Demystifying Sickle Cell

By Sharifu Kiragga Tusuubira 

Sickle Cell is the world's commonly known genetic diseases affecting every community, religion and social class. A study carried out by Uganda Sickle Cell Rescue Foundation (USRF) in collaboration with researchers from Clarke International University revealed that for the vast majority of people, exposure to and knowledge of Sickle cell disease (SCD) stemmed from their family and friends.

This study also indicated that health professionals were the least likely source of Sickle cell-related knowledge. Families and friends have been identified as precipitators of stigma and discrimination coupled with myths/beliefs about Sickle cell disease. One participant in the study was quoted to say that ‘my parents always cautioned us from marrying from a certain family because they always heard somebody hospitalized'.

This calls for the need to demystify Sickle cell in Ugandan communities starting with the commonest myths.

Myth one: Sickle cell is a curse in the family

Sickle cell is a condition in which the cells responsible for carrying oxygen in the body or red blood cells are not shaped as they should be. A normal red blood cell should be round disc-shaped but in this condition, they are shaped like Sickles or the crescent moons.  This condition is genetically passed on thus it is not some kind of curse that requires visiting witch doctors.

Myth two: Sickle cell occurs because a person married a partner from another tribe or is always brought by the mother of the child 

To have a child with this condition means that ‘both parents' are carriers or have genes for Sickle cell. Previous studies have proved that 13.5 % of Uganda's total population are carriers, meaning every one in seven people are most likely going to genetically pass on this condition. Ideally, this is not exclusive of the tribe and as such marrying a partner from another tribe is not the cause of the baby's Sickle cell.  This means that each one of us has a responsibility to know their Sickle cell status/genotype and to encourage couples that are about to get married to take a Sickle cell test for screening is the only way to prevent Sickle cell disease.

Myth three: Sickle cell is contagious  

Any contagious disease arouses a fear due to the belief that one is going to contract it. However, as earlier highlighted Sickle cell is an inherited blood disorder. This means that a person cannot get Sickle cell through associating or having sexual intercourse with another person living with the disease. No one should, therefore, stigmatize anybody having a Sickle cell.

Myth four: People with Sickle cell are ‘Sicklers'

A simple search on Google would show that ‘Sickler' means someone with Sickle cell disease however from my understanding the term was devised from the fact that the red blood cells are Sickle shaped. However, the ‘sick' part in the word has dominated this meaning and seems to be the usual reference when the word ‘Sickler' is mentioned. The connotation is that "one is always sick" which is wrong! A person can be sick but this doesn't stop them from being a productive member of the society - thus labeling people living with Sickle cell as "Sicklers" perpetuates stigma unjustifiably. 

Myth five: People with Sickle cell die at a certain age

In Uganda today, up to 80% of those born with Sickle cell would die before their fifth birthday. This is attributed to a lack of comprehensive care, which is a prerequisite for holistic management and care for the people living with Sickle cell.  However, one of the oldest Ugandans living with Sickle cell today is a 63-year-old lady who is currently being supported by the Uganda Sickle Cell Rescue Foundation.

In one of the school outreaches, a student living with Sickle cell was quoted to say "I dropped out of the biology class because the teacher said people with Sickle cell disease die before the age of 20". It is wrong to believe that because a person has Sickle cell they are dying anytime soon. Just like any illness, a person with a Sickle cell can die at any age.  

Myth six: People with Sickle cell cannot become parents

It is a common belief that Sickle cell disease makes one infertile. Yes, there could be Sickle cell related complications that could cause one to be infertile e.g priapism - a complication that causes painful erections mostly in men.

If not managed, this could lead to penile dysfunction (or a condition where one is not able to get an erection or keep it firm enough for sex).  However, the occurrence of this complication is up to 3.6% in patients less than 18 years of age meaning that it is a rare occurrence. This, therefore, disregards this myth because the vast majority of males with Sickle cell are able to father children. 

Similarly, women with Sickle cell can get pregnant and conceive children normally. However if pregnant, such a pregnancy is referred to as a high-risk pregnancy. This means that either the mother or the baby might be at a high risk of exposure to health problems before, during or after delivery.

Typically, special monitoring or care throughout pregnancy is needed.  Sarah who lives with Sickle cell once sought our intervention when a midwife at one of the big hospitals asked her "Do you want to die? Why did you get pregnant?" Such a midwife and many others, deny our daughters the chance to enjoy a family life because they scare them with death if they got pregnant.  The sickle cell, therefore, doesn't necessarily cause one to become infertile.

Myth seven: People with Sickle cell should not work

The vast majority of people believe that once a person has Sickle cell they will always be sick while others believe such a person will be very weak to work. Like any other person in society, a person living with Sickle cell can be productive. Employers should therefore not deny persons living with Sickle cell chance to employment.

It is a high time people living with Sickle cell challenged the narrative and started telling their own stories because they are normal people who are contributing to the development of our country.  Having Sickle cell is not a death sentence, and as such, people living with it have rights to independent thinking, participate freely in community activities or further agency and enjoy the same privileges like every other citizen. Together we can fight Sickle cell and address its associated stigma.

The writer is a Mandela Washington Fellow living with Sickle cell and working with Uganda Sickle Cell Rescue Foundation.