Haemophilia: ‘Our stories living with bleeding disorder’

When Mrs Olajide Saviour, 36, and her family members circumcised her first son, they thought they were carrying out a normal and routine cultural practice for male children in most parts of Nigeria. However, two hours later, the eight-day-old baby continued to bleed profusely. He was rushed to the hospital, transfused with blood and the […]

When Mrs Olajide Saviour, 36, and her family members circumcised her first son, they thought they were carrying out a normal and routine cultural practice for male children in most parts of Nigeria.

However, two hours later, the eight-day-old baby continued to bleed profusely. He was rushed to the hospital, transfused with blood and the wound was sutured.

The bleeding didn’t stop till two weeks later.

She then remembered that her younger brother had suffered the same fate after circumcision. He was rushed to the hospital too.

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“The people at the hospital then couldn’t identify the problem and my family began to think it was a spiritual attack. He bled for weeks before it stopped, “ she narrated.

With these experiences, she was afraid to circumcise her second son, and had to use another method that does not involve a cut.

However, she over-came the fear and decided to circumcise her third son.  He was transfused with blood.

Her first son was six years old when the three boys were finally diagnosed of haemophilia.

Haemophilia is a rare inherited medical condition in which the ability of the blood to clot is severely reduced, causing the sufferer to bleed severely from even a slight injury.

Olajide added that it is not easy living with three boys with the condition as even the slightest cut could cause severe and even life-threatening bleeding.

However, she added that since their diagnosis access to drugs through the Haemophilia Foundation of Nigeria (HFN) has helped them tremendously.

“As at the time my children were circumcised, we didn’t know about the disease or the treatment called factor concentrate that helps people with the condition.”

She also narrated that a teacher beat her son in the palm of his hand at school and he began to bleed in the brain and had to undergo treatment.

“Sometimes my children suffer joint pains, and they may not be able to walk. So, my children are on the medications and take them twice a week for prevention. When a mothers notice prolong bleeding after circumcision of their sons, they should suspect haemophilia and go to the hospital. Because the child can bleed to death. When they play and get cuts, they also bleed. There is need for more awareness among parents. The awareness should also be taken to schools and churches,” she said.

She lamented that the drugs and injections are very expensive and sufferers are only able to access them through the HFN in Nigeria.

Idris Bello, 16, who suffers from the type of haemophilia called haemophilia A , says he was diagnosed in 2016 and has grown to become a global ambassador for the disease.

He said, “Factors have helped me, because before I started receiving it, I was having joint pains and also immobile. I learnt that I bled for six months after my circumcision. My parent took me to different General hospitals in Minna and Suleja in Niger state. There was no factor then so I was been given blood transfusion. I have had more than five blood transfusions from birth till now. It has not been easy.

“We need the government to help in buying factors for us, because right now, we are on donations. Donations are not sustainable. Currently, the funding from some companies have begun to dwindle.”

Another haemophilia sufferer, David Edembe, 16, said his school mates and teachers are baffled with his condition.

“Growing up with haemophilia was not easy; with my academics and need to do what my fellow mates are doing especially with emergency visits to the hospital and endless pain throughout my childhood. I am living on medications.”

He also said government should support parents and caregivers providing care for haemophilia saying it is not easy living with the condition.

David’s mother, Nkoyo, whose two sons suffer from haemophilia  said David was diagnosed at eight days when she took him for circumcision.

She said the medical staff at the hospital where she gave birth, didn’t know what hemophilia was.

She said, “Imagine a child of eight days going to theatre and coming out more than five times. They were wondering, what the problem was. They even had to give him vitamin K. It was later in the journey that I got to know that even Vitamin K is very dangerous for people living with hemophilia. It can kill them, but we thank God he survived.

“So, we are pleading with the federal government, the Federal Ministry of Health, they should come to our aid, because it’s not a small journey. I am a single parent with two boys living with this   bleeding disorder. At times, two of them will be in the hospital at the same time.

“ Sometimes they stay away from school, and it affects their grades. At time, other children bully them, because they are quiet. They don’t know that they are quiet, because they are trying to stay out of trouble. The last time a child pushed him on the wall, he hit his head. And, I spent so much money.”

 

What is haemophilia?

Dr Udo Christiana, a consultant haematologist and director   North Central Haemophilia Treatment centre, located at the National Hospital, Abuja, said haemophilia is a rare disorder, and involves the inability of the blood to clot properly or on time.

She said, “It’s not that it doesn’t clot at all. It clots But when you have a cut, because you have an abnormality In the clotting factor, you can bleed easily. You. bleed for a long time as compared to a normal person.”

She further explained that haemophilia is an inherited disorder, and an X-linked disease that affects the clotting proteins.

She said, “ We have two types of haemophilia; hemophilia, A and B. Apart from haemophila, there are other bleeding disorders like von Willebrand disease which is the commonest bleeding disorder.

“So, while haemophilia majorly affect the males. Females can be carriers. There are also female haemophiliacs.

“When we say haemophila is an X- linked disease, it means it is transmitted through the X chromosomes. For females, they carry the XX chromosomes, while the males have the XY chromosomes. So, haemophilia, is carried in the X chromosomes, and that’s why  it is called an X-linked disease.

“When a woman with an abnormal X chromosome gives birth to a boy , she will most likely  transmit the abnormal X chromosome to him. So, when a man has the abnormal X, he presents with the disease.

“The reason why  girls are always carriers is because they have two X chromosomes. If one is abnormal, and the other is functional, it can produce the normal clotting factor that is needed to prevent  bleeding.”

 

Women and girls also bleed

Dr Udo Christiana said while the disease is common in men, women and girls also suffer from it.

She said, “ there are also haemophiliac females, and that is why we said both girls and women can also bleed.  Women suffer from von Willebrand disease, and  can also bleed.

“And then we also have girls that maybe have heavy menstrual period. They may likely also have the bleeding disorder. As well as those of them that have real haemophilia.”

The haematologist  also highlighted that when a woman has two abnormal  X- chromosomes,t the girls she gives birth to can also have haemophilia, noting, “That is why some girls can present with a severe form of the disease.”

She said that inspired the theme of this year’s World Haemophilia Day “Women and girls also bleed”. The  World Haemophilia Day is marked on April 17 every year .

While saying there is need for equitable access to care for women and girls, she said there are  about 100 hemophilia patients registered  at the centre.

Diagnosis of haemophilia according to the CDC includes screening tests and clotting factor tests. Screening tests are blood tests that show if the blood is clotting properly. Clotting factor tests, also called factor assays, determine the clotting activity of factors VIII and IX in the blood. Factors VIII and IX are proteins in the blood that help form clots and stop bleeding.

Treatment of haemophila

She said haemophilia can be mild, moderate and severe in sufferers.

She said, “ Those with severe symptoms, their factor levels are very low. The normal protein factor level is about 150. So when someone has less than 1% he has severe hemophilia, and then from childbirth, he or she can just bleed, or bleed post circumcision. Even without circumcision, they bleed severely.

“For the moderate, the level is between one to 5%. So most of these ones don’t bleed Until maybe there is  a cut or they go to the dental clinic and then the dentist is working on their teeth and notice the bleeding , or there’s any form of trauma or a cut.

“For those who have mild haemophilia the factor level is from 6% to about 40%. Those ones, usually, they may never know, until they go for surgery or  any other procedure.”

She said the treatment for haemophilia basically is to replace the  protein or clotting factor that is deficient.

She stated that, “If the person has haemophilia A,  Factor eight concentrate is used for treatment and the bleeding will stop. If the person has haemophilia B , which is factor nine deficient,  Factor nine concentrate, is infused in the patient and the bleeding will stop. Other non-factor concentrators can also be used for treatment. There are also newer drugs that are being used. Once or twice in a week as prevention, even before they bleed.”

She added that the best  treatment is gene therapy meaning that the  gene affected is transmitted back to the patient to produce the right proteins. However, she added that this form of treatment is very expensive and not yet available in Nigeria.

She said, “Already the factors are very expensive and the government of Nigeria is not providing these factors. Some pharmaceutical companies Support the World Federation of Haemophila which in turn donates to patients in Nigeria. Most patients can’t afford it.”

She said HFN  provides free testing when it has support and goes for awareness in rural areas but added that most testing for haemophilia is done in some teaching hospitals in the country.

 

Low awareness and high cost of treatment

Dr Uju Patrick, a medical practitioner and haemophiliac, said he lost 17  members  of his family before he took the bull by the horns to get to the root of the cause of the deaths.

He said he first observed prolonged bleeding from cuts including one when he was in the university that for months lasted months.

 

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He said he also bled when blood is drawn from his arm for test at the hospital.

 

Dr  Patrick, who is also the coordinator of the Haemophilia Foundation of Nigeria (HFN), North Central Chapter said following his diagnosis he realized that those persons who died from his maternal family  actually suffered from haemophilia.

 

 

 

He lamented that  majority of haemophilia cases in Nigeria are undiagnosed because of low awareness.

 

 

 

The medical practitioner said  that only 1, 000 cases of hemophilia are registered with the foundation in Nigeria. With many lives continually being  lost as a result.

 

 

 

He said there was also  dearth of treatment facilities

 

 

 

He spoke during a recent awareness walk on haemophilia organized by the Haemophilia Foundation of Nigeria (HFN) , North- Central chapter, in collaboration with the National Hospital, Abuja in commemoration of this World Haemophilia Day.

 

 

 

He also lamented that patients  face  challenges with delayed access to donated drugs due to procedural hurdles from the  Nigeria Customs Service (NCS) and the National Agency for Food and Drug Administration and Control (NAFDAC),  sometimes leading to expiration.