A Royal Disease in Nigeria
Incidences of excessive or abnormal bleeding were first recorded hundreds of years ago. The Talmud, a collection of Jewish rabbinical writings on laws and traditions, from the 2nd century AD, stated that baby boys did not have to be circumcised if two of their brothers had previously died from the procedure. The New Testament of […]
fatima damagum
Incidences of excessive or abnormal bleeding were first recorded hundreds of years ago. The Talmud, a collection of Jewish rabbinical writings on laws and traditions, from the 2nd century AD, stated that baby boys did not have to be circumcised if two of their brothers had previously died from the procedure. The New Testament of the Bible mentioned a woman who had hemorrhaged for 12 years, before touching the hem of Jesus’ garment, when she was healed. Abu Khasim, a 10th century Arabian physician, described families whose male relatives died from uncontrolled bleeding after trauma.
Hemophilia is probably one of the best-known single‐gene disorders in the eyes of the public, not because of its frequency (others are more common), but because it occurred in the royal families of Europe. Queen Victoria was a carrier of the disease and had a son, Prince Leopold, who suffered from it. At least two of her daughters were also carriers and passed it on to their children. In total, at least nine male descendants of Queen Victoria had hemophilia (there may have been more, however, the disease was an embarrassment to the monarchy, who generally kept as quiet as possible about it, and some that died in infancy may not have been reported). Hence the nickname, ‘royal disease’.
Haemophilia is a sex-linked disorder, where men express the disease and women are carriers. Haemophilia A, factor VIII (8) deficiency, has an incidence of 1/10,000 and haemophilia B, factor IX (9) deficiency, has an incidence one sixth of haemophilia A. In Nigeria, there are about 1000 people with bleeding disorders.
As can be imagined, the problems associated with Haemophilia in Nigeria are very complex.
Firstly, diagnosing the problem itself is an issue. The skills and equipment needed to accurately diagnose haemophilia, and the specific types are limited to a handful of tertiary and specialist hospitals. Many hospitals lack the capacity and therefore rely on basic tests like clotting time, Prothrombin time and mixing studies to make educated guesses.
Many a time, especially in the rural areas, diagnosis is made post mortem. This usually occurs after traditional practices like circumcision and uvulectomy. One Mallam Musa (No offence intended) who comes from a generation of barbers, and who has been circumcising boys for the past twenty years in a distant village in Bulkachuwa (again, no offence) will suddenly cut the foreskin of a boy resulting in profuse bleeding. Despite applying pressure, the boy will continue to bleed and bleed until he dies. The people in the village will chuck it down to fate and continue with their lives. The father will then have another son and take him to the same barber or another one for circumcision, and it is not until this index child bleeds out that it suddenly dawns on the family that the bleeding is not ordinary.
After diagnosis, comes the problem of treatment. Many years ago, haemophilia was treated with whole blood transfusion. Gradually scientists discovered the use of plasma concentrate, then cryoprecipitate. Today, haemophilia is no longer a life-threatening disease and in most developed parts of the world, sufferers have access to treatment with recombinant factor VIII or IX concentrate. However, this is not the case in Nigeria.
Synthetic factors 8 and 9, which is the main stay of treatment is not available in the Nigerian market because of the cost. The drugs are obtained from the Haemophilia Foundation of Nigeria (HFN) which receives donations from the World Federation of Haemophila (WFH). The WFH in turn receives donations from International Donor agencies and pharmaceutical companies. So far, Nigeria has received almost N2 billion worth of haemophilia medication from the WFH. Neither the government, nor indigenous foundation, talk less of individual has ever donated to the HFN.
Haemophilia is a stupendously expensive illness to treat. A simple example is tooth eruption. Typically, children start shedding their teeth at around the age of six. A young child will come to the parent with a tooth in his hand and declare that his tooth fell off while playing. What do we do? Nothing. Sometimes, we may tell the child to rinse his mouth or applying cotton wool to the bloody gum. Two minutes later, the child has forgotten and is running around.
Not so in children with haemophilia. That tooth that has shaken loose will be accompanied by so much bleeding that he will have to be taken to the hospital for transfusion. To treat with synthetic factor 8 or 9 costs about N1-2 million. Now calculate that amount with the number of teeth that the child will shed before he becomes an adolescent.
See what I mean?
Another layer of complexity in managing haemophilia apart from diagnosis and treatment in this country is government bureaucracy (AKA corruption). When Nigeria, through the HFN, receives medicines, they are cleared by customs and shipped to NAFDAC for testing and authentication. By Law, NAFDAC is supposed to test each vial from a batch and when confirmed, shipped to various hospitals that have since indicated interest for administration to patients awaiting treatment.
Seems like a seamless process, yes? Lai lai!
Since March 2024, medicines in NAFDAC have not been released and are awaiting clearance. They are still ‘testing’. This means that patients awaiting treatment have been on hold for the past five months!
For the last five years, the Haemophilia Foundation of Nigeria (HFN), in collaboration with the World Federation of Hemophilia (WFH), has engaged the federal government of Nigeria through the Ministry of Health to advocate for the adequate care of people with bleeding disorders (PWBDs) in the country. And yet, nothing.
Nigerians living with bleeding disorders are like you and I. They, equally, have a right to life and a right to quality health care. The country needs to show commitment to officially include bleeding disorders in its health policy and strategic plan.
Can someone please beg Oga at the top?