Primary immunodeficiency occurs in people with immune systems that are either damaged or missing at birth. The condition appears in the form of “common infections“. This carries the risk of a medic sometimes only treating the infection while missing the underlying cause. The condition becomes more apparent as infections can reoccur. The risk here is that, untreated, vital organ damage, physical disability, or even death can occur.
Some 250 different genetic defects of the immune system are recognized by the World Health Organization as a Primary Immunodeficiency. Globally it is estimated that one in 1200 people have the condition (which means 6 million people worldwide affected). When a defect in the immune system is inherited (carried through the genes), it is called primary, or inherited, immune deficiency. This contrasts the condition to infection with HIV, which leads to acquired immune deficiency. The video below provides further details:
To alert parents and medical staff, the Jeffrey Modell Foundation worked with the American Red Cross to come up with 10 warning signs about primary immunodeficiency. The warning signs have since been translated into more than 50 languages.
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With children the 10 warning signs are:
1. Four or more new ear infections within 1 year
2. Two or more serious sinus infections within 1 year
3. Two or more months on antibiotics with little effect
4. Two or more pneumonias within 1 year
5. Failure of an infant to gain weight or grow normally
6. Recurrent, deep skin or organ abscesses
7. Persistent thrush in mouth or fungal infection on skin
8. Need for intravenous antibiotics to clear infections
9. Two or more deep-seated infections including septicemia
10. A family history of Primary Immunodeficiency
A list of signs is also available for adults.
The launch of the warning signs in Canada is part of World Primary Immunodeficiency Week, which this year runs between April 22nd – 29th, 2017. Around 29,000 Canadians suffer from primary immunodeficiency and it is though about 70 percent are undiagnosed. Raising the profile is important since, with early diagnosis, care and treatment, individuals can live full and rewarding lives. Treatment includes the administration of plasma derived products (the infusion of immunoglobulins). Human Immunoglobulins are purified from human plasma/serum with the buffers used being phosphate or tris buffered saline coming with slightly differing salt concentrations. Such products are manufactured through aseptic processing, meaning microbiological control is a key quality attribute.
The warning signs support the work of Immunodeficiency Canada, which has been in operation since 1999. The organization exists to provide patient support, education, and research with the aim of finding a cure for the condition.
As part of the global week-long event, a petition has been set-up by the campaign organization Global Primary Immunodeficiencies which is requesting governments to take steps to understand, diagnose and manage Primary Immunodeficiency Diseases in their respective countries.