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.