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SICKLE CELL DISEASE AND COVID-19

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“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. 

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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.

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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

CHIEF PHARMACIST

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COCOA CLINIC 

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International character of Ghanaman and more

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Ghanaians can be found in every part of the world

The average modern Gold Coaster born and bred in Sikaman is an adventurous creature. Go to any part of the world and you’ll find Ghanaman diligently slugging it out either in the snow or in hot sweltering weather, earning a decent living.

Go to Guinea and see Ghanaman in the garb of a herbalist, curing the sick of various ailments. He makes the Guinean understand that not all diseases are caused by James Lucifer alias Jimmy Satan. On the contrary, most diseases are caused by kooko which can even attack the human nose and turn it upside down.

That kooko is also the cause of poverty and the internal bleeding of personal back-pocket economies. And that kooko can be cured using the chemistry underlying Sikaman herbal medicine which has been blessed by the gods of Larteh.

And before Ghanaman starts dispensing his herbal concoctions, he must attract the passing crowd with some Sıkaman-originated choreography, akin to a combination of adowa and agbadza, such that the dancer can easily dislocate a shoulder and start crying for his mother.

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When the crowd is enough to form a quorum, Ghanaman must perform magic as a side-attraction, and proceed to sell his drugs. The drugs are genuinely made from potent herbs which the Pharmacy Board of Sikaman says it has no confidence in. Believe me, Ghanaian herbalists are practising in neighbouring countries like Guinea, Cote d’Ivoire, Sierra Leone and Benin, while we are discouraging them from practising in Sikaman.

Go to Las Palmas and see Ghanaman as a fisherman. He sails on Korean-owned boats and is forced to eat garlic and raw tuna for radiant health. The garlic is also supposed to protect him against witches. But sometimes, he is not good bedfellows with some Korean crew members, and once in a while a fight breaks out.

The typical Korean is a Kung Fu expert and before displaying a golden dragon data. He must first terrify Ghanaman with a Korean dance which consists of wild arms and leg throwing. But Ghanaman is usually not daunted. Why has he been eating cancer kenkey all years without developing cancer? It mean is a superhuman capable of countering Kun fu side-kicks to the ribs.

Anyhow Ghanaman’s most potent arsenal lies in his fists. If he is a southpaw, it mean left fist is probably insured and can cause damage. When Ghanaman lands a cassava blow and it lands on a Korean chest, it has the effect of a cruise missile. If there is no ceasefire a funeral cannot be ruled out.

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Greenland

Go to Alaska and find Ghanaman there. Greenland, Iceland, Brazil, Argentina, Madagascar, everywhere and you’ll find him. He has become an international citizen and is generally lauded for his hardwork, sincerity and honesty.

However, some bad nuts are also condemned and despised.

The world and all its parts and corners are for the human race, and anyone can choose to live anywhere and earn a decent living so long as he doesn’t misconduct himself or become a social misfit and a law unto himself.

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Sıkaman for instance accepts foreigners, and we are so liberal that people just come in through our borders and we welcome them with the best palmwine in town and goat light soup. Who cares about passports and visas? Our hospitality is, as it were legendary.

So we have Liberians, Nigerians, Sierra Leoneans, Togolese, Europeans, Americans, Scandinavians and Koreans in Sikaman.  And some of them have misinterpreted our overzealous accommodation to mean “foolishness” and are misconducting themselves.

The newspapers carry stories of some unscrupulous Nigerians stealing, defrauding and collaborating with their Sikaman counterparts to rob people. You’ll find some good and law-abiding Liberians around but some other Liberians are in the business of fraud, robbery, money printing, money doubling, anything criminal.

Ghanaian hospitality can no longer be extended to such people. I do not think Ghanaians will be treated kindly either, when they misbehave anywhere in the world. Those who come from outside with the intention of bringing down their evil deeds and to practise them here professionally must be getting ready because Ghanaians are getting fed up with the menace.

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These days, you’ll find strange people following you as soon as you emerge from a bank. If you are not careful, they’ll wrestle your briefcase from you and speed away in a waiting taxi.

Someone was just saying that many foreign criminals are flocking to Ghana because in their countries, they are enable to operate either because they are being looked for by the police for offences committed or fear instant justice at the hands of mobs. Ghanaians, it is said, are humanitarian in dealing with criminals.

Well, in some African countries, a thief who is being chased runs directly to the police station in his own interest and begs the officer at the counter to lock him up. Otherwise he’ll be lynched or be burnt to death. In Ghana, it is the opposite. The thieves run far away from the cop stations.

The criminals must reform and stay peacefully in Sikaman or quit. No court case!

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This article was first published on Saturday September 5, 1998

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The architecture of existence: How we live and die

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​The human condition is defined by two absolute, biological bookends: the commencement of complex cellular animation and its ultimate, irreversible cessation.

For centuries, medicine treated life and death as strictly separate states. Today, modern pathology and molecular biology reveal a more profound truth: the mechanisms that allow us to live are the exact same mechanisms that dictate how we die.

​Understanding this relationship is no longer just a philosophical pursuit. It is a clinical necessity for managing public health in the 21st century.

Part I: How we live – The mechanics of animation

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​At its core, biological life is a continuous, high-stakes battle against entropy (the natural tendency of systems to degrade into disorder). To maintain form, consciousness, and function, the human body relies on three fundamental pillars:

1. Cellular respiration and energy production

​Every second of human life is powered by the conversion of oxygen and glucose into Adenosine Triphosphate (ATP), the universal energy currency of our cells. This process occurs within the mitochondria.

Without a constant supply of ATP, the microscopic pumps that maintain electrical gradients across our cell membranes fail, causing cellular collapse within minutes.

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2. Homeostasis: The balancing act

​To survive, the internal environment of the body must remain incredibly stable, regardless of external chaos. This balancing act, known as homeostasis, regulates:

​Blood pH: Strictly maintained between 7.35 and 7.45.

​Core Temperature: Ideally kept around 37°C (98.6°F).

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​Electrolyte Balance: Precise ratios of sodium, potassium, and calcium that allow nerves to fire and muscles to contract.

​3. The Autonomic Nervous System (ANS)

​The ANS acts as the invisible conductor of life, split into two complementary branches:

Part II: How we die – The path of cessation

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​Death is rarely a singular event; it is a progressive biological cascade. Whether caused by old age, chronic illness, or sudden trauma, the final pathways of mortality generally follow a predictable trajectory.

​1. The cellular horizon: Senescence and Apoptosis

​Every time a human cell divides, the protective caps at the ends of its chromosomes-telomeres-shorten. Eventually, telomeres become so depleted that the cell can no longer divide safely. It enters senescence (a state of permanent arrest) or triggers apoptosis (programmed cell death). This gradual, systemic loss of cellular renewal is the underlying engine of aging.

​2. The clinical cascade of somatic death

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​When the body can no longer maintain homeostasis, somatic death (the death of the organism as a whole) begins. This occurs in a distinct sequence:

1

Clinical Death

Zero to four minutes

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The heart stops pumping blood, and respiration ceases. While breathing and circulation have ended, the body’s tissues still contain residual oxygen and cellular energy reserves. At this stage, resuscitation is often still possible.

2

Brain Death (Infarction)

Four to six minutes

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Deprived of fresh oxygenated blood, the brain’s highly sensitive neurons begin to starve. Lacking ATP, the brain cells lose their electrical charge, swell, and begin to burst. Permanent, irreversible neurological damage occurs within minutes.

3

Biological Death

Hours to days

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The organs fail entirely. Without central control or blood flow, individual cells throughout the body consume their final energy reserves. Enzymes that once aided digestion begin to break down the body’s own tissues from the inside out, a process known as autolysis.

Part III: The modern collision of life and death

​In the modern era, the boundary between how we live and how we die has blurred. Historically, humanity died primarily from infectious diseases and acute trauma. Today, global mortality is dominated by non-communicable, chronic lifestyle diseases.

Modern Pathology: How we live (the cause) how we die (the result)

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Cardiovascular disease, chronic stress, poor diet, and lack of sleep keep the sympathetic nervous system hyper-activated, damaging blood vessels .Occlusion of coronary arteries leads to myocardial infarction (heart attack) or ischemic stroke.

Metabolic syndrome over nutrition and physical inactivity overwhelm cellular mitochondria, leading to systemic insulin resistance. Multi-organ failure, chronic kidney disease, and severe vascular complications.

Neurodegenerative Disorders Chronic systemic inflammation and sleep deprivation prevent the brain from clearing metabolic waste. Gradual loss of synaptic connections, leading to dementia and the eventual loss of basic autonomic functions.

Conclusion: The unified theory of health

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​Ultimately, how we live directly shapes the path of how we die. The micro-choices of daily existence-how we manage emotional stress, the quality of our rest, our communal connections, and our nutrition-either preserve the integrity of our homeostatic systems or accelerate their decline.

​True medical advancement is shifting away from merely extending the final, frail stages of biological death. Instead, the focus is turning toward optimising our living years-ensuring that the intricate, beautiful machinery of human animation runs smoothly until its natural, quiet conclusion.

By Robert Ekow Grimmond-Thompson

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