Primary immune deficiencies (PI) are a group of rare genetic diseases that affect the immune system, making a person immunocompromised. The immune system is the part of the body that protects a person from infection. For most people, the common cold is rarely more than a minor inconvenience; however, for patients with PI, the common cold can be a life-threatening event. There is no cure for PI. The treatment is intravenous immunoglobulin (IVIg), which is made from donated plasma. The plasma is collected from people when they donate blood. It takes approximately 300,000 blood donors to make one batch of IVIg. Intravenous immunoglobulin gives patients a transient immune system; it gives them necessary antibodies to help fight infection. A patient with PI will need to receive replacement immunoglobulins (IgG) at least once a month, every month, for the rest of their life.
Since these diseases are so rare, it often takes years before a patient is properly diagnosed. Even once a patient is diagnosed, it can be difficult to get proper medical care because most doctors have never heard of primary immune diseases, never mind know how to treat them. Proper and early diagnosis is essential to a good quality of life and prognosis.
One correction . . were not positive CVID is genetic. The best thinkers believe CVID is a bunch of different, over 150, illnesses lumped into a classification called CVID. That is why it is so variable.
ReplyDeleteThe current believe is there is a genetic component but there need to be some sort of a trigger mechanism, environmental, virus, or something we don't yet understand for an individual to have their B and T cell stop functioning.