LAUSR

Over the last two decades treatment options have drastically improved for patients with pulmonary arterial hypertension (PAH). In the recent times, there is renewed interest in dry powder inhaler (DPI) based inhaled therapies in the treatment of PAH. PAH patients are well known to have respiratory and other muscle weakness either related to the disease itself or due to the underlying diseases like connective tissue disease (CTD). CTD PAH patients are at particular disadvantage as there is a concern if they have enough strength to press the buttons on the inhaler device, needed to pierce the drug capsule inside the device. Additionally, CTD PAH patients develop hand deformities making it difficult to use devices. To our knowledge, this is the first study to systematically examine the pinch force strength needed to pierce the capsule in DPI devices in patients with PAH. We enrolled 35 patients and our results showed that all PAH patients were able to generate enough pinch strength needed to pierce the capsule regardless of the etiology of PAH.