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Living with and managing sickle cell disease

By Tayo Oredola
21 October 2018   |   4:38 am
Timileyin Edwins is the co-founder of CrimsonBow Sickle Cell Initiative (SCI) a non-governmental organisation (NGO). She is a 31-year-old sickle cell warrior who, in spite of all odds, has managed to live “a normal life” as opposed to what the society...

Creating awareness on sickle cell anemia

• Stigmatisation Worsening Pains, Travails Of Persons Living With Sickle Cell Disease
• Nigeria Now World’s Capital Of SCD
• Victims Of Genetic Disorder Clamour For More Haematology Clinics

Timileyin Edwins is the co-founder of CrimsonBow Sickle Cell Initiative (SCI) a non-governmental organisation (NGO). She is a 31-year-old sickle cell warrior who, in spite of all odds, has managed to live “a normal life” as opposed to what the society thinks about persons living with the genetic condition.

Edwins like others battling the condition, despite being in the 21st century with perceived remarkable scientific progress in the understanding of Sickle Cell Disease (SCD), are still subjected to diverse forms of stigmatisation by the society. Sickle Cell Disease is a hereditary blood disorder, caused by an abnormality in the oxygen-carrying protein haemoglobin found in the red blood cells. The abnormal haemoglobin because of the mutation forms crescent or sickle shapes, which make it not flexible, like the normal oval shaped haemoglobin.

The non-flexibility of the sickle shapes can cause it to easily stick to the vessel walls to cause a blockage that hinders the flow of blood. When this happens, oxygen can’t reach nearby tissues, and this manifests in either acute or chronic pains, especially at the joints, which is referred to as crisis, the hallmark of the condition.Warriors as the sufferers are often called are confronted with societal stigma, where people wrongly assume that each of them has a date with death sooner than later.

According to Edwins, Nigeria is the capital of sickle cell in the world because it has the largest population of people living with the condition worldwide, but owing to widespread stigmatisation, a lot of them stay quiet, suffer and die in silence.The CrimsonBow’s boss whose mother happens to be a sickle cell warrior in her 50s, runs the NGO not only to bring succour to incapacitated sufferers, but to bridge the awareness gap, which has been described by most experts as the greatest hurdle.

She noted that those who assume that sufferers were weak and incapable of being great hold very wrong notion about this class of persons.
Ademola Samson Adewoyin of the Department of Haematology and Blood Transfusion, University of Benin Teaching Hospital, in his research titled, “Management of Sickle Cell Disease: A Review for Physician Education in Nigeria (Sub-Saharan Africa),” said SCD affects about two to three per cent of the Nigerian population. This simply means that about four million Nigerians are living with the genetic disorder.

The scary report added that about 30 per cent of Nigerians are equally carriers of the disease, meaning that more than 40 million Nigerians have the sickle cell trait, with about 150, 000 children born yearly with the condition in the country.In 2006, the World Health Organisation (WHO) declared SCD as a problem of major public health significance and a burden that must be addressed if recent improvements in overall child survival are to be consolidated.

SCD according to WHO is the sixth commonest cause of death among Nigerian children, as an estimated 6.4 per cent, or no fewer than five deaths are from the disease.People with the sickle cell traits who are also referred to as carriers of the disease are those who have one sickle haemoglobin gene (S) and one normal gene (A), thus AS.

SCD, termed an ailment of the blacks, is one of the most common genetic diseases worldwide, with between seven to nine per cent of the world’s population as sufferers and it predominates the sub-Saharan Africa, East Mediterranean areas, Middle East, and India.Thus, the high prevalence rate in Nigeria, being the most populous black nation in the world is not accidental. Some advocates argued that despite its prevalence in the country, way more than cancer and HIV, yet it doesn’t get more publicity and funds.

Chief Consultant Psychiatrist and Head of Child and Adolescent Unit, Federal Neuropsychiatric Hospital, Enugu, Dr. Muideen Bakare, is a SCD warrior in his 40s and he told The Guardian that living with the condition is tantamount to dwelling in challenges and overcoming them amid stigmatisation and discrimination.He said stigmatisation is one of the cardinal things to fight when it comes to the scourge of SCD among African children because the world view of Africans had long been manipulated and taught to embrace stigmatisation and the trend could be found in most Nigerian societies.

Bakare in his book titled, “A Story of Sickle Cell Disease, Stigma, Poverty, Hope and Resilience in Sub-Saharan African,” recalled how his childhood was laden with suffering from the condition amidst poverty and the associated stigma.

According to him, many around him thought he would not survive into adulthood, a development, which he says is a common observation among children with SCD born and raised in sub-Saharan African countries, including Nigeria.Bakare whose father was an SCD warrior too described the condition in his book as fighting a war, stressing that the condition remains a battle of the physical and psychological.

“In my primary school days, my classmates gave me a derogatory nickname ‘ominikun’, meaning water filled abdomen because of my big stomach which was caused by my enlarged spleen, a results of the condition. So I had little confidence, as inferiority complex and low self esteem were my companion.”

The consultant psychiatrist, who was motivated to read medicine because of his frequent hospital visitations, recollected how his third and fourth years in the medical school were quite eventful as a result of crises, occasioned by excruciating pains, despair, and how a medical doctor at Jaja Clinic, University of Ibadan (UI) told him, ‘you are a sickler, what are you doing studying medicine and surgery in the university, you that would soon die.’

Bakare encouraged warriors to think highly of themselves, be positive and courageous, not only to face the condition and move ahead, but also to overcome what people say, stressing that doing so would lead to greatness. Also narrating her story, Omolola Ogunrinade who has been living in South Africa for 14 years, where she moved to from Nigeria, explained that after being diagnosed at five, the condition forced her to develop the kind of inner strength that helped her overcome fears, pains, stress and ridicule.

“I spent most of my childhood days being bed-ridden, and crying as a result of excruciating pains from crisis, and I was always laughed at in school for my swollen hands and legs, and for carrying my medication in makeup purses.” Ogunriade who developed avascular necrosis (death of bone tissues due to lack of blood supply) at the hip had to undergo two major hip surgeries that have left her limping, and for her, “each day is a struggle with physical pains, especially in my hip.”

She cited instances where some organisations rejected her when she was job hunting in Nigeria saying, “We are often marginalised in the society and this makes it difficult for us to even find the right life partners.”

Comparing her experiences between living in South Africa and in Nigeria, she explained that the ailment is invisible in South Africa since so many people don’t know about it. This she noted is the cover for her. Currently working with an NGO as a project coordinator, she believes it is easier for her to manage the condition in South Africa because it is “well managed here. I have relative peace living here because I am not labeled.”

Mrs. Adesola Ekem, mother of eight-year-old Ebere Ekem, who is living with SCD, feels helpless whenever her daughter is in crisis because she can’t stand seeing her wail while in pains.“Ebere was diagnosed as SS at four, but her twin sister is AS. I felt very bitter and didn’t want to accept that, but I couldn’t watch my child die, so I accepted fate and started finding help for her,” Mrs. Ekem told The Guardian at her residence in the Isolo area of Lagos State.

Ekem, a petty trader and tailor admitted to being ignorant of her genotype and that of her husband until their daughter was diagnosed with the disorder.Apart from the exorbitant cost of managing her daughter, especially her medication and diet, Mrs. Ekem also confessed to being very protective of her baby owing to fear of her being stigmatised and discriminated against until she was more enlightened.

Having herself somewhat liberated, she now attends the sickle cell clinic at the Isolo General Hospital on Wednesdays for further education on the genetic disorder and medication for little Ebere. While applauding the initiative, she appealed for government to create more clinics because the ratio of health personnel to patients remains yawning. In spite of the dire health challenges that she has to battle with, Ebere’s teacher says she is glad to have her in her class because she is “a gentle and good student.”

She added that when her parents reported her condition to the school authorities, Ebere’s seating position was changed to a more ventilated location in order to enhance her comfort.“I also monitor her play with other students, as well as, ensure she hydrates well as instructed by her mother,” said Ebere’s teacher.

Senior Lecturer and Consultant Hemoto-Oncologist at the Department of Child Health, the University of Benin Teaching Hospital (UBTC), Dr. Magdalene Odunvbun, affirmed that children with the condition should be feed between five to six times daily with nutritious and balanced diet because unlike other children, their bone marrow works extra to produce red blood cells between 10 to 20 days instead of the normal 120 days.

She stressed the importance of balanced diet to their system because red blood is what carries oxygen in the bloodstream to remove carbon dioxide.During an interaction with another person living with SCD, who is currently battling end stage kidney disease as a result of excessive ingestion of analgesics to suppress crisis pains, she narrated how her mother and sisters in-law were mean to her and called her derogatory names like agbana (spendthrift).

The lady, a medical doctor, who prefers to remain anonymous, recalled how her marriage lasted only a year and four months because of undue interference by her in-laws.“And now I am back home struggling to survive as I have been abandoned by my husband.” Thirty-seven-year-old Henry Nwokobia, a laboratory staff at the Lagos University Teaching Hospital (LUTH) is lucky not to experience any form of stigmatisation whatsoever at work because there is understanding.

“But one thing about most persons living with SCD is that they tend to try, or are always trying to prove that they are able to do what others are doing. And for some, that constant trying leads to crisis, while for others like me, we go the extra mile but never wind up with crisis as a result.”

Nwokobia who recalled how constant crisis affected his life in his 20s, expressed disappointment that most warriors find themselves striving to prove to the society that they are up to the task, even at the detriment of their health most times.“Others find themselves trying to prove to their bosses they are not pity employees, but capable and qualified ones like other staff, and some of us are caught up in trying to prove to a prospective spouse that we are strong and not lazy or too sickly to avoid being rejected,” he added.

He said: “A number of my female friends have faced stigmatisation from mother-in-laws and husbands’ relatives, and for most of us, it’s like no fertile soil for true love to sprout,” he stated.He admitted falling into depression once in a while and even losing the zeal to go ahead with life years ago. This, he said led to him not caring much for himself even though he knew the implications were dire for his general wellbeing.

Dr. Bakare shares Nwokobia’s view when he maintained that often, sickle cell persons face adjustment problems, which ultimately lead to depression, in addition to the anxiety of what tomorrow holds for them. In extreme cases, this leads to suicide. According to him, the age range most prone to such psychological adjustments is the adolescent stage because that is the period where an average sickle cell person is open to depression and anxiety because they begin to want to discover themselves.

He said it is appropriate for sickle cell patients to go through psychological counseling in many areas at a point in their lives in order to build up on their complexes.Unfortunately, despite the country having the highest number of people living with this condition, little is being done to curb further occurrences as awareness is still lacking.

For instance, SCD forms a small part of the clinical practice of most general duty doctors in the country, and there is gross absence of dedicated sickle cell centres. Thus, making it difficult to keep abreast of current knowledge and practices in the treatment of the condition.

Edwins is particularly dissatisfied with the support of government to the condition, describing it as poor, and responsible for lack of specialised care for sufferers.She considers herself lucky to spend an average of N5, 000 on medication monthly, and when she has reason to visit the hospital, expenses are covered by her HMO. The average Nigerian sufferer is not this lucky.

There are also not enough general practitioners in the nation talk-less of SCD specialists. This is why Nwokobia is advocating that government should build more heamatology clinics with state-of-the-art equipment, manned by trained sickle cell health personnel. He suggested that such clinics should be located in every local council in the country, or one built to serve two local councils, as well as, equip primary healthcare centres to handle such cases in order to ease the burden on secondary and tertiary centres like LUTH.

The Chief Executive Officer (CEO), Sickle Cell Foundation of Nigeria, Dr. Annette Akinsete, is full of commendation for the Lagos State government for the N200m support to facilitate the establishment of a bone marrow transplantation unit at the Lagos University Teaching Hospital (LUTH).

Describing it the highest a government has done in support of the course, she informed The Guardian that it was high time the government stepped up to the plate and walk the talk by making provisions to fund sickle cell programmes, by supporting NGOs that already doing the work. The new transplant centre in LUTH is expected to commence work by the end of this year.

She said it was appropriate for governments to come up with appropriate policies, and show directions for them to follow, just as she expressed that they would be willing to join forces with governments to implement such as foot soldiers. On the Genotype Screening and Test Bill, which passed its second reading at the National Assembly since last year, an indifferent Akinsete remarked that the bill would only just help in putting a law in place, but would not help in taking care of sufferers.

But the Executive Director (ED), Sickle Cell Advocacy and Management Initative, Miss. Toyin Adesola, who is also a sickle cell warrior in her 50s has a contrary view. In fact, she is of the view that even though the bill is in the right direction, the right people should be involved because he who wears the shoes knows where it pinches most.

Adesola stressed that handing the entire process to those that do not know sufficiently about the disorder or what sickle cell is all about won’t help matters in any way. Instead, government should consult people living with the condition, and who understand the condition better, “else the bill would not mean anything and won’t have any relevance.”

Akinsete’s position appears to be shared by CrimsonBow’s executive, Edwins, who also commends the government for bringing up the bill, but doesn’t think it would address salient issues affecting sufferers because “the bill is like an infringement on people’s rights of choice.” Instead, she urged government to focus more on awareness creation, which would help people to make informed choices, because the disease has remained endemic in the country due to lack of proper awareness, funds and government policies.

The last few decades have witnessed remarkable scientific progress in the understanding of this genetic disorder and improved clinical insights have heralded developments in that area. Modifying interventions such as chronic blood transfusions, hydroxyurea therapy, and haemopoietic stem cell transplantation have also been made.

Haemopoietic stem cell transplantation popularly known as bone marrow transplant, a very expensive procedure, which is affected by age in most cases has, however, been proven by studies to be the only permanent cure for the condition. However, a source from the UBTH told The Guardian that Dr. Godwin Bazuaye, the first Nigerian doctor to carry out the procedure in 2011 at the hospital is in the process of establishing a private centre due to lack of government’s support for the haematology unit.

Despite all the scary stories about the disease, 47-year- old Mrs. Bridget Augustus, a sickle cell carrier believes that it is not all doom for sufferers because with appropriate information, care, support and the God factor, there is hope for sufferers. She also wants the society to stop stigmatising persons living with the condition.