Coronavirus: sixteen times more lethal for kids with intellectual disabilities

Four studies about the effects of the novel coronavirus COVID-19 on individuals with intellectual and developmental disabilities all conclude the same thing: its effects are more severe and lethal for these patients.

16X more lethal

The first study sought to determine the effects of COVID-19 on patients with intellectual and developmental disabilities (IDD) as compared to patients without IDD. The researchers reviewed data from a research network, identifying 29,808 patients without IDD and 474 patients with IDD, 5% of which had Down syndrome.

The comparison found that both groups had the highest percentage of cases at ages 18-74. But, when it came to children, those with IDD made up over 25% of all cases as compared to just 8% of children without IDD. Worse, while the overall case-fatality was almost the same for patients with IDD (5.4%) and without IDD (5.4%), for children, the case-fatality rate was 1.6% among those with IDD as compared to less than 0.1% of children without IDD.

Both of those percentages seem relatively low, but that’s the challenge of percentages. Appreciate that if your child with IDD contracts COVID-19, he or she has a greater than 1-in-100 chance of dying; for kids without IDD, their chance of dying from COVID-19 is less than 1-in-1000.

Graphically, these percentages are depicted at the top of this post to show the wide difference between a 1.6% death rate and a 0.1% death rate. What this means is that children with IDD have a SIXTEEN TIMES greater chance of death from COVID-19 than all other children.

And, while lower, the study found that for those aged 18-74 with IDD had a case-fatality rate that was 1.6 times higher than for those of that same age group without IDD.

Higher death rates for IDD & Autism

One of the authors of the previous study, Scott Landes, an associate professor at Syracuse University, was quoted in an NPR report on the impact coronavirus is having on individuals with IDD in New York and Pennsylvania.

Specifically, for those who live in group homes, Landes says they are four times more likely to contract the coronavirus and almost twice as likely to die as compared to the general population.

The bar graphs in the graphic in this section are from public records on all individuals who receive care through the respective state’s department for developmental disabilities. In actual numbers, as of June 2, Pennsylvania had 801 confirmed cases and 113 deaths among people with IDD. In New York, 2,289 tested positive for COVID-19 and 368 have died.

Down syndrome genetics may contribute to severity and lethality

Joaquin Espinosa, of the Linda Crnic Institute for Down Syndrome & Department of Pharmacology at the Unversity of Colorado, wrote a perspective entitled: “Down Syndrome and COVID-19: A Perfect Storm?” The reason for the ominous title is Espinosa’s examination of the genetics of Down syndrome and how they may contribute to the severity of coronavirus if contracted.

The first half of the opening paragraph portends the perfect storm:

Individuals with Down syndrome (DS) show widespread and chronic immune dysregulation. This population shows increased rates of diverse autoimmune conditions, including autoimmune thyroid disease, celiac disease, autoimmune skin conditions (e.g., alopecia areata, psoriasis, vitilligo, atopic dermatitis and/or eczema, hidradenitis supporativa), and type 1 diabetes. At the molecular and cellular levels, individuals with trisomy 21 show clear signs of inflammation in the absence of any detectable infections, such as elevated levels of potent inflammatory cytokines and chemokines, and changes in diverse immune cell types indicative of hyperactive, pro-inflammatory cellular states. In addition, individuals with trisomy 21 show more severe consequences during lung viral infections, such as increased rates of hospitalization during respiratory syncytial virus (RSV) and H1N1 influenza A infections, as well as increased rates of mortality from bacterial pneumonia and sepsis.

(citations omitted).

Those who follow the COVID-19 news may be aware of the theorized “cytokenetic storms” that are believed to contribute to the inflammation and death from the coronavirus. The theory is that the coronavirus initiates an immune system response of “cytokines” that then overreact and begin to attack healthy systems of the body. This is why immune system suppressing drugs are in trials as possible treatments for minimizing the impact of the coronavirus.

Unfortunately, for Down syndrome, at the genetic level “several genes encoded on chromosome 21 have established roles in immune control, and their overexpression could contribute to the general immune phenotype of DS.” Specifically, “people with DS show significantly elevated levels of key cytokines”.

To demonstrate the level of sophistication and complexity of Espinosa’s review, here is the last element in listing these immune system genetic phenomena of Down syndrome:

Lastly, metabolomics studies of both plasma and cerebrospinal fluid have revealed that individuals with DS display dysregulation of the [interferon](IFN)-inducibe kynurenine pathway of tryptophan catabolism, leading to elevated levels of quinolinic acid, a neurotoxic tryptopphan catabolite.

In the end, Espinosa concludes: “This body of evidence indicates that the IFN response, which is key for both mounting antiviral responses and initiating and amplifying the cytokine storm, is much more active in people with DS.”

So, the extra 21st chromosome that is the genetic basis for Down syndrome has immune system genes that are overexpressed making the cytokentic storm associated with coronavirus infection and death more likely. Espinosa cautions that his perspective is just that, a viewpoint, not an empirical finding, and therefore “should be approached with skepticism.”

That is, until there is empirical data supporting his theory:

COVID-19 Hospitalizations more frequent and severe in Down syndrome

In a study posted on June 2, 2020, researchers reviewed all cases of COVID-19 in the Mount Sinai Health System in New York City. Their findings support Espinosa’s hypothesis and corroborate the studies of the IDD population as applied specifically to Down syndrome.

The researchers first looked at all hospitalizations over a period of time and identified what percentage of those were patients with Down syndrome. These were just general hospitalizations, i.e. what is the incidence rate that someone with Down syndrome would be one of their health system’s patients. Based on that incident rate, they expected that out of the 4,615 total patients hospitalized due to COVID-19, just one would have been a patient with Down syndrome.

Instead it was six patients with Down syndrome.

This equated to an 8.9-fold higher risk of hospitalization with COVID-19 for patients with Down syndrome as compared to patients without DS.

Further, supporting Espinosa’s theory, their findings “may indicate that a subset of DS patients had higher levels of soluble inflammatory markers”. Consistent with that finding, (quoting):

  • Patients with DS were more likely to suffer from all the COVID-19 induced complications surveyed, including acute respiratory distress syndrome (ARDS), acute kidney injury (AKI), sepsis and encephalopathy.
  • [H]ospitalized patients with DS were more likely to receive mechanical ventilation and ICU care … .
  • Hospital stay was longer for patients with DS[.]
  • Two patients in the DS group (33.3%) and two patients in the control group (6.7% died).

More severe effects, including an almost 5X greater death rate, lead to the conclusion: “The COVID-19 patients with DS hospitalized at Mount Sinai thus had more severe disease progression and longer hospital stays than their non-DS counterparts.”

Vigilance required

All of these studies came out in a span of weeks between the end of May and beginning of June 2020. This is at the same time as all states, to one extent or another, began to relax quarantine/lockdown measures and started to “re-open.” It also coincides with the protests throughout the nation against racial discrimination by police in the wake of the George Floyd killing and, locally here in Louisville, Breonna Taylor.

Just as the prohibition against large pool parties and gatherings were to tamp down the spread of the virus, as more and more people leave quarantine and return to more regular patterns, states are seeing new spikes in cases of the coronavirus.

The studies summarized here support the guidance I wrote about at the outset of the pandemic based on how individuals with Down syndrome suffered in previous respiratory disease pandemics. Individuals with Down syndrome are at high risk, at all ages.

If our loved ones with Down syndrome get this “awful virus”, as my son calls it, odds are that it will be awful, more severe, and with greater chance of death. Therefore, vigilance is required to look out and care for our loved ones with Down syndrome, starting with our own actions to minimize our own exposure and infection.

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