Sickle cell disease still not given adequate attention

A trip to a traditional healer is what ensues once one is diagnosed and informed that they have sickle cell disease.  

There is still a lot of myth that surrounds sickle cell, Daniel Roy O’dur the Executive Director Uganda Sickle Cell Rescue Foundation says.  

“There is little or no information. When informed of a case of sickle cell they instead say they were bewitched.”  

But there are consequences to that kind of thinking. “When the family chooses to try a traditional healer, in the long run the person with Sickle Cell Disease (SCD) develops more diseases such as malaria, infections and anaemia that worsen their situation,’ O’dur says.  

“It all comes to lack of information. There is a lot of stigmatisation with sickle cell hence the denial,” Tracy Nagawa Project Coordinator Uganda Sickle Cell Rescue Foundation says. 

“Our work to raise awareness is limited we cannot be everywhere.” 

Sickle Cell Disease (SCD) is a major genetic disease that affects red blood cells. The inherited red blood cell disorder causes unusually shaped red blood cells like a sickle and can be fatal.  

The sickle shaped cells can stick to blood vessel walls blocking the flow of oxygen to organs, causing unbearable pain episodes known as crises, that can last up to a week or more.  

The global burden of SCD has not been fully addressed, reports have indicated. In Uganda, 13.5% of the population has a sickle cell trait, according to a 2016 Ministry of Health finding.   

It means one in every seven people has the sickle trait. In that one carries or has inherited a single copy of the gene that causes sickle cell disease.   

Another one in every 150 people has the disorder, SCD.   

Northern Uganda has the highest prevalence of sickle cell at 18.6 percent, a 2014 study by the Ministry of Health, Makerere College of Health Sciences and Cincinnati Children's Hospital says.  

East and Central regions come second with a sickle cell prevalence of 16.7%. The Mid-Eastern and South-Western regions have a sickle cell trait prevalence of 16.5% and 4.1% respectively.   

In Sub Sahara Africa about 240,000 children are born with SCD annually over half 50-80% of these children die before their fifth birthday which is reason to scale up awareness and screening at health centers for sickle cell.  

Insufficient attention to sickle cell  

June 19, 2022 was World Sickle Cell Day commemorated under the theme “shine the light on sickle cell.”  

Although SCD is a major public health concern in Uganda, the interventions that are currently in place are not sufficient to address the current burden, O’dur reveals.  

“Current interventions are targeting doctors. Nurses who are the first contact when one has crises (excruciating pain episodes) as a result of sickle cell are neglected,” O’dur says.  

“In lower health centers you can’t easily get a doctor but you will get a nurse. Everybody (doctors, nurses) should be trained on how to manage SCD,” he says.  

Furthermore, dissemination of information about SCD is not well tailored.  

“Information should be customized and shared in all local languages to reach the last person. Not everybody has access to radio and radio talk shows, its best to also use religious and traditional leaders to communicate and reach every person,” O’dur says.  

He also says, funding to SCD is lacking unlike other noncommunicable diseases (NCDs). “No one is giving SCD attention, it needs to be funded.”  

Further more systematic screening for SCD using a simple blood test is seldom done and diagnosis is often made when a child presents with severe complications.  

Although there is some free testing offered, testing for sickle cell can go for sh25,000 or even sh105,000 not all people can afford it, O’dur says. 

“One time at Kiswa KCCA Hospital, we found a family treating malaria, yet the child had chronic pain and other conditions. They were told to test for sickle cell disease, but they said they had no money. Had it not been our Uganda Sickle Cell Rescue Foundation to intervene and facilitate the free tests, the family would not have established that their child had sickle cells,” O’dur says. 

He calls for hydroxyurea to be given free of charge at every health centre. 

Hydroxyurea makes red blood cells bigger. It helps them stay rounder and more flexible and makes them less likely to turn into a sickle shape.  

“Each tablet of is sh1000 and one has to take it for months or for life in a country where one may not afford sh500 to buy a painkiller like Panadol. It’s too expensive.”  

“When the pain intensifies in a person with sickle cell you see them crying because of the unbearable pain but the drugs are not there in all health centers and not everyone can afford it.”  

“The treatment for pain in persons with sickle cell is morphine its expensive and it has to be administered by two nurses because if it’s just one nurse and they are not upright, they may not inject it all but keep some for commercial gains hence its administered when there are two nurses present,” O’dur says. 

More challenges  

The consequence of sickle cell disease is not only living with chronic unbearable pain for life but also developing systematic complications which those with sickle cell frequently succumb to.  

When it comes to treatment-based services we still lag behind. The only treatment is fluid resuscitation, Nagawa says.  

Extra fluids are routinely given as adjunct treatment, regardless of the individual's state of hydration with the aim of slowing or stopping the sickling process and thereby alleviating pain.  

She says health workers still have little knowledge on sickle cell. “Health centers also don’t have sickle cell centers. For example, one has to move from kaboong to Kampala to get treated for SCD.”  

Nagawa says better infrastructure is needed in every corner of the country to manage SCD.  

“The waiting time that takes place in the health facilities is painful. People with SCD need to be attended to fast. The pain go through nobody can understand unless you are in the shoe of the persons suffering from sickle cell.”