I have addressed other forms of PI, as well as CVID, throughout the blog. Focus here is to attempt to be a simplistic as possible, with focusing on Common Varable Immune Deficiency (CVID). Primary Immune Deficiency, also referred to as PI throughout the blog, is an absent or an immune system in chaos. The immune system is designed to protect the entire body as a whole. The immune system is responsible for millions of processes, chemical, biological, and significantly affects major organs. Autoimmune Disorders are common with CVID, and referred to often, as comorbities. Autoimmune Diseases are a result of the body’s immune system, attacking itself.
Symptoms can vary widely within the PI population. I have witnessed some of the most common symptoms of the general PI community. Of prevelance, the most common symptom is the inability to mount a defense from a germ (virus, fungal, bacterial), and the resulting aftermath, serial bacterial infections. Serious, chronic, long-lasting illness pursues after what most deem a simple “virus”. That virus has the capacity to quickly become life-threatening pneumonia. Additional Illnesses (complications and/Or autoimmune) and symptoms lead to more diagnoses, and become more chronic and progressive. Often Diagnosis is delayed by decades, and the damage from consistent infections is irreversible. Extreme, fatigue. Not just fatigue, chronic and debilitating, what seems a simple task, is insurmountable. The “spoon theory” derives from this crushing state of existence. At a conference, every patient in the room complained of the diabolical exhaustion. Researchers are aware of this all time consensus, but do not offer any answers. Gastrointestinal difficulties are also a common symptom. As well, as autoimmune diseases. This is not exclusive, many suffer with intense “complaints “. While others suffer in another body part or system and is as severe, but a different body origin or system is the source. After reviewing many updates, the resulting Illnesses, appear to be linked between 2-3 specific types. All co-morbid diagnosis, are the RESULT Of an immune system that is absent, not functioning, and in chaos.
The Jeffrey Moddell foundation primarily focuses on pediatric PIs, as of now. JM offers this flyer has a quick reference of symptoms of a PI.
Plasma, the “white” (it’s actually yellow), liquid component of blood, contains what called “immunoglobulins “. Immunoglobulins are a specialized proteins, produced by B cells, that develop into plasma cells. These proteins Develop through a maturation process, into antibodies. Antibodies are the body’s defense against invaders, fungal, bacterial, and invading microbes. The major antibodies include immunoglobulin G (IGg), immunoglobulin A (IGa), immunoglobulin M (IGm). Low levels of immunoglobulins, results in inadequate antibodies. Inadequate antibodies leaves the person unprotected from invading Illnesses, thus, constant illness, and an immune deficiency. Additionally, the immune system attacks itself, research, does not understand the correlation between a defeciency and why the
A blood test determines abnormalities with the IGG, IGA, Or IGM. If defeciencies are positive, a diagnosis of hypogammaglobulinemia. Further testing is required to determine if and to what type of an immune disorder is present. Current criteria for a CVID diagnosis indicates a defeciency in IGG, and deficiency in IGa or IGm. “Titers”, which means through serum testing, antibody responses are recorded for vaccinations. If a patient has CVID, their immune system does not ever create antibodies to any or most vaccines. The “acquired or memory” cells are absent, the titers certifies the patients immune system has not produced any immunity to immunizations. This is an indicator that the immune system does not have working “memory”, and defective. Full panels of serum testing are completed, a patient may gain insight as to what specific immune cells, or pathways, are deficient.
Always secure and keep a copy of all of your blood work. I monitored my “general” labs for a few years (pre diagnosis). The GENERAL LAB results (without specifically testing for immunoglobulins was KEY to my diagnosis). My lab results were constantly out of range for years in two areas 1) ALBUMIN 2) GLOBULIN the ratios were always marked out of range. I researched what this meant, and never was able to locate any information. When presenting the abnormal ranges to my MD at the time, it was dismissed. If the above results are abnormal on “NORMAL” WBC draws, contact a CLINICAL immunologist. Another tip: I also had abnormal co2 blood results for years, I am not sure if or how this was related. Point is, secure and keep all of your labs, blood, x rays, MRIs, etc. I found lab work from 10 years prior that indicated IGG numbers were clinically low, and no one ever questioned it! Advocate!
A detailed breakdown as follows: