Andrew and Edith Otokhina have lost two children to the sickle cell disorder. And three of their remaining children are also affected. FOLASHADE ADEBAYO writes on the pain that they and other carriers are going through
Love was the motivating factor when Andrew and Edith Otokhina got married in 1979. The lovebirds, who did not know they both inherited the AS gene, went on to have seven healthy children. Or so the couple thought.
Life went on smoothly for the family until October 2010 when Tony, the first son of the family, died. His sister, Mary, also died of complications arising from blood transfusion six months later in March 2011. It was thus an understatement to say the couple were inconsolable.
During the course of these life-changing and tragic events, the family went for a diagnosis where it was discovered that only two of the surviving five children are free of the sickle cell disorder.
Speaking to our correspondent recently in Lagos, Andrew, 65, and Edith 54, shared their touching story and how they emerged from the long night of grief.
“It was not a normal thing to check your genotype in 1979. None of the children exhibited any symptom until much later. They were healthy children. All I noticed was that Tony used to have joint pains whenever he played football. We warned him to stop but he would not listen and would go out to play once we were out of the house. Tony died in 2010 and Mary, six months later. Of course, we were devastated. Tony died at 23. He graduated with second class upper degree in Law. Mary was writing her final thesis when she died,” Andrew said.
Quack doctor
For Edith, the fact that Mary died of mismanagement makes her death more painful. “My husband and I travelled to our home town when Mary had a crisis. Her friends took her to a private hospital in Lagos. She told us on the telephone that the doctor told her she needed blood transfusion even though she told him she checked her Packed Cell Volume days earlier. We told her to go ahead because we did not want to lose her. We never knew he was a quack doctor. We rushed back that same day and she told us that we should go home and rest, that she only had chest pain. We left that night, only for the doctor to call us early the next morning that she was dead.
“I almost lost my mind when Mary died. I just went to church and I think I envisioned Mother Mary looking unhappy when her son, Jesus, was being crucified. Then I knew it was God’s way of telling me that I am not alone. Also, my remaining children were very sad. They said my reaction showed that I did not love them as I do their late brother and sister. I had to take heart. However, I would never have married my husband if I had known that he had the AS trait,’’ she said.
Though the couple have three other children living with the disorder, they have since forged ahead by establishing a non-governmental organisation, TonyMay Foundation, in the name of their late children, to sensitise the public and promote advocacy for the sickle cell disorder. Together, and as individuals, the couple noted, the bruises of that traumatic past were healing and they were committed to turning the tide against the disorder.
More Nigerians conquering the sickle cell
Despite cases of fatalities, it has been observed that more Nigerians who inherited the sickle cell haemoglobin are hitting middle and old age. Sitting in the cool ambience of her home in the Igando area of Lagos on Monday, her aged mother napping in a nearby couch, Niniola Philips-Adeleye prepared to attend to this reporter when her phone rang. Ripples of sweat ran down her face seconds after picking the call.
“Please, excuse me. This caller is one of us,’’ she said. Talking to the caller, Philips-Adeleye, spoke in soft tones. “How much is the surgery? N700,000? Don’t worry, I don’t have money but we will rally help. The money will come in good time. The surgery will be successful and you will fare better. I had the same problem in 1995 when I had my last child and my limb was to go for it. My doctors said it was a 50:50 chance and that I might not be able to walk again. But here I am today,’’ she added.
Days and weeks have turned into months and years for Philips-Adeleye, who is living with the sickle cell anaemia. At 51 (she clocks 52 next month), the lady belongs to a select club of middle-aged people living with the disorder in the country. The mother of four has shamed her childhood doctors who told her not to expect to live beyond 16 years.
But the woman also admits that she gave up on herself while growing up. “A doctor told my mother I would not see my 16th birthday. I never knew I would turn 18 years myself. I was so surprised when I got to 25 and you could imagine my amazement when I turned 40, then 50. The older I get, the better I feel. Maybe it is because I have come to know what is good or bad for me. However, I think God is just gracious to me. Look at that picture on the wall. That was my only sister. She died at age 36 of complication from sickle cell disorder,’’ she explained.
Looking at the survivor, her skin and even teeth gleaming against the glow of the afternoon sun, it was hard to believe that she was once a regular face in hospitals. The professional caterer said she had become better with age, and is now able to arrest any crisis before it starts.
“I still have crisis, but they are now very far apart. I have not been to the hospital in the last two years. I was at my church for a conference two days ago and I sensed a crisis. If I had laid down, it would have been a bad case and my roommates would have been scared. But I sat up against a wall and stayed still for one hour. Lying down would have made it worse. Anytime I sense a shooting pain in my spine, I sit up and take a glass of water every 10 minutes. The pain is gone before you know it,’’ she said.
These days, Philips-Adeleye has found fulfilment mentoring anxious children and young adults living with the sickle-cell disorder. But she is not alone.
Music to the rescue
When she clocks 50 in September, the Executive Director, Sickle Cell Advocacy and Management Initiative, Toyin Adesola, will join Philips-Adeleye in the club of middle-aged Nigerians living with the sickle cell disorder. She was born in 1965, when medical and general knowledge about the condition was limited. According to her, she had once contemplated suicide to escape her world of excruciating pain. Since she was diagnosed with the disorder at the age of two, Adesola, who is the daughter of a former Vice Chancellor, University of Lagos, the late Prof. Akin Adesola, has had more than 47 years of leg ulcers, bed sores, pain episodes and sundry infections that come with living with sickle cell anaemia.
Like Philips-Adeleye, Adesola has somewhat developed a coping mechanism for her pains.
“I either listen to music or call my friends. It helps a lot having something to look forward to. When I am in crisis, my laptop is often beside me. If you wallow in self-pity, the pain will consume you. It is a choice as to whether you live or die. I always say that what may kill a person living with sickle cell anaemia depends on the state of medical care from the service providers, the family and the individual himself.
“When I go to your hospital and you do not have oxygen, I will shout. I always tell parents to educate the friends of their children. They will be there when you are not,’’ she said.
Contrary to the long-held belief that people living with the disorder die in their prime, findings by our correspondent indicated that, like Philips-Adeleye and Adesola, more Nigerians are defying the verdict.
In an interview with our correspondent, the founder, Sickle Cell Foundation, Lagos, Prof. Olu Akinyanju, discloses that there are more elderly Nigerians living with the disorder who are quietly thriving in their professions.
“We have a high court judge who retired at the age of 60 years and is now a grandmother. There is a 66-year-old man living with sickle cell anaemia who is on our board of directors. There is a commissioner in Akwa Ibom State. Someone told me that there is a governor who is living and aging well. It is just that people do not want to be identified and you have to respect that.
“But I have also seen lawyers, doctors and dentists who are living well with sickle cell anaemia. They are all into different kinds of jobs. People are living longer because they know what to avoid. If you dance all night, you are probably going to have a crisis the next day,’’ Akinyanju said.
A condition and its many afflictions
According to the World Health Organisation, the sickle cell anaemia is a major genetic disease in many countries in the sub-Saharan Africa. The international body adds that more than 300,000 babies are born with the disorder of the red blood cells each year across the world.
But, with 200,000 children born annually with the condition, Nigeria is adjudged to have the highest number of people living with the disorder in the world. However, the condition affects much more people as more than 40 million Nigerians (about the population of Kenya and the combined population of Libya, Tunisia, Gambia and Senegal) are said to have the AS trait.
With a huge population of people living with the AS trait, experts said the country risks an increased population of Nigerians born with the sickle cell disorder. This, they said, could only be prevented with informed relationship choices and the more expensive prenatal genetic diagnosis for married couples living with the disease.
Indeed, selective relationship has been a recurrent factor in the cocktail of sickle cell prevention and awareness programmes in Nigeria and other affected countries. But, investigations by our correspondent also showed that many Nigerians living with the disorder deal with the daily pangs of health conditions such as physical handicaps, pain episodes, drug addiction as well as discrimination in the family and work places.
It was learnt that cases of leg ulcers, blindness, hearing loss, kidney failure, lung tissue damage, stroke and damage to the spleen and liver abound among people living with the disease.
Experts are also worried that a majority of the sickle cell community, especially children and teenagers, are not only nursing various physical handicaps (some of them nurse leg ulcers for 10 years) but are also addicts of pain killers.
Our correspondent learnt that some addiction cases may result in psychiatry challenges. In a report by two physicians, AB Makanjuola and P. Olatunji, published in the African Journal Online, the experts warned doctors to be cautious in prescribing pain killers for patients. The report is titled, ‘Sickle Cell Anaemia in Patients: A Report of two-case Vignets.’
“Two case reports of Pentazocine abuse seen in a psychiatry clinic at the University of Ilorin Teaching Hospital, Kwara State, were made. It was found that both people who were sickle cell anaemia patients were first given intramuscular Pentazocine in private hospitals during an episode of bone pain crisis. They claimed that though the bone pain crisis subsided after a few days on admission, they continued to feign pains in order to be given Pentazocine because they enjoyed its dysphoric effect.
“There were features of Pentazocine dependence as shown by intense craving for the drug, excessive sweating, body (not bone) pains, signs of needle pricks on the body, sudden extravagance, begging for money, stealing and poor academic performance, among other things. There was no clinical evidence of bone crisis or complications of sickle cell anaemia. It was concluded that more than before, clinicians should be cautious in the use of analgesics, which have the potential for addiction in sickle cell anaemia and chronic pain patients.’’
Adesola, who works with affected children and teenagers, said drug addiction in the sickle cell community was a worrisome trend that might have even led to the deaths of some members.
Like Philips-Adeleye, Adesola said she had mastered the art of managing her crises without resorting to pain-killers, even if it was paracetamol.
“I fear my crisis but I fear addiction more. If you are pumping yourself with narcotics, what happens if you inject yourself with the wrong drug, or if the dose is too high? I am afraid of being dependent on drugs. Some of us got into this because they do not get adequate attention in the hospital. So, they learnt to inject themselves to escape the pain but this is breeding addiction, which is a huge problem on our hands now,’’ she said.
On his part, Akinyanju thinks pharmacies have a share of the blame. He said correspondence with the Federal Ministry of Health and the Lagos State Ministry of Health in the past had not yielded intervention of any kind.
“Addiction is another story entirely. One of these drugs can even be bought over the counter in pharmacies. And these are controlled drugs. We have written to the relevant ministries and all we got was that they were looking into it. Pharmacies are not supposed to sell these drugs without prescription. This should be enforced. Even if you are a doctor but not registered in England, you can’t buy these drugs without prescription over there,’’ he said
The WHO said the burden of sickle cell anaemia was even more telling on children who are under five years, with a corresponding economic frustrations on struggling parents who do not have any social support from government.
For instance, the United States Centre for Disease Control and Prevention reported that the sickle cell disease triggered 75,000 hospitalisation, which cost $475m (about N110 billion which is half of the 2015 budget for the health sector) between 1989 and 1993 in the United States.
While calling on government to inaugurate a social fund for sickle cell patients, Adesola said hospital admissions and cost of treatment would be much higher in Nigeria, which has the highest incidence of the disorder in the world.
“You can imagine the trauma parents go through. I had some form of support because my dad was a professor of gastroenterology and my mother was a nurse. But the general situation is that a lot of families are barely existing. This is a country where the majority live on less than $2 a day. Many families have come to see their children as a burden. If the financial capability is not there, they get frustrated and transfer that to the poor child.
“We know that antiretroviral drugs for people living with HIV/AIDS are from donor bodies, but government should intervene to bail out parents. We still do not have newborn screening mechanisms in this country. So, parents have to wait for a child to manifest symptoms and, unfortunately, it is only early diagnosis and treatment that reduce crises and premature death. I can tell you that there are parents in this country with two or three children with the disease,’’ she said.
An ‘unfinished business’
As far as the Federal Ministry of Health is concerned, sickle cell anaemia in Nigeria remains an ‘unfinished business.’ The Permanent Secretary, Mr. Linus Awute, said this at an event to mark the World Sickle Cell Day last month in Abuja. According to him, the ministry cannot adopt a forceful approach in ensuring that people living with the disorder do not marry. He said government would rather increase the sensitisation and awareness drive in the prevention of the disorder.
“Tackling sickle cell disorder is an unfinished business of the MDGs and would need to be prioritised in the post-2015 development era. Ours is a democracy and you will find that before people talk about marriage, they are already in a relationship. That is why we do not have that kind of confrontational approach.
“We believe strongly that people who marry and later begin to see these complications in their children are usually unaware of what would be the associated consequences of that marriage. For such people, if they were aware, the story may be different,” Awute noted.
While they do not disagree with this approach, activists and experts in the field of human genetics are urging relevant authorities to address pertinent issues such as stigmatisation, addiction, and subsidy for the sickle cell community, among other interventions.
Hear Akinyanju, “We need a sickle cell policy which is a document to which you can hold government accountable. America, with a low incidence rate of the disease, passed a sickle cell anaemia law in 1972 and backed it with $25m for implementation.
“The average life expectancy of sickle cell patients at that time in America was 14 years, now, it is about 60 years. Life expectancy has also increased in Nigeria, but it is not for the entire population of people in the sickle cell community.”
He added, “The problem is that government does not want to spend money. There is no way you will have a health policy without releasing money to implement it. The current governor of Delta State, Dr. Ifeanyi Okowa, sponsored a bill to that effect when he was a senator. But that bill was never passed.”
Also, in a recommendation put together by the Sickle Cell Foundation, government has been told to develop guidelines for the diagnosis and treatment of the disorder as well as the urgent introduction of infant screening for early diagnosis.
Among other things, the organisation seeks the recognition of “a cadre of Sickle Cell Nurse Specialists, as exists in England and America, to improve our capacity to deliver better health care and education to affected individuals, families and other members of the Nigerian public.”
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