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: