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Dealing with Sickle Cell Disease in Modern Society
Despite counseling by medical experts to intending couples not to marry partners with incompatible genotype so as to reduce cases of sickle cell anaemia, over 150, 000 children are born annually in Nigeria with the disease according to World Health Organisation records. Blessing Ibunge reports on the inherent challenges therein
A non governmental organisation, Nirvana Initiative in partnership with Hacey thought it wise to sensitise the public on sickle cell disease and best way to manage the crisis if one is a carrier.
The group through Sickle Cell Awareness and Health Foundation, stormed the University of Port Harcourt Teaching Hospital (UPTH) where many with sickle cell trait and their parents disclosed their challenges.
Understanding the Sickle Cell Disease
Sickle cell disease is an inherited blood disorder, caused by flawed hemoglobin, an essential component of the blood largely composed of iron and protein.
Dr Casmir Ifeanyi, an Infectious Disease expert and Moleculera Biologist, who explained how a person can inherit the disease said “The protein in the globin has two chain, the alpha and the bitter chain. The globin comprise of amino acid and each of them have their positions. So usually, on the sixth position of the amino acid chain, is glutamic. Is a mutation which can be a disorder arising on its own, there can be substitution of glutamic acid with valine.
“When that happens from one parental source, then the person will have the carrier status which is the X-gene in one. But when it happens and the individual is unfortunate to inherit this abnormal hemoglobin from both parent, it means the person will have the homozygous status of SS and that means the person will now have the sickle cell disease”.
“When that sickle cell happens the blood cell are unable to pass freely in the blood vessels. This can lead to a whole lot of disease condition: pain, hemolysis and even stroke and death.
“So, because there are triggers like malaria, infection and stress, which will potentiate or increase the likelihood of it sickling when it discharge with oxygen to deliver at the tissue”.
Speaking with THISDAY on the status of Sickle Cell disease in Nigeria, he explained that it is largely a preventable disease, stating that the approach to it is premarital counsel when couples are advised to know their genotype and follow the one that is compatible.
Dr Ifeanyi further explained that “If an individual is AA, that individual can marry anybody. If an individual is AS that individual must not marry AS because if that happens that individual will have the chances of producing by probability one SS, two AS and one AA per every probability of one over four child birth.
“But the point is that this is not like a normal probability where an individual determines what happens. So you can even have four life birth and all of them will be SS and is why it is advised that people who are AS do not go ahead to marry same genotype partner.
“The person who is AS can safely marry someone who is AA and you wouldn’t expect them to have an SS.”
Dr Ifeanyi also disclosed that sickle cell disease is more prevalent and predominant amongst the West Africa Coast, largely Africa and India; adding that the thalassemia disease is common along the Mediterranean and Arab.
“There are local and international data of people living with sickle cell disease. Example, according to the World Health Organisation (WHO) about 150, 000 children are born annually in Nigeria with sickle cell disease.
“Out of this 150, 000 over a hundred thousand of them do not celebrate their fifth birthday. So, it is one of the leading cause of deaths amongst children. And now, the challenge is that many of them who got to do their fifth birthday, some do not progress beyond 25 years, the fact is that a sickler is perennially ill disposed, debilitated.
“The point is that AS person marrying a AS person is creating a problem to themselves not only but a societal problem, they are going to put more burden on our very wicked structure and in Nigeria we do not have an advanced technology and facilities to manage sickle cell disease.
“In developed world today, you can do a stem cell therapy, you can do a bone marrow transplant and you can resolve the diseases of sickle cell but you will not be able to change the Hemoglobinopathy”.
Possible Intervention to Reduce Cases of Sickle Cell Disease in Nigeria
In as much as the federal government is tackling some challenges as regards to security of the country, politics, oil and gas, Dr Ifeanyi has noted the social economic implications and the health burden associated with sickle cell as better imagine than experienced.
Dr Ifeanyi said is a preventable disease condition, just as he stressed that Sickle cell disease is real, adding that “the disease is debilitating. A sickle cell offspring destabilize and can destroy marriages”.
He urged that “There is a bill actually about sickle cell disease trending in national assembly. I expect that the bill should expeditiously passed, but one of the essential thing that should be in the bill is that those who are with incompatible genotype should not be allowed to marry in Nigeria”.
Experiences of People Living with Sickle Cell Disease
At the sensitisation and give out food package programme by Nirvana in Port Harcourt, some people living with SCD, as well as their parents shared their experiences.
When asked how she feels amongst her peers knowing she is living with sickle cell trait, Miss Prayer Orounwo, a 29-year-old graduate of Agricultural Science, stressed “its actually about yourself. I have grown to have self confidence.
“My genotype is something I tell somebody at the first time. As my friend, I will want you to know everything about me should in case something happens, I won’t want you to be ignorant of what to do. I don’t feel ashamed of it because is not a death sentence at all. So I live my life normally, I go out, I have friends, I go to work.
“I had my last serious crisis when I was in primary six, I think I was 12, I was writing my common entrance my mum had to be going through and fro with me. I didn’t stay in the hospital because I don’t like staying there, it makes me get sick the more.
“But since then, the only thing I feel is headache, dizziness, tiredness maybe when I have stressed and I know what to do most time. I try to take a long rest, I eat, I get blood belting. So its been really good with me (she laughs)”
Miss Nwokocha Goodness, a 22 year old undergraduate of English Language, Rivers State University, expressed “When I was younger I was feeling devastated, sometimes frustrated but as time goes on I start feeling better.
“I am not depressed as before because there are situation that are worse than my own. I just take life the way I see it, I appreciate it.
“My parent feel down knowing I have sickle cell disease. Most times my mother blame herself for being ignorant about the whole situation but even at that she still takes care. She does what needs to do to help me relief the pain”.
Goodness who felt bad that her crisis always arises during her birthday explained “Before I used to have crisis every month but now is when I stress myself much, examination period and my birthday, every May. Anytime my birthday is coming up the crisis increases and it become torturing”.
Also sharing her experience Somto Omekukwu, 22 years years old and a graduate of Plant Science and Biotechnology, said being a sickler has limited her from things she passionately desire to do.
“I have been relatively stable most of my life but it has its challenges. Sometimes plan something and you woke up not able to do it that day. You learn to manage the challenges and move on with your life.
“I like sports but most time I am not able to do as much as I was to. My parent realized that I am a sickler when I was about a year old and my mum was already pregnant with my little sister who also had sickle cell.
“My immediate younger sister died about five years ago but I have another sister that have sickle cell disease, we were three but one had died”.
Rukvwe Aride, 27 years old graduate of Biochemistry and Chemistry Technology, recalled how he felt the first time he knew about his status.
Aride who a serving National Youth Corp Member in Rivers State, expressed “I felt not really good when I knew about my status because I am wondering why me” We are three in my family by my mum: my elder brother is AS, my younger brother is AA and I am SS.
“But as time went on, I began to live with it and felt this is my fate, so I had to work on myself. The crisis usually come when I was much younger because of academic stress but I learned how to control myself, taking a lot of water, vegetables and basically taking my drugs at when due.
“When relating with my peers I feel timid sometimes but I found out all about it myself. I knew how to relate with my friends. I knew who to tell or who not to tell”.
A mother of eight years old disclosed to THISDAY how his family sold a landed property to save their son.
“I noticed my son is a sickler when he was 8 months old. Then, sometimes the body will swollen, he sometimes cries too much, so we brought him for test in UPTH where he was discovered to have sickle cell disease.
“Whenever the crisis come, we normally bring him to hospital in two to three months interval. The situation has cost us so much, we had to sell our land property during one of the crisis to save his life”.
Commemorating World SCD Day
Meanwhile, Dabata Omubo Pepple, Executive Director of Sickle Cell Awareness and Health Foundation, explained that the gesture by the NGO to reach people living with the disease was in commemoration the world sickle cell day.
Pepple who is a nurse with UPTH stated that “Is a day set aside to create awareness about the disease, to remind people that the disease is congenic condition and not a contagious disease. It is preventable. Also to remind people with the disease that they can do alot with their life”.
The nurse further revealed: “The Nirvana Initiative is doing a welfare package. A way of reaching out with people living with sickle cell disease with a welfare package which has to do with food reminding them that food is important in their day to day and also to support them”.
According to WHO, about 150, 000 children are born annually in Nigeria with sickle cell disease. Out of this 150, 000 over a hundred thousand of them do not celebrate their fifth birthday. So, it is one of the leading cause of deaths amongst children