Down Syndrome and COVID-19: An Especially Vulnerable Group

By Stewart J. Lawrence

Research shows that people with Down syndrome are three to ten times more likely to die from COVID-19 than the general population. They are also far more likely to develop complications from the disease. But until recently, the Centers for Disease Control, citing mixed scientific evidence, was reluctant to designate Down syndrome as one of the disease conditions “prioritized” for the vaccine. Down syndrome advocates, led by the National Down Syndrome Society, have lobbied the CDC for new protocols, but it was only last month that the CDC formally amended its guidance to include those with Down syndrome.

Still, getting everyone with Down syndrome vaccinated isn’t easy. Some states are more compliant with the new CDC guidance than others. In Maine many with Down syndrome were already vaccinated under the pre-existing priority for those living in group homes. But those living with their own families had fallen through the cracks. Thanks to more advocacy, all Down syndrome adults are now on Maine’s priority list.

But not in many other states, especially in the South. In Florida, for example, the COVID vaccine, until recently, was only available to healthcare workers, people 65 and older and patients in long-term care facilities. “We’re not a huge community, and we understand that. But we’re just as important as any other individual and every other family member. And it’s critically important that our community has that option to get the vaccine,” said Jeffery Leach, board president of the Jacksonville Down Syndrome Association in an interview with NBC News 4/Jacksonville in late January.

The special vulnerability of those with Down syndrome seems undeniable. And that vulnerability seems to increase sharply by age. One study conducted in Great Britain with more than 1,000 people with Down syndrome found that their vulnerability increased sharply by age. Those 40 and older exhibited a mortality rate of 51 percent compared to just 7 percent for those under 40. “At about the age of 40, things are getting really bad … [with] a mortality rate comparable with those older than 80 in the general population,” says Anke Huels, a biostatistician at the Rollins School of Public Health at Emory University, who led the UK study.

Why are people with Down syndrome at such a high risk? It comes down to biology. Those with Down syndrome have larger than average tongues, smaller jaws, relatively large tonsils and adenoids and weaker throat muscles, all of which leaves them more vulnerable to respiratory infections — including COVID-19.

Basic genetics is also partly to blame. People with Down syndrome have three copies of a gene on chromosome 21, TMPRSS2, which codes for an enzyme that allows the coronavirus to invade human cells more quickly.

Another factor is the way interferon, a signalling protein that regulates the body’s immune response to viruses, functions in those with Down syndrome. There is some evidence that it may actually strengthen the initial immune response — but people with Down syndrome also exhibit interferon “hyperactivity” — especially as they age — which ends up compromising their immune response as the disease progresses.

With this panoply of weakened defenses, it’s no wonder that those with Down syndrome — an estimated 200,000 people nationwide — are dying from COVID-19 at such relatively high rates, experts say.

Undoubtedly, more research on how COVID-19 affects those with Down syndrome is still needed. For example, some of the latest research suggests that children with Down syndrome may be just as vulnerable as adults to COVID-19 infection and mortality. That finding could help shape the next phase of the vaccine roll-out targeting younger age groups. But it may take more persistent advocacy to ensure that those with Down syndrome don’t find themselves left off the list of those needing special attention once again.

Stewart J. Lawrence is a trained sociologist and a Washington, D.C.-based consultant and policy analyst. He writes frequently on science and public health issues with a special focus on at-risk communities.

This originally appeared in the COVID-19 Observer on May 13, 2021.