Dear Mr. Sparks,
In April of 1996, my husband, Gary, and I went to my obstetrician's
office for a routine ultrasound. We were so excited, I was 19 weeks
along and had just begun to feel Adrienne's life in my belly. I was not
sure that the ultrasound was necessary because my husband and I were
both young and healthy with no reason to believe that there would be any
problems. During the ultrasound we told the sonographer that we did not
want to know the sex of our baby. We wanted to be surprised when the
baby was born. The sonographer began to seem distracted during the
ultrasound and then abruptly dropped the doppler and excused herself
from the room. Gary and I thought briefly that something might be wrong
with the baby but agreed that it was more likely that the sonographer
had something going on in her personal life that was causing her to act
so strange. When the sonographer returned about 10 minutes later with my
obstetrician in tow, they both had tears in their eyes and struggled to
tell us that our baby had a very severe case of a rare birth defect
called diaphragmatic hernia, (left-sided). My first question was "Is our
baby going to die," the answer was a silent spillage of tears down
their faces. That began one of the most painful and surreal weeks of my
life (along with the weeks following her birth). We were sent to see a
neonatal specialist who performed an amniocentesis, (which was normal),
and advised Gary and I to terminate the pregnancy in the form of an
induction and live birth. The doctor told us to prepare to offer our
child a peaceful death in our arms rather than to be born full-term and
begin a struggle for life that she could not win. She told us there was a
good chance Adrienne would be stillborn if I did carry her to
full-term. If she did win the battle, she would live her life as a
vegetable. As we drove home after that day of hell, we realized that our
lives would never be the same; the loss of a child seemed unbearable. I
wasn't sure how I would survive the death of our child and her funeral.
It seemed so unfair to terminate a life without ever giving her a
chance, but how could I condemn her to a life as a vegetable for my own
selfish reasons? How could a doctor know that she would not live before
she was even born? As we prepared for the termination and funeral, we
found out the sex of the baby and named her Adrienne, it seemed to fit
her perfectly even though we had never met her. As we walked the site
next to my grandfather's grave where Adrienne was to be buried, the
child living so strong in my belly began to flip and kick using all her
might. It was a very intense message from Adrienne that terminating was
not the right thing to do. The next day we met Dennis Lund, MD at the
Children's Hospital in Boston who told us that the information we had
been given was very outdated and untrue. There was so much hope for CDH
babies, no matter how severe their case (Dr. Lund agreed, after seeing
the ultrasound pictures of Adrienne, that she had one of the most severe
cases he had seen). He gave his apologies for what we had been through
from the time of the first ultrasound to the time we saw him (which was
only a week, but it seemed like an eternity). He told me to keep myself
as healthy as I could in order to deliver Adrienne in the best shape
possible. He explained that we would need to be prepared to have
Adrienne taken from us at birth and she would need ECMO immediately,
then surgery and a long hospital stay involving many high-tech
procedures and machines to save her life. We were prepared and so
ready...anything to save our baby and offer her the happy life we had
dreamed of for her. We went to Boston for Level 2 ultrasounds every two
weeks for the duration of the pregnancy and were always encouraged to
hear that her heart continued to look good. During those ultrasounds we
saw her sucking her thumb, doing little dances and we even saw her
crying once. She had such a personality, even then.
On Saturday night, during a hurricane, at 1:36 a.m. on August 31, 1996
Adrienne was born after 30 hours of labor and a consequent emergency
C-section at Brigham and Women's Hospital in Boston. As Dr. Lund had
warned us, she was taken from my body and put on a respirator within
seconds and then rushed away. I got a chance to look at her as they
wheeled her past me and I said "Be strong, Baby Adrienne." She looked at
me with big, focused eyes and I knew that I had a very strong, willful
baby. The name Adrienne was perfect for her. We were told to get some
rest and we would be called in a few hours. In a few hours, we received
our first communication from Dr. Jay Wilson, CDH expert at BCH. He had
been with Adrienne for hours at that point and had put her on VA ECMO,
and within a couple of hours, VV ECMO. He was monitoring her closely, he
told us, and she was in good hands. It didn't take us long to realize
what an extraordinary man Jay Wilson was. He was compassionate and
caring and wasn't afraid to shed some tears with us as he explained how
rocky the roads were and that he would do everything he could do to save
our very ill daughter.
After 10 days on ECMO it came to an abrupt end following 3 days of
cycling with very little lung function. The circuit had a clot in it and
it needed to be either replaced or discontinued. Since this was
happening when we were en route to the hospital and were unreachable,
Dr. Wilson made the decision to let her try to "fly." When my mother
and I arrived at the hospital, we received the information from the
secretary at the front desk that Adrienne was off ECMO. We weren't sure
what that meant. Within a few minutes of our arrival, Dr. Wilson emerged
from Adrienne's cubicle and told us he was nervous, but changing the
circuit and putting her back on ECMO would not be beneficial to her
since she was nearing the end of the safety zone on ECMO. Much to
everyone's surprise, she made it on a hi-fi oscillating ventilator!!
After a week of hi-fi she began a spell with pneumothoraxes that nearly
claimed her life, one right after the other for 3 days. Dr. Wilson told
us to be prepared that this could be the end, but he would continue to
pull from what he called his "bag of tricks." Some of the things he
tried on Adrienne in the next couple of weeks were not approved by the
government and considered very experimental, but Dr. Wilson told us that
they were our only hope and we trusted him fully. He is the most
intelligent, compassionate doctor we have ever known and he gave her his
life for a good four weeks until she began to stabilize. His wife must
be equally as compassionate and caring. At two months old, after a week
of failed attempts to get off the conventional ventilator, she made it!!
It was during the big flood in New England of October 1996 and I cried
as I watched the taxis floating down the streets in Boston on the news
as I sat in my home in Kennebunk, Maine. After all that time, I couldn't
get to her on the day she was extubated. The nurses kept in close
contact with me that day and told me how well she was doing on oxygen.
During the two months Adrienne spent at the Children's Hospital, my
mother and I commuted daily to Boston during the week and on the
weekends my husband and I made the commute together.
On November 3, Dr. Wilson told me that Adrienne was stable enough to be
transferred to his associates in Portland, Maine at Maine Medical Center
so that she could be closer to home. She still had some severe fluid
issues and required daily Lasix, she was having a difficult time weaning
from Morphine and was having withdrawals no matter how slow the wean
went. She had survived some blood infections secondary to the central
line but was doing pretty well. Dr. Wilson told us that the next issue
before Adrienne could go home was feeding. After 2 months of pumping and
throwing away the majority of my breast milk, I wanted to nurse her. I
wanted to offer her something normal and motherly. When we got to
Portland on November 4, I did not bond as well with her doctor there as I
had with Dr. Wilson - who could ever live up to Dr. Wilson? We argued
about what to feed Adrienne and how to feed her. I wanted her to have
breast milk, he thought formula would be better. I wanted to try to
nurse her, he thought that tube feedings were much safer and could
monitor her fluid intake better. Eventually I nursed her, but only once.
She latched on immediately and looked so peaceful but it came up as
fast as it went down, only much more painfully. Her severe case of
reflux had been showing its ugly face for a few weeks at that point. She
continued to have breast milk through her various types of feeding
tubes until she was nine months old, at which point, I had had it with
pumping. As the two months crept past that she spent at Maine Medical
Center, her doctor (Dr. Browne) and I came to respect each other even
though we didn't always agree. After placement of a jejunostomy tube
during Thanksgiving week of 1996 and the rehabilitation from that
surgery, Adrienne came home on December 20, 1996. What a Christmas we
had... The j-tube was placed in order to keep Adrienne nourished through
the winter, Dr. Browne did not think she was strong enough to have the
nissen fundoplication to correct her reflux at that point and thought we
should wait until spring. Dr. Browne did not want us to feed Adrienne
orally through the winter, for fear of aspiration. Through the winter,
our home was kept as sterile as possible and people were screened
carefully at the front door for any colds. Adrienne remained oxygen
dependent around the clock until after her fundoplication in April 1997,
at which point she only needed oxygen to sleep. The fundoplication was
done at the same time the g-tube was placed. A minor surgery was
performed shortly after that to remove the j-tube and the central line, a
surgery which I was able to participate in.
Adrienne stopped breathing on the way home from my parent's house one
night because her cannulas had disconnected from the oxygen tank. I will
never forget her labored, slow breaths as I looked at her purple body
and begged her to breathe while waiting for the ambulance to arrive. My
husband was not with us during this episode but rounded the dark corner
on his way home to find our front lawn covered with ambulances and
flashing emergency lights. The winter she came home was racked by scary
blood infections (from the central line), constant reflux of the bile
from Adrienne's empty stomach (the food was going directly into the
intestines via the j-tube), and complete oxygen dependence. It was not a
restful winter, to say the least. Since then, Adrienne has survived a
significant case of external hydrocephalus which we thought was going to
need to be shunted, dumping syndrome/hypoglycemia, a painful retching
disorder with frequent bouts each day, the inability to eat by mouth due
to dysphasia and lack of skills, recurrent ulcers from the g-tube,
monthly IV placement and respigam treatments, SVC syndrome with
subsequent heart surgery and hardware placement and a small hole in her
heart (which healed itself when she was 3). Every couple of years until
she is full grown the hardware used to keep her SVC vein open will need
to be enlarged. When she is full grown she will need a full
reconstructive surgery on her SVC vein. She graduated from oxygen at 26
months old!!
Over the years we had in-home PT, OT and speech several times a week in
order to help Adrienne catch up developmentally. When Adrienne was 3,
we were fortunate enough to find a warm and loving private duty nurse in
our hometown to be a part of our lives, after searching unsuccessfully
for 18 months to find a nurse we felt comfortable with. Linda is a
wonderful mother to her own three children and she is a very caring and
compassionate nurse. Until we found Linda, Gary, my Mom and I were the
only ones who took care of Adrienne. Having Linda in our lives has
allowed me to take on some responsibilities outside of our home knowing
that Adrienne is in good hands, and it has provided Adrienne with a new
extended family at Linda's house. Linda has accompanied Adrienne to
preschool for 2 years and will continue going to school with Adrienne to
provide medical support when Adrienne enters public kindergarten in the
fall of 2001. We LOVE Linda!
Through it all, Adrienne has been lucky enough to grow into a normal
child with a few lasting medical issues. Most people are amazed to find
out that she has medical problems and was unable to eat by mouth until
she was 3 ½ years old. We feel so lucky to have found Dr. Wilson. At a
CDH clinic visit in 1999, Adrienne put her "G-G" (that's what she named
her blankey) in Dr. Wilson's lap and laid her head on her G-G. It was
touching to watch as he stroked her curly hair and smiled at her. It was
a perfect picture of the compassionate and caring doctor he is and the
trust Adrienne has in him. I have always felt that he loves her and
cares about her life very much.
Sincerely,
Adrienne's mom, Jenn Dumas (Maine)
