, especially (as if cancer is just one disease, like appendicitis), is one that regularly grabs the imagination. But one must always be cognizant of the fact that however relevant these “cures” might be, their impacts are mostly incremental and, in any case, require time to mature into full-fledged remedies.

The cure that was announced last week must, as in other similar examples, be the subject of a reality check. Nevertheless, this is one announcement that should be taken very seriously because the procedure directly targets the engine that drives the terrible clinical consequences of the disease. In the report, French researchers describe how they harvested blood stem cells from a 13-year- old boy suffering from the disease and inserted normal haemoglobin genes into them. They then returned the modified stem cells back into the boy, who subsequently began to make normal red blood cells. Fifteen months later he is free of all the symptoms of the disease. Pretty good, when you consider that for most of his young life he had suffered severe symptoms. Everybody is now talking excitedly about how this treatment can be rolled out to all those hundreds of thousands of patients throughout the world suffering from sickle cell.

Conceptually, the procedure is simple enough. But in practical terms, it could be devilishly complicated as demonstrated by the huge number of researchers that pulled off the treatment. Indeed, when I looked at the report, I had to give up counting the named individuals and institutions that collaborated in the work. I can say, though, that their number, combined, might well exceed the entire scientific and medical capital in some countries where sickle cell is prevalent.

Another point: This is just one case. That of a13-year-old located in a well-resourced setting and is a long way from signalling that the treatment can be extended everywhere, even to other rich countries, never mind into the swamp-like conditions that prevail in poor ones. To show that the treatment can be applied elsewhere calls for a clinical trial that would involve many patients preferably from more than one big institution. Undertaking such would require time and money. We’re probably looking at, may be, five years, if not longer, not to mention the necessary amount of cash.

In the mean time we do have other measures which, though not curative provide much relief for patients. These are the things we should focus on at present. But even here, there is an urgent need for peoples and governments to step up and do their duty.

Tell Fren Tru

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