Primary Immonodeficiency is uncommon but not RARE! These diseases are made by clinical diagnosis with supporting laboratory reports.
- 1 in 1,200 people with more than 10 Million worldwide
- 4 times more common than hemophilia
- 5 times more common than Cystic Fibrosis
- more than 200 PID
- PID in adults almost as common as in children; however in children often more severe.
- Warning signs are similar- allergic , inflammatory or autoimmune and may not present with infections from weakened immunity.
- in more than 25% of cases, diagnosis takes 20 years from onset of symptoms
Immunity is from Latin word ‘immunitas’ from legal prosecution offered to Roman senators while in office. Protection from outside world such as pathogens, reaction to allergens and elimination of cancer cells while maintaining self-antigens.
Immunity has two parts. Innate is most primitive and has no memory. Adaptive immunity is recently evolved and 2nd line in defense. It has a memory and is the defense against microbes and cancer.
Adaptive immunity involves B cells and T cells. They are found in blood, lymph nodes and stem cells.
CVID is the most common PID in adults. I have Specific Antibody Deficiency- IgA. That means I don’t make good quality immunity antibodies in the mucus membranes of my body. Currently, we can only take IgG antibodies which provide my body backup.
my WONDER WOMAN JUICE: Immunoglobulin therapy goal isn’t to fix but to give us good quality antibodies. Immunoglobulin 10,000 to 60,000 donors in each pool. IgG is separated by ethanol fractionation and treated to prevent viral transmission. Then, it is stabilized by amino acids. The process takes 9-12 months from the plasma to the shelf. Its a very complicated process and 98% of the final product is usable IgG in the normal distribution (IgG1, IgG2, IgG3 and IgG4).
I’ve recently switched from a once weekly subcutaneous IVIG product to a newly approved monthly subQ called Hyquvia. This Baxter product is very exciting for me both from a connivence and from a clinical standpoint. Stay tuned for a post with lots of information on that. Meanwhile. Here’s a pic from today’s conference with Chris Wilson of Baxter and Hank Schwyzer of NuFactor Specialty Pharmacy. You guys ROCK!
3rd Annual Patients with Primary Immunodeficiency Education Day – Newport Beach, California
FACULTY
Sudhir Gupta, M.D., Ph.D. Professor of Medicine, pathology & Laboratory Medicine, and Microbiology and Molecular Genetics.
Chief, Basic and Clinical Immunology, University of California, Irvine
Co-Founder, Foundation for Primary Immunodeficiency Diseases (FPID)
Joseph Church, M.D. Chief of Clinical Immunology and Allergy. Children Hospital of Los Angeles. Professor of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles
Leman Yel, M.D. Associate Professor of Medicine (Emeritus), University of California, Irvine. Global Medical Director, Baxter Biosciences, Westlake Village
Neena Kapoor, M.D. Division Chief, Bone Marrow Transplantation and Research Immunology, Children Hospital of Los Angeles. Professor of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles
For more information: FPID.org
