Guidelines and Recommendations for Families Affected by MWS

The Mowat-Wilson Foundation has received a number of questions about how coronavirus disease 2019 (COVID-19) may affect individuals with MWS. We hope that you and your family are staying safe and healthy. During this time, the best source to turn to for information about your family member with MWS is your family member’s primary care physician, who will know your family member’s medical history, as well as local/state guidelines for protection against getting COVID-19.

The Mowat-Wilson Medical Advisory Board also realizes that many families are concerned about the risks of contracting COVID-19 and how this virus may affect their relatives with MWS differently. Individuals with underlying health conditions (including cardiovascular, pulmonary, and immune problems, to name a few) are thought to be at increased risk of having a more difficult time fighting off the virus, although age is also thought to be a factor, with individuals under age 20 years accounting for many fewer serious infections or deaths compared to those older than age 40 years. However, these are generalizations, and each individual is unique, as is the risk in each community based on local/geographic factors.

Many are wondering when it will be “safe” to return to some types of typical activities (i.e. going out in public, etc.). We encourage all families to be aware of their local/state/county/country guidelines regarding social distancing, the need to wear masks in public, and the slow reopening of businesses/schools. If an individual is unable to abide by the social distancing and/or mask requirements or recommendations, it may not be in their best interest to be about in larger public areas, even if those areas have been reopened. Additionally, individuals who explore their environment using their mouth (putting objects into their mouth) may have an increased risk of becoming infected with the virus if they are placed back into a daycare or school setting, particularly if that setting includes other individuals with special needs who also explore the environment using their mouth.

The medical factors that lead to a worse outcome are not completely known, although some underlying health conditions seems to predispose individuals to being sicker. While we do not know all factors that may lead to more severe illness or death, some factors that could increase this risk and are more specific to individuals with MWS are the following:

1. Those with a history of previous blood clots or clotting issues. This is rare in individuals with MWS.
2. Those with absent spleen or who have been told that their splenic function is not normal. Fortunately, most individuals with MWS do not have a known problem with their immune system.
3. Those with congenital heart defects; discussion of the actual heart defect and specific risks would need to take place in conjunction with the affected person’s primary care physician and cardiologist.
4. Any person with obesity (also, extremely uncommon in individuals with MWS).
5. Any person with baseline breathing issues (also rare in those with MWS who do not have a congenital heart defect).

Individuals with MWS may also be at increased risk for various types of “accidents” (including having a seizure, falling, etc.) that could put them in contact with first responders or other individuals in the general community who are trying to help. Before returning to more normal activities (school, daycare, day programs, etc.), it would be ideal for families to have a contingency plan in place on how to handle each risk-related event based on the individual’s risk factors and history and on how to address these issues with contact with others in the school setting and elsewhere. Then if “accidents” occur, there is a plan in place to address the individual’s needs without excessive exposure to others and the environment.

Many individuals with MWS have a history of seizures or currently have a seizure disorder that is treated with medication. Even if your affected family member does not have a history of seizures, we encourage you to read the statement below, as it applies to many individuals with chronic health conditions who may be taking prescription medication. The following is an excerpt from a statement put out by the American Epilepsy Society:

“The American Epilepsy Society (AES) urges its members and their patients to be aware and cautious while keeping the coronavirus disease 2019 (COVID-19) situation in perspective. In addition to monitoring US Centers for Disease Control and Prevention (CDC) statements and directives, healthcare providers (HCPs) should closely monitor statements and actions of state and local health departments.

Patients should be instructed to work with their HCP’s office and pharmacy to initiate prescription refills at least one week before they anticipate running out of medications for 30-day prescriptions and at least two weeks in advance for 90-day prescriptions. While not always possible under payer practices, where feasible and warranted by individual patient situations, HCPs may wish to consider writing 90-day rather than 30-day supplies of medications to ensure that patients have a sufficient quantity of medications on hand.

In a reminder of usual best practice, providers should encourage patients to work with their pharmacists to synchronize prescription refills of all medications, not just antiseizure medications. In addition, patients should be advised to discuss home delivery options with their pharmacy. These measures can minimize public exposure, in the event that public health authorities limit travel or public activities. Epilepsy centers and HCPs should also consider readiness for utilizing remote care options, if possible; i.e., telephone, telehealth, and electronic health record (EHR) messaging.

Viral illnesses may be a trigger for seizures for some, and patients should have an up-to-date plan for managing breakthrough seizures. If medications are involved in this plan, patients should ensure that they have a supply of these medications on hand, with expiration dates at least six months away.
Health professionals may wish to monitor drug shortages via the FDA Drug Shortages web page or the American Society of Health-System Pharmacists (ASHP) drug shortage tracking system which can now be integrated into EHR systems.

Additional advice for patients with epilepsy and their families is provided on the Epilepsy Foundation web page, Concerns about Coronavirus. AES will continue to monitor the impact on its members and their patients of the COVID-19 situation and will update information and guidance as needed.”

We know this is a stressful time for everyone and we hope that you and your family stay healthy.