Copyright © 2012 Isabella Nicole Kurek Memorial Foundation
All rights reserved. Questions, comments or broken links, please contact
Copyright © 2013 Isabella Nicole Kurek Memorial Foundation, Inc.
All rights reserved. Questions, comments or broken links, please contact
By Joanna and Harlan...
general, Isabella’s condition has notably deteriorated.  She has suffered multiple broken bones, had severely debilitating
bouts of diarrhea due to her medications, and has experienced episodes of complete respiratory arrest.  She is only able to
tolerate sitting for very short periods of time, which has dramatically limited the use of her power wheelchair and her ability
to participate in school.  Isabella’s strength has deteriorated and she is visibly so much weaker and seems so fragile.  Our
struggles with the insurance companies and nursing care providers are the only things that have, unfortunately, remained
stable.  As Isabella has deteriorated, the battles we choose to fight have shifted.  Many of the frustrations mentioned earlier
in her story seem rather irrelevant now, given our new concerns about her condition.  The last year and a half has provided
many frightening moments, but has also given us some wonderful experiences we will always remember.

As mentioned before, shortly before Isabella’s fourth birthday, she suffered her first broken bone. Her leg healed well and
the cast was removed. Afraid of an underlying orthopedic disease, we performed a bone-density study, but the results were
inconclusive. Ten months passed without any further episodes. Then, in a 3 month span from July to October 2008, she
suffered five consecutive fractures. First, she broke her right femur above the knee; again, with no history of trauma. While
this leg was casted, she broke her right arm, just below the shoulder. This fracture was the only one we could identify the
cause. It happened  while changing her into her pajamas. The location of the fracture in her arm didn't allow for casting and
it was allowed to heal without specific treatment. Four weeks later, while her right leg was still casted and her arm was
healing, she broke her right ankle, just below her cast. Her cast was lengthened to incorporate her foot and things
continued to heal. Three weeks later, Isabella re-fractured her left femur, in the same location as a year earlier. One week
later, she re-fractured her right femur and had full casts on both legs.

At this point, we knew we needed to address her weak bones, if at all possible. We were referred to a pediatric
endocrinologist at Yale, Dr. Carpenter. He repeated the bone-density study and confirmed osteoporosis, secondary to
disuse atrophy. No muscles pulling on the bones leave them very weak. He offered two different medications which could be
helpful to restore bone density and strength, although neither offered predictable results. There wasn't much experience
using either medication in children; they are more commonly used to treat osteoporosis in women. One of the choices was
an injectable drug, Pamidronate, which required an IV and a day admitted to the hospital. Given our past experiences with
hospitalization, we ruled out this option. The other drug, Fosamax, is given orally (or via a g-tube) each week and can be
done at home.

We chose to go with the Fosamax but we weren't able to continue it for long enough to see an effect.  Despite varying
dosages, Isabella developed chronic, severe diarrhea, dehydration and lethargy while on Fosamax and we had to
eventually give up.  Because we were unable to complete the treatment we never repeated her bone-density study.  But
interestingly, she has not suffered another fracture over the last eight months.  Maybe it helped, but more likely, her
decrease in activity is the main contributing factor.  She is moved to and from her wheelchair much less often, offering less
chance of an accidental bump on the leg… one of the few benefits of her increasingly sedentary lifestyle.

Just when we finally gave up on the Fosamax and got her chronic diarrhea under control, Isabella developed a C.diff
infection that lead to weeks of more diarrhea.  It took three courses of different antibiotics, probiotics, and many trips to the
gastroenterologist before we were able to stop it.  It was very discouraging to see her so weak and dehydrated.  You could
tell she felt horrible; she was so quiet and lethargic.  We spent most of the winter of 2008 treating her.  Part of Isabella’s
ongoing treatment for her tracheitis infections is a low dose of daily antibiotic.  This did wonders for her tracheitis, but
probably contributed to her C. diff infection and we have since had to stop the treatment.  Since making the change, her
bowels have normalized but her tracheitis has become much worse.  It is a frustrating reality that we can’t treat one problem
without making another worse.  It is a daily tightrope walk that is getting more and more difficult.

Isabella’s Public School career has been another up and down process and serves as an example of how our priorities
have shifted.  Because travel to and from school is so difficult in the winter months, she continued to be home-schooled by
her wonderful special ed teacher, Ms. Gina, until March 2008. At that point, she returned to her regular pre-school program
and finished out the school year with success. She graduated with the rest of her classmates in an adorable little ceremony.
For kindergarten we decided we wanted her in a mainstream classroom as much as possible.  We never realized how
difficult it was going to be to get her the support and attention we felt she deserved. The school district is obligated to
provide each special-needs student with everything they require to obtain an education, but they sure seem reluctant to
follow through. It seems like we were involved in countless, frustrating meetings with the district to coordinate her program.
We had to negotiate everything: school bus pick-up and drop-off times, the number of hours she could tolerate in class,
rest times and locations, air-conditioning in the classroom, private changing area, staff training and provision of assistive
technology devices, nursing care, her curriculum, special ed, speech and PT just to name a few. The truth is, because of
Isabella’s “fragile” condition, the school was scared to have her there and they did everything they could to drag their feet
when it came to providing for her. This became even more obvious when she had her first major crisis while at school. She
loved being in class and interacting with her friends and we were fighting so hard to maximize the amount of time they would
let her be at school… then it all came crashing to a halt.

On October 21, 2008, Isabella stopped breathing.  She was at school with her personal nurse, began to stress, and
snowballed into full respiratory arrest.  It was one of the most terrifying experiences of my life.  That single episode brought
home the reality of her condition like never before.  It showed us how fine the line is between life and death.  For some
reason, we always thought “the end” would come as the result of some long-fought battle with pneumonia, or some similar
slow deterioration.  We always thought we would see it coming.  This episode caught us all by surprise and forced us to
acknowledge the possibility that she could be fine one moment and we could lose her the next.  In the weeks leading up to
this, the school was already starting to show some trepidation handling Isabella.  She would be sent to the nurse’s office
and we would receive a call every time she began to show the first signs of stress, or became flushed.  These were
everyday experiences for us at home, and we knew she could be easily settled back down with a little suctioning and
attention.  The school wanted us to leave work and come pick her up every time this happened.  We could easily see they
were uncomfortable dealing with her and would rather just have her stay at home and not be their responsibility.  The call I
received while at work on October 21st seemed like just another annoying, false alarm.  The school nurse called to tell me
Isabella was starting to stress and they felt like we should come pick her up.  She was due to get on the bus to come home
very soon and I was trying to determine if this was a real crisis when I heard in the background “we are going to call 911.”  
Then they hung up on me.  My heart fell to the floor and I immediately left work and drove the very short distance to her
school.  I arrived before the ambulance and found Isabella in the nurse’s office.  My Baby was pale, limp, and a horrible
shade of blue.  She was completely nonresponsive and in full respiratory arrest.  I was shocked to see she was still in her
wheelchair.  Her personal nurse was paralyzed with fear and did nothing to help her.  I immediately moved her from her
chair and laid her on the nurse’s bed and began deep suctioning her and shook and started chest PT.  She slowly started
to regain consciousness and her color started to improve.  Shortly after, the ambulance arrived and I rode with her to the
emergency room.  By the time we arrived, Isabella was back to normal, just a little sleepy.  After a couple hours of
observation, the doctors consulted with our pediatrician and they elected to let us decide when we felt comfortable leaving.  
We took the opportunity and brought her straight home.  That evening, our dog, Ivy, stayed by her bedside and wouldn't
move, even to go outside.  She knew something was wrong and seemed to be protecting her.  Isabella continued school for
another week.  We received phone calls every day from the nurse’s office telling us she was starting to stress.  I finally
couldn't take it anymore and we took her out of school.  I was terrified every time the phone rang that it would be another
911 call and, honestly, our confidence in her nursing care had vanished.  We knew she wasn't safe at school any longer.

In the nine months since October, she has suffered three more episodes of respiratory arrest.  Luckily I was home each
time and was able to revive her in a similar fashion.  None of the episodes required a trip back to the hospital.  What has
become frighteningly apparent is how easily she can fall into these episodes.  The worst one was early one morning.  I had
just checked on her and went for a shower.  Within a few minutes all her alarms began screaming and I jumped out of the
shower to find her in complete arrest.  She was once again pale, limp, and blue.  My husband and I were able to revive her
with suctioning, stimulation, and oxygen through the ambu bag.  We discovered later that she had dropped a toy she was
playing with and couldn't reach it.  This made her cry and she quickly snowballed into arrest.  Her condition has become so
fragile and tenuous.  It is frightening to think this can happen to her at any moment.

One of the factors that contributed to Isabella’s crisis at school was her inconsistent nursing care.  Nurses come and go.  
Some of them have been excellent and we trust them like members of our family.  Some of them have been asked to leave
before the end of their first day.  Most fall somewhere in between.  It seems like as soon as we get comfortable with a really
great nurse, something happens and they can’t continue.  We have lost nurses to other companies; we have lost them to
maternity leave, and one even moved out of state.  There always seems to be, however, a long line of less-than-mediocre
nurses ready to take their place.  We have had nurses that were afraid to handle and move Isabella, nurses that were
surprised they were expected to bathe her in the morning, surprised they were supposed to read to her and play with her.  
We have also had nurses that thought it was ok to sit outside reading a book while Isabella was left alone in her room.  
Some have been so completely cold and clinical it makes you wonder why they even work in pediatrics.  Through the last
four years I feel like we have seen it all.  Isabella’s nurse the day of her crisis at school was one of those that was afraid to
handle her, for fear she may break.  This kept her from removing Isabella from her wheelchair when the crisis began.  The
simple act of laying her down may have prevented her arrest.  The nurse left the case shortly thereafter.

The nursing care companies have also been difficult to work with on many occasions.  Often it seems like they are
unmotivated to supply us with nurses.  It can take weeks to find a replacement when one nurse leaves.  I have lost many
days of work because of this frustrating process.  On two occasions, two different nursing companies have failed to
properly invoice our insurance companies and we have been left holding the bill.  One company sent us to collections for
$5,600.  We ran out of insurance coverage towards the end of the year and the company never informed us.  They just
kept sending the nurses and it wasn't until months later that they handed us the bill.  We were paying more for a day of
nursing than I made for a day at work.  Had we known, I would have just stayed home.  In a different year, another nursing
care company handled the same lapse in insurance coverage a little differently, but with the same disastrous result.  We
have two insurance companies and combined, our benefits still don’t allow for nursing coverage the entire year.  The
second nursing company failed to bill our secondary insurance when the benefits from the primary insurance company
became exhausted.  They said they did this on purpose because they knew we would run out of days later in the year.  So
instead, once again, we got the bill.  We have since stopped using both companies, but unfortunately, that leaves us with
only one other nursing company in our area.  If we end up on bad terms with them, we will have burned our last bridge.

I get tired complaining about our insurance companies, but I just can’t stop.  As mentioned before, the nursing hours they
provide are less than we need to get through the year.  We don’t need a lot of coverage.  We only use the nurses four
days a week for eight hours; the time I am at work.  We never have nurses in the evenings, weekends, or holidays.  Even
so, we can’t make it through the year.  I can’t begin to imagine how we would cope if we required 24hr coverage.  The most
ridiculous thing is that the insurance companies would be willing to pay to have Isabella hospitalized, rather than pay for
home nursing.  The cost comparison is staggering, but it all depends on the break-down of the benefits.  We know families
that can’t take their children out of the hospital because their insurance doesn't allow for nursing care.  The sheer stupidity
of this policy is amazing.  For the last four years our insurance companies have been trying to reduce our coverage by
reinterpreting their definition of the words “home nursing care” and “shift.”    It seems as though “skilled nursing care” is
quite different from “home nursing care” and whichever one we happen to have just isn't the right one.  Then there is the
ambiguous “shift” to consider.  When the Child Advocate scolds the insurance company for trying to deny our nursing days,
they counter by changing their definition of a nursing “shift.”  Shifts started out as 12hrs, easily allowing for our little 8hr
day.  Then it was discovered that shifts are really only supposed to last 8hrs; still ok, but better make sure we get home
from work on time, or we will be charged for a second day.  Then the insurance companies realized a shift is actually just
4hrs; eight hours would be just too tough on those poor nurses.  Can you imagine the stress and fatigue required to care
for a severely handicapped child for longer than four hours without a break?  Now it seems that our little 8hr day requires
TWO days of nursing benefits.  Each year we go through the exact same process with the insurance companies and the
Child Advocate.  Each year the insurance companies relent and say, “Ok, we will cover you for this year, but NEXT year,
you are on your own.”  Then we do it all again.

On a positive note, our home care provider, Apria, has softened and is waiving the cost of renting Isabella’s ventilators
(seeing how our insurance doesn't cover such fancy equipment).  I take back all the terrible things I said about Apria in the
previous installments of Isabella’s story.  Unfortunately, however, we are back to relying on two ventilators.  The Miracle
Ventilator we found at the NEAT Market turned out to be a lemon.  It worked very nicely for about a year before suffering a
circuit board failure.  We found a very nice repair service that was willing to work with us as private owners (something
nobody apparently does), but despite their best efforts, it never functioned properly again.  Thankfully, Apria came through
for us and Isabella still has the protection of a second, back-up ventilator on hand.

We continue to try to keep active with Isabella, even though it has become more difficult to travel with her.  We were able to
bring her to visit our family in Maine again last summer.  She went whale watching in Bar Harbor for the second time and
was still scared of the whales.  She would close her eyes and turn her head away whenever the guide pointed one out.  We
also spent time exploring Moose Point State Park and kayaking in Lake Saint George (Isabella skipped that part).  Most of
our time was spent in the woods, at the quiet home of my husband’s parents.  While there, Isabella had the opportunity to
experience her first real campfire and hot dog roast.  Good thing she is still not using oxygen.

Our biggest adventure of the year was a winter trip to Florida.  This would be Isabella’s second time to Orlando and her first
experience driving there.  With all of her medical equipment and the cost of flying the entire family, we decided it would be
easiest to drive with Isabella and to fly the other family members.  The planning and coordination involved was
monumental.  We had three adults and four children, including Isabella, that were making the trip.  My husband and I
stuffed the minivan full with Isabella, our son, Reilly, all of the medical equipment and supplies, and as much luggage as we
could carry and drove 23 hours to Orlando.  We stopped once for the night and arrived intact early the next day.  My sister,
Anna, and our two daughters, Natalie and Emma, arrived by plane that evening.  We had a wonderful week visiting the
parks all day and playing in the swimming pool all night.  At the end of the first week, Anna, Reilly and Emma flew back
home and Isabella’s father flew down to spend a few days with her and Natalie.  My husband and I then drove home with
Isabella while Natalie stayed the rest of the second week in Orlando with her father.  At the end of the week, the two of them
flew home together.  All things considered, Isabella handled the trip like a champ.  The drive was a little tough, but dividing
it into two days made it tolerable.  The weather was beautiful while we were there and we all shared a vacation that will
always be remembered.  On a sad note, a couple of days before our trip ended, we learned that our dog, Ivy, passed away
while boarding back at home.  She died during emergency surgery to treat gastric dilation and volvulus (GDV/bloat).  We
are all going to miss her.

We have also continued to visit our local aquarium and zoo as often as we can.  Isabella has attended many family parties
this year as it seems everyone is getting married or having babies.  When we are not able to get away, we try, at least, to
get Isabella outside and into the fresh air and sunshine.  As time goes by, she is becoming less and less tolerant of these
things.  She is having more difficulty sitting in her wheelchair or stroller for extended periods of time without becoming
uncomfortable.  Often, as soon as she gets outside, she asks to go back to her room.  Her recent episodes of respiratory
arrest make it particularly stressful to travel.  We always bring her emergency supplies with us, but it is frightening to think
what could happen.  We have another trip to Maine planned for this summer and we are hoping she will still be able to
enjoy it.  Sadly, we know there will come a time when this becomes impossible.  Until then, we will treasure the experiences
and build memories that can never be taken away.

Isabella continues to see her pulmonologist, Dr. Egan and gastroenterologist, Dr. Glassman regularly.  We are continuously
tweaking her nebulized antibiotics to combat her chronic tracheitis.  She was forced to discontinue the low-dose oral (g-
tube) antibiotic which was working so well, due to the development of her C. diff infection.  Now we are limited to the
nebulized antibiotics and are having a more difficult time keeping the infections controlled.  Her latest therapy consists of
two weeks on and two weeks off of nebulized tobramycin.  Time will tell.  We continue to see the gastroenterologist to help
determine the amount of formula Isabella needs each day as she continues to grow.  He also helped us battle the C. diff
infection which finally ended her nearly two months of diarrhea.

As Isabella’s condition has changed, so has the priority of her individual problems.  Her lungs have become our biggest
concern.  Honestly, I suppose we always knew they would.  In the process, we have had to sacrifice some of our earlier
battles.  She is no longer able to comfortably wear her thoracic brace and her scoliosis has markedly progressed.  Her
osteoporosis makes physical therapy too dangerous and her range of motion has suffered.  We no longer see the
orthopedist or the endocrinologist.  As mentioned before, her trouble at school and our frustrations with the school district
have forced us to keep her at home.  So many of the things we fought for no longer seem relevant.  The day-to-day grind
and general uncertainty have taken their toll.  Not a day goes by that I don’t wake up wondering if today will be the day; the
day she has a crisis she can’t recover from, the day I lose her.  I try not to focus on it.  I know I have a family that needs me,
a life beyond the reach of SMA, but at times it is so hard to remember.  The lack of control is frustrating and something with
which we continue to struggle.  We all just try to enjoy the good days and find the strength to endure the bad.  We will have
to wait to see what tomorrow brings.

Continues at Part 5