Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR)gene which lead to impaired chloride (Cl-) movement across the plasma membrane. Individuals with CF typicallyexcrete sweat with substantially higher Cl- and sodium (Na+) concentrations than healthy individuals. CF can leadto multi-organ manifestations, either primarily or secondarily (i.e. bronchiectasis, diabetes mellitus, osteoporosis,malnutrition, depression). Exercise is recommended as adjunctive treatment for many of these manifestationsand exercise capacity has been shown to be an independent factor in CF survival. With the recent approval ofhighly effective CFTR modulators (HEMT) for 90% of CF patients, it is likely that survival for CF patients willincrease substantially and it is anticipated that many patients will survive into their sixties. The efficacy of HEMThas led to some unintended consequences such as that some patients have become obese (BMI>30) and manyhave become less adherent to their daily airway clearance regimen and rely on exercise for airway clearance.These factors underscore the importance that regular vigorous exercise will play in ongoing care of CF patients,which is now expected to last many more decades. Since excessive water and electrolyte losses can contributeto dehydration and electrolyte imbalance, CF patients are at high risk for severe dehydration and electrolyte losswhich can have deleterious consequences and limit intensity of exercise. There is substantial data that hasdescribed sweat rates and electrolyte loss in response to exercise in normal athletes, however, there is limiteddata for CF patients. We recently reported a novel skin-interfaced wearable microfluidic device and smartphoneimage processing platform that enables analysis of regional sweating rate and sweat chloride loss in athletesand CF pediatric patients in a mode that is comfortable and imperceptible to the wearer. We hypothesize thatcontinuous electrolyte and sweat rate measurements captured with our soft wearable microfluidic sensors andsmartphone application (called the CF Patch System) will provide actionable hydration and electrolyte repletioninformation for adult CF patients during exercise. The CF Patch wearable platform offers a groundbreakingapproach for the quantitative and continuous assessment of CF hydration and health conditions. Thefoundational insights from this work will enable new care management paradigms for CF patients in remotesettings.
Public Health Relevance Statement: Project narrative:
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR)
gene which lead to impaired chloride (Cl-) movement across the plasma membrane. Individuals with CF typically
excrete sweat with substantially higher Cl- and sodium (Na+) concentrations than healthy individuals. There is
substantial data that has described sweat rates and electrolyte loss in response to exercise in normal athletes,
however, there is limited data for CF patients. The present proposal addresses this unmet need by deploying
novel wearable sweat sensors that provide quantitative and actionable hydration and electrolyte repletion
measurements for adult CF patients taking CF therapies. The foundational insights from this work will enable
new care management paradigms for CF patients in remote settings.
Project Terms: <21+ years old> |
