Living in Holland...

I am going to do something a little different for this week.  My son, Jack, is very ill.  I am very stressed and very sleep deprived.  Therefore, I am going to let someone else write my blog entry for me.  A mother, whose child also has Noonan Syndrome, posted this on her Facebook page last week, and never have I been more moved.  Never have I so totally and completely related to something that someone else has written.  People often ask me how I cope with knowing that my kids can't grow or gain weight, that they see specialists frequently instead of just annual physicals, that they'll never go horseback riding or play football, or that they can't ever get over a stomach bug or a sinus infection without the assistance of a hospital stay.  I wish that I could share this with all of them.

While I loved this story, I wanted to make sure it was legit. I did my research, and I was thrilled with what I found.  The writer, Emily Perl Kingsley, has been a writer for Sesame Street since 1970.  Her son has Down Syndrome, and she played integral part in featuring children with disabilities on the show.  In doing so, she provided a voice for special needs children and their parents.

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WELCOME TO HOLLAND  by Emily Perl Kingsley

c1987 by Emily Perl Kingsley. All rights reserved

I am often asked to describe the experience of raising a child with a
disability - to try to help people who have not shared that unique experience to
understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip
- to Italy. You buy a bunch of guide books and make your wonderful plans. The
Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some
handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your
bags and off you go. Several hours later, the plane lands. The stewardess comes
in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm
supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and
there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting,
filthy place, full of pestilence, famine and disease. It's just a different
place.

So you must go out and buy new guide books. And you must learn a whole new
language. And you will meet a whole new group of people you would never have
met.

It's just a different place. It's slower-paced than Italy, less flashy than
Italy. But after you've been there for a while and you catch your breath, you
look around.... and you begin to notice that Holland has windmills....and
Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all
bragging about what a wonderful time they had there. And for the rest of your
life, you will say "Yes, that's where I was supposed to go. That's what I had
planned."

And the pain of that will never, ever, ever, ever go away... because the loss
of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy,
you may never be free to enjoy the very special, the very lovely things ...
about Holland.

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I have been living in Holland for quite awhile now.  It took awhile to get comfortable, but I am happy here.  It is quite beautiful.  And like Ms. Kingsley, I have met a group of people that I never would have met, from all across the world.  I have been involved in a network of other mothers, who have children like mine.  The support that we have provided to each other, and the understanding that we all share has been instrumental in learning to live the life that we now live.  Two of these mothers that I have become close to, have children in critical condition right now.  Two little girls are fighting for their lives, on opposite sides of the globe, connected only by a genetic condition.  Their mothers are among the bravest and most faithful of anyone who has ever spent time in Holland, and my heart aches for them. Holland is a much easier place to live when your children are there with you. There is no worse feeling in the world, like the moment you kiss your child goodbye and place him in the hands of his doctors.  The waiting and the uncertainty is pure agony.  But you do it with the hope that your child will come back to you.  It's that hope that gets you through, the hope of holding your child again. As a special request from your dedicated blogger, please say a prayer today that these little girls recover and get to come home to the people who love them. 

Abby and Bella, so many people are thinking of you and sending you strength right now... please get well soon.

Listen to Your Heart...

Today, I am devoting my blog to Congenital Heart Disease Awareness Week (February 7-14).  I am not going to bore you with statistics.  Instead, I am going to tell you a story in the form of a timeline.  I am going to tell you a specific chain of events that could have gone in an entirely different direction.  This is a true story.

February 21, 2008:  My son, Henry, is born, almost six weeks premature. He is sent to York Hospital NICU for underdeveloped lungs.

February 29, 2008:  Henry is discharged from the NICU.  The doctor on duty tells us that he has slight murmur, which is very common in preemies.  He tells us to follow up with our pediatrician.

March 3, 2008:  Henry's one week checkup at Pediatric Care of York.  Dr. Moore hears Henry's murmur and tells us that it doesn't sound bad, and is probably an innocent murmur.  However, PCY's policy is to never ignore a murmur, so he refers us to a Cardiologist at Hershey Medical Center and tells us not to worry.

April 15, 2008:  Hershey Medical Center performs an Echocardiogram on Henry, and determines that he has Pulmonic Valvular Stenosis (PVS), a congenital heart defect.  Devastated, I go home to Google "PVS," and read the words "Noonan Syndrome" for the first time.  I decide to ask PCY about it at the boys' next appointment in August.

October 25, 2008:  Henry and his brother Jack test positive for a mutation in the PTPN11 gene, confirming a Noonan Syndrome (NS) diagnosis.  PCY refers us to Johns Hopkins Genetics Clinic.

December 8, 2008:  Johns Hopkins Genetics examines Henry and Jack and gives us a list of specialists that they will need, as part of their treatment of NS.  One of the specialties is Hematology, as NS patients often have blood clotting disorders.

January 14, 2009:  Dr. Takemoto, Pediatric Hematologist at Johns Hopkins, discovers through blood work that Henry and Jack have Factor VIII deficiencies and Von Willebrand's Disease.  Bottom Line:  Their blood has difficulty clotting.

September 24, 2009:  Nineteen month old Henry wakes up from a nap with a diaper that has over-flowed with blood, leaking down his legs, and soaked into his pants.  (You may have heard this story.)  In a panic, I rush him to the ER of York Hospital.  Unable to find a source of the bleed and unable to stop it, the doctors at York call Dr. Takemoto.  Dr. T explains the severity of Henry's bleeding issues, and it is decided that Henry will be sent to Hopkins via ambulance.  By the time my husband and I arrive at Johns Hopkins, Henry's heart rate is at 214BPM.  We are told that because of the blood issues, if Henry doesn't receive clotting meds and a blood transfusion immediately, he will be in heart failure within an hour.  Four days after our arrival at Hopkins, following an admission into the PICU, several blood transfusions, and multiple tests, the source of the GI bleed is found.  Henry had been born with a Meckel's Diverticulum (MD), a small, rare growth outside of the stomach in the intestines.  It ruptured, causing an intestinal bleed.  A MD rupture is usually not life threatening... as long as the blood clots.  The amazing surgeons at Johns Hopkins successfully remove the MD, and save Henry's life.  Henry comes home a week after surgery, where he has been spoiled rotten ever since.



When Henry was first admitted to Hopkins, I received word that an acquaintance of mine, who was also a patient there, had just received life changing news.  Like my sons, she had been born with Congenital Heart Disease, only hers was very severe.  She had endured numerous surgeries, and had been on a waiting list, for a new heart, for years.  She received her new heart, from a donor, the same day that Henry's MD was removed.  It was nice to see the familiar faces of her family in the surgical waiting room, even though it was under such dire circumstances.  As I think back to all of us waiting, for our loved ones to pull through their surgeries, I thought of the one thing that had brought us all there.  We were united by the heart.

Henry's scar is on his stomach.  The disorder of his blood is what almost took his life.  But it was his heart, and that beautiful murmur of his, that caused his timeline to continue. 

The murmur led to the Cardiologist. 
The Cardiologist led me to the Internet.
The Internet led to Noonan Syndrome.
Noonan Syndrome led to Hematology.
Hematology saved Henry's life.

Henry was one of 2% of the population born with a Meckel's Diverticulum.  It was going to rupture eventually.  He was born with blood that doesn't clot.  If we had not known that, Henry would died twelve hours after the rupture, before they even would have had time to run the proper tests.  The doctors at Johns Hopkins saved my son, but I also give credit to Pediatric Care of York.  If they had treated Henry's murmur as innocent, and waited until symptoms were presented, he would not be here with me, today.

Cheesy love songs, jewelry commercials, and Hallmark cards often tell you to "Listen to Your Heart."  You have no idea how good that advice is.  In honor of Congenital Heart Disease Awareness Week, listen to your heart.  Listen to your child's heart.  If a doctor tells you he hears a murmur, insist on an Echocardiogram.  It may not be innocent, and it may lead to a discovery of a more serious underlying condition.  Football players go into Cardiac Arrest on the field at 15 years old, because of an undetected CHD.  Babies go into Congestive Heart Failure, before they've even seen a Cardiologist.  I dream of a time when all infants receive Echos before leaving the hospital, after birth.  It is too serious of a condition to ignore.  Promote Congenital Heart Disease Awareness.  Listen to your heart.  My son's saved his life.

Pajama Time...

Pad-pad-pad-pad-pad-pad-pad-pad-pad-pad-pad.

All parents are familiar with that sound.  It is a distinct sound.  It is a sound that warms my heart.  It is a sound that I am listening to, right now.

Pad-pad-pad-pad-pad-pad-pad-pad-pad-pad-pad.

Ahhhhh.... the sound of covered little feet, scurrying about in footed pajamas.  There's nothing like it.  The actual sound my vary from child to child, due to texture and size, but the feeling remains the same. 

I remember wearing footie pjs.  However, footed pajamas of the 1980s were basically made of steel wool and the plastic always seemed  to be hanging off of the bottoms of mine, providing no grip whatsoever.  So, I probably sounded more like, pad-flop-pad-flop-pad-flop-pad-flop-THUD, as I ran (and fell) throughout our house.  Fortunately, now that I have had children, the manufacturers of footie pajamas have started making them out of 100% Cotton.  They also created rubber-like gripies on the bottoms of the feet, instead of poorly gluing heavy plastic socks to the suits, as they did with the ones I used to wear.  Itchy or not, I still loved my footie pjs.  As my mom would zip me up, I felt like she was putting me into a superhero suit.  I'd tuck my boobah (my blanket), into the back of my neck to act as my cape, and I'd zoom down the hall to save the universe.  Good times.

I've longed to buy a pair for myself, now, as an adult.  I am pretty sure that if I pin myself down into a sports bra, I can squeeze into a size 14/16.  My husband always responds to this idea with a very stern, "Absolutely NOT," which I think is extremely unfair.  It's been pretty cold lately.  He may just find a surprise under the covers in a few weeks.

My love of footie pajamas has gone so far, I tend to wear them on my boys year round.  It's not uncommon in our house to go into their rooms on an August morning, to see a sleeping half naked child with a sweaty pair of pajamas crumpled on the floor, next to the bed.  Michael says that I'm going to suffocate them.  But I fail to see how something as lovely as a pair of footed pajamas would do any harm to a child.

I think that the appeal lies in the fact that they make a child look younger.  Footie pjs are something that they wore as babies, that they still wear as children.  They grow out of the bunting nightgowns, they refuse to wear cute hats, and before you know it, you pick them up and that familiar crinkle of a diaper under their bottoms is gone.  But when I zip them into a footed suit, and fold them into my arms, they become my snuggly soft babies all over again.

I once said that I wish I could bottle up the smell of my children after a bath, put it around my neck, and wear it for always.  It brings me such joy to get my boys out of the tub, slather them in Johnson's baby lotion, and dress them for bed.  The other night, after their bath, the boys padded down the hallway with their boobahs tucked into the back of their necks, off on yet another mission.  I wish I could bottle up their smell.  I wish I could freeze that moment to have with me all of the time.  The thought of them growing older, no longer believing that they are superheroes, and refusing to wear footed suits to bed, is devestating.  Hopefully, though, it will be a few more years before that happens.  For now, I am going to be sure to take advantage of every moment, while they are still my snuggly soft boys.

Our area was blanketed with ice last night, and the storm is to continue until tomorrow afternoon.  The National Weather Service has advised against all unneccesary travel.  The boys and I are going to cuddle up on the couch and watch movies all afternoon.  Oh, and I have decided that today is going to be a Pajama Day.  All Day.  I've got to enjoy this time while I can.

"Now all around the room in one big line, wearing our pajamas and looking so fine. It's Pajama Time!"  - Pajama Time, by Sandra Boynton