Rare heart condition threatens girls’ lives

Monday, September 2nd, 2019 00:00 |
Beth diagnosed with Patent Ductus Arteriosus, heart condition where a vessel in the heart does not close soon after birth. She need at least Sh600,000 for treatment. PD/Wangari Njuguna

Wangari Njuguna and Sandra Wekesa

As thousands of students countrywide prepare for the national exams, the fate of two pupils from Maragi Primary School in Kiharu near Murang’a town, hangs in the balance due to a rare health condition.

Beth Wanjiru and Lilian Wanjiru, both aged 13, have holes in their hearts — a condition that has affected their learning and has seen them in and out of the hospital. 

Occasionally, when they get weak, they stay home, and when in school, they isolate themselves something their teacher, Nancy Wairimu, says is necessary to protect them. “Their condition is fragile and we don’t allow them to play with others because we don’t know what might happen to them,” she adds.

According to their medical reports, both of them have a condition known as Patent Ductus Arteriosus (PDA), a heart defect in which an extra blood vessel formed at birth to help with foetal blood circulation fails to close shortly after birth. Beth not only has two holes but also suffers another congenital heart disorder known coarctation, the narrowing of the large blood artery (aorta). 

How it forms

Dr John Maina, the paediatric cardiologist at Josma Health Services and lecturer at Kenyatta University Medical School, says before a baby is born the lungs are not functional, meaning blood bypasses the fluid-filled lung thanks to an extra vessel called ductus arteriosus.  However,  when a baby is born, the lungs open up and it is filled with air. 

Beth and Lilian Wanjiru were diagnosed with Patent Ductus Arteriosus,  heart condition where a vessel in the heart does not close soon after birth. They need at least Sh600,000 for treatment.   PD/Wangari Njuguna

Dr Maina explains that the ductus fails to close after birth in cases where babies are born prematurely, are too small, are born with the umbilical cord around the neck, are born with a lot of stress or did not cry well. This means there will be an abnormal transmission of blood between two major heart vessels: aorta and pulmonary artery.  It also means the lungs will not be able to pump enough oxygen to the rest of the body.

In the case of premature birth, babies’ muscles are not able to close properly as they lack enough strength to constrict, thus keeping the ductus open.“That is why they might need surgery or drugs administered for it to close,” he says.

Time taken for the hole to close depends on its size: if it is large, it will require surgery. However, if it is small, administering medicine such as indomethacin anti-inflammatory drug is necessary. “If medication is not administered within two weeks, it may not work on the infant,” he cautions. 

Dr Maina says about 10 per cent of all congenital heart diseases globally is projected by PDA, meaning that 1 in 1,000 life birth comes with symptoms of PDA that normally are treated when the child is still young.

The surgical mortality rate in premature infants ranges from 20 per cent to 41 per cent. With the availability of antibiotics, a low-risk surgery and catheter technique to handle the condition, the mortality rate appears to be quite low, except in the extremely premature infants. It is estimated that if left untreated, the mortality rate for PDA is 20 per cent by 20 years, 42 per cent by 45 years, and 60 per cent by 60 years. 

Risk factors

In Kenya, however, there are no statistics, but survival rates decrease in patients with large holes.  In Beth’s case where she has two holes in her heart, there is no known clear cause. Dr Hassan Adan Ahmed, a Cardiology Fellow at the Aga Khan University Hospital in Nairobi, says genetic factors play a role in such heart conditions. 

“PDA can also occur along with other heart defects. Some risk factors include: premature birth, family history and other genetic conditions, Rubella infection during pregnancy and being female as PDA is twice as common in girls than boys,” he adds. If the condition is left untreated, the blood pressure in the baby’s lungs might increase (pulmonary hypertension) and the baby’s heart might enlarge and weaken.

Depending on the size and age of the baby, the condition can go undetected even in adulthood. However, a large hole can cause signs of heart failure soon after birth. In childhood, the signs are varied and might include poor eating, sweating with crying, breathlessness or fast breathing, tiring faster and rapid heart rate.

“The major issue children encounter mostly is a difficulty while breastfeeding, because they flood the lungs with the whole process of feeding. Other than that, the children feed slowly and cry a lot,” Dr Maina says.

Funds appeal

He adds that the cost depends on the hole size and type of treatment. “Costs depend on the size of the hole. For starters, patients need to pay between Sh7,000 to Sh10,000 for electrocardiography, the test to determine how big the hole is,” he says. For surgery, the amount can go up to Sh600,000.  

For the two teenagers, the cost of required surgery is too steep. Margaret Wangui, Beth’s grandmother, relies on casual jobs for a living. “I used to take her for medical check-ups, but it became too expensive and we had to discontinue,” she says.

To treat Beth’s condition, the doctors, in 2015, recommended catheterisation (insertion of a tube) of the aorta as she awaited the major surgery. The procedure costs Sh25,000, but her grandmother could not raise it.

Susan Wambui, Lillian’s mother, is always worried about her daughter when she is in school for she has had, on several occasions, been called to rush her to hospital.

Phyllis Kamau, the school’s headteacher says they have consulted various hospitals and the cheapest they have gotten is Sh1.2 million to rectify the condition for each child. They are currently on the lookout for funds to help alleviate the pain and allow the teens to continue with their studies.  The two Wanjirus are determined to study and achieve their dreams to be

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