"What do I need to do (or know) as a person with Sickle cell disease?" The enquirer is health care student. In response I came across nice piece written by Nitin et al published in the Pan African Medical Journal (Vol 36, May -Aug 2020). The World Health Organization (WHO) has identified sickle cell disease (SCD) as a major concern of public health significance. It has been estimated that around 5% of the global population carry Sickle Cell Trait genes. About two-thirds of the sickle cell disease patients of the global burden reside in sub-Saharan Africa. COVID-19 Pandemic caused by Corona virus 2 (SARS COV2) is having a devastating effect on socioeconomic and health indicators in counties worldwide. The additional financial burden of supporting health care management system in tackling COVID-19 impact at the same time preventing mortality rate of COVID-19 deaths is a matter of great concern to all.
The pathogenesis of the sickle cell disease is attributed to the polymerization of the deoxygenated haemoglobin S(HbS). The polymerization leads to alteration in the normal biconcave shape of the red blood cells making them rigid and more prone for intravascular haemolysis. As a consequence of repeated hypoxia driven polymerization of HbS there is development of cyclic cascade leading to blood cell adhesion, vaso-occlusive crisis and ischaemic reperfusion injury. SCD patients may develop complications such as Acute Chest Syndrome, pulmonary embolism and stroke
About two thirds of new borns born with SCD worldwide are found in sub-Saharan Africa. The sickle cell gene HbSS is commonly identified in Africa in SCD while HbSC and HbS/ beta+thalassemia has been observed in West Africa. SCD had led to the death of about 50-90% of the affected as the disease remained undiagnosed during the childhood. The various studies done in Africa were found that SCD patients have higher mortality rates. In Ghana-the programme to enhance health care for sickle cell disease is a big relief.
The development of knowledge of understanding the pathology and management protocol of SCD has been helpful in management of the disease. The presence of malaria, undernutrition and other infectious diseases also contribute towards mortality rate in Africa. Of late it has been seen that because of the devoted and dedicated health care services provided by the health personnel the mortality rates are declining and this life-threatening disease of children is now progressing to chronic disease of the adult.
It has been observed that pulmonary functions are decreased in SCD. Lung functions are compromised in patients of sickle cell disease and Sickle Cell Trait (SCT). Repeated chest infections in SCD and SCT lead to alteration in geometry of lung parenchyma and physical properties of elastic and collagen fibres thus decreasing pulmonary function parameters such as Forced Vital Capacity, Forced Expiratory Volume and Forced Expiratory Volume 1%. Moreover the pulmonary vasculature is highly sensitive to hypoxia (absence of enough oxygen) driven micro-occlusion of pulmonary vasculature which along with cell adhesive changes may cause pulmonary hypertension and further compromise lung functions]. Persons with SCD have an increased susceptibility to infection. The impaired leucocyte function and humoral and cell-mediated immunity loss have been reported to account for the immunocompromised state in patients with sickle cell disease. The SCD patients being immune compromised are more prone for recurrent chest infections. The major cause of mortality in patients of SCD is acute chest syndrome, pneumonia and acute respiratory distress syndrome.
COVID-19 is the acronym for corona virus disease 19 and has been termed as SARS-COV-2 by International Committee of Taxonomy on Virus (ICTV). The common clinical manifestations observed in patients of sickle cell disease include cough, fever, shortness of breath, loss of smell perception and loss of taste sensation. Most of the patients of COVID-19 may have a mild course of disease while few may develop severe clinical manifestations. The clinical manifestation of severity in COVID-19 patient includes Acute Respiratory Distress Syndrome (ARDS), Pneumonia, Multiple Organ Failure, Septic Shock and Sepsis. The severity of pneumonia manifests with dyspnoea (difficulty or laboured breathing) and tachypnoea (abnormally rapid breathing)
COVID-19 infection can worsen the pulmonary manifestation in SCD patients especially in those having pulmonary complications such as Acute Chest Syndrome, Pulmonary Hypertension and ARDS. COVID-19 infections in SCD can also increase morbidity and mortality risk in these patients.
The main cause of concern in patients of SCD is that these patients are immunocompromised and may suffer from both acute and chronic complications which require hospitalization and close contact with the medical system. There is overlap in clinical manifestations of fever and lung disease in COVID-19 and SCD. The increased complications will amplify health care utilization-e diagnostic, management and logistic challenges. In view of the above facts it is necessary for health care workers to educate SCD patient registered in their areas regarding care and precautions to be taken during COVID-19 pandemic to prevent getting affected with COVID-19 infection. Although the education applies to everyone, there should be more emphasis for persons with SCD.
All persons with SCD need to be educated regarding COVID-19 signs, symptoms and mode of spread. They should be explained regarding the increased risk of contracting COVID-19 infections in them due to their immunocompromised state. All patients of SCD should be advised to strictly adhere to social distancing, isolation polices, use of face mask, and frequent hand washing with soap to prevent COVID-19 infections. They should keep adequate medication of SCD such as analgesic and antipyretic drugs, hydroxyurea. They can be advised regarding use of clinical thermometer at home as fever is common sign in SCD patient and thereby these persons can take appropriate precautions and medication after seeking telephonic consultation with their health care providers. They can use pharmacy home delivery services in case they require medication during emergency situations.
Until then regularly/daily consume polyphenol-rich cocoa. It is been useful for persons with SCD.
DR. EDWARD O. AMPORFUL