Give it a shot! Cooties, and Christmas Party for Two, Apart

Jeff sat over his pile of morning pills, his “second breakfast”, while our 4-year-old granddaughter observed.

“Grandpop, which one is your Grumpy Pill?” she asked.

“This half of a pill,” Jeff told her.  He splits a 20mg tablet of Prednisone and he thought she was referring to the pill with the unfortunate side effect of irritability.

No wonder he is grumpy sometimes.  From the start of his day taking care of himself is a time-consuming and frustrating series of tasks.  Donning compression stockings (while gloved to protect his weak fingernails), injections (twice a day for 10 days earlier this month), wound care (dressing old and new wounds), creaming his face, swishing his mouth, flushing his PICC line and taking a mountain of pills.  He contends his morning ritual is much more involved than doing hair and make-up.

And wounds happen so easily, each one an additional wound since the old ones don’t really heal completely.  He even injured himself while sleeping.  A tug on the blankets and oops!  His hand slipped off and he gave himself a fat, split lip.  I heard him mumble, “Yeah, that’s not going to heal.”  

Bone marrow biopsy results were a bit of a let-down even though there was good news – no evidence of leukemia and his blood is 98% Donor Nicole’s!  There was also no indication of why Jeff’s platelets have been slowly, steadily dropping for months.  Still, Dr. Porter was pleased with the results so we tried to be okay with it, too.  I told Dr. Porter that Jeff didn’t seem as well as he did three or four months ago.  He and Nurse Heather said medications and everything he has gone through could account for that.  

But…  things suddenly began turning around. 

When edema suggested – and an ultrasound confirmed – blood clots in his leg, Nurse Heather asked, “Have you ever given yourself an injection?” 

“Not on purpose,” Jeff quipped.

Heather laughed and said the home nurse would provide injection instructions.  However, the home nurse wouldn’t visit for another four days and Jeff needed to start the blood thinner immediately.  After negotiating with the pharmacist to pay out-of-pocket for three days’ worth of syringes pending insurance approval, we watched a YouTube video on self-injecting and Jeff was set. 

The video said to avoid injecting in the same spot.  Hmm.  How to remember the location of the previous injection site?  We needn’t have worried.  Jeff woke up the next day with a five-inch purple circle marking the spot.  Fortunately, that didn’t happen every time.  Boy, did he bleed!  The sheets, towels, light switches, faucets, doorknobs and Jeff’s clothes all had what Jeff called “(Donor) Nicole’s precious blood” dripped – and flooded - on them.  The injection sites bled through the Band-Aids and each day, of course, another couple of Band-Aids were added to his belly.  They did not sufficiently contain Nicole’s viscous gold.  I am glad there was no reason for CSI to visit our home.  There would have been questions.

There is still some edema but Jeff will take blood thinner in pill form for several months.  And the marked improvement in platelet counts (due to the blood thinners, maybe) satisfied Dr. Porter enough to order removal of the 15-month-old PICC line!  Merry Christmas to Jeff!  No more weekly blood draws, no more silicon shower sleeve.  Extra medical supplies donated to the local animal hospital.  Done.

Another recent success was with dermatology.  Jeff had the worst flare-up of his painful, bumpy, red face on a day he was scheduled to see Dr. Porter in November.  It was so dramatic that Dr. Porter noticed it while passing the exam room that Jeff, well-masked, was waiting in.

Dr. Porter immediately sent pictures to Dr. Samimi, the dermatologist.  Dr. Samimi’s biopsy of one of the bumps showed three things going on:  yeast, mites (he has cooties) and folliculitis.  She switched Jeff’s treatment from Ivermectin cream (which burned his skin) to Ivermectin pills instead.  The insurance company required pre-authorization.  Isn’t “authorization” given when the doctor prescribes it?  Anyway, insurance will cover only 9 pills in three months.  His entire course was only a week, “seven pills today and seven pills in a week”.  Fortunately, it was inexpensive and since Jeff’s condition was acute, we just paid for it.  Why, you might ask, does the insurance company have to pre-authorize a drug if it isn’t expensive?  Evidently it is because of the mis-use of Ivermectin for COVID.  The good news is, Ivermectin seems to have worked.  His face isn’t completely normal but it doesn’t hurt anymore and looks a whole lot better.  Recent pictures of Jeff’s improvement impressed Dr. Samimi.  “Drop Prednisone to 5 mg,” she messaged.  Woohoo!  Grumpy Pill, halved!

Dr. Porter says that Jeff might have adequate antibodies for COVID.  The antibody test that was done in April gave results that seemed less than stellar but at that time not enough information was known about what constitutes a good number.  Jeff, now having received three full doses of the vaccine all while immunosuppressed, is still not comfortable taking too many risks.  He can do without COVID, thank you very much. So when I had another potential work exposure four days before Christmas, we isolated from each other per CDC guidelines for households with someone who is immunocompromised.  We are in separate bedrooms, use separate bathrooms, eat in separate rooms (though within talking distance).  Jeff cooks and handles clean dishes and clean laundry; I handle dirty dishes and dirty laundry.  We each contributed to our Christmas dinner but we cooked our respective dishes by taking shifts in the kitchen.  We go for walks – masked and socially distanced – so we can be together sometimes.  We are trying to make the best of the situation while awaiting my PCR test results - just a few days.

We hope that you are making the best of the pandemic, too.  Merry Christmas and Happy New Year! 

Lotions and Potions, Flushes, Swishes and Sloshes, and a Year-old PICC Line

“Grandpop you need lotion for your face.”

Granddaughter Rosie had noticed the flare of GVHD all over Jeff’s face.  The same spots remain always but are alternately quiet or active.  It was particularly bumpy and red and Rosie was certain she could offer advice that would help.

“Rosie, if you knew had many lotions and potions I have for my face, you wouldn’t believe it can still look like this,” Jeff lamented.

Steroid creams, moisturizers and dermatological creams cannot tame this beast.  Weirdly, Jeff enjoyed a few days of calm on his face after he started swishing his mouth with the new formulation of Dexamethasone the oral specialist prescribed for GVHD of the mouth.

“Whoa!” I said one morning at breakfast.  “Why do you think your face looks normal?”

“I was wondering the same thing,” Jeff said.

We joked that maybe changing the treatment for the inside of the mouth somehow helped the outside (his face) as well.  “Normal” didn’t last long so our silly theory was debunked.  Jeff “swishes and sloshes” his two oral rinses three or four times a day and that seems to manage the GVHD mouth sores.  He hasn’t had too many problems with sores down his esophagus so maybe these rinses are working better for him.  Jeff alerts me when he is about to “swish and slosh” because he has to keep the stuff in his mouth as long as he can before spitting it out and he will, for the duration, communicate with grunts and hand motions.  He has to time the two rinses apart from each other and from meals or snacks.  (Getting these magic elixirs refilled required three trips to our pharmacy which was, sadly, short-staffed due to COVID and, therefore, three days behind in filling prescriptions!)

Jeff calls me at work when Dr. Porter enters the exam room at his regular hem-onc visits.  Last week Dr. Porter discussed the kidney biopsy (no GVHD there, nor cancer), a COVID booster (get one as soon as you can) and a slow steroid taper (a month at a time, currently at 5 mg).  My questions answered, new instructions noted, I left the call.  Then Jeff remembered the question that was most on his mind, “When can this PICC line come out?  It’s been in there for a year.”  

For a year he has had to tend it, covering it in a silicone sleeve to take a shower and flushing it with heparin every day.  Dr. Porter said, “Oh!” as if he didn’t realize how long ago the PICC line was inserted.  PICC lines often don’t last this long and its longevity may be due to the good care Jeff and our granddaughters give it.  When the girls are with Jeff, they fight over who gets to press the syringe for the flush.  Fortunately, there are two tubes so at least two of the three girls can have a turn on any given day.

If Jeff’s numbers (blood counts, liver and kidney numbers) remain steady and pretty good, the PICC line can come out in a month, Dr. Porter thinks.  Then the Home Infusion nurse would no longer need to come weekly to change the bandage and draw blood.  Then Jeff’s blood would be tested monthly.  That will be liberating for him!  

Jeff seems to be getting stronger although he still needs naps sometimes.  Unfortunately, our four-year-old granddaughter, who spends two days a week with Grandpop, seldom needs a nap.  The two of them are working it out.  Sometimes he wins, sometimes she does.

Our family is undergoing some changes.  Our youngest son moved out (again) and we are empty-nesters (again).  It suits us.  We will miss our Jeopardy whiz/lawn-service/hardware deliveryman/technical consultant/pizza-picker-upper but we will manage. 

Another family member entered hospice care – beautifully, gracefully and with the love and support of her family.  She and Jeff have commiserated over their cancer experiences and though she is strong, it is difficult to know that her journey will very likely be a painful one.  Still, she intends to “go out laughing” and here’s hoping that isn’t for awhile yet and that she gets to have many, many more laughs.  Prayers for her, if you will, for all those dealing with the ravages of cancer and cancer treatment, and also for their caregivers.  

If you are so inclined, please support our friends, Michael and Monique, in their fundraising effort for the Leukemia and Lymphoma Society, Light the Night: https://pages.lls.org/ltn/epa/BucksCo21/mriottovkk. 

The Merits of Compliance

Jeff cleaned off the nozzle of the spray paint can I’d managed to clog.  He handed it back to me and said, “I can fix anything.”

“Except yourself,” I teased.

“I’m working on it!” 

When Jeff visits a specialist for the first time the new doc is likely to comment on Jeff’s willingness to do what it takes to be well.  His medical record is marked “Compliant”.  We can’t imagine saying “no, thank you” to efforts to improve health although Nurse Heather says that happens a lot.

The report from a recent skin biopsy gave welcome news - not cancer - but left questions.  Jeff’s case was reviewed by the Dermatopathology Consensus Conference which sounds mighty important for a group who could only narrow it down to about three unpronounceable possible diagnoses.  There was a referral to an Oral Specialist (a totally different part of the anatomy but somehow, weirdly related).  Jeff scheduled an appointment in November - the best they could do, he was told.  Dr. Porter’s office intervened and voila! Jeff got the message, “Come next week.”  

In a flood.  Jeff’s symptoms are concerning and it seemed important to keep the appointment.  We texted Daughter Kim and asked for suggestions for an alternative route since major roads, including the Vine Street Expressway, were closed due to flooding.  The ride was round-about but Jeff got there in reasonable time, an hour and a half.

After a couple of young docs interviewed and examined Jeff, Dr. Stoopler arrived in the exam room.  One look inside the mouth and Dr. Stoopler confirmed GVHD.  We suspected this because all the symptoms on the head (skin, eyes, mouth, throat) had been getting worse as the steroid taper continued.

“Do you have trouble swallowing?” Dr. Stoopler asked.

So far, the lesions and swelling have not impacted Jeff’s ability to eat enough although he sometimes has to take it slowly.  He thought he was imagining feeling obstructions in his esophagus but Dr. Stoopler believes he has sores all the way down and referred him to an ENT specialist at Penn.  

Dr. Stoopler’s diagnoses included “stomatitis” and “mucositis”, thus increasing our vocabulary once again.  He said GVHD of the mouth is hard to maintain and prescribed a different formulation of the Dexamethasone mouthwash and added an anti-fungal mouthwash.  He gave Jeff a list of foods to avoid including cinnamon, garlic, vanilla and mint (including mint toothpaste), as well as the more obvious ones – tomato sauce, citrus, red wine.  For fun, Jeff added broccoli and brussels sprouts (he likes both of those).

Upcoming visits include follow-ups with dermatology, the oral specialist, hematology/oncology, ophthalmologist (tear ducts were plugged), oral surgeon (check on skin graft).  Also, dentist, a physical with his primary, initial visit with the ENT specialist and weekly visits with the home nurse.  This is part of what it takes and, as we know, Jeff is compliant!  He calls himself a “professional patient”.  

This summer has been an odd one:  COVID scares, tornadoes, hardly any days at Judy and Mark’s pool, maintaining vigilance to minimize COVID risks, and spending an inordinate amount of time just the two of us.  We are happy we see our children and grandchildren regularly.  They add color to our social calendar, for sure!

Good news: 

• Liver and kidney numbers are fairly good and fairly stable despite a bout of acute kidney failure a couple of weeks ago. 
• The steroid taper is down to 2.5mg and, unless instructed otherwise, will be zero by Thursday.
• We completed our goal of walking the entire Delaware Canal Towpath.  Last weekend we walked a couple of miles on the Delaware and Raritan Canal on the New Jersey side of the river.  We might set a new goal to hike the NJ side.  Stay tuned.
•  Almost all of the things damaged or lost in a power surge on July 1^st (including central vac, garage door opener, hot tub, dehumidifier, lights, routers, TV…) have been replaced or ordered.
• Jeff started a project at Friend Dan and Jennifer’s house which is challenging for him but he is determined to do the work.  We are thankful for Dan and Jennifer’s patience and willingness to help the contractor haul heavy things.

We hope you are all well and happy!  Take care of yourselves and be thankful for something every day.  

Bubblewrap, Bullseye and a Big Boom

“I need a medic,” Jeff said, flopping into a kitchen chair and pointing to his bloody leg.

 

Keith went for the Band-Aids.  Neither of us was overly concerned.  Jeff’s arms and legs are covered with wounds.  The slightest bump against a piece of furniture or brush against a box lid while reaching inside can draw blood.  Harder bumps produce instant hematomas (maybe there is another name for these) where blood immediately pools just under the skin in a wide area around the cut.  With each addition, his skin looks a bit more scary. We learned that some patients wrap their arms to prevent these wounds.  I am considering whipping up sleeve protectors like old-timey bookkeepers wore. 

 

“You just want to wrap him in bubblewrap, don’t you?”  Keith asked.

 

“I do.  I just wish he would stop doing things that make him bleed.” 

 

This, of course, is unrealistic since opening a box is hazardous. 

 

After a slow start, the steroid taper is moving along nicely.  Liver counts remain steady.  The Prednisone dosage is down to 25mg and side effects are lessening.  Sleep is improved, hunger is somewhat better controlled, glucose level is improving, platelets are creeping up.  Jeff is a tad less irritable and I think he sweats less although he isn’t so sure of that.

 

I caught myself imagining the day when the PICC line, inserted in his left arm on October 1^st, could be removed.  Jeff has been dreaming of that day, too.  Since about October 2^nd. 

 

Jeff’s complaints are often general, “I can’t catch a break,” “Nothing works the same,” or “I just wish I felt better.”  Well, it does seem as if the poor guy can’t catch a break. 

 

I noticed a large oval mark on the back of his thigh and asked him what he did.  He gets wounded so often and so easily that he doesn’t always know what happened.  He remembered bumping into a shopping cart.  I told him it was red, not bruised.  The spot looked so odd that I snapped a picture.  Jeff looked at the picture and wondered if it could be Lyme Disease.  It was oblong and didn’t have a defined bullseye.  Neither of us knew what to make of it.  Four days later when the spot became painful to him, I snapped another picture.  I sent the pictures to his dermatologist who has been keeping tabs on his GVHD skin symptoms. 

 

She replied quickly, “Any fever, chills?  Have you been hiking?”

 

We’ve been using bug spray on our hikes but…  Three weeks of antibiotic were prescribed.  Dr. Samimi wrote, “I want to highlight stomach upset and risk of sunburn...”  Dr. Porter’s office asked us to make them aware of any side effects so they can be dealt with as they come up.

And we lost some electronics in a lightning strike. My first clue that we were hit, besides the heart-stopping BOOM, was our central vacuum spontaneously turned ON in the basement. When I went downstairs to turn it off, the basement light didn't work. As I used the flashlight of my new cell phone to inspect the vacuum, a chirp notification came in and I fell to my knees!  I screamed, then I laughed when I realized the "whistler" was not standing beside me.  Hearing my dark-basement freak-out, Jeff sauntered downstairs to see what was going on. So far we think we lost at least one  TV, two light fixtures, the modem and at least one router. And the central vacuum doesn't turn off. It will remain unplugged for now. Replacing surge suppressors and all things ruined will keep us busy for a while. I'm glad I have a long weekend to get the internet up and running before I need it for work next week.  

Ain't life grand?  

10-Year Survival, Remission and the Impatient Patient

“I’m tired of being sick,” Jeff told me in a rare admission of frustration.

“I bet you are,” I told him.  “I don’t think I would be as patient a patient as you have been.”

It’s odd to have had this conversation when we’d heard some very, very good news this week.  We’d waited very patiently (really and truly) for the results of the last bone marrow biopsy.  We believe this was the 13th or 14th biopsy since diagnosis in 2010.  We didn’t expect bad news.

The results came in dribs and drabs over the last couple of weeks.  As each of the many tests performed on his marrow were completed, we’d get an email saying that a new test result had been posted on Penn’s portal.  One test showed 1% blasts (immature cells in the marrow) which Jeff feared was bad.  I thought we all probably have blasts.  Dr. Porter confirmed that under 5% blasts in the marrow is normal.  In fact, Dr. Porter was so pleased with the results that he said Jeff should consider himself in remission!

Dr. Porter noted that there was still one test result that wasn’t in yet – the chimerism test which shows how much of Jeff’s marrow are Nicole’s cells.  By the end of the week we received the “New Test Result” email.  I checked the portal to find a welcome number, >99% Donor Cells!

Why, then, isn’t Jeff happier?  Last week’s blood work showed only a marginal improvement on one of his liver counts and no improvement on another.  Dr. Porter was comfortable reducing Prednisone by 10 mg to 50 mg and will reevaluate after this week’s labs.  More of Jeff’s issues are steroid-related rather than caused by the disease the steroids are supposed to treat.  GVHD is causing: abnormal liver function, blurred vision (tear ducts are scheduled to be blocked to help this), some skin issues and brittle nails.  Side effects of Prednisone include:  irritability, large muscle weakness, sleep disturbance, irritability, profuse perspiration, dehydration (resulting in leg and hand cramps), irritability, high glucose levels, low platelets, easy bruising, weak voice, dizzy spells, Moon Face and irritability.  And constant hunger.

Jeff has always had a healthy appetite but now prepares large meals which he manages to consume, often leaving no leftovers.  He scrounges for snacks (avoiding sugar) and gets hangry.  I keep hard-boiled eggs on hand for when he is suddenly ravenous but he adds them to our large meals as if they’re a side dish.  I tried a new recipe for healthy banana bread that uses a little bit of honey and chocolate chips.  To avoid him eating the whole loaf in a couple of sittings, I froze some of it.  He eats a chocolate chia pudding I made with coconut milk, a “treat” he would eschew under normal circumstances.  Nuts, removed from his diet by the kidney specialist a few years ago, are now back on his plate.  We bought Gatorade and a bag of avocados for replacing potassium and other nutrients depleted by profuse sweating.  

Over the last few days I noticed Jeff was frequently slurping saliva, a quick phlooping action.  When I asked him about it he sassed, “Oh, it’s just something I do to annoy you!”

I waited a moment to respond to Steroid-Jeff, “I thought it might be a new side effect that you may want to mention to Heather.”

Later, when Real-Jeff returned, we discussed what might be causing excessive saliva in his mouth.  I Googled the issue and learned some medications cause it – none of the many Jeff is taking, however.  It could be a muscle issue, I learned.  Except he can swallow without difficulty.  We puzzled over it and put it on the list to ask Nurse Heather.

Jeff pondered some more and came up with a theory:  Steroid-induced “Moon Face” puffs his cheeks away from his gums and teeth creating a cavernous area that somehow prevents drool from being absorbed in a normal way.  I think he might be onto something.  And what, pray tell, will be the solution for this? 

A blood test was run to find out whether Jeff developed any COVID antibodies since his vaccination; he was “stuck” in March – squeezed precariously between his last treatment in February and the start of immunosuppression (Prednisone) in April.  Evidently, he had a “weakened immune response” and developed only the IgG antibodies and not the IgA antibodies.  Not enough is known about this response but Dr. Porter feels that Jeff likely has some protection against the virus.  We will continue to be careful.

Since our last post we celebrated May birthdays including Jeff’s 10-year Transplant Anniversary.  I made a chain out of the hospital bracelets that Jeff saved.  We shared a cake with our kids and grandkids to celebrate Jeff’s and Kim’s shared anniversary/birthday.  That was the extent of the celebration.  If the pandemic were in the rear-view mirror we would have had a big party.  Maybe next year…

Our canal towpath hikes continue although at some cost to Jeff.  After a hike we push Gatorade, water and potassium-rich foods to replenish nutrients lost through copious sweating and not sufficiently managed by drinking water en route.  We continue this treatment (plus Epsom salt foot baths) until his legs and hands stop cramping.  When I suggest we cut a hike short or propose not doing it at all Jeff says “We have to.”  Our goal is to finish the last 11 miles (X2 because we do up-and-back hikes) to reach Easton.  A few more trips should do it.  Tramp on.

Steps for Life!

I’d been telling family and friends that Jeff, Nicole and I would be sharing the virtual stage with Broadway star, Jackie Burns (Elphaba/Wicked among other roles).  The talented actress introduced us during the kickoff of the Gift of Life/Steps for Life Virtual 5K!  There was an awesome donor/recipient meeting which was done live (and continued in the chat room immediately following their live introduction) and then our pre-recorded - and geniusly- edited - interview.  Since our interview was done the week before, we could simply sit back and watch ourselves.  It was fun to get messages from our family and friends that evening and in the days that followed.  We are thankful for the love and support shown to us and Gift of Life. 

If you missed the event, do not fear!  It is available at Gift of Life/Steps for Life Virtual 5K Kickoff (the festivities begin at about 16:00).  The campaign continues through May 24^th.  Our team page is Bist Du Stark? Are You Strong? We have done well so far and appreciate all of our donors.  Not everyone has an easy time finding a match.  Jeff was lucky that a match was found for him quickly when he needed one.  

Daughter Kim, Daughter-in-law Theresa and our granddaughters joined us for another Delaware Canal Towpath walk on Saturday.  We wore race bibs for the Gift of Life Virtual 5K.  We ladies walked for Jeff/Dad/Grandpop/FIL while Jeff walked in honor of his donor, Nicole.  None of us will win any prizes unless there is one for most-turtles-counted. Granddaughter Rosie counted more than 50 turtles!  It was a gorgeous day to celebrate life!  Thanks again, all to all of our team supporters.

Well, now, let’s talk GVHD and steroids.  Jeff has been watching his diet in an attempt to ward off one side effect of steroids – high glucose levels.  Jeff and I eat, more or less, the same diet.  He has lost 16 pounds.  Please don’t ask the question that is sure to come next.  The answer is zero!

Jeff now takes steroids three ways – eye drops, mouth gel and oral Prednisone.  The Prednisone has been tapered twice and he is now taking 60 mg per day.  The ability to sleep no matter what – a strength of Jeff’s – is greatly impaired.  Four hours of sleep a night for someone used to getting 8+ is not pretty.  Add steroid-induced irritability and, well, mild-mannered Jeff sometimes hides.  Jeff tried to describe to me what it feels like to be both grumpy and grateful but couldn’t seem to describe it to his satisfaction.  I suggested that he increase his Melatonin dosage.  Nurse Heather started him at 10 mg when he began Prednisone.  Jeff decided to try 15 mg which yielded his first decent night’s sleep in weeks.  The next morning he woke up expressing gratitude and joy, not just for a good night’s sleep but for a good and joyful life!  How lucky are we?!

Unfortunately, the second night at that dosage was not so successful.  Try, try again.

I heard “Dexamethasone” while listening to a Webinar on COVID and blood cancer.  Dexamethasone is given to COVID patients in serious condition. Why did I know that drug?  I opened the kitchen cabinet where Jeff’s pharmacy is kept and saw that, yes, Dexamethasone is what he swished around his mouth for GVHD of the mouth and throat.  Dr. Porter said to swish it as long as possible.  Jeff got quite good at communicating with hand signals and grunts during his sustained swishing.  The stuff worked very nicely. He is left with only one persistent sore which is now treated with gel mixed with that gritty stuff we used to put in our babies’ mouths for teething.  When I couldn’t find it in the baby aisle the pharmacist directed me to the oral hygiene aisle.  Evidently, the pain reliever in it makes some babies stop breathing.  Geez!

Dr. Porter is pleased that Jeff’s liver counts are better but wishes they were good.  Besides sleep issues, other side effects that Dr. Porter attributed to Prednisone include weak voice, dizzy spells, dehydration and nocturia, also “reactive” kidney and CO2 numbers and dropping platelets.  Another bone marrow biopsy is scheduled for May 24^th after which time we’ll learn whether the steroids can be tapered again.  Dr. Porter plans on getting Jeff on Tacrolimus as soon as possible because he can’t stay on high doses of Prednisone for too long. 

Whenever I hear “Tacrolimus” I remember asking a nurse at Penn why Jeff’s Tacrolimus was the only IV drug brought in a glass bottle.  The answer was, “Because it would eat through a plastic bag.”  Naturally, I have mixed feelings about oral Tacrolimus.  I still think of it as pretty nasty stuff. 

Last month I hosted a writing workshop with BMTInfonet.org as part of their annual symposium.  I was touched by the expressive writing done, particularly by two women who described what it was like to live with chronic GVHD of the eyes. One of the writers described her vision exactly as Jeff had, “like looking through water”.  When the other writer said she had three eye doctors in four states I became concerned that chronic GVHD of the eyes could become Jeff’s plight.  Jeff visited his eye doctor last week and scheduled a follow-up in a month.  BMT survival takes a lot of specialists. (Jeff hasn’t complained about this for some time.)  What’s one more?

The adventure continues.

Hitting mile markers with the family

Moonface, Here I Come

Like glue to a first-grader, a little bit of GVHD is good but a lot is - surprise! -  a big mess.

Jeff has been growing more and more despondent.  Mouth pain and ulcers in his mouth and throat make mealtime less than enjoyable at times.  Shoulders sagging, he dutifully swallows his many pills.  Sighs, head-shaking, more sighs.  He complains of blurry vision.  A weird rash appeared on his torso. 

He'd begun a couple of new medications around the time new symptoms appeared.  He completed two courses of Amoxicillin prescribed by the oral surgeon with a week in between.  And he started Ursodiol (“liver flusher”).  We began to think that his mouth ulcers and skin rash were an allergic reaction to medication.  After all, Donor Nicole is allergic to many antibiotics, including Amoxicillin.  As today's oncology appointment approached we began to believe the skin rash and mouth/throat ulcers, together with the high liver counts, were a good indication GVHD had arrived in force.  

We were right.  At today’s visit Dr. Porter had Jeff pull his lower lip down. 

“Now the upper lip.”  

Jeff did so but Dr. Porter had intended him to have both lower and upper lips pulled back simultaneously. Jeff awkwardly exposed his teeth looking like a horse under a vet’s examination.  

“That doesn’t look bad,” Dr. Porter said.

“Yeah, but they don’t feel like my lips.  They’re dry and numb.”

According to Dr. Porter, Jeff's blurry vision is also attributable to GVHD.  They will cancel the liver biopsy which was to happen tomorrow (to rule out any other reason for the high liver counts and was important to do if only the liver was affected), and the pre-procedure COVID test.  Stop Ursodiol which didn't appear to do anything for his liver anyway.  Add steroid mouth elixir, high dose of oral steroids, Bactrim 3X a week.  Continue antifungal and antiviral meds.  And check blood on Monday to see whether it is safe to begin tapering the steroid dose.  

Seven vials of blood were drawn.  Seven precious vials of what Jeff calls “Nicole’s blood.”  Results:  white blood cells in the normal range, Hg nearly normal, platelets dropped a bit and, of course, very high liver counts.  

Jeff’s primary question for today’s visit was not answered favorably.  He had high (unreasonable) hopes that Dr. Porter would allow his PICC line to be pulled out.  Oh, the disappointment!  He seems more upset about keeping the PICC line than about the warning to reduce sweets while on steroids.  

GVHD is what Jeff's medical team was watching for, expecting.  Dr. Porter considers it a good thing.  He knows what to do about it.  Still, we know how tricky it can be to get off of steroids and, in the meantime, adjust doses, treat side effects – and develop moon face.  It is unfortunate.  We’ll deal with it.  

A Little GVHD

We are relieved to have had our second doses of COVID vaccine.  Jeff had a sore neck, shoulder and arm the first night and vomited once the next morning.  I remembered too late that our pharmacopeia includes plenty of anti-nausea medications.  As soon as Jeff’s tummy settled a bit he took a Compazine pill and was fine thereafter.  He was perturbed to have had a reaction to the vaccine (“What next?!) particularly since I had only a very mild headache and almost no pain at the injection site.  I rolled towards him in bed and he griped jealously, “You can lay on that arm?!”  Later I gloated by waving my arm all around.  It isn’t fair, I know.

For two weeks Jeff’s liver counts were elevated, indicating Graft vs. Host.  We began to worry that Jeff would have to start steroids for GVHD before his second COVID vaccine or before his body would have time to build antibodies.  On Wednesday Jeff had his second vaccine in the morning and had labs drawn at home in the afternoon.  The results were posted on Penn’s portal on Thursday morning – even  higher liver counts.  Ugh.  Nurse Heather – via Bluejeans video conference - assured us that “a little GVHD is okay” as long as it accompanies GVL (Graft vs. Leukemia).  So far it does not have to be treated.  Heather says we don’t want steroids to suppress the T cells.  (Nicole’s lovely T cells are doing such nice work; Jeff’s blood is again 100% donor cells!)  If it becomes necessary to treat for GVHD, then we will deal with it.

Heather told us about a drug that is standard for some transplant patients with GVHD of the liver.   Actigall/Ursodiol acts by flushing the liver and might be a reasonable alternative to steroids in Jeff’s case.  Dr. Porter later agreed and prescribed it.  Jeff says it’s a blessing to live long enough to have new drugs and treatments available to him.

While discussing Jeff’s sore breasts, Heather said she was glad the pain was bilateral, less chance it could be cancer.  She searched for the last time his hormones were checked.  She laughed and said she’d brought up a pregnancy test from 2011 which, oddly, is part of a panel of tests used for all patients.  “It was negative,” she quipped.  At his doctor visit later this month, hormones will be checked. 

We were able to report that the rosacea has been manageable since the dermatologist changed Jeff’s medication.  When Heather asked what Jeff is doing to take care of himself, he said he’d had only a half a beer three weeks ago (now no-beer due to the liver issue), eating fruits and vegetables and some sweets and treats.  We told her about yoga and our walks.  Jeff told her his feet hurt, are super-sensitive/numb, and is concerned it may impede his ability to go on long walks.  She recommends Epsom salt baths and Vitamin B6.  And more yoga.  She told Jeff he is doing well, “You’re on autopilot.”

He laughed and said, “Heading for a crash?”

“No!  Smooth sailing!” 

We went on another walk along the canal towpath, getting in shape for a 5K (walk, don’t run!) for Gift of Life in May in the Philadelphia area.  Nicole, Jeff and I have been asked to participate in the virtual opening ceremonies.  We are pleased to tell how Gift of Life connected Nicole and Jeff and how Nicole gave Jeff the gift of life not once, but twice! 

Body Parts

Jeff was doing well enough to drive to the city, I thought.  He is alert and clear-headed, lasts longer before requiring a nap, and is stronger.  When I suggested he drive himself to his in-person appointment with Dr. Porter, he was surprised. 

Some of what it takes

“Do you think I can?” 

“Yes, don’t you?”

“I guess I can!”

He was like a teenager being granted car privileges for the first time, excited and a little apprehensive.  

In Dr. Porter’s office, Jeff called and put me on speakerphone.  Dr. Porter is very happy with Jeff’s blood counts.  Platelets and white blood cells are in the normal range and hemoglobin is inching towards normal, in the 11s now.  Jeff reported that he is still getting winded when climbing stairs but Dr. Porter says he can no longer blame it on the hemoglobin.  Time to get in shape! 

I referred to my list of questions and asked if Jeff could enjoy a beer once in a while.  Dr. Porter said while watching for GVHD he would prefer Jeff avoid alcohol which can give confusing results on blood tests.  They will need to watch his liver closely in this window of 1-3 months post-DLI.

“A half a beer on occasion would be okay,” Dr. Porter conceded.

Not a problem.  Jeff isn’t much of a drinker but would enjoy a beer – or half of one – now and again.

Jeff also reported some weird issues with various body parts that he might have mentioned a month or two ago but didn’t.  He had something on the roof of his mouth for months until one night while eating tortilla chips, it disappeared.  Dr. Porter looked at the area and referred him to his dentist.  A semi-annual visit with the dentist was already scheduled for later that day.

The dentist took an x-ray, diagnosed an abscess, prescribed an antibiotic and said Jeff needed to have the molar extracted in the next couple of weeks.  There is bone loss in the area and it is quite infected. 

The first oral surgeon we called wanted to know if Jeff needed hyperbaric treatment prior to the surgery, how many rads he received during his full-body irradiation in 2011.  We checked with Dr. Porter who deferred to the oral surgeon.  We were left wondering, “Is hyperbaric treatment really necessary?”

After consulting with my sister Judy (a retired hygienist) and, through her, a dentist whose opinion she values (a former boss), we decided that it was better to get the molar out sooner rather than waiting for a series of hyperbaric treatments.  We called another oral surgeon who could accommodate Jeff right away.  Jeff was asked which of their two surgeons he would like.  Jeff, not familiar with either one of them, quipped, “The good one.” 

The surgeon did not want to be too hasty.  Jeff assured him that Dr. Porter had cleared him for surgery – better not to have an infection!  I presented the lab results on my phone and, finally, he was satisfied.  The man was thorough and, although he lacked bedside manner, we appreciated the care he took. 

The tooth was easy to extract as Judy said it would be, given the bone loss in the area.  It came out in one piece and yet Jeff thought there were a lot of tooth bits flying around.  He has had relatively little pain in the area since.

Another weird body part issue he might have mentioned but didn’t:  sore breasts.  Nurse Heather joked, “Are you ovulating?”  Jeff thinks that’s a sign that your medical professionals know you really well, when they can joke about anything.  Dr. Porter says we will have to pay attention, wait and watch.

Yet another weird issue: facial rash.  We’d thought it was mask-ne (acne in the mask area) and Dr. Porter agrees.  Nurse Heather, however, remembered reading a report from the dermatologist that said he had “quiet” rosacea.  Well, it isn’t quiet anymore.  The right cheek has splotches that can be subtlety pink or flaring red, pimply or hive-like, slightly raised or quite swollen.  Sometimes his eyelid, nose or forehead is also affected.  The changes occur rapidly and often throughout the day. 

“I’m like a mood ring,” Jeff says.

Jeff and I have both gotten our first COVID-19 vaccines.  It is embarrassing for me to admit that I have gotten my vaccine - not old enough, not sick, maybe not my turn.  Jeff, of course, is Phase 1 and eligible for a vaccine.  Our family is relieved Jeff finally got vaccinated before the potential for GVHD, thanks to a tip on available appointments from Niece Melissa.  Family members believed I should be Phase 1, as caregiver to an immunocompromised person.  However, that does not qualify me in Pennsylvania.  Son Kerry insists I qualify in Phase 1 because of my BMI.  I rationalized; maybe that is a valid qualification for me.  I have been concerned about my fitness at a time when we are supposed to stay healthy to fight COVID should we contract it.   I had not been exercising as usual because Jeff is often eager for company after my at-home work day.  If we exercised at all, it was at his level and speed.  So, I decided to Own My Obesity and schedule an appointment. Kerry grabbed an appointment for me one week after Jeff’s.  Because Jeff got the Moderna vaccine (2^nd dose at 4 weeks) and I got Pfizer (2^nd dose at 3 weeks) one week after Jeff’s, our second doses are on the same day!

Through our friend Michael, I was put in touch with BMT Infonet, a post-transplant group out of Chicago.  They invited me to facilitate a writing workshop at their annual symposium in April.  I’ve had my training for the virtual event and look forward to hosting probably three sessions, probably caregiver groups, to spend time exploring and practicing writing techniques that are useful for healing.  There is a waiting list of 150 people for these sessions.  Obviously, there is a need.  What a gift to be able to spend time with other people connected to bone marrow transplantation and who are interested in writing.  Visit BMTInfonet.org.

First Towpath Hike of 2021

Jeff and I take increasingly longer walks around our neighborhood.  Today we resumed our Delaware Canal Towpath hikes with a 5-mile trek.  Jeff was proud he could keep up and last that long.  He was a little stiff at the end but wants to get out there again very soon!

Thank you for caring about Jeff and me.  We are grateful every day for the people we have supporting us, near and far. 

Feeling the Love

Nurse Heather described DLI (Donor Lymphocyte Infusion) as uneventful and I was unprepared for the emotional toll it would take.  The infusion itself wasn’t concerning but we watched the weather forecasts, wondering whether those beautiful cells could make their way from sunny Florida to wintry Philadelphia – and us from home to Philadelphia – between snowstorms.  Well, truth be told, I suppose there was a certain amount of angst about getting to the other side of DLI regardless of the weather.

Wednesday was collection day.  Donor Nicole and her husband, Scott, were at the Gift of Life Marrow Registry headquarters in Boca Raton Florida for the occasion.  They’d flown in early to enjoy some time with Scott’s family in the area and to have a nice beach get-away. 

During a 7-hour process, Nicole’s entire blood volume was circulated through a machine twice (!) to collect lymphocytes for Jeff.  Both of her arms were occupied so she binge-watched a TV series.  Scott was kind enough to post pictures on FB – Nicole hooked up to the machine, a wall of pictures of Nicole during her marrow donation 10 years ago and of she and Jeff at the Steps for Life Walk/Run where they first met, and Nicole watching the video that we were asked to prepare for her.  The tears in her eyes as she watched our video sparked many of us up north to shed a few tears ourselves.  The last photo Scott posted was Nicole holding the bag of T Cells and modeling her new Gift of Life Donor T-shirt.  (“I know you did it for the swag”, I later teased her.  She replied, “and the trip to the beach!”) 

Meanwhile in Pennsylvania, I was working.  I should have taken the day off because my mind’s ability to concentrate was impaired by my excitement and anticipation (a la Christmas Eve my entire life) and Jeff, I think, wanted company while our phones kept dinging with well wishes from family and friends. Jeff’s Home Infusion nurse came to change the dressing on his PICC line and to draw blood.  She joined in our excitement and indulged Jeff as he proudly showed her the pictures Scott had posted on FB.  And we got a phone call from the transplant coordinator at Penn to tell us that his infusion appointment had been changed from 8:30 to 11 and a 9 a.m. lab appointment was added so they could type and cross his blood.  We knew that would add hours to the day.  So be it.  And we watched weather reports and contemplated staying at Daughter Kim’s place in the city overnight.  In the end we did not.  Only 1” of snow was predicted.  The main roads would be fine. 

A Gift of Life staffer told Nicole the cells would be sent via courier to Philadelphia.  We figured the later appointment would allow for potential weather-related flight delays.  Maybe everything would come together as planned.

On Thursday we arrived early at Penn’s Perelman Center.  We didn’t have any problems getting into the city despite the weather reporters stressing “allow extra time”. We ate our breakfast in the car before Jeff went to his lab appointment.  They were able to take him early so that he was finished with his 9 a.m. appointment before 9 a.m.  We waited an hour in the Perelman Center’s public area until Jeff, eager to get things going, suggested we walk over the bridge to the hospital and wait for his 11 a.m. appointment in their waiting room.  On the bridge, we followed a woman in a lab coat carrying a red and white cooler.  Jeff said, “That could be my cells or that could be her lunch.”  We were tempted to ask. 

The hospital lifted some COVID-19 restrictions and this was only the second day they allowed companions in the Apheresis and Infusion Center.  The staff at the reception desk all looked up from their work and smiled at us.  I wondered whether they knew it was Jeff’s special day.  Despite arriving one hour early, we hadn’t even settled into chairs in the waiting room before Jeff was called and we were shown to a room.  Nurse Dana said the infusion would begin at noon at the earliest because of the time-consuming type and match that had to be done on Jeff’s blood and Nicole’s T cells.  I asked if they would prefer if we came back later.  She assured me the room was ours for the day.  Jeff watched TV and I read a book.  We also exchanged texts with Nicole (I believe she may have been a little anxious about all this, too) and family (ditto).

I asked Nurse Dana a question that had occurred to us.  Previously, when Jeff’s blood and donor blood were checked for antibodies, we thought if there was a problem the lab would simply go to a shelf of blood products and test a different donor for matching antibodies.  In this case, what would they do if there was a mis-match with Nicole’s?  Today she is Jeff’s one-and-only.  Dana said she wasn’t sure how to answer the question and she would have a pathologist come to explain it.  Well, we got an education from a very nice, very animated Penn professor.  The upshot is, should there be a problem, they would know to watch for a reaction.  They’d administered Tylenol and Benydryl, I believe as standard procedure.  When the lab was within one hour of completing their work, they alerted Dana so she could begin giving fluids.  An hour of fluids, almost an hour for infusing T cells (slow rate and large volume - three times the usual amount for some reason), another hour of fluids.  Jeff slept through most of this.  4 p.m. Done.

Jeff felt no different.  He felt well enough to watch our youngest granddaughter on Friday.  On Saturday we did nothing.  N.O.T.H.I.N.G. 

Lab results from Wednesday’s at-home, pre-DLI blood draw were posted to Penn’s portal.  WBC much improved, 3.1!  Hg 10.3!  Platelets 98,000!  All heading towards normal!  Nurse Heather had told us that the biopsy showed 96% of his marrow is Nicole’s (pre-chemo it was 76%).  It seems like Nicole’s T cells only have a little bit more work to do, chomp the remaining cancer cells.

Jeff to Nicole:  I hope your cells are hungry.

Nicole to Jeff:  They are!

Jeff theorizes that his body already recognizes Nicole’s cells and that GVHD may not become a problem for him.  Nurse Heather says they really don’t have any idea because Jeff’s situation is rare:  ten-year bone marrow transplant survivor, subsequent DLI infusion, marrow and T cells from the same donor…

Strange:  Jeff is suffering from Mask-ne, blemishes on his face in the mask area.  At least that is what Dr. Amy thinks it is.  It is quite persistent in two spots.  Jeff says if it had happened after DLI he might have suspected GVHD of the skin. 

Stranger yet:  A week after finishing treatment (hopefully forever) ads for Venclexta began popping up on Jeff’s FB feed.  Really?  Are you supposed to ask your doctor if this medication is right for you?  Well, Genentech, impressive/creepy ad-targeting but you are a little late to the party.

Many, many thanks to everyone who has been praying for Jeff, sending messages of love, and supporting Nicole’s birthday fundraiser for Gift of Life.  We enjoyed reading the messages Nicole’s family and friends posted on FB in support of her wonderful-ness and appreciate their prayers as well.  We are feeling the love!  XOXO

Happy Valentine’s Day!

Car-antining and “Boca Baby!”

For months we’ve known that Donor Nicole would be asked to donate T cells for Jeff.  Finally, last week Nicole received her official notification from Gift of Life that she is needed.  She’s been eating less sugar and more greens to keep her blood healthier (Isn’t that sweet!  A double-blessing – for her and Jeff!).  T Cell collection day will be the same day as infusion day but she is in Georgia and we are in Pennsylvania.  I remembered that she and her mother were flown to New York for collection of her bone marrow 10 years ago so maybe…

I texted, “Where will the collection take place?”

Nicole’s reply, “Boca Baby!”

Ah, the beautiful new donation facility at Gift of Life Marrow Registry in Boca Raton, Florida!  https://www.giftoflife.org/posts/post/gift-of-life-opens-new-headquarters-in-boca-raton  We are so happy for Nicole and her husband to get a little get-away.  Jeff’s nurse says Nicole will probably feel fine after the donation (unlike bone marrow donation which knocks the donor down a bit).  She and her hubby, Scott, will actually be able to enjoy themselves!  Donation Day is very near her birthday so she thanked US for the GIFT!  Ha!  Who is gifting whom?

After two units of blood on January 4^th and 5^th Jeff has not needed transfusions during this last cycle of Vidaza/Venetoclax.  And some of his numbers are beginning to turn in earnest.  Hemoglobin has been holding steady in the 9 range since receiving those two units of blood.  Blood transfusions only last for a couple of days so when Jeff maintained Hg in the 9s I suggested we try to find that donor with the magic blood!  As time went on it became evident that the improvement was lasting.  And Jeff’s platelets even wandered into the normal range once!  He still has almost no white blood cells but because his platelets are improving Dr. Porter gave the go-ahead for some deferred maintenance. 

Dermatology:  Dr. Samimi froze some spots on his face and shoulder and removed a weird cell on his back.  It often seems the spots that most concern me are nothing to her.  I hadn’t noticed anything on his face while the benign spot on his back was, well, ugly.  She deftly took care of it as well as the pre-cancerous ones.

Flexible Sigmoidoscopy:  Although the bleeding that concerned Dr. Porter in October/November had stopped, it was still worth having a look-see.  All good.

Dental:  Magic mouthwash is wonderfully numbing for treatment-related mouth pain but one particular tooth has been sore persistently.  Dr. Snead ground it down so it isn’t in the bite as much.  He theorizes that Jeff is grinding his teeth at night.

Jeff has dubbed my working-in-the-parking-garage-at-Penn “car-antining”.  Prior to today’s bone marrow biopsy, Jeff and Nurse Heather called me from the exam room while I was car-antining.   We were able to ask some questions about the upcoming T-Cell infusion.  That day, probably February 10^th, Jeff will go to Penn’s Stem Cell Lab in the hospital’s Ravdin building for outpatient infusion.  The infusion of Nicole’s cells may take no more than 20-30 minutes, depending on the volume.  Since this is so similar to treatment infusions, Heather describes the day as “uneventful”. 

Four to six weeks later we’ll be anticipating Graft vs. Host Disease (GVHD).  Jeff will be monitored for GVHD of the liver, gut or skin.  We’re familiar.  Jeff had GVHD of the liver requiring immunosuppressants for several years after his bone marrow transplant until the disease settled down and left.  The tricky part will be the timing of his COVID vaccination.

Heather says Jeff needs to register for the vaccine and he needs to get it.  However, his white blood cells need to be higher and he cannot be on more than 10mg of steroids when he gets the vaccine or it may not be effective.  It seems unlikely that he will be able to be vaccinated in that four- to six-week sweet spot - after T cells and before GVHD.  We’ll see.

Heather told us that some people yell at Penn’s staff for not providing the COVID vaccine. The hospital gets doses for their employees but not for the general population, even cancer patients.  We know Jeff will have to wait his turn and trust that Bucks County is doing the best they can with the limited doses they have available.

Jeff turned spoons on the lathe again for Dr. Porter and Heather. Heather wasn’t in the office on the day he left a spoon for her so Jeff asked, “Did you get your spoon?”

Heather was focused on the bone marrow biopsy she was about to perform using a “spoon” so the question initially puzzled her.  Then she realized which spoon Jeff was referring to and laughed.  “Yes!”  She thanked Jeff and said her husband really, really likes wooden spoons.

Jeff was buoyed by Nurse Heather’s encouragement today.  There’s a medical professional who treats the soul as well as the body!

Another ray of sunshine:  only weekly (instead of twice weekly) blood draws will be required going forward. 

Happy Birthday to Jeff!

What a day to start Cycle 4 of Vidaza/Venclexta!  Dr. Porter says Jeff’s birthday is a good day to start what we hope is the last treatment cycle because it seems to be working.  He thinks Jeff should be ready for Nicole’s T cells by early to mid-February!  And he observes, “You’re getting old and that’s a good thing.”

Jeff’s birthday gifts included dinner prepared by Son Kerry and DIL Theresa and delivered to our door, handmade cards from our granddaughters, a sweater vest from Daughter Kim, a silly video from Donor Nicole and texts and messages from family and friends including an early greeting (early to us in EST) from Friend Steve in New Zealand.

Birthday “treats” continued at Penn’s infusion center.  We left the house at 6:50 a.m. for what Jeff believed would be a short visit, just the Vidaza infusion.  Alas, his hemoglobin was 7.2 and he needed a blood transfusion. 

Jeff texted me in the parking garage, “How many people can say they got blood for their birthday?”

It takes such a long time to type and crossmatch his blood and check the donor blood for antibodies.  He waited three hours for the blood product during which time he was offered – and accepted – a MASSAGE!  What a treat!  They infused only one of the two units required (the other will be given following his treatment tomorrow).  His short visit turned into a whole-day affair. 

I kept myself busy for the duration.  I worked for three hours until my laptop lost power, picked up Jeff’s prescription at Penn’s specialty pharmacy, went for a long walk, got lunch, listened to an audio book and took a catnap. Finally, Jeff returned to the car at 4:30!  We got home at 5:30 p.m.  Somehow, the day was more tiring for me than for him. 

The compulsion to be creative and productive is strong in Jeff.  While hospitalized in October, he designed a floor lamp. Since then, little-by-little, he spent time in his shop turning parts of the lamp on the lathe, shaping other parts, assembling all with a giant screw from a bookbinding press we’d acquired at an auction many years ago.  The result is a great piece.

Some of the care Jeff requires is reminiscent of a baby’s needs:  soft foods, naps, baby powder, soothing.  My baby puts himself down for a nap and remains there reliably for hours, expresses gratitude for soft foods prepared and appreciates cooing and backrubs.  Although this journey is not a picnic we consider ourselves lucky to have each other.