#HEALTHTALK 15: "The pain of sickle cell motivates me": Pearl Makinde shares her story

Blood is the fluid found in the body of vertebrates. It is the transport medium in the body which carries oxygen and nutrients to the cells, while also bringing out metabolic wastes.

Genotype is the genetic make up of an individual. Humans have different blood types. There are three different alleles for human blood types known as Alleles A, B and O.

Human blood type are determined by co-dominant alleles. An allele is one of several different forms of genes that is responsible for variation in heredity. It is found in the DNA. Every individual  must have inherited them  from their mother and father.
Possible blood types are AA, AO, BB, BO, AB, and OO.

This special edition of #HealthTalk features Titilayo Pearl Makinde, the President of Ilorin Warrior's Hub, and the founder of Pearl Sickle Cell Initiative, in the maiden publication of the #HealthTalk Interview Series.

According to Makinde, "Pearl Sickle Cell Initiative is a non-profit organization that stands for advocacy and management of sickle cell, and is currently based in Kwara state".

"Ilorin Warriors Hub was born out of the Pearls Sickle Cell Initiative, and is focused on being a support system for persons living with sickle cell in Ilorin strictly."

"The group offers medical as well as psychosocial support to persons in the hub. We hold programmes to discuss what we cannot discuss with people who do not live with sickle cell and share ways to overcome stigma and lots more."

During the interview, she says a lot about what sickle cell means, it's effects and treatments, and the work she and her team have been spearheading at the Ilorin Warrior's Hub and the Pearl Sickle Cell Initiative, to promote sickle cell awareness, management and prevention in Kwara state.

This article is based on that interview, and the full video is provided herein also. Please watch and also subscribe to our YouTube channel to get notified whenever new videos are released.

Sickle cell anaemia

A normal red blood cell takes the shape of a circle, but when they take a Cresent or sickle shape, they become sickle sells. If that happens, red blood cells will be in short supply in the person's blood.

Insufficient red blood cells causes anemia.  It may interest you to know that your body produces new cells daily, and the red blood cells on the average live for 120 days. This is why blood donation is good .

Sickle cell is a blood disorder characterised by abnormal shape of cells.

Normal red blood cells are round; but in people living with sickle cell, the shape of the cells form a crescent or sickle shape as earlier said.

Sickle cell is inherited when parents with incompatible genotypes come together to make babies.

In sickle cell, the cells, due to their shape, get clogged and cause pain episodes. This is commonly called crisis, and the pain varies in levels. Depending on the patient, the trigger and other factors, the pain can vary from mild to severe.

Sickle Cell is a blood disorder and this means the individual gets to live with the disorder daily.

Effects of sickle cell

The impacts are numerous. Sickle cell is life-threatening and very expensive to manage.

There are different kinds of crisis or sickle cell complications. But the most common is the pain crisis.

When blood can't get to parts of the body due to clogs or blockages, pain may occur in that part of the body, and is usually caused by certain triggers.

The pain may last from a few hours to days or even months, and severity differs for individuals.

Sickle cell pain crisis are indescribable. In the video, Makinde, a sickle cell Warrior herself, explains it by comparing it to using a hand blender or grater to blend.

She paints a mental picture of someone holding the grater and forcefully grinding a human being's fingers slowly, and then decides to go throw the victim into a grinding machine.

One can only imagine!

She goes further to say that, "Until one is in pains, they may never really be able to explain it; and when one is in pains, you are focused on the pain and not how to describe it".

Free genotype tests are organized yearly by the Pearl Sickle Cell Foundation in different communities in Kwara state. 

Triggers

Anything can trigger a crisis. They include cold, heat, high altitudes, infections, dehydration, stress, etc.

Unfortunately, no matter your level of carefulness to avoid your triggers, still, you come down with a crisis.

Who is a sickle cell Warrior?

Pearl explains that the word 'Warrior' is globally adopted by the sickle cell community for people living with sickle cell.

This was aimed at replacing the word 'sickler' which people who live with sickle cell consider derogatory.

Are Warriors stigmatized?

Stigmatization of Warriors happens everywhere and everytime, says Makinde. "At the hospital, market place, schools, religious centres, workplace and even in the family."

However, the most common ones are employer stigma and marital stigma.

Because they are believed to be unable to work or deliver, employers often decline an offer of employment upon discovery of the medical condition of sickle cell Warriors.

Also, because of the belief that Warriors are frail and sickly, most employers reject people living with sickle cell at the mere mention of it.

Sickle cell statistics

According to Makinde, the total number of sickle cell births worldwide and annually amounts to 300,000 (see WHO data), and Nigeria alone accounts for approximately 150,000 of these births; not just in Africa but in the world!

This is a whooping 50% of sickle cell births worldwide! Among other things, this is a testament to the fact that most Nigerians do not take genotype compatibility as a serious consideration in choosing a spouse and/or making babies.

There is a need to create awareness and to put a stop to this.

Sickle cell awareness programs are being organized in schools and religious centers by the Pearl Sickle Cell Foundation 

The statistics are high in Nigeria due to ignorance, teenage pregnancy, pregnancy from rape, religious beliefs, and the belief in genotype change.

Also, for a country with the highest number of sickle cell births, one can tell that the number of individuals living with sickle cell in such a country would be outrageous. According to the Coalition of Sickle Cell Non-governmental Organisations in Nigeria, there are about 40 million healthy carriers of the sickle cell gene in the country.

This is why the need to run inclusive sickle cell awareness projects to cater for people who live with the disorder, and to dispel myths around it cannot be overemphasized.

Something needs to be done.

Sickle cell myths

People have different beliefs about sickle cell anaemia.

Number one myth is usually that people who live with sickle cell die young or early.

Fact: This may have been true in the days of low medical and technological advancements. But today, there are medical options and technological upgrades that have helped many patients to manage the disorder better. There are sickle cell Warriors in their 50s, 60s and even 80s who are well and alive currently.

Other myths are:
People who live with sickle cell are infertile. But that's not true.

That people who live with sickle cell should not receive educational support.

That people who live with sickle cell are always thin, slim or lanky. This isn't true as there are lot of sickle cell Warriors who are not lanky nor slim, but plum.
Therefore, genotype can only be determined by a blood test and not by the observation of body size.

Sadly, some religious organizations who ask for genotype test results of intending couples do so late, especially when it's barely weeks to the wedding and almost nothing can be done.

Treatment

Some Warriors may not need hospitalizations during their lifetime. But others may need it frequently. Some may also need blood transfusions yearly or bi-monthly.

Sickle cell complications are numerous and management for them may be expensive.

Some sickle cell warriors live with multiple Complications daily, such as Leg Ulcer, Avascular Necrosis, Priapism, Retinopathy, etc. And all of these require certain treatments.

For those with leg ulcer, their average cost of healthcare would differ from someone with avascular necrosis and osteomyelitis at the same time.

For leg ulcer, one is looking at cleaning the ulcer atleast twice a week. With avascular necrosis, it's an extra cost.

Managing sickle cell can be too expensive. Monthly routine drugs require between N2,000 - N30,000, depending on the supplements the individual uses.

Intending couples with genotypes such as AS, AC and AB or other variants with a trace of sickle cell are advised to marry people with AA genotype. This should help in bringing sickle cell to the barest minimum.

Also, early diagnosis, can save lives. Get your baby tested.

How should sickle cell Warriors be treated in the society? 

Everyone must see them first as HUMANS who deserve to be given equal opportunities to live, just like other humans too.

In the same way that stigmatization against those living with HIV/AIDS was tackled through aggressive campaigns, stigmatization against sickle cell Warriors should also be fought against.

Titilayo Pearl Makinde and her team during a Drug Donation at the University of Ilorin Teaching Hospital, Kwara state. 

There should be more support from the government and NGOs, for those living with sickle cell, especially through the provision of free drugs, legal protection from discrimination/stigmatization, etc.

In a nutshell about sickle cell

Sickle cell is inherited when two parents with the sickle cell trait come together to make babies. Or say when two people with incompatible genotypes come together to make babies, they are at risk of having a child with sickle cell.

There are over 700 different types of genotype in the world.
But the most common six types in Nigeria are: AA, AS, AC, SS, SC and CC, with some traces of SB and AB.

From the above, it is clear that SS is not the only sickle cell type in the world, even though it is the most common type in Nigeria. There are people who are SB+ in Nigeria but are very rare to find.

Variants of sickle cell are SS, SC, SE, SO, SD, SB.

So, if someone tells you they have sickle cell, it doesnt mean they are SS, they may be one of the other variants. Only a proper genotype test will tell which they are.


Lilian Eze is a Nutritionist-Dietitian and teacher in Lagos, Nigeria. She is also a Lead Content Creator at The Paul Anunaso Blog, and can be reached at lilian.thepaulanunasoblog@gmail.com

Chukwubuikem Paul Anunaso is a CNBC Africa opinion writer and civil/structural engineer in Lagos, Nigeria. He is also the editor of The Paul Anunaso Blog, and can be reached at anunaso.cp@gmail.com