Our clotting disease triggered by the sun

Julie Chedeye (right) with her 16-year-old daughter Shalom Mwakha at their home in Mulwanda Kakamega County on November 20, 2020. [Collins Oduor, Standard]

When she conceived her second born, Julie Chedeye could not hide her excitement. She would soon be a mother of two. But her excitement was short-lived. Within a few weeks, she started feeling unwell, and her doctor recommended bed rest. This was 16 years ago and Julie was just 28.  

“I started feeling out of sorts, and when the doctor ordered me on bed rest, I quickly made the changes I needed to make that possible. My greatest fear then was losing my baby.”  

Luckily for Julie, the bed rest was enough to get her back in gear and the baby, Shalom Mwakha was born beautiful and healthy. It was when Shalom turned four that the cracks began to appear.

“My active and bubbly child suddenly began getting these attacks. Her body would shiver uncontrollably and she would have difficulty walking. She walked on her toes.”

A few weeks after the first symptoms showed, Shalom developed a high fever  and was rushed to the hospital where a paediatrician managed to bring her temperature down.

“After observing her, the medic informed me that it seemed Shalom had inherited “something” from me but couldn’t confirm what it was.”

After a series of tests and consultations with doctors, Julie was told that Shalom possibly had bronchitis, a respiratory illness. She was put on treatment.

But at the age of six, her allergic attacks worsened. She walked with a limp. And when they took her back to hospital, the medic recommended a Doppler test. This is a test that measures the amount of blood flow in the veins and arteries.

The test revealed blood clots in the legs and she was referred to a paediatric rheumatologist who ordered more tests. An MRI of the brain showed several clots with one ventricle of the brain completely blocked. The results confirmed that she suffered from anti-phospholipid syndrome (APS).

Dr Charles Omondi, a rheumatologist, says that APS is a rare genetic disease with no cure. It is a condition where the body reacts against its own generated proteins.

“The syndrome manifests when the body produces protein substances which trigger reaction against normal tissue –lack of self-tolerance. APS can be triggered by an infection, ultra violet rays and smoking.”

“To be sure it is anti-phospholipid syndrome, there must be presence of clots in any part of the body, which manifests with a severe headache which does not go away even with medication. We check for antibodies to confirm if one has APS then scan the body parts for clots. If clots are found, drugs are prescribed,” says Dr Omondi.

Pregnant women suffering from the condition could lose up to three pregnancies all in their first trimester or at 34 weeks. The patients also experience uncontrollable convulsions.

With the diagnosis made, Shalom was immediately started on a drug regime to handle the clots. Since then, she regularly undergoes a test to check the level of blood thickness in the vessels. This helps monitor the likelihood of formation of clots.

“What makes the treatment expensive are the tests which cost more than the drugs. The tests are done bi-monthly with each costing an average of Sh2,000 in public hospitals,” says Dr Omondi.

The drugs also present some side effects like bleeding. 

“The drugs, some steroids, used by the patients, increase the susceptibility of patients to viral infections such as Covid-19.”

A family affair

In the course of managing Shalom, Julie also started developing similar symptoms; body jerks with lots of body pains that need sedation to abate.

Her husband George recommended she gets tested in the US and India on the advice of local doctors. In India, she was treated for back pain while in the US they found out that she had an enlarged heart and was put on medication.

But three years ago her condition worsened and she was rushed to Kisumu Specialist Hospital where tests found clots in her lung arteries (pulmonary embolism). Like her daughter, she had APS.

“It was a shock to us to find out that it was the same syndrome, yet she had been put on different medications that had little impact on the problem,” says George.

Doctors advise that patients avoid smoking, minimise sun exposure and if they have to be out, to wear sunscreen religiously.

“Also, there is need to avoid risk of mouth infections which are known to spur APS attacks.”

While APS happens sporadically in a population, it can sometimes be hereditary and George suspects that it runs in their family.

“Julie’s grandmother succumbed to blood clots in her left leg. So maybe that is the link.”

Today, mother and daughter are on a drug regime that includes a cocktail of painkillers to manage the condition.

Before Covid-19 struck, they would both be admitted to hospital at least once a month but now they practice home-based care.