31 October 2022 — Our immune systems serve as our natural defense mechanism that helps us fight infections. But some individuals are born with problems in their immune system with more than 400 types of immunodeficiencies or Inborn Errors of Immunity (IEI) that have been identified. Some IEIs cause problems at birth while others manifest later in life.
People with IEI are likely to become very sick from infections.They are part of the general grouping of immunocompromised individuals who must be vaccinated and provided with additional protection compared to the general population.
Episode #118 of the Stop COVID Deaths Webinar Series took a closer look at the IEIs to help identify these patients and know how to refer to specialists who can help manage them; and discuss the effects of COVID-19 on patients with IEI. Recent studies in the Philippines show that there seem to be more cases of IEI in the Philippines, hence the importance of keeping a close watch on children who have severe COVID-19 symptoms.
Dr. Fatima Joanna Santos, head of the Immunology Council of the Philippine Society of Allergy Asthma and Immunology (PSAAI) said that just like other bodily systems, the immune system also ages, called immune senescence, that makes the cells less responsive to vaccination and decreases their proliferative capacity. They also become less effective in making strong and specific antibody responses resulting in less antibody production after a new vaccination or infection.
She noted that changes in our innate immune system somehow create a persistent inflammation and may lead to a more severe course especially in the presence of other pre-existing comorbidities like heart or lung disease, hypertension, or diabetes.
Dr. Santos, advisor of the Philippine Organization of Patients with Primary ImmunoDeficiency (PHILPOP), cited studies showing that in most countries, males were more prone to develop symptomatic SARS COV2 infection because of the different levels of inflammatory mediators. Women are known to mount stronger immune responses against viruses and vaccines, and exhibit superior immune mediated tissue repair capacities.
Dr. Santos presented the following warning signs which will make one suspect IEIs:
She pointed out that if IEIs are not diagnosed on time, 88,760 Filipinos will miss out on the following: better management of associated conditions like autoimmune diseases and cancer; infection prevention through vaccines (including COVID-19) and prophylactic antimicrobials; and blood products, genetic counseling, and support groups. The two patients whose experiences were shared by Harris Baraguir and Katrina Villareal during the webinar are both members of PHILPOP, an active support group.
The risk factors, according to Dr. Santos, are increased lethality with each decade; co-morbidities (chronic lung/heart disease), obesity, diabetes, hypertension that may lead to more severe course; and being male as males are more symptomatic and exhibit less immune response than females.
Dr. Santos warned that a breach in the immune system can mean serious consequences. A high index of suspicion plus basic laboratory tests are keys to IEI recognition and timely referral to the immunologists that will impact on patient mortality and morbidity.
The combined understanding of mechanisms and the effect of COVID-19 that can lead to better prioritization of resources for specific risk groups are keys to optimal therapeutic approaches in dealing with infections among high risk patients. The optimal management of infection can significantly improve survival
and quality of life among patients with IEI.
Cynthia M. Villamor
