On June 19, the world again gave attention to sickle cell. Women’s Editor, TEMITOPE OJO, writes on the avoidable disease that is claiming lives.
Adeola, 28, died five years ago after childbirth. According to her husband, Lekan, she carried the SS genotype but rarely fell ill. This was why, though they both knew he too carried AS gene, still went ahead to get married. They were optimistic they could be lucky not to bring forth a child with the SS gene or even where they did, the baby might just be lucky not to fall ill frequently, like the mother.
Similarly, Margaret, 32, died months after she had her son in the United States. She too was a sickle cell patient.
Sickle-cell disease (SCD), also known as sickle-cell anaemia (SCA) and drepanocytosis, is the world’s most commonly-inherited blood disorder, characterised by an abnormality in the oxygen-carrying haemoglobin molecule in red blood cells. Its signs, symptoms and complications have similarities with practically every disease known to humankind.
Many SCD patients have either died or are living with severe pains on daily basis.
SCD is an extremely debilitating medical condition. It is characterised by chronic pain, with frequent bouts of acute severe pain that requires large amounts of narcotics that often provide incomplete relief. It is also marked by social stigma that makes others, including medical professionals, view sickle cell patients as ‘drug-seekers’ due to their need for large amounts of pain medication.
According to experts, essentially, patients have poorly oxygenated red blood cells in the shape of a farm tool called sickle. Patients’ cells are hard, sticky, and sometimes travel backwards, according to the Centers for Disease Control and Prevention (CDC) of the United States. Cells get stuck this way and clog blood flow, leading to “pain and other serious problems such as infection, acute chest syndrome, and stroke”.
For the sickle cell patient, any day without pain is a good day!
Experts say patients have an annual average of three episodes of severe pain that requires hospitalisation and narcotics.
Although SCD is the most common genetic blood disorder worldwide, it receives comparatively meagre research funding. Each year, approximately 500,000 babies worldwide are born with it, with children of African, Latino, Mediterranean and South Asian descent most impacted.
Nigeria, by virtue of her population, is the most SCD endemic country in the world, with an annual infant death of 100,000 representing eight per cent of infant mortality in the country.
According to Chairman, Sickle Cell Foundation Nigeria (SCFN), Prof. Olu Akinyanju, patients’ pain is real and is triggered by damage to major parts of their bodies, including the heart, lungs, kidneys and brain. Also, they are very susceptible to infection, which can be life-threatening. These organ damage-induced bouts of pain and infectious episodes are two causes of mortality at a very young age.
He stated that over 40 million Nigerians are healthy carriers of the sickle cell haemoglobin ‘AS’ trait. According to him, rarely is there a family in this country that has not been grilled or is not being grilled by SCA.
In Nigeria, factors like ignorance, lack of access to good healthcare services, stigmatisation, poverty and superstition have contributed to the mortality rate of this blood disorder, Akinyanju asserted.
There is a need, he said, for national guidelines on sickle cell diagnosis and treatment to help improve the life expectancy of people with SCD.
He called on government at all levels, stakeholders and the media to provide advocacy to help reduce the burden of the disease.
“There is also need for government to provide intensive funding for researches, (as well as) equitable and efficient treatment.
“The role of the media in providing advocacy for enlightenment and awareness campaigns cannot be undermined.
“We want to get to a place whereby SCD disease will be regarded like other diseases such as anaemia, hypertension and diabetes. People with SCD can also live a normal life and have longevity with modern treatment,” he said.
Since the early 1990s, universal newborn screening programmes include testing for sickle cell (as part of hemoglobinopathy test). While newborn screening helps the youngest among us, many parents are aware of their own sickle cell status.
The life span of someone with SCD is about 40 years if they live in the U.S.
Yutaka Niihara, MD/CEO of Emmaus Life Sciences, explained that in underdeveloped countries, where there’s limited access to medical care, 90 per cent of patients won’t live past age five.
Newborn screening is crucial, so that treatment may begin early. Infants with SCD are given daily antibiotics to prevent serious infection and closer monitoring to guard against complications. Care for SCD also includes hydration, pain medication, and blood transfusions.
Relief, especially for SCD-related pain, is limited. Some experts recommend patients drink plenty of water, keep their body temperature regulated, and avoid high altitudes and low oxygen levels. For more severe cases, the CDC says hydroxyurea is an option, a medication designed to reduce the number of painful episodes. However, the medication boasts serious side effects and anyone taking it should do so under a doctor’s supervision.
Even the multiple blood transfusions that keep red blood cell levels up pose health risks with increased transfusions overloading the body with iron. Too much iron can lead to liver, heart, and pancreas damage, the CDC found
The only known source of long-term relief for SCD patients is either bone marrow or stem cell transplants, which on their own are very risky.
Being vocal about the realities of living with SCD has a positive domino effect.