• Experts list how to survive condition
By Romanus Okoye
There have been questions as to how long people with sickle cell live. Some have said it is below 40. Other said it is under 60. And yet others said, 70 years.
On this, a report once declared: “Among children and adults with sickle cell anemia (homozygous for sickle hemoglobin), the median age at death was 42 years for males and 48 years for females. Among those with sickle cell-hemoglobin C disease, the median age at death was 60 years for males and 68 years for females.”
However, events have proved that people living with sickle cell could live as long as others, despite the crisis they go through. Many have lived more than 70 years. Indeed, the story of people living with sickle cell is a moving one.
Ms. Toyin Adesola, executive director, Sickle Cell Advocacy & Management Initiative (SAMI), has lived with sickle cell disorder (SCD) for over 51 years. Telling her story, she said living healthy with SCD was more than taking drugs.
“I have found that healing from chronic disorders like sickle cell or any health challenge requires not only drugs but also a state of preventive care. It requires the physical, mental and spiritual capacity to make positive healthy choices; placing more emphasis in one area while neglecting another can result in stress, poor health and the inability to cope with the demands of one’s daily life,” she said.
Giving tips on how to survive, Adesola said: “One needs to have a cautious healthy lifestyle. Cautious, in the sense that we have to be mindful of what we eat, how we think, how we relate to our environment and our relationship with God.”
She emphasised healthy eating, stating” “Healthy eating involves eating variety of foods that give you the nutrients you need to maintain your health, feel good and have energy. These nutrients include protein, carbohydrates, fat, water, vitamins and minerals. Do you know that breakfast is the most important meal of the day? The effect of leaving our stomach empty can result in mental and physical problems.
“Not only do we need to eat breakfast, we must maintain a balanced diet. A good balanced diet requires us having more vegetable and fruit than any other thing.
“Adequate intake of water cannot be overemphasized. Simple health issues like fatigue, headache, body pains and impaired performance can be due to dehydration. It is also important we prepare home cooked meals as much as possible, eat less of fried foods, and eat smaller quantities and more frequently.
In specific term, she ;listed food and things that should be avoided. “Avoid junk foods, sugar, loaded drinks, beverages and alcohol. I used to wonder why my stomach was often bloated, excruciatingly painful and sometimes leading to frequent stooling until I discovered that I was mildly lactose intolerable due to intake of dairy milk,” she said.
Adesola said soya and almond milk are good substitutes for regular dairy milk, explaining that there are people who encounter issues due to their intolerance of gluten, which can be found in wheat products.”
On what people living with sickle cell go through, she said: “There is no denying the fact that living with sickle cell disorder takes its mental and emotional toll on you. Having people understand what you are going through can be difficult. This then tends to increase your doubts and fears, thus increasing the tendency to fall into some form of depression.”
According to her, the secret of health, for both the mind and body, was “not to mourn the past, worry about the future and not to anticipate trouble but to live in the present moment wisely and
earnestly,” adding:
“Living in the present and not focusing on negative words, but being positive each day reduces the effect of emotional stress, which can lead to crises.”
Another person who has lived healthy with sickle cell is Mrs. Modupe Popoola, born in 1958. In her family, she is the only one with Hb SS- sickle cell disorder, which was diagnosed at the age of five months at the University College Hospital (UCH), Ibadan.
Telling her story, she said: “I have two brothers that are AA and a sister that is AS,” she said.
Mrs. Popoola, who is now 58 years old, said it was more severe to live with SCD when she was much younger, explaining that with experience and medical improvements now, managing SCD generally had become much easier.
Said she: “I can manage myself better now. I remember when I was to travel to England for the first time. My father was reluctant for obvious reasons. But surprisingly, I did not have any crisis
throughout my stay.”
On how she has survived over the years, Popoola said: “My parents and siblings were very caring, supportive and protective. They never wanted anything untoward to happen to me. This level of support helped me to cope to a large extent. I remember my mum went to see my school principal before I started secondary school.”
She said her Hb SS genotype did not deter her from school activities, though she knew her limit. “I was very active in school and in sports. I played the netball and was the shooter for my team. But I was conscious of my limits, which was why I refused to play for the school team,”
she said.
How does she overcome stigmatization? She said people living with sickle cell should be confident and determined as well as not pay attention to what people say.
“When I was in primary school, my friends called me ‘abiku.’ But I always retorted that they were ‘olodo,’ meaning ‘empty heads’ as the condition did not impact on my intellect. At every point, I used my experience with stigmatisation as a stepping stone.”
She noted that another challenge was that most parents cannot cope with the burden of care of affected children in Nigeria due to poor welfare infrastructure and prevailing high poverty levels.
Her survival tip is this: “There is need to empower affected persons, especially the less privileged ones. Affected persons should seek treatment in hospitals where they can get appropriate care, especially where there are hematologists. Do not self-medicate. Seek counsel from genetic counselors when in doubt.”
Meanwhile, experts have revealed steps to be taken to forestall ugly situations that could complicate living with SCD. One of such steps, according to them, is the assessment of stroke risk in HbSS children. According to Dr. Motunrayo Adekunle, consultant Pediatric Hematologist, Babcock University Teaching Hospital and Professor Olu Akinyanju, chairman, Sickle Cell Foundation Nigeria, a non-invasive and painless ultrasound screening of the brain by trained personnel, using Transcranial Doppler Ultrasound equipment, could detect the risk of stroke in these children within 30 minutes.
“Children aged 2-16 years should have this screening done at least once a year. Transcranial Doppler ultrasonography is available in Lagos, Ibadan, Kano and some other locations following the recent training of sickle cell doctors from six geopolitical zones in Nigeria,” one of them said.
Another improvement that raises hope for those living with Sickle Cell Disorder is Bone Marrow Transplants. It provides permanent cure for the terminal disease.
Bone Marrow Transplants do not need chemotherapy, making it less risky for adult sufferers, often thought to be too weak to survive chemotherapy. According to scientists, patients receive immunosuppressive drugs before the transplant, along with a low dose of total irradiation, a treatment that is much less harsh and with fewer potentially serious side effects than chemotherapy.
“The recipients still need to take immunosuppressant drugs for at least a year. This lowers your body’s ability to fight infections,” said Victor Gordeuk, Director of the Sickle Cell Centre, University of Illinois.
Also speaking on Bone Marrow Transplant, Prof. Christopher Ndugwa, a paediatric consultant and board member of the Uganda Sickle Cell Rescue Foundation (USCRF), said sickle cell disease was curable through bone marrow transplant.
He, however, pointed out that even without a transplant, a child could lead a normal life if he/she have early diagnosis and go for regular hospital assessments every three months, even when they are not sick.