Results:
Of the 73 survey respondents, 10 patients experienced COVID-19 infection
(13.7%) [Table 1]. Seven of these ten patients (70%) reported
germline SBDS mutations. The median age of the COVID-19 positive
patients at time was diagnosis was 16.5 years (range 1-37 years). Of the
three patients who both developed COVID-19 infection and received a
COVID-19 vaccine, all of them received the vaccine following COVID-19
infection, two to six months following infection (median 5 months). None
of them experienced serious vaccine-related adverse events. All
vaccinated patients received two mRNA vaccine doses. All patients in the
SDS Registry who developed COVID-19 were symptomatic of infection with
the most common symptoms being respiratory, such as congestion or cough
(70%), or fever (60%). The median duration of symptoms was 4.5 days
(range 2-100 days; excluding an outlier of 100 days, range 4-14 days).
Only one of ten patients required hospitalization, and this patient was
one year-old (PT-6). She was hospitalized two days after symptom onset
and was hospitalized for four days. She did not require supplemental
oxygen, noninvasive positive-pressure ventilation, or intubation and
mechanical ventilation. She did not require admission to the intensive
care unit.
Of the survey respondents who developed COVID-19 infection, none of them
reported having a pre-existing condition other than SDS. Pre-existing
conditions listed in the survey included hypertension, diabetes
mellitus, asthma, and routine need for immune globulin (IVIG).
Respondents were able to report other conditions that were not listed.
No patients who developed COVID-19 infection reported a history of
tobacco use or vaping. Six of them (60%) reported regularly taking
pancreatic enzymes, and two of them (20%) reported regularly
administering granulocyte colony-stimulating factor (G-CSF) prior to
COVID infection (PT-8 and PT-9). One patient reported a history of a
hematopoietic stem cell transplant over 15 years ago (PT-4).
Survey respondents were asked if they received any of the following
COVID-19 infection treatments: remdesivir,
hydroxychloroquine/chloroquine, azithromycin, anakinra, tocilizumab,
corticosteroids, immune globulin, or the investigational COVID-19
monoclonal antibody, bamlanivimab. One patient (PT-10) received
remdesivir, and one patient (PT-4) received systemic corticosteroids.
One patient (PT-5) developed “COVID toes” – swelling, discoloration,
and/or pain of the toes associated with COVID-19 – and was treated with
aspirin and topical corticosteroids. She also reported the longest
duration of symptoms out of all respondents, 100 days. No patients
required pRBC or platelet transfusion during COVID-19 infection, or new
administration of G-CSF. No patients developed a venous thromboembolism,
multisystem inflammatory syndrome in adults (MIS-A), or children
(MIS-C).
In the cohort of SDS registry patients described, most reported having a
short duration of symptoms that did not require hospitalization or
result in serious virus-related complications. Although the Centers for
Disease Control and Prevention (CDC) recommends receiving the COVID-19
vaccine even after developing COVID-19 infection, only 30% of patients
with SDS and COVID-19 infection subsequently received the vaccine; three
of whom were ineligible to receive the vaccine at the time of survey due
to age less than 12. Of the 63 survey respondents who had not developed
COVID-19 infection, 18 (28.6%) of them received at least one dose of
the COVID-19 vaccine; 15 of whom (83.3%) received two doses. Of the 18
respondents who received at least one COVID-19 vaccine dose, arm pain
was the most common side effect (66.7%), followed by fatigue (50%),
fever and/or chills (38.9%), and muscle aches (33.3%). Other side
effects listed in the survey were rash and anaphylaxis; no respondents
reported experiencing these symptoms. Five of 18 recipients (27.8%)
reported experiencing no side effects. Thirteen (72.2%) of 18
vaccinated respondents received the Pfizer vaccine while the remainder
received the Moderna vaccine.