Reallly? So long?

Hello friends.

I suppose some of you have wondered if I have either perished or simply dropped off the earth. It seems to be some of both, and I admit to being rather down tonight. The past 15 months since I wrote last have proven quite difficult: I am taking my fourth different formulation of immunoglobulin, one of which brought with it two very serious cases of Aseptic Meningitis Syndrome (AMS); our insurance situation abruptly changed, so that well we do still have insurance – and I suppose it is fairly good insurance – the costs of it for some of the things I end up experiencing all too often have climbed more than 1000% (such as extensive in-patient hospitalizations); I have not yet finished my dissertation, as there are days and sometimes even weeks when I feel to unwell to even rise for meals; roughly a month ago I was placed on home health services, switched to subcutaneous immunoglobulin infusions, and put on notice that these four walls will become very close and too familiar (my last IgG numbers tanked and I promptly caught my fourth case of pneumonia in 27 months); and, in the meantime, I have been diagnosed with two additional digestive tract illnesses that will require surgery as soon as possible, but which cannot be considered until I have more strength – hence twice-weekly physical therapy as I am able. It’s a lot to deal with.

I will try to take this all in the order in which I listed it.

Mid-November 2013, I received my usual IV dose of Privigen. That Friday I came down with intractable migraine – or so I thought. As a lifelong – and now daily – migraineuse, I simply assumed this was yet par for the course. However, by the next evening, I simply could not see, I could not think, I could not bend my neck or turn my head, I could not touch my toes, I could barely remember the previous several days. Light made me scream despite the pain it caused. Clearly something was radically wrong. My husband realized how different this was from anything else I have ever experienced in our marriage and he rushed me to the emergency room. Without hesitation, I was whisked back to a room, hooked up to monitors, set up with an IV, and had a large amount of blood drawn. A chest x-ray and head CT soon followed, as did a urine sample. I do not know how much time passed in a blessedly dark and quiet – negative-pressure isolation rooms assist with more than communicable disease containment – but my husband stated that very quickly after I returned from CT, the physician returned and stated that he was nearly certain that I had either meningitis or encephalitis, and that he recommended an emergency lumbar puncture. I think I remember telling my husband this was the right course but I cannot remember for certain; I do remember he signed paperwork and I was glad despite the pain I knew was coming; someplace in the back of my head either diagnosis made sense. Again, time made no sense, but it seemed as though it was also a very short time between the lumbar puncture (which I do not remember hurting, possibly because everything else hurt so desperately) and the diagnosis of meningitis. They believed it was viral, though I did have a bacterial load. However the bacterial count was not high enough to consider bacterial meningitis – thank goodness, because we really had waited too long had it been bacterial meningitis. Were that the case I would likely have been dead already.

I was moved to a room on a medical/surgical floor next to the ICU, and given copious IV fluids and pain medication on demand (though I believe my husband said that during the first 24 hours I was given pain medication on a four hour schedule – I do not remember). A few days later I was released from the hospital, but we returned almost immediately because the headache became suddenly much worse and I could not tolerate any changes in position or even going to the restroom. Any change of pressure or movement affecting my head hurt, perhaps even worse than the original meningitis. Rush return to the ER.

This time I remember being extremely combative and refusing to cooperate with nurses and physicians. My spouse (let us just call him S from now on – that is so much easier) was able to work with me to allow the nurse to insert the IV line, but he said I was virtually incoherent and very combative with the doctor, refusing to remove the layers and layers of turban-like cloth I had wrapped around my head to shut out light and sound. Eventually, I suppose with a sedative and pain medication on board, he was able to conclude his physical exam, and was certain that this was a continuation of the meningitis, and possibly a leak of cerebrospinal fluid from the lumbar puncture several days prior. I do not think I have ever gone to a room so quickly from the emergency room.

A day and a half later, I was still in unrelieved pain, finding it difficult to communicate and still struggling with combativeness. This was Thanksgiving week in America (I know I have some followers overseas, so this translates to the last full week in November; Thanksgiving Day is that Thursday). S had much of the week off work anyway due to the holiday, and he rarely left my side. Given that what was being prescribed was ineffective for the most part, and narcotics for making me exceptionally nauseous, we wanted to come up with a different treatment possibility. As the anesthesia group felt I was to medically unstable for an attempted blood patch, the covering doctor that weekend (I think the weekend actually begin on Friday due to the holiday) came up with a unique solution: she prescribed massive doses of IV Tylenol for a 24 hour period, a wide open IV line with nutrients and very dilute Phenergan as I was so nauseous it was difficult to cope (I have great difficulty finding nausea relief, and Zofran is simply ineffective), and absolutely flat on my back bed rest, including a Foley catheter – still with the option for narcotic relief, which I refused. Evidently, IV Tylenol works by a different mechanism than oral Tylenol, and while it can take a little longer for relief, it does seem that this is an excellent alternative to narcotic regimes in certain situations. But, I still fought with irritability and anger which we worked with medically, though crying jags constantly occurred despite the pain they caused. This was one of the worst things I have ever gone through in my life. I kept insisting on being released against medical advice, but the physician and S soothed me enough to make it through each additional day. I knew I was being played, but I also knew S would not let me go home if he could help it.

By the end of the weekend I was weak but at least medically stable. I was released home and for the most part slept for two weeks. My intention had been to complete the rough draft for my dissertation no later than the end of November – and I was well on my way before I became ill – but, well recovering I found that I was unable to think clearly in my short-term memory had been affected. My immunologist and I decided mutually to attempt the Privigen one more time, and four weeks to the day from my previous infusion, I received another IV dose. Unfortunately, history repeated itself. And this time, it was Christmas week. Such luck! I was finally released, feeling very ragged, and more than a little defeated, right around New Year’s Eve.

We chose to switch medications at that point; obviously the Privigen and and my metabolic system were not getting along. We made the decision to go with IV Gamunex-C, and after a week of exhaustion I began to feel much better than I have felt in decades. By the end of the month however, I felt as though I had been hit by a tank; it was as though the medication had simply run out on me. Another two months of IV trials, and we decided as I hinted above, to go with the subcutaneous version. The very low IgG count, obviously, had a great deal to to with that. That was four weeks ago.

The past four weeks have also been a bit of a blur. I am exhausted all of the time, and find it difficult to take interest in much of anything due to the exhaustion. What had been, in December and January, “simply” short-term memory loss and difficulty reading had become good memory, but no reading, no writing, just a constant fog of exhaustion. I assume I am adjusting to the medication – at the moment, for my sanity, I feel that is what I must assume. I will be going in this week for my first blood work since starting the subcutaneous version and will know early the following week – or perhaps by the end of next week – where my IgG levels reside at this time. I suspect they are lower than usual, and this downward trend (and it has been a trend for several months) remains quite scary to me. My IgG has not once gotten above 853, and while that I suppose that is more to celebrate than the low of 164, everything below 700 feels like a loss – a big loss. I DID have one exceptional days this week, though. Thursday, I felt almost well. But I must have lifted one too many plates, or typed one character too many, or sat up for one minute too long, or moved one muscle too many in physical therapy, because since then, everything has reverted to that backward-sliding, zero-sum game I seem to be playing.

While I sit here in the bathtub dictating this – yes, dictating – I am so very grateful for Dragon Dictation – my cat purrs, and my body relaxes. It seems that bathtubs and cats remain among my few joys right now. The sun aggravates me, not because it is sunny, for I enjoy that, but because it is springtime, and I am a gardener who cannot garden and cannot tolerate sunlight due to medication.

Amongst all of this are the never ending medical bills, increased insurance premiums, a deductible – which is something, actually, we have never had, increased co-pays, and what seem like constantly increased costs of living. Meanwhile, I have been told it is improbable to impossible that I will ever return to teach in the classroom again – a talent and joy I never expected nor knew I had until a decade ago. I feel as though the rug has been pulled out from under my feet. I cannot even imagine a time when I will be able to have stamina or the reliability to teach online for a full semester again; I have not once managed to be well enough even six weeks in a row to be reliable even on a day-to-day basis. This well and truly sucks.

Or, to be far more specific, this well and truly sucks impossibly big boulders through a very narrow and bendy straw.

There have been far more illnesses and far more difficulties then I can possibly relate in this space at this time. For one, it just seems too overwhelming, and second, how am I to ever keep this all straight? I have all of the medical records, I have all of the notes, I have all of the blood test results, I have all the data. But for all of that, I cannot tell you the toll it has taken. I am glad for the extra year I have been given somehow – I was told so many times I would not live, and certainly not to my birthday last year – but, I do wonder if there will ever be a time when I can look back and say I have truly lived. It seems as though that ended so many years ago.

I smile and I effuse and I express positivity, and on that basis so many have taken me less than seriously. I know no other way to cope in real life. If I do not try – if I do not at least look like I am trying – life seems even less inviting. Perhaps it is good, then, that you cannot see my face, that you cannot be led astray by a smile and straight teeth. Perhaps it is well that I can write.

Because, perhaps that is all I can do.

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It’s been a rather extraordinary road

I’ve had a rather frustrating – and thoroughly angering – few weeks.

Last Monday I fired the rheumatologist / immunologist who has done nothing but sit on his thumbs and spin. Finagled a work-in with another group. I had actually met three docs in the group at another time and *very* much liked them – so a good feeling all the way around there. So, we hit it off and are heading toward them taking full care on the 1st of January.

Well, Friday night, I received the oddest of odd calls: it was a group asking me to rate my experience with CIGNA Home Delivery. Huh? I haven’t ordered front them in three or four years – and certainly not recently! I hung up with this survey group and called CIGNA to find out what was going on.

Ends up that the doc I had fired had gone ahead and ordered the Gamunex and it was sitting in his office! I was pretty hot about it! But there was really nothing to be done. I had come down with an ear and sinus infection, and have been rapidly feeling crappier and crappier.

So, that is what I am doing. In about two hours my infusion will begin.

I’ll fill in details another time… But I wanted you to know this much, anyway.

One in blah… blah … blah ….

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Filed under allergist, chronic illness, CIGNA / health insurance companies, CVID, deficiency, depression, ethics, gastroenterologist, hematologist, hope, IgA, IgG, IgM, immunoglobulin, immunologist, infection, knowledge, migraine, pain, relief, rheumatology, support

Here it is December – and I am coming out as The Grinch

Anger topped my charts yesterday.

When was it I was so upbeat and stated that I had been approved for everything – the works, including the Gamunex-C, etc?

October? Late September?

Well, guess what. They STILL have not ordered the medication. I am out here hanging, living on borrowed time and hopes I will not contract something else, while my rheu/imm doc’s office implodes on itself. So, I spent yesterday (Friday – I realize that by the time I post this it may not be even Saturday HERE anymore, either!) calling other practices in town to find SOMEBODY – ANYBODY – to take on my case. I have some good leads, but given that I was calling on Friday, well… most had already taken off for the weekend.

This is unconscionable.

Yet another kidney infection since I last wrote. Yet another chest infection. Yet another sinus infection. And then, with all the antibiotics, a huge, multi-organ yeast infection. I am so angry I could scream! What does it take to get anything done around this medical community!? My death?

I don’t often allow myself to think of the mortality rate of pneumonia patients with CVID. I’ve had pneumonia three times this year, though, and am not only run-down, but at an astronomically higher risk for a fourth bout than virtually anyone in the “average” population. The sinus infection will not abate, and I am back to horrendous diarrhea. My usual food intake each day consists of part of a potato, and a small bowl of frosted mini-wheats with a banana on top. And I work all day to drink, drink, drink a mixture of Sprite and Ensure Clear (The peach Ensure is nauseatingly sweet, but the blueberry pomegranate more than makes up for it!). And that is IT. I haven’t been able to manage much of anything else for how long? It’s hard to remember what it feels like to actually sink my teeth into something really and truly solid and tasty. I try very hard to not grind my teeth in frustration – any of the frustration – or the emptiness.

I’ve lost so much weight that when I reached into the closet last week for dress-up clothes I found that none of my nice blazers fit – including a gorgeous filigreed velvet one I have dearly loved. I pulled on a pair of jeans yesterday and found that they have gone from tight last year to nearly falling off this year.

baggy clothes december 2012-001

Image

Here I am yesterday just as I came home from an appointment – dissertation-related. While I am not skinny, I most certainly do not weigh the 287 to which I had once ballooned. 122 pounds down so far… and no stop to the slide yet… I would show you my face – but right now I am fairly jealously guarding who I am.

So now I will morph away from my anger and see where I end up by the time I decide to stop this for tonight.

I purchased the coat, above, at a thrift store, along with several other items; just cannot afford to replace my wardrobe in its entirety every couple months or less, so I concentrate on purchasing nice items – more on the dressy end – at local thrift stores. Everything else is… baggy or incomprehensibly large. I have $400… $500… $600… $700 suits (no, I *never* paid that!) in the front of the house, awaiting resale, not to mention the few remaining designer clothes I had modeled (they had been carefully fitted to me when I was doing private/boutique modeling at one of the big Chicago merchandisers – and since my body shape has changed dramatically, I don’t think they will ever fit properly again) that are still in the attic. In my closet I have a few items; in the large drawer in which I keep my knit and other “fold-able” clothing only a few things actually fit; and, on the shelves in the closet… well almost nothing. I have to be quite creative to be comfortable. Most everything I feel truly comfortable wearing is lined up on the shower curtain rod in the bathroom so it is easy to quickly mix and match when I get up late because I am too damn tired to move.

Working on the dissertation is difficult, but it *is* progressing. I just wish I could plan each day’s schedule in advance. I have purchased some software recently to assist getting things done – terrible pain in my joints and muscles of late – and especially helpful right now is Dragon software from nuance.com; I am speaking portions and pieces and parts of my work – as well as things like this. It was easy to pick up, and I am grateful for the ease of at least that.
You know what? Talking my dissertation almost feels like teaching.
And that, my friends, feels good. I am glad for the little things.
Love,
One of the 200 Among the 54,000 Among the 72,000,000

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Resource Information

As I receive healthcare benefits through CIGNA, their website is a go-to for me for policy statements and other data I need to fight through their red tape. Fortunately, they have been very helpful in the some half-decade during which they have covered us. May I direct you to two items?

One is this site http://www.cigna.com/individualandfamilies/health-and-well-being/hw/medical-topics/common-variable-immune-deficiency-nord807.html

The other is this .pdf file. I have highlighted the information relevant to my situation – you may find there is other information on there that could help you in your specific situation.

CIGNA_coveragepositioncriteria_Immune_Globulin_Subcutane

Note this language:

Although the exact prevalence of PID is unknown, an estimated 25,000 to 50,000 people in the US have the disease. Approximately 400 children are born with serious PID in the US each year. Although PID can act on any part of the immune system, approximately 50% of cases involve antibody deficiency.

So, according to my understanding, all PID is not equal. If 50% of PID cases involve antibody deficiency (basically the definition of CVID), then we are looking at a US population of between 12,500-25,000 total with the disease (see caveat below). This also means, if the logic follows, that, of the 400 children born with PID, roughly 50% of the cases, or roughly 200 births, involve antibody deficiency.

Caveat: Since CVID is only one of several types of PID fitting the 50% criteria, the actual number of individuals with CVID is a subset of the 50%… perhaps as few as 25% of that subset – or more. The prevalence is not well know.

SO, while we are fairly certain that half of the PID population has antibody deficiency, the amount of that deficiency class is yet smaller. The most accurate statement about it I can make is that we are of 200 births per year – and likely between 25-75% of that…

Makes us rare gems, doesn’t it?

Love,

One of less than 200 yearly births – or part of a population of 12,500 to 25,000 in the US today (might as well dispense with the One-in-54,000 stuff….)

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For Matt – and For Me, Too, I Think…

Matt –

Pardon me for a moment as I single you out and offer you some love and support for the very tough time you have been having. This is, perhaps, one of the cruelest diseases I know, not because it is necessarily fatal, not because of the cost of the treatments associated with it, but rather, because of the sheer callousness of so many of the medical professionals who see us – and dismiss us out of hand thinking we are hypochondriacs of the worst kind. We all know there is something so not right within us – but as you so clearly pointed out regarding stupid assumptions and unhealthy “diagnoses”, I am actually to the point that if one more damn person decides to state that our illness has anything to do with somatoform problems of any sort, I think I will take them by the throat and send them to the raptors, fully trussed and ready for dinner. I have zero tolerance for this junk. I just wish I didn’t always have to be my own advocate… but nobody else – even Love – can refer back over 44 years of medical records in the way in which I can. Now that there is a diagnosis, though, I am a tiger and simply will not take “no” for an answer on most anything and everything. I am almost a jerk about it.

Fired two GI docs in the past two months over it, even. I’ve found a great GI doc – and an esophageal specialist – in the same practice – where I had never thought to look. I think they will work out well.

Yes, the lack of energy is horrible. Does it physically and mentally hurt to kick yourself out of bed most mornings? Nobody has ever fully understood that, but the pain can sometimes take my breath away. That may not be a symptom everyone has, but it is the most consistent of the ones I have notices throughout  my life, because I face it every single day without fail – even when I feel reasonably well.

I have wondered about the chronic sinus infections coupled with the “allergies”. Before I ended up with the rheumatologist / immunologist I am seeing now, I was seeing one of the best allergist/immunologists in town. He couldn’t find a single verifiable actual allergy, though I was constantly in a very reactive state. Between the two of them I have come to understand that our immune systems are so wonky that they just throw into the arena reactions to anything and everything; the immune system can’t properly recognize most anything in our environments, and when it find that it’s irritated, there’s no telling what reaction we will experience.

And, as with you, things have consistently – and more recently, rather alarmingly – gotten worse. As you said, an increase in the frequency of illnesses coupled with the fact that they hit us so much harder. My rheu / imm explained it to me this way: our bodies have been fighting bogus enemies for so long that when something really comes along (and sometimes, just for the heck of it), our bodies react much less positively – and much more self-destructively – though the immune system has no idea what it is doing, frankly. I know when I finally hit that point that I stopped socializing, stopped going out in public, stopped teaching in the classroom (something I dearly, desperately love), stopped being me.

And yes. I have always always always slept more than anyone else I know or have heard about. So glad to realize now that not only am I not alone – but that there is an honest-to-goodness reason for it. A legitimate – albeit very serious – medical reason. It’s not the depression (and yes, bi-polar as well) I have fought all my life. It is actually the other way around.

Move to Oregon? Really? Nice place, nice people – but what a convenient way for that doc to evade any sort of responsibility!

I, too, started this fall with a dx of hypogammaglobulinemia. It wasn’t until I saw the rheu/imm that I had even ever heard of CVID.

I also must admit that I gave a rueful chuckle at your description of your schlepy medical file! I mentioned in my post earlier today that I am in the process of scanning all the medical records I have in-house and that I am close to filling a 16G flash drive with it all. Once I pick up all the records from August through present I will be well over that. I don’t know if I should laugh or cry.

My insurance company has, fortunately, been quite accommodating and helpful. Then again, I think I may have spoken in the past three years to every rep the have in the United States. I have their phone numberS on speed dial, alongside the names and positions of those whom have been most helpful.

I see you have been taking gammaguard – I wonder if the Gamunex will be a little less side-effect-filled.

You know, you said something I have been thinking for some time: I have been just certain that part of the reason my tummy refuses to work properly is due to “improper” gut flora, and it is quite, well, fascinating to find that it appears that this may be a common issue.

Do you find yourself doing research and advocating on behalf of what you think doctors should be doing as part of their JOBS? While I realize this is not your problem, this illustrates things well, I think: I had an emergency hysterectomy 2 1/2 years ago. I had almost never had periods ever in my life, and we had been unable to have children. I developed severe pain and heavy bleeding suddenly in mid-July, and was on the table within a little over a week (the wait time is usually 4-6 weeks or longer). While still under anesthesia, they slapped a progesterone-based hormone patch on my belly in an effort to prevent hot flashes. However, within a matter of months I was experiencing hot flashes but HOT HOT HOT FLASHES that never ended – I was so hot that last winter I *never once* wore a sweatshirt, sweater, sweat pants – nothing like that; my summer clothes received continuous demand! Well, I finally sat down, reviewed the female hormonal cycle, and realized that, not only had I lost a massive amount of estrogen when I had a serious breast reduction, and I had lost a huge amount of estrogen-producing fat stores when I lost all this weight, AND I had almost never experienced progesterone in my life (I stimulated my periods twice a year with a short and miserable run of progesterone)… so… it dawned on my that what I needed was not the dang progesterone – but ESTROGEN. Sat one of my docs down, explained it to her, and she looked at me – the chagrin obvious – and wrote me a scrip for estrogen. Needless to say, I feel far better on that note than I have, well, to be honest, in my life. Crazy to need to think these things through because you know your body better than nearly anyone else…

And one other thing.

Congratulations. It is one of those crazy miracles in the world when you and someone you love so well manage to find each other. Congratulations and best wishes. You are both winners.

Much love,

One of the rare 200 of the 54,000…

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This One’s a Doozie

Whoo-boy…

I don’t know when this particular rocky road became so boulder-filled, but I swear I hit a cliff face without a helmet going 100 late September / early October.

You see, I don’t even remember most of the last week in September.

The last time I really wrote anything (back in August) I was feeling pretty rough, and either that day or the next landed in the hospital for several days. Bowel issues and endless nausea – it’s not good when all your body wants to do is expel, expel, expel – and yet not. But things seemed reasonably ok when I was dismissed just as September came around, and I returned home to rest, then started to add to my schedule, little by little.

Managed to do some research for my dissertation on-site in a couple archives and was really optimistic things were going better and – WHAM – I started back in with the severe nausea and vomiting (I had been nauseous and occasionally vomiting the whole month – that hasn’t substantively changed in more than 11 months). This time had a different feel to it. I wasn’t starting from a good place – I was still tired from the August / September hospitalization, and I had not been able to build any reserves. Especially concerning, I was unable to consume even the regular chocolate Boost that had been sustaining me since last December (December 2011). Not to be nasty, but it was as though I had lost the ability to break down any of the contents of the Boost and was “returning” the “meal” entirely encapsulated in this vile, nasty yellow vomit. And vomit and vomit. More than a gallon a day. I stopped processing any and all oral medications; they came back as encapsulated foam and bits of pills. Even water seemed impossible.

By Sunday, September 23, I was struggling in many ways as I never had before. Then utter weakness set in and I sometimes didn’t make it to the bathroom – just mere steps away. I wanted to cry but was too dehydrated to make tears. Very frustrating feeling, actually. Very.

Managed to make it to my general practitioner’s for a shot of Phenergan in my butt Monday early morning (no, I did not drive), but never stopped vomiting – the meds didn’t even slow it down. We tried Phenergan Gel, dissolvable Zofran… lots of things… to no avail. BTW Zofran has NO effectiveness with me at all. And that is something I cannot seem to get through the thick skulls of ER docs and nurses. I would rather have a big, painful shot of phenergan in my butt than spend the time and money waiting for Zofran to “work”.

Either Monday or Tuesday (Monday, I think), I was vomiting in the toilet and absolutely could not draw a breath. I don’t actually remember passing out, but I do remember hitting my head on the outside of the pedestal sink, then waking up sometime later, again vomiting, this time at the base of the pedestal. No idea how long I was out. When Love came home I didn’t remember to tell him; he would probably have rushed me to the hospital at that point, but I was getting more and more confused and dehydrated. Terrible.

Somehow Tuesday came around, and I must admit I am terribly unclear what happened next. I’ve been sitting here talking with my husband with a notepad in hand, and this is the best sketch I can make; almost all of this is from his memory:

Evidently, the primal part of my brain realized I was in trouble because when he came home from work I was on the phone calling doctors for advice and guidance. I called our GP and the response was Go. Go NOW. You CANNOT afford to wait one more minute.

I checked the ER Wait Time (6 minutes rolling average at the time!) and scheduled the ER appointment (yes! You can do that here!). Love said that I scheduled the ER time two hours ahead so there would be an opportunity for him to eat dinner (how funny! And how sweet, I think, somehow; I was – and am – more concerned for his welfare than mine) but he really pushed me out the door – I crashed and crashed and crashed. He managed to wheel me into the ER (how did he get me out of the car?), sign me in, and go park the car, but at some point just after he returned to the ER waiting area I tried to get up and lie down full-length on the floor. He diverted me, and I ended up on a bench (?), vomiting. (I think it may have been two of those ER bolted-together seats – I don’t know). He says they triaged pretty quickly, and dumped me into a room quite quickly after *that*; evidently I was passing out as I sat in triage. I remember being terribly, terribly cold – and that I was so dry and weak that I couldn’t vomit anymore – the vomit was like a white, sticky web and just dribbled out my mouth, making me choke and panic. I remember him saying “Calm down. Breathe in through your nose. Stop fighting the vomit. Let it come. Let it come. Calm down. Breathe in through your nose.” Over and over again. I think he was scared silly, frankly.

Fortunately – how did THIS happen two ER visits in a row? – they managed a peripheral line on the first try. Love said it was pretty amazing – I vaguely remember his “WOW!” And then meds. I have no idea what they gave me, but it seemed to help ease pain and nausea. Quick admit, though – I was in a room within three hours. Clearly critically ill.

Love says the ER doc was concerned about the accumulated radiation of the past several years. Random thing. And yes, there is room for concern. Just an odd time and thing to say in the midst of a crisis.

In room and intense nursing care. I do know they called the crisis team several times while I was there, but I couldn’t tell you when during the week. But, frankly, I was ready. If I went, I went. The only thing I worried about was that Love would be alone and I feared for him. So terrible.

Well, Sunday evening one of the nurses came in with a floor-nurse phone and handed it to me; the doctor was on it and wanted to know if I wanted to come home. I said something along the lines that I would like to but didn’t know if had the energy to do so. Next thing I knew, I was being dismissed. Stunned doesn’t cover it – I still couldn’t keep down solids or liquids!!!

Spent the rest of the week vomiting violently, and Saturday we went to one of the minor med locations in two. I was so dehydrated that my bp was 73 / 39. Once again rushed to the ER.

Terrible, horrible, miserable time starting an IV (8 big, bruised attempts)… and things just deteriorated from there in my nursing and general medical care. Didn’t matter I hadn’t eaten or kept down anything since the middle of September – they made their own assumptions, and my care suffered. And example? I started complaining of serious pain at the IV site – but it wasn’t until I was dismissed 37 hours later that they removed the offending issue – and by the next afternoon I had developed a bad site infection. Anyway, one thing led to another – and how – and I ended up being booted out of the hospital – still unable to eat or drink. (At that point they had also convinced themselves I was forcing myself to vomit! I am sorry, but when you gag and dry heave for HOURS on end, and then, when the puke comes, you vomit incessantly for hours, that is not a joy ride… pardon me, but they are asses – and I feel fortunate that I have the ability to choose a different hospital provider within my city).

I have been home since then, except for the endless rounds of doc appointments. At one point I lost 20 pounds in 45 days – and had been trying desperately the whole time to at least hold steady (I’ve lost something like 53 pounds since this time last year. Hah. Holding steady on WHAT planet?). No, I am not emaciated – and that damned “you look pretty good, so I don’t think we need to concern ourselves with IV nutrition or anything like that yet” crap is just getting to me, getting to me, getting to me. Have been able to get down a little more than “usual” this week – but everything is punctuated by nausea – and some vomiting. I worry that I am developing some sort of illness-related anorexia: I am so completely and utterly frightened and scared of the idea of vomiting even One More Time that I would rather not eat than risk doing that. There have been days when a glass or two of juice / tea have been all I can get down – and go through days and days on end of being unable to eat. Better luck the past week, and there have been three days when I have been able to get down “breakfast, lunch and dinner” if only in the most rudimentary fashion (several bites here, there, and another). It’s a rare thing to actually be hungry – but that’s the worst. I cannot manage my meds – and certainly  not in the proper dosages. I swallow what I can of he enteric-coated ones – and everything else is crushed.

Been calling the immunologist’s office for weeks trying to figure out where we are on actually GETTING STARTED on the IVIg – and can’t get the person in charge of my case to even call me back. I have another kidney infection, have had another bout of pneumonia AND I feel lousy today. Frankly, they are going to regret not calling me back, because I am going to devote tomorrow to getting answers and being the boil on the butt of a cavalry officer. I have been approved for the IVIg. I have been approved for Gamunex. At this point the hitch is, evidently, that the person in charge of all the approvals and ordering, etc., has been looking for the “most cost-effective way of doing this.” As I told the office today, I couldn’t care less about cost-effectiveness at the moment; I need help – and NOW. I just cannot afford another major infection. With a third bout of pneumonia under my belt and the 11th kidney infection of the year? No. Stop futzing around and work for cost-effectiveness next round. We have other priorities right now.

The dissertation is coming along, though, and I should finish my solid rough draft mid-month next month.

At least something is coming along.

A more cheerful post coming in a few… but I had to get this out. I am sure I am not alone in this treatment or these attitudes. Why does most of the medical community think they can romp all over us, assume we are mentally defective drama queens who have some level of Munchhausen syndrome (and act callously accordingly), give an inappropriately-low level of treatment because we “look just fine” (trust me – when I look like crap, I will, quite literally, be on death’s door…), and get so annoyed with us that they FIRE us from their practices? This should never be acceptable – and especially so with people with a clearly-documented illness – set of illnesses. The “medical records” rolling bag I schlep to each and every appointment is full to overflowing – and I am currently working (in between dissertation work) to scan every single document onto my hard drive – and onto a 16G flash drive. The time is not far away that I will need a flash drive double that size. But for now? Well, we do what needs doing.

Thank you for your support – and for your selflessness in sharing your own stories. I really don’t know what I would do without knowing you are there.

By the way. I was reading CIGNA’s white paper on CVID and found that the type of CVID I have is found in only roughly 200 live births each year. Will need to find that paper and repost here. Astonishing, that.

Much love,

One of the yearly 200 of the 54,000. I’m a rare gem. 😉

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Weary wind doth blow

I tried posting an update a few weeks ago, in between hospitalizations, but I just couldn’t manage it. I’ve been too sick. I hit the backspace key on two separate occasions, wiping out everything. Will be doing all but this post in Word first from now on.

However, no more testing needed; we’re moving forward with insurance approval for Gamunex. Finally.

Will write more, but I will be doing it offline, so it may take me until tomorrow to get something here. I’m pretty tired – and tire even more easily – since this last week in the hospital. I cannot imagine feeling decent, though the doc pretty well promises a health I have never had if I am able to tolerate the Gamunex.

What a thing this is.

Love,

A very tired minority

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A Thought

 

A thought tonight, if you will; it is turning out to be unexpectedly quite difficult on the health end of things.

Thank you so much.

Love,

One in Fifty-Four Thousand

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Considering Mortality

Albert Einstein: “The strange thing about growing old is that the intimate identification with the here and now is slowly lost; one feels transposed into infinity, more or less alone, no longer in hope or fear, only observing.” (1953)

And again: “To think with fear of the end of one’s life is pretty general with human beings. It is one of the means nature uses to conserve the life of the species. Approached rationally that fear is the most unjustified of all fears, for there is no risk of any accidents to one who is dead or not yet born. In short, the fear is stupid but it cannot be helped” (1953)

And again: “Now he has departed from this strange world a little ahead of me. That means nothing. People like us, who believe in physics, know that the distinction between past, present, and future is only a stubbornly persistent illusion.” (1955)

______________________

Some nights Einstein prowls in my head – not because I am as smart – or perhaps as honestly and fully as human – as he, but because he reflected on the human condition so honestly. He was also always ready – and sometimes even relished sometimes – to admit he had made mistakes. He was constantly as fascinated by the things in which we fail as those that succeed; there is so much education in the simple mistake. May we all be as humble in our learning.

But I have truly had a rotten day. And I while I am not willing to be terribly introspective within myself, I am going to try and push it out here.

I’m scared. That is the single most important monster I face in my life right now. Or at least the one that looms largest. I’m scared that 8 weeks of chemo will be too much for my body to handle. I am scared I won’t make it to IgG replacement therapy that would actually make me feel decent for the first time in god-knows-when. I’m scared I won’t live to be by my husband’s side as we grow old. So much fear can really sit on a person’s mind pretty hard.

I remember meeting the now-deceased :::sad::: Neil Armstrong many, many, many years ago. He had asked me if I had goals. I said, “Yes.” His response was, “Good. Always work toward your goals.” He asked me if I had any questions for him.

The only one I could think of was, “Why?”

He pondered it a moment, and then said, “Because it was my job, and as part of my job we made goals many hadn’t considered. It was my job.”

The last thing I asked him was, “Did you enjoy it?”
He kind of ducked his head and said, “Yes. I suppose I did.”
I am pondering this deeply today, not just because Mr. Armstrong passed away a few hours ago, but because I think I need to force myself to refocus on goals – real goals.
  • I know, with pinpoint accuracy, that I want to complete my PhD.
  • I know I want to continue to pass on the absolute joy and wonderment I feel when I teach.
  • I know I want to continue to present new things to new people, whether in writing, in a virtual arena, or in person. Those things that make me go “tick… EUREKA!”
  • I know, with the fullness of my heart, I want to enjoy the thrill of growing old beside my husband.
  • And I know I want to live. Not live sick. Not live partially. But wholly in the moment for the rest of my life, whether I die tomorrow – or 40 years from now.

THOSE are the things that are important to me, when you get right down to it

The last two are, by far, the most important. But I am doing that right now. Well, I confess – not all the time. That worry thing really gets me, and I open myself up to the fear, the scary, the doubts.
How do you move along when faced with a critical health condition?I know that many weeks ago I told my husband that, just because I couldn’t get out of bed some days, it didn’t mean that my mind wasn’t still active and growing and questioning and thinking. Maybe that is important right now. I am still imagining.
Last night one of our cats presented us with that most unwelcome of feline pests, The Flea. It took me a moment to catch it and … uh … put an end to it. But afterward, just as we were falling asleep, I murmured something to my husband about the fact that the flea was so tiny – its mass and size so small – yet it could jump thousands of times better and farther than its tiny little body might suggest. I wondered aloud at the physics of it, the math involved, the possibilities of how we could learn from this little beast I had dispatched with such ruthlessness. I went to sleep pondering if some form of it’s biological ingenuity might be put to use on places like Mars in order to be able to explore more of its surface. Yes. I fell asleep inspired by The Flea. Odd, that.
I guess inspiration comes from one place… and goals another. Or is it the other way around? Perhaps it is a chicken-and-egg infinite loop series. I don’t know. I don’t think I will ever know…I am just so very grateful for the people in my life – those who have touched me in passing, and ones with whom I pass time. Health kinda shrugs away when you consider the relationships you’ve enjoyed and the things you have shared whether from within – or without. Even those tiniest of little things that inspire ideas – or a smile.

And when you have dreams for the future.

Much love,

One in Fifty-Four Thousand

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Let’s Start at the Very Beginning – That IS a Good Place to Start!

I was born many, many moons ago – many eclipses and many changes in the earth have occurred. (:::laughing at myself::: Sounds like a bad “It was a dark and stormy night” Bulwer-Lytton Contest Winner – or runner up, at least!)

The reason I put it that way is because there are days when it seems as though I have been here for hundreds of years, moving in and out (mainly in) of fevers and illnesses and pain and discouragement.

That’s not to say my life has been bad. No, that wouldn’t be accurate. I have enjoyed so very many things, and reveled in experiences and situations of which I suspect few even dream. I’ve known famous, influential, rich, poor, average Joes and Joelles, stunningly brilliant, dregs of the human condition, joyous, sad, fascinating, dull – among so many, many others. I have  traveled widely. And perhaps most of all, I have known the very best love life has to offer – that of a steady companion and partner in life – my very best friend – who may very well be gentlest, most compassionate, and kindest individual I have ever known. I have known, above all, great joy as  I have traveled these roads. I have lived a good life so far.

And yes, I have been terribly, horribly, devastatingly ill more and more often from infancy to the moment of this writing, until it seems as though all I experience now is a constant stream of scary germs to be fought, or die. It’s that simple.

CVID is a disease that involves a whole family, not just the person with the diagnosis. It’s frustrating, it’s incredibly debilitating. It keeps you from firmly committing to meetings, and classes, and dates, and friendly get-togethers. It surprises – unpleasantly – even in the course of even an hour. It disappoints, it angers, it depresses. Actually – I cannot at the moment find anything good to say about it … except that it has finally been diagnosed. It is the root cause of most of my illnesses, and as I alluded to in a prior post, it has placed me on the “hypochondriac” list for far, far too many years. Even with all the good, this still lurks.

I remember an incident in childhood – just a moment in time. It was a Sunday morning and, as per usual, I was completely wiped out. It hurt to even roll over – but I couldn’t explain that adequately. Next thing I knew, my mattress was on top of me on the floor; I’d been unceremoniously tossed out of bed. It hurt, not so much that I had gone top over teakettle (or whatever one does when landing with a mattress on the floor…), which was probably pretty funny – but it was because nobody understood what was happening to my body. Another “lazy” moment.

I graduated second in my high school class some time ago <– a long time ago. I had worked very hard academically, and I excelled in some other areas as well. But it had come with a price: at least 30 days of missed school a year, every year from Kindergaten on up. The principal drew up paperwork two years out of three in high school to hold me back a year – and we fought it and fought it and fought it; I had never been out of school without a doctor’s note. Literally. The Americans with Disabilities Act didn’t come in until well after I had graduated, so it was quite difficult to push the boundaries much. But, we did, and I graduated on time.

Getting there was tough.

As with many IgG- babies, once I refused the breast, I promptly came down with a litany of infections. Strep – 13 times my first year and what seemed constantly even after I had my tonsils and adenoids removed (BTW – when my tonsils and adenoids were removed, it was June, I was well, and things appeared normal. However, once I was under and the surgeon made his first cut, infection spewed out, and I ended up very, very ill for nearly a month. IgG- does this.). Pneumonia – I just rotated through my 7th and 8th rounds of it. Juvenile Rheumatoid Arthritis for ten years. I mean – now that I know what this damned disease is, I can look back and see every single sign I should have seen – but even in my region this is a tough diagnosis, and the Cleveland Clinic didn’t exactly do wonders, either. My diagnosing doctor sees between 2-4 new patients with this a year – in a large metropolitan area advancing into a large hinterland. He may not be the only diagnosing doctor – but he is the only one I have found here. Ten minutes away. Opposed to 10 hours of travel to Cleveland for naught. Strange how things work out, sometimes.

However. In the meantime:

  • I’ve had a hematologist tell me I am making this up (despite blood work nobody could fake and results sitting right in front of him).
  • I’ve had two doctors fire me for being “too complicated”
  • I’ve had a neurology GROUP fire me because I was “too demanding”

And so it goes. You have no idea how much I want to tell these groups and individuals “Up yours!” In the case of a few of them I plan to do some “doctor education” – but the rest? Pfft. Begone. I am proactive in my health – and have been especially so the past two years. I need answers. Or I am going to die. It’s that simple.

Bounce back a little:

I managed to work and attain my Master’s degree at the same time, but my health suffered – badly. Before I (rather abruptly, due to something that was horrifying and was said not even about me but to me) resigned, I had two heart surgeries and the stress was unbelievable. Nobody needs to feel on edge all the time on a job. Actually, I cannot imagine why someone would yell at an employee or say some of the things that were said. But that’s another story for another day – and only if I feel like wasting some breath. So, I took two months, rested, recuperated, and went to another school, in another career path. It was actually the path I had originally followed, but from which I had been derailed for a while. (and yes. at that time I had my diploma, my BA (in two areas), and a Master’s)

Started in a Master’s program at the new institution – did quite well, actually – 4.0 – and was allowed to move directly in the PhD program upon completion of the first year’s work. I was tossed in front of a classroom my first PhD day and bit my way through the first semester, until I really began to feel as though I had found what I want to do. I enjoyed the classroom immensely and the course work as well. Actually, it felt, somehow, like coming home. I was happy.

Then, second semester of teaching I ended up in the hospital for a month – yes, a month. Three surgeries and this “mystery” diagnosis they seemed to only be able to label as “IBS” – irritable bowel syndrome – and back home to recuperate. But, things just weren’t on a good track, medically. I kept it together at work and school, and toughed through some pretty tough three-hour classes – both ones I took, and ones I taught.

Going before my committee for comps was the worst single moment of my life; I was terrified. When I was told I had passed them, I was sitting in the anteroom, dark, not lit, at a table on a hard chair thinking I had ruined my life. But, my chair came and told me, kissed me on the cheek and told me I was on my way. I went to the administrative offices and did paperwork, then went to my office and cried for more than half an hour. Finally called family and let them know I had passed when I was no longer gulping air and hiccuping.

You know what came next.

I became ill again. Immediately. Managed to get the end-of-semester grades in on time, then just plopped out for a month, almost unable to move. Further, the stress of the situation, once relieved, seemed like a void in my life. I knew I was supposed to switch gears, and I certainly knew the focus (well, mostly!) of my dissertation topic. I felt, though, like I was grasping at straw in a burning barn. Not the beautiful-smelling new-mown hay, not the first bales of the season the rafters.

I have only seen one barn burn in my life, and while it was an amazing sight – it had been struck by lightning only moments before it became engulfed – it was tragic; animals died, and a season’s crop of feed and bedding was ruined. One heck of a lot of hard work. That was how it felt, though: amazing yet somehow discombobulated. It took forever for it to dawn on me I had passed comps.

That was the last big thing that happened in my life before my grandma’s death: I was able to tell her I would gain my doctorate.

I decided in 2010 that I would make that year my “Get Well” year. I arranged for some elective, but major, surgery… and, quite unexpectedly, ended up with a second, emergency, non-elective surgery 7 weeks later. The first surgery went quite poorly, and I had nothing left to fight the second. Landed me on my butt for quite a while, and I had to cancel a slew of speaking engagements I had lined up for Fall Term. After finally finding my ABD PhD feet I was knocked – hard – to the ground.

The path from there has been far from straight. I have had to give up teaching – even online – even online courses I developed – and focus on the dissertation and my health.

At the beginning of this calendar year, I sat down with my chair, knowing how ill I was, and told him that, if it were in my power, I would make certain that at least I had graduated before I succumbed to this – there were nights I would ask, “Is this the last breath? Is this what it feels like to die?” I taught online for two months and ended up in Cleveland, so sick I could barely move. I had gone on my own, without a support person (until J – thank you J!) and made tests and appointments – 8 of them – in two days. The next day was a Saturday, and I realized I was in big trouble because I couldn’t breathe right – and I couldn’t remember my birthday. I had tried to eat up there, but everything was a no-go, and had only managed some water and a little Sprite. And, perhaps, a few harvest cheddar SunChips. I know there were still some in the bag when I threw it out. J rushed me to their ER and I just couldn’t think what to say. I just opened my wallet and said, “Here.”

After a really and truly nasty 26-hour ER visit – it ranks up there AT THE TOP of all the hospital experiences I have ever had – I made arrangements to come home the next day. I made the reservations while in the ER, and was home the same time Monday. My birthday, actually. And it was that day I chose to stop teaching entirely. Not only was it in the best interests of the students, but it was in mine as well. And I cried and cried and cried – for days.

And that is where I will wrap up tonight. I never thought I would see my birthday – now I have hope for Thanksgiving and beyond. I am on one medication now that doesn’t seem to have helped (he said it would kick in within 48 hours if it were going to do anything), and the next step is a rather nasty chemotherapy drug. Point A to Point B seem to have been shoved aside to make for a new “Point B” with an uncertain Point C and beyond. We’re aiming for IgG replacement therapy before the end of the year. I hope so. I am exhausted as I have never been.

BUT

My husband is known for his dapper hats. If the chemo drug makes my hair (my very thick hair) fall out, then it all goes, and I will join him in bald-headed wonderment.

It will be a new experience.

With much love,

One in Fifty-Four Thousand

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