The Only Guy

Our daughter Molly left protest-laden Madison, Wisconsin and headed to Chicago via bus and arrived around noon today.  Steve's liver biopsy was scheduled for 2:30.  Luckily his Mom and sister were visiting when Transport arrived at noon (don't ask, it happens) so they accompanied him to Interventional Radiology via the underground tunnel until Molly and I got to Northwestern.

The liver biopsy went without a hitch, about 10 minutes for the procedure. They gave him Versed as well as Fentanyl. No nausea.  His south end is good. Only vomited once today, this morning.  Starting IV nutrition tonight.  Bumped his anti-nausea drug, Zofran, from 4mg/6 hrs. to 8mg/8 hrs.  Increased his IV fluids.  Goal is to stop both ends from exploding.

Today's funny comment:  His IV stand is on wheels.  So you take Waltzing Mathilda with you to the bathroom.  If you are in a BIG hurry, you have to kick off the bed covers, and wheel the stand into the bathroom, drugged and possibly wobbly.  The IV stand has electric pumps with battery back ups.  You have to also unplug the IV stand, wasting precious moments.  Steve figured out today that he has enough electrical cord to race to the john without unplugging, and that was a major win.  Some days it's the little things like that that make a difference.

We are getting used to The Only Guy in sentences.  His nurse said he is the first adult patient she has ever given Desferal (chelator) to in 17 years of nursing. He is The Only Guy she has had to check a ferritin level for.  I asked, "so how do the other patients get rid of their extra iron?  Put it in an envelope and mail it??"  His labs were great:  

WBC 8.6

Hemoglobin 9.7

Platelets 303

Ferritin has dropped to 3663 (Normal <400, he was as high as almost 9000)

Bilirubin is UP to 9.4 which isn't so wonderful

He is on a strong steroid, so that suppresses the immune system, his white cell count (WBC) was 10.3 a day earlier.  He told me the Stem Cell Team, during rounds, is 90% sure his symptoms are truly GvHD and they are (and have been since a week ago Monday) treating him with steroids to combat the GvHD until the liver biopsy results confirm their suspicions.  Taking him off Exjade, the first chelator, was the easiest thing to do first.  He is back on the Tacrolimus and was resting very comfortably when Molly and I left at 6:30 tonight.  He was very, very weak today but mentally sharp and positive about his health outlook, counting on a rally soon.

No talk of discharge or surgery.  The team told him there is "no play book at this point," everyone is different and they will do their best to handle bumps in the road as they appear.  They want him to feel better before heading to the OR.

That's it for today, thanks for the read.

Holding Pattern

Not much to report tonight, I spent the day at home working on paperwork and made a nice dinner for Kevin and I. 

Steve spent the day resting after a good night's sleep.  Late afternoon he was transported to Prentice on the 15th floor, his old stompin' grounds.  He knew both his RN and his PCT, so he felt like a celebrity.  They are keeping pain and nausea meds going, and he said he feels better than yesterday.  Only one episode of vomiting.  I hope that clears quickly.  He did have Desferal overnight to keep chipping at the ferritin level. 

He asked for IV fluids and has been drinking water like mad. The surgical resident, Dr. Fronza, paid a visit today and said they are a go for Friday, but deferred to Dr. Altman's opinion once she returns in the office tomorrow and evaluates Steve's current level of health.  His hemoglobin is over 12.0....imagine.

Thanks to all of you for your words of concern, prayer and help.  I have been meaning to tell you how flabbergasted I am when I see or speak to people who not only read the blog, but can quote from it!  I've sure learned a lot about medical things, and even more about people.  Closing tonight with Steve's nap companion, our Chocolate Lab, Cooper.

...And Back Again

Sunday and Monday were rough, and I was really looking forward to our home health visit.  The Desferal infused with no trouble on Saturday, yet Steve begged me to take Sunday evening off in case the drug was making him feel even worse.  If you are in on the "please refresh my water" joke, he was in full swing over the weekend.  Our icemaker was running 24/7, and so was I, hahaha.  

His nausea turned into vomiting on Monday, not often, but still consuming him emotionally.  I started playing with ideas of admission.  Dug out some Ativan from backstock to see if that would help his stomach.   He hasn't eaten since Friday.   After home health left, I started making calls to the hospital.  Everyone was in agreement that coming in was a good idea.  Dr. Altman is out of town, and was contacted.

His labs from today show improvement in liver enzymes and more big improvement on the CBC side again.  They did a ferritin level but no one seemed to have the result as of late Monday.  His bilirubin also moved up, bleh.  Much improvement on his "south end" frequency, so some good news there.

Bless Steve's cousin, Bill.  Our sewer line has clogged for the third time since Steve's illness, and Bill was going to come over after work and do the dirty work.  Instead, he became my wingman, and a fine wingman he was!  We got to the ER at Northwestern around 7, got seen at 8 on the Mezzanine Level, and taken to a room in Feinberg around 11.  Prentice is still full up, and the plan is to transport him when room becomes available.

While in the ER, Steve's nausea and pain were well managed, and he was able to chill and even joke a bit.  Dr. Altman called me on my cell phone from who knows where (not Munich, that's later) and told me of her plan.  Our ER doc said Altman e-mailed a "very detailed chart and plan" of Steve's care to them.  (HAHA.) She feels very badly that Steve has been suffering and wants to get a handle on this now.  At first she thought about starting him back on (anti-rejection drug) Tacro in the morning; by the time he got to Feinberg, orders were for the Tacro to start tonight.   She feels that his elevated ferritin lab value may NOT be due to just iron, it may also be from liver inflamation, and the sharp decline was possibly from steriods, not necessarily just the Desferan.  There was mention made of a liver biopsy coming, which would give a definitive answer on GvHD as a cause of the GI issues.

Don't know what this means for surgery this week.  Goodnight.

Days of ('Roid) Rage

Simply cannot believe it was just Thursday that I last posted.  It has been a whirlwind of activity here.  Steve's ferritin (iron overload) level was not acceptable at such a high, dangerous level.  Normal is <400, his was teetering near 9000.  Iron can deposit in the liver and/or heart and cause organ failure...you get the picture.  He was feeling sicker....

The first dose of Desferal has to be given in a controlled hospital setting for both health and insurance reasons, understandable.  All outpatient facilities at Northwestern as well as Christ were booked for Thursday and Friday, so we went onto a waiting list for inpatient rooms at Prentice/NW.  I was, quite frankly, relieved, as he seemed medically fragile and not at a level I felt able to handle at home.  He doesn't seem well enough to have surgery of any type, so this was a great opportunity to get plumped up and game-ready.  The call came at 10 on Friday (2/18) morning.  He was vomiting at home before we left the house.  ::SIGH::

On a humorous note, this was his 11th admit.  We school that place!  He adjusts the thermostat, switches out the hospital pillow for the home pillow,  I set up his clothes/shoes/blanket.....the intake questions are answered with military execution, and then I had to recite his med names, dosage, and frequency by heart.  I have to close my eyes to do that.  Had an outstanding hospitalist, Dr. Burke, and she was snappin' and tappin' her way through *the process* of hospital admission.  Patience is truly a virtue.  We were told that Steve is one of only 2 patients at NW (think that's right) that have received Desferal SC (subcutaneous).  Basically, they insert a small-gauge needle into his stomach "fat" which does not currently exist, and very, very slowly infuse the med over about 12 hours.  Dr. Altman was on a flight to Munich for a leukemia conference, and calls were made to cross t's and dot i's.  I left at dinnertime.

No one knew how he would react to the infusion due to the Hereditary Spherocytosis and the elevated ferritin and bilirubin levels.  That ALL changed this morning.  Steve's ferritin was reduced by HALF in the first dosage.  UNBELIEVABLE!!!!!!  His hemoglobin is now at 9.4!!  Awesome.  Bili is still over 7.  Of course, he didn't sleep worth a hoot.  Needed a wheelchair to get downstairs for the ride home.  Thanks to Dave S, who was my wingman today, we make a great team! 

Steve is really suffering from the steroids today, and tonight was really tough.  He is on 40mg Prednisone x1 daily, 3mg Entocort x 2 daily, and 5ml Beclomethosone x4 daily.  The second two are often prescribed for Crohn's Disease patients, helps calm the GI.  None of our practitioners are sure why his GI is such a bad state so quickly.  Many possibilities, and we are on course to knock it out.  

If you have never dealt with a steroid patient, well......let's just say it is interesting.  You get screamed at, verbally abused, mocked, criticized, and then for fun, they sob and wail.  Sometimes they are totally silent and demand the same from their caregiver.  Sometimes they are manic and gabby.  Can't eat.  Eat like a horse.  Their thought processes go haywire.  The emotional rollercoaster is awful to watch.  He was afraid we were running out of comfort food today, so I ran to the store and tried to buy food I thought he would like.  So he asks, "what did you buy?" and then he didn't want to eat.  It's a living hell.  I try to give him his meds and he is sick to his stomach.   Yet the meds are to help the upset stomach.   If you get off task with the 'roid dosage, he crashes.

So tonight is dose #2 of Desferal.  There is a little battery-operated pump that will be our bedmate for a few nights.  The Desferal attaches to the iron and causes the body to excrete the iron overload through urine and poop. I can't help but feel that some of his GI disturbances are from both holding and dumping the iron.

We hope to see improvement in his health tomorrow.  I have to get him downtown once before his surgery on Friday  for his pre-op evaluation, which, quite frankly, is a pain in my Lithuanian ass.  Good news is that his INR level, which is the lab test they do to determine bleed-out risk during surgery, is "PERFECT."

Roll With The Changes

I can't believe how many things have happened since my last update.  Let's see, there was a blizzard, our first-born turned 21, Green Bay won the Super Bowl, and Steve had a MAJOR, MAJOR rally with his health.  

As last reported, Dr. Altman stopped Steve's anti-rejection drug and had Neupogen shots being given daily.  Numbers started creeping up, most importantly his white cell count, which was mysteriously not behaving at all.   Steve's last red cell transfusion was Friday, January 28, and his hemoglobin level was pretty good-ish going into that transfusion.  He has not had ANY blood since that date.  I think 10 days was the record before this.  Steve was sleeping well, eating like a pig, getting chores done (imagine!) and was enjoying feeling well again.  He went from looking sick to looking like a skinny guy.  If you had just met him, you might not know he had even been ill.  His hair, right now, is like puppy fur and doesn't have the reddish cast it used to have.  Of course the grey is coming in, but in a nice manner.

On Monday, February 7, his CBC results were:  

WBC 6.3 (ANC 4.7)

Hemoglobin 7.1 (10 days post-transfusion)

Platelets 120!

Much like a mother sending her child to school for the first time, Steve went alone to clinic on the 10th.  He felt great, and it was time.  He had a bit of a swagger goin', and the staff was happy to see him flying on his own power.   Labs from February 10th:

WBC 8.9 (ANC 7.3)

Hemoglobin 7.0 (hanging in there!)

Platelets 148

However, once the chem panel came back, the tune changed.  Dr. Altman called later that day and said that while it certainly looked like the marrow is starting to work and produce cells in larger quantity, she was concerned about newly elevated liver enzymes and a bump in bilirubin.  She asked that he come to Northwestern on Monday (2/14) and have ultrasound imaging of the liver as well as another blood draw.  Many, many things could be factoring into elevated enzymes.   She told me to discontinue the Neupogen shots as his white count was FINALLY normal.  At that time she also discontinued his magnesium supplements.  That was a long time coming.  (Good mag = good energy!)

As this past weekend progressed, Steve's general health began to tank.  His color got bad.  If you have known him a long time, you know the look.  It's orange.  His eyes jaundiced.  He looked gaunt.  I convinced him to take IV fluids on Saturday and Sunday just to keep dehydration at bay.  He began to tire and take to the bed.  We cancelled dinner reservations at his favorite restaurant, the one he dreamed of eating at while going through chemo.  He wasn't able to stay for Kevin's Boy Scout dinner, had to leave.  He looked sick.  My gut feeling was that we were in trouble.  We were.

Monday's scan was fabulous.  The tech told him he looked great (internally speaking) and that he should be an "ultrasound model."  Seriously, they need bodies when new equipment is delivered and demoed to the staff.  He was flattered.  Labs had been drawn before the scan.  I had a feeling we would go home and get a call back, so I got my Snappy Gutsy Patient Advocate Hat on and ever-so-politely pushed for a visual assessment of Steve's look up in clinic.  We were accommodated in Lurie.  (Thanks to one of my home health nurses for egging me on, BLESS YOU!!)  He had lost 6 pounds in 4 days.  "I'm wearing lighter shoes," he says.

This was one of those (many) times where Steve and I have locked horns.  The staff sees this often in married couples, and while it can be humorous, a la The Honeymooners, they have to have exceptionally fine emotional radar and people skills to read both the patient and the caregiver.  The patient felt he was just fine, only tired.  The caregiver had her arms and legs crossed while cocking her head to the side and rolling her eyes.  Dr. Altman quipped, "I feel some anger?"

At this time I will mention that over the past 2 weeks he has had much increased lower GI activity.  If this was MY health, you would have an Excel chart of time, consistency, color, smell, and some fun pictures of the dog hair on the bathroom floor.   To protect his privacy, I will say that I have been concerned and alarmed.  I report his stats to the nurses and Dr. Altman, they nod in agreement, and we "watch" as this could be the precursor of GvHD (graft versus host disease) which must be nipped as early as possible.  Monday's CBC results:

WBC 8.4 (ANC 6.5)

Hemoglobin 7.7 (Whoa, going UP?!)

Platelets 199

During our visit on Monday, I pressed for a discussion about his iron chelator, Exjade.  While a wonderful, new drug, it does have a black box warning of stomach bleeds and liver failure, but mostly in elderly and medically comprised people.  Steve, since the weekend, was having stomach pain along with the lower GI issues.  We went home with two steroids and a liquid compound that helps the stomach.   The steroids are to fight possible GvHD.

At 9:15 Monday night, Dr. Altman called the house, and she was very concerned.  Steve's liver enzymes were not good at all, very elevated.  (I know jack on this value, hasn't been in my all-star lineup) and his bilirubin had skyrocketed.  A normal bilirubin level in an adult is 1.0.   Steve's had been 1.0-1.5 during his chemo/transplant.  Thursday the 10th it was 1.8.  Monday the 14th it was 7.2. She pulled the Exjade and said, "I want him to have his spleen removed next week." 

Possible reasons for elevated liver enzymes and/or elevated bilirubin:

• Liver less able to handle toxins from Rx
• NEW cells are making spherocytes (irregularly shaped red cells) as Mary, our donor, also has Hereditary Spherocytosis
• Spleen can't destruct the spherocytes fast enough as he only has 20% splenic function after 2 splenic embolizations, which creates a back-up of sorts
• Exjade (iron chelator)
• Extremely high ferritin level (iron overload) from excessive red blood transfusions.  I believe Dr. Altman said a normal ferritin level was <400; Steve's is 8000+  This can cause liver or heart damage if left untreated.  The Exjade does not seem to be bringing the level down.

In a nutshell, Steve's transplant is working so well that his other condition, the HS, can't keep up.  Both Dr. Altman and Dr. Jayesh Mehta, Director of Stem Cell Transplantation, conferred and agreed that Steve has significant hemolysis and needed the splenectomy NOW versus later.

Today we had a surgical consultation with Dr. Alexander Nagle, a GI surgeon.  Dr. Nagle is operating on an off day to accommodate Steve (and don't fool yourself, to please Dr. Altman, HAHA! Bless her heart for advocating for him!) and plans on a successful laproscopic procedure.  Steve was told it was a 75+% change of scoping, yet we know there is always a chance of an open conversion.  With the spleen, open surgery is now referred to as a "hand assisted" (GROSS!!!) laparoscopic procedure.  Think Napoleon.  Dr. Nagle will remove Steve's spleen and gall bladder.  There is some indication on the ultrasound of gall stone formation in the gall bladder, so it's gone.  He will also do a hernia repair while he's there.  I am going to try to throw in a tummy tuck and scar reduction if I can, shhhhhh, don't tell Blue Cross.

Dr. Altman quickly ordered 3 major vaccinations and Steve received them this afternoon.  Remember,  his natural immunities were lost during consolidation chemotherapy.  He will have to have all his childhood vaccinations again...normally they don't immunize until the one year post-transplant period, however, a splenectomy trumps the waiting period.  Today (February 16) his labs were improved:

Bilirubin went down to 5.8 from 7.2 (good)

Hemoglobin is up to 9.1 (Olympic)

Platelets are 252 (surgeon will do the happy dance)

WBC went down to 4.8 with ANC of 4.0 (no biggie, she said)

Liver enzymes went down considerably, but still too high

Surgery is scheduled for Friday, February 25. 

A few closing thoughts on the spleen.  Removing the spleen does not cure anyone of Hereditary Spherocytosis.  The body will still create the sphere-shaped cells.  It will cease to destroy, or hemolyze, the red cells, curing the chronic anemic state.  While Steve's spleen has been the cause of a lot of grief to him, it may have saved his life during his leukemia chemo and stem cell transplant.  Steve's immune system was destroyed from the chemo.  The spleen filters microencapsulated bacterial infections and other harmful infections.  Who knows what he may have contracted without his spleen for protection???  We will never know.  What I *DO* know is this:  If you Google image "Steve Reppen's Spleen," you will see this: