This world has seen its fair share of love superheroes- think of the man born in Nazareth who took 64 lashes(lost count cry-watching The Passion Of Christ) to Jack of Titanic whose love story grossed over 1.4 billion dollars in movie sales and finally to the “Legend of Bantama” who died contracting incurable gonorrhoea. One guy didn’t get a top-three shoutout. The reason being; despite his incompatible sickling genotype, he took a not-so-brilliant decision – in hindsight – to conceive a child with an incompatible partner. This guy gets a full feature on this blog because he makes me ask a troubling question:

Is love ever enough?

The paradox of Love: The Ugliness behind love
Photo Courtesy Lemuel Hearts Advocacy for Sickle Cell Anaemia

SICKLE CELL DISEASE IN REVIEW

Save the stress understanding this. check out the post below.

I really wish I could go to the world’s end to explain all the protein chains, gene mutations and abnormally inherited alleles responsible for causing sickle cell disease. However, a good friend of mine who runs a great medical blog wrote a simple digestible blog post on Sickle Cell Disease. To get a grasp of what follows in this blog post, you should take a look.


Read: Sickle Cell Disease Oversimplified I: The Basics

ALL THE “BAD” DECISIONS TWO LOVERS CAN MAKE

As the saying goes, ‘one day we will all fall knee-deep in love’. Forgotten the author of the quote(Lion King maybe?) but the question is, will it come at the cost of life-long suffering for an unborn child or an inevitable uncomfortable future? The choice is yours if you want to play a game of chances. However, if you have a child(SS), you probably might be thinking, does this child have a life expectancy cap? Great question. You aren’t alone. A guy on an incredible informative Whatsapp page I am on asked this:

Myth or Fact? Let’s bust it!!!!

A CURIOUS CASE OF SICKLE CELL DISEASE

Every myth has an iota of misguided truths somewhere in there. What would really make a sickle cell patient die at an early age(said specific age)? To truly understand this question, watch this short clip on where the trouble starts that puts the life of sufferers at great risk.

From the video up there, these defective red blood cells don’t last long compared to the 120 days lifespan of healthy red blood cells, thus breaking down rapidly leading to anaemia. Now, the dangerous part is-abnormally shaped sickle-shaped red blood cells stick together blocking important blood supply channels(vessels) thus depriving vital organs of oxygen and blood. Acute conditions arising from Sickle Cell Disease summed up is what is called “Sickle Cell Crisis”. The major cause of mortality in SCD patients.

Photo Courtesy Lemuel Hearts Advocacy for Sickle Cell Anaemia
THE TRAGIC DEATH OF PRODIGY
Albert Johnson alias Prodigy of Mobb Deep of the US, performs during the Open Air Frauenfeld music festival on Saturday, July 9, 2016, in Frauenfeld, Switzerland. (Gian Ehrenzeller/Keystone via AP)

In June 2017, while performing at the Art of Rap in Las Vegas, Prodigy was hospitalized following an acute crisis on stage. He would die the following morning at age 42. If you don’t know who Prodigy is- he was one of the most iconic rappers in the hip hop scene. Forget Logic and Childish Gambino, this guy owned the rap scene from the 90s and was part of the West Coast-East Coast feud that led to the death of 2pac Shakur.

Prodigy was SS and had been battling complications of this disease throughout his life. Citing his battle with sickle cell the reason he had a nihilistic view on life.

DID YOU KNOW?

Prodigy sparked the theory, a highly powerful secret society existed- The Illuminati. And spent a good chunk of his rap career speaking publicly against it even to the extent of accusing Jay Z of being part of the alleged secret society.

The death of the rap artist Prodigy of Mobb Deep fame at only 42 following a lifetime of suffering from sickle cell disease, was a reminder of the devastating cost of the sometimes fatal genetic disorder.

THREE DEVASTATING SICKLE CELL CRISES

VASO-OCCLUSIVE CRISIS

When blood vessels clog up, this leads to deprivation/reduction of blood supply and ultimately oxygen supply to target organs. This leads to death of tissues(necrosis), intense pain(bone pain, abdominal pain, etc) and often organ damage. Sufferers are susceptible to strokes. A vaso-occlusive crisis involving organs such as the penis(priapism) or lungs(ACS) is considered an emergency and can lead to death.

SPLENIC SEQUESTRATION CRISIS

Your spleen is like the laundry of your entire circulatory circuit. Its function is to mop up defective red blood cells. Due to occlusion of its somewhat narrow blood vessels, most Sickle Cell patients do not have proper spleen function. A lack of proper functioning spleen leaves sufferers susceptible to infection from encapsulated microorganisms(strep. pneumonia etc).

Now the destructive part of this crisis is: by the time sufferers get out of childhood, there has been significant infarction to their spleens. Since the spleen acts as a filtering system, entrapment of red blood cells(normal and abnormal) occurs within it. This causes the spleen to enlarge(splenomegaly) and become painful. Now don’t forget these red blood cells(normal) are needed for systemic circulation! With entrapment, sufferers can go into hypovolemic shock(reduced blood volume). Sequestration crises are considered an emergency. If not treated, patients may die within 1–2 hours due to circulatory failure.

ACUTE CHEST SYNDROME

It is defined by at least two of the following signs or symptoms: excruciating chest pain, fever, cough, respiratory symptoms, or hypoxemia(low oxygen levels).

Acute Chest Syndrome is the second-most common complication and it accounts for about 25% of deaths in patients with SCD!

BUSTING THE MYTH

Let’s toss the myth into the bin! Sickle cell suffers do not have a specific age at which they die. There isn’t any life expectancy cap on their heads. Yes, they are prone to life-threatening conditions like the crises you saw a few paragraphs above which has a high mortality rate. However, there is hope! With the advancement in medicine and drug research, many would have the chance to live a long normal life as normal people do.

HOPE IN THE MIDDLE OF THE STORM

It’s been 61 years down the line since the discovery of the mutation responsible for sickle cell, and 30 years since scientists found a compensatory mutation — one that keeps people from developing sickle cell despite inheriting the mutant genes.

Well, the thing is, unfortunately, sickle cell majorly strikes the “wrong” kind of people, including African-Americans, and Africans thus seeing massive starvation of funds for research.

The good news is, in the last 4 years significant progress has been made towards providing a cure! “There are huge numbers of drug companies finally putting money into this,” said Dr Mitchell Weiss, chairman of Haematology at St. Jude Children’s Research Hospital, who is developing a genome-editing approach, using CRISPR-Cas9, to cure sickle cell. 

GENE THERAPY TO THE RESCUE!

The most encouraging human data so far have come from a genetic therapy being tested by Cambridge, Mass.-based Bluebird Bio. In March, the company reported that a boy who received the gene therapy in October 2014, when he was 13, had been able to stop taking medication that helps alleviate symptoms and has not needed to be hospitalized with a sickle cell crisis (as Prodigy was in the days before he died). Nor has he suffered the crushing pain or bone and tissue damage that results from the inability of sickled blood cells to carry oxygen.

STATNEWS.COM

“People say we’re within 10 years of reaching the goal of a cure, and maybe less.”

Dr. Charles Abrams of the University of Pennsylvania and past president of the American Society of Hematology

THE END

10 years or less? That’s incredible news! While we wait patiently on this side of the globe for a possible cure in the not so distant future, you have that absolute responsibility to make good choices in choosing a life partner! If you are a sufferer, keep hydrated, and get help fast when in crises!

Photo Courtesy Lemuel Hearts Advocacy for Sickle Cell Anaemia

You can join the discussion and support the awareness campaign by following the amazing woman and doctor championing the advocacy for Sickle Cell Anemia awareness. Follow her facebook page.

Dr. Yvonne Woyome

Facebook page: https://web.facebook.com/yvonneantwikusi

VERDICT: MYTH!!!!

Got any questions on Sickle Cell Disease? Feel free to leave a comment in the Comment section below, and don’t forget to subscribe to notifications(for comment replies).

Cheers, DrC.