Individuals & Families

COVID-19 & TSC Resources

Welcome to the TSC Alliance’s COVID-19 & TSC Resources page for people with tuberous sclerosis complex (TSC), their families and loved ones, healthcare professionals and others. Please visit this page often as we will continually update it as needed.

If you or a loved one has tuberous sclerosis complex, be sure to scroll down this page to read and/or download our Coronavirus Disease 2019 (COVID-19) Questions and Answers (FAQs).

COVID-19 Virtual Town Halls

The TSC Alliance partnered with The LAM Foundation to host a Virtual COVID Vaccine Update Town Hall on Thursday, August 19, 2021. Peter Crino, MD, PhD of the University of Maryland Medical Center, Nishant Gupta, MD of the University of Cincinnati Medical Center, Darcy Krueger, MD, PhD of Cincinnati Children’s Hospital and Joel Moss, MD, PhD of the National Heart, Lung, and Blood Institute at NIH discuss the recent announcement from the FDA authorizing additional vaccine doses and the implications for TSC and LAM communities. They also provide an update on the Delta variant and current mask recommendations.

The TSC Alliance partnered with The LAM Foundation to host a Virtual COVID Vaccine Update Town Hall on Tuesday, April 20. Peter Crino, MD, PhD of the University of Maryland Medical Center and Nishant Gupta, MD of the University of Cincinnati Medical Center provided updates on the current COVID-19 vaccines in the TSC and LAM communities.

COVID-19 Resources

Click the following links to access additional information about:

TSC Alliance COVID-19 Vaccine Position Statement – Update

November 16, 2021

COVID-19 Vaccination Addendum:

The FDA has authorized the use of Pfizer’s COVID-19 vaccine, under an Emergency Use Authorization (EUA), in individuals 5 years of age and older. In addition, for those 18 years and older, the CDC has approved an individual’s choice of COVID-19 vaccine as a booster. Some people may prefer the same vaccine type originally received, whereas others may choose a different booster.

FAQ: New eligibility of COVID-19 vaccine for individuals 5 years of age and older.

Does my child need to stop taking any medication prior to the vaccine?

No. Ongoing research provides no evidence to alter or stop any medication including mTOR inhibitors prior to the vaccine. We strongly defer recommendations to the prescribing providers preference.

Will my child’s seizures worsen with the vaccine?

It is possible to have a breakthrough febrile (fever) associated seizure due to any vaccination. However, there is still no evidence that any of the COVID-19 vaccines lower seizure threshold. Always defer to your seizure action plan defined by your current provider.

Do I need to get my child’s antibodies tested after vaccination series has been completed?

Currently there is no evidence that support antibody level testing after completion of the vaccination series. At the time of this addendum, there is no consensus on antibody levels required for a protective response. We strongly defer recommendations to the prescribing providers preference.

Can my child receive the vaccine at the same time of their other childhood vaccinations including the seasonal influenza vaccine?

Yes. The American Academy of Pediatrics confirms that COVID-19 vaccines can be co-administered on the same day as other routine vaccinations – or within 14 days – of other vaccines. If your child is on an altered schedule of vaccinations or there are additional concerns, we strongly recommend discussing this with your local pediatrician and pediatric neurologist.

When my child receives the vaccine will they be immune from contracting the virus?

No. To be considered “fully protected” you must complete the vaccination series. Two weeks following the last (2^nd) vaccine injection, you are considered “fully vaccinated.” Remember, vaccines reduce transmission; however, those vaccinated can still become infected. In fully vaccinated individuals (completed vaccine series and 2 weeks post-last injection) the associated infections are milder and prevent serious illness. The goal of vaccination is to decrease the severity of complications associated with the illness.

If my child had COVID-19, should they still get vaccinated?

Yes. Studies in adults have provided evidence that detectable antibodies levels are variable following “natural” COVID-19 infection, where some have protection for only a few weeks. Furthermore, the risk of reinfection after natural infection is 2 to 5 times more likely, which raises the need to consider vaccination.

How is the Pfizer vaccine different for ages 5 – 11 than the other available vaccines?

Currently, Pfizer has the only approved vaccine for this age group. The dose is one-third of the dose for the 12+ age group (10 micrograms vs 30 micrograms) and is safe and effective.

If my child is currently 11 but will turn 12 before the second dose, what should I do?

If your child received the smaller dose, you should complete with the smaller dose to complete the series. Unlike other medications, this vaccine is not weight dependent. The dose is based on immune system maturity. Furthermore, do not wait until your child is 12 before starting the vaccination series

What are most common side effects of the vaccine for ages 5-11?

Compared to adults and children age 12+, children ages 5-11 showed similar side effects such as, fatigue, headache, muscle pain and soreness at the injection site. Fever and chills were not as common as compared to the other age group trials. The most common adverse events are lymphadenopathy (swelling of the lymph nodes) or hypersensitivity (rash/dermatitis). In the event these occur we recommend contacting your local health care provider.

You can also self-report any adverse event through the vaccine adverse event reporting system (VAERS) at https://vaers.hhs.gov/index. If your child is experiencing a medical emergency, you need to seek immediate assistance from a healthcare provider or call 911. VAERS is for data tracking purposes and does NOT provide medical treatment.

Given TSC cardiac manifestations, is my child at higher risk of vaccine induced myocarditis?

Myocarditis is inflammation of the heart caused by viral and other pathogens. Myocarditis due to COVID-19 vaccine is milder as compared to classic myocarditis, covid 19 virus, or MIS-C myocarditis. In continuing clinical studies in children 5–11-year-old with the Pfizer vaccine, there has been no reported cases of myocarditis. Given the uniqueness of TSC, personal concerns and other health related issues, we strongly recommend discussing this with your local pediatrician and TSC providers.

FAQ: Individuals 18 years of age and older boosters and mixing of boosters

Does TSC qualify me to receive a booster?

We strongly encourage you to have further discussion with your prescribing provider as qualification due to TSC, is dependent on the individual manifestations. Currently, boosters are approved for people 18+ years of age with an underlying health condition, particularly those with severe immunosuppression (e.g., certain cancers, organ transplant, HIV). Those who take mTOR inhibitors for TSC/LAM specific indications could have a lower immune response. Ongoing studies provide some evidence that individuals with TSC/LAM on a single therapy mTOR inhibitor exhibit an appropriate response for the 2-dose mRNA COVID-19 vaccination series, or the 1 dose of Johnson & Johnson shot.

When should I get a booster?

It is encouraged that you receive a booster vaccine when available (6 months after mRNA series or 2 months after Johnson & Johnson shot). This is due to waning antibodies over time and risk for complications associated to those with underlying health conditions associated with TSC. For those who have severe immunosuppression (such as certain cancers, organ transplant, and HIV), 3 shots were required to generate a desired antibody response to complete their primary series and will require an additional booster 6 months for the time of the last shot. This is also being referred to as “4^th booster.” We strongly encourage further discussion with your prescribing provider preference.

What booster is recommended for those with TSC?

All booster vaccines are shown to provide protection. Those who had an mRNA-based vaccine should continue with an mRNA-based booster if available locally. Ideally, if you tolerated the Pfizer vaccine for your first series, it is reasonable to continue with that manufactured vaccine. However, if locally Moderna is only mRNA vaccine available, it is safe and effective to mix from your primary series to your booster.

For those who took Johnson & Johnson adenovirus-based vaccine, there is evidence to support wider protection from an mRNA-based booster. If Johnson & Johnson is the only vaccine booster available, it is completely fine receiving this booster shot. Either mRNA-based vaccine can be used. We strongly defer recommendations to provider preference and encourage discussion on the options with your local or TSC/LAM expert providers.

Are there any differences to the booster shots than the primary series vaccines?

Pfizer and Johnson & Johnson both are utilizing the same dose for their booster shots and the primary vaccines. Moderna booster dose is reduced by 50%.

What are the most common side effects associated with the booster shot?

Fever, headache, fatigue, and pain at the injection site of the shot. These symptoms usually resolve within 24 to 48 hours and are normal response to a vaccine. Most common adverse events are lymphadenopathy (swelling of the lymph nodes) or hypersensitivity (rash/dermatitis). In the event these occur, we recommend you reaching out to your local health care provider. You can also self-report any adverse event through the vaccine adverse event reporting system (VAERS) at https://vaers.hhs.gov/index. If you are experiencing a medical emergency, you need to seek immediate assistance from a healthcare provider or call 911. VAERS is for data tracking purposes, and you will not receive individualized medical treatment.

Can I/my adult child receive the vaccine at the same time of the seasonal influenza vaccine?

Yes. The CDC confirms that COVID-19 vaccines can be co-administered on the same day as other routine vaccinations – or within 14 days – of other vaccines.

Medical Review Note

This information was reviewed and approved by:

Peter B. Crino, MD, PhD, Chair, TSC Alliance Board of Directors
Darcy A. Krueger, MD, PhD, Chair, TSC Alliance Professional Advisory Board
Mustafa Sahin, MD, PhD, Chair, TSC Alliance International Scientific Advisory Board and Co-Chair, TSC Alliance Science and Medical Committee
John J. Bissler, MD, TSC Alliance Professional Advisory Board
Nishant Gupta, MD, Director of LAM Clinic Network
Joel Moss, MD, PhD, TSC Alliance Professional Advisory Board
Elizabeth Petri Henske, MD, TSC Alliance Professional Advisory Board

For additional questions or concerns please reach out to Ashley Pounders MSN, FNP-C,TSC Alliance Director, Medical Affairs at apounders@tscalliance.org or 240-472-4302.

Disclaimer

This content was created for general informational purposes only. The content is not intended to be a substitute for professional medical advice. The risk profile for each individual is unique, and immunity from COVID-19 may be affected by factors such as age, chronic health conditions, and other medications. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding these recommendations.

Download the Updated Position Statement

August 18, 2021

The United States continues to face an unprecedented public health challenge during the COVID-19 pandemic with more than 617,787 lives lost in 18 months. Throughout this time frame, the global medical and scientific community has rallied together to address detection of the virus, learn and implement best practices in clinical care, and develop vaccines to prevent or lessen infection and serious symptoms. Three vaccines (Pfizer, Moderna, and Johnson & Johnson) have received emergency use authorization (EUAs) from the FDA to slow the spread of COVID-19 and improve the protection and outcomes of the public. ALL U.S. citizens 12 years of age and older are eligible for vaccination. At the time of drafting this document, over 167 million individuals (~50% U.S. population) have been fully vaccinated. Ongoing clinical trials will hopefully extend eligibility for those younger than 12 years of age in the coming months.

Take Home Messages

If you have not yet received a COVID-19 vaccine, get vaccinated as soon as possible, even if you have already had COVID-19 infection.
You do not need a prescription by your physician to get the vaccine.
If you are currently taking Everolimus or Sirolimus for TSC, stay on these medications unless otherwise directed by your TSC clinician(s).
If you are currently taking Everolimus or Sirolimus for TSC, and you have already received two doses of COVID-19 mRNA vaccine (Pfizer or Moderna), you are eligible for an additional, third dose of the vaccine, a “booster” vaccine.
The TSC Alliance recommends a third vaccine dose for TSC individuals currently treated with Everolimus or Sirolimus who have previously received either the Pfizer or the Moderna vaccines.
The current recommendations are to receive the same kind of vaccine for the third dose that you received for the first two doses i.e., Pfizer for Pfizer, Moderna for Moderna). Mixing vaccines is permitted if same vaccine type is not available.
There is no recommendation yet regarding an additional dose for those individuals who have received a single dose vaccine such as the Johnson and Johnson vaccine.

Considerations for an informed choice on getting vaccinated

Vaccine Response and mTOR Inhibitors
Vaccines train the immune system to recognize and help clear infectious agents such as viruses from the body. In such a response, the cells of the immune system develop antibodies against pieces of the virus called antigens. These antibodies attach to the antigen on the virus, providing a signal to the immune system cells to attack and remove the virus from the body. In the case of COVID-19, approved vaccines train the immune system to recognize COVID-19 antigens called “spike protein.”

Everolimus and Sirolimus are members of a class of drugs called mTOR inhibitors and are commonly used to treat brain, kidney, and lung manifestations of TSC, including subependymal brain tumors, kidney angiomyolipomas, epilepsy and LAM. The concern with those who take mTOR inhibitors (e.g., Everolimus and Sirolimus) for TSC is that this class of medicine might suppress the immune system (immunosuppressant), lowering the body’s ability to mount an effective attack (e.g., antibodies) against pathogens causing infections. Studies in people taking mTOR inhibitors along with other immunosuppressive drugs to prevent rejection after organ transplantation concluded that those individuals were less likely to have an effective immune response even after receiving the appropriate vaccinations. These studies provided strong supporting evidence to encourage the FDA to approve a third dose of vaccination in individuals taking immunosuppressants including Everolimus and Sirolimus.

The extent to which mTOR inhibitors alone reduce the production of an effective immune response following vaccination is not well understood. It is likely that patients on mTOR inhibitors alone mount a significantly better response to vaccination compared with other immunosuppressed patients such as post-transplant individuals who generally tend to be on multiple immunosuppressive medications. Small independent studies (non-published) in the TSC and LAM communities have demonstrated this response but require large scale validation. Given this uncertainty combined with the potential for severe complications in patients with TSC after COVID-19 infection, our consensus recommendation is for TSC patients on mTOR inhibitors to receive the additional (third) dose of vaccination. The consensus group also recommends continuing to take mTOR inhibitors for disease management and not to stop unless directed by your healthcare specialist.

COVID-19 Vaccines in Individuals with TSC
As part of the mission of the TSC Alliance to keep you informed, we compiled recent information regarding the safety and effectiveness of COVID-19 vaccination, vaccine hesitancy, vaccination response on mTOR inhibitors, long-term COVID-19 challenges, and school barriers in those living with TSC. With the large amount of information and misinformation available, our job as leading expert providers, researchers and advocates is to provide accurate information for you to make a well-informed decision regarding the benefits and safety of vaccination.

Why should individuals with TSC be vaccinated?
Unless otherwise contraindicated, everyone should receive a COVID-19 vaccination since all commercial COVID-19 vaccinations lower your risk of contracting COVID-19 and developing severe COVID-19 complications including hospitalization and need for the intensive care unit. They also reduce your chances of spreading the infection to others. TSC is a multi-system disease that may put individuals at risk for severe COVID-19 illness. It is known that COVID-19 virus can cause inflammation of bodily organs. Multiple studies have shown the neurological impact of COVID-19 including persistent headaches, dizziness, mental fog, and difficulty articulating words. Those with epilepsy may be at risk for loss of seizure control or worsening seizures. Individuals with LAM and other associated pulmonary conditions may experience pneumonia or other severe illness due to the virus. COVID-19 may stress the kidneys, and those with high blood pressure or existing kidney disease are at an increased risk of developing more severe kidney disease, including kidney failure. It is especially important to prevent additional kidney damage in individuals with TSC and polycystic kidney disease (PKD). For those who need to receive the COVID-19 vaccination and their other childhood vaccinations, these vaccines can be administered at the same time – or within 14 days – as other vaccines. If your child is on an altered childhood vaccination schedule or there are additional concerns, we strongly recommend discussing this with your local pediatrician and or pediatric neurologist.

Why should family members get vaccinated?
It is important to understand those who are not eligible for a vaccination or choose not to be vaccinated are vulnerable to COVID-19 infection, and therefore their protection from the virus is dependent on all individuals in close living conditions or proximity take measures such as vaccination and wearing masks to prevent the spread. Family members, friends, teachers, aids, and others getting vaccinated significantly reduce the transmission of the virus to those who cannot receive a vaccination or achieve an adequate immune response.

Previous History of COVID-19 Infection – Should I still get vaccinated?
The simple answer is “yes”. Antibodies can be detected for about 4 weeks after having COVID-19, although it can take several months in some people. Data show antibodies are less consistently detectable after COVID-19 infection compared to after vaccination. We are starting to understand that natural antibodies mainly attach to one protein region while vaccination can produce antibodies attaching to several protein regions, creating better chance of protection against new virus variants that may emerge. We also know that re-infection rates are 2x higher among unvaccinated individuals with past COVID-19 infections compared to those who did get vaccinated after a COVID-19 infection. A study by the CDC demonstrated that the natural immune response did not protect against reinfection as well as vaccinations. In addition, the CDC also found that individuals who had not been vaccinated and developed a second COVID-19 infection, had more severe clinical COVID-19 illness.

Vaccine Hesitancy
As with any new treatments or recommendations it is natural to be skeptical. With the overwhelming amount of misinformation on social and other media outlets, and inconsistency of recommendations over the last year and half, we understand many remain cautious, and it is important we clarify common misconceptions. mRNA vaccines (Pfizer and Moderna) have been studied and researched by scientists since the 1990’s.

All approved COVID-19 vaccines have been through rigorous and controlled clinical trials to establish both the safety and effectiveness. Clinical trials with Pfizer, Moderna and Johnson & Johnson vaccines evaluated the safety and effectiveness in over 150,000 participants. To put this in perspective, key clinical trials for the approval of mTOR therapy for SEGAs and renal angiomyolipomas (EXIST-1 and EXIST-2) included approximately 235 clinical trial participants.

These vaccines have mild side effects that are considered a normal response (e.g., injection site pain, headache) and typically resolve within a few days. Severe vaccine side effects occur in less than 0.5% of those vaccinated. Generally, vaccines are safe with no evidence to support long term complications. Further, there is nothing in mRNA vaccines that would carry potential long term side effects. For short term effects, as with any vaccination that can lower seizure threshold, it is possible to have a breakthrough febrile (fever) associated seizure. However, there is currently no evidence that any of the COVID-19 vaccinations worsen preexisting seizure baseline. The consensus group continues to defer to your seizure action plan defined by your current provider. Finally, you cannot contract COVID-19 from any of the commercially available vaccines.

Third Dose of COVID-19 Vaccination (“Booster”)
On August 12 and 13, 2021, the FDA approved emergency use authorization (EUAs) for, and CDC recommended, additional doses of mRNA COVID-19 vaccines following a primary series in immunocompromised individuals. This amendment applies to the Pfizer COVID-19 vaccine for those 12 years old or older and Moderna COVID-19 vaccine for those 18 years old or older. Those who received the Johnson & Johnson COVID-19 vaccine are not eligible for an additional dose at this time due to insufficient data. Ongoing research is being conducted to ensure optimal vaccine protection for those who received the Johnson & Johnson vaccine, and recommendations will be updated once sufficient data is available.

The Centers for Disease Control (CDC) has prioritized an additional (third) dose of COVID-19 vaccine for the following immunocompromised people:

Active or recent treatment for solid tumor and hematologic malignancies
Recent of solid-organ or recent hematopoietic stem cell transplants
Severe primary immunodeficiency
Advanced or untreated HIV infection
Active treatment with high-dose corticosteroids, alkylating agents, antimetabolites, tumor-necrosis (TNF) blockers, and other biologic agents that are immunosuppressive or immunomodulatory
Chronic medical conditions such as asplenia and chronic renal disease may be associated with varying degrees of immune deficit

Who is eligible for additional dose of the vaccine?

Individuals with TSC who are on immunosuppressive biologic agents (Everolimus or Sirolimus)
18 years of age or older who received two doses of the Moderna vaccine or 12 years of age or older who received two doses of the Pfizer vaccine
Those who received the second dose of the Pfizer or Moderna COVID-19 vaccine series at least 28 days prior to receiving a third dose
All attempts should be made to receive the same mRNA dose as the primary series, however if that is not feasible, an additional dose of the other mRNA vaccine is permitted.
Contact your local pharmacies to schedule your additional dose. You do not need a physician prescription to receive the additional dose.

I have TSC but am not on Everolimus or Sirolimus. Do I qualify for an additional vaccine dose?
No, the current recommendations are only applicable to patients with TSC who are on Everolimus or Sirolimus or qualify under one of the other above-mentioned CDC criteria. It is likely that in the near future, all of us will require an additional dose of the COVID-19 vaccination to boost our protection.

Public Health Measures to Protect Individuals with TSC
Regardless of vaccination status, proper and basic public health measures should continue to be followed. It is strongly encouraged that all individuals wear a mask indoors when not at home and when in large crowds outdoors. Continue to stay 6 feet apart from others that do not live within the same dwelling. Avoid crowds and poorly ventilated spaces. Close contacts of those living with TSC should be strongly encouraged to be fully vaccinated against COVID-19.

In addition to masking and social distancing, we understand that many individuals are faced with profound barriers and concerns as schools and work are returning to in person environments. Nationally individual states and school districts have conflicting recommendations that make decisions even more difficult. Each individual situation is unique and requires carefully thought-out interventions to ensure safe return to school for social development and education, and fair and equal access to day and vocational programs for TSC individuals and the challenges with masking and unvaccinated individuals.

Here are some helpful tips that can easily be requested and added to individualized education plans (IEPs) and other individualized plans:

Request arrival and dismissal times where there is reduced population
Limit exposure during high-risk activities such as eating in the classroom and sharing of common items and avoiding communal items such as water fountains.
Request all staff to always wear masks around high-risk individuals
Request for HEPA Filter for classroom
Request for Clorox 360 and other room cleaning devices to be used daily
Request education to custodial staff to pay special attention to classroom
Limit multiple room exposures and request additional services to come to classroom
Highlight importance of seizure action plan as viruses that lower seizure threshold might be first sign of impending infection
Proper notification to parent / guardian when there has been direct infectious exposure to at risk individual

Additional Resources and Recommendations
As the COVID-19 pandemic persists and new variants are emerging such as the Delta variant, we will continue to update and provide accurate information to the community. Variants such as Delta are proving that reinfection and breakthrough cases are possible, even in the vaccinated population. This stresses the importance that we keep our community safe. Despite showing breakthrough COVID-19 cases, those who are vaccinated are having less severe and fatal consequences with quicker recovery times. Many geographical areas are facing unprecedented hospitalization records across pediatric and adult hospitals. More than ever, the TSC community must remain diligent with prevention measures.

Antibody testing after vaccination is not clinically necessary unless advised by a specialist. For those who wish to know their antibody response, we urge them to discuss with their local healthcare team to determine whether and how to be tested. Those who are already enrolled in the TSC Alliance Natural History Database and have had antibody testing and wish to have your antibody testing including in the study, please contact biosample@tscalliance.org.

Medical Review Note

This information was reviewed and approved by:

Peter B. Crino, MD, PhD, Chair, TSC Alliance Board of Directors
Darcy A. Krueger, MD, PhD, Chair, TSC Alliance Professional Advisory Board
Mustafa Sahin, MD, PhD, Chair, TSC Alliance International Scientific Advisory Board and Co-Chair, TSC Alliance Science and Medical Committee
John J. Bissler, MD, TSC Alliance Professional Advisory Board
Nishant Gupta, MD, Director of LAM Clinic Network
Elizabeth Thiele MD, PhD, Massachusetts General Hospital
Joel Moss, MD, PhD, TSC Alliance Professional Advisory Board

For additional questions or concerns please reach out to Ashley Pounders MSN, FNP-C, TSC Alliance Director, Medical Affairs at apounders@tscalliance.org or 240-472-4302.

Disclaimer
This content was created for general informational purposes only. The content is not intended to be a substitute for professional medical advice. The risk profile for each individual is unique, and immunity from COVID-19 may be affected by factors such as age, chronic health conditions, and other medications. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding these recommendations.

Download the TSC Alliance’s Updated Position Statement

TSC Alliance COVID-19 Vaccine Position — Addendum

May 18, 2021

Pfizer’s COVID -19 vaccination has now been authorized for use by the FDA under EUA in individuals 12 years of age and older, concluding that there is no evidence suggesting a need to change or delay medications because of vaccinations.

Do I need to stop taking my mTOR before my vaccine?

Outside of a small solid organ transplant study, we currently do not have consistent evidence to change mTOR inhibitor management during vaccination phases. We strongly defer recommendations to the prescribing provider preference.

After my vaccination should I have my child’s antibodies tested?

Outside of a small solid organ transplant study, we currently do not have consistent evidence to suggest obtaining antibody testing after the vaccination phases. We strongly defer recommendations to the prescribing provider preference.

Will my child’s seizures worsen with the vaccine?

As with any vaccination that can lower seizure threshold, it is possible to have a breakthrough febrile associated seizure. However, there is currently no evidence that any of the COVID-19 vaccinations worsen preexisting seizure baseline. Defer to your seizure action plan defined by your current provider.

My child has advanced end-stage renal disease and is being worked up for a renal transplant. Can my child still get vaccinated?

Yes, it is recommended. We strongly defer recommendations to the local transplant team for further directions. (Reference; National Kidney Foundation)

Can my child receive the COVID-19 vaccination at the same time of their other childhood vaccinations?

Yes. The American Academy of Pediatrics confirms that COVID-19 vaccines can be administered at the same time – or within 14 days – as other vaccines. This is contrary to the previous recommendations that COVID-19 vaccines should not be given within 14 days of other vaccines. If your child is on an altered schedule or there are additional concerns, we strongly recommend discussing this with your local pediatrician and or pediatric neurologist.

Medical Review Note

This information was reviewed and approved by:

Peter B. Crino, MD, PhD, Chair, TSC Alliance Board of Directors
Darcy A. Krueger, MD, PhD, Chair, TSC Alliance Professional Advisory Board
Mustafa Sahin, MD, PhD, Chair, TSC Alliance International Scientific Advisory Board and Co-Chair, TSC Alliance Science and Medical Committee
John J. Bissler, MD, TSC Alliance Professional Advisory Board
Nishant Gupta, MD, Director of LAM Clinic Network
Elizabeth Petri Henske, MD, TSC Alliance Professional Advisory Board

For additional questions or concerns please reach out to Ashley Pounders MSN, FNP-C, TSC Alliance Director, Medical Affairs at apounders@tscalliance.org or 240-472-4302.

TSC Alliance COVID-19 Vaccine Position Statement

December 18, 2020

The United States has faced unprecedented public health challenges during the COVID-19 pandemic. Tragically, this virus has claimed more than 300,000 lives in the U.S. Amazingly, vaccines from several pharmaceutical companies, including Pfizer and Moderna, have been recently developed, evaluated for safety and effectiveness, and granted emergency use authorization by the FDA for rapid deployment across the country. The vaccines confer >90% effectiveness against SARS-CoV2 (the virus that causes COVID-19) and are largely viewed by experts as safe for adults over the age of 16.

The Centers for Disease Control (CDC) has targeted prioritization of COVID-19 vaccinations for the following demographic sub-groups:

Healthcare personnel
Workers in essential and critical industries
People residing in congregate housing situations such as nursing homes
People at high risk for severe COVID-19 illness due to underlying medical conditions
People 65 years and older

The vaccine is not yet intended for administration to children under age 16.

COVID-19 Vaccines in Individuals with TSC

Adults (16 and up) with tuberous sclerosis complex (TSC) will be able to receive COVID-19 vaccinations in the upcoming months. As part of the mission of the TSC Alliance to keep you informed, we compiled the following information regarding the safety and efficacy of COVID-19 vaccination in individuals facing the multi-system challenges of TSC including LAM, kidney disease, epilepsy, and autism. Ultimately, getting the COVID-19 vaccine will be a personal decision, but the current data suggests the risk:benefit ratio of this vaccine is highly favorable with effectiveness greater than 90%. It is highly likely that receiving this vaccine will protect individuals from severe COVID-19 infection, and it seems prudent for use in individuals with TSC.

The TSC Alliance asked a number of TSC clinical experts across the U.S. to evaluate the safety of the COVID-19 vaccine in TSC to help patients, families, and caregivers evaluate the vaccine in an informed manner. Of course, no trial to-date has specifically assessed the COVID-19 vaccine in TSC patients, so these opinions reflect best assessments of current knowledge and medical literature.

What Are the Public Health Goals of COVID-19 Vaccination?

The CDC endorses the following public health goals for COVID-19 vaccination:

Decrease death and serious disease as much as possible
Preserve functioning of society
Reduce the extra burden the disease is having on people already facing disparities
Increase the chance for everyone to enjoy health and well-being

Is the Vaccine Safe in TSC Patients?

Our TSC experts have reviewed the existing data from human COVID-19 trials and agree the vaccine is safe for administration in TSC patients. The CDC identifies at least 11 medical conditions that confer an increased risk of severe COVID-19 infection, meaning high likelihood of hospitalization, intensive care unit requirements, and even death. While TSC is not listed, chronic kidney disease and chronic lung disease are listed and provide support for the need for vaccination in TSC.

Can I Contract COVID-19 from the Vaccine?

No, both the Pfizer and Moderna vaccines are not live vaccines. Both use a messenger RNA (mRNA) technology to induce immunity, so it is not possible to acquire COVID-19 infection from either of these vaccines.

Will the Vaccine be Effective If I am on a mTOR Inhibitor Such as Everolimus (Afinitor) or Sirolimus (Rapamune)?

There is no specific data addressing the safety or effectiveness of the COVID-19 vaccine in patients receiving everolimus (Afinitor) or sirolimus (Rapamune). The concern is whether the immunosuppressive effect of these medications would alter the safety profile or effectiveness of the COVID-19 vaccine. Analyses of patients who are immunosuppressed for other reasons such as cancer or treatment with immunosuppressant medications to control organ transplant rejection suggest the COVID-19 vaccine will be safe with no added side effects and likely to be effective, or at least more effective than getting no vaccination. Interestingly, some medical literature suggests everolimus (Afinitor) or sirolimus (Rapamune) may actually enhance the effectiveness of vaccines such as the influenza (flu) vaccine.

What About Side Effects of COVID-19 Vaccines in TSC?

The COVID-19 vaccines are associated some mild-moderate side effects. For example, some individuals will experience only pain at the site of the injection, while others may experience fever, chills, malaise, or headaches. These symptoms typically last approximately 24 hours and stop spontaneously. You can take over-the-counter medications such as acetaminophen (Tylenol) to help reduce symptoms. There is no evidence these side effects will be more severe in individuals with TSC or that TSC patients are at risk for more severe or dangerous side effects. In a very small number of individuals with known allergic hypersensitivity reactions (i.e., those who carry an EpiPen for allergic reaction to peanuts, eggs, or shellfish), there is a chance you might have an allergic reaction to the vaccine. The risk-benefit ratio of COVID-19 vaccination in these individuals should be discussed with care providers. Other unexpected or more severe side effects are not out of the question with any vaccine, including any of the COVID-19 vaccines, but we suspect these will be rare and that the benefits of vaccination to our patients, their family members and close contacts, and society as a whole, far outweigh the likelihood of the risk of any severe, unexpected side effects.

Will Children be Vaccinated?

To date, large trials in children under age 16 have not been completed. The current vaccine approval is for individuals >16 years of age. We expect that children and early teens eventually will get vaccinated for COVID-19, when distribution channels and supplies allow for it and after gathering more safety and efficacy data on the vaccine in adults. It is likely that the FDA will want to assess safety data from a future vaccine trial in children.

Should I Receive the COVID-19 Vaccine?

The decision to receive the COVID-19 vaccine is a personal one. However, in view of the clear and documented effectiveness (>90%) of the vaccines in clinical trials and relatively benign safety profile, it is highly likely the COVID-19 vaccine will lead to immunity from COVID-19 infection. Since COVID-19 infection can lead to hospitalization, long-term post-infection consequences to bodily function, and death (>300,000 Americans have died from COVID-19), all clinical indicators and prudence would dictate you and your loved ones should receive the vaccine to protect you from COVID-19 infection.

If I Get the Vaccine, Will Everything be “Normal Again”? Can I Stop Social Distancing? Can I Stop Wearing a Mask?

The CDC and other experts have stated clearly the COVID-19 vaccine is yet another means to prevent COVID-19 infection and should be viewed as additive to social distancing and mask wearing. Data to-date demonstrates maximum immunity following the COVID-19 vaccine will not be attained until 2 weeks following the second vaccination or about 5-6 weeks after the first vaccination. Currently it is recommended that even after you have received the vaccine, you should continue practicing social distancing and mask wearing, avoid large social gatherings, continue good handwashing practices, and quarantine yourself if you have been exposed to someone with known COVID-19 infection, or if you develop symptoms of COVID-19 infection, such as fever, chills, cough, loss of taste or smell, diarrhea, headache.

Medical Review Note

This information was reviewed and approved by:

Peter B. Crino, MD, PhD, Chair, TSC Alliance Board of Directors
Darcy A. Krueger, MD, PhD, Chair, TSC Alliance Professional Advisory Board
Mustafa Sahin, MD, PhD, Chair, TSC Alliance International Scientific Advisory Board and Co-Chair, TSC Alliance Science and Medical Committee
John J. Bissler, MD, TSC Alliance Professional Advisory Board
Nishant Gupta, MD, Director of LAM Clinic Network
Elizabeth Petri Henske, MD, TSC Alliance Professional Advisory Board
Elizabeth A. Thiele, MD, PhD, TSC Alliance Professional Advisory Board

Novel Coronavirus Disease 2019 (COVID-19) Frequently Asked Questions and Answers (FAQs)

Updated December 4, 2020

The Department of Health & Human Services (HHS), in coordination with NIH and Operation Warp Speed, has launched a central web portal called Combat COVID (https://combatcovid.hhs.gov/).

The portal is a one-stop resource to help members of the public and doctors find information about different stages of COVID-19 illness, NIH-supported COVID-19 prevention and treatment clinical trials, and locations to donate plasma. The website provides clear and easy-to-understand information for:

People who have never had COVID-19 who may be interested in joining a vaccine or other prevention trial
People who have COVID-19 and want to participate in a treatment clinical trial
People who have recovered from COVID-19 who may be interested in donating plasma
Doctors and other healthcare providers who want more information to guide their patients who have COVID-19

Visit combatCOVID.hhs.gov to learn more.

Updated March 20, 2020

View the video recording of our TSC/LAM VIRTUAL TOWN HALL: A COVID-19 UPDATE with Drs. Peter Crino, MD, PhD, University of Maryland School of Medicine, Frank McCormack, MD, University of Cincinnati School of Medicine, Darcy Krueger, MD, PhD, Cincinnati Children’s Hospital Medical Center, and John Bissler, MD, St. Jude Pediatrics Research Hospital, who share considerations for impact of COVID-19 on management of TSC and LAM.

Updated May 20, 2020
Due to quickly evolving information, recommendations may be subject to change and will be updated as needed.

The TSC Alliance prepared these FAQs to address concerns from the TSC community and healthcare professionals regarding the novel coronavirus disease 2019 (COVID-19) outbreak. For more comprehensive and up-to-date information refer to the Centers for Disease Control and Prevention (CDC) website.

SYMPTOMS OF COVID-19

People with COVID-19 have had a wide range of symptoms reported – ranging from mild symptoms to severe illness. Symptoms may appear 2-14 days after exposure to the virus. People with the following symptoms may have COVID-19:

Cough
Shortness of breath or difficulty breathing
Fever
Chills
Muscle pain
Sore throat
New loss of taste or smell

This list is not all possible symptoms. Other less common symptoms have been reported, including gastrointestinal symptoms like nausea, vomiting, or diarrhea.

RISKS OF ILLNESS

1. Can I get sick by touching a surface or object that has the virus on it?

According to the CDC, coronaviruses are generally thought to be spread from person-to-person through inhalation of respiratory droplets produced when an infected person coughs or sneezes.
It may be possible for a person to become ill by touching a surface or object that has the virus on it and then touching their own mouth, nose, or eyes. Wash your hands often and try not to touch your face unless you have just washed your hands.
- Use bar or liquid soap and water and wash for at least 20 seconds.
- If soap and water are not available, use an alcohol-based hand sanitizer with at least 60% alcohol.

2. Who is at higher risk for serious illness from COVID-19?

People aged 65 years and older
People with a serious chronic medical condition such as:
- Diabetes
- Heart disease
- Lung disease (e.g. lymphangioleiomyomatosis [LAM])
- Hypertension
Individuals on immunosuppressants (see below)

3. What should people at higher risk of serious illness from COVID-19 do?

The CDC recommends contacting your healthcare provider to ask about obtaining extra necessary medications* and supplies in case of an outbreak of COVID-19 in your local community, which may require you to stay home for a prolonged period. You may want to consider a mail order vendor if you cannot obtain extra medication.
Keep away from others who are sick.
Put distance between yourself and other people outside of your home.
Stay at least 6 feet (about 2 arms’ length) from other people.
Avoid touching your eyes, nose, and mouth with unwashed hands.
Wash hands often with soap and water, especially after you have been in a public place, or after blowing your nose, cough, or sneezing.
If an outbreak occurs in your community (e.g. workplace, school, church), stay home as much as possible and follow any specific recommendations made by local authorities.

*FDA DRUG SHORTAGES: The TSC Alliance is not aware of any seizure medication shortages caused by COVID-19. Refer to the FDA Drug Shortage website for current information.

4. Should I wear a facemask to prevent contracting COVID-19?

The CDC recommends that everyone 2 years and older wear a cloth face covering that covers their nose and mouth when out in public. Wearing a mask is intended to PREVENT the spread of the virus if someone is infected but does not have symptoms.
Anyone who has trouble breathing or unable to remove the face covering without assistance is exempt from this recommendation.

5. Is it safe for me or my loved one to go to work or school?

Refer to COVID-19 updates from your local government websites for guidance. Continue to maintain physical distancing of at least 6 feet between yourself and other people, wear a facemask, and wash your hands often with soap and water. For reference see the CDC’s guidance on Returning to Work.
The CDC has a school decision-making tool to help you assess your child’s risk of returning to school.
Contact your local school’s administration to see what steps they may be taking to keep students healthy. For reference, see the CDC’s Checklists for In-Person Classes and Virtual or At-Home Learning.

6. Is it safe for me or my loved one to travel by car or airplane?

The CDC recommends limiting non-essential travel as much as possible. In the absence of symptoms or recent exposures, it is safe for you and family members you live with to be in a car together. Be careful to maintain good hand hygiene and distancing at rest stops along the way and take care to avoid large crowds at restaurants or pit stops. Be particularly vigilant about hand hygiene before entering your vehicle. Try to limit public transportation such as airplanes, buses or trains to essential trips while following strict hand hygiene and social distancing. Please refer to the CDC and Johns Hopkins maps for the latest information on affected areas.

7. Is it safe for me or my loved one to continue going to clinic appointments?

Each institution/clinic has specific rules and recommendations regarding clinic visits. Many are conducting telehealth visits when possible to protect the clinic staff’s and your health. If you have questions or concerns about an upcoming clinic appointment, we recommend contacting your TSC Clinic for further instructions.
Please refer to the CDC and Johns Hopkins maps for the latest information on affected areas.

QUESTIONS ABOUT COVID-19 AND TSC (May 12, 2020)

1. Does TSC put me at greater risk for COVID-19?

The sample size is limited. Based on a handful of reported infections in patients from a small number of large TSC Clinics in the United States and abroad, there isn’t any evidence currently that having TSC puts one at greater risk for COVID-19. We continue to monitor for new reported cases and will update the TSC community should infection or complication rates change.

2. Does everolimus/sirolimus put me at greater risk for COVID-19?

There is no evidence yet. More studies needed to answer this question.

3. Should I keep taking everolimus/sirolimus for epilepsy or SEGA?

The current position from TSC care providers is to continue taking everolimus/sirolimus for seizures or SEGA. If you develop COVID-19, you would want to have a discussion with your doctor to decide if you should suspend your treatment for a period of time.

4. What happens if I develop COVID-19?

The presence of TSC will probably complicate the course of the illness because of potential involvement of other organs, TAND, and current medications you are taking. This will require a conversation with your primary care doctor and a TSC expert at a regional TSC center, if possible.

5. Can COVID-19 make my seizures worse?

If you have a high fever, are receiving antibiotics, or are unable to take your seizure medications, your seizures may worsen.

6. Will COVID-19 affect SEGA growth?

There is no evidence that COVID-19 infection will cause SEGA to start growing or to grow faster.

7. Will COVID-19 affect autism?

There is no data yet showing that COVID-19 infection will worsen autism or intellectual disability; however, those with an infection may develop a febrile or systemic illness, which can make some of those symptoms worse.

8. For those who are taking immunosuppressants should there be any concerns about taking the vaccine when it becomes available?

It depends on how the vaccine is generated, e.g., live attenuated, inactivated, or recombinant, or a combination antigen/antibody. There are different vaccine strategies – so we need to wait and see which one(s) will be deployed in the United States.

9. Should people who are on immunosuppressant drugs self-isolate even when stay-at-home orders are lifted?

We recommend people with TSC continue practicing safe social distancing when possible.

10. Do any of the treatments proposed for COVID-19 interact with antiepileptic medications or mTOR inhibitors?

There is a possibility that some anti-seizure drugs such as phenytoin (Dilantin), carbamazepine (Tegretol), phenobarbital, and primodone may activate liver enzymes that metabolize certain drugs currently in trials to treat COVID-19 such as remdesivir and rotinivir. Thus, if you are taking any of these AEDs, the level of remdesivir in the body may be lower. Further studies will be needed to define pharmacological interactions between mTORi and COVID-19 anti-viral agents and mTOR inhibitor.

USE OF AN ORAL IMMUNOSUPPRESSANT DRUG
(e.g. Afinitor®, Rapamune®, Zortress®, everolimus, sirolimus, Acthar Gel®, steroids)

I have LAM, should I stop taking sirolimus (or other mTOR inhibitor drugs)?
Whether being on mTOR inhibitors, such as sirolimus or everolimus, increases the risk of complications from COVID-19 is not known. Given the potential for lung function decline of sirolimus, pulmonologists strongly recommend that people with LAM do not stop taking sirolimus as a precautionary measure during COVID-19. Certain circumstances, such as active infection with COVID-19, may necessitate a dose reduction or interruption in sirolimus use. In such circumstances, the decision to change dosing or hold sirolimus should be made on an individual basis in close consultation with your LAM physician.
My child or I am taking Afinitor® (or other mTOR inhibitor drug). Do I stop it?
Based on current CDC statements and how recent influenza outbreaks like the H1N1 in 2009 were managed, it’s recommended to stay on drug unless your child/you or an immediate family or close contact (e.g. schoolmate or work colleague) is diagnosed with COVID-19. Local school/community authorities who provide different recommendations specific to the community/region should also be followed. Consult with your doctor for further guidance.
My child is taking Acthar Gel®. Should I continue to administer?
If taking Acthar Gel or steroids for infantile spasms, do not discontinue without discussing with your healthcare provider.

RESOURCES AND REFERENCES

American Thoracic Society Patient Information Sheet

The CDC: Coronavirus Disease 2019 (COVID-19)

Johns Hopkins COVID-19 Global Cases Map

Cystic Fibrosis Foundation

The LAM Foundation: COVID-19 Updates for the LAM Community

MEDICAL REVIEW NOTE

These FAQs were reviewed and approved by Peter Crino, MD, PhD, University of Maryland School of Medicine; Darcy Krueger, MD, PhD, Cincinnati Children’s Hospital Medical Center; and Mustafa Sahin, MD, PhD, Boston Children’s Hospital.

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