6 minute read
Haemophilia: Becoming Haemo-aware
By Godfrey Ekisa Osang’ir
The central nature of blood functions in the human bodies cannot be underestimated, nor overemphasized, as the red fluid defines the health and life of every soul that is alive.
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Blood has many functions one of which is to ensure a well regulated balance between bleeding, forming a stable clot and the body breaking down this clot to prevent blockage of blood vessels; a process called hemostasis.
Hemophilia is an inherited bleeding disorder that results in those affected having low levels of clotting factors that are important to avoid prolonged bleeding. Hemophilia A is more common and these patients have clotting factor 8 deficiency whereas Hemophilia B which is less common; patients have factor IX deficiency. Hemophilia can also be acquired where patients develop antibodies to the clotting factors therefore resulting in bleeding. There are other bleeding disorders such as von Willebrands disease.
Hemophilia has a male predominance due to how it is inherited (X-linked recessive pattern) and women are mostly carriers meaning they have one X chromosome with the abnormal gene and the other is normal therefore they do not manifest with disease.
People living with hemophilia are at high risk of spontaneous bleeds mostly into the joints such as the knees, elbows and ankles or prolonged bleeding and bruising during physical activities. This may also manifest with prolonged bleeding after minor procedures like tooth extraction. Life threatening bleeds can also result such as bleeding into the brain, vomiting blood or bleeding into the throat or neck which can result in breathing difficulties.
Dr. Victoria Kithinji- Medical Officer prepairing a haemophilic patient for Factor Infusion procedure
PHOTO | STEVE MUTUKU
Dr. Kibet Shikuku, Chairman of Kenya Hemophilia Association and a lecturer at the Universty of Nairobi, Pathology Department says that for the all-important red fluid to clot, the body needs special proteins called clotting factors and blood cells called platelets.
“Normally, platelets clump together to form a plug at the site of a damaged or injured blood vessel. In people with bleeding disorder, however, the clotting factors or platelets don’t work the way they should or are in short supply,” Dr. Kibet says.
A bleeding disorder is a condition that affects the way a human’s blood normally clots. The clotting process also known as coagulation, changes blood from its liquid state to solid.
“When a person is injured, blood normally begins to clot to prevent a massive loss of blood. Congenital deficiencies of plasma proteins involved in blood coagulation generally leads to life-long bleeding disorders,” Dr. Kibet affirms.
Though are statistics indicate that one in a million people have hemophilia and efforts must be put in place to support those with this condition, to live a normal life. Early diagnosis is key in ensuring proper management and avoid or reduce complications.
The KNH Haemophilia and Bleeding Disorder Clinic was launched in 2014 through the support of donors to provide comprehensive care to patients with haemophilia and bleeding disorders. The clinic is currently located in Clinic 17 and runs from Monday to Friday from 8am to 5pm.
The clinic serves both adult and paediatric patient population. It is staffed by nurses and a medical officer trained in Haemophilia patient care and work with both paediatric and adult haematologists. The success of this clinic has been hinged on support from the KNH management, Kenya Haemophilia Association (KHA) and donation of factor by the World Federation of Haemophilia (WFH). To date the clinic registry has 302 patients who follow up.
In a move aimed at giving a lifeline to people living with hemophilia, Kenyatta National Hospital in collaboration with the University of Nairobi conducted a week-long circumcision initiative from 28th March to 1st April 2022.
A total 55 male patients, including 25 adults and 30 minors underwent the circumcision exercise, which was aimed at painting a smile on the special group that has endured pain and anguish, especially whenever faced with a cut that exposes them to life threatening bleeding.
Thirty-eight-year-old Joshua Mutinda, a hemophilia patient who benefited from the circumcision exercise has been living in fear of the cut, a situation that sidelined him from his peers. He states “since most of them do not understand what really it is to have hemophilia as a condition.”
“The aftermath of the circumcision procedure was such an exciting moment for me. The experience was so refreshing that I felt like a new lease of life had been injected into my existence,” an elated Mutinda says, as he beams into the future that equates him with his peers, especially in an environment and culture that traditionally circumcises men. “Male circumcision in people with hemophilia is possible and safe when performed be trained surgeons and under the cover of clotting factor concentrates,” says Dr. Matilda Ong’ondi, Consultant Physician and Clinical Hemato-oncologist at the KNH and Head of Hemophilia Clinic. “This has been a great success with amazing team work from a multidisciplinary team that included surgeons, hematologists, nurses, lab technologist among others. In addition there was participation from teams from other counties and Tanzania with a hope of facilitating the same at county levels,” Dr. Matilda added.
This exercise was possible through local and international partners who all played pivotal roles. The Kenyatta National Hospital who provided a ward dedicated to the exercise and ensured it was well staffed as well as the surgical team who dedicated theatre space for the weeks exercise. The World Federation of Hemophilia (WFH) who provided the clotting factor concentrates, Roche facilitated inhibitor testing for all the patients, NovoNordisk Hemophilia Foundation and WFH that assisted in logistics for training during the exercise and Kenya Hemophilia Association who coordinated and organized logistics for the exercise and training.
The Kenyatta Hospital continues to live up to its motto; “We listen, we care” and this week it was by meeting a special need in people living with Hemophilia.
A paediatric Haemophilic patient being prepared for the cut
PHOTO | STEVE MUTUKU
