About Primary Humoral Immunodeficiency (PI)
Nearly all of us get an infection once in a while. Perhaps it’s a minor cold, cough, or a cut that gets infected—or it may be something more serious like pneumonia.1
Most people expect to recover quickly from an infection. Our body’s immune defenses (sometimes with help from certain medicines) can usually eliminate germs that cause infection and protect us against new germs in the future. Others are born with an immune system that is without certain defenses required to fight infections. This is called primary immunodeficiency (PI).1
The 10 warning signs of primary immunodeficiency2*
Excerpted from Jeffrey Modell Foundation.
The Impact of PI
Primary immunodeficiency disease consists of more than 400 rare, chronic disorders involving a compromised or incomplete immune system. These disorders are not contagious and often present at birth (due to hereditary or genetic defects) and can impact any race, age, or gender. While some disorders impact multiple components of the immune system, others can impact only one, resulting in the inability of the body to fight infection.3
Exact prevalence is unknown but, in the United States, it is estimated that 1 in 2000 children and 1 in 600 households (or an estimated 150,000 to 360,000 people) are diagnosed with PI.4
Those suffering with PI often get one infection after another. These infections may never really improve as expected and may recur over time. In more severe forms of PI, even certain infections considered "less serious" in individuals with normal immune systems may cause serious, life-threatening infections in those with PI.1
Also, certain PIs are associated with immune system disorders such as anemia, arthritis, or autoimmune diseases. Other PIs increase the risk of cancer, stunt growth, or impact the heart, digestive tract, or the nervous system.1
Missed Work and School Days
In the 2008 Immune Deficiency Foundation National Survey of Patients, patients reported that they missed an average of 36.8 days of work or school prior to diagnosis. In the same survey, only 10% of the 1030 responding patients said they had never missed any days of school or work prior to diagnosis.5
Seeking Treatment for PI
If left untreated, PI can lead to serious health issues. If someone in your family has PI, it is important to have other family members checked for signs and symptoms and evaluated for PI as soon as possible.
This information is not intended to be a substitute for professional medical care. You should consult your family physician or pediatrician for specific information on the diagnosis, treatment, and clinical care of patients with PI.