…Victims labelled “children of the night”
BRIDGET MABANDA/ADMIRE MASUKU
SYMPTOMS upon first exposure to sunlight: Freckling on sun-exposed areas. Dry skin. Pimples resembling blisters develop into spider web-like vessels and eventually dark brown tumours.
If your child is showing these symptoms, you need to see a medical practitioner – they are associated with a rare, incurable genetic condition: Xeroderma pigmentosum. The condition is hereditary and develops in young children. In most cases, diagnosis is often done late.
The condition renders the body unable to repair damage caused by ultraviolet radiation, leaving the skin too sensitive to sunlight. In the absence of sun protection, their skin ages, becoming dry, rough and atrophic, according to an article in the Oprhanet Journal of Rare Diseases. In extreme cases, it results in skin cancers, blindness, neurological disorders and premature death.
To date, seven children have succumbed to the condition, according to the Xeroderma Pigmentosum Family Support (XPFS), a non-governmental organisation that looks after the lives of those diagnosed with ultraviolet light conditions.
The organisation started operating informally in Zimbabwe in 2017 and was formally registered in 2019.
Apart from assistance from the organisation and its funders, very little is being done to provide support to the victims by the government.
On 26 November last year during a press briefing, and in a tone that showed little attention is being paid to the condition, the Ministry of Health and Child Care appealed to hospitals to work with the XPFS Zimbabwe to support children with the condition.
“I urge hospitals, where possible, if they can also chip in and allow XPFS to use a selected room as XP registration at their premises,” said Dr John Mangwiro, the deputy minister of Health in a speech read by Dr Wenceslus Nyamayaro.
The deputy minister’s tone, and his appeal that XPFS be given space to operate in hospitals already paralysed by a shortage of medical professionals, displayed a casual approach to an emerging and potentially disturbing health problem.
Dr Mangwiro made a desperate plea: “I now call upon the corporate world and other related bodies’ support in terms of funding, through donations–cash or in kind.”
He called on stakeholders to help “construct or rent a house to accommodate XP children when they come for treatment in Harare.”
Kudzai Lucy Matura, a 26-years-old XP patient who has been living with the condition since birth but was diagnosed at the age of 11, said XP patients were being shunned.
“XP patients usually miss their appointments in Harare as some of them come from faraway places. They end up sleeping on the streets. Many people do not want to accommodate them because of their condition,” she said.
“Due to stigmatisation, we feel like second-class citizens. People call us all sorts of names, for example some say we are vampires; we are ghosts. Our eyes are different from those of other people and that causes them to view us differently.”
While health professionals display a lackluster attitude to the condition, cases continue to grow.
Between 2019 and 2020, a total of 88 cases of children with Xeroderma pigmentosum were recorded.
Chenjerai Takaedza, a Masvingo-based male teacher who lost his 11-year-old son in August last year to the condition, says it is difficult to get treatment.
In a poignant voice, he narrated how he and his wife frequented Gweru Provincial Hospital from the time the child was one-and-half-years-old in 2009.
He was only told after 11 years (last year February) that his child had the condition. All this while, health professionals were fumbling for answers and experimenting with her child.
Parents whose children have XP feel neglected and question the competence of health professionals in dealing with the condition.
“It is distressing that the cases continue to grow yet medical practitioners are failing to diagnose the disease at an early stage,” said Chenjerai.
“In most cases, by the time they do so it will be too late.”
Pauline Mapuvire, the founder of XPFS, says “what is killing the children is not the condition, it is late diagnosis.”
“Most families seek medical attention when it is too late because they associate the condition with witchcraft. Ignorant of the actual cause of the condition, most families seek help from witchdoctors and prophets who usually worsen the situation by delaying treatment,” Mapuvire said.
She said the condition makes children active during the night when there is no sunlight. During the day, they remain indoors. As a result, “they are called moon children or children of the night.”
Unable to cope with the level of ridicule, Mapuvire says some parents “end up hiding their sick children in the house”.
“They eventually die a painful death locked up in the house,” she said.
Research shows that when detected early, effects can be reduced through wearing protective clothing, using sunscreen, and regular consultations with dermatologists, ophthalmologists, neurologists and dentists. But all these are just life-prolonging measures which do not constitute a cure.
XP patients need regular check-up but most parents can hardly afford the costs. Fatima Muzize, an XP caregiver, says as part of the treatment procedure patients have to use expensive, specialised creams beyond the reach of many.
“50ml of Vaseline Dermatology Formula lotion is going for US$20. Skin care doctors are charging exorbitant fees for check-up,” she said, adding that some of the creams are not found in Zimbabwe.
Regrettably, some marriages have collapsed after children are diagnosed with the condition.
According to XPFS, three quarters of 88 known mothers whose children have the condition were deserted by their husbands. They are blamed for bringing a “curse” upon the children.
Unable to handle the situation, suffering from abandonment and being accused of bewitching children, the majority of them suffer from stress-related medical conditions.
“As pressure mounts on these women, they end up suffering from high blood pressure as well as mental health diseases,” Mapuvire said.
Their plight is worsened by the fact that very few health practitioners have experience in dealing with the condition. Poverty of information on the condition is widespread, and even so in health corridors.
Donald Mutangadura, a specialist dermatologist based at Baines Medical Centre in Harare, says some of the cases are not referred to dermatologists because doctors attending to XP patients have insufficient knowledge on the condition. XP is not taught in medical school.
“It’s just a sentence in the whole syllabus,” says Mutangadura.
Opinion on the causes of the condition is divided.
It is also believed to be common in the Middle East where consanguinity is practiced (marriage between people with blood relations). But Mutangadura says the condition is inherited.
“If parents have recessive genes, they can give birth to a child with Xeroderma Pigmentosum,” he said.
Mutangadura said in Zimbabwe, at least one case is recorded in approximately four to five months and “some cases are not referred to hospitals.”
The average life expectancy is shortened for many individuals with XP due to the increased risk of cancers, says Anna Mary Nyakabao, a clinical oncologist with the Parirenyatwa Group of Hospitals.
XP is found on all continents and racial groups.
However, statistics vary from one race to another and from continent to continent. XP incidences vary from 1 in 20 000 in Japan to 1 in 250 000 in the United States of America, and approximately 2.3 per million live births in Western Europe, according to an article in the Oprhanet Journal of Rare Diseases.
