Approximately 13,000 Canadians suffer from Primary Immunodeficiency.
Your Immune System is a vast internal network of cells, tissues and organs whose job it is to protect your body from harmful invasion by foreign bodies such as viruses, bacteria and toxins. Primary immunodeficiency (PI) refers to an immune system that is either broken or completely missing from birth. It is not acquired after birth from infection or accident. It is a genetic malfunction, unique to an individual. Early diagnosis is crucial. Untreated PI can lead to serious damage to organs, physical disabilities and, in the most severe cases, death.
There are warning signs of PI, like recurrent infections of the ears and skin, pneumonia, bronchitis and sinusitis. For some, the first infection will be serious and life- threatening—a definite red flag that there may be a problem with their immune system. Some will suffer recurrent infections from infancy. However, some infants with PI will not show symptoms early on due to Immunoglobulin A (IgA), an antibody that plays a critical role in mucosal immunity. Babies receive IgA through breast milk from the mother or from antibodies that cross the placenta in the womb and remain in the infant’s body until four to six months of age when normal infants start to make their own antibodies. In some cases, warning symptoms will not show up until later in life, sometimes as far along as middle age.
Diagnosis begins with the understanding and recognition of the warning signs. Are there more infections than normal? Do infections recur after treatment with medications? Do infections not respond to usual medications? The infections involved differ for adults and children. Please see the charts to the right. All too often the significance of the warning signs is overlooked, sometimes because they may seem quite mild.
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