The burden of living with sickle cell anaemia

Across the border, Patrick Bwire and Patricia Bwire struggle with a chronic illness in the family. The two residents of Namuwongo, a Kampala suburb in the neighbouring Uganda, are struggling with twins suffering from sickle cell anaemia. In an article published by Uganda’s Daily Monitor recently, 10-year-old Rofina Pauline Adong and Regina Petronila Apio were paralysed after suffering a stroke as a result of sickle cell anaemia. The first one to fall sick was Adong, aged two years then, followed by her sister, Apio, two years later. “I was at home one morning when I suddenly heard a loud cry from the bedroom and rushed there only to find her (Adong) lying still on the bed, with the right leg and arm stiff,” Bwire says. Initially the doctor prescribed a cream to help relax her joints and relieve the pain but Adong’s condition never improved until later when doctors at Mulago Hospital confirmed that she had sickle cell condition. Adong’s left leg and arm had also stiffened and she could hardly speak. As if the pain was not enough for this family, the limbs of the other twin also started stiffening and eventually lost her speech at the age of four. Because of the pain and discomfort, Adong cries a lot throughout the night making it very difficult for the rest of the people in the house to sleep. But that is not all. The cost of treatment is simply very expensive. The twins, however, are not the only ones suffering from sickle cells. Although the magnitude of the problem of sicklecells in Rwanda is unknown, it is estimated that 300,000 children are born each year with sickle-cell anaemia worldwide. Research published in The Lancet shows that the most affected countries in Africa are Nigeria and Democratic Republic of Congo with an estimate of 91,000 and 40,000 annual births respectively. What is sickle cell anaemia? Sickle cell anaemia is a blood condition acquired as a result of inheriting two sickle cell genes in their recessive form, one coming from either parent. Sickle cell patients (sicklers) lack enough healthy red blood for carrying adequate oxygen throughout the body. Unlike normal red blood cells that are flexible and free flowing within the blood vessels, sickle cells are rigid and sticky. “This kind of cell orientation easily blocks blood flow within the vessels,” says Jean Paul Nsengiyumva, a nursing officer at the sickle cell clinic at Kibogora Hospital. He adds that sickle shaped cells lack capacity to accommodate enough oxygen limiting its supply to vital body organs. “The blockage combined with insufficient supply of oxygen to other body parts results into localized pain depending on the body part,” he adds. According to Mayo Clinic, the pain also occurs in other areas such as bones but other signs include swollen hands, feet, frequent infections that damage the spleen, vision problems and delayed growth. Although there is no conclusive evidence on its cure, sicklers, too, have a life. Living with sickle cell anaemia Dr Charles Sindabimenye, a physician at Doctors Plaza in Kimironko, says that sicklers survive by adhering to medical prescriptions as a way to relieve the pain while preventing further complications. Sindabimenye, who is currently treating four sickle cell patients at the clinic, says most of them are foreigners. “Two patients are from Nigeria, one from Kenya and the other from Congo,” he says. He maintains that since sickle cell anaemia has no conclusive cure, the suffering of one individual will affect the rest in the family. “Definitely individuals within the family are forced to sacrifice time and spend a lot of money on treatment,” Sindabimenye adds. Managing the disease Treating a disease like sickle cell also causes depression among other psychological effects especially on the side of the parents. Dr Charles Mudenge, a psychiatrist from University Teaching Hospital, CHUK points out that in one way or the other, terminal illnesses psychologically affect families. “Definitely, a child with sickle cells cannot be like the other children since most time family members are forced to provide adequate attention,” Mudenge says. He, however, advises that during this condition, psychologists are of a greater value to support family members. Thamar Nyiransabiyaremye, a supervisor in charge of immunization at Kibogora Hospital, says that having sickle cell anemia should not be cause for depriving children of the other health procedures. “On top of their treatment, sicklers should continue to receive immunization packages just like the other children,” Nyiransabiyaremye says.

Health

The burden of living with sickle cell anaemia