The “Polar Bear” on Nobscot Hill

“Be Prepared.”    BSA Scout Motto

One Friday afternoon in January the thermometer was dipping below ten degrees Fahrenheit and Scott was loading his pack with long underwear, fire starters and matches.  He was going on his first Polar Bear campout!   I am half convinced the reason boys like to camp and endure the elements is so that they can play with fire.  Excuse me…build fires.

We did not have proper camping equipment for such a night, but his brother Greg was too young to go so Scott took his sleeping bag and planned to sleep with one bag inside of the other.   There were Coleman lanterns, camp stoves, cooking equipment, etc.  Water was packed in a thermos for the middle of the night cornstarch ritual.

On Saturday afternoon the campers returned.  When they came into the house they smelled of sweet wood smoke and looked a shade darker then when they left.  The brothers and I were eager to hear the stories.  Yes, it was bone-chilling, their feet were cold, but they took turns keeping the fire going all night.  The unanticipated hitch was that, “The lid to the thermos with my water for my cornstarch was frozen shut in the middle of the night.  Dad had to breath on it to get it to thaw.”  Wow!  Awesome.

Lesson Learned: You can’t think of everything.

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Can You Bake a Berry Pie?

“It is simple, come with me.”  The Simple Prince  by Jane Yolen

The smell of a fresh New England blueberry pie permeated our family’s campsite one hot afternoon, tempered by the evergreens that surrounded us.  Out of place, honestly.  Our family prides itself in being unusual in all sorts of ways.  Glycogen Storage disease just adds dimension.

How does one decide to bake a pie in the woods anyway?  Well, it all started with a hike which I chose not to take.  Three boys and their father came running back into camp with the bottoms of their pint sized shirts filled with hundreds of tiny blueberries not much larger than tapioca pearls which is typical of wild blueberries in the Northwest region of the United States.  “Do we have enough!”  The gauntlet had been thrown down!  My kids had no doubt in their mind that I could accomplish this request.  Their concern was if they had picked enough. “It sure looks like it,” was my response.

The boys were eager to get started. They knew from experience that in the woods this would require teamwork.   We needed a fire.  Not just any fire.  We needed a hardwood fire that would burn slow and even for 45 minutes after coals were formed.  Their dad was the master of fire and they were his disciples.  For the next 30 minutes their was wood gathering, reflector oven designing, wind checks and so on.

I always camp with the basics (flour, butter and sugar) and pies require nothing more. The crust was rolled out with a tall tin cup on a picnic table covered with a red and white checked vinyl cloth. The lack of humidity in the air was perfect for pie crust. The kids cornstarch is my thickener of choice for blueberries. I just wished I had known back then that corn syrup should have been my sweetener of choice for kids with GSD. The crust bottom was placed in a round cake pan I had packed for making French toast and filled it with tiny sweet blueberries tossed in cornstarch.  After the top crust was sealed and crimped  I carefully cut vents in the shape of three wheat shafts replete with kernels, the mark of my mother and grandmother.

The pie was placed on a flat rock at the edge of the fire next to the reflector oven made from sticks and aluminum foil.  It was carefully rotated over the next 45 minutes to ensure even baking. It was perfectly imperfect!   Only one side got burned , a little.  “Mom, this is so cool,” commented at least one of the boys.

Living with GSD can be unrelenting.  There is an air of freedom in the woods. Preparing a family to go camping, especially one with special medical needs, is much work, but worth every minute.   Can you bake a berry pie in the woods?  It is simple, come with me.

Lesson Learned: Being successful just outside our comfort zone translates to competence, creativity and confidence in the possibilities of other areas of one’s life.

Camping in October

“We choose what we choose.  I can smell the Jasmine in the hills.”  The old wise man sitting on the Night Train to Kathmandu

The leaves crunched under our feet as we walked the trails in the state forest.  Who knew honey colored Timberland work boots were manufactured to fit such small feet. Four of them stepping over stones, roots and branches.  Scott had a brother now. There was the occasional trip and fall and right back up again. The out of doors makes one hearty.   There is nothing like the vista as a reward for climbing a peak no matter how small.  This was the first camping trip I remember attempting with a child who required continuous overnight feeding.  I can’t wait to tell you hear how much fun we had.

The tent was missing a stake, but before long a substitute was created from a stick with a jack knife.  A woven rug outside the tent removed the woods from our feet.   A burette hung from the center pole and the clear tubing extending from a rotary pump on the canvas floor across the small sleeping bag where Scott would be sleeping right next to me.  I am sure this sounds familiar to some of you.  An orange extension cord ran from the car to the tent to supply electricity to the pump that would be feeding Scott all night.  A tiny electric flashlight with the shape of a Coleman lantern dimly lit the cosy space.

After the excitement from the hike up to the lookout, eating from mess kits and enjoying a  campfire, there was much giggling and wiggling as I helped my sons to bed.   It must have been 9pm before the troops were settled.  Prayers were said, the pump was turned on, I sang a few children’s songs and all was well in Zion.  I thought.

About midnight I awoke to the smell of stomach acid.  Great!  A leaking connection or a gastrostomy pulled out.  Did I bring the stylette?  The details are foggy like my head at the time, but supper half digested was evident by the cold slimy feel as I ran my hand under Scott’s belly in his sleeping bag.  I saw that the level in the burette was higher than when I went to bed.  Oh brother!  I was pumping this little boy’s stomach.  I quickly re-taped the connections, opened the flow of glucose to replace what I had taken, checked Scott’s breathing,  and placed Scotty on a towel and cleaned up the best I could.  After checking the direction of the tubing through the pump literally 10 times, I tried to get comfortable again, but the smell and the guilt kept my mind doing gymnastics.  I was so happy to see the glow on the tent walls that marked the sun rising to rescue me from this hell.

After the boys’ morning pee (all of them including dad) on a tree not far from the tent, the boys dad prepared the fire and I cooked breakfast. Ok, I admit it.  There is nothing like the smell of bacon on a campfire.  Yum.  Eggs…not so much.

It was time to wash up.  I tied the special toiletries apron made from dish towels around each boys’ waist.  The bottom third was folded up and sewn to create “just right sized” pockets for toothbrushes, paste, soap etc. Nothing had to be put down on dirty surfaces in the latrines.  Woods dirt and bathroom dirt are two different kinds of dirt in my book.   We started our short walk to the cement block wash house.

Exhausted from no sleep, the acrid smell of stomach acid lingering in my nose, feeling like I have a layer of sweat and dust between my underwear and my skin, thinking about the work to clean everything up when I got home were clouding my vision.   I attempted to sound enthusiastic about the beautiful new day, the sun shining through the trees and the smell of pine needles.  We exhaled with our lips pursed so we could see a translucent stream of condensation flow from our mouths.  Science lessons abound in the wild.

As we approached the low impact color cement block wash house, bugs lay dead on the ground, others hung from the spider webs near the yellow bug light outside the entrance, and the slow creak of the screen door to the latrine echoed through the air like a scene from the Adams family.  I remember it as clear as if it was yesterday.  The kids steps were two to my one and Scott exclaimed with glee, “Mom, we are having so much fun! ”  I threw my head back and laughed out loud!

Lesson Learned: Children view their world with fresh eyes and curiosity.

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.  http://www.uic.edu/classes/bms/bms655/lesson5.html   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.

Lead Kindly Light

“Lead kindly light amid encircling gloom; Lead thou me on!  The night is dark, and I am far from home;Lead thou me on!  Keep thou my feet; I do not ask to see the distant scene.  One step enough for me.”   -John Henry Newman

I had an operator interrupt a phone conversation my husband was having to tell him our daughter was not out of the woods like the doctor had promised.  That she was lost forever.  We were nearly 1000 miles apart.  No embraces, just tension over questions to which I had no answers.  No, it had nothing to do with GSD.  I don’t know if they gave her enough glucose.  Was she comfortable? I didn’t know.  I had not been allowed to stand by her side like before.

I called Dr. Crigler to tell him the news. After a pause he said it was the saddest news he had every heard.  Last week from a wheel chair, recovering from a broken hip, he recalled that day and remarked as he respectfully bowed his head, “That was tragic.”

The autopsy confirmed it was Legionnaire’s disease (http://www.cdc.gov/legionella/patient_facts.htm).  Not sure where it came from.  It doesn’t matter now.  Natalie was dead.  October twenty-fourth would never be the same on my calendar.

Natalie’s Dad arrived by plane; the funeral was planned; we bought plot 257 in Section N at the cemetery.   That is real estate we never wanted to own.   Three papers across the country carried the news; Los Angeles Times, Marlborough Enterprise and the Midland Daily News.

My sister-in-law meticulously ironed the white smocked Polly Flinders gown Natalie had worn the day she was blessed and given a name.  She wore it again as she lay in her small white casket tucked in with a tiny pink and white pieced quilt LInda and her friend had worked on far into the night.  I had no idea how important that quilt would be over the years.  It comforts me to know it is draped over her chest as she lies beneath the winer snow.

The wake was surreal.  I felt like an actor doing and saying the things I was supposed to say.  Young mothers didn’t want to come through the line. I was called over to accept their condolences.   Most of my friends were still in college so their parents came to pay their respects.  I couldn’t fathom how so many people got the word.

My Father and brothers were the Pall bears to a box so small it looked like I could have carried it myself.  A child 1 year and 24 days old doesn’t require much to return to God.  The man who blessed her to be well gave the sermon and my friend Linda gave the eulogy.

The station wagon that brought us to Michigan drove us home in silence.  I remember nothing about that trip.  Life goes on.  Trite but true.  God bless of everyone.

Lesson Learned: You can do what you must…one step in front of the last.

The Rest of the Story

“Now you know….the rest of the story.”    Dave Berry, Columnist

Natalie was not taken by GSD. She died of Legionnaire’s Disease; a tragic encounter with a gram negative bacteria.

I could have started my story here and left out the sad beginnings, but then you wouldn’t know the whole story.  As my close friends have read this blog, some of them were not aware of the details of this brief but significant season that colored my life.

The posts that follow tell the story of managing difficult situations in a disease that is relentless yet very livable.  The posts will no longer be linear, but written in topics that create branches of a tree;  oddly analogous to how scientists define glycogen which is stored in the liver in tree like structures.

With the risk of being abstract, if you take away a bit of wisdom from these stories it would be like cleaving a glucose molecule from a strand of glycogen.   In reading further I am hoping my stories will give you the “glucose” you need to move forward…. to grow as individuals in the process of helping your children along their journey.

Dr. Weinstein et. al. are making GSD a manageable disease with a cure on the horizon. It is thrilling to watch this long awaited miracle unfold.