Since 2012 every March 21 is celebrated World Down Syndrome Day, a vindicative date whose main objective is to encourage the development of educational strategies and social policies aimed at raising public awareness of Down syndrome.
As a consequence of the health crisis caused by COVID-19, in the last year several research groups have been interested in learning about the effects of the virus on people with Down syndrome.
Several studies have consistently concluded that age is a key risk factor that increases mortality from COVID-19. People with Down syndrome (DS) age prematurely with a life expectancy of around 60 years.
Thus, it is presumed that an age of 40 years for a patient with DS could be equivalent to 60 years for a person without DS, as pointed out by Dr. Viviana Arreo del Val, Child Cardiologist at the Congenital Heart Disease Unit and the Hospital Universitario La Paz.
The report published by the Trisomy 21 Research Society points out that children with DS without previous pathologies do not seem to present an increased risk of mortality, a fact that Dr. Arreo del Val welcomes given the low incidence of mortality that has been demonstrated.
The effects of COVID-19 in Down syndrome patients
Several experts have indicated on numerous occasions that high-risk factors, such as age, male sex, obesity, diabetes mellitus and arterial hypertension, affect patients with DS similarly to the rest of the population. However, Dr. Viviana Arreo del Val clarifies that patients with DS have a higher prevalence of these comorbidities (obesity, diabetes and congenital heart problems), so the risk of having a serious disease seems higher.
The figures for hospitalized patients indicate that patients with DS also present greater pulmonary complications, to which the doctor replies that this could be explained by the fact that these patients usually present greater hypotonia of the muscles responsible for facilitating breathing, in addition to the fact that they frequently suffer from gastroesophageal reflux that favors respiratory infections (pneumonias), present craniofacial alterations that make intubation difficult, and have an immune system with a greater predisposition to generate uncontrolled pulmonary inflammation.
Not only does this happen with COVID-19, but an increased risk of pulmonary complications has also been seen when infected with other viruses such as influenza.
The Scientific Society for Down Syndrome Research noted that the main causes of morbidity from COVID-19 in people with Down syndrome were hemorrhage and multiorgan dysfunction.
The doctor adds that in COVID-19 infection it has been seen that there is a first phase in which the symptoms depend on the viral infection and a second, more serious phase, in which complications appear due to an exaggerated activation of the immune system that tries to defeat the virus and which we call cytokine storm. This “super activation” of the immune system can affect any organ. According to some published studies, people with Down syndrome have a more uncontrolled immune system and this could justify this multi-organ dysfunction as the main cause of death in these patients.
Why do people with DS have more difficulties in coping with COVID-19?
A study published in October 2020 in the Annals of Internal Medicine, conducted in a cohort of 8 million adults, concluded that people with DS were 4 times more likely to be hospitalized for COVID-19 infection than the general population and 10 times more likely to die.
She believes that in part, this may be explained by the different genetics that people with DS have, as it influences how their immune system is regulated. Another study published by authors Ilario De Toma and Mara Dierssen, suggests that trisomy 21, specifically the triplication of a gene called TMPRSS, facilitates the entry of the virus into cells (which would explain the greater ease of virus infection).
They also found other altered genes in people with DS that are responsible for generating an exaggerated immune response and triggering the cytokine storm that is largely responsible for the development of complications including death.
Changes and adaptation to the new healthcare scenario
Dr. Viviana Arreo’s experience as a pediatrician has allowed her to see how her pediatric patients with DS have adapted perfectly to the recommendations of the new normal. And not only them, but also the rest of pediatric patients and children in our society.
For her, they are an example to follow, especially for those irresponsible people who, at this point and with the information they have, are still breaking the rules and making it difficult for us to finally control this pandemic.