Nursing, Bottles & Diapers, Oh My!

“Every new beginning comes from some other beginnings end.”   -Roman philosopher, mid-1st century.


Riding home from the hospital, I got that familiar feeling in my gut.  That feeling of fear and awe, that I was responsible for the survival, nurturing and education of this tiny being who we named Scott.  The only difference was that this “tiny being” would “never” grow out of his need to eat every few hours.

(The word “never” is a strong word that seemed accurate 34 years ago.  Just this year a company in the United Kingdom patented a product called Glycosade that is allowing people with this disease to sleep much longer than was previously possible.)

After nursing for a few months Dr. Crigler suggested it was time to switch to a formula laced with glucose.  In 1978 glycogen storage disease was still being treated with continuous glucose during the night and very frequent feedings during the day.  Frequent meant every one and a half hours with this little guy.   If he didn’t take in some form of glucose his familiar sweet, inquisitive spirit would wilt and droplets of perspiration would form on his nose and forehead.   If I waited too long his elfin hands would tremble as if he had palsy. Children with glycogen storage disease Ia cannot raise their own blood sugar efficiently.

Before he was one year old Dr. Judah Folkman, the Surgeon in Chief at Boston Children’s Hospital, cut into Scott’s small abdomen to perform a gastrostomy.  Now we had the capability of infusing glucose into him all night by way of a pump, just like his sister Natalie.  Now he could sleep without being awaken.

Probably out of necessity he learned to say words early.  He would come to me and say, “Mama, ‘feetsies’,” which he has since explained meant that his feet were going numb or tingling; one of the signs of severe hypoglycemia.  His favorite foods were chicken and rice, which turned out to be perfect for him.

I became more organized. Eight bottles of formula with added glucose would be lined up on the bottom shelf of the refrigerator door everyday; all different colors.   When he could walk I allowed him to take his own bottles from the refrigerator.

Occasionally he would not finish a bottle and the next one would start early. I tried to keep track of which bottle he took at which time.  Sometimes I found half drank bottles in the toy box or hidden in his stuffed animals. These were his first steps towards autonomy.

Rainbow after the Storm

“The soul would have no rainbows, had the eyes no tears.”  John Vance Cheney

Darryl got a new job, I was pregnant and we became the proud owners of a stately little brick cape on a hill in an old section of town.  The anticipation of having a baby was clouded only vaguely by the possibility they would have GSD.  Glycogen Storage disease is autosomal recessive.   Every one of my pregnancies would have a 1 in 4 chance that the baby would be born with the disorder.

I worked for a temp agency, received good prenatal care and spent hours preparing our house for the new beginning of our family.   The pregnancy was uneventful, except for the baby squirming inside me when he heard his father’s voice.

We lived though the blizzard of 1978 that encapsulated Boston for a week.  As spring melted and summer came into full bloom, so did I.  One pleasantly warm morning in August I put the finishing touches on our baby’s crib.  After closing the lid on the paint can with a few taps of the hammer I stood at the counter to prepare a peanut butter sandwich when my water broke.  No lunch for me.  The time had arrived to welcome our new little person.

My husband was out of the office and there were no cells phone, so I called my 62 year old neighbor who was a father of five.  Without a question he whisked me off to the hospital 30 minutes away.  I walked into the hospital feeling strong and ready for the next chapter of my life.  Sometimes it is good to be 22.

Seven hours later the pediatrician was present in the delivery room as well as a team of doctors and nurses.  I wasn’t sure how they thought they could help.   My delivery was considered high risk because of a previous sibling with glycogen storage disease.  An IV maintained my blood sugar so baby would not begin his life with hypoglycemia…just in case.

Our son was born and laid on my belly.  I can still remember the warm weight of his body on my body.  Scott was born with a normal blood sugar that dropped within a few hours.  His tiny heels became blue from lancets sticks.   His desire to survive was strong.  He nursed like a trooper.

The attending physician, residents and my pediatrician all had opinions about how this infant should be nourished. I knew that lactose in milk should be restricted, but I also new there needed to be balance.  I wasn’t going to nurse him forever.  I wanted to nurse my little one for all sorts of reasons.  I felt like I had failed Natalie and wanted this baby to have a stronger immune system. I wanted some control over what was happening…again.

The rocking chair cradled my sore body as my new little son with GSD hung onto my breast, sucking life into himself. Doctors had to move their discussion out of the nursery.   I am guessing they didn’t want me to hear how divergent their opinions were about what was or was not good for my son.   Dr. Crigler told me I could decide, so I did.

Lesson Learned:  Even when a group of people have the same information, they can disagree on how to move forward.