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About Primary Immunodeficiency Disease (PI)

About Primary Immunodeficiency Disease (PI)

About Primary Immunodeficiency Disease (PI)

PI is a class of inherited genetic disorders that causes an individual to have a deficient or absent immune system due to either a lack of necessary antibodies or a failure of these antibodies to function properly. PI has an estimated prevalence of 1:1,200 in the United States, or approximately 250,000 people. According to the World Health Organization, there are over 250 different presentations of PI.

PI patients are more vulnerable to infections and more likely to suffer complications from these infections. As patients suffering from PI lack a properly functioning immune system, they typically receive monthly, outpatient infusions of IVIG therapy. Without this exogenous antibody immune support, these patients would be susceptible to a wide variety of infectious diseases.

PROUD SUPPORTERS OF:

THE 10 WARNING SIGNS OF PRIMARY INMUNODEFICIENCY1

  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 PI.

Implications for Patients with PI: 2013 IDF National Patient Survey

63%

of respondents reported having asthma, 13% have COPD

46%

of PI patients reported they suffer from chronic lung conditions

40%

of PI patients report lung infections and other infections in the prior 12 months

6%

of PI patients reported being hospitalized in the prior 12 months due to lung impairments

One infection is one too many! Each time a PI patient gets a serious infection, irreparable damage occurs.

Risk Factors for Infection in PI

Risk Factors for Infection in PI

  • Type and severity of immune deficiency
  • Age
  • Impaired pulmonary function
    • Bronchiectasis
    • Asthma
    • History of respiratory infection/environmental conditions
    • Chronic lung disease

ADMA is an advocate for the PI patient. We pledge to champion for protection against the risk of infection, and to develop therapies that meet their needs through tailored plasma pool compositions.