By: Meredith Ruppert, RN
In partnership with World Primary Immunodeficiency Week celebrated this week (April 22-29th), The Immune Deficiency Foundation is asking everyone to join in to make April Primary Immunodeficiency Awareness Month. People with conditions called primary immunodeficiencies require a regular infusion of IVIG to replace antibodies that their bodies do not produce. IVIG (intravenous immunoglobulin) contains immunoglobulin G and is provided intravenously; a subcutaneous version of immunoglobulin G is also available.
As many as 250,000 people have a primary immunodeficiency, while thousands more go undiagnosed. People with a primary immunodeficiency are more susceptible to infections and illness, which can in turn progress to debilitating diseases. To prevent these chronic infections from causing irreversible damage, early diagnosis is imperative. This need for early diagnosis has fueled organizations such as The Jeffrey Modell Foundation and The Immune Deficiency Foundation in their efforts to educate the public and encourage awareness about these conditions.
Primary Immunodeficiency Warning Signs The first step to correct diagnosis? Knowing the warning signs of primary immunodeficiency:
- Four or more new eary infections within one year.
- Two or more serious sinus infections within one year.
- Two or more months on antibiotics with little effect.
- Two or more pneumonias within one year.
- Failure of an infant to gain weight or grow normally.
- Recurrent, deep skin or organ abscesses.
- Persistent thrush in the mouth or fungal skin infection.
- Need for intravenous antibiotics to clear infections.
- Two or more deep-seated infections, including septicemia.
- A family history of primary immunodeficiency.
If you or someone you know is affected by two or more of the above warning signs, speak to a physician about the possibility of an underlying primary immunodeficiency. Diagnosis is made by history, physical exam, and blood and immunoglobulin level tests.
Once a diagnosis is made, treatment can begin. It is estimated that 70 percent of patients diagnosed with primary immunodeficiency are treated with IVIG. The infusion of IVIG acts as a replacement for what is absent from the patient’s immune system. IVIG is a continuous replacement therapy throughout the life of a patient with primary immunodeficiency.
Ultimately, the goal of treatment for primary immunodeficiency is for those diagnosed with it to lead a full, active life. They should be able to participate fully in school, work, and social activities. The treatment will result in a decrease of the number and severity of infections. The appropriate treatment will produce the least amount of side effects, while being a regimen that works for each patient’s schedule and lifestyle. The constant research, development of new products, and education of pharmacy and care providers are all integral parts of this goal.
Think Zebra The Immune Deficiency Foundation, which is the national patient organization dedicated to persons living with primary immunodeficiency diseases, urges patients and their doctors to “think zebra.”
In medical school, many doctors learn the saying, “when you hear hoof beats, think horses, not zebras” and are taught to focus on the likeliest possibilities when making a diagnosis, not the unusual ones. However, sometimes physicians do need to look for a zebra. Patients with primary immunodeficiency diseases are the zebras of the medical world.
If you have an infection that is: • Severe: requiring hospitalization or intravenous antibiotics • Persistent: won’t completely clear up • Unusual: caused by uncommon organism • Recurrent: keeps coming back • Runs in the family
Then ask your physician to: THINK ZEBRA!
Sources: Immune Deficiency Foundation, The Jeffrey Modell Advisory Board
Bio: Meredith Ruppert, R.N. is the Regional Director for Business Development for BioPlus Specialty Pharmacy