‘Sickle cell disorder should be treated as national health emergency’

Health experts have advised Nigerians living with Sickle cell disorder (SCD) to see the disease as a national health emergency and work towards reducing the burden of the disease.

Statistics show that 150,000 babies are born yearly with sickle cell and over 100,000 die before their fifth birthday in Nigeria. An estimated 50 million people carry the sickle cell trait and are at risk of passing the disorder to their offspring.

With the average life expectancy of individuals in Nigeria living with sickle cell at only 20 years, the need for access to effective treatment and cure has never been more urgent.

They noted sickle cell could be prevented through genetic counselling and genotype screening among intended couples. This will enable them to know their genotype before getting married, and avoid having babies with sickle cell disease.

The National Director/Chief Executive Officer of Sickle Cell Foundation Nigeria, Dr Annette Akinsete, who disclosed this to The Guardian, said every Nigerian is directly or indirectly affected by sickle cell disorder.

She explained that if Nigerians learned more about the disorder, including the cause, and shared accurate information about the disease it would be reduced.
Akinsete said the government should provide practicable and implementable policies and laws to reduce the burden of the disease. She urged Nigerians to create awareness about the disease, as deaths caused by sickle cell are out of ignorance and preventable, saying, “Parents should take their babies to the hospital whenever they notice signs and symptoms of sickle cell anaemia or any other related ailment.”

Akinsete disclosed sickle cell is an inherited disease and genetic disorder. It has to do with genes, lineage and heredity from parents to their children. “We always tell people to stop discriminating and stigmatising against people living with sickle cell disorder because it is not contagious and infectious.”

According to her, the United Nations has mandated every government to take action to prevent sickle cell, adding that the government should provide quality, affordable, accessible sickle cell treatment in Nigeria as non-governmental organisations (NGOs) and other relevant stakeholders should support efforts of government in combating sickle cell in the country.

Akinsete noted that Nigeria has had policies since the 80s but should be backed by guidelines and funding for sickle cell disorder control. She said the foundation provides screening for mothers and unborn babies to know the genotype of the unborn baby before they are born into the family.

In terms of providing drugs to patients, she said drugs for the treatment of sickle cell are expensive and the government should fund it to reduce the burden parents go through while treating their children.

The Global Action Network for Sickle Cell and Other Inherited Blood Disorders (GANSID) Regional Lead– Africa, Mr Olajide Ayoola, said people with sickle cell disease face multiple challenges internally and externally.

He said the internal challenges result from inheriting a gene that distorts normal blood shape from round to sickle. “This inheritance manifests in having all sorts of head to (whole body), complications, the most common one being localised or generalised pain. The pain may last hours to days or weeks, and sometimes not be amenable to the most potent painkillers.”

Ayoola, who is also a sickle cell sufferer explained that other challenges associated with sickle cell disorder include tiredness, chronic shortage of blood, slow growth, paleness, and visual difficulties.

He noted the external challenges are those imposed by their kin, family, friends and society at large, such as stigmatisation and discrimination, mockery, rejection, and name-calling. “This can birth feelings of interiority, erode self-esteem, and even engender depression.”

He said the most recent cure for sickle cell disorder is casgevy, which gives hope of a one-time cure by gene editing. “There is hope for a cure, but it would not be for everyone either because they cannot afford it or because their geographical location precludes them from accessing such treatments.”

He said this would allow individuals and families directly or indirectly affected, estimated to be more than 100 million worldwide to download and share the free editions provided.

He noted the geographical distribution of sickle cell disorder is estimated that 20 per cent of the world’s population carries abnormal haemoglobin. “Sickle Cell and its variant hemoglobinopathies affect all races but it is more prevalent among Africans and tribal Indians at home or abroad.

He said it also affects Arabs, Jews, Hispanics, and southern Europeans with an estimated three to five million citizens living with sickle cell anaemia and more than 50 million with Sickle Cell Trait (SCT).

Olajide said the United Nations (UN) encourages robust sickle cell disorder awareness activities worldwide, particularly in countries that are more severely impacted.

It encourages governments at all levels, corporate institutions and charities to support research and public education, as well as alleviate the impact of disease on individuals and families.

Chairman/Founder Sickle Cell Foundation Nigeria (SCFN), Prof Olu Akinyanju, who said sickle cell is the most common genetic blood disorder in the world, called on the private sector, international organisations, individuals, persons with sickle cell their families and friends to act.

He said the foundation was established to address gaps in the control of sickle cell in Nigeria, including capacity building, research policy development and implementation, and monitoring and evaluation which are necessary for the sustained management and control of sickle cell disorder.

He said the foundation has supported governments at all levels in Nigeria through Public-Private Partnerships (PPP), notably via its affiliations with the Lagos State University Teaching Hospital (LUTH)/College of Medicine Lagos State University Teaching Hospital, the Federal Ministry of Health and the governments of Lagos, Edo, Delta, and Kano States.

On the global stage, he explained the foundation continues to partner with the World Health Organisation (WHO) and remains in consultative status with the United Nations Department of Public Information (DPI).

He said the foundation has, over the years, provided free or highly subsidised services to persons living with sickle cell in Nigeria. Other related healthcare services include stroke prevention for children with sickle cell, genetic counselling, and psycho-social counselling.

He said the foundation provides Nigerians access to cure for sickle cell and has established a bone marrow transplant centre in partnership with LUTH and Vanderbilt, the University Medical Centre Nashville, Tennesse USA with support from the Lagos State government and charitable individuals.

He said the foundation is championing mini blood donation to save lives, especially for persons living with sickle cell disorder. “The mini blood donation drive encourages voluntary blood donation, while still increasing the number of blood bags in the blood bank.”

Akinyanju noted sickle cell remains a significant public health challenge globally, affecting nearly 100 million people and accounting for over 50 per cent of deaths among those with the most severe form of the disease.