The Saga of Sam

The Saga of Sam

Wednesday was back to the routine; school, homework.  The family is shell shocked at this point.  We function on sheer determination.  Our direction is unclear.  Somewhere prior to here, I had told you that we couldn't be concerned with the day-to-day panics.  We had to take one day at a time and expect trauma.  It was part of our healing.  We agreed to look at where we were on July 4th.  Hopefully, we would sit on the beach at Cape May and see the positive trend.  The Penicillamine would have time to work.  The liver function would return.  By Christmas, we would look back and say what a bad dream that was.

That evening you complained of an upset stomach.  You were up all night.  Rubbing your back seemed to help.  There was little or no sleep.

I took Shaun and LT (our neighbor) to the bus in the morning.  While waiting, Mom beeped me.  I had started to wear a beeper recently to keep constant contact with you and work.  I waited for the bus; Mom beeped again with three "1"s after our home number.  We had agreed to use this as an emergency signal.  I left Shaun and LT on their own, and returned home.

You had vomited a cup or so of bright red blood.  Mom called Dr. Schwarz and she instructed us to meet her at the JHU ER.  Fortunately it was still early (7:00 A.M.) and the Jones Falls expressway was relatively clear.  You and I took a distressed but uneventful ride to Hopkins.  We always took the JFX to Guilford Avenue to Monument Street to JHH.  Monument Street is a mess and will be rebuilt soon.  To date it has been an annoyance, now it is frightening.  We bounce gingerly down Monument Street to the ER.

We arrived, checked in, and you headed to the bathroom.  A young doctor, Martin Hanawalt, stopped you.  You told him you needed to vomit.  Time ran out and you vomited a large amount of old and new blood.  It was quite a scene - like something from TV.  Martin grabbed you and ran to the treatment room.  Blood was everywhere.  This probably was the first time I questioned your recovery.  The ER staff worked quickly.  IVs were started and a Blakemore tube was readied.  A Blakemore tube is a large tube with a bladder that is inflated after insertion into the esophagus to stop gastrointestinal bleeding (GI).  It is an ominous-looking instrument.

Your throat was numbed, the tube lubricated, and you sat up.  I stayed in the room, but was relegated to a corner.  Your fear was obvious.  Your fear was my fear.  Your pain was my pain.  They pushed the Blakemore down your throat, you choked it back up.  They tried again, you choked it back up again.  This is insanity.  Sickness has changed to suffering.

Dr. Schwarz arrives.  Sanity restores.  She stops the Blakemore and calms you - us.  Mom arrives.  Dr. Schwarz begins transfusion of two units of blood and admits you to the Pediatric Intensive Care Unit (PICU).

This was one of the worst experiences of my life.  I'll never forget the look in your eyes during the tube insertion.  I'll never forget the helplessness.  The bond with Dr. Schwarz was sealed today.  Again and again, she will be the calm in the midst of the storm.  This storm was not yet over.

Kathleen Bull was our PICU nurse.  She had the dubious task of inserting a Nasal-Gastric (NG) tube, through your nose and down your throat.  We've had enough of tubes by now.  You are uncomfortable and distressed through noon, but finally fall asleep.  Sr. Schwarz has asked to talk to Mom and me.

The PICU is on the 7th floor.  There is a small consultation room with a love seat, two chairs and a telephone.  I thought we would discuss the plan, medications, and sources of bleeding.  I figured that the bleeding was caused by the medications, given all the vomiting and nausea you had been through.  Mom settled into the love seat, I into a chair beside Dr. Schwarz.  Dr. Schwarz said that you had three of the four indicators of children who do not make it to transplant.  Ascites, low bilirubin, low cholesterol are serious signs.  The GI bleed was caused by varicose veins in your esophagus (varices).  The GI bleeds always stop.  She had consulted the Wilson's Disease specialists in NY, and they agree with her decision to do a liver transplant.

I can't believe I'm sitting in this chair.  I belong next to Mom, but I had no clue this would be a transplant discussion.  We both cried.  I moved next to Mom.  Dr. Schwarz held Mom's hand and said, Dr. Colombani, the transplant surgeon would talk to us.  She described transplants as "miraculous".  I would not forget that word.  Dr. Schwarz left.  She was planning to do sclerotherapy at 3:00 P.M. to start to heal the varices.

A million questions were going through our minds.  Liver transplant was mentioned in the ER on the first day in March.  That week we had agreed to put you on the waiting list as a precaution.  It was not something we ever believed would be necessary.  In March you were "healthy", now on April 28, we are planning a liver transplant.  Unbelievable!  Quality of life is on our mind; Penicillamine, Wilson's Disease, liver transplant, rejection --- what are we doing to you?  As our minds cleared, we questioned Dr. Schwarz.  Good news - the transplant cures the Wilson's Disease - it is localized to the liver.  It's hard to believe, one of the things I feared most, now cannot happen soon enough.

Dr. Colombani, the head of the pediatric transplant team, stopped in.  If Dr. Schwarz is the calm in the storm, Dr. Colombani is the calm within the calm - really laid back.  Dr. Schwarz said he has a quiet control of everything around him, especially in the Operating Room (OR).  He explained the system.  Time on the list was important and you had not been on the list very long.  Some people are on it for years.  Clinical condition is important.  "One" is home without complications, "two" is home with complications, "three" is in the hospital, and "four" is on life support.  You are a "three".  He mentioned that with infants, they often do "living related" transplants.  The Mom or Dad donates a small portion of their liver to the infant.  They must match bloodtype and have the right anatomy.  That would be a possibility with you, but you would need a whole left lobe.  They have done this in Japan and Chicago.  Dr. Colombani has no doubt they could do it here.

Dr. Shwarz did the sclerotherapy and you were sedated for the rest of the day.  She planned to repeat the procedure on May 13.  Then, she will do it in the OR.