Why Sickle Cell disease is not death sentence

James Muori's mother Margaret Mukando. [Brian Kisanji, Standard]

Within the span of a week, the boy experienced episodes of fainting and passing blood in his urine.

One evening, when the episodes became overwhelming, Mukando sought treatment at Banja Dispensary in Hamisi.

It was at this hospital that a clinician promptly took action and referred the frail young boy to Jaramogi Oginga Odinga Teaching & Referral Hospital (JOOTRH) in Kisumu.

"By the time I reached JOOTRH, my boy was practically lifeless. He couldn't speak, and his breathing was faint," Mukando said.

Mukando continued narrating how she pleaded with the matatu driver to accelerate the Public Service Vehicle she had boarded from Gambogi.

"The journey of almost 20km felt like an eternity. I kept urging the driver to avoid any stops or picking up other passengers because I was afraid that my son would die in my arms," Mukando said.

Upon arriving at the hospital that evening, the doctors immediately sprang into action and stabilized young James. After undergoing various clinical tests in 2015, James received a diagnosis of sickle cell anemia.

Sickle cell anemia is one form of SCD, a non-communicable disease that is inherited.

To James's mother, it felt like a miracle that her son survived that terrifying ordeal.

Since then, the boy has been on medication and management protocols to cope with the lifelong illness. Due to the search for appropriate treatment and disease management, James had to miss school for nearly two years.

He is currently in seventh grade, and his mother says he is intelligent and enthusiastic about learning.

"My son is doing well. You wouldn't even know he has Sickle Cell Disease. He excels in school and is very healthy," Mukando said.

However, amidst the joy of surviving a near-death experience, Mukando partly blames herself for not being aware that both she and her husband were carriers of Sickle Cell Disease.

Medical research indicates that SCD is a genetic condition that is present from birth and inherited.

According to the Centers for Disease Control (CDC), Sickle Cell Anemia develops when the body produces an abnormal form of the protein (hemoglobin) in the blood that carries oxygen.

SCD is a genetic condition that is present from birth and inherited. [iStockphoto]

The abnormal hemoglobin (HbS) causes red blood cells to adopt a crescent shape, resembling sickles. These cells tend to clump together, leading to blockages in blood vessels, causing pain and tissue damage.

In this case, James inherited the condition from his parents, who, after testing, were both identified as carriers.

Dr. Frederick Okinyi, a specialist in blood disorders and a lecturer at Nairobi University, emphasises the importance of genetic counselling for couples planning to marry in order to address this genetic condition. He also emphasises the need for health facilities to be equipped to manage SCD.

"Anyone over the age of 20 who intends to have children should undergo screening and testing for SCD," Dr. Okinyi said.

He said most parents, like James' parents, are unaware of their SCD status, which puts their newborns at risk of developing the disease.

He further urges people of all ages in Kenya to get tested for SCD to effectively plan for their health.

According to Dr. Okinyi, early diagnosis and treatment are crucial, and individuals with the disease can lead full and active lives.

In Kenya, as per the World Health Organization (WHO), around 14,000 children are born with sickle cell every year, while globally, the number exceeds 300,000.

The symptoms and complications of SCD vary among individual patients.

The majority of individuals with SCD experience symptoms that can vary in severity, including mild to severe pain, swelling of the hands and feet, frequent infections, delayed growth, and vision problems.

To address these symptoms and manage the condition, infants and children with SCD typically undergo a treatment plan that includes vaccinations and antibiotics. This approach aims to prevent potentially life-threatening infections, such as pneumonia.

Western, Nyanza and Coast counties residents are classified as being at high risk of developing sickle cell diseases.

One tablet, for example, hydroxyurea, costs Sh50. The medication roughly costs between Sh3,000 and Sh3,500 a month, which is beyond the reach of many families.

Patients with SCD and medics in the field complain that the county governments have not invested well in the treatment of the disease.

Dr. Okinyi, who is also the chairperson of the Sickle Cell Federation of Kenya, advocates for the addition of drugs such as hydroxyurea to the list of essential medicines covered by NHIF.