Sad tale of sickle cell ravaging Lango

The burdens of a rural home can be daunting but can be worse if there is a sick soul in the home, especially if the condition is chronic.

In Lango sub-region in northern Uganda, worse stories have been told but the burden that has been presented by sickle cells is pain in itself.

Stories of how men have fled homes, accusing their wives of bringing “misfortunes,” are littered all over.

Data indicates that more than 100 cases of child neglect and desertion are recorded at Lira Central Police Station on a weekly basis. Sixty of these in Dokolo District alone have been reported to be sicklers.

However, no direct data has been linked to the desertions but there is belief that the trend is a result of the burden presented by the sickle cell condition.

In March, Dorcus Apio of Kakoke of Ojwina Division in Lira Municipality, woke up to a shocker. Her husband had fled their home after they confirmed their two-year-old daughter to be a sickler.

“The condition was detected in February, and my husband said I had brought misfortune in their family. He fled and has since not given me support,” Apio says.

Apio is not alone. In Acaba Sub-county, Oyam District, a three-year-old girl was in January abandoned at her grandmother’s home after testing positive for sickle cell.

At 50 years, Joyce Okullo is ageing and looking frail but she has to take care of the three-year-old whose status has to be observed every Friday at Lira Regional Referral Hospital.
“Her eyes always turn yellow,” Okullo explains to the health worker, who every Friday is at the hospital to conduct specialty tests.

But beyond the social disconnections is the challenge that comes with treating a sickler.
Sekondina Alobo has for 14 years been treating her daughter, who at only one year tested positive for sickle cell condition.

“It has been an expensive experience. The drugs are hard to get. Most times it gets frustrating when I come to the hospital and there are no drugs,” she says.
But the frustration aside, for 12-year-old, Elizabeth Ajok, it has been a painful journey, which doctors associate with low fluid levels.

“I always feel a lot of pain all over my body,” Ajok says.
In Uganda, more than 20,000 babies are thought to be born with the sickle cell disease annually, but accurate data is unavailable.

The Ministry of Health has chosen Lira Hospital to be a referral centre for sickle cell complicated cases. The Lango sub-region and Alebtong District have the highest prevalence.
Experts believe sickle cell prevalence is high in areas with high rates of malaria.

On average, there are at least two sicklers per 10 families in Lira District, and these have reported home desertion in the last two years, Philomena Okello of Lira Hospital, says.

“We have even involved legal minds to help. We are getting so many reports of men abandoning their families,” she says.

Managing the disease, she says, has become difficult because of ignorance exacerbated by the stigma vented towards sicklers.

“When they look at the pain sicklers go through, the parents get traumatised. It is a painful burden,” Okello says. Besides the pain, Florence Ocen, a pediatrician at Lira hospital, says sickle cell comes with a lot of trauma.

But how can sicklers be made to feel better about their status?
The most important aspect is sensitisation.

Fine, Ocen says, the condition could be manageable but it is important to avoid it, if that possibility is there.

“Before getting into committed relationships that could involve bearing children check for traits of sickle cells,” Ocen advises, noting that it is advisable that carriers do not get into a relationship that might involve producing children.

The sickle cell threat

Sicklers have abnormal red blood cells and according to Ocen, they live a highly fragile lifestyle.

Most of them are extremely weak because of the very low blood level manifested in the yellowish skin display.

Because sicklers experience low oxygen supply, they get swellings resulting from blood blockages.

“They live a sickly life that is characterised by pain,” Ocen says, highlighting the burden that comes with the condition.

At Lira Hospital, a sickle cell association has been formed to source support for victims.
Sicklers, it has been found out, are resistant to malaria because they carry a gene that protects them from the infection.

Ana Ferreira of the Gulbenkian Institute of Science in Oeiras, Portugal, conducted a research in which she discovered that a mouse that had been injected with a single gene of sickle cell had grown resistant to malaria.

The research found that the gene is not only protected from malaria but fights any form of intrusion even when it has been forced its way into the body.

Treating
Preventing painful episodes. Avoiding possible triggers such as drinking plenty of fluids, wear appropriate clothing not to get cold as well as avoid sudden temperature changes, such as swimming in cold water. If you get more pain, a medication called hydroxycarbamide taken in a capsule form is recommended.

Preventing infections. Most sicklers will need to take a daily dose of antibiotics (usually penicillin), often for the rest of their life. Long-term use of antibiotics won’t pose any serious risks to a sickler.

Bone marrow transplants. Stem cell or bone marrow transplants are the only cure for sickle cell disease, but they are not done very often because of the significant risks involved.