Coronavirus (COVID-19) and Noonan Syndrome

Brett | Mar. 27, 2020

The current Coronavirus (COVID-19) pandemic has created a great deal of stress and anxiety in not just the Noonan Syndrome (NS) community but the general public as well. Many people and carers of people with NS may be concerned about how their condition influences their risk of contracting COVID-19. This Blog Post will explore COVID-19 in the context of NS and how it may relate to you or someone you provide care for.

What is COVID-19?

COVID-19 is a ‘new’ strain of Coronavirus. Coronaviruses (CoV) are a large family of viruses that cause illness ranging from the common cold to more severe diseases such as Middle East Respiratory Syndrome (MERS-CoV) and Severe Acute Respiratory Syndrome (SARS-CoV). A novel coronavirus (nCoV) is a new strain that has not been previously identified in humans.

Coronaviruses are zoonotic, meaning they are transmitted between animals and people.  Detailed investigations found that SARS-CoV was transmitted from civet cats to humans and MERS-CoV from dromedary camels to humans. Several known coronaviruses are circulating in animals that have not yet infected humans. Best contemporary evidence suggests COVID-19 was transmitted to humans from either Pangolin or Bats and this occurred within China in late 2019.

Common signs of infection include respiratory symptoms, fever, coughing, shortness of breath and breathing difficulties. In more severe cases, infection can cause pneumonia, severe acute respiratory syndrome, kidney failure and even death.

Standard recommendations to prevent infection spread include regular hand washing, covering mouth and nose when coughing and sneezing, thoroughly cooking meat and eggs. Avoid close contact with anyone showing symptoms of respiratory illness such as coughing and sneezing.

Are people with Noonan Syndrome at higher risk?

NS is not a uniform disease; it is a condition caused by missense mutation of multiple genes and the effect these mutations have functionally is further altered by environmental and other epigenetic influences. COVID-19 will affect people with NS differently based on their individual mutation and co-morbidities (other ailments). However as a general rule:

Noonan Syndrome does not necessarily increase your risk of contracting COVID-19

Immunosuppressive Conditions

However… there are many secondary conditions and ailments that occur at a greater frequency in NS than in the non-NS population and these WILL increase your risk of COVID-19. If you or a loved one with NS experiences these illnesses/conditions than they will need to exercise extra caution during the current COVID-19 pandemic. We will explore these now…

Cancer

The increased risk of all childhood cancers in NS is 8.1 times higher than the general population. Thankfully, this risk drops to 3.5 times by adulthood.  In regards to cancer incidence within NS the population, cancer can occur in as many as 10% of children with NS. If you want more information regarding Cancer and Noonan Syndrome you can read our Blog on this topic here: https://noonansyndrome.com.au/cancer-and-noonan-syndrome/.

If someone has cancer (particularly cancers that impact the immune system or blood, like leukemia), the disease itself can compromise the body’s immune response. Cancer treatments such as Chemotherapy can further impact the immune system and increase an individual’s vulnerability not just to COVID-19 but all viral and bacterial illnesses. If you are an individual currently undergoing cancer treatment, have recently completed cancer treatment or have received a cancer diagnosis it is imperative you contact your Doctor immediately should you develop any COVID-19 symptoms.

Autoimmune Conditions

There are a few autoimmune conditions that are more common in NS and these will affect immunity and increase the risk of contracting COVID-19. These conditions (in no particular order) are Lupus (SLE), Autoimmune Thyroiditis and Autoimmune Hepatitis. These conditions not only inhibit immunity but the treatment for these illnesses often require immune-suppessive medication which further heightens the risk of contracting viral pathogens such as COVID-19. If you would like more information on the Autoimmune Diseases that occur more frequently in NS you can see our Blog here: https://noonansyndrome.com.au/autoimmune-diseases-and-noonan-syndrome/. People with the aforementioned Autoimmune Diseases may not mount a ‘normal’ immune response to a pathogen such as COVID-19 so it’s important to see your Doctor at the first sign of any symptoms (fever, cough and shortness of breath).

Hypogammaglobulinemia

Hypogammaglobulinemia is a problem with the immune system that prevents it from making enough antibodies called immunoglobulins. Antibodies are proteins that help your body recognize and fight off foreign invaders like bacteria, viruses, and fungi. Without enough antibodies, you’re more likely to get infections such as COVID-19. Hypogammaglobulinemia occurs at a higher rate in people with NS than in the non-NS community. People with Hypogammaglobulinemia may not mount a ‘normal’ immune response to a pathogen such as COVID-19 so it’s important to see your Doctor at the first sign of any symptoms (fever, cough and shortness of breath).

I have one or more of these conditions… what should I do?!

It’s recommended that people who are immunocompromised follow the same general precautions as non-immunocompromised people (see below) such as washing your hands frequently, sneezing and coughing into the fold of your elbow, avoiding touching your face and people who may be ill, disinfecting/sanitizing surfaces regularly, and staying home if you’re sick. Immunocompromised individuals should also avoid all non-essential travel and avoid crowds as much as possible during the current COVID-19 pandemic.

Lowering your risk of COVID-19

Hygiene

It sounds inherently obvious and rather simplistic but the best way to prevent contracting and spreading COVID-19 is to practice good hygiene. Here’s what you can (and should) do:

  • cover your coughs and sneezes with your elbow or a tissue
  • put used tissues straight into the bin
  • use alcohol-based hand sanitisers
  • avoid touching your eyes, nose and mouth
  • clean and disinfect frequently used surfaces such as benchtops, desks and doorknobs
  • clean and disinfect frequently used objects such as mobile phones, keys, wallets and work passes
  • increase the amount of fresh air available by opening windows or adjusting air conditioning
  • wash your hands often with soap and water, including before and after eating and after going to the toilet

The last step of washing hands seems rudimentary but many people don’t wash their hands effectively. Here’s the step-by-step guide to washing your hands, including how long you should wash your hands:

  • Wet your hands with clean, running water (warm or cold), turn off the tap, and apply soap.
  • Lather your hands by rubbing them together with the soap. Lather the backs of your hands, between your fingers, and under your nails.
  • Scrub your hands for at least 20 seconds, which is about the length of time it takes to sing the “Happy Birthday” song from beginning to end twice.
  • Rinse your hands well under clean, running water.
  • Dry your hands using a clean towel or air dry them.

Social Distancing

Social distancing reduces risk by preventing the primary route of transmission of COVID-19 (droplets in the air from a cough, saliva or sneeze). It needs to be practiced in public, at home and at work.

Social distancing in public means people:

  • stay at home unless is absolutely necessary
  • keep 1.5 metres away from others
  • avoid physical greetings such as handshaking, hugs and kisses
  • use tap and pay instead of cash
  • travel at quiet times and avoid crowds
  • avoid public gatherings and at risk groups
  • practice good hygiene (see above)

Steps for social distancing in all homes include:

  • stay at home unless going out is absolutely necessary
  • keep visitors to a minimum
  • reduce visits to the shops — instead, buy more goods and services online if you can for pick-up, pre-order or delivery
  • carefully consider what travel and outings are necessary, both individual and family
  • regularly disinfect surfaces that are touched a lot, such as tables, kitchen benches and doorknobs
  • increase ventilation in the home by opening windows or adjust air conditioning

Steps for social distancing in the workplace include:

  • stop shaking hands to greet others
  • hold meetings via video conferencing or phone call
  • put off large meetings to a later date
  • hold essential meetings outside in the open air if possible
  • promote good hand, sneeze and cough hygiene
  • provide alcohol based hand rub for all staff and workers
  • eat lunch at your desk or outside rather than in the lunch room
  • regularly clean and disinfect surfaces that many people touch
  • open windows or adjust air conditioning for more ventilation
  • limit food handling and sharing of food in the workplace
  • avoid non-essential travel
  • promote strict hygiene among food preparation (canteen) staff and their close contacts
  • consider if you can reschedule, stagger or cancel non-essential meetings

Conclusion

Noonan Syndrome (NS) is a varied and multi-dimensional genetic disease with a myriad of clinical presentations which means how COVID-19 affects people with NS differs greatly from person to person. If an individual with NS doesn’t possess any of the conditions listed in this Blog they are at no larger risk of contracting COVID-19 than a person without NS.

The current COVID-19 pandemic has increased stress, anxiety and reduced access to care and community support networks for many people with NS. It is important to stay calm, follow the direction of your Medical Practitioner and relevant State and Federal Health Departments and most of all stay well. In the circumstance where you are at increased risk, most localities have introduced measures to help people with compromised immunity procure medications and groceries so that these people don’t increase their risk of contracting COVID-19. If you’re in Australia you can find information on these services here: https://www.health.gov.au/resources/publications/covid-19-national-health-plan-primary-care-home-medicines-services-to-support-the-response-to-covid-19

Please reach out to us if you have any concerns or questions regarding COVID-19 and how it relates to your Noonan Syndrome journey.

References

https://www.health.gov.au/news/health-alerts/novel-coronavirus-2019-ncov-health-alert/how-to-protect-yourself-and-others-from-coronavirus-covid-19/social-distancing-for-coronavirus-covid-19

https://www.shape.com/lifestyle/mind-and-body/immunocompromised-patients-coronavirus

https://www.healthline.com/health/hypogammaglobulinemia

https://www.mayoclinic.org/diseases-conditions/lupus/symptoms-causes/syc-20365789

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