SICKLE cell disease (SCD) continues to be the most common genetic disorder in Jamaica, with one out of every 150 babies born alive having the condition.

SCD may cause many complications, including some that could be severe and potentially life-threatening. Individuals may experience severe painful events, infections, stroke, and a pneumonia-like illness that can be quite dangerous.

As people get older, they can have damage to their organs, such as kidney disease, leg ulcers, heart failure, and chronic lung disease. The effects of the illness can begin from infancy and the various complications will wax and wane throughout the lifespan of the individual.

Individuals with SCD also face many psychological, emotional and social challenges.

The painful crises which can begin in their childhood may result in frequent hospitalisations and absences from school or work. The unpredictability of severe attacks can lead to fear and anxiety. Individuals experience more fatigue and lower energy levels and hence may not be able to participate in sports and other physically demanding activities.

All of these things can lead to a sense of isolation, loneliness and depression. Many people require strong painkillers, such as opioids, for relief, and are often wrongfully accused of being drug-seeking by families and health care professionals. This is unfortunate, as painful events are very severe and the need for relief is critical.

Often because they have a lot of fear of facing this stigma, patients do not want to seek help. This may lead to severe life-threatening problems being missed and they may present late to hospital for care.

The flip side of the coin is that those individuals who have more psychological and social problems also have more severe complications related to the disease. For example, individuals with SCD who have greater emotional and stress-related problems have more frequent and more severe pain events.

Local studies from the Sickle Cell Unit in Jamaica point to the many psychological and social problems faced by our patients. Adolescents are seen to engage in risky behaviours, such as drinking alcohol and unprotected sexual contacts. They have poor body image and hence may experience depression and occasionally have thoughts of suicide. Their overall quality of life is impaired and even parents may not be fully aware of the emotional impact the disease is having on their children.

Unfortunately, many of these problems are found to be worse in girls than in boys.

Adults living with SCD also state that they have a sense of loss. They feel their life is not the same as others who are living healthy lives. They also describe that they don’t always feel they can control their illness. These feelings, along with the stigma and discrimination they often face at work, health care facilities and in their communities, lead to high levels of depression and loneliness among them.

Individuals who have more severe disease, such as those with more frequent pain events or leg ulcers, as well as those who are unemployed or have poor social support, experience a worse quality of life and higher depression. People with SCD have more difficulty making friends or forming intimate relationships and many times are hesitant to disclose their illness status in an effort to do so.

Whereas the medical fraternity now has a much better understanding of how to manage this disease and its complications, attention to the mental and social health of these individuals is still inadequate. Many times, individuals with SCD are not disclosing these issues till probed.

Patients presenting for routine check-up visits to their health care facilities should be screened and assessed for psychological, emotional and social issues. If such problems are identified, then referral to a psychologist or social worker can be of great benefit.

Families, schools, workplaces, and communities need to be familiar with the problems that children and adults living with SCD may face, especially if they have such individuals present in their lives. They have the opportunity to offer much support and hence assist these individuals in leading much happier and healthier lives.

Families and friends can be a great social support network by providing understanding and quick attention to medical needs when their loved one with SCD has a medical complication. Being included in activities which are safe for them and not being bullied, ostracised or treated as if they are weak helps individuals to feel less lonely and happier.

Schools and workplaces can learn more about the illness as some people with SCD have unique needs. For example, a child with SCD might have to get restroom breaks more frequently as he or she produces a lot more urine, or the child might miss school due to hospitalisations and will need special attention from their teacher to catch up with their school work. Adults may not be able to work in very cold environments or perform very physically exerting jobs, so alternative tasks may need to be assigned to them.

In short, if families and communities continue to educate themselves and seek further information from health care specialists and from the individuals living with SCD, then we can really move forward in improving the quality of life of people living with this devastating illness.

Monika R Asnani, DM (Fam Med) PhD (Epidemiology), of the Caribbean Institute for Health Research — Sickle Cell Unit at The University of the West Indies, Mona. E-mail: monika.parshadasnani@uwimona.edu.jm