Coming to terms with having an immunodeficiency has been both a burden and a blessing, and a marathon all wrapped into one. I’ll be quite honest, my life has not been easy to say the least. From a medical standpoint, it has been a train wreck. Like in my earlier posts, you can easily see how much I’ve gone through to get to this point.
My life ultimately changed August 2018 after being readmitted back into Porter Adventist fighting sepsis for the 3rd time. The re-admitting physician coming into my room and saying “Are you aware of having a compromised immune system?” Shocked I replied with “This is the first time anyone has ever said that to me.” Of course the first thing that shot into my head was my worst fear, HIV, or AIDS. Even though the possibility of me having HIV or AIDS was absolutely none, I asked him back directly, “Like HIV or AIDS?”. “No”.
This sent my mind into WTF mode. He then started to explain to me how the immune system works. That’s when I learned what immuglobulins were, and what they were for. “I’m not an expert at the immune system, but I think you need to look into your immune system and follow up with an immunologist” he said. After being discharged the second time, the 3rd re-admission back to Porter, I was assigned a different physician. I had filled him in on my immunologists name, and got the two talking together as I was fighting of yet another episode of sepsis, and colitis on top of that. More bloodwork was done, looking at IgG, IgA, and IgM. From the original levels of 562, I started to look at the numbers (IgG & IgM), and I noticed the numbers were well below “normal”, and going down. My immunologist started telling my attending physician what to do, and what to run at this point. We pulled TDap titers, the Diptheria antibodies were non-existent, like I’ve never gotten the shot in my life. At this point, the colitis was resolving, and I contacted my immunologist directly and begged her to get me transferred over to UC Health. Which is where she wanted me to go for my immune workup. She mentioned ordering IVIg, but wanted that to happen once I got to UC Health.
After I arrived at UC Health, they pulled my immuglobulin numbers again, and IgG went down by 100mg/dL in a matter of 2 days, and IgM by 5mg/dL. The admitting physicians at UC Health got me an Immunology consult the next day. Sitting with 2 members of Immunology, they agreed that a full immune workup would be reasonable after my discharge. I had yet another septic episode roughly a week and a half after being discharged again.
4 weeks after being discharged, September 20th, I sat at UC Health’s Allergy/Immunology department sitting there just wondering whats going to happen. They started off with the usual tests, CBC (Complete Blood Count), which for the first time in months, was back to “normal”. I met my new immunologist, along with a few members of his team to talk about the plan ahead. I got a pretty good education on what immuglobulins are, and what they do. I showed them the rash that I was getting on & off, which they did take an immediate interest in. Then we discussed how we were going to proceed with determining “why” my immune system wasn’t working. Was is truly broken? Or malfunctioning? My immunologist rattled off a battery of tests to another physician sitting at the computer. Tests ordered:
Tetanus & Diptheria Antibodies
Immuglobulin Panel (IgG, IgA, IgM)
Pneumavax 23 Antibodies
Memory B Cell CD27/CD19/IgD
TSH Ultrasensative
Neutrophil Function Testing
Haemophilius Influenza B IgG Antibodies
Lymphocyte Enumeration of TBNK
It was probably the most blood taken out of me ever, I counted over 14 vials of blood. At this point, I was so used to getting IV’s, and getting my blood drawn, it was no big deal. After taking my blood, the immunologist vaccinated me with both the Pneumavax23 and TDap vaccines. He then said, we have to wait at least 6 weeks, and take another look to see what happened with the antibodies. As I drove home from my appointment, the UC Health app just started going nuts with results from the tests that were ordered. It was non-stop for at least an hour. “New Test Result Available”. I started to just hover around my phone looking at things I had no fucking clue what they were, or what they meant. Results were:
Diptheria – No Protection
Tetanus – Protected
Immuglobulin Panel
IgG – 498 (Low)
IgA – 224 (Normal)
IgM – 33 (Low)
Pneumavax 23 – Basically no protection
Memory B Cell CD27/CD19/IgD – normal
TSH Ultrasensative – Normal
Neutrophil Function Testing – Normal
Haemophilius Influenza B IgG Antibodies – No Protection
Lymphocyte Enumeration of TBNK – Normal. My prior hospitalization CD4|8 was really high
So basically, my immune system cannot produce enough immuglobulin G and M. Thats what the results meant. But what did that really mean? It was finally an answer to all my medical problems. Constant and recurring infections. Eating antibiotics like they were candy. I got a message a few days later from my immunologist wanting to meet up before my departure to Mayo to discuss results. CVID was the diagnosis. He also mentioned that once I get to Mayo to have my immuglobulins re-checked, as both the Pneumavax23 and TDap vaccines should boost my IgG levels.
I get to Mayo more towards the end of October. I met with my primary physician who looked at my health history. We spoke in depth about what has happened in 2018. It was like a job interview, took almost 3 hours just sitting and talking about all the hospitalizations, infections, etc. Then he setup my appointments with:
Dermatology
Immunology
Infectious Disease
Oral Maxiofacial Surgery
Focusing on just the Immunology piece, I met with the immunologist. He was quite pleasant and seemed quite interested in my immunodeficiency. He went over using this scoring algorithm developed by Dr. Cunningham-Rundles (JACI 2014), and concluded that my lab score was 9/10, and my clinical score was 10, and that IRT (IVIg Replacement Therapy) should be considered. He spoke to me in depth about how the IRT works, and how often, and how it would benefit me. I recommended that once I return back to Denver, to follow up with immunology at UC Health to start working on getting the IVIg ordered. He handed me a bunch of documentation on IVIg, as well as my score sheet, and I was on my way. Well, at least I was heading in the right direction for once. He pulled both IgG and IgM labs from me, which to my emotional breakdown, showed it going backwards. Completely opposite of what it should have done.
I returned back to Denver with the confidence that IVIg was the way to go, that’s when my immunologist looked at the IgG/IgM results, and ordered the IVIg therapy. Here was his response:
“Yes, you responded to the pneumovax
Yes, your response to diphtheria is considered to be a non-responder. However, with the diphtheria, your value rose from 0.1 to 0.9. So, even if it did not reach the threshold of 1.0, you did have a 9-fold rise. So, in my mind this is a sub-optimal response.
Also, the H. Influenza titers are sub-optimal.
I will write for IVIg at this time. Hopefully, it will be approved.
That said, there are some other tests I want to perform at the next visit. Getting IVIg will not interfere with these tests.”
Then things got weird after that message. Dead silence from my immunologist. IVIg got denied. I was frustrated, but was at the point to where I needed to return from work. I still continued to feel worn out, fatigued, but I just pushed through it. I saw my immunologist again in December, and to my surprise, my IgG increased to 543, well, maybe my immune system is recovering on its own. My immunologist reversed course and said that he no longer supports my need for IVIg.
January 2019, IgG continued to increase to 563. My immunologist retracted his support for the IVIg stating that the oral infection is what was causing my low immune system numbers and said that “this is probably the normal level you’ll be at.” I was somewhat confused by that, but from a logical perspective at the time, it made sense. My immunologist stated that he didn’t need to see me anymore, as it looks like my immune system is coming back around. I just made the assumption that things are just going to improve, I actually started to get some energy back, however, that was short lived.
The fatigue returned in February, another sinus infection, and my upper teeth started to hurt. I could feel my heartbeat in my teeth (which were already root canaled). I reached out to my Oral Surgeon to perform another Cone Beam CT scan of my mouth, and again, more infected teeth. Thats when I told my Oral Surgeon that we need to dig deeper. He referred me out to an ENT (Ear Nose Throat) Doctor.
In March, I met with my ENT’s NP who restored my faith in medicine, and in people. Sitting down and speaking with her was an experience I haven’t had in the medical field (Minus Mayo), in a very long time. She took the time to actually listen, understand, ask questions, ultimately treating me like a real person, not just “another” patient. After I explained the issues from 2016 till now, she went into immediate action. Not only interested in my immune issues, she looked at CT scans I had had previously at both Swedish Medical Center and Aurora Medical Center prior to me coming to the office. She noticed that I had an osteoma growing in the upper left portion of my frontal sinus, and that could be contributing to my immune issues as well as the bad headaches, and neck pain I was experiencing. She orders a CT and MRI of my head, as well as orders a full deep dive of my immune system (something which my immunologist refused to do). I went downstairs to have the blood drawn and I was on my way.
The pain still continuing in the upper portion of my mouth I reached out to my Oral Surgeon again, and agreed that the teeth needed to go. Wife took me to my appointment, and an hour later, they were gone. 3 more previously root canaled teeth gone.
My final meeting with my immunologist was really odd and quick. He didn’t really seem interested in seeing me anymore, but I asked him to pull 1 more IgG test just to make sure, and to my surprise, it dropped backwards to 472. I messaged my immunologist about the 100mg/dL drop, and his response was:
“This could reflect 1) lab variation or 2) state of hydration.
Remember we have checked your specific IgG antibodies to vaccinations and they are OK. The specific IgG values are more important than the total.
That said, I will place an order for an IgG test. Please have that done in 2-3 months. If it is still trending downwards, you could be seen here again for further eval.”
Cigna being Cigna denied the CT and MRI, but my ENT’s PA spoke with Cigna via a peer review, and things were approved. So I met with the results of the tests, and thats when the PA referred me to their own immunologist. I also showed her the results of my IgG levels after the teeth being extracted, which really concerned her. I had an emotional breakdown in her office. The pain, the fatigue, the disappointment in UC Health, everything just hit me at once. She reassured me that she will make sure that their immunologist will take a fresh look at it.
I sent the results from the workup showing the IgG deficiencies as well as the IgA deficiencies to my old immunologist and his response was:
“The question is do you need IVIg. Some docs may put you on this.
From a risk:benefit ratio question, I felt this is not indicated for you at this time.
If this new doc wants to do this and can get it approved by your insurance, I understand.”
After reading the response from my old immunologist, I was disgusted. I turned back to Mayo, and emailed them the workups from UC Health, and their disagreement with treating me for IVIg, I got a formal letter of recommendation from Mayo, and gave this to the new immunologist that I was seeing:
“This letter is being provided at the request of our patient. He was seen in our division for clarification of an underlying immune deficiency as a contributor to his recurrent infections. He has a complicated medical history with recurrent infections of the jaw, multiple caries, mastoiditis, recurrent sepsis, recurrent cellulitis, and recurrent pneumonias between 2008 and 2015. I do note that he has had five x-rays in 2018 for suspect pneumonias, but radiologically there was no evidence of such.
Immune workup done elsewhere as well as Mayo showed a reduced immunoglobulin IgG at 451 mg/dL (lower limit of reference range 767 mg/dL) and a reduced IgM at 23 mg/dL (lower limit of range at 37). Immunoglobulin A is within reference limits. Nineteen of 23 serotypes to pneumococcus are above the consensus value of 1.3 mcg/mL. He had an appropriate antibody response to tetanus toxoid IgG at 3.77 and a diphtheria toxoid IgG non-responder or suboptimal value at less than 0.1 IU/mL, which subsequently has gone up to 0.7 IU/mL after vaccination. He also reports a recent episode of mastoiditis and continues getting sick with infections.
Using the clinical scoring algorithm developed by Dr. Cunningham-Rundles (JACI 2014), his laboratory score is 9/10, and based on his history, his clinical score would be 10, and thus consideration for immunoglobulin replacement as a trial for a year may be considered reasonable. Our patient seeks a letter summarizing our conclusions, impression, and results. Other providers have in the past recommended a more conservative approach, which may also be okay, but given the burden of recurrent infections endorsed by our patient, a trial of immunoglobulin replacement (given the lack of other specific treatment options) is reasonable. I defer to your clinical judgment and expertise for the final opinion in this matter. Please not hesitate to contact me for further discussion.”
A week later I had my CT and MRI done. Confirmed the osteoma is now golf ball sized.
Then later that week, I met with my new immunologist. I spoke with him for about 20 minutes going over my entire medical history from a child till now. Between that, and all my immunological workups, and the letter from Mayo, he stops. “There’s no need for you to talk about this anymore, I have heard and seen enough” he said. He started to talk about CVID (Common Variable Immunodeficiency Disorder), and I sort of stopped him. I started to talk about all the research I was doing, the symptoms I was having, it was like the stars aligned. He was somewhat taken back that I had a pretty good understanding of the immune system. Thats when he said “You are textbook CVID, its very clear from a lab and clinical perspective that your immune system is malfunctioning, and you need to get on IVIg like it was 10 years ago yesterday.” I lost it, again. This has been a long hard road for me, and it felt like I was finally going to “make it”.
After fighting for so long to be treated for CVID, I finally got the support I needed. It felt surreal that my new immunologist cared more than I expected. With respect to the random rashes that I was getting, he said “thats your immune system doing that, I’ve seen that plenty of times in patients”. After that we toured the infusion center, and I met his PA who will assist in overseeing my care. I was absolutely elated that I was finally going to get the therapy I so desperately needed.
As I posted last, my IVIg was approved, and I start April 11th. The infusion will be about 5 hours. My IVIg is Octagam 5%. The dosage I will be receiving each infusion will be 500ml, which for the curious, will cost about $4000 for the IVIg itself, and I would assume another $500-700 for the infusion center services. With that aside, I am hopeful that this will help fight the random infections, and give me back some energy.
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