Sunday, January 06, 2013

On twitter feeling happy like I discovered hydroxyurea therapy or something

I decided to devote some time to thinking about malaria and sickle cell anaemia this month. 
Started by checking out the origins of quinine and artemisinin online.  Both were 'discovered' by local folk using them naturally hundreds of years ago, then rediscovered and sold in more deliberate forms. 

Moved on to sickle cell where as one would expect, people have tried going into the stem cells for red-blood cell production and switching the one mutated point in patient's DNA right back. 
Read from bottom to top

Read about the wide variation in how rough people with this genetic disease have it: from not-so-bad in many of the Arabian occurrences to regular killer in Africa. 
I don't really like twitter but yeah I'm @tosinbird
And of course, there is a treatment based on that: hydroxyurea is said to be so effective that it's a surprise not more people are using it. 
Treatment, yay!
Maybe I'll stop there for now. 
But lots of questions still - remember the prophylactic 'Sunday-Sunday medicine' we used to take for malaria in boarding school - what was that about?  
What other cool manipulations can prevent the occurrence of sickle-cell and its related suffering?  Nowadays we tell people that 'AS shouldn't marry AS, only marry AA' but how easy is that to understand?  And for those who understand it, I've seen how painful it can be for a young couple to have to break off their engagement after a drug test says they're both AS.  More than 20% of Nigerians are either AS or SS according to this paper.


t said...

Some more nice information on sickle cell:
1. Moringa, oh famous Moringa, shows anti-sickling in the lab and may be useful for treatment. Moringa oleifera Lam. (Moringaceae) grown in Nigeria: In vitro antisickling activity on deoxygenated erythrocyte cells Comments? This is a 2012 paper so you could contact the researchers directly or even work with them.

2. Other plants with properties that make them useful in treatment of sickle cell

3. Something about unripe pawpaw, seeds of the pigeon pea, and other natural substances click here.
Folkloric history has it that an aqueous extract of the unripe fruit of pawpaw is also used as a home remedy in Southwestern Nigeria for patients during crises. Its evaluation showed that its water extract after 48 hours showed that it was capable of inhibiting and reversing sickling of HbSS red blood cells. The possible antisickling compounds were suspected to be organic acids released after the fermentation of the fruits for 48 hours.

4. A list of sickle cell organizations / foundations in several states and regions of Nigeria and other parts of Africa, with names and phone numbers of contact persons. Compiled by Sickle Cell News/Journal: AFRICAN SICKLE CELL NEWS & WORLD REPORT.

5. How effective is genetic counselling? See one study. Also, not sure I understand why keeping family size small helps lower the aggregate number of people with sickle cell disease. Explain?

Have fun!
I guess when this is solved there are new, cool, frontiers to move on to...

t said...

Nice paper with a review and details of how fetal haemoglobin (total level and % of red blood cells with high HbF level) affects the sickle cell experience. By Idowu Akinsheye, Martin H. Steinberg, and others.
Read here

In summary, the authors write: "HbF has beneficial effects in sickle cell anemia. The contrast between asymptomatic persons with HbS-gene deletion HPFH and symptomatic patients with sickle cell anemia with similarly high HbF levels suggests that, if it were possible to induce high HbF levels in most sickle erythrocytes and if this could be done before organ damage occurs, one might expect the disease to be “cured.” Presently, this is not possible, but a better understanding of how HbF levels are modulated might suggest new therapeutic approaches and combinations of HbF-inducing agents that could allow this goal to be met. "