Sickle cell disorder: What options are there for warriors?
Growing up with Sickle Cell Disorder for Chimezie Agumbah, a Lagos based warrior used to be liken to living with a death sentence hanging over her head.
This pathetic victim of circumstance who was just so unlucky used to throw countless pity parties, while the only people that turned up were pain, sadness, grief, doubt and a deep dark fear of what might or might not be beclouding her sense of judgment.
While narrating her warrior story, Agumbah said, “I can categorically state that this journey hasn’t been one bit easy, but one thing I never asked was why me? Because I wouldn’t pray this on anyone else especially my siblings. I used to wish I could take their pain and add it to mine; sometimes I felt their pain, by mere association”.
“In secondary school, there was this literature book we were reading titled “Mezie the ogbanje boy”, my classmates changed it to Mezie the ogbanje girl because of how I would be healthy one minute and reeling in pain the next, an endless circle which most people didn’t understand,” said Agumbah .
Speaking further, she opined that her journey was uncertain and scary; however, she enjoyed the best support system from her parents. “My family is the best, and my very small circle of friends. My parents care tactics is on point, and I wouldn’t trade that for anything. Somethings I have been tempted to just blame them and withdraw, but their love and care won’t even give room for that. I don’t regret being born with SCD, my only regret is that I let my insecurities and fears keep me down for a long time.
“I have always been closeted about SCD, but I feel now is the right time to come out and lend my voice and support in any way I can. Encouraging others is akin to encouraging myself because the battle is real. I’m a warrior and warriors don’t cringe from battle, but most importantly I’m a victor because God’s got me. My faith…His mercy, I don’t want to die fighting, I want to live winning.
“Near death experiences, major surgeries, organ removals, the stigma and shame, the fear that keeps us from soaring and just being who God has called us to be; this and countless more can’t be said in one sitting, all warriors have a different story to tell and need to tell it, the world needs to hear us.
“Even with all the awareness out there, children are still being born with SCD…the people doing this need to hear and see what “We” go through so we can end this once and for all. Everyone should be allowed to come into the world with a chance at living and not a death sentence hanging over their head,” said Agumbah as she calls for support to eradicate SCD.
Living with a cell disorder could be a challenging moment for parents, care-givers, and guardians. However, to help reduce and share such burden, the global community comes together June of every year to create awareness on Sickle Cell Disorder, cell distortion that is caused by inherited abnormal hemoglobin, which causes the oxygen-carrying protein within the red blood cells to malfunction. 19 June is therefore celebrated as World Sickle Cell Day, a day set aside by the United Nations to create awareness about sickle cell disorder.
The World sickle Cell Day is to spread accurate information about sickle cell anemia, aimed at educating the public on the misinformation about sickle cell as a death warrant that has been widely held among the generality of Nigerians.
Nigeria joined the rest of the world to commemorate the 2018 World Sickle Cell Day as designated by the United Nations to draw attention and create necessary awareness to the problems posed by sickle cell disorder.
Toyin Adesola, Chairperson, Coalition of Sickle Cell Disorder NGOs, Lagos State/Executive Director Sickle Cell Advocacy and Management Initiatives (SAMI) in commemorating the World Sickle Cell Day said the coalition is working to sensitive Nigerians on the need to recognise the disorder as a major public health problem in Nigeria.
According to her, about 1-in-4 people in Nigeria are healthy carriers of the sickle cell gene and over 150,000 children are born annually with symptomatic sickle cell anemia, which corroborates with Kharya statement on the high prevalence of sickle cell in Nigeria.
Annette Akinsete, National Director/CEO, Sickle Cell Foundation of Nigeria said the Red Umbrella Walk is to mobilise everyone including the government to pay attention and fund projects on sickle cell disorder. “The coalition of NGOs is to strengthen our campaigns and we believe that this will make our voices to be louder to attract the attention of the government and corporate organisations,” said Akinsete.
Sickle Cell Anemia: disease or myth?
Sickle cell anemia (sickle cell disease) is a disorder of the blood caused by inherited abnormal hemoglobin (the oxygen-carrying protein within the red blood cells). The abnormal hemoglobin causes distorted (sickled) red blood cells, which make the sickled red blood cells fragile and prone to rupture.
When the number of red blood cells decreases from rupture (hemolysis), it results in anemia. This condition is referred to as sickle cell anemia. The irregular sickled cells can also block blood vessels causing tissue and organ damage and pain.
Sickle cell anemia is one of the most common inherited blood anemias. The disease primarily affects Africans and African Americans.
Over the years many people have held the opinion that sickle cell is an attack from the village, with little or no knowledge that it is actually an inherited disease that affect the red blood cells in the human body.
Sickle cell anemia is inherited as an autosomal (meaning that the gene is not linked to a sex chromosome) recessive condition. This means that the gene can be passed on from a parent carrying it to male and female children. In order for sickle cell anemia to occur, a sickle cell gene must be inherited from both the mother and the father, so that the child has two sickle cell genes.
The inheritance of just one sickle gene is called sickle cell trait or the “carrier” state. Sickle cell trait does not cause sickle cell anemia. Persons with sickle cell trait usually do not have many symptoms of disease and have hospitalization rates and life expectancies identical to unaffected people. When two carriers of sickle cell trait mate, their offspring have a one in four chance of having sickle cell anemia. (In some parts of Africa, one in five persons is a carrier for sickle cell trait.)
Options available for warriors
Gaurav Kharya, Senior Consultant, head pediatric hematology oncology, immunology and bone marrow transplant, Artemis Hospital, in a statement said 85per cent of sickle cell carrier are in Africa, while Nigeria has the highest burden of sickle cell globally. According to Kharya, genetic matching is key to bone marrow transplant; however, there is currently no local registry for voluntary donor, which is a major challenge in finding donor for bone marrow transplant.
Kharya recounts how blood transfusion was administered two months earlier to bring down his patient’s sickle cell level as direct transplant would have increased a chance of rejection. According to him, the patient’s father (donor) had to undergo high resolution Human Leukocyte Antigen (HLA) to check bone marrow compatibility.
“After 14 days, his chimerism (DNA testing) showed 99.45 per cent bone marrow precursor cells from his father. The patient showed very good recovery and new marrow started working on day 12. He was discharged on day 20, having sickle haemoglobin level decreased with 10 per cent post-transplant. It is almost three years now and the patient has not shown any signs of a relapse,” said Kharya.
According to Kharya, with half-match bone marrow transplant, even patients who have no siblings to donate bone marrow for them can be benefactors of this treatment.
Speaking on the success rate of the bone marrow transplant, Kharya who have been involved with Nigerian warriors in the last three years have a 75 percent success rate, while his patients have enjoyed improved quality of live with no risk to the donor. “Interestingly, 70% – 80% of donors are carriers,” Kharya remarked.
Conditions that promotes Sickle Cell.
Red blood cells are manufactured in the bone marrow. Their unique biconcave shape increases their storage capacity for hemoglobin molecules that carry oxygen. They also make the cells pliable and soft so they can squeeze through the tiniest blood vessels in the body. In sickle disease, the red blood cells form an abnormal crescent shape that is rigid, causing the red blood cells to be damaged.
The cells are not malleable enough to get through tight spaces, and this can increase the risk of forming blood clots in the small capillaries of different organs causing the potential for organ damage. However, the sickling (distortion) of the red blood cells in sickle cell anemia?
Sickling of the red blood cells in patients with sickle cell anemia results in cells of abnormal shape and diminished flexibility. The sickling is promoted by conditions which are associated with low oxygen levels, increased acidity, or low volume (dehydration) of the blood. These conditions can occur as a result of injury to the body’s tissues, dehydration, or anesthesia.