25 Days, A Lesson in Immunity, and Progress

It’s somewhat sort of hard to believe that I’ve had to wait a literal eternity for the second surgery. Since about March of 2023, I started to push Dr. Hepworth for surgery.

This problem, however, has been going on for a very long time, probably 2+ years. I remember asking Hepworth to look around last year when the sialendoscopy was happening, but it just seemed like he was too bothered to.

The swelling under my tongue has been around since 2019. I found an old card in my desk, where I write down talking points when I go to doctors. It’s sad that this has all been dismissed for so long.

I often take a selfie at least once a week to show progress of my face and neck. Late in 2021, my face felt like it was going to explode. The sheer amount of pain going through my face was something else. Between my eyes, my forehead, my cheeks, along my jawline, and my entire neck. The worst was around my left ear.

Every morning when I would wake up, I would be immediately greeted and reminded with pain. The only thing that would calm it down was antibiotics. But of course doctors wanted to gatekeep the ones that worked the best. Vancomycin was one of the IV antibiotics that were the most beneficial. I only received it sparingly. If I convinced an ER doctor to give it to me, that was the only way I could get it. A single 2g dose would calm things down for the next 2-3 days, but afterwards, the feelings would begin to return.

When COVID hit me, luckily (weird to say), I was declared septic. When a patient is declared for sepsis, this opens up the top drawer of any drug that can immediately benefit the patient. Typically this includes Vancomycin, Ceftriaxone/Cefepime, 2L lactated ringers (no not milk). The goal is to try and get the body immediate help. Sepsis is nothing to mess around with. Once sepsis sets in, time becomes critical. When the body begins to ring the 5 alarm fire inside, everything overreacts. As a consequence, your body begins to shutdown non-essential things.

There are several blood work labs that can be drawn to look at what could potentially be the cause, but again, just so everyone is aware, most labs that are drawn are non-specific. They dont say what the cause is, only shows the effect. Most people seem to think that if a Complete Blood Count (CBC) is abnormal, that doctors will know exactly what to do. On the contrary, it just shows in a generic sense of what your body is doing. Smart doctors typically will order deeper investigation tests to try to narrow down to find the cause.

For me, unfortunately, I have to tell doctors what labs to run, what to look for in those tests. Why? Because my immune disorder caused all sorts of confusion with test results. Over the years, I’ve had to be the one whom pays close attention to them. I know what to to look for based off of how I feel.

Let’s sidestep quickly and let me explain my logic before we jump off of this fun cliff.

Common Variable Immundeficiency is a congenital birth defect where B cells, the cells responsible for FINDING infection are either non-existent, or severely impaired. B cells, or B lymphocytes, their primary responsibility is to generate antibodies. These antibodies are then used throughout your body to find pathogens, viruses, or foreign substances. To further explain, these B cells on the surface have antigens that quite simply have a lock & key mechanism. Antigens which find a “match”, will call up various immune responses to help rid the body of this “foreign invader”.

For all intensive purposes, B cells are the primary defence mechanism of the human immune system. B cells are the primary signalling system that finds infection. Without them, the human body will overlook these foreign invaders. B cells do have a life cycle. All cells within the human body, they all come from stem cells, which is the primary building block of anything found in the human body. Stem cells can be essentially made into whatever.

When looking specifically at stem cells, as they relate to the adaptive immune system, as you can see, B cells come from stem cells. Again, stem cells are the raw material with your genetic encoding carried within.

This is how your body can differentiate what is you, and what is not. Without it, your immune system would just attack everything.

With my specific condition, my body DOES create B cells, but they do not do a good job at generating antibodies. To make up for this severe shortfall, antibodies are carried within human plasma. Immunoglobulin, is contained within human plasma (in normal people it’s quite abundant). Immunoglobulin carries all antibodies, good, and bad. Regardless, people whom donate plasma, is where immunoglobulin can be derived, separated, extracted, and processed to create the product I receive. Immunoglobulin is not blood type specific, so this is how I can receive and “borrow” someone else’s immune system regardless of blood type.

When a foreign invader is found in the human body, the antigens found on the B cells bump up against this foreign invader, generates the antibody protein specific to what it’s found, whether it’s bacteria, a virus, or other things. These antibodies that are generated then bind to the foreign invader.

These binding sites are specific to the foreign invader. Now with that being said, there are 5 different types of antibodies your B cells produce.

Antibodies are classified into 5 differentiated types. Each antibody type, serves a different purpose based on the situation. Those types being IgG, IgA, IgM, IgE, and IgD, easy way to remember the 5 different types is think of “GAMED”.

IgG or Immunoglobulin G, is the most abundant antibody found in the human body. It makes up roughly 75% of the total antibodies found in human serum. Specific to this antibody type, there are 4 subclasses that make up the total amount of IgG. IgG1 and IgG2 makeup almost 90% of the total. IgG is found in human blood, and extracellular fluids. Think of IgG being the primary antibody type that can activate the classical pathways of the complement system (I’ll talk about it later)

IgA or Immunoglobulin A, is the second most abundant antibody found in the human body. As the previous antibody IgG, IgA makes up roughly 15% of the total antibodies found in the human body. There is a contrasting difference however, IgA is found in your saliva, tears, respiratory tract, gastrointestinal tract, and mother’s breast milk. There are trace amounts of IgA found in blood as well. There are two subclasses associated with IgA.

IgM or Immunoglobulin M, is the largest, and first antibody to appear in the response of initial exposure to an antigen. Think of IgM as the first responder to infection, but as an infection progresses, this antibody is found less. This antibody type is found everywhere within the human body, just as the previously 2 mentioned antibody types.

IgE or Immunoglobulin E, this antibody’s primary responsibility is to find allergens, parasites, certain forms of cancer, and toxins. When found, IgE triggers histamine release from mast cells. IgE can also be responsible for anaphylaxis. There should be a very low amount of IgE detected in blood, as too much of this antibody realistically is a bad thing.

IgD or Immunoglobulin D, has for a long time been a mystery within the immunological community. This antibody type to this day is not well understood. For the most part, IgD’s purpose is to signal B cells to activate. Outside of this observation, IgD still remains somewhat of a mystery.

So that’s a small overview of B cells, antigens, and antibodies. Again, this is the monitoring/signalling portion of your immune system.

There are 3 distinct versions of your immune system, innate, passive, and adaptive. Innate is what you’re born with, and adaptive being what you acquire as you grow. CVID, unfortunately, impacts both of these systems. Passive immunity is something that is borrowed, such as your mother’s breast milk, can only be temporary.

From an innate perspective, the B cells that my body produces, has a severe flaw in their function. They just don’t produce antibodies, which would be innate related, from an adaptive perspective, the other malfunction of my B cells is their ability to learn, retain, and find infections.

I am not an anti-vaxxer by any means. Immunizations are good for anyone. It helps train your immunity without having to actually become infected with certain pathogens. I’m pretty sure tetanus would be a nightmare to deal with on its own. With that being said, vaccines carry information which can be used by your immune system to generate antibodies and have the ability and tools to fight certain infections, think of it as the plans to the death star.

Unfortunately for my case, most vaccines or immunizations do not work. Polysaccharide based vaccines specifically won’t be learned by my immune system due to the defects found within my B cells. When I was diagnosed with CVID, I had to go through a vaccine challenge where I was given several vaccines, and my titres were checked pre/post vaccination. The outcome of this challenge, is that my body failed horribly. It didn’t really learn anything, not really respond like a competent immune system should.

If you feel like crap after a vaccine, that’s a good thing. That means your body is responding to essentially what it would be like if it was the real deal. There are several different types of vaccines, ones which are considered “live” are a huge no-no for those of us with CVID. It would cause some pretty life threatening issues, which I really want no part of.

Your body generates, and regulates it’s immunity in several ways. Several organs help generate the three distinct portions of your immunity. The largest organ when it comes to your immune system is your skin. Which is everywhere. Other immune related organs and where they are:

Adenoids. Two glands located at the back of the nasal passage.

Bone marrow. The soft, spongy tissue found in bone cavities.

Lymph nodes. Small organs shaped like beans, which are located all over the body and connect via the lymphatic vessels.

Lymphatic vessels. A network of channels all over the body that carries lymphocytes to the lymphoid organs and bloodstream.

Peyer patches. Lymphoid tissue in the small intestine.

Spleen. A fist-sized organ located in the belly (abdominal) cavity.

Thymus. Two lobes that join in front of the windpipe (trachea) behind the breastbone.

Tonsils. Two oval masses in the back of the throat.

Unfortunately, as I was growing up, the “popular” thing to do was when you were young, was to have your tonsils, and adenoids removed as they were previously thought as “useless”, which in terms of today, was completely wrong. In my specific case, doctors thought my tonsils and adenoids were making me sick, which was why they were removed. In hindsight, they should have done their due diligence before yard saling 2 vital pieces of my immune system. Realistically, it was CVID the entire time causing all my problems. But back then, CVID went under the radar of most doctors due to realistically the lack of education, and understanding the human immune systems functions, and was furthered by the ineptitude of doctors wanting to just write serious symptoms off as one offs, or psychological, or made up. Today, this still happens, and I’m living proof of it.

It’s only taken me 43 years of my life to get doctors to remove their heads from their asses, and pay attention to fact, not myth.

So up until this point, I’ve really tried to explain CVID in a nutshell, what’s the real reason why I keep getting sick. Constantly fighting chronic infections, some of which have almost killed me. I spent a pretty good amount of time on the B cell side of the immune system. Is there more?

Absolutely there is.

You would probably be familiar with red blood cells, as you would know, if you get a cut in your skin, blood appears. It’s redness comes from a reaction when exposed to the outside air. Red blood cells are the primary life preserving cell that delivers oxygen to the remotest regions of your body, and also carries carbon dioxide back to be exchanged in your pulmonary system for oxygen.

There is another cell that follows the same path, visits the same places. Those are known as your white blood cells. Invisible to the naked eye, but crucial to your survival.

To put it simply, your body must do preemptive planning should an infection, or foreign invader make it past the first barrier of your skin. Leukocytes or white blood cells are always in your body. Too much of them, indicates infection. Not enough, some infections / cancers could be present.

White blood cells (WBC) can be broken down into several types. Each WBC plays a specific role or function depending upon the situation.

Phagocytes are the primary cell that is used to destroy invading organisms. These cells are found at the site of an active infection. These cells are delivered by the bloodstream, and can enter effected tissues. 1 liter of blood contains roughly about 6 billion phagocytes.

Neutrophils are a phagocyte that are specifically geared to fight bacteria. These cells are typically the most abundant. The antimicrobial action of these cells is more potent, and can use differing microbidcidal techniques. These cells can generate reactive oxygen or use antimicrobial proteins to destroy the microbes it may come in contact with.

Macrophages are a phagocyte that are the garbage collectors of the human body. These cells scavenge the human body looking for dead cells, as well as other microbes. These cells use nitric oxide to destroy these types of material.

Eosinophils are a special type of white blood cell that act against foreign invaders by releasing inflammatory molecules and cytotoxic cationic proteins. These cells have great effect in fighting parasitic invaders. One thing to note about these cells, is they can damage your own tissues. These cells can also be called into action when an allergen is present. If you have asthma, these cells are the reason why you have issues breathing.

B Lymphocytes are the memory portion of your immune system. They help your body remember a previous infection. These cells are also known as B Memory Cells. They tag antigens for destruction with antibodies that signal the attack side of your immune system.

T-Cells are an important white blood cell that are a type of lymphocyte where their primary function is to help your immune system fight germs and protect you from disease. There are two main types. Cytotoxic T-cells destroy infected cells. Helper T-cells send signals that direct other immune cells to fight infection.

The last portion of your immune system, is known as the complement system. This system can also be used to fight bacteria, viruses, or infected cells. The complement system has the ability to cascade itself to help clear microbes and damaged cells, promote inflammation, and attack a pathogens cell membrane. As much as I would love to explain the complement system, it’s a really deep rabbit hole.

That was a lot, but these are some of the things I have had to learn and educate myself on things you’d expect a doctor to know, and fully understand. Surprise surprise, they don’t. It’s really sad when I have to explain these things ARE COVERED during their education. A basic knowledge of the immune system should be a requirement of all doctors, I don’t care what type of doctor you are. This is crucial material.

Sorry if that was a long winded dive into the immune system, but these things are important to me when it comes to my complex healthcare.

As for progress, since things have become “challenging” with the disability company, I decided that it’s best to return to work on a restricted basis until surgery. I will be going back to work on October 2nd, and going back out on disability after October 25th, as that’s when Hepworth’s surgery will take place at Presbyterian St. Luke’s.

I am hopeful that this will be the final crucial piece to this absurdly crazy nightmare that I have been trapped in for the past 5 years. Advocating for myself, and finding the team of doctors I have now really shouldn’t have taken this long, but it did.

Circling back to why I went on the immunology rant, my labs are just hard to interpret. Performing antibody tests on someone whom doesn’t generate them, but receives them through other means will create a lot of false flags.

Prior to starting IVIg, every antibody test was negative. Nothing. Everything auto-immune was tested, negative. Which is probably why doctors were going the psychological route. Because they weren’t finding any cause, little did they know, they were looking in the wrong place.

Going through all my old labs there is a pattern, and it’s easy to see. My WBC was always somewhat high. Neutrophils being the one that was consistently high. When we get into the pathology of the dermatology side of things, everything removed from me indicated chronic inflammation. Even the mucocele removed from my mouth, inflammation. So here we have 2 things that are a constant theme.

Every septic episode, or even when the severe infections started, lactic acid was ALWAYS elevated. Yes it’s normal to have it in your body, when you work out, your body produces it, but with proper hydration, it goes away. My lactate has always been elevated. Everytime. After IV antibiotics, it drops down. So there is even more evidence showing there is an infection, it’s just not presenting the way doctors expect because my body doesn’t produce proper amounts of antibodies. When all this started, my IgG was at 350, the normal range begins at 750. My IgM was non-existent.

And no. This is absolutely not due to me being dehydrated. Even all the immune workup labs showed my CD4/CD8 is always elevated. That value has never come down. When Sarid dove into the prostaglandins, my D2 was through the roof. Every lab points towards infection, inflammation of unknown origin. But I can tell you where it is, it’s in my face, mouth, and neck. I feel it every day.

Between the labs proving out a definite CVID diagnosis, and Mast Cell Activation Syndrome (MCAS), those two diagnosis’ are key. I’ve proven my immune system is shot, and overreacts to things. But everything that remains all points to a chronic infection. I still don’t care what a CT doesn’t show, or an MRI.

I’m sure when Hepworth opens up things, he’s gonna find it. I’m hoping that I get to draw on my face where everything is. Because if I wake up and I still feel the shit in my left cheek, chin and under my tongue, at this point I’ll probably lose my shit. Hepworth will listen. He values my input. I trust that he will rid me of this once in for all.

This is why the surgery on October 25th is imperative. This will remove the infection once and for all. Hopefully giving my immune system a much needed break. It’s been doing overtime for far too long. My body is a constant reminder that something is wrong, you can’t discount that. I don’t care what labs show, you’re looking in the wrong place anyways.

As for things coming up, Dr Dobbs whom is the oral surgeon I met with 2 weeks ago, ordered a bone scan / SPECT/CT. This scan will definitively determine if there is bone involvement in my jaw/skull. This will be useful if we need to intervene with more surgery to remove infected bone should that be the case. The scan will happen October 3rd. I’ll be injected with a radioactive tracer in the morning, then return to Swedish mid-day to sit in a scanner for an hour. Hopefully I just fall asleep. I’ve never had a scan done like this before, so this is new to me.

I still haven’t gotten my IVIg infusion, which was supposed to transition to Soleo home care, however, they didn’t complete the authorization in time, and I need to go back to the infusion center for the final dose before I transition to home. I’ve been trying since last Monday to get someone from Immunoe to get me on the schedule, but that didn’t happen. “We will call you back” or “our schedulers are out to lunch” or just insert whatever stupid excuse you want use use as to why nobody is calling me back.

So I had enough, I just got off the phone with one of the people at Immunoe, and explained the situation. I told her that Dr. Sarid needs to be notified, as well as the practice manager as this is really unacceptable. I need the infusion every 3 weeks. It needs to be consistent. Again, it’s not my fault that people cannot do their jobs, so I’ll just push this long. I’m not going to wait for these lazy people to decide to do something.

I’ll see Dr. Sorenson the week after to follow up with the kidney stone surgery. I’ll bring the specimen jar with so that they can send the contents off to a lab, but I’m pretty sure they will come back as calcium oxalate, as they always have.

That’s it for today. I’ll post an update again soon. 25 Days to go.