As told by Claire' s mom....

Our daughter Claire is a fun-loving and silly three year old who believes she is a princess.  She loves playing dollhouse, making up magical fantasies, and building castles out of blocks.  She also enjoys driving her hot pink power wheelchair through the park and the back yard.  Claire has Spinal Muscular Atrophy, and although her mind is unaffected her muscles are quite weak.
During Claire’s first year of life we were unaware that anything was wrong.  We recognized that she was frequently congested, struggled to gain weight despite normal feeding patterns, and that she sweated heavily in her sleep.  However, she was meeting her developmental milestones on time so we assumed that her other issues were not concerning.  Sadly though, when Claire stopped crawling and pulling up on furniture we were no longer able to deny that there were more significant challenges facing her.
In the course of searching for a diagnosis for Claire, she endured a variety of tests and procedures.  One procedure was an MRI in which Claire had to be sedated.  Despite her frequent congestion, failure to thrive, and sleep sweats, it had never occurred to us that Claire had respiratory issues.  However, during the MRI Claire struggled to maintain her oxygen saturation and her respiratory rate and heart rate were irregular.  The physician attending the procedure suggested a sleep study might be beneficial.
Soon after Claire’s MRI, we finally were given a definitive diagnosis of Spinal Muscular Atrophy Type II.  As sleep disorders are common with this disease, our neurologist agreed to order a sleep study for Claire.  Her sleep study revealed that because her respiratory muscles were not strong enough to take deep breaths, Claire had to breathe quite rapidly in order to maintain her oxygen levels.  The rapid breathing also caused her to build up excessive carbon dioxide which triggered her brain to have Claire stop breathing momentarily (sleep apnea) to balance out the carbon dioxide.  Claire was struggling so much to breathe while sleeping that sleep was essentially exercise for her and was the main cause for her sweating during sleep and poor weight gain.  For this reason, Claire’s pediatric sleep specialist, Dr. Dominic Gault, recommended that Claire begin using BIPAP when sleeping.
Claire was 22 months old when we began acclimating her to her BIPAP mask.  She initially liked wearing the mask and pretending to be an elephant but was terrified when we hooked the mask up to the BIPAP machine and began blowing air through it.  We tried games such as having everyone in the family take a turn putting the blowing mask onto your face, placing the mask on stuffed animals, and feeling the air blowing on your hand.  Although Claire loved putting the mask on Minnie Mouse, she was absolutely not going to allow us to place it on her!  Eventually Dr. Gault’s office had us bring in Claire for a mini trial of placing the mask on Claire’s face and then turning on the airflow.  Although she resisted at first, she did calm slightly, and we were sent home with a schedule that gradually increased the amount of time Claire was to wear the mask each night.  It took about a month of wearing the mask in bed before Claire finally learned to fall asleep with her BIPAP. 
Claire has been successfully using BIPAP for just under two years, and we feel strongly that it was well worth the frustration and hard work in getting her to accept it.  Her regular use of BIPAP allows her body to know what a deep breath should feel like so that she will fight for deep breaths when off of BIPAP.  Also, the airflow keeps her lungs flexible and open and her congestion has disappeared.  Since she’s not working so hard to breathe during sleep, her heart rate is slower, she’s not burning excessive calories, she’s no longer sweaty when she wakes, and she is able to grow and gain weight.  As parents we’re also very grateful that our daughter wakes up with energy to play and live life.  Treating Claire’s sleep apnea through BIPAP does not cure her Spinal Muscular Atrophy, but it does greatly improve her quality of life and overall health.