She looked forlorn as she walked into the consulting room. On her face was etched misery, anguish and agony. She was carrying a boy who looked about 5years. He was in pain, his right knee was swollen and he looked pale. I knew the story will be long and truly it was.
It began 10years earlier when she married her village boo. There had been serious and protracted resistance to the relationship by her boo’s mother who made several threats but ultimately, love prevailed. A truce was reached and the wedding held. The first child came soon enough, a girl who grew steadily and in good health. Then the second child, a boy, came and the troubles began.
The delivery was by a traditional birth attendant who subsequently carried out the circumcision on the eight day of life. The boy started bleeding soon after from the circumcision site and all efforts made to stop it proved abortive. By the time they got to hospital it was too late to save the child. Cause of death…excessive blood loss. That was the medical one….
The widespread belief was that the death was Jazz induced. Naturally the mother in-law was at the top of the list of suspected culprits.
Two years later, the second son came along. Circumcision went well but then as he began walking and with the attendant constant falls, swellings began appearing around his legs. Injuries too were bleeding for too long. By the time he got to 5years old, repeated painful knee swellings became a constant. After several failed spiritual and traditional interventions, she decided to come to the hospital. Full examination and clinical assessment suggested Haemophilia.
Haemophilia a genetic or inherited medical condition that cause abnormal or exaggerated bleeding and poor blood clotting. At some stage in history, it was called the Royal Disease. Queen Victoria, Queen of England from 1837 to 1901 was a carrier and her daughters passed on the faulty gene to members of the royal families of Germany, Spain, and Russia. Alexandra, who became Tsarina of Russia in the early 20th century when she married Tsar Nicholas II, was a carrier. Their son, the Tsarevich Alexei therefore suffered from haemophilia and that was how they dell under the spell of Rasputin. But that is another story
Males carry one X chromosome while females carry two, so while males are commonly affected females are usually JUST carriers of the disease. Females are protected because they carry two X chromosomes. Now you understand why the female child was spared but the males were affected fuelling the belief that someone was after the male children. In rare circumstance, the odd female might have haemophilia…..the long explanation can cause headache . so let me spare you that headache……paracetamol no dey cheap again oh.
The severity of symptoms varies among affected individuals. Symptoms include nose bleeding, post circumcision bleeding, joint swelling from bleeding. The main danger is uncontrolled internal bleeding that starts spontaneously or results from an injury.
If haemophilia is suspected in an individual, simple laboratory tests for determination of the levels of Factor VIII or Factor IX activity in their blood sample is advised. In countries with better organised health systems, if the mother is a known carrier of haemophilia, testing can be done before a baby is born. This is called prenatal diagnosis.
The first reported diagnosis of haemophilia in Nigeria was made in December 1969. Currently 12,000 – 14,0000 Nigerians are estimated to be living with haemophilia but then awareness sadly has remained very low. Treatment involves replacing the missing factors in blood to prevent excessive bleeding but the cost of treatment and management of complications CAN be very expensive.
Do spare a thought today for the child with haemophilia and his parents.