Tag Archive | glycogen Storage Disease

God Bless Alice

“The best laid schemes of mice and men often go awry.”   Robert Burns

The performance was starting in an hour and I found myself doing the usual check.  Children fed and in their sleepers, pump set up clean, rescue supplies available, information about how to reach us and French horn by the back door.  My black dress probably should have been dry cleaned, but who notices what the musicians wear in the pit anyway.

Alice knocked on the front door a few minutes before we expected her and the kids were all giggles and silliness about spending time with the “girl” teenager across the street who knew how to ride a unicycle.  Alice was one of eight children whose mother was a nurse so I always felt confident that if she had problems she could request back up.  Goodnight kisses, two little boys waving “bye-bye” through the back porch window and their daddy and I backing down the sloping driveway on the way to a concert.  Playing my horn was a “filling the vessel” activity for me.  It was a night out alone one night a week, and more during dress rehearsal weeks, to refresh and have a change from the unrelenting responsibilities of raising a family and children with GSD.

Most of the other musicians were already warming up their instruments when I walked quickly to the front of the auditorium to take my place in the horn section.  I noticed my husband found a seat in the mid section not too far from the aisle.  I took my horn out of the case, put the mouth piece in place and blew warm air through the cool metal.  Business as usual.

About 45 minutes into the performance I noticed a police officer walking down the aisle toward the front of the auditorium.  That was weird.  Who needs police protection for a stage play like Oklahoma?  In seconds the police was bending down to talk to me.  The baby sitter needed me.  I quickly left the pit and relayed the message to my husband who immediately left the auditorium to take care of what ever Alice needed.

Back in my seat, I nervously kept playing my part and counting measures of rest.  What could have happened?  3-2-3-4  Did someone need stitches?  4-2-3-4 What did Alice need that her mother could not help her with?  5-2-3-4

I later was told that the boys had been horsing around and Scotty’s gastrostomy had been pulled out.  Old fashioned gastrostomy’s were about 10 inches long and had to be coiled and taped to the skin.  Apparently the tape was not a match for the two pre-school boys doing who knows what. My husband had gone to the pay phone (yes, there was life before mobile phones) to call Alice.  She told him the problem and he talked her through replacing it.

The problem was remedied, my husband returned to his seat in less than ten minutes, and the orchestra played on.  God bless Alice!


Lesson learned: You must care for yourself in spite of unseen risks.

A Trip to the Fire House


“Nothing in life is to be feared. It is only to be understood.”  Marie Curie

“Mommy, what’s happens if there is a fire in the night and the fireman doesn’t know how to unhook me from my pump?”   Compassion rose to my throat and I took my son into my arms.  “Sweetheart, that is such a good question.”

My next response was a pat answer about how they would figure it out and that they would probably take him pump and all.  Then I pictured the safe guards I had initiated to secure the lines filled with glucose for a sleeping, dreaming, rolling four year old.  Tape on the connection between his tube and the main line.  Tape on the bed posts to prevent the tubing from pulling the pump off the dresser.   His Dr. Denton’s sleeper with their custom button hole added near the ankle at the bottom of the zipper where I snaked the tube to meet his G-tube.  He had thought of a safeguard I had not.

The little boy may not know from were this fear sprung (http://www.health.harvard.edu/newsweek/Childrens_fears_and_anxieties.htm) or that the word “fireman” was not politically correct, but he understood the permanence of his nightly infusion.  Never a night passed without the ritual of connecting his gastrostomy to a long line of green tubing and his personal Folkman pump.

The next week my son and I had an appointment with the Fire Chief.  We told him where we lived, the basics about glycogen storage disease and the life sustaining nature of his night time infusion.  We took supplies to demonstrate how to disconnect and secure his G-tube.  Written details were given to this caring man in uniform who time proved would never need to  preform the duties.  In addition, the chief put our home on a registry that guaranteed if the city lost electricity our home would be a priority.  Brief, simple and important.

Lesson Learned: A child trusts you to keep him safe.