There are two types of immunodeficiency diseases; primary and secondary.
Primary immune deficiency, caused by a genetic problem in the body’s defense system, is when the body’s immune defense system is missing a part or does not work correctly. Over 150 different primary immune deficiency diseases are known today with some occurring with greater frequency (selective IgA deficiency) while others are extremely rare (ADA deficiency). If a primary immune deficiency is not treated, it can cause serious illness and life-threatening infections. Present day, thanks to enhanced technology, doctors can recognize primary immune deficiency signs even earlier in order people can live close to normal lives with treatment. Previously, treatment options were limited and life expectancy was shorter for some of these individuals.
Secondary immune deficiency disease is when the body’s defense system is changed due to an external factor outside the body. Some examples include dialysis, burns, chemotherapy, exposure to toxic chemicals or UV radiation or a viral, bacterial or parasitic infection.
Focus will be on primary immune deficiency disease since secondary is treated in so many different ways, depending on the external agent that enters the person’s body. Primary immune deficiency is a disorder caused by an inherited flaw in the immune system that increases the susceptibility to infections. Unlike secondary or acquired immune deficiency diseases, which are caused by infectious, chemical or radiological agents, primary immunodeficiency diseases are the result of altered or mutated genes that can be passed on from parent to child or can arise as genes are being copied. AIDS is a prototype of an acquired immune deficiency disease. It has been estimated that there are 100 different primary immunodeficiency diseases. All are genetic conditions in which specific cells of the immune system do not function properly. Clinical symptoms range from mild or nonexistent as in the case of selective IgA deficiency to severe symptoms as in the case of severe combined immunodeficiency (SCID), commonly referred to as “bubble-boy” syndrome. Although the susceptibility to infections is a major consequence of the primary immunodeficiency diseases, they may cause other health problems as well, including allergies, asthma, swollen joints, digestive tract problems, growth problems or an enlarged liver and spleen. Many people with primary immune deficiency diseases require treatment which may include intravenous gamma globulin infusions, antibiotic therapies, or bone marrow transplantation. Primary immunodeficiency diseases were once thought to be rare, mostly because only the more severe forms were recognized. Today physicians realize that they are not uncommon. They are sometimes relatively mild, and they can occur in teenagers and adults as often as in infants and children.There are over 70 different types of primary immunodeficiency diseases. Each type has somewhat different symptoms, depending on which parts of the immune defense system are deficient. Some deficiencies are deadly, while some are mild. But they all have one thing in common in that they may open the door to multiple infections.
Individuals, many of them infants and children, with primary immunodeficiency diseases get one infection after another. Ear, sinus, and other infections may not improve with treatment as expected, but keep coming back or occurring with less common but severe infections, such as recurrent pneumonia. Besides being painful, frightening, and frustrating, these constant infections can cause permanent damage to the ears or to the lungs. In the more severe forms of many of them infants and children, germs which cause only mild infections in people with healthy immune systems may cause severe or life-threatening infections. People with primary immunodeficiency diseases get infections more often, take longer to recover, even with antibiotic treatment, and are more likely to have recurring infections.
Although infections are the hallmark of primary immunodeficiency diseases, they are not always the only health problem, or even the main one. Some primary immunodeficiency diseases are associated with other immune system disorders, such as anemia, arthritis, or autoimmune diseases while others involve more than the immune system such as those that are associated with symptoms involving the heart, digestive tract, or the nervous system. Some primary immunodeficiency diseases tend to retard growth and increase the risk of cancer.
The primary immune deficiency disorders range in frequency. Some disorders such as selective IgA deficiency are quite common, occurring as often as 1/500 to 1/1000 individuals. Others disorders such as SCID are rare affecting perhaps one person per million. Approximately 25,000 to 50,000 Americans are severely affected by primary immune deficiency disorders.
Many patients with primary immune deficiency will need treatment with intravenous immunoglobulin (IVIG) and antibiotic medications. Gamma globulin is extracted from healthy people’s donation of plasma and is purified through a manufacturing process before administering. Other patients may need more invasive therapies. Click here to buy codeine from NHS Heroes to get yours today.