In the Infusion Rhythm

Wednesday - August 24, 2016

CVID is chronic. I'm in it for the long term.  This month, next year, for all months to come (and I hope there are many ...).  Like diabetes, my body needs regular "topping off" to function "normally". Insulin is administered multiple times a day for people with diabetes. Gamma globulin is infused less often to CVID "zebras" like me who do not produce our own immunoglobulin, whose immune system has basically run out of gas.  We are empty.  Our bodies just do not produce immunoglobulin to fight infections and we are constantly sick.  My immune system seems to have run dry in my mid 20s but it took 30 years of coughing, pneumonia and bronchitis, shingles, hashitoxicosis, and lymphoma before all the puzzle pieces came together, an immunoglobulin panel revealed my lack of immunity, and a treatment plan was developed.

Thanks to the wonders of modern medicine, I can boost my immune system with the gift of immunoglobulin derived from generous blood donors.  In choosing to have gamma globulin delivered via IVIG directly into my veins, I have opted for an infusion that should provide me some immunity from infection for a full month. Subcutaneous infusions are administered weekly (or biweekly through a new delivery system) through injections into the fat layer of the abdomen.  I confess that the decision between IVIG and subcutaneous options came down to scheduling and convenience. Once a month seems MUCH less invasive than a weekly schedule.  Yes, it's a full day but it's one and done. I also am spared of a subcutaneous "sac" of liquid waiting to be absorbed overnight. Also, how do you travel? How can you feel untethered from the relentless weekly schedule of that multi-hours infusion? So, I am happy with our choice of delivery system yet remain fundamentally wary of what I am doing once a month.

I am still unnerved about welcoming foreign blood products into my veins once a month. We simply don't know what we don't know. HIV-AIDS?  Mad Cow? Transfusions of tainted blood spread these and other diseases before the medical community was aware of the threat. It would be naive of me to trust completely that there is no risk. I am exposed to donors' immunities as well as possible pathogens. Yes, I will eventually borrow others' immunity to Zika as it becomes endemic and builds in the population. Yet medical knowledge has limits. Any rose-colored glasses were shattered (and then ground into the pavement) when multiple medical teams advised that a risky allogeneic Bone Marrow Transplant was absolutely necessary for my survival only to have Johns Hopkins say "sit tight", to hear UCSF agree that the BMT advice was "protocol but no longer advisable", and to remain lymphoma-free 4 years later without that extremely dangerous procedure. Consider me a cautious skeptic of all things medical. "Completely safe."  As far as we know.  For now.  I'll take that with a heaping teaspoon of salt and say "sounds good!".

So far, so good. No side effects and no illnesses.  Also, since I am trying (with limited success) to avoid Google, I am calm.  As was the case with my lymphoma and chemo, I am offering this overview of my benign, mundane CVID experience as a counterpoint to all the horror stories online.  All's well. Nothing to report. Smile, be grateful, and be hopeful.

Bottom line? I have not been sick all summer... BUT that is par for the course for these non-school months when I am quarantined from a classroom of children. The true test will come after Labor Day when I am fortunate once again to be working with young children in the petri dish of an active classroom. I am optimistic that my boosted immune system will do the trick and that I will avoid the debilitating coughs that are the soundtrack of my school year!

Still Crackling

Friday - April 22, 2016

A follow-up visit to my primary care physician confirmed no fever, revealed some improvement in my lungs, but surfaced some "crackling" that remains in my lower lobes.  The Levaquin has worked but this infection is pretty well dug in.  I will go back next week for another check with the understanding that I may need another course of antibiotics when this 10-day course is complete. 

I also received a firm line in the sand for the future:  just as in my neutropenia-watch post-chemo days, a 100.5 fever means an immediate trip to the doctor.  No more waiting.  When I hit that number, I get in the car.  Now and forever, I am not to wait it out.  This stubborn woman will no longer argue with the thermometer and trust in the efficacy of ibuprofen.  The thermometer will dictate my actions, not my brain or my gut.

Good news!  I have had no apparent side effects from the IVIG!  Yes, I am certainly tired but I am fine with powering through fatigue.  Also, THIS fatigue is probably closely tied to the pneumonia.  That being said, Monday is pretty well prepped and I am determined to rest up again this weekend.  That will be at least 3 weekends that this pneumonia has laid me low ... but I AM feeling on the mend :)

First Infusion; Pneumonia Makes it Tricky

Wednesday - April 20, 2016

I had intended to post as I used to - with my pre-chemotherapy Rituxan infusions - from my infusion chair, as a productive diversion from the monotony of serving as a receptacle for the drip drip drip of the pump.  My body had other ideas.  Yesterday's pneumonia diagnosis did NOT disrupt today's first IVIG but it did make it a lot trickier and less comfortable.

The infusion room will be my monthly destination for perhaps the next 6 months.  At that point, my infusion rate may be sufficiently established to avoid side effects and my "trough level" (starting IgG rate) may be at the low end of normal (800 versus my current "atrocious and dangerous" sub-150) when I sit down in the chair.  Then I may be able to have an RN deliver the infusions at home.  The infusion room is a lovely, quiet space with treetop views and a sweep of Western Avenue in Friendship Heights.  Everyone was professional and attentive; the WiFi was robust and, best of all, the RNs and Dr. came in and out to change bottles, adjust rates, take vitals while I slept without noise and with a minimum of disturbance and disruption.  Hallelujah!  Great care!  No headaches, no light headedness, no flu-like symptoms, lots of sleep.

As for the low point of the day, my pneumonia caused a severe reaction as the immunoglobulin for this first ever  IVIG began to build in my system and to attack the lurking bacteria in my lungs.  The result was severe tremors, so rough that I almost fell out of the chair at one point and was blocked from falling by the attentive RN who was evaluating me.  The infusion was stopped immediately, saline was run, prednisone and ibuprofen were prescribed, and we waited 45 minutes for those very disconcerting body spasms to dissipate.  Then we started up again at the lowest infusion rate; we weren't even halfway through the first of my 4 bottles of immunoglobulin when the tremors / spasms caused the pause.  Ah, well.  "It's going to be a long but less-eventful day," my immunologist said.  And that's when I just settled down to nap ....

"Baxter" to the rescue :) - a good omen

Another interesting point is that the immunoglobulin mix I am receiving has been scrubbed of all IgA.  I only show a trace of IgA over repeated tests thus demonstrating that my immune system really does not produce enough IgA on its own for my body to recognize it.  While my skeletal ranks of IgG recognize the IgG reinforcements flowing in during my infusion, research shows that IgA might not be recognized in a depleted system such as mine resulting in the infused IgA triggering anaphylactic shock.  Best to be avoided.  I shall soldier on with only wisps of IgA and hope that my bolstered IgG will work overtime.

So today was 7 and a half hours in the infusion room.  How do I feel?  Well, I am pretty wiped out from the pneumonia itself as well as the IVIG which is also supposed to produce fatigue.  But from the point of view of my immune system, today is the best that I will feel until my next infusion on May 18.  The half-life of the immunoglobulin sets in immediately and my levels will decline steadily until we build them back up in a month.  Think "300" everyone!  That is my goal "trough level" for May 18!

Time to Get Well

Saturday - April 16, 2016

Never could I imagine that so much time would pass before updating.  Sorry but I have been busy coughing - and working - and then coughing some more.  Time for friends?  No.  Time for fun?  No.  Too busy coughing.

Happily (?), this cancer blog appears to be shifting into a CVID blog.  Of course, I remain profoundly grateful that my wretched lymphoma remains in remission.  Almost four years since that Stage 4 diagnosis and I remain cancer-free!  1 more year and I may be able to breathe more easily.... But speaking of breathing, that brings up the topic of coughing and ...

... I am certainly not well.  This school year has been an intellectual and emotional delight but a true health challenge.  I have been coughing (productively) non-stop since Columbus Day.  Prior to that I had a good 8 days but had been coughing since the schoolwide Open House held prior to Labor Day.  That's a LOT of coughing and in the last few weeks, I have begun to feel ... depleted, tired, ready to feel better.  Generally it's just that annoying (productive) cough - especially in the morning - but there have been some notable highlights such as a very vivid, eye-catching subconjunctival hemorrhage, a thrown-out back, and a recent cracked rib.  Last weekend, I was coughing so hard I couldn't keep food down and this weekend I am battling a high fever that has just broken (that's what 16 hours sleeping and medicating can do!).  Joe expresses frustration that I hold it together for the school week and then crash on the weekends - he's right.  It's how I keep going.  I focus on the fun I have at work and immerse myself in the job to push down concerns about my health.  It worked when I taught through the medical mystery of my 4+ months of migraines and double vision and it has worked this year as I simply CANNOT rid myself of this deep (productive) cough.  But now I enter a new phase of medical intervention.

On Wednesday, I begin monthly immunoglobulin therapy to bolster my deficient immune system.  Since August's CVID diagnosis, I had months of debate about what kind of infusions to get and where to have treatment.  Joe and I met with an NIH-affiliated immunotherapist in February.  He was very firm that despite my appearance of good health and excellent (!) lung function (can you believe it? We were ALL surprised), given my extraordinarily low immunoglobulin levels, I was risking "catastrophic" results from a swift moving infection.  He used "catastrophic"multiple times even though the first time he used the word, it pierced my stubbornness and got my attention.  "Catastrophic" is a persuasive word.

So now the paperwork for this immensely expensive treatment has been vetted and approved by insurance and I am finally on the schedule.  I am so incredibly grateful to all the blood donors who make my treatment possible:
"Ig is prepared from the plasma collected from a large number of normal individuals, usually between 10,000-50,000, who have been carefully screened to make sure they are healthy and do not harbor certain infectious diseases."
Whatever hesitation I might have felt has evaporated in the face of this constant (productive) coughing and my growing fatigue.  Because I want the latitude to travel and be free of a weekly medical procedure, we have decided to go with intravenous monthly infusions at an infusion center rather than more frequent subcutaneous infusions at home.  Wednesday's first infusion will be an all-day affair and the first of ... well, I will need them for the rest of my life.

So, I have reached a level of acceptance and am ready to share some aspects of life as a zebra on CVID boards and sites.  I still receive mail from the NHL Cancer sites and there indeed appears to be crossover between lymphoma and CVID.  It's a chicken and egg situation.  Whatever connection may exist should be more public so I am keeping the blog as one full piece rather than splitting it into two separate medical journeys.

Relieved to be diagnosed.  Grateful to be starting treatment.
Thrilled to stop coughing!
Finally!
Please!