Diagnosed with Common Variable Immune Deficiency (CVID) in 1994, I soon became a patient advocate for this often misunderstood and undiagnosed illness. Oddly enough, the organization I first worked with was the U.K. is based in, and possibly a resident of, the US.
My contact in the U.K. they are more experienced with CVID than their counterparts in the US, and the US patient support a lot of immune forms in youth. /a> failure.
Call me a brat, but at the ripe old age of 25 I wasn’t quite ready to throw in the towel. Medical knowledge about CVID has been greatly improved – patient associations such as basic immune deficiency.
Patients, however, continue to experience delayed diagnosis and treatment and problems with insurance coverage.
Contents:
–Overview of immune deficiency
–CVID Symptoms
–CVID Diagnosis
–Difficulties in Diagnosis
–CVID Treatment
–Treatment Complications
— Living with CVID
–Child-friendly Help
Hypogammaglobulinemia – Overview
Hypogammaglobulinemia essentially means an antibody deficiency, and includes numerous primary immune deficiency diseases such as Common Immune Deficiency (CVID). Unlike AIDS or chemotherapy-induced immune deficiency, immune deficiencies are considered to be primary innate, possibly genetic.
One of the rarest forms of hypogammaglobulinemia, a severe immune deficiency, has been nicknamed the “child-in-a-bubble disease” – a somewhat popular example known to moviegoers.
Both Common Immune Deficiency (CVID) and Variable IgA deficiency, when the antibodies are decreased, not absent, are much more common. The approximate rate for CVID is 1:50,000 people and the rate for IgA deficiency is as frequent as 1:750 people.
As immune system is quite complex, CVID often involves additional defects in the immune system.
Common Variable Immune Deficiency (CVID) is mild to life threatening: dependent on resistance to infection, success of treatment, and if complications occur. Patients also have a significant risk of developing certain cancers and autoimmune diseases.
Children with CVID are typically born with an immune deficiency, but an adult attack of CVID is not uncommon. The exact cause of CVID is unknown, but genetic factors are involved.
Understanding Immune Globulins and antibodies in CVID
(Refer to the end of the article for a child-friendly explanation)
immune system of the five main types of antibodies called immune globulins, two of which are commonly affected. in CVID: immune globulin G (IgG) and immune globulin A (IgA). IgM deficiency occurs less frequently, although up to 50 percent of the time.
Each type of antibody plays a different role in the response to infection:
- – IgA is the main protector of mucosal linings, including the mouth, stomach, respiratory tract, intestines and external reproductive organs.
- – Igg is the main defense of the body throughout the body. Some CVID patients mount a very common and dangerous bacterial response to no antibody — causing pneumonia, tetanus, whooping cough, and colds, for example.
Signs of common variable immune deficiency
- – bacterial, viral or chronic-sinusitis or damage to the upper respiratory tract, bronchiectasis
- – fatigue
- – expansion of the lymph gland
- — hair loss
- – infections or disorders of the digestive tract, diarrhea
- – anemia
- – enlargement of the spleen
pain or arthritis joint or arthritis (perhaps due to infection
Other Complications:
- – lymphoma
- – autoimmune disease
- – premature death
Note that infants with primary immune deficiency have other problems, such as failure to thrive, due to immature immune systems physical and developmental developments .
Symptoms of mild CVID and IgA deficiency can be similar. However, there is no treatment for IgA deficiency except in the case of infection with antibiotic therapy observation.
Diagnosis of common variable immune deficiency
The test is done by measuring the levels of immune globulin in the blood. Additional tests such as antibody response tests and a CT scan of the sinuses or lungs may also be ordered.
CVID often goes unnoticed until symptoms are more severe in the second to fourth decade of life.
Chronic infection and damage to the lungs and sinuses may occur well before diagnosis.
Difficulties in Diagnosis
Whether the test is underutilized or the entire immunoglobulin level is misunderstood, both contribute to diagnostic delay.
Additionally, total IgG or IgA counts can be misleading, and these levels alone are not necessarily indicative of a patient’s health status.
Each type of protein is immunologically divided into subclasses, sometimes one type is produced in abundance while one or more other subclasses remain deficient or absent. A more specific test, called the IGG class level, is often necessary to diagnose CVID.
Additionally, CVID care is very expensive (thousands of dollars a month). Insurance companies may deny treatment if antibody levels are considered “low enough,” and doctors may be misled about what constitutes CVID.
Treatment for Common Variable Immune Deficiency
Some of the body’s antibodies can be replaced by what is known as intravenous immune globulin (IVIG). IVIG is the most elegant blood product from a pool of selected donors. Other forms of administration, such as subcutaneous injection under the skin, are less commonly involved.
While IgG antibodies can be replaced, no replacement is available for IgA.
IVIG is best administered on a bi-weekly to monthly basis. Infusions typically last a few hours and seated like flu-like symptoms are common.
therapy is not an immune cure for CVID; Generally, IVIG therapy is a lifelong treatment that helps patients better fight off infections and complications of the disease.
Most patients are healthier on IVIG, and some lead more normal lives than the discomfort attributed to the infusions.
Almost all CVID patients eventually experience some form of weakness, mild to severe, and maintain higher than normal infections.
Older CVID patients who receive IVIG fare better. With adequate treatment, sinus and lung damage and lung and other complications can be prevented.
Prompt administration of antibiotics, with necessary and regular evaluation by an immunologist or specialist, is another crucial form of treatment for CVID patients.
Treatment Complications
Delayed administration of IVIG and preemptive administration of diphenhydramine HCL (Benedryl) can greatly reduce infusion-related reactions. Ibuprofen or aspirin may be recommended.
More serious or life-threatening reactions to IVIG occur in a minority of patients. The latter group cannot receive due to immune globules therapy natural antibodies that react to the amounts of IgA or otherwise reduced. Substances contained in immune cells.
Children and CVID
Children with CVID have different educational, medical and support needs than adult patients with CVID. Just the problems involved with exposure to germs at play or school are great. In addition, peer pressure can make a child feel inadequate. For additional help, consider support groups with dedicated information for parents and children. Parents may find both the Jeffrey Modell Foundation and the IDF quite helpful.
For a fantastic explanation of children’s regulated immune systems, see “Our Immune System” by the Immune Deficiency Foundation (IDF). By the way, many adults also like it.
