Hemophilia: Raising children with the bleeding disorder

Agnes Kaddu shows her son Joel Mawejje’s swollen knee. She says whenever Joel falls on his knees, they swell due to internal bleeding and in most cases the swelling does not shrink back to normal. Photos by Esther oluka

What you need to know:

  • When a patient has low levels of Factor VIII, we term that as Hemophilia A while deficiency (low) levels of Factor IX is called Hemophilia B
  • Between one and five per cent activity, that is moderate hemophilia. Then, between five and 40 per cent activity that is mild hemophilia

“He was lying in a pool of blood on his bed,” Agnes Kaddu recalls.
When Joel Mawejje was seven months old, he was circumcised. Upon returning home from hospital, Mawejje’s mother, Kaddu, breastfed him before eventually taking him to bed. A few moments later, Kaddu went to check on her son and what she saw sent chills down her spine.

Upon close inspection, Kaddu realised that blood was emerging from the circumcised wound. The worried mother rushed her son to a nearby clinic in Kitintale, a Kampala suburb. At the clinic, Mawejje was given some medication and the wound dressed.

“But even after all that, the wound continued bleeding without stopping,” Kaddu says.

Kaddu was advised to take her son to another health facility. The toddler was then rushed to Kibuli Muslim Hospital. By the time they reached, Mawejje was pale and his body so cold after losing a lot of blood. Right then, he was given a blood transfusion. That day, Mawejje’s father had travelled to the village for a burial and that’s why the responsibility of looking after Mawejje was solely on Kaddu.

Mawejje was discharged later that day. However, upon reaching home, Kaddu says, the wound starting bleeding again.

“I had no choice but take him back to hospital and the wound was dressed again,” she says adding that the wound did not heal for an entire month, hence, making hospital visits a back and forth routine.

“The doctors could not understand what was going on with my son,” she says.
In fact, one intentionally pricked Mawejje’s ear as a way of checking and confirming whether the uncontrolled bleeding would also likely happen in other body parts. “Just that small prick refused to stop bleeding until it was tightly covered with a plaster,” she says.

As Mawejje continued to heal from the circumcision wound, Kaddu began suspecting that her son had hemophilia even before he was diagnosed. The reason for her suspicions came from her family history of losing two brothers to the condition.
“The same symptoms Joel exhibited are the same my brothers had before they died. All I remembered is that even the smallest wound on them would bleed nonstop,” she says.

The diagnosis
One day, during his earlier childhood, Mawejje fell down and developed a swelling on his knees. When the swellings stubbornly refused to subside in a year, Kaddu and her husband decided to take their son to Mulago National Referral Hospital where they saw a hematologist (a doctor who specialises in the diagnosis, treatment, and prevention of diseases of the blood).
The hematologist referred them to LMK Medical Laboratory in Kampala where tests were done before it was later confirmed that Mawejje had hemophilia B, severe. At the time, he was five years old.
Kaddu was surprisingly relieved that her suspicions of her son having hemophilia had now been confirmed. At least now, the couple knew what they were dealing with.
Another scare came when Mawejje fell down and broke a tooth. “We tried to control the bleeding using cotton wool in vain,” she says. Mawejje was later admitted at Mulago National Referral Hospital for about two weeks.

According to Dr Philip Kasirye, a pediatrician and hematologist at Mulago National Referral Hospital, the only way doctors can manage the condition is by giving the patient artificial factors that stop the bleeding. He adds that most of the factors being used at the moment are donated by World Federation of Hemophilia under the Hemophilia Foundation of Uganda.

Mawejje is 10 years old and has two younger siblings; a girl (eight) and a boy (three). The three-year-old also has hemophilia B, severe after getting diagnosed at five months old. Although the girl, unlike the boys, is fine, Kaddu suspects that she must be a carrier of the disease.

Hemophilia has no cure, and, for this reason, Kaddu says she always tries to look after the boys the best way she can.

“I am always cautioning them to be careful, especially when they are playing. I am always afraid for them to get cuts because even the slightest can become very bothersome,” she says.
However, Kaddu is always prepared with first aid just in case of any accidents.

“Whenever any of the boys gets either a cut or swelling, I immediately apply ice to control bleeding. The ice actually works by either reducing the swelling or stopping the blood flow,” she says.

On occasions when the bleeding fails to stop, Kaddu seeks immediate medical attention. She says the disease has mostly affected her eldest son. In fact, she even shows me his swollen joints that have not shrunk back to

“Whenever he falls on his knees, they swell as a result of internal bleeding. One may think the swelling has pus, yet, it is blood. Whenever these joints swell, they hardly go back to their original size, hence affecting mobility,” she says.

Kaddu has another brother, Gideon Dradu, 19, who also suffers from the same condition. Dradu, who currently stays with his sister, says he was diagnosed with the disease at the age of five. Before the diagnosis, the doctors thought he had sickle cell anaemia.

But after the right diagnosis, Dradu says the doctors started according him the right treatment. Regardless, he still faces a number of challenges in his daily life.

“Whenever I would hurt myself and blood starts flowing, it would not stop until I sought the appropriate treatment,” he says.
Dradu says one time, in the past, after hurting his right foot, it bled continuously and took about five months to heal causing the slight limp he now walks with.

But for Kaddu, her hope is that one day, a permanent cure for hemophilia will be found.

What is hemophilia?

Dr Philip Kasirye, a pediatrician and hematologist at Mulago National Referral Hospital, says.

“Hemophilia is a hereditary or genetic blood disorder that relates to prolonged or uncontrolled bleeding. Symptoms may manifest as swellings on the ankle and knee joints, especially after an injury. These swellings are a result of bleeding under the skin. In reality, patients lack factors that enable the formation of fibers that hold the clot. The disease mostly manifests in males who are born with it. However, it may also occur due to environmental factors that stress the body.
Part of the diagnosis involves measuring factors basing on Factor VIII (8) and Factor IX (9) which are more associated with hemophilia. When a patient has low levels of Factor VIII, we term that as Hemophilia A while deficiency (low) levels of Factor IX is called Hemophilia B.

Type A is more common than B. The types are graded. If someone has less than one per cent activity of any those factors, that is severe hemophilia. That is why you will find some patients being diagnosed with Hemophila B, severe. Between one and five per cent activity, that is moderate hemophilia. Then, between five and 40 per cent activity that is mild hemophilia. Lastly, if one has factor activity above 40 per cent, that is almost normal hemophilia.