Why…?

Why does it all feel so calm now?

I was formally diagnosed Thursday with CVID – a very serious genetic immune disease that, while not curable at this time, does have clinical options to improve my quality of my life. I’ve – as I am certain all who have received this dx also have done – been reading voluminously about it. It seems so strange, though: now that I know what it is, I feel oddly peaceful and, well, *relieved*, punctuated by sudden, short bouts of great anger. It’s not about who or what I could have been before now (I am in my 40s and completing my PhD, already fairly well-known in my field).  But, had to resign from an adjunct position at a University and another accredited online degree program mid-semester this last Spring because I was just *that* ill. Yet, of course, it was all in my head. Or so they said.

I have been to doctors in other states (having, quite possibly pissed off the entire specialist community in my own region with my dogged persistence and refusal to accept either “I don’t know” or “It’s in your head”; if I *ever* hear the term “somatoform” – E-V-E-R – again, it will be too soon and will likely met with a violent reaction.). Somehow, while exploring others’ blogs since then with that nasty four-letter acronym search term, CVID, I get the impression mine is a common experience on the road to diagnosis. It’s also no wonder, therefore, that we tend to deal with depression, and  have difficulty standing steady on all things relationship (Thank goodness for my husband and our 20+ years of marriage, though! He’s a saint!)

The relief I feel right now is not “just” a diagnosis; honestly, I fear the future as much as I finally look forward to it. This time last week I was really and truly ready to drive as far as I could on my tank of gas, then walk my way along a riverbank until I could walk no more – and spend life watching the ripple of the water, the fish, the frogs, the birds, the stones themselves… and just drift off without a care left in the world. Not a suicidal ideation, actually – not in any way – but rather the desire to have a choice – one, single choice – in how I wanted to enjoy myself for as long as I could breathe. I cannot explain it better than that. Peace has been hard to come by.

However, I had a follow-up appointment last week with an allergist / immunologist who, on a hunch, had rechecked basic IgG, IgA and IgM levels. Now, I had a huge number of tests run at a ding-dong hematologist’s office at the end of December. Had I been with someone who was on the ball about things like this, it would have been a pretty instant diagnosis, then more tests to determine severity and course-of-action. Obviously, that did not happen – even though when I went to the January appointment I had a huge barrage of illnesses:

• Severe pneumonia
• The pressure from the fluid in the bottom of my left lung actually had enlarged and elongated that lung – it was bilateral, but this was the left side – to such an extent that it pressed from the inside on my pancreas and left kidney. I’m still fighting the kidney infection.
• Nasal swab-confirmed influenza
• A serious bilaterally-diffuse mycoplasma infection.

The ding-dong hematologist was HIGHLY dismissive – even with all the hospital results – AND the results of the blood tests he had had run *right in front of him!* ALL he said was that I needed an iron infusion for “some anemia.” Pffft. Eventually, by the last time I saw him, he had decided my problems were somatoform; somehow, according to him, because I was raped when I was 10 I just “don’t cope” and “stress my immune system with unresolved floating angst.” Wow. @@ Hell of a thing to say! Talk about unfounded pop psych!

Was rushed to Cleveland Clinic the next month; it was clear that things were declining steeply and we decided to go sooner rather than a later that might not ever come. Came away from there with a diagnosis of dysphasia (“nutcracker esophagus” with the addition of malfunctioning upper and lower esophageal sphincters) with which I do agree; I have had digestive issues for years and years and years, and had been vomiting 10-15 times a day since the beginning of December. Still do have GI issues to beat the band (I’m seeing a lower GI specialist next week) and the only thing – and still almost exclusively the only thing – I have able to tolerate is chocolate Boost. Yes. I have been on Boost since December, which we now get as Target brand with the addition of plenty of scoops of chocolate Quik; there’s only so much of that stuff you can consume without going utterly batty. I used to love chocolate, actually…

Returned home (a long drive and two flights later) utterly, totally exhausted. I just… collapsed. At that point I realized someone else had to teach these students what I, obviously, could not due to my illnesses, and resigned. That really was one of the hardest things I have ever done. I absolutely love teaching, and cannot imagine myself doing anything but research in my PhD area —– and teaching. If I never do anything else, I will look upon educating hundreds and hundreds of students as the most important thing I have ever done professionally. It has been a distinct honor to do so, and all I can think of is finishing the dissertation (nearly there, actually) and going back into the classroom. We will see what we will see.

The utter agony of even rolling over in bed led to stiffness and migraines – but getting up was often far worse. Pain gathering my body to sit. Pain standing. Pain walking. Pain and exhaustion, pain and exhaustion.

And infection after infection.

Many infections later, I ended up, yet again, in the ER for a critically bad bowel impaction (from the base of the large intestine almost to the top of my small). When I was released from the hospital, I went in and saw my GP. This GP was “new” to me – I had been with another (and frankly, better) GP for some 17 years, and I made a conscious choice to step away there for a year or two and then come back together so we could evaluate the body as an entire, connected organism, with a better focus and new ideas. Well, I hadn’t quite anticipated doing it that week – but the “new” GP sent a certified letter immediately after I saw her stating (and I am paraphrasing) that I was “too complicated” for their practice and that it was “in my best interests” to leave their practice, that she would only be available for emergencies until the end of the month, and after that, she wanted no contact with me beyond notification of where to send the files.

:::stunned::: Yes. I was fired from my doctor for crime of being sick.

So I quickly made arrangements to go back to my original GP, who is one of the best, most pragmatic clinicians I have ever had the pleasure of knowing. A pure joy.

Next thing I knew I had developed my fourth bout of shingles – second one on the year, and a different place. Then, I tripped in the dark in the den and landed squarely on my knee – which, of course, became infected inside and out.

And two weeks later? Another round of pneumonia.

My GP sat me down at that follow-up visit and said, “I have known you so long, and I am so sorry I need to have this conversation with you, but I think we need to think about some things.” She proceeded to acknowledge that I have a sensible and clear living will and medical power of attorney – but (quite gently) asked whether our Will is in order. I didn’t get angry, I wasn’t really shocked. I knew I was dreadfully ill. I asked her why, though. I mean, I knew – but I needed to hear it. She told me that I was so very ill at that point that, were I to contract one more serious illness on top of the pneumonia I was attempting, struggling, fighting to conquer, I could and likely would die within days.

It never really seemed as though that was at all unreasonable or shocking. I have been sick all my life, and I know I do look at bit like death warmed over right now. I used to be a model and it has been a rare day to go out without makeup since I was in college (undergrad) – but I haven’t worn makeup except on two occasions in the past month. I just feel that utterly awful.

 

At least I brush my teeth. :::chuckle:::

 

My dear doc (We’ll call her Dr. “W” for being Doctor Wonderful) did ask me to return to the allergist / immunologist, though, just to see if he might be able to pull something out of his 40+ years of practice that might even be comforting – we were beginning to think that things really were getting down to comfort measures, and I secretly wondered at what point she was going to call hospice. Well, that’s when the allergist / immunologist reran the Ig series. He called me the moment he received results and said he needed to see me – immediately. I saw him last Wednesday, and we initiated an antibody challenge series (damn insurance companies…).

 

I really didn’t feel as though I should go anywhere Thursday – I was worn out – but had two appointments and really didn’t want to cancel, both out of respect for those two clinicians and because I really don’t want to rack up more co-pays without at least going in and touching base – especially if this were to be a terminal situation. Being sick – and especially this sick for this long – is a horrible drain on family finances and impossible to budget for. I mean – how do you save on a hospital visit that saves your life? You don’t.

So, Thursday I went to the docs. The first one I saw was very, very concerned not by my demeanor. On one hand, I was pragmatic, and determined to finish up the things I needed to do to return a full draft of my dissertation to my committee so I could at least get through the oral defense as quickly as possible and perhaps (and yes, this is what I was thinking) be awarded my degree posthumously. Her worries were, though, based in the radical physical changes she saw that had occurred over the month since I had last seen her. When I saw her partner, who practices in a related field, she took a look at me, refilled what scrips I needed, and then – to me she somehow just looked very angry – ordered me to go sit in the waiting room. All I could think was that – well, all I could foresee – was being fired from yet another practice. But I was just so far past caring that I just sat there.

She came out about ten minutes later and said, “You need to be at this doctor’s office ::pointing to her card, on which she had written down a doctor’s name, address and phone:: within the hour. He is expecting you. Now. Go. Call me after you are seen and we will make a return appointment.”

I thought, “Dangit! (not the actual word!) I am far too tired to do this.”

But, he was a rheumatologist / immunlogist – all wrapped into one. Maybe he might have an idea – and again I was thinking palliative care. For the sake of at least final clarity, I went.

Now, as with so many who deal with this THING that never seems to be acknowledged, never differentiated, never diagnosed, I carry medical records with me when I am going to go on an appointment – whether it is with an individual physician or the ER. I should say MEDICAL RECORDS. A lot of them. Files. A lot of them. I gave Dr. “X” (for X marks the spot!) everything he asked for, and he sat down with me, went through every single blood test result from the past year; discussed my medications, diagnoses, symptoms, etc.; went through the list of every single surgery I have ever undergone; asked questions about childhood illnesses; performed a brief, but very focused, physical exam. Everything he needed to evaluate it.

He was most interested in all the immunology workups I had with me – especially those from the ding-dong hematologist. Thank goodness I held onto the test results with an iron fist. Being a pack rat, in this instance, paid off.

And he had an answer. For the first time in my life, there was a definitive answer.

I have CVID.

Dr. X told me the basic information, what I needed to know to get us moving, what additional tests he was ordering, what I needed to know to learn more (on the Internet, natch’), and what he proposed for treatment.

Now, I have to level with you. I had suggested CVID to the ding-dong hematologist – and it was dismissed out of hand. Somatoform was easier, I guess. So I had put it aside even though it felt right. I didn’t pursue it further. It just no longer seemed worth the time, effort, energy or care.

Well, now that I know what it is I feel more peaceful and certainly more purposeful.  I have bursts of astonishing anger sometimes, but I feel otherwise somehow at ease in my skin in a way I never have been ever before in my life.

The anger is somehow not so much about the fact that it took so long to be diagnosed per se, but rather that it has taken this long to prove that I don’t lie, that I am not a shirker, that I am not a dawdler… I AM NOT A HYPOCHONDRIAC. THIS IS NOT IN MY HEAD. THIS IS NOT FAKING OR IDIOCY FOR ATTENTION… THIS IS REAL. This health doomsday course I have been on has been quite serious – and now that I know, it has been far more serious than I had imagined. (I’ll talk more about my relevant health background at another time – saying these things, here, is far more important at the moment.)

Oddly enough, my greatest relief right now is not so much in the hope I have for a better life with treatment, but rather that I can look every single person who has ever doubted me, who has ever thought any of those things about me – employers, academics, friends, family – in they eyes and state clearly and for the record that I do, indeed have an illness, and I am not the assumption you have made about me. I no longer feel as though my ethical framework – or my honesty – is in question. Because, for me, nothing more matters than the ethical life lived.

And nobody can ever question that part of my being. Ever. Again.

That is it in a nutshell for now. I’ll catch up with you soon.

Sincerely,

One in Fifty-Four Thousand < — the ratio of cases of CVID in the general population today. I read earlier today – but haven’t had an opportunity to confirm the numbers against the current population of the US – that only roughly 6,300 people in the United States are fighting this disease – and just 140,000 worldwide. There may be many, many more, but very few know how to diagnose, much less treat, this condition. Education is the key to solution.