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!

A Shift in Perspective

Saturday - September 19, 2015

So, this 3-year chronology of my lymphoma treatment and remission is morphing into a CVID blog....  Hmmm....  Who knew?  I suppose I need to change my "About Me" section and post another link on the CVID message boards.  I confess that as I lay up for the weekend trying to stave off my first respiratory infection of this school year, I am buffeted by mixed feelings.

Relief:  Hope exists for healthier living.  My VCU immunologist phoned on Thursday with news that my vaccine challenge test dovetails with my second round of blood tests (showing miniscule and alarming levels of immunoglobulin).  My blood has had a low response to both the tetanus and pneumonia (yikes!!) vaccines he administered on August 24.  These slam dunk results mean that insurance should cover the expensive immunoglobulin infusions that I need (weekly?  monthly?  TBD) to restore my immune system to some level of function.  With these infusions, the goal is to "avoid bronchitis,  pneumonia, and most respiratory infections."  He added that I should have colds at the rate of a person with a normal immune system.   That sounds absolutely GRAND!  I am flooded with relief.  Won't it be magnificent to live most of the year without lung congestion!  These many years of constant coughing have worn me down emotionally.  Which leads me to ...

Worry:  I may have already damaged my lungs.  54 is relatively late for a CVID diagnosis.  My father, Joe, and my sisters have always maintained that I have a "weak immune system."  My father told me that for years and he passed away in 1995 so this issue has been long-standing.  I believe I was relatively healthy through college but certainly remember coughing like a fiend when I was working as a management consultant in the mid 80s.  Coming down with shingles at age 25 in 1986 is consistent with the decline in my immunoglobulin.  I have a consult with a pulmonologist when I return to see the immunologist at VCU on Oct. 19.  Before my lymphoma, I used to joke that my lungs would be the death of me.  After cancer, my thoughts about my ultimate demise obviously shifted to the insidious specter that permeated my lymphatic system  - a relatively unappreciated and unnoticed part of our bodies! Now my lungs have reasserted their need for medical attention.  With treatment, CVID is completely manageable.  I am hanging my hat on THAT!

Guilt-free:  My wracking coughs are indeed NOT a cause for concern to the public, no matter how horrid I sound.  Whatever virus or bacteria is afflicting me will probably not impact others because the vast majority of the world has the protection of those handy immunoglobulins which will efficiently summon up an immune response and protect against infection.  I get sick.  YOU don't.  The immunologist emphasized that I may sound wretched but I am generally harmless.  "Patients with common variable immunodeficiency are subject to recurrent infections, especially those caused by microbes that don't normally cause disease in normal persons."  Good to know.  Maybe I should wear a sandwich board to reassure everyone?  I know I sound horrible, gross, scary ....

Squeamish:  I am NOT looking forward to a regimen of subcutaneous infusions for the rest of my life.  I spent too much time on message boards last night and this morning.  TMI, folks!  Too many photos!  Maybe I can gratefully digest this detailed information later.  At this point, I can't read any more horror stories about side effects (migraines!  NOT wanting to return to that!) and administration problems (broken needles?  Oh Lord ...) so I am returning to my self-imposed internet-surfing ban.  Megan can be my surrogate :)

Supported:  A community of zebras is already giving me valuable advise on finding a CVID specialist in the DC area.  Thank you, internet!  Thank you, Facebook!  The 2 referrals I received from VCU have been outed as rude and rushed by multiple sources online.  Excellent physicians, for sure, but I have been spoiled by compassionate, professional, courteous, and engaged medical professionals in 4 hospitals since 2012.  As a consumer and client, I have a choice.  I choose professional, polite, and approachable medical practitioners.  A third referral is reaping praise from multiple references with plaudits for manner, demeanor, and engagement.  Guess who I will call first?

"People with CVID are called Zebras because doctors are taught in medical school, “If it looks like a horse and sounds like a horse then think horse–not zebra” (as in a rare disease). CVID patients are the exception! We are Zebras! Because CVID is very rare, some doctors overlook it and misdiagnose it as something else because the symptoms are identical to other diseases."

Clear Scans and a New Diagnosis (?)

Sunday - September 13, 2015

Almost three weeks after my PET scans and MRIs and many many days of playing telephone tag with my oncologist in Richmond, I finally looked at my online health portal - my scans are all clear.  Phew.  Of course, I realized - intellectually - that bad news would have been transmitted swiftly, follow ups would have been quickly vetted and put into place, and a personal call to deliver bad news would certainly have preceded posting bad results online but ... - emotionally - I was not so sure.  Logging onto my portal seemed risky.  A very bad idea.  Seeing bad news in print without the support of a physician would have been a blow so ... I avoided it until this morning when I let my head and my logic win the day.  All clear.  Okay.  Breathe out.  Move on.  Nice to get a confirmation that lack of outside symptoms matches lack of evidence of lurking lymphoma sneaking back into my bones, my orbits, my nodes.  Relief.....

That emotion of relief is countered somewhat by another piece of health news delivered that Monday morning in Richmond.  While I will share more news as this upcoming path becomes clearer, my oncologist's keen listening to my regular mantra of "I get horrible colds; I can't shake them for up to 6-8 weeks; it can end in bronchitis or pneumonia" resulted in him running an immunoglobulin panel in my blood work.  This test had never been run on me before and revealed that ... I really don't have any immunoglobulin.  Barely perceptible numbers.  We're talking single digits.  After getting those blood results, he called me at 7 pm and left a message to call his cell phone.  Ummmm.... while he sounded completely calm and upbeat, NO ONE wants to have an oncologist call at night and ask you to call back on his cell.  No cancer news; just news that I seem to have negligible traces of IgG, IgA, and IgM.  He already had scheduled a referral to an immunologist at VCU.

In following up with the VCU immunologist and confirming these blood results in another test, a diagnosis of Common Variable Immune Deficiency ( CVID ) appears likely.  Treatment?  Think diabetes management but rather than daily insulin infections, CVID can be managed with monthly or biweekly subcutaneous infusions.  The CVID explains a lot.  Not only does it shed light on my weak immune system, it is consistent with my shingles, my thyroid autoimmune mysteries, and even my lymphoma.  The BEST news that emerges from this fresh information is that CVID makes the lymphatic system very active and that my stage 4 designation might have been overstated.  CVID "lights up a PET like a Christmas tree", just like stage 4 lymphoma.  CVID can "overlay" with lymphoma.

So, now that stage 4 designation feels less likely.  I remember the difficulty in harvesting a lymph node.  I recall the clear bone marrow biopsy despite the stage 4 designation.  And then ... I cringe when thinking how CLOSE I was to an allo Bone Marrow Transplant with its 25% mortality rate. Oh my.

Medicine = art and science.  We are still in a nascent era of medical discovery and treatment.

Time to find care up here in the DC area.  It looks like I will be searching for a CVID specialist.

Closing Down California Living

The hounds sacked out while I cleaned around them

Tuesday - June 30, 2015

Well, that's it for our beautiful perch on the hill.  This afternoon I walked the hounds on the fire road above Sausalito, soaked in the glorious view of Angel Island and Richardson Bay one last time, spied the UC Berkeley Carillon across the bay, and locked the doors behind me.  Time to move on - and I am pretty much exhausted!  It has truly been weeks of preparation and organization for this move - our 22nd! - with much weeding out and recycling before the packers came last week.  In California, MUCH can be recycled or deposited for safe disposal and I took full advantage.  The move itself was a challenge due to our location high above town on a steep and narrow road.  One truck got stuck getting out of our parking pad and then had a flat tire from the exertion and contortions.  The next day, our boxes and furniture had to be shuttled down the hill and across to Mill Valley where they were then transferred to the transcontinental truck.

Since the apartment emptied out on Friday, we have been in temporary housing in 2 lovely Air B&Bs for five nights.  Enjoying new lovely Marin locales.  Trying to rest.  We also took advantage of our last few days by enjoying wonderful outings to Sonoma, Point Reyes, and Half Moon Bay as well as final visits to favorite restaurants.  Leaving behind the amazing vistas, weather, and food of Marin is going to be pretty, p-r-e-t-t-y hard.  Will I find another destination to match the peace and beauty to match Point Reyes?  Will pizza ever taste so good?  Yes, we are excited to return to Northern Virginia but we will miss much about the beautiful Bay Area!

Monday it was down to dirty details.  Two full days of scrubbing and cleaning, mopping and dusting, and trying to leave our rental in white glove condition.  Let's just say that I am ready for a (coast to coast) vacation!

Leftover cancer drugs safely disposed of in CA! 

So farewell, California.  Thanks for two years of sunshine, Megan-time (!) and good health.  Thanks for that unequalled restorative, calming, ever-changing view from our perch on the hill.  Hurricane Gulch was very good to us.  I will fall asleep at night remembering the whitecaps and the wind, hearing the Golden Gate foghorn and the barking of sea lions, seeing the wave of fog spilling over Wolfpack Ridge and the outline of Angel Island against a moonlit sky.  A feast for the senses that will continue to soothe me in my memory.

Tomorrow we hit the road heading east!

Farewell to UCSF Oncology

Fabulous vista.  Who can spot the Golden Gate bridge?

Thursday - May 14, 2015

Today was my last quarterly check-up at UCSF.  These 2 years, I have felt well cared for and included in my care decisions at UCSF Hematology.  I believe I have had thoughtful care from a collaborative and reflective teaching hospital team.  The conversations feel like dialogues rather than pronouncements.  They also go the extra yard to treat all of my immuno-compromised self.  A perfect example is today when I presented with one of my epic going-on-6-weeks upper respiratory illnesses.  Although I had been to the UCSF clinic on Tuesday for this nasty, horrible cough / cold, the oncology clinic did a full work up on me - again! - and then referred me for a pertussis swab (despite an absence of whooping)  "just to rule anything out."  Today we also discussed transferring my care BACK to VCU and my original oncology team.  Carolyn had suggested I travel south to Richmond every few months rather than finding a new team in the DC area.  My UCSF oncologist agreed that this plan made a lot of sense and then presented his view on future decision points e.g., scans and bone marrow transplants.  We also agreed that if things head south, I skedaddle up to Johns Hopkins.  Sounds like a plan.  Now it's time to start transferring all of my records - another indication that our move is in full swing.

Wonderful News but ... Still Too Raw to "Time Hop"

Friday - April 24, 2015

Today is a glorious day for our sweet Megan!  Her plans for year 6 of her 7 year PhD strategy have come to fruition with a Fulbright Award to study at Nanjing University.  She is relieved and happy, happy, HAPPY! And of course Joe, Jonathan, and I are proud (but not too surprised!) family cheerleaders!  After all, one could say that Megan is an inspired, creative Dragon baby with the nose-to-the-grindstone tendencies of her Ox parents.  She is ready to tackle those Nanjing archives, learn the erhu in her spare time, and write, write, write!

And in the midst of this swirl of positivity -- there it is .... in the not-so-far back of my mind, I think "thank God, I am here WITH HER when she got the news."  Not just "here" in the sense that we happened to be together in the San Domenico faculty workroom when she opened the email, but "here" as in "still alive" to see our girl glow with happiness at a long wished-for goal attained.

Wow.  That sense of "almost" never quite goes away and this highly recommended "On This Day" Facebook app confirms recent history.  Three years ago today might have been my absolutely most excruciating point of pain.  I literally crawled up the stairs into bed ... and got up the next morning to the salvation that was my first grade classroom.  Unbelievable.

"Christine Ammirati

April 24, 2012 at 7:02pm · Williamsburg, VA ·

Shattered. A head MRI with a bone-crushing headache is immeasurably worse than a head MRI while on prednisone and a manageable headache. Taking emergency pain med, curling into a ball, and hoping for deep, healing sleep.

17 Comments

So... time to disable this well-intentioned app, cuddle up with our amazing girl and the dogs, message Jonathan with laments about the Mets, feel grateful, and be in the moment.  It's still too soon to have surprise looks back and that day in particular still seres.

Virginia Dreamin'

Tuesday - March 31, 2015

This classroom view will be missed!

We are once again in countdown mode to yet another move.  The reality of our return to the familiar faces and environs in the Arlington area softens any discomfort or frazzle.  We are headed home!  Our lease is up at the end of June and on that day or soon thereafter Joe, the hounds, and I will turn the VW eastward and head off to the muggy and comfortable neighborhoods of northern Virginia.  Given that our days are ticking off steadily, we are now tackling the tasks of selling our Williamsburg house, buying a house in Mclean or North Arlington, prepping for our physical pack-up and move, and finding a happy new school for me to call home in Northern Virginia.

Another necessary task is finding an oncologist.  UCSF has been an amazing experience.  I feel regret at leaving my medical team behind.  So what's next?  I am toying with the idea of going in full steam at Johns Hopkins since it will be my medical recourse if I relapse.  However, Joe advocates for optimism and avoiding the round trip to Baltimore.  Another box to tick off as we organize our departure.  Do I choose convenience or top of the line Hopkins experience?