Since the first child with antibody deficiency was successfully treated over 30 years ago, much progress has been made in detecting and treating immune deficiency diseases (IDD) in children and adults. At first, doctors knew only about the severest and fatal cases of IDD, such as the Houston "Bubble" Boy or Fatal Granulomatous Disease of Childhood. Fortunately, these diseases are very rare and prolonged lifespan or even cures are now possible for some of these patients. More recently, acquired immune deficiency syndrome (AIDS) has caused great concern and fear, bringing about much attention regarding immune deficiency diseases — some good and some not so good.

There are many different kinds and causes of IDD in children and adults, of which AIDS is only one cause. Other IDDs are not contagious and cannot be spread to other people. In fact, healthy people with infections may pose a threat to patients with immune problems, since the latter are unable to fight infections effectively.

Background

There are four major components of the immune system: antibodies, immune cells (lymphocytes), phagocytes, and complement defense. All of these components are very important in helping to detect, inactivate, and kill the organisms that cause infection.
When the immune system is not working properly, patients may experience:

Frequent infections
Chronic lingering infections
More severe infections
More complications from these infections

While chronic infections and congestion might be a sign of immune problems, there are other more common causes. These include:

Increased exposure — especially if the child has many brothers and sisters or goes to nursery school/day care centers where infections are easily passed around
Normal variation — some people just get sick more often than others without any known reason
Allergies and asthma — one of the most common causes of chronic congestion, upper respiratory tract "infections," "bronchitis," and "pneumonia"
Secondary causes — some people with other diseases such as diabetes, kidney disease, cystic fibrosis, or sickle cell anemia are more prone to infection

Antibody Deficiency

Where immune deficiency is a cause, abnormalities in antibodies are the most common reason for chronic infection. While some children and adults with antibody abnormalities can have severe infections such as life-threatening pneumonia, sepsis (blood poisoning), or meningitis (inflammation of the brain), most patients have less severe problems.

These include:

Chronic ear infections
Chronic sinusitis
Throat infections
Chronic viral bronchitis and pneumonia
Chronic diarrhea

While these infections are usually not life threatening, they can cause considerable illness resulting in numerous operations (tonsillectomy, tubes, etc.), doctor visits, and school or work absences.

Tests

There are a number of tests used to determine whether immunity is intact. These include:

Antibody levels (blood tests)
Antibody function (blood tests)
Tests to find out whether you can kill fungi or bacteria (skin tests, blood tests)
Tests determining whether allergies are also present (skin tests, blood tests)

Treatment

Treatment varies from avoidance and caution, judicious use of antibiotics, appropriate and cautious use of vaccines, and infusion of immune globulin.

Antibody infusion treatment has advanced considerably in recent years to the point where many individuals with IDD can lead normal lives. While effective, this is an expensive and inconvenient treatment. No cases of AIDS or CMV have been transmitted.

If you have any questions, contact our office: Omaha: 402-391-1800 Lincoln: 402-464-5969