Educating myself and others about immunity

Today is education day.  I’ve started to delve deeply into IVIg and it’s uses with respect to the immune system.  In previous posts, there is absolutely a clear indication of a TNFRSF13B mutation within my mother’s side of the family.  It affected my sister in the form of GBS.  My mom’s brother, Crohns.  Again, my entire life I have been constantly using antibiotics.  Whether I am fighting off yet another respiratory infection, pneumonia, teeth infections, I was diagnosed with CVID or Common Variable Immunodeficiency Disorder.

The path to getting my diagnosis wasn’t easy.  Most of my issues are attributed through my immune system just giving up, and not being there for me.  Now for someone reading this, this isn’t acquired, this isn’t an STD, this isn’t HIV, or AIDS.  My immune system is malfunctioning, and does have some function.  My immune system tests shows some defects within the B cell, which essentially is the signaling cell that there is an infection and deploys antibodies and signals other portions of the immune system to destroy the infection via NK (Natural Killer) or T cells.  There are several different types of T cells:

CD4 Helper – These help activate cytotoxic T cells and macrophages.  Think of this as the signaling aspect of your immune system.

CD8 Cytoxic – These cells are responsible for releasing the attack on the infection.

Memory CD4|8 – These cells are more long lived.  Essentially this is the memory should a re-infection occur against a previous pathogen either bacterial or viral.

Natural Killer – Bridge the gap between the adaptive and innate portions of your immune system.  Once activated, they can mimic the functions of both CD4 and CD8 T cells.

A. Bone marrow: The site in the body where most of the cells of the immune system are produced as immature or stem cells.
B. Stem cells: These cells have the potential to differentiate and mature into the different cells of the immune system.
C. Thymus: An organ located in the chest which instructs immature lymphocytes to become mature T-lymphocytes.
D. B-Cells: These lymphocytes arise in the bone marrow and differentiate into plasma cells which in turn produce immunoglobulins (antibodies).
E. Cytotoxic T-cells: These lymphocytes mature in the thymus and are responsible for killing infected cells.
F. Helper T-cells: These specialized lymphocytes “help” other T-cells and B-cells to perform their functions.
G. Plasma Cells: These cells develop from B-cells and are the cells that make immunoglobulin for the serum and the secretions.
H. Immunoglobulins: These highly specialized protein molecules, also known as antibodies, fit foreign antigens, such as polio, like a lock and key. Their variety is so extensive that they can be produced to match all possible microorganisms in our environment.
I. Neutrophils (Polymorphonuclear PMN Cell): A type of cell found in the blood stream that rapidly ingests microorganisms and kills them.
J. Monocytes: A type of phagocytic cell found in the blood stream which develops into a macrophage when it migrates to tissues.
K. Red Blood Cells: The cells in the blood stream which carry oxygen from the lungs to the tissues.
L. Platelets: Small cells in the blood stream which are important in blood clotting.
M. Dendritic Cells: Important cells in presenting antigen to immune system cells.

Now with a little education out of the way on how your immune system works, let’s focus on my specific condition.  CVID.  My condition impacts both G. and H. portions of the image above.  Specifically looking at my B cell functions, my body cannot produce enough antibodies for both IgG, and IgM.  Also, I have an IgA subclass 2 deficiency, however my overall IgA is good.

So what the hell is the difference between these B cell antibodies, think of “GAMED“:

IgG – a class of circulating antibodies predominant in serum, produced by plasma cells and memory cells in response to pathogens and other foreign substances

IgA – a class of antibodies predominant in respiratory and alimentary tract secretions and in saliva and tears, functioning as the body’s first line of defense against invading foreign substances especially by neutralizing viral antigens and by preventing the adherence of bacteria to mucous membrane surfaces.

IgM – a class of short-term circulating and secretory antibodies existing as an aggregate of five antibody molecules, having a high affinity for viruses.

IgE – a class of antibodies most abundant in tissue spaces, involved in the expulsion of intestinal parasites and causing allergic reactions by activating the release of histamines and leukotrienes in response to certain foreign antigens.

IgD – a class of antibodies present as an antigen receptor on most cell surfaces and predominant on human B cells.

Now with all that out of the way.  You should have a basic understanding of the human immune system (now you can see how much I have educated myself into all this).  IVIg or (Intravenous Immuglobulin, is mostly made up of IgG, and trace amounts of IgA, and IgM.  Now, each IVIg product is NOT the same.  Some can be used both in an IV fashion, and in a SC fashion (Subcutaneous).  Now, IVIg or SCIg IS NOT A BLOOD TRANSFUSION, however it is derived from the plasma pool which is blood.  The creation of IVIg or SCIg typically takes anywhere from 1,000 to 15,000 blood donors to create a batch of IVIg or SCIg respectively.

Now for my first infusion, I have been prescribed Octagam 5%.  Now for the details of what this is comprised of.  The manufacturing process for Octagam 5% liquid isolates IgG without additional chemical or enzymatic modification, and the Fc portion is maintained intact. Octagam 5% liquid contains the IgG antibody activities present in the donor population.

IgG subclasses are fully represented with the following approximate percents of total IgG:

IgG1 is 65%
IgG2 is 30%
IgG3 is 3%
IgG4 is 2%

Now Octagam just like other IVIg products, again, contains trace amounts of both IgA, and IgM.  It also contains Maltose as a stabilizer for the product specifically.  Other IVIg products use a wide varying array of stabilizers.

So those are the facts of what I have, and what I am facing.  Now notwithstanding, I have no control over my bodies immune response.  I am constantly fatigued.  My body is always at war with something.  Since 2015, the disorder has rapidly progressed to this point.  I always thought that my body would eventually come around, but when 2018 hit, thats when I really learned that I was in trouble.  Between being hospitalized several times for sepsis, which by the way is a VERY serious condition, cellulitis, unknown bacterial infections, this got me to the point of finding an immunologist that could help put me on the path of finding stability, and healing.

Now with that being said, I am not going to get better.  I am not going to get cured.  I am going to now start being able to mount a defense against things we come in contact every day, and now have a fighting chance to lessen my risk of going back to the hospital.  Now with the IVIg comes the pros, and cons of being treated with it.  The pros, there’s only one, having an immune system capable of actually doing something.

Cons (covered in previous posts):

Common side effects of all IVIg products:

mild headache
dizziness
tired feeling
back pain, muscle cramps
minor chest pain
flushing (warmth, redness, or tingly feeling)

Rare to serious side effects of IVIg products:

Aseptic Meningitis
Anaphylaxis
Renal Failure

Now the above Cons can be experienced during the IVIg infusion, or days after.  I will need to keep an infusion log of several different aspects of my therapy going forward.

• IVIg Specific Items
• Brand of IVIg
• Amount Infused
• Date of infusion
• Lot # of the IVIg product

People always ask me what they can do to help me.  Well, to that I think I respond with donate blood.  If it wasn’t for those people whom are healthy, and contribute to the plasma pool, this product, and treatment wouldn’t be possible.  Thats all for now, I hope that this will help people understand CVID better, and what I have been going through.  I am hopeful that the IVIg will benefit me in many ways, and get be back to as “normal” as possible, but we will just have to see how this goes.