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Copyright © 2013 Isabella Nicole Kurek Memorial Foundation, Inc.
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By Joanna and Harlan...
I was thrilled when I first discovered I was pregnant with my second child. We had been trying for over a year. I even started
seeing a fertility specialist and had begun the first round of testing. This pregnancy was a little different from my first one. I
felt pretty sick for the first four months; the nausea and vomiting just wouldn't stop. The baby, however, seemed healthy
and there were no concerns. Everything was normal on my first ultrasound and we were pretty sure it was going to be a girl.
I was so excited!

In early December, around my seventh month of pregnancy, I started to experience some changes. My body started
swelling. My ankles became so swollen that I couldn't even wear my shoes. I also experienced awful headaches and
nausea, but I saw my OB/GYN regularly and there were no obvious problems.

On December 25th we had Christmas dinner in our home. The entire family joined us and we had a wonderful time. I
started experiencing my first contractions early in the day. At first I thought it was just a false alarm; I wasn't due until the
end of January. As the contractions became more frequent, I called the hospital and they told me to come right down. Since
I was already in my 35th week, they wouldn't try to stop the labor.

I arrived at the Emergency Room around 10 pm and was admitted right away. My blood pressure was very high (175/150).
The monitors showed very mild contractions, but upon exam, I was already 8 cm dilated. The doctor told me I was suffering
from toxemia (pre-eclampsia) and they were going to have to induce me. The resulting contractions were awful, but after
only three of them, I was ready to push. An episiotomy allowed things to move along faster. And then she came… my
beautiful, baby girl. 5 lbs, 1 oz, 18 inches long; the tiniest and most precious thing I had ever seen. We named her Isabella
Nicole. She was perfect!

We had to stay in the delivery ward for observation for the next 24 hours. My blood pressure was still very high and they
were worried that I may seizure. We were finally released on Sunday, December 28th, 2003. I was still on medication and
suffering from periods of dizziness, but I couldn't have been happier to be home. Isabella’s big sister was so proud and
completely adored her right from the start.

When Isabella was one month old, she contracted RSV. We avoided hospitalization but had to treat her at home with
Xopenex in a nebulizer every 4-6 hours. Her cough started to improve almost immediately. At her two month check-up, the
pediatrician noticed some laxity in her hips and we were referred to an orthopedist. I was worried she may need to wear
supportive braces, but everything turned out to be normal.

One day I was changing Isabella’s diaper and noticed a huge bulge in her left groin area. I immediately saw the pediatrician
and was referred to a pediatric surgeon for an inguinal hernia repair. The day of surgery was awful! I was so scared that
she wouldn't make it through the procedure. It was the longest hour of my life. Little did I know at that time. If I only knew
what was going to happen next…

At her four month check-up, I mentioned that she seemed to be breathing very hard and that she wasn't rolling over or
holding her head up yet. The pediatrician recommended we start physical therapy, but I wasn't ready. I thought she was
just weak from the surgery and that if we gave her enough time, she would catch up on her own.

Two months passed by… still no rolling over. I also noticed she wasn't moving her legs much and thought there had
possibly been some nerve damage as a result of her surgery. I was really starting to become concerned about her. When
we saw the pediatrician at her six month check-up, he referred us to a neurologist. As soon as we saw the neurologist, it
was obvious he knew what the problem was. He kept asking me questions, which at the time, I thought were pretty weird.
The following day blood was drawn and tested for muscle enzymes and SMA. I tried to get some answers from the
neurologist about the disease, its progression and prognosis. He avoided answering all of my questions and just kept
telling me that we shouldn't jump ahead of ourselves and wait for the results. He also told me that for the follow-up exam
and review of the test results, I should come with my husband.

Well, I couldn't just go home and wait. It was going to be three weeks before the results came back. That day I came home,
got on the Internet, and discovered the most horrifying thing. I looked up SMA and the first sentence I read was
“…number
one killer of infants and toddlers under the age of two…”
 I was shocked. I broke down into tears and grabbed my baby girl
and hugged her so tight that she started to cry. I couldn't believe it!  This was my Baby Girl!!! The only person that was
there for me when I needed it the most; she would listen to what I was going through without judging me. She was the
perfect example of unconditional love.

I printed out some articles and waited for my husband to come home. All the symptoms matched… tongue fasciculations,
lack of head control, no rolling, hypotonia, etc. As soon as I told my husband about the disease, he was in denial. I also told
my sister and my best friend. They were both very supportive. I decided to keep it from the rest of my family until the results
came back. Those three weeks seemed to take forever. She seemed to be doing fine. We started physical therapy and it
seemed to be helping. Whatever she couldn't do physically, she would make up for with her beautiful smile. That smile was
always there, regardless of what was going on around her.  

On August 19th, 2004, we had a 5 pm appointment with the neurologist to review the test results. My husband and I didn't
talk much prior to the consultation; he was angry with me for even considering the possibility that Isabella may have the
disease. My sister came with us to the appointment to offer support. We were all sitting in the office as the neurologist
confirmed the diagnosis. I was trying to be strong. I didn't want my baby to know how weak and helpless I felt at that
moment. She most likely had Type I SMA, but it would require a muscle biopsy to confirm Type I, vs. Type II or III.  I refused
to put her through those additional tests.

We had to break the news to my parents and the rest of our family. On the way back from Isabella’s appointment, we
stopped at my parents’ house to pick up our older daughter. The whole family was there and we gave them the news.
There was silence and a lot of crying. I decided to write a letter to our family and friends explaining Isabella’s diagnosis. It
was very difficult. I asked everyone to respect any decisions we should make regarding her care, and to be supportive in
any way they felt comfortable. I also included an article describing the disease and her prognosis. I noticed that people
started avoiding contact with us because they didn't know what to say or do. I couldn't blame them. Some just sent cards
with their wishes or prayers. Everyone was shocked.

I scheduled an appointment with a pulmonologist to address her difficulty breathing. Her first x-ray showed a mucus plug in
her right lung, but it wasn't a concern at that point. We arranged for a pulse-oximeter and a nebulizer to be delivered to our
home. She was started on Xopenex and Pulmicort to keep her lungs healthy and to keep her breathing easily.

Next came a visit to the gastroenterologist. Isabella had already been treated for acid reflux with Zantac, but the doctor
wanted to try a course of Prevacid before doing anything else. Three weeks passed by without improvement. She wasn't
eating as well as she used to and she had completely stopped eating at night. The next step was to admit her to the
hospital to run some tests. In order to confirm that she actually had reflux, a nasogastric pH probe was placed. This
measured the pH in her esophagus over a 24 hour period. She did, in fact, suffer from reflux. The next day she had an
upper GI barium study to rule out damage to the esophagus. Thankfully, the results were normal. She was discharged with
a nasogastric feeding tube in place. We were only to use it to supplement her night feedings. A pump and formula were
delivered to the house that day. Things seemed to be fine.

The following day, Isabella developed a fever.  We saw her pediatrician immediately and he was concerned by the sound of
her chest.  We were sent for x-rays which confirmed aspiration pneumonia of her right lung. She started antibiotics and we
continued the nasogastric tube feedings. Swallowing became progressively more difficult for her and she eventually
stopped taking anything by mouth. Her difficulty swallowing made it necessary for us to suction her saliva, so she wouldn't
choke. We carried a bulb syringe with us everywhere we went. Within a few days, the pneumonia and fever began to
resolve.

During this time, however, she began vomiting more frequently.  Initially, it was only once a day, but soon she began
vomiting after every feeding, especially at night.  We went back to the gastroenterologist and he referred us to a surgeon
for placement of a gastric feeding tube and a fundoplication (NISSEN procedure), which would limit further reflux and
vomiting, hopefully preventing additional aspiration pneumonia.

Isabella suffered her first real respiratory crisis one morning, shortly before her surgery was scheduled to take place. Her
mouth filled with saliva; her face turned very pale and her lips and tongue were blue.  Her oxygen saturation levels dropped
into the 70’s. I suctioned as fast as I could and tried to talk to her as calmly as possible. It seemed like it lasted forever, but
she soon recovered. We rushed to the pulmonologist and follow-up x-rays showed worsening of her pneumonia; half of her
right lung field was infected.  Her antibiotics continued and we arranged to have an oxygen tank and concentrator delivered
to our home. There were no additional crises prior to her surgery date and we felt that she was actually improving.

The NISSEN procedure and placement of the g-tube was scheduled for October 29th, 2004 at 9 am. Isabella was examined
by the anesthesiologist upon admission and there were concerns about her lingering pneumonia. The surgeon and the
anesthesiologist agreed that the procedure should be postponed and that Isabella should be admitted to the hospital for
treatment.  After an hour of waiting, we were finally moved to a room in the pediatric ward.  Isabella’s condition seemed to
be deteriorating by the minute.  Her oxygen saturation dropped into the low 70’s and she was put on supplemental oxygen.  
Hours went by and we still had not seen a doctor.  Isabella had not eaten anything since the night before, she was
dehydrated and hungry.  I thought she wasn't getting enough attention and I was starting to get angry.  After chasing down
the nurses, an IV was finally established, but without doctor’s orders, fluids couldn't be administered.  All the while, Isabella
became weaker and more exhausted.  Apparently, she was too sick for surgery, but not sick enough to receive attention
once admitted to the hospital.  I couldn't sit by and watch this any longer.  An emotional break-down on my part finally
prompted some overdue attention.  Nurses and doctors swarmed in and initiated IV fluids and antibiotics.  X-rays showed
her pneumonia had worsened; it was a good thing the surgery had been postponed.  Later that afternoon, we began
feeding her (partial parenteral nutrition, PPN) through her IV. The pulmonology team was consulted and she was started
back on her nebulizer medications and chest physical therapy.  Isabella finally began to settle down and seemed
comfortable for the first time that day, but I still hadn't seen a smile since we left the house.  I just hoped that the night
would go smoothly and that she would be happier tomorrow.

That night was awful.  I have never in the past 10 months seen her so miserable.  Since having the nasogastric tube
removed that day, she wasn't taking anything orally.  She was receiving PPN through her IV line, but it didn't seem to be
satiating her hunger or thirst.  Isabella didn't sleep more than 30 minutes the entire night.  She desperately wanted to drink,
but would start choking and coughing if given more than a couple of drops of water at a time.  We spent the entire night
rocking her, giving her water from a syringe… drop by drop.  She constantly needed oral suctioning because of her
difficulty swallowing.  Her fever persisted despite regular doses of Tylenol and Motrin.  She was exhausted, but wasn't able
to sleep for more than a couple of minutes at a time, before waking up and crying.

We spent the next three days in the pediatric ward, with what I thought was too little attention and not enough aggressive
care.  Multiple IV catheters were placed, and ultimately failed, before a central line was established.  Thankfully, this was
accomplished under mild sedation and a local anesthetic.  Isabella slept through the entire procedure.  The central line
allowed us to upgrade her PPN to TPN (total parenteral nutrition), optimizing her nutritionally.  Later that day, her heart rate
began to elevate (210-220 BPM) and her respiratory rate increased to 55-60 breaths per minute.  Her oxygen saturation
fell into the high 60’s and she was transferred immediately to the pediatric ICU (PICU), where the attention to her care
dramatically increased.

I was impressed with the PICU team and very relieved that she would be cared for and observed more closely in this
environment.  She saw more doctors and nurses in her first 10 minutes in PICU than she did throughout her first three days
in the hospital.  I finally felt that she was getting the attention she deserved.  Initial x-rays in PICU showed a worsening of
her pneumonia and a new area of collapse in her right lung.  Her antibiotics were changed and she was placed on positive
pressure ventilation.  After just a few hours, Isabella seemed to be improving dramatically.

After over two weeks in the hospital with agonizingly slow improvement on her x-rays, occasional oxygen desaturation into
the low 80’s, and lots of deep suctioning, I was about to lose my mind.  However, I knew I needed to stay strong and not
show Isabella any signs of sadness or anger.  Still no surgery date scheduled and lots of confusion.  I began to have mixed
feelings about all of this.  Most of the days she would smile and make noises; she seemed to be really happy and
comfortable, although, during her occasional times of crisis, she seemed absolutely miserable.  The overwhelming feeling
was one of a loss of control.  All of the specialists were looking out for their own best interests, often conflicting with each
other.  The surgery team wouldn't operate until her lungs were healed enough to optimize her recovery, but the longer she
went without surgery, the more likely she was to relapse, further postponing any surgery date.  It seemed like a vicious
cycle, and we were stuck in the middle.  The doctors’ time-frame seemed to be measured in weeks to months, while mine
was measured in days.  We just wanted to get her home and start the healing.  I can’t even describe the frustration we felt.  

On Sunday, November 14th, I decided to take a break from the hospital and spend some time back at the house while my
sister stayed with Isabella. She was fine all morning; smiling and happy as can be. I received a phone call from the nurse
only a few hours after leaving the hospital.  Isabella’s blood oxygen was desaturating and she needed to be deep suctioned
numerous times. Thirty minutes later the attending doctor called to tell me she had failed to improve. She was still having
trouble breathing and was failing to maintain her saturation. He suggested intubation would be the best way to stabilize her.
I tried to stay calm and gave them permission to do anything necessary to help her. However, I hoped they would be able to
wait the hour it would take me to get back to the hospital. I just wanted to see her one more time before she was sedated
and intubated.  Luckily, things seemed to have calmed down quite a bit by the time I arrived back at the PICU. Isabella had
recovered and the intubation was averted.

After much debate between ourselves, the respiratory team, and the surgeons, Isabella’s surgery was finally scheduled for
November 17th.  Her lungs had healed as much as they were going to, and we all feared another set back might take place
if we continued to wait.  We made the difficult decision to perform a permanent tracheostomy at the same time as her
gastric surgery.  Considering the progression of her respiratory compromise since entering the hospital, we knew it was
only a matter of time until the tracheostomy would become necessary. We also knew that because of her pneumonia, they
might have trouble extubating her after surgery, making a tracheostomy necessary regardless. Everyone agreed that
accomplishing both procedures under a single anesthetic event would be in her best interest, so the decision was made.

The day before surgery, we experienced one of our most frustrating confrontations with the nursing staff.  Isabella had
been receiving Tylenol and Benadryl on a nightly basis in order to help her sleep.  For some reason, the night before
surgery, her nurse decided Isabella had been on these medications for too long.  It seemed that the “as needed” note
failed to make it to that day’s Doctor’s Orders.  The battle to keep her comfortable had begun.  The next morning during
rounds, I had to fight to continue her Tylenol.  Isabella had a difficult time with her morning chest PT and seemed
uncomfortable.  Her heart rate and respiratory rate became elevated and she couldn't fall asleep.  She would close her
eyes for only a moment before waking up crying and moaning.  She was finally dosed with the Tylenol after three hours of
my pleading with the staff.  I was very angry and frustrated.  Because of miscommunication between the doctors and
nurses, Isabella had to suffer.  I just wanted her to be comfortable and I wanted to see that beautiful smile one more time
before she was taken into surgery.

Her surgery was scheduled for 1:30 pm, but she wasn't transferred to the OR until 2:30.  It was very hard to see her go.  I
gave her a big hug, a kiss, and told her I loved her.  Now all I could do was wait.  The surgeon came out to update us as
soon the NISSEN procedure was completed and the gastric tube was placed.  Isabella did incredibly well under anesthesia
and her surgeries were uncomplicated.  We had to wait a short time for the EENT surgeon to perform the tracheostomy, but
within an hour, Isabella was in recovery and doing well.  I was so glad it was over.  By the time we made it back up to our
PICU room, Isabella had already arrived.  This was the first time in two weeks that I could see and touch her beautiful face,
free of all the feeding and respiratory tubes.  Isabella had begun to open her eyes and look around, but she was still very
disoriented.  She had a wonderful night postoperatively.  There were no episodes of desaturation and her pain was well
controlled.  She was still quite sedated and slept well.

The next week in PICU passed quickly.  48 hours postoperatively we began feedings through her gastric tube, initially
Pedialyte followed by a true enteral formula.  She tolerated the feedings well and her stomach was emptying normally.  We
began to see the first signs of her regaining some of the weight she had lost.  Her first trach tube change occurred 5 days
after surgery and her ventilator status was downgraded from BiPAP to CPAP and her oxygen supplementation was reduced
to that of room air.  At this point, Isabella was transferred from ICU to the respiratory ward, which we viewed as a huge step
in the battle to be discharged.  Unfortunately, we soon learned the frustrations were only beginning.

Isabella spent over two weeks in the respiratory ward while we tried to convince her doctors we were capable of taking care
of her at home.  Her condition remained stable and unchanged throughout the rest of her stay in the hospital, but they
wouldn't discharge her until we fulfilled an exhaustive check-list of tasks.  Before this experience, I naively thought decisions
about her care would be made by me, with the guidance of her doctors.  I soon realized that once admitting her to the
hospital, all control was forfeited to the doctors and nurses.  I thought I could politely decline the doctors’ suggestions that
we obtain in-home nursing care. I thought I could politely decline their recommendations that she spend an additional
month in ICU while I was “trained” to use the medical equipment I had already been using for months.  I was surprised to
learn I had no legal rights to simply “sign her out against doctor’s orders.”    We were prisoners in the hospital until they
saw fit to release us.

We were told we could not be released until we secured nursing care at home, whether I wanted it or not.  Obviously, I was
unable to judge my own ability to care for my daughter.  Additionally, it wasn't good enough that I had mastered the tasks
involved in her daily care (ventilator maintenance, trach changes, suctioning, etc.), I needed to have three other “potential
caregivers” pass all the tests, just to be safe.

Initially upon transfer to the respiratory ward, I was told to expect a two month learning period before Isabella could be
discharged.  We fought exhaustively to have her sent home after two weeks.  By then, I think they were all happy to see us
go.  Thankfully, Isabella remained happy and stable throughout the process.  I don’t know what to expect with our transition
back home, or what the future will hold, but I do know we are ready for the challenge.  Isabella continues to smile and play,
which encourages us all to cherish each day as we forge on ahead.

…….. If you have a child, please give them a kiss and let them know that you love them.

Continues at Part 2