The Pfizer/BioNTech and the Moderna vaccines use messenger RNA (mRNA) technology. The vaccines do not contain viruses or protein, instead they contain a small piece of viral genetic material that instruct our cells to produce a portion of the spike protein. The Janssen and the Oxford/AstraZeneca vaccines use a viral vector approach, where a portion of the SARS-CoV-2 gene that is responsible for making the spike protein is inserted into a genetically-engineered harmless human virus that enables the human body to temporarily make the spike protein. The end result from both of these approaches is a priming of our immune system to where it is better prepared to protect us when faced with the real virus.

The Pfizer/BioNTech, Moderna, and Oxford/AstraZeneca vaccines require two doses to obtain maximal benefit, while the Janssen vaccine is a single shot. The Pfizer/BioNTech vaccine requires a second dose 21 days after the first dose, the Moderna vaccine requires a second dose 28 days after the first dose, and the Oxford/AstraZeneca vaccine requires a second dose 4-12 weeks after the first dose.

Although even one dose of the vaccine confers some protection against COVID-19, both doses are needed to obtain maximal sustained protection. As such, it is imperative that patients take both doses of the vaccine.

Neither of the above-mentioned vaccines are live vaccines. As such, there is no risk of acquiring COVID-19 infection from vaccination.

The Pfizer/BioNTech and the Moderna vaccines have a greater than 90% efficacy in providing protection against COVID-19. The Janssen vaccine is roughly 70% effective at preventing moderate/severe COVID-19, while the Oxford/AstraZeneca vaccine is approximately 60% effective in preventing COVID-19 infections.

It is not possible to compare the efficacy of different vaccines without head-to-head clinical trials. Furthermore, the clinical trials for these vaccines occurred in different geographic regions and at different time points with varying incidence of COVID-19, which could explain the differences in the reported efficacy among these products. LAM patients should take the earliest available vaccine that is offered to them.

The most common side effects from the COVID-19 vaccines are local reactions such as injection site soreness, pain and swelling. Other common side effects include (in decreasing order of frequency) fatigue, headache, chills, joint pains and fever. These reactions are generally worse after the second dose than the first dose, and worse in younger subjects as opposed to people ≥55 years of age. The majority of these side effects are mild-to-moderate in nature and self-limited with a resolution time of 1-2 days. Over-the-counter medications such as Tylenol (acetaminophen) and NSAIDs (e.g., ibuprofen) may be needed for symptom relief in some cases and are OK to use. Patients with history of serious allergic reactions should discuss the safety of vaccination with their healthcare providers. It is possible that with more widespread vaccination, some other unexpected side effects or serious adverse reactions to the COVID-19 vaccines may occur, however, we expect these to be infrequent. Overall, the COVID-19 vaccines appear to be both safe and effective for the vast majority of recipients.

The COVID-19 vaccination is being rolled out in a staggered fashion with the initial supplies being provided to healthcare personnel and people residing in long-term care facilities. The next group of people being prioritized for vaccination includes teachers, first responders, other essential workers outside of health care, people ≥65 years of age and people at risk of serious complications from COVID-19 such as people with underlying health conditions. LAM patients, by virtue of their lung disease, will fall in this category. While the exact timelines for vaccine availability for this group are constantly evolving depending upon the available supplies and authorization of more candidate vaccines, it is likely to be available for most LAM patients by late spring/early summer 2021.

The safety and efficacy of COVID-19 vaccination has not been well studied in patients taking immunosuppressive medications such as mTOR inhibitors. Although there is no direct evidence, there is no reason to think that the side effect profile from vaccination will be different in patients taking mTOR inhibitors versus patients not on these drugs. The major concern with vaccination in patients on mTOR inhibitors pertains to their efficacy, i.e., will the mTOR inhibitors impair the immune response to the vaccination leading to suboptimal protection? While the exact answer to this question is not known, it is likely that patients on mTOR inhibitors will mount at least some response to vaccination and derive at least partial immunity from COVID-19 as opposed to the prospect of no protection without vaccination. As such, we recommend that LAM patients should take the COVID-19 vaccine regardless of their use of mTOR inhibitors.

Previous infection with COVID-19 provides you some natural immunity against re-infection. However, the efficacy and duration of natural immunity to prevent COVID-19 reinfection is not well understood. At the present time, we suggest getting vaccinated for COVID-19 even if you have previously been infected, similar to the recommendation for Shingles.

It is common for viruses to mutate and evolve over time. We do not know if the currently available vaccines will provide the same degree of protection against some of the newer variants/strains of SARS-CoV-2. However, the vaccines are likely to confer at least some protection against the variants. As such, the best course of action is to get vaccinated with the earliest vaccine available to you.

Yes. While the availability of COVID-19 vaccination represents a landmark moment in our quest to quell the pandemic, the staggered roll out of the vaccine and the time it will take to mount an effective response to vaccination (~2 weeks following the second dose) implies that we need to continue to take all the necessary precautions such as hand hygiene, social distancing, and mask use in order to maximize our chances of staying safe and controlling community spread.

What are the typical symptoms of COVID-19?

The typical symptoms of COVID-19 include fever, body aches, cough and shortness of breath. Other symptoms of COVID-19 may include headache, fatigue, sore throat, and loss of taste or smell.

How can I tell the difference between my normal LAM symptoms and symptoms from COVID-19?

It may be difficult in some situations to distinguish your LAM-related symptoms, such as shortness of breath from COVID-19 symptoms. Some clues that might help include the presence of new symptoms such as body/muscle aches, fever, sore throat, headache, and loss of taste or smell. Increased intensity of your chronic symptoms such as shortness of breath may suggest COVID-19. If in doubt, contact your healthcare provider to discuss.

What should I do if I have symptoms suggestive of COVID-19?

Contact your LAM physician if you have symptoms suggestive of COVID-19. For the majority of the patients (probably >80%), COVID-19 causes a mild-moderate flu-like illness that does not require hospitalization.

Seek immediate medical attention if you have any of the following: worsening shortness of breath, persistent chest pain, bluish lips or face, confusion, or trouble arousing. Note that this list is not all-inclusive. If in doubt, err on the side of calling and talking with your healthcare provider.

How do I get tested for COVID-19?

There are two types of testing for SARS-CoV2 (the virus that causes COVID-19) infection. The first is a test for the presence of viral RNA, or PCR testing, that is used to determine if active viral infection is present. At the present time, COVID-19 PCR testing is generally being limited to patients who have symptoms. The second type of test is an antibody serology test, to determine if you have previously been infected with SARS-CoV2. That test is being refined to be more accurate and specific, and will soon be more generally available. The hope is that this test, and viral neutralization tests that will follow, will enable us to determine which patients are immune from future infection with SARS-CoV2.

Please contact your LAM physician if you think you might have COVID-19. Depending upon your degree of symptoms and the local / state policies and test availability, they can guide you towards the next steps.

To gain control of this outbreak, widespread PCR testing, contact tracing and quarantine of the exposed and isolation of the infected is necessary. In a lot of areas, PCR testing is currently being limited to patients with moderate-severe symptoms who might require hospitalization and patients with mild symptoms are being advised to stay at home and not undergo diagnostic confirmation. This situation is likely to change over time with improved access to testing capabilities and the development of novel testing platforms.

Does sirolimus put me at increased risk of complications from COVID-19? Should I hold sirolimus until COVID-19 has passed?

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 off sirolimus, we strongly recommend that LAM patients do not stop taking sirolimus as a precautionary measure during the outbreak. 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.

Can sirolimus help prevent/treat COVID-19? Should I take sirolimus to prevent/reduce my chances of catching COVID-19?

The role of sirolimus as a preventative or treatment agent in COVID-19 is not clear and is the subject of active investigation. The decision to start sirolimus should be based on indications to treat LAM, and LAM patients should not start taking sirolimus solely for the purpose of preventing or reducing their chances of catching COVID-19.

Should I take hydroxychloroquine to prevent/reduce my chances of catching COVID-19?

The efficacy of hydroxychloroquine to prevent or treat COVID-19 is not clear and the drug has significant side effects. Given the lack of proven efficacy and the prospect of potential adverse effects, multiple professional societies and The LAM Foundation recommend against the prophylactic use of hydroxychloroquine to prevent COVID-19 outside the setting of a clinical trial. The same advice applies to the use of hydroxychloroquine as a treatment for COVID-19 — use outside of a clinical trial is not recommended.

Is it safe for me to continue to use a nebulizer at home?

A nebulizer can generate aerosols making it easier for the COVID-19 virus to spread. As long as you do not have symptoms of COVID-19, it is safe for you to continue to use your home nebulizer but be careful not to expose others who may be at risk for infection. Metered dose inhalers are not considered aerosol generating and are preferred. If you have symptoms suggestive of COVID-19, please talk to your LAM physician to discuss this question.

Is it unsafe for me to take painkillers such as ibuprofen?

Non-steroidal anti-inflammatory drugs (NSAIDs) like ibuprofen or naproxen pose theoretical risks to those with COVID-19. Although there is no direct evidence to support this idea, we are avoiding their use in hospitalized patients with the disease. There is no evidence that taking NSAIDs increases the risk of catching COVID-19. Please do not alter your regimen or stop your medications before consulting with your healthcare provider.

Is it unsafe for me to take Angiotensin Converting Enzyme (ACE) inhibitors such as lisinopril?

Angiotensin Converting Enzyme (ACE) inhibitors such as lisinopril pose theoretical risks to those with COVID-19, but there is no direct evidence to support this idea. There is no evidence that taking ACE inhibitors increases the risk of catching COVID-19. Please do not alter your regimen or stop your medications before consulting with your healthcare provider.

Do I need to purchase a pulse oximeter?

Pulse oximeters are used to monitor oxygen saturation and are most helpful in titrating the amount of oxygen in patients on supplemental oxygen. There is no proven role of monitoring oxygen saturation by pulse oximeter as an indicator of contracting COVID-19, and because of inherent variability in the test, it is as likely to frighten as inform. For LAM patients who have symptoms of COVID-19 and who have been advised to stay at home, periodic monitoring of oxygen saturations by a pulse oximeter may be useful to make decisions regarding the timing to seek immediate medical attention.

Is it safe to travel for my routine LAM clinic or other healthcare appointments?

The risk of traveling for clinic visits is dependent upon the local spread of COVID-19 in your community. In addition, each institution/clinic has specific rules and recommendations regarding clinic visits, and many are continuing to conduct visits remotely using Telehealth. Please discuss the timing and format for upcoming appointments with your healthcare provider. If your disease course appears stable, chances are that your clinic visit can be postponed or conducted remotely via Telehealth.

Should I postpone my routine bloodwork such as sirolimus/everolimus monitoring labs?

The decision to modify your bloodwork routine should be made in close consultation with your healthcare provider taking into account the local prevalence of COVID-19. For some patients who have been on a stable dose of sirolimus/everolimus for a while, it may be appropriate to reschedule routine monitoring for a later date. For patients who are newly starting sirolimus/everolimus or have had medication changes that are known to interact with sirolimus/everolimus, the benefits of monitoring may outweigh the risks. Please discuss the timing and frequency of individual bloodwork monitoring with your LAM physician.

Is it safe for me to perform pulmonary function tests (PFTs)?

Similar to clinic visits, most institution/clinics are following specific guidance with regards to reopening PFT laboratories for routine visits. Those with the most urgent needs, such as preoperative patients or post-transplant patients, will be the first to be offered PFTs. For patients who are due for a routine PFT but are doing well, it may be appropriate to postpone and reschedule to a later date. Some patients may be able to use home spirometry to monitor their disease. Please discuss the timing and format (home spirometry vs. PFTs) of your upcoming PFT assessments with your LAM physician.

Should I continue going to pulmonary rehabilitation visits?

Many pulmonary rehabilitation programs remain closed at the time of this writing. Please discuss continued participation in a pulmonary rehabilitation program with your rehab team and your LAM physician. It may be possible to replace in-person pulmonary rehab with home-based exercise regimens until safe reopening of rehab facilities is possible. Download an at home exercise program written and published by Dr. Mary Beth Brown, PT, PhD and Morgan Kelly, PT, DPT from the University of Washington here.

Can I continue my clinical trial appointments as scheduled?

Safety of clinical trial participants takes precedence over maintaining a prespecified trial schedule. Please contact your study team to devise an individualized plan for your study activities, and to discuss alternatives such as remote/tele visits, home-based testing such as spirometry, utilizing local testing facilities in lieu of travel to study sites, and advice regarding continuation of study drug(s).

I am a lung transplant recipient. What steps should I take to protect myself?

Lung transplant recipients may be at higher risk of complications from COVID-19. You should continue to stay sheltered and avoid unnecessary travel or contact. Stay in close communication with your transplant team regarding further guidance on your medication and disease monitoring.

I am on the lung transplant waiting list. What steps should I take?

Patients on the lung transplant waiting list belong in the high-risk category and should continue to stay at home as much as possible. Stay in close communication with your transplant team to obtain individualized guidance with regard to further testing and transplant evaluations. Depending upon the local spread of COVID-19 in your community and your disease severity, the timing of your transplant may be altered.

Is it safe for me to go to work?

Follow your state / local policies and above-mentioned tiered guidance when making this decision. In general, working remotely, if possible, is the safest strategy until the virus is under control in your community or a vaccine has been developed and implemented. The answer to this question is also dependent upon other factors such your lung function, your underlying health conditions, and the ability to distance at work. Please discuss return to work strategies with your healthcare provider.

What if I can’t work remotely?

Certain jobs may not be conducive to remote work. Please discuss with your employer to ensure adequate safety precautions are in place before going to work. Continue to follow hand hygiene, avoiding touching your face, and social distancing principles to prevent/reduce your chances of acquiring COVID-19. Contact your healthcare provider if you need a medical note to excuse you from work.

I work in a healthcare setting. What should I do?

Depending upon your profession, working in a healthcare setting may increase your chances of coming in contact with people who have COVID-19. Hospitals and other healthcare places have instituted rigorous cleaning and social distancing policies to limit the spread of the virus to healthcare personnel. Follow your institutional policies with regard to the appropriate use of personal protective equipment (PPE). Discuss your individual circumstances with your healthcare provider to make decisions regarding your ability to safely go back to work.

Should I wear a mask in public?

The current CDC guidance recommends masks when going out in public, especially when venturing into confined spaces where proximity to others may be difficult to avoid such as grocery stores and pharmacies. Cloth face masks are appropriate for this purpose. Check the CDC website for information on the best practices of cloth face masks here. Special masks such as N95 and surgical masks are in short supply and should be reserved for use by medical professionals. Wearing a mask might worsen some LAM patients symptoms such as shortness of breath. This effect is likely effort dependent and more likely to happen with more vigorous activities such as exercise. Getting acclimated to mask use in the comfort of your home and slowly escalating the use outside your home might help overcome this for a subset of patients. For others, it may be ok to not wear a mask when outside in well-ventilated areas, for example while taking a walk outside. However, as much as possible, you should try and wear a mask in confined spaces such as grocery stores, or limit your excursions to such places. Slowing your pace at these places may also help. Please remember that the biggest utility of masks is to reduce the chances of spreading COVID-19 to others. Wearing a mask is not a substitute for the preventative steps such as hand hygiene and social distancing as outlined above.

Should I stockpile on medicines and supplies?

LAM patients in the moderate-high risk category should have access to enough medicine and supplies that allows them to shelter in their homes for a few weeks, if needed. For all LAM patients, it is prudent to plan ahead and limit the overall number of trips to grocery stores or pharmacies. Follow the CDC guidelines to protect yourself while shopping here. There is no shortage of sirolimus/everolimus, or for any of the commonly used inhalers by the LAM patients, at the current time. A list of the current drug shortages can be accessed here.

Can I go outside during the pandemic?

It safe for LAM patients to go out on walks in well-ventilated areas. Follow social distancing guidelines when going on walks. Avoid touching surfaces such as playground equipment. Wash your hands after coming home.

Is it safe to order take-out and have groceries delivered?

Ordering food via take-out is safer than dining in a restaurant and delivering groceries to your home is safer than a trip to the grocery store. Follow social distancing guidance during these interactions. Paying by credit card in advance may limit physical interaction. Remember to wash your hands with soap and water after handling outside items such as food and grocery deliveries.

Should I wipe down everything in packages and deliveries?

Although the risk of transmission by handling packages and deliveries is very low, it is a good idea to wipe surfaces and outside objects with regular household cleaning sprays or wipes. The risk can be further reduced by leaving non-perishable items outside the home (such as in the garage) for 2-3 days before bringing them inside. Remember to wash your hands with soap and water after handling outside packages and deliveries.

Can I 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 and limit public transportation such as airplanes, buses or trains to essential trips while following strict hand hygiene and social distancing.

What has been the experience of LAM patients with COVID-19?

We are only aware of a few LAM patients who have been infected with COVID-19. This low incidence is likely due to the overall rarity of LAM. Overall, these patients have done well and recovered without requiring prolonged hospital stays. The long-term effects of COVID-19 on lung function in patients with LAM are not clear. We are gathering information about LAM patients who get diagnosed with COVID-19 in collaboration with the Tuberous Sclerosis Alliance Natural History Database. Please contact Jo Anne Nakagawa at jnakagawa@tsalliance.org or The LAM Foundation, if you have been diagnosed with COVID-19 and would like to contribute to this endeavor.

This pandemic has caused a lot of anxiety for my family and me. What can I do?

The CDC has posted guidance to address the anxiety and stress that are common in many individuals due to the uncertainty and risk posed by COVID-19 that can be found here. The LAM Foundation recently hosted two wellness webinars that can be accessed here, and the staff is always available to lend a listening ear and think through potential stress-relieving strategies.

I’m worried the person I live with could get infected. What should I do?

Hand hygiene, social distancing, and following the preventative steps outlined above is the best strategy to reduce your chances of acquiring and spreading COVID-19. It is important to impress on all members of the household that their adherence to these principles protects everyone in the home. Some front-line workers have been spending the bulk of their time in a room that is separate from the rest of the family during periods when they are in direct patient contact, and for up to a week afterward. Others have been living apart. These more extreme measures are isolating and add to the stress of COVID-19 mitigation, and each family must make their own strategy that balances risk, mental health and quality of life. For caregivers, those who see an occasional COVID-19 patient with full personal protective equipment (PPE) under well controlled circumstances might adopt a more modest, ‘strip and shower approach’ upon returning home, whereas those assigned to a large, overwhelmed COVID-19 unit might decide to stay in a hotel while on service. By analogy, for LAM patients, those with mild lung function impairment who are living with a COVID-19 caregiver with limited exposure might decide on the more liberal approach above, while those in the high risk category living with a healthcare provider who interacts with COVID-19 patients on a more regular basis might opt for the more extreme approach. The trade-off between risk and quality of life is a spectrum, and individual situations differ widely. The CDC offers guidance for those living with someone who has COVID-19 that can be found here.

We have young children at our home. What steps can we take to protect them? How can we protect ourselves from getting infected?

Although serious illness has been reported in a few cases, the majority of the kids do not tend to become ill from COVID-19. However, children may spread the virus to other members of the household. Parents should become familiar with and educate their children about the principles of hand hygiene and social distancing, as well as other measures to prevent the spread of infection such as regular use of masks, frequent use of hand sanitizer, avoiding group activities which are not conducive to social distancing such as playdates, cafetaria, library, or gym visits. The CDC offers helpful guidance on talking with children about COVID-19 that can be found here.

Depending upon the local prevalence and occurrence of outbreaks of COVID-19, schools may remain closed for an indefinite period. In the meantime, schools are providing opportunities for virtual and distance learning. As schools open, parents should emphasize the importance of hand hygiene and social distancing with their children. Teachers are busy devising learning strategies that are conducive to social distancing, such as staggered schedules, and avoiding crowded spaces like cafeterias, gyms, or libraries. Children should be encouraged to wear a cloth facemask as much as possible during school and should wash their hands immediately after returning from school. Clothes should be washed with the warmest appropriate temperature setting. Dryer heat (usually >120°F) is also very effective at killing the virus. Frequently touched surfaces in the home should be disinfected often. Social gatherings with other kids should be limited to essential school activities. Parents should reassure their children we are safe when we ‘follow the rules’, that the pandemic will have an end, and the life will return to normal.

What is herd immunity?

Herd immunity is the indirect protection from infection that occurs when the majority of the population is immune to a communicable disease. For example, if 80% of a population is immune to COVID-19, 4 out of 5 subjects who encounter an infected individual won’t get sick and the disease is prevented from spreading in the exponential manner that occurred in this first wave. Current estimates suggest that at least 70% of the population will need to be immune in order to confer herd immunity against COVID-19. Development and widespread implementation of a vaccine is the safest way to establish herd immunity.

When will a vaccine to prevent COVID-19 be ready?

Multiple international efforts are underway to develop a vaccine against COVID-19. However, candidate vaccines will need to be tested in humans before widespread implementation. While the exact timelines are not clear, widespread availability of a COVID-19 vaccine is unlikely before the end of 2020.

What should I do if I haven’t heard from my LAM physician regarding advice to protect myself from COVID-19?

Most pulmonologists, including LAM Clinic directors, have hundreds of patients under their care and are also assisting their respective institutions with responding to COVID-19. While it may not be possible for LAM physicians to contact every patient, given a little time to respond, most will be available to answer your questions and you should not hesitate to contact them. The LAM Foundation will periodically release COVID-19 guidance documents to try to address patient queries/concerns. The LAM Foundation is also planning to host informational webinars to disseminate key information pertaining to COVID-19 and its implications for the LAM community.

What steps are being taken by The LAM Foundation in response to COVID-19?

The LAM Foundation is committed to the safety and well-being of all LAM patients, and will continue to work tirelessly to advocate for the LAM community in these trying times. Resources provided by the Foundation include:

1. The LAM Foundation has created a monthly education series called LAMposium In Your Living Room. The first session was very successful with greater than 200 attendees. For more information on upcoming LAMposiums In Your Living Room click here.
2. Staff at the Foundation are available for calls and inquiries.
3. Several COVID-related videos and guidance documents are posted here.
4. The LAM Foundation has partnered with the Tuberous Sclerosis Alliance to produce webinars, meetings, and capture the natural history data in patients who are diagnosed with COVID-19.