‘Sickle Cell Disease not death sentence’

A medical officer at the Ghana Institute of Clinical Genetics (Sickle Cell Clinic), Dr Grace Amoh, has cautioned the general public against discrimination targeted at people with Sickle Cell Disease (SCD).

She said the disease was a genetic blood disorder that could be prevented and managed, yet till date people still held various misconceptions with some even attributing it to “evil forces.”

Dr Amoh, who was speaking to The Mirror, as part of the Sickle Cells Awareness Day celebration last Wednesday, explained that people living with SCD faced a lot of health and emotional challenges, therefore such stigmatisation only added to the burden on individuals and affected families.

Contrary to some beliefs that people with the disease lived a short life, she noted that with support from family such persons could live a long and quality life if the disease was diagnosed early and managed properly.

What is Sickle Cell Disease?

SCD is an inherited blood disorder that affects red blood cells – the cells responsible for carrying oxygen from the lungs to all parts of the body.

Unlike persons without the disease whose red blood cells are rounder and flexible, people with the disease have stiff and sickle-shaped red blood cells that makes it difficult for oxygen distribution.

This may lead to episodes of pain, referred to as crisis with symptoms like swelling of feet and hands, and can also damage organs in the body.

 Comprehensive structure

Dr Amoh said they were also advocating a comprehensive approach to providing care for people with the disease.

She said in most cases, the disease was diagnosed when it had reached an advanced stage, making it difficult to be managed.

“Some health facilities provide screening for new-borns so that their status is captured at an early stage. Beyond this, we need a national policy and plan to manage the disease.”

This, she said, included appropriate facilities and trained personnel, adequate diagnostic tools and treatment for patients with SCD.

Do you know your status?

SCD  is passed through the bloodline of people who have it. It is possible for people to have only one sickle cell gene, so they do not have the disorder themselves. These people may pass that gene to their children who are then at risk of contracting the disease if their partner also has the gene.

Dr Amoh advised that it was important for partners to know their status before deciding to have children.

“Most  parents of the patients we attend to had no idea of their status prior to giving birth. Carriers of Sickle Cell genes are healthy and so most of the time it hits them hard when they realise their children have the disease.

“Our focus is to create awareness and get people to know their status before having children, adding that these days some churches insist on will-be couples  checking  their status before getting married.

“ In the past, the commonest test was the sickling test but now we recommend haemoglobin electrophoresis, a blood test that measures different types of a protein called haemoglobin in your red blood cells.

“A simple sickling test will only pick someone with haemoglobin ‘S’ but does not give the actual genetic makeup,” he explained.

 Not an easy journey

Ms Rosalind Amoh, a journalist, had gone for a routine medical screening for her four-month old baby, the last of her three children when he was diagnosed with SCD.

She recounts that  her son who is now 10 showed no sign of the disease till he started to crawl and move around.

She said the journey had been tough and challenging, as by age five, the boy had to undergo splenectomy, a surgery to remove the entire spleen as it was not functioning properly.

“He has had very severe crisis but thankfully two years ago, he was put on some medications and he has been a bit more stable.

 “I am always willing to share our experiences and advise people to know their status before they decide to have children.

“It is emotionally, physically and financially draining. If you do not have support, you will break down. Depending on the severity of the crisis, you could be on admission for two weeks or over and within that period as a parent you will not be able to work and you also deprive your other children of care,” she stated.

Fortunately, her son is always able to catch up with his mates at school despite the many days of crisis