Rare blood disorder that plagues my life – von Willebrand Disease

By Milliam Murigi On Mon, 19 Aug, 2019 08:00 | 3 mins read
Molline Atieno explains her struggle with the vWD, a rare blood disorder. Photo/MILLIAM MURIGI
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    Molline Atieno started nosebleeding at Kenyatta National Hospital in 2010 when visiting her convalescing brother, it was not such a big issue for she was used to nosebleeds.

    It comes in four types:  vWD type one, which is common and has mild symptoms such as nosebleeds and bruising. Type two has mild to moderate symptoms and is broken down into 2A, 2B, 2M and 2N, depending on quality and quantity of the vWF.

    Haematologist, Dr Kibet Shikuku, says pregnant women with vWD need close monitoring by a multi-sectoral team that includes obstetricians/gynaecologists and blood specialists.

When Molline Atieno started nosebleeding at Kenyatta National Hospital in 2010 when visiting her convalescing brother, it was not such a big issue for she was used to nosebleeds.

She had had them nearly everyday since she was four years old, an annoying occurrence her parents repeatedly dismissed as insignificant. However, this particular incident prompted a more detailed test on her and other family members. It was established that the family had a bleeding disorder known as von Willebrand Disease (vWD).

“My parents used to think that it was not a disease, but a family condition because our grandmother used to have recurrent bleedings and she didn’t get to go to the hospital,” she adds.

 Fatigue and nausea

VWD happens when clotting protein known as the von Willebrand factor (vWF) is missing or is defective. VWF helps platelets mesh to form a clot to stop bleeding. However, in people with vWD, it takes longer for blood to clot and for bleeding to stop.

It comes in four types:  vWD type one, which is common and has mild symptoms such as nosebleeds and bruising. Type two has mild to moderate symptoms and is broken down into 2A, 2B, 2M and 2N, depending on quality and quantity of the vWF.

Type three has severe symptoms including spontaneous bleeding episodes into joints and muscles.  Acquired vWD occurs in adults, especially after diagnosis of autoimmune conditions such as lupus and after taking certain medication.

Research has shown the disease, also known as angiohaemophilia, runs in families and mostly affects people with blood group O. Symptoms may delay in girls until their first period. 

Though, they were taught how to prevent and manage the bleeding, Atieno never took it as a major problem until her first period at 16. Her periods were prolonged and could even last for a week or more, leading to fatigue and nausea due to loss of iron.

“I had signs and symptoms since I was four years old, now I am 34  and  it was never really looked into,” she says. After going through the same problem repeatedly, she decided to find out how to manage her condition.  

No statistics

During her online research, she met Laurie Kelley,  who runs an educational forum for individuals and families dealing with haemophilia, another bleeding disorder related to vWD, in the United States.

She was invited to the US, and after listening to Kelley’s story on how she managed to bring up her three children, one of whom lived with the condition, Atieno realised the condition was manageable.

“At first I was afraid that someone with a bleeding disorder could not give birth, but after seeing how such women are raising their families, I was encouraged,” she adds. 

Upon her return to Kenya, Atieno joined a support group to increase awareness of vWD. Currently, she is a member of the Kenya Haemophilia Association and Jose Memorial Haemophilia Society of Kenya, which educates, creates awareness, and provides treatment and support research on haemophilia and other blood disorders. They also provide factor concentrate on persons with haemophilia to prevent and manage bleeding. 

Atieno is a mother to a baby boy. Because of the vWD complications, a specialist doctor monitored her throughout the pregnancy. It was a difficult time for her as she used to bleed frequently, leading to a premature delivery at just seven months. Her baby is a constant reminder of how blessed she is.

Haematologist, Dr Kibet Shikuku, says pregnant women with vWD need close monitoring by a multi-sectoral team that includes obstetricians/gynaecologists and blood specialists.

These specialists should also draw up a delivery plan to ensure the patient does not bleed out as well  as check the child to determine if they have vWD.

Whereas it is estimated that up to one per cent of the world’s population suffers from vWD, Dr Kibet says the number of people with vWD in Kenya is unknown due to lack of awareness.

The lack of a central repository and specialised centres to effectively manage the condition also contributes to this lack of statistics.  “The treatment involves giving clotting factor concentrates to prevent bleeding and bleeding-related complications in people with vWD but each case is handled on its own to allow a proper analysis of how the condition presents,” he  adds.

Kibet also notes that vWD can be passed down from parent to child of either sex. However, in some cases, a spontaneous change in the vWD gene before the baby is born can cause it.