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Isabella Nicole Kurek
2008 & 2009
Isabella’s fourth birthday has
come and gone. In fact, another year and a half has
passed since our last update. In 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 three month span from July to
October of 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
drugs 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 of
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 become 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, a
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 homecare
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 hotdog 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.
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