Giving Life to Indigent Sickle Cell Carriers
The Omotoke Gambari Foundation recently reached out to 63 children living with sickle cell anemia with the aim of celebrating them despite their health challenges and indigent situation. Sunday Ehigiator writes
The recent death of popular radio presenter, Tosyn Bucknor, from health complications relating to sickle cell anemia has again brought to the fore that the disease is one of the leading causes of death globally, especially in sub-Saharan Africa.
Sickle cell anemia is an inherited form of anemia; a condition in which there is not enough healthy red blood cells to carry adequate oxygen throughout the body.
Normally, the red blood cells are flexible and round, moving easily through the blood vessels. In sickle cell anemia, the cells become rigid, sticky and are shaped like sickles or crescent moons. These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.
Although, there’s no cure for most people with sickle cell anemia, treatments can relieve pain and help prevent problems associated with the disease.
The disease is caused by a mutation in the gene that tells the body to makes the red, iron-rich compound that gives blood its red color (hemoglobin). Hemoglobin allows red blood cells to carry oxygen from the lungs to all parts of the body. In sickle cell anemia, the abnormal hemoglobin causes red blood cells to become rigid, sticky and misshapen.
The sickle cell gene is passed from generation to generation in a pattern of inheritance called autosomal recessive inheritance. This means that both the mother and the father must pass on the defective form of the gene for a child to be affected.
If only one parent passes the sickle cell gene to the child, that child will have the sickle cell trait. With one normal hemoglobin gene and one defective form of the gene, people with the sickle cell trait make both normal hemoglobin and sickle cell hemoglobin. Their blood might contain some sickle cells, but they generally don’t have symptoms. But they are carriers of the disease, which means they can pass the gene to their children.
Despite increased awareness, statistics have shown that a lot of work is still needed to be done to achieve its extinction.
It was against this backdrop, that Omotoke Gambari established her foundation, with the mandate to promote awareness of the Anemia, care for carriers, and provide adequate support to the carriers here in Nigeria.
According to Omotoke, the recent get-together with its 63 children living with sickle cell anemia, with the aim of celebrating them despite their health challenges and indigent situations, was a perfect way to end the year after the hassles experienced in the year, and also a thanksgiving to God for life despite their obvious condition.
At the party, which was held on a beautiful little lawn in Osborne Estate, Ikoyi, the giggle, excitements, laughter and happy noise of the children who jumped on bouncing castles and rode on fun-train, was satisfactory, as carriers are also worthy of a happy life despite their condition.
The foundation launched in August this year is already a home to 63 kids with this genetic defect. Among her objectives and activities since founding the organisation is to educate the patients as well as their parents; since many of them are less privileged and uneducated.
From providing hospital care when they go into crisis, to giving free drugs to the kids, monthly meetings which is both advisory and supportive, conducting Trans-cranial Doppler scan (TCD) for children who are two – 16 years who are most at risk of having a stroke, and also offering free treatment to all children concerned, the foundation has been of cardinal impact in the lives of the 63 carriers, breathing in them a new life.
Omotoke, who is also conditioned by the genetic misdemeanor, while further speaking with THISDAY on the conditions of a’ sickler’, said “I was somewhat lucky because I am privileged to come from a comfortable home; I received all the attention and care I needed, observed and treated whenever the need arose.
“Complications come in different forms in a patient. It is important to understand that the term ‘sickler’ is from the word sickle which is the abnormal shape of the cells in the patients; as a result, these cells could get stuck in the blood vessels. Getting stuck in the wrong places could lead to organs failures and infections.
“The weather could also lead to complications; a patient need not stay in either a very cold or very hot environment. Malaria could also lead to complications, and so anti-malaria vaccines are advised. In the case of injuries, an SS patient does not heal at the same rate with that of a ‘normal person’.
“In many extreme cases, there is constant need for blood transfusions, in a bid to flush out the SS blood cells and replace with other healthier ones.”
According to a Retired Matron and Genetic Counselor, Mrs. Olabisi Olaiyi Adefisan, “one of the health complications associated with persons living with sickle cell disease; especially in males is painful unwanted erection. It occurs to boys/men within ages four to 45 years. It usually comes at night.
“Another complication that could arise from this condition is leg ulcer. This is common in sickle cell disease. Although this could happen to any gender, studies have shown it can be prevalent in males compared to females.
“Leg ulcers are a chronic painful problem. They result from minor injury to the leg area; predictably, because of relative poor circulation of blood, compounded by sickling and micro-infarcts. Healing is delayed and infection becomes established.
“The risk associated with sickle cell disorder could be classified into conditional and standard risk.
“Conditional risk is a situation of being free from stroke for the next six months while standard risk on the other hand is being free from stroke for the next one year. The only way to know what risk a carrier belongs exactly is by conducting the TCD scan.”
She added that signs and symptoms of sickle cell anemia vary from person to person and changes over time.
She further posited that, “Anemia as earlier said is another symptom associated to carriers. Sickle cells break apart easily and die, leaving one without enough red blood cells. The red blood cells usually live for about 120 days before been replaced. But sickle cells usually die within 10 to 20 days, leaving a shortage of red blood cells.
“Without enough red blood cells, human body can’t get the oxygen it needs to feel energised, causing fatigue.
“Episodes of pain are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints. Pain can also occur in your bones.
“The pain varies in intensity and can last for a few hours to few weeks. Some people have only a few pain episodes. Others have a dozen or more crises a year. If a crisis is severe enough, one might need to be hospitalised.
“Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers and other causes.
“There is also painful swelling of hands and feet. The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.
“Another is frequent infections. Sickle cells can damage an organ that fights infection (spleen), leaving you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.
“Other is delayed growth, and Vision Problem. Red blood cells provide the body with the oxygen and nutrients you need for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers. And as, tiny blood vessels that supply the eyes may become plugged with sickle cells, this can damage the retina; the portion of the eye that processes visual images, leading to vision problems”.
Management of Sickle Cell in Children
To manage sickle cell disease in children, it is often advised that carriers should ensure to go to all doctor’s visits and share any concerns or new symptoms, avoid peculiar pain crisis triggers, such as extreme temperatures or stress, talk to the doctor about which activities are right for you and which you should avoid, avoid smoke, drinking of alcohol, or use of illegal drugs, drink lots of liquids and get enough rest, and as well as letting an adult know right away if they don’t feel well.