COVID-19 continues to persist in the community at a relatively low level, mainly in the form of Omicron subvariants. Omicron subvariants are highly transmissible but at this time not causing disease of worse severity than the original Omicron variant. Please be mindful of your local community’s spread of COVID-19 and continue to take all precautions such as social distancing, wearing a mask, avoiding crowded spaces, and frequent hand washing. Upgrading from cloth masks to a surgical mask or a N95-type mask is recommended, especially when coming in contact with other people in indoor settings. The following summarizes and updates key concepts related to COVID-19 that are relevant to individuals with LAM.

Outcomes following COVID-19 infection in patients with LAM

An international group of investigators compiled and analyzed data from patients with LAM around the world looking at outcomes following COVID-19 infection. This study included 91 women with LAM: 77 with Sporadic-LAM and 14 with TSC-LAM. The study was a true international representation of the LAM community and included patients from the United States, Brazil, Europe, and Japan. About half of the patients in this analysis were on an mTOR inhibitor (sirolimus or everolimus) at the time of their infection, at an average sirolimus dose of 2mg daily. Somewhat unsurprisingly, patients on mTOR inhibitors had worse lung function compared with untreated patients (patients with more severe disease tend to be prescribed mTOR inhibitors more commonly than patients with mild disease).

The major findings from this study were:

1. the overall outcomes following COVID-19 in patients with LAM were similar to the general population, with an approximately 1% risk of mortality (dying),
2. reduced diffusion capacity on pulmonary function testing was associated with an increased risk of hospitalization or the need for supplemental oxygen following COVID-19 infection, and
3. the overall outcomes were similar in patients who were taking mTOR inhibitors versus those not on mTOR inhibitors.

These data were derived prior to the widespread availability of vaccines and other antiviral treatment options, and likely represent an assessment of the complications following infection with the alpha variant (the first wave of COVID-19). While the implications of these results for the current Omicron variants are not fully known, the availability of vaccines and current treatment options should translate to improved, or at least no worse, outcomes for patients with LAM as compared with the above-mentioned data.

What to do if you have tested positive

Isolate yourself from other family members. Most patients with COVID-19 have mild disease and are able to recover at home. Take plenty of rest, stay hydrated, and use over-the-counter medications such as acetaminophen for symptom control. Inform your physician(s) about your diagnosis so they can guide you towards the best next steps taking into account your disease severity and other medical conditions. If you have a finger oximeter, it is advisable that you monitor your oxygen levels and report any significant decline (< 90% if not currently on oxygen or inability to maintain saturations > 90% on your usual oxygen flow rate).

Antivirals: The oral antiviral medication Paxlovid (nirmatrelvir plus ritonavir) has been approved by the United States Food and Drug Administration for the treatment of COVID-19. This drug is meant for the treatment of adults with mild-to-moderate COVID-19 who are at high risk for progressing to severe illness, and should be administered as soon as possible after a positive test and within 5 days of symptom onset. More information on this drug is available here and here.

It is possible that there might be potential drug-drug interactions between the Paxlovid and your other medications, so it is essential that the prescribing provider review all your medications before prescribing the drug. Notably treatment with Paxlovid can raise the blood levels of sirolimus, requiring that you either hold sirolimus or reduce the sirolimus dose for the duration that you are on Paxlovid. If you are taking sirolimus, please make sure to discuss this with your healthcare provider.

A second antiviral medication, Lagevrio (molnupiravir) has received emergency use authorization by the FDA. However, currently available evidence suggests that it may not be as effective as Paxlovid in preventing progression to severe infection. For this reason, it should only be prescribed in situations where Paxlovid is either unavailable or contraindicated.

Vaccination

Vaccination remains the best public health measure to protect people from COVID-19, slow transmission, and reduce the likelihood of new variants emerging. The newly updated (2023–2024 formula) COVID mRNA vaccine booster (both Pfizer and Moderna) offers more specific protection against Omicron subvariants. The new shots are designed to protect against XBB.1.5 and should also protect against more recent virus strains, including EG.5 and BA.2.86. If you haven’t received the updated (2023–2024 formula) booster dose, you should seriously consider getting it. More details on COVID-19 vaccinations can be found here.

Research indicates that receiving both a flu vaccine and a COVID-19 booster vaccine during the same visit is considered safe. According to a study released by the CDC, individuals who received a flu vaccine and an mRNA COVID-19 booster vaccine concurrently had a slightly higher chance of experiencing reactions such as fatigue, muscle soreness, and headaches in comparison to those who received only a COVID-19 mRNA booster vaccine. However, most of these reactions were mild and resolved quickly.

Certain immunosuppressive medications can compromise the response to vaccines, initially raising concern that individuals taking sirolimus might have an inadequate response to COVID vaccines. Somewhat surprisingly, however, a study that compared response to vaccine among individuals with LAM who were or were not taking sirolimus demonstrated that the majority of those on sirolimus mounted a response identical to that of individuals not on the drug. Preliminary studies in kidney transplant recipients taking sirolimus also support the notion that sirolimus does not impair response to COVID vaccine. Based on this available evidence, it is not recommended that sirolimus be held prior to receiving the COVID vaccine.

The future vaccination strategy for COVID-19 is unclear. It is likely that COVID-19 will stay with us and that we will likely need an annual vaccination similar to the flu shot. We will continue to closely monitor the COVID-19 situation and will provide regular updates as new developments arise.