Sickle Cell Disease is a genetic blood disorder in which the normal, circular red blood cells present as abnormal 'sickle' shaped cells. This reduces the amount of oxygen that the blood carries to all parts of the body. The sickle-shaped red cells may also block the flow of blood in tiny capillaries thereby depriving cells of both oxygen and nutrients. The numerous complications include: anaemia, excruciating pain, fever, infections, strokes, injury to the lungs, kidneys, eyes and other organs, severe fatigue and retarded growth, among others. There is no cure for Sickle Cell Disease. It is often considered a 'Black people's disease' but Hispanic, Mediterranean (Turkey, Greece, Italy), Arab and South Asian people are also affected.
Of all the symptoms, pain may be the most challenging. Pain can occur in the abdomen, lower back, knees, joints and extremities – several times per month and lasting for 4-6 days, each time. This recurrent pain results in frequent contact with health professionals, repeated absence from school/work, and often leads to depression, low self-esteem, anxiety, negative thinking, social isolation and poor quality of life. The onset of pain is unpredictable. It is associated with infection, dehydration, physical exertion, exposure to cold weather and stress. Pain is usually treated with rest, fluid consumption and medication, but medications have side effects and do not always work. Hence, alternative coping strategies, such as stress management, should be considered.
A painful crisis can be triggered by an immediate stressful event or by an event that occurred a couple of days before. Pain due to an immediate stressful event can last for two days. Pain that was not associated with a stressful event can be exacerbated by subsequent stress. Thus it is important to understand how the body responds to stress and to learn positive ways of managing it.
The stress response begins in our brain – what and how we think! The good news is that, we can change the way we think – the way we respond to stress. We can change the way we think about ourselves, the world and others by using cognitive-behavioural techniques (CBTs). Then, when dealing with situations, or people, that we cannot change, cognitive-behavioural techniques will enable us to respond in healthier, more positive, ways.
CBTs can transform negative-thinking pessimists into positive optimists and this, interestingly, can alleviate the perception of pain. Negative thinking is associated with depression and anxiety. Depression, anxiety and anger are associated with more severe pain requiring hospital visits, contact with health professionals and medication. Patients with frequent painful episodes, requiring frequent health-care use, generally have the highest mortality rates. Negative thinking can kill!
Optimism and positive emotions (happiness, pleasure, joy), enable patients to maintain their activity, even when they are experiencing pain – and this lasts for several days. Positive mood is associated with fewer hospital visits and contact with health professionals, less medication and fewer absences from work/school. Thus patients must be taught positive stress management techniques from an early age. Furthermore, friends and family should encourage them to remain positive during painful episodes as this will relieve anxiety, alleviate depression and improve their mood.
Cognitive-Behavioural Techniques include building self-esteem, assertiveness training and effective communication. Low self-esteem is associated with high rates of depression and anxiety, while high self-esteem enhances one's ability to cope with stress in positive ways. Assertiveness and effective communication will help patients to navigate the barriers that they often encounter – especially barriers due to racism and ignorance.
Additional techniques that enhance coping skills include belly-breathing, guided imagery, calming self-talk, relaxation, yoga, distraction, music, art therapy, scheduling pleasant activities and reinterpreting pain sensations. Progressive muscle relaxation shows much promise. When used in combination with abdominal breathing and guided imagery, patients report significant, or complete, relief from pain. These techniques can be learnt in a short time but require daily practice to be effective.
Patients with Sickle Cell Disease who use cognitive and behavioural coping strategies experience lower levels of pain, fewer hospitalisations and emergency room visits, less need to contact health-care professionals and fewer absences from work/school. To enhance efficacy, CBTs require collaboration among the major 'players' in the patient's network. These techniques can be modified to suit different illnesses, gender, culture and ethnicity.
P.S. I recall my wise Mother saying, "If you can't change a situation, ask God to change you so that you can better cope with the situation, then it won't kill you or make you sick". Since it is said that 'God helps those who help themselves', without knowing it, my Mother was promoting Cognitive-Behavioural Techniques.