Sickle cell and cognitive decline in children

Sickle cell and cognitive decline in children

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While reading about sickle cell in children, I came across a long and interesting article which I decided to highlight some noteworthy parts from the article for us to learn more.

Many studies have been done over the years regarding the cognitive impairment that children living with sickle-cell disease go through.  Sickle cell is a multi-faceted disease associated with episodes of acute illness and progressive heart damage.  The sickled cells can block blood and other nutrients from travelling through veins and arteries causing pain and loss of blood to the brain, organs and extremities.

Pain is a common consequence of sickle cell disease. Pain in the form of vaso-occlusive episodes can begin as early as six months of age and can be both frequent and severe. Pain can be associated with an increased impairment in daily activities such as school and play as well as potentially impairing adjustment in physical, emotional, recreational, educational, and vocational activities in daily functioning.

Children who suffer from this condition have a lot of health related as well as social and emotional complications associated with this disease.   Lack of adequate oxygen and blood supply to the brain as well as brain infarcts (strokes) can contribute to the prevalence of cognitive impairment and decreased health-related quality of life seen within children who have sickle cell disease.

Recurrent episodes of vaso-occlusion (pain) and inflammation result in damage to most organs, including the brain, kidneys, lungs, bones, and cardiovascular system, which become apparent with increasing age.   This is viewed as a serious deficit in children because it not only leads to many health-related problems, such as irreversible damage to organs as well as ischemic (restriction of blood supply and shortage of oxygen and glucose to cellular metabolism) injuries of the limbs, bowel, cutaneous tissues and even the heart and brain which can result in death.

One report addressed the possibility of developmental delay in preschool children with sickle cell disease.   In another study, further evidence of cognitive impairment in children with sickle cell disease can be seen in an extensive study of 373 patients with varying classifications of the disease (255 with haemoglobin SS and 118 with haemoglobin SC). The participants were given a series of neuropsychological tests such as Wechsler Intelligence Scale for Children (WISC-R or WISC-III) and the Woodcock-Johnson Math and Reading Achievement Tests, Full-scale IQ (FSIQ) for complete cognitive capacity and an MRI. The researchers found that “children and adolescents with SCD between 6 and 18 years of age have a significant deficit in neuropsychometric performance associated with silent infarction and a significant decline in performance with age in certain areas.”

This shows that family socioeconomic factors are significant predictors of functional outcomes demonstrating that family income can cause social stress on the child contributing to lower physical activity and depressive nature. At the neighbourhood and community level one can see how the family and neighbourhood socioeconomic distress was predictive of physical activity and healthy emotional development.

Management of the quality of life for children with SCD can be difficult to deal with and can create heightened anxiety and reduced confidence.   There are many physical complications caused by sickle cell disease. However, acute chest syndrome is the most prevalent and perhaps the most uncomfortable for those with SCD.  Acute chest syndrome (ACS) is an important cause of morbidity and mortality in sickle cell disease.   Acute chest syndrome is the second most common cause of hospitalisation in patients with sickle cell disease and is responsible for up to 25 per cent of deaths.

The frequency of presenting symptoms was age-dependent with fever and cough being more common in young children (age 2 to 4 years) and the incidence of chest pain, shortness of breath, chills, productive cough, and hemoptysis increasing with age. This is significant because even though young children do not initially experience pain, as they grow older they will experience all the symptoms that adults do. Therefore, since there is no cure for sickle cell disease and the complications that come with the disease, it is important to intervene to make the quality of life for children with SCD better by not having to experience pain associated with acute chest syndrome.

Early intervention is important and should include, “Antibiotics (pneumococcal and atypical pneumonia cover), careful IV fluid maintenance, to prevent dehydration and avoid overhydration, effective and safe analgesia, bronchodilators and transfusion.” If ACS can be seen early and early interventions can be made, it is possible that children with SCD and ACS may not experience pain.

Progress has recently been made in the development of drugs that inhibit K+ (potassium) and water loss from SS red cells and thus cause a reduction in the intracellular haemoglobin concentration, which results in sickled cells.  Sickle cell disease is a condition that causes many health-related problems such as irreversible damage to organs, acute pain and ischemia of the extremities. Through careful research, it is concluded that children with sickle cell do suffer from cognitive impairment from; (1) recurrent micro infarction of the central nervous system; (2) chronic hypoxic damage to the brain or diminished pulmonary function; (3) sub-acute brain damage that occurred during bouts of hypoxia associated with events such as aplastic crisis, acute chest syndrome, and obstructive sleep apnoea; and (4) chronic nutritional deficiency associated with increased metabolic demands. These recurrent instances directly result in compromised neurocognitive function.

In conclusion, the quality of life that children living with sickle cell disease have is directly affected because of illness from the disease. Children that come from low socioeconomic communities and neighbourhoods don’t have the adequate resources to play and engage in physical activity. Therefore, they are impacted physically, emotionally and socially. Moreover, children with sickle cell disease as well as their parents have self-reported lower health related quality of life in comparison to healthy children from extensive tests, illness, missed days of school/work and lack of physical activity.


If you would like to get in touch with me about this article or about sickle cell, do so, via email: [email protected]  And do checkout my blog:  My book on sickle cell – How To Live With Sickle Cell and my other books are available for purchase on

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