PRIMARY IMMUNODEFICIENCY

Overview and Facts

Primary immunodeficiency disorder weakens the immune system, which makes infections and other health issues more likely to occur. Furthermore, many individuals with primary immunodeficiency are born without parts of the body’s natural defenses or with a malfunctioning immune system, making them more vulnerable to infection-causing microorganisms.

Types and Symptoms

TYPES

Types of primary immunodeficiency include:

Autoimmune Lymphoproliferative Syndrome
CARD9 Deficiency
APS-1
Common Variable Immunodeficiency
BENTA Disease
Congenital Neutropenia Syndromes
CTLA4 Deficiency
GATA2 Deficiency
Chronic Granulomatous Disease
Glycosylation Disorders with Immunodeficiency
Caspase Eight Deficiency State
DOCK8 Deficiency

SYMPTOMS

More common illnesses persist longer or are more difficult to cure than infections in people with a healthy immune system are signs of primary immunodeficiency.

Moreover, some of the indications and symptoms of a primary immunodeficiency include:

Internal organ inflammation and infection
Low platelet counts or anemia
Bronchitis, Pneumonia, sinus infections, meningitis, or ear infections
Hunger, constipation, diarrhea, and nausea
Autoimmune disorders are all lupus, rheumatoid arthritis, and Type 1 diabetes.

Diagnosis & Medications

DIAGNOSIS

Tests to detect immunological disorders include:

Blood tests. These are used to measure the number of blood cells and immune system cells in your blood to see if the levels of infection-fighting proteins (immunoglobulin) in your blood are normal.

Prenatal testing. Parents who have children with a primary immunodeficiency disease may wish to have their future pregnancies checked for specific immunodeficiency disorders.

TREATMENT

The following are treatments for primary immunodeficiency:

Treatment for managing infections:

Infection treatment. Antibiotics must be used quickly and aggressively to treat infections. Moreover, antibiotics may be needed for a longer period than is typically recommended.
Infection prevention. Long-term antibiotics are required in some instances to avoid respiratory infections and irreversible damage to the lungs and hearing.
Symptom treatment. Ibuprofen (Advil, Motrin IB, and others) may help relieve infection symptoms by acting as pain relievers, decongestants for nasal congestion, and expectorants to thin mucus in the airways.

Immune-strengthening therapy:

Immunoglobulin therapy. In this therapy, immunoglobulin is either inserted under the skin or through an IV. This is done since immunoglobulin is made up of antibodies that help the immune system fight infections.
Interferon-gamma therapy. Infections and cancer cells are immunologically confronted by the presence of interferons, which are chemicals in the human body that can be identified in a laboratory.
Growth factors. This helps boost the number of immune-boosting white blood cells in the body.