COVID-19 Largely Mild in 4 LOPD Patients With Pulmonary Weakness

Yedida Y Bogachkov PhD avatar

by Yedida Y Bogachkov PhD |

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Infection by the virus that causes COVID-19 led to mild-to-moderate symptoms in four people with late-onset Pompe disease (LOPD) and pulmonary involvement, despite the increased risk of severe complications in such cases, scientists reported.

“COVID-19 infection can result in a benign course in some patients with LOPD. However, individuals with LOPD remain at high risk and should receive COVID-19 vaccinations and exercise precautions to avoid exposure to COVID-19 infection,” the investigators wrote.

Their report, “COVID-19 Infection in Patients with Late-onset Pompe Disease,” was published in the journal Muscle and Nerve.

The COVID-19 pandemic that started in December 2019 has infected around 269 million people worldwide, and led to about 5.3 million deaths. People with pre-existing medical conditions (especially those affecting the respiratory and cardiovascular systems), a weakened immune system, or who are not vaccinated against the virus have been hospitalized at particularly high rates.

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How the SARS-CoV2 virus that causes COVID affects those with neuromuscular disorders such as LOPD is not entirely understood. Respiratory muscle weakness, however, is known in some LOPD cases.

“Because morbidity and mortality in LOPD are primarily driven by respiratory involvement, we examined the impact of COVID-19 on these patients,” the University of California, Irvine, researchers wrote.

Their report detailed four confirmed cases of COVID-19 in LOPD patients in California. All were white men on nightly non-invasive breathing support due to diaphragmatic insufficiency — dysfunction of the diaphragm, the dome-shaped muscle that expands and contracts in breathing.

The patients’ forced vital capacity (FVC) — how much air a person can exhale forcibly after a deep breath — ranged from 47%  to 86% (normal FVC is at least 80%).

Reported COVID-19 symptoms were fever, fatigue, muscle pain, diarrhea, cough, nasal congestion, loss of sense of smell, loss of appetite, chills, and difficulty breathing. These symptoms lasted for six days in two patients and for 15 and 16 days in the other two.

The first patient was 42 years old and had a pre-COVID FVC under 50%, indicative of severe disease. Diagnosed with Pompe four years earlier, he alone had COVID symptoms that included mild shortness of breath (dyspnea). The second patient was the oldest at 71, with a disease duration of more than 20 years and no longer able to walk unassisted but having a near-normal pre-COVID FVC of 77%.

Patient three, 52 years old, had a high pre-COVID FVC of 86% and a disease duration of five years, while the fourth and youngest patient, 37, had been diagnosed six years ago and had a pre-COVID FVC of 76%.

One of the two men with FVCs of “around 75%” had low inspiratory pressure — the pressure measured during inhalation — and was documented as having extremely low diaphragmatic pressure, indicating weakness of the diaphragm, the report noted.

High-dose corticosteroids were given to the first and fourth patient, and that youngest man was also treated with COVID-19 monoclonal antibodies. The other two were treated only with acetaminophen — a commonly used pain reliever and fever reducer.

Three of these four men fully recovered from all COVID symptoms over one to three months of follow-up. The oldest patient, treated with acetaminophen, reported persistent Pompe-related weakness and fatigue following COVID-19 infection.

No notable post-COVID changes in FVC were recorded in three patients. The youngest did not undergo a FVC test following his infection; he was also the only patient followed for one month.

Although these four men had pre-existing LOPD with pulmonary involvement and were at risk for severe COVID-19 infection, they “all had mild/moderate disease and did not have additional pulmonary complications, hospitalization, or significant worsening of their neurological illnesses,” the investigators wrote.

These cases “demonstrate that a benign course may occur in some neuromuscular patients, who may not experience any persisting post-COVID symptoms,” they added.

“It is possible that early institution of corticosteroids and treatment with monoclonal antibodies prevented further worsening of symptoms and disease in patients 1 and 4, but this is speculative,” the team noted.

The 2021 wave of COVID infections, the scientists added, came “at a time of improved understanding of disease management, and were associated with lower morbidity and mortality. This may have played a role in the apparent benign course in our patients.”