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The differences were evident beginning January 12,
1999 when James was born. He was my second child. Everything went
well with the pregnancy, and I was healthy, so I never imagined
the journey that lay before us. James was jaundiced. No big
deal we thought, until we were sent straight to a special pediatric
hospital. My husband, Jim, and I walked the halls of Children's
Hospital of Philadelphia (CHOP) in disbelief. We met super intelligent
people, all who new about our 4-day-old son, and called him by
name, "Sweet Baby James." Test after test...hour after hour."Rule
outs" came from a long list of horrible could-be liver diseases.
James was diagnosed with biliary atresia, a condition
that is a result of the malformation of the ducts that carry bile
from the liver to the gall bladder. At the age of three weeks,
James underwent a surgery to "correct" his condition by connecting
a portion of his small intestine directly to his liver that, in
theory, would alleviate his "plumbing" issues. (The operation is
called a Kasai).
From February to June 1999, James received a lot
of medical attention. James got an NG tube for nutrition due
to failure to thrive, along with follow-up gastro visits for his
liver. We watched his yellow color and monitored his sucking. We
held his tiny arms for blood "sticks" and learned about hepatic
function, liver panel, and bilirubin. Progress was only adequate.
At the end of July 1999, James underwent a hernia
operation, and following, he lost the vast majority of his muscle
control. He couldn't lift his head. He couldn't suck his bottle.
He couldn't grasp his hands and feet. The medical staff was confused
because this hypotonia was not consistent with his "liver-related"
issues.
As a result of not bouncing back like he should from
the hernia repair, and with elevated liver numbers, the following
week, James underwent liver exploratory surgery. The result, the
doctors informed us, was that James' liver was severely scarred
and a transplant was imminent. Why hadn't the Kasai worked?
James had no reserve and after a second dose of morphine, he suffered
respiratory failure. The feeling of powerlessness overwhelmed
me as I watched the medical staff revive my son. There lay my
infant boy, limp in the nurse's arms and all I could do was pray.
I hoped that God was with him to give him the strength to stay with
us. I hoped that it WAS God's will. I hoped that James would survive.
James survived! Thank the Lord!
Our life of normalcy turned to that of hospital visits,
tests, learning medical terminology, and worry beyond compare. We
watched our son daily. We learned all his normal behaviors and could
sense any dangers. We became pros at placing tubes, drawing meds,
working machines. We became night owls...listening and distinguishing
the nighttime bells. Our lives had changed.
In October 1999, James was having a bath with his
sister, Gabrielle, then 3˝ years old. I took James out, changed
him, "hooked him up" to his feeding pump and placed him in his swing.
(He loved his swing). About 20 minutes later, I heard a cry that
seemed odd. I went to James, and grabbed him out of the swing. He
looked like he was choking, so I pulled the NG tube from his nose.
His breathing didn't seem right, and as I yelled for my husband,
Jim, James' lips turned purple. James stopped breathing! I laid
him on the bed and screamed out "Call 911." Jim gave the info
to the paramedics on the phone. I started giving mouth to mouth.
I panicked, but could remember, "Tilt the head, back. Give quick
short breaths." But this was my son, my baby. "My God, please don't
let him die," I thought.
After half a minute, James started crying, I knew
this was a good thing. The rescue squad arrived and took over
assessing James, and giving him oxygen. James was transported to
CHOP.
This was the first of many hospital transports over
the next two years. We became quite attuned to James' signals. We
knew when he was happy. We knew when he was hot (with fever) and
not so hot. And we knew when he had to go (to CHOP). And go quickly!
After many more tests, James was diagnosed with a
fairly rare metabolic condition. Initially it was thought to
be LCHAD, but now we know it is specifically, Tri-functional Protein
Deficiency (TFP). It is a Fatty Oxidation Disorder, and gone
undetected can cause liver, heart, retinal and muscular damage...even
death. Treated with glucose and special diets, the child can
live a healthy life. Many states are trying to expand Newborn
Screening tests. And many families are trying to spread the word
of awareness, since some pediatric doctors are not even knowledgeable
about these disorders. And some, although aware, are not familiar
with treatments.
James was placed on the liver transplant list as
a Status One. We stayed in the PICU (pediatric ICU) of CHOP
for eight weeks. We waited for James to come off the ventilator.
We waited for him to be fever-free. We waited and listened to all
the helicopters coming in the night. We waited for a liver! We prayed
it would come. We prayed that someone's misfortune would be our
son's blessing!
December 29, 1999. The day had come! Jim and I left
James' sleepy head to grab a bite in the hospital cafeteria. It
was about 12 noon when our nurse came to find us. "James will
be getting a liver. They've got one. It will be today. We've
got a lot to do. Yeah!!!"
Oh how the chills ran up our spines.and smiles to
our faces. Our miracle will happen. James will be fixed! He will
be well again. We raced to the room and everyone prepared James.
More blood tests, paperwork, and preparing meds. The nurses worked
like a well-tuned machine. Everything was in order.
It was about 11:00 pm when everything was getting
underway. The red cooler arrived. The delivery man said "God bless
you." The doctor said "...All looks good. Get rest. It will be a
long night." How could we sleep?! I hugged my baby and gave him
kisses. "Mommy loves you. Mommy loves you," I whispered. "You fight
in there. You be strong." The operating room door shut. And I latched
onto Jim and cried in his arms. Will I ever see my baby again? Will
he make it? What if there's a complication? What if...?
The waiting room was cold. We sat in the hard light
blue cushioned chairs for hours. Soda and snacks kept us awake.
The silence, kept us asleep.in our fears, in our hopes. We waited
for the updates. All was going well. And finally about 5:30 am,
the doctor came before us. James made it! The new liver was in!
"This is it. It's all over," I thought. He made it. He's a survivor!
I surely was wrong that it was over. That day
we held our baby's hand and must have sung, "You are my sunshine"
about 300,000 times. He looked miserable like he was beaten. He
was puffy. He was weak. He had tubes and more tubes. His bandages
covered his torso. But he had his new liver! Right?
Early evening arrived, and with the doctor's rounds
came the blow. "The graft is not functioning," he said. He
repeated it. What the hell does that mean? I couldn't believe what
I was hearing. I didn't understand. What it meant was that the liver
was not working. The liver somehow didn't survive! Maybe this and
maybe that...but what it meant was that James was dying. After
all the waiting.after the fight.after all those hours..."The graft
is not working," haunted my thoughts. I've got to be dreaming.
We weren't dreaming! Reality was all but too true.
James got placed back on the transplant list again ~ Priority in
the country. And we waited, but this time only for hours. By the
grace of God, a liver was born. James underwent his second transplant
less than 36 hrs from his first. This time it was not an exact
blood type match. Will it work? Will he live? Will he survive another
surgery?
James survived. And as we slept most of New
Year's Day, we silently thought Happy New Millennium!
One week later, James was in severe rejection,
and again underwent surgery. The surgeon needed to be sure the
issue wasn't clogged connections. They were fine. James' body just
was rejecting this foreign object. This foreign object that he needed
to live!
On Jan 12, James turned a year old in the hospital
bed of the PICU. We celebrated with balloons.with a cake.with
a sedated child.and with an unlit candle that brought many tears.
James was in pain. Our sweet Baby James lay silent because that
was all we could do for him. Make him sedate. And pray. We weren't
sure if James would make it. And if he did, did his brain suffer
damage? Would he be our James? The doctors and nurses were our
therapy. God was our pillar of strength.and definitely watching
us all.
James pulled through again. He was our survivor.
Yet, at the end of February, James got a virus, "It's EBV (Epstein-Barr
Virus).like mono. With tiders in the "millions," they said. The
quantitative number didn't mean much except that it was really bad.
"It will make him really weak. It's like a lymphoma sort of thing."
We were beyond crazy. The tears and horror filled my head for days.
I was frozen in time. I was still waiting for Thanksgiving. How
could this be? He was supposed to just need a new liver! Right?
What could be done? Something has got to be done! I wasn't ready
to lose my son. And James...he wasn't done fighting.
"We could always get another liver, but we can't get
another body," the surgeon said. And so we agreed to give up the
liver. All anti-rejection drugs were stopped. We prayed for healing.
We prayed his body would fight the virus and not the new liver.
We waited. We watched. We prayed some more.
During James' fight with "OKT3" a horrible drug that
compromised James' body, and breathing and life...my body ached
with pain. Mentally and physically I was exhausted...I was having
contractions...I was having another baby!
That's right, Samuel, our third child was born,
February 26, 2000. He lived in his stroller his first couple
of months of life. He reveled at the beeps and lights and hissing
of oxygen. He was a baby. He was also the recipient of a defective
gene. Samuel also had TFP! I would take turns holding each
of my boys.
Singing lullabies. Changing diapers. Placing NG tubes.
Wondering what was next! Wondering what was God's plan.wondering
if we would all come home.wondering and standing still in time.
Was it Spring? I had hardly noticed.
From February to June (of 2000), James (and Sam and
I) remained at CHOP with a few interim "welcome home" days. James
went through more bouts of rejection, horrible drugs, several viruses
and thousands of "procedures." Sam, on the other hand, was a
"healthy" baby thanks to James. He had been tested at birth
and treated with IV glucose as a precaution. He was in "preventative
mode rather than repair mode like James."
Finally the EBV titers went down. We were on our
way home. James was a survivor. James was a hero.
"The first year is the hardest," I remember everyone
saying. They did warn us, and they were right. In a year the most
consecutive time spent home was about 12 days! Imagine that. Two
weeks then a hospital stay. We learned the ins and outs. We were
regulars. We were a family with a chronically ill child. And
in spite of all the chaos, our family made it. We stayed strong.
We stayed secure and we stayed sane. (Well, almost.)
Then came February 2001. More hospital stays.
Samuel had a scheduled admission for his g-tube placement and James
followed him due to a routine cold. "He needs some IV glucose,"
we thought, "48 hour rule out!" God had another plan. James had
cardiac arrest. For 2 hours, we watched the team "treat" the
code blue. Hours went by as doctors pumped drugs into James. The
doctors prepared us that evening. "...We don't think Jimmy will
make it this admission." The words struck like daggers to my
ears. The shock and the panic consumed our hearts. "He just had
a cold," I remember shouting in tears. The familiar faces were all
a blur. Some strange metabolic phenomenon.unknown causes. Unanswered
questions. More worry and more prayers. And unbelievably, more miracles!
More chances from God! James survived again.
It is now August 2001. And life is changing again.
Our family has endured. And James continues to be a rock.
The medical regimen has become our life. Hospital visits have become
the norm. Tubes and pumps and therapy and oxygen...all just normal
routine. James works hard at getting strong. He has good head control
now and tries to bear weight on his arms. He can roll and he can
babble. He speaks with his eyes and his smile will brighten any
room. He has therapy daily and even takes swimming lessons. He is
very proud of his accomplishments. His road ahead is long, but
thanks to the miracle of organ donation he has a road to travel.
Thanks to genetic research he can be safe for now. He has a chance
for a good life.
And Sam has been saved by James. We are grateful
for that. Sam is developmentally fine, but also has a nutrition
(g-tube) regimen due to the lack of appetite, the inability to fast,
and the lack of experience in chewing. He's our crazy boy, and will
surely be James' best friend.
And Gabrielle. She is the angel in our story.
Our outstanding daughter, Gabrielle, now 6, who is really all...survivor,
hero and angel. She has fought through her brothers' hospitalizations.
She has fought through mom and dad whisking away almost weekly.
She has fought through holidays, birthdays, vacations and celebrations
in the hospital. She has learned about "Level 6" and how sick a
child can be. She has been versed in medical apparatus that even
some adults haven't understood. Yet she is a happy wonderful child.
She is secure and she loves both her brothers, although silently
wishes for more attention. She plays "Barbies," recites "MaryKate
and Ashley" and eats...not through a tube. She is mentally and physically
healthy. Sometimes she even teaches us, and calms our fears.
She is very deserving. She is an angel!
There is a story to be told of organ donation,
genetic tests and special needs families with FOD (Fatty Oxidation
Disorders). Not to mention family, trust, parenthood and relationship!
If I had the eloquent and thorough words of John Irving, I would
write a book, but for now, I seek your writers, your editors. Help
raise awareness. Help tell the story of Sweet Baby James...and
the survivor, the hero and the angel!
"There is memory almost foreign.
There is fear, now routine.
There is hope.
And there is peace in being home."
~ MaryAnn Raccosta ~
Sincerely,
MaryAnn RaccostaŠ8/8/2001
jraccosta@hotmail.com
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