World PI Week kicked off 22 April 2021. It joins together the efforts of organizations across the globe to promote awareness of primary immunodeficiency diseases. In this week, there are worldwide initiatives to improve the recognition, diagnosis, treatment and quality of life of people with primary immunodeficiency diseases.
Primary immunodeficiencies (PI) are a group of more than 400 rare, chronic disorders in which part of the body’s immune system is missing or malfunctions. They are not contagious but are caused by hereditary or genetic defects, and although some disorders present at birth or in early childhood, they can affect anyone regardless of age or sex. Some affect a single part of the immune system; others may affect one or more system components. Furthermore, while the diseases may differ, they all share one common feature: each result from a defect in one of the body’s normal immune system functions.
The immune system’s primary function is to protect against infection; therefore, a defect in the immune system means that the affected person is more vulnerable to infection. The infection can be in the skin, sinuses, throat, ears, lungs, brain or spinal cord, or urinary or intestinal tracts, and the increased vulnerability to infection may include repeated infections, infections that will not clear up or unusually severe infections.
This results in enduring recurrent health problems and often developing serious and debilitating illnesses. Fortunately, with proper medical care, many patients live full and independent lives.
Specific PI Diagnoses
There are more than 400 primary immunodeficiencies recognized. You can find more information here. https://primaryimmune.org/specific-pi-diagnoses
You should be suspicious if you have an infection that is:
• Severe – requires hospitalization or intravenous antibiotics
• Persistent – will not completely clear up or clears very slowly
• Unusual – caused by an uncommon organism
• Recurrent – keeps coming back
• Runs in the family – others in your family have had a similar susceptibility to infection
If any of these words describe your infection, the Immune Deficiency Foundation (IDF) recommends that you ask your physician to check for the possibility of PI.
Living with PI
Primary immunodeficiency diseases affect people in different ways, but like everyone else, individuals with primary immunodeficiency diseases need to feel a sense of accomplishment and purpose and contribute to the world around them. To best manage your life and your health, you need to educate yourself about your disease, build a collaborative relationship with your healthcare providers, and take care of yourself physically and emotionally.
Here are some factors that those with a PI should keep in mind:
• Choose a quality healthcare team.
• Take advantage of resources.
• Build strong social relationships.
• Connect with others like you.
• Maintain a positive attitude.
Living with PI and COVID-19
The first knee-jerk reaction of PI sufferers was to “batten down the hatches”, so to speak. There was no knowing how hard this unknown quantity was to hit the community. However, interestingly, the risks of severe disease and death are not markedly increased in people with PI. According to an amalgamation of international studies, mortality rates are overall comparable to those without a PI.
The majority of persons with PI should get the vaccine, but only after checking with their immunologist. Most types of PI produce a normal antibody response to the vaccine. Even those with antibody deficiencies should benefit from a slight antibody response or a T cell response, mitigating the disease’s severity.
How to get involved
Go to the following links for some inspiration on how to get involved and help spread the word about PI:
• Social Media http://www.worldpiweek.org/resources/sample-tweets-and-posts/
• Audience Specific Toolkits http://www.worldpiweek.org/get-involved/audience-specific-toolkits/