Photographer campaigns to raise awareness about disease dubbed a silent killer

Growing up Lea Kilenga knew that she was different from other children. Sickle cell disease was not new to her family as her two elder sisters had been diagnosed with it.

“The doctor knew about the condition with my sisters and automatically requested a haemoglobin electrophoresis test when I was three months and that is how I was diagnosed,” she says.

Her childhood was characterised with a lot of hospitalisation and daily medication. She also witnessed the pain her older sisters went through during a crisis.

“For a long time I thought that being hospitalised and being on lots of medication was the norm for many children until my younger brother was born and I realised that he did not need so much medical attention and the differences started becoming clearer,” she recalls.

Sickle cell disease (SCD) is an inherited condition affecting the blood and various organs in the body. It affects the red blood cells, causing episodes of sickling, which produce episodes of pain and other symptoms.

Sickle cell disease prevents oxygen from reaching the spleen, liver, kidneys, lungs, heart, or other organs, causing a lot of damage.

When sickle-shaped red blood cells get stuck in blood vessels, patients can have episodes of pain called crises. Other symptoms include delayed growth, strokes, and jaundice.

Crises occur when it is cold, too hot or when a person is dehydrated or their immunity is low.

The full realisation of how different she was came when Ms Kilenga was in Standard Six and her academic performance started declining sharply.

“I still had not accepted myself. I was ashamed to be different and that people noticed that I was not like any other child. My parents, however, were very supportive and I was able to complete KCPE,” she says.

While most parents whose children have been diagnosed with sickle cell prefer to keep their children as day scholars so as to keep a close eye on their health, Ms Kilenga is thankful that her parents took her to a boarding school as this helped her learn what identified her as an individual other than the ailment.

“My school was very supportive and gave me the flexibility I needed to manage the condition. I immersed myself into performing arts and drama, which helped me regain my confidence and self-esteem,” she adds.

It is with this in mind that 25-year-old Ms Kilenga has embarked on the ambitious 10003 Warrior Project, a photographic narrative that she and her team hopes will enable them capture 10, 003 raw real-time portraits and stories of sickle cell warriors around the country.

The 18-months long project is also a platform that encourages a healthier lifestyle for sickle cell disease management as well as raises awareness on a disease Ms Kilenga refers to as long forgotten in the country.

“Very few people know about sickle cell. There are a lot of misconceptions and poor care is given to the vulnerable group, mainly because there is no data on the number of people affected. Even many insurance companies fail to provide medical cover to such patients,” she says.

Every month, she and her team of three hold a photo shoot for people with sickle cell at social enterprise Pawa254, and four weeks into the project she has taken photos and stories of 25 people.

At the moment their greatest challenge is lack of funding to not only create awareness on the disease but also travel across the country to areas where the disease is considered endemic.

“The other challenge we are facing is getting warriors to participate in the project because we wholly rely on their willingness to come and speak out. Convincing them that this will eventually create policy changes with regards to how the disease is dealt with in the country is a bit hard,” she confesses.

In her interactions, she admits that first time parents of children with sickle cell disease are most afflicted by the lack of information on management of the disease.

“The initial acceptance of the child’s condition is normally the hardest and some parents end up becoming overly protective in an unhealthy way, feeling that their children cannot lead normal lives. It is also a costly disease to maintain and the frequent health complications and hospitalisations can be financially draining,” she says.

At the moment the youngest child she has photographed is 15 years old and while some of the warriors have gone on to live to more than 50 years, she believes that bringing out stories of younger patients will help in changing the attitudes of parents towards the disease, which the children are more likely to pick up on ensuring they survive and achieve their potential.

According to Prof William Macharia, a doctor specialising in paediatric haemato-oncology (blood and cancers in children) at the Aga Khan University Hospital, the distribution of the gene that leads inheritance of the disease is mainly found in western Kenya, Nyanza and at the Coast.

“Actual number of people within the population carrying the disease is not estimated because no population base work has been done to determine the kind of blood individuals have, but in countries like Jamaica where the population is screened at birth to even determine their haemoglobin type it is easier to come up with a prevalence rate for a particular geographical location,” he notes.

Prof Macharia says in instances where both parents are carriers of the abnormal gene, with every birth there is a 25 per cent chance that the child could be a sickler (have sickle cell disease), 50 per cent chance that the child could be a carrier of the gene without it manifesting into disease and a 25 per cent chance that they have the completely normal AA haemoglobin type.

“If you have four children all of them could be healthy, all or some of them could be carriers and in some unfortunate cases all of them could be sicklers,” he explains.

He is quick to note that while the prevalence of the disease at the Coast and in western Kenya is enough to warrant neonatal screening, the procedure is costly and most infants get sick and die quickly without being diagnosed.

“Without the neonatal screening programme at birth most health practitioners, especially in remote parts of the country, will rely on the clinical signs manifesting in the child,” he says.

Some of these symptoms include low blood levels during the first few months of life, a lethargic child, poor feeding and complications like the heart beating very fast, breathing problems or swollen tummy from an enlarged liver.

Symptoms like the hand and foot syndrome that develops in some sicklers aged between four and six months are also clear to pick up on. Often the limbs of these children swell painfully without a clear indication of what the problem and the children have pale eyes.

Another indication that could be a manifestation of the disease and which parents should be alert to seek further medical attention on is when a child keeps having frequent infections, especially accompanied by anaemia.

“Most parents do not know what is causing the problem in their children until a medic suspects a diagnosis and a haematologists identifies the SS haemoglobin in a blood test. Such interpretations are unlikely to happen in hospitals below the district level,” says Prof Macharia.

Unlike in healthy individuals where red blood cells live for between 100 -120 days, cells affected by sickle cell survive for only 40-45 days increasing the risk of blood levels going down. It is for this reason that a daily dose of folic acid supplements is prescribed to help in replenishing the cells.

Often the sickled cells are sticky and can cause blockage within arteries blocking the supply of blood to the tissues and causing painful crises. Patients are encouraged to increase their daily fluid intake to make the blood less viscous, especially on hot days when there is a possibility of losing water through sweat, vomiting or diarrhoea.

Many infections

“The spleen can also get compromised over time and the weakening can prevent it from containing the haemophilus and streptococcus bacteria, but in children under two years we put them on daily penicillin in addition to the pneumonia vaccine and the haemophilus influenza vaccine. After the age of two we administer another vaccine that covers more subsets of the bacteria and which is repeated every 3-5 years,” he says.

On a cold day, blood vessels constrict increasing the risk of blockage and sicklers are advised to avoid places of extreme low temperatures and those with low oxygen concentrations as this may lead to painful crises.

Prof Macharia advises parents to monitor if the child is taking enough fluids from observing the colour of urine, which tends to be clear if they are taking enough fluids and brownish when they are not.

The disease, he says, can be managed if children take their medication, eat healthy balanced diets and healthy snacks as they expend more energy than their healthier counterparts.

He also urges parents to be vigilant monitoring their children’s tummy on the size of the liver and spleen to see if their are enlarged.

“Bleeding into your organs is one of the most fatal crises that can happen as blood gets trapped into the liver or the spleen causing them to enlarge making the belly bigger than usual and which can also be painful. When this occurs the patient can easily go into shock and there is high probability of brain damage if there no blood supply,” he explains.

With proper care the doctor says it is possible for children to grow and lead a healthy life where the number of crises reduce to once every two years.