Sickle Cell Disease (SCD) is a genetically inherited blood disorder characterized by the presence of sickled red blood cells (caused by a single mutation) that can get stuck in capillaries and cause clots. People with SCD must receive lifelong treatment and are at a heightened risk from clotting and tissue/organ ischemia. Viral infections (including influenza and COVID-19) can lead to complications in people with SCD.
What causes sickle cell disease?
Sickle cell is an inherited disease caused by a defect in a gene.
A person will be born with sickle cell disease only if two genes are inherited—one from the mother and one from the father.
A person who inherits just one gene is healthy and said to be a “carrier” of the disease. A carrier has an increased chance of having a child with sickle cell disease if he or she has a child with another carrier.
For parents who are each carriers of a sickle cell gene, there is a 1 in 4, or a 25 % chance of having a child with sickle cell disease.
How is sickle cell disease diagnosed
Along with a complete medical history and physical exam, you may have blood and other tests.
Many states routinely screen newborns for sickle cell so that treatment can begin as soon as possible. Early diagnosis and treatment can reduce the risk of complications. Haemoglobin electrophoresis is a blood test that can determine if a person is a carrier of sickle cell, or has any of the diseases associated with the sickle cell gene.
COVID-19 and SCD
People with SCD are classes as extremely clinically vulnerable due to being immunocompromised and are therefore strongly advised to be extremely cautious during the pandemic in adhering to the rules regarding shielding, social distancing, frequent handwashing & mask-wearing when out in public to minimize the risk of contracting COVID-19.
This is because the risk of adverse health effects and complications is greater in people with SCD. People with SCD have impaired immunity which can arise from functional hypersplenism and systemic vasculopathy – all increasing the risk of thrombosis (clotting) – which in itself is a common symptom and complication of COVID-19, especially in younger individuals.
Specific case studies have shown different outcomes depending on whether the SCD patient is of advanced age, has pre-existing comorbidities, or any SCD-related complications within 1-3 years or not. In general, people who have had SCD-related complications and crises close to contracting COVID-19 and/or have 1 or more comorbidities are at a far greater risk of developing more severe COVID-19, ICU admission, and in some cases, death. This is very much in line with influenza infection and disease severity, though the evidence for COVID-19 severity is recent and still emerging.
SCD and COVID-19 vaccination
As patients with SCD are classed as clinically vulnerable, patients must be vaccinated at the earliest possible opportunity to minimize the risk of contracting COVID-19, and more importantly, reducing the overall risk of severe disease and death. The two main mRNA vaccines; Pfizer and Moderna, have been shown to be extremely safe and effective in the vast majority of people, including those with SCD.
Recently, the Oxford-AstraZeneca and Johnson & Johnson vaccines have been in the news for their supposed links to increased clotting. However, these events are incredibly rare, and the benefits of vaccination far outweigh the risks associated with vaccination or COVID-19 itself, and therefore even people with SCD are strongly encouraged to get these vaccines.
In summary, patients with SCD who develop COVID-19 are at an increased risk of moderate to severe disease, especially those that are older and those that have pre-existing underlying health conditions.
What causes a higher risk of infection for COVID-19
Recommended that crowded indoor spaces should be avoided. When indoors, increasing the rate of air change, decreasing recirculation of air and increasing the use of outdoor air can reduce transmission. The WHO recommends ventilation and air filtration in public spaces to help clear out infectious aerosols.
What are the symptoms of COVID-19?
Common symptoms of COVID-19 begin two to 14 days after exposure. They include fever, tiredness, and dry cough. Other symptoms include loss of taste or smell, nasal congestion, sore throat, muscle and joint pain, chills, vomiting, nausea, and diarrhea.
Most of these symptoms are usually mild, and about 80% of people who get the virus will typically recover without needing any special treatment.
However, it is estimated that about 15% become seriously ill and develop breathing difficulties, and 5% become critically ill and require intensive care. Symptoms of serious COVID-19 include shortness of breath, loss of appetite, and pain or pressure in the chest.
People over the age of 60 and those with underlying medical conditions such as diabetes, heart and lung issues, cancer, and high blood pressure are most at risk of becoming seriously ill.
Common questions, people asking with the answers
Can COVID-19 enter into the body by the hands?
Hands touch too many surfaces and can quickly pick up viruses. Once contaminated, hands can transfer the virus to your face, from where the virus can move inside your body, making you feel unwell.
Why are older people at significant risk of COVID-19?
Although all age groups are at risk of contracting COVID-19, older people face significant risk of developing severe illness if they contract the disease due to physiological changes that come with ageing and potential underlying health conditions.
Which organs are most affected by COVID-19?
The lungs are the organs most affected by COVID-19 because the virus accesses host cells via the receptor for the enzyme angiotensin-converting enzyme 2 (ACE2), which is most abundant on the surface of type II alveolar cells of the lungs
Can COVID-19 be transmitted through food?
There is currently no evidence that people can catch COVID-19 from food. The virus that causes COVID-19 can be killed at temperatures similar to that of other known viruses and bacteria found in food.
Can the coronavirus disease be transmitted through water?
Drinking water is not transmitting COVID-19. And, if you swim in a swimming pool or in a pond, you cannot get COVID-19 through water. But what can happen, if you go to a swimming pool, which is crowded and if you are close to other people and if someone is infected, then you can of course be affected.
What are some potential long-term effects of COVID-19?
Some early studies suggest that ten to twenty percent of people with COVID-19 will experience symptoms lasting longer than a month. A majority of those who were admitted to hospital with severe disease report long-term problems, including fatigue and shortness of breath.
What are the complications of COVID-19?
Septic shock, and death. Cardiovascular complications may include heart failure, arrhythmias (including atrial fibrillation), heart inflammation, and thrombosis, particularly venous thromboembolism. Approximately 20–30% of people who present with COVID-19 have elevated liver enzymes, reflecting liver injury.
What are the effects of the COVID-19 pandemic on mental health?
Bereavement, isolation, loss of income and fear are triggering mental health conditions or exacerbating existing ones. Many people may be facing increased levels of alcohol and drug use, insomnia, and anxiety.
Meanwhile, COVID-19 itself can lead to neurological and mental complications, such as delirium, agitation, and stroke. People with pre-existing mental, neurological or substance use disorders are also more vulnerable to SARS-CoV-2 infection ? they may stand a higher risk of severe outcomes and even death.
What should sick individuals do?
If symptoms are present or a COVID-19 diagnosis is confirmed, the following steps should be taken to prevent the spread of infection:
Stay at home, preferably in a separate room not shared with others, and isolate yourself, with the exception of getting medical care.
Avoid public areas and public transport.
Wear a mask if you have to be around people.
Limit contact with pets and animals.
Avoid sharing personal items.
Cover coughs and sneezes with tissues and dispose of them properly.
Sanitize hands regularly.
Disinfect surfaces such as phones, keyboards, toilets, and tables.
Advice for family members and caregivers
o Family members and caregivers of people with sickle cell disease should take appropriate precautions and extra care to avoid bringing COVID-19 home. They also should constantly monitor patients for any potential symptoms of the virus.
o They should wear gloves when coming in contact with blood or body fluids of patients with a COVID-19 infection and thoroughly wash clothes and highly touched surfaces such as counters, doorknobs, bathroom fixtures, and toilets.
o If family members and caregivers show symptoms of COVID-19 themselves, they should avoid coming in contact with the patient until the self-isolation period is complete.