Primary immunodeficiency (PI) is a category of genetic defects and disorders that impact how the immune system functions. There are hundreds of different types of PI—over 450 and counting, with more being identified thanks to advances in genetic testing and ongoing research about these conditions.
Because PI interferes with the normal functioning of the immune system, a person with one of these conditions is at an increased risk for infections, such as viral or bacterial infections. They will also be at an increased risk of becoming more severely ill when infected and having infections that are more difficult to treat.
Below, we look at some strategies to prevent infections when managing PI.
Follow your treatment plan
As with any health condition, the most important thing a person living with PI can do is work with their healthcare provider and follow their treatment plan. Treatment for PI will depend on the type. Treating and preventing infections is a key focus of treatment, and your healthcare providers will be your best source of information about your treatment options and strategies to stay healthy.
Treatment options for PI can include:
- Immunoglobulin replacement therapy, infusions of antibodies that help the immune system function and help the body fight infections.
- Antibiotic or antiviral medications to treat or prevent viral and bacterial infections.
- Hematopoietic stem cell transplant (HSCT), a procedure that has the potential to cure certain types of PI. This is typically only recommended in cases where PI is causing severe illness or is life-threatening. This is also known as a bone marrow transplant.
- Gene therapy, treatments that alter and correct defective genes that cause specific types of PI are being studied in clinical trials.
Take precautions to prevent infections
In addition to working with a healthcare provider and following treatment as directed, people living with PI should take steps to protect themselves against infections. Here are some strategies that can help prevent infections:
Some basic strategies for preventing infections:
- Stay up to date on vaccines. Talk to your healthcare provider about what vaccines they recommend. Also talk to your providers about upcoming travel plans— additional vaccinations may be recommended when traveling internationally, depending on your destination
- Wash your hands often, especially after being out in public. Use soap and hot water and scrub for at least 20 seconds each wash. Use hand sanitizer that contains at least 60 percent alcohol if you are unable to wash your hands.
- Avoid touching your face, especially your eyes, mouth, and nose.
- Avoid people who are sick. Avoid crowded places where you are more likely to be in contact with a person who is sick, especially during times when flu and COVID-19 cases are high.
- Wear a mask if you are going out—especially if you are going to be in a crowded place. Well-fitting N95 or KN95 masks offer better protection than cloth masks or surgical masks.
- Avoid sharing food, beverages, drinking glasses, utensils, and bottles with others. Avoid contact with napkins, tissues, or other items that have been used by others.
- Follow guidelines for safe food handling and preparation. This includes washing produce and cooking meat to the recommended internal temperature. Food safety is another topic to discuss with a healthcare provider, and they may be able to provide you with guidelines.
- Wounds can also lead to infections. Talk to your healthcare providers about how to clean and bandage a wound and steps to help prevent a wound from becoming infected.
- Talk to your healthcare provider about swimming and recreational activities that involve water. People with certain types of PI will need to avoid recreational water spaces, where there is a risk of exposure to water-related illnesses.
You should also talk to your healthcare providers about what to do if you start to feel sick or have symptoms of an infection. It is important to have an emergency plan in place. This plan should include a list of symptoms to watch for, contact information for your healthcare providers, and guidelines on when to seek care and when to seek emergency medical care.
Sources: Immune Deficiency Foundation. What is PI... + 16
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