Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare, fatal, segmental premature aging syndrome caused by a mutation in LMNA that produces the farnesylated aberrant lamin A protein, progerin. This multisystem disorder causes failure to thrive and accelerated atherosclerosis leading to early death. Farnesyltransferase inhibitors have ameliorated disease phenotypes in preclinical studies. Twenty-five patients with HGPS received the farnesyltransferase inhibitor lonafarnib for a minimum of 2 y. Primary outcome success was predefined as a 50% increase over pretherapy in estimated annual rate of weight gain, or change from pretherapy weight loss to statistically significant on-study weight gain. Nine patients experienced a ≥50% increase, six experienced a ≥50% decrease, and 10 remained stable with respect to rate of weight gain. Secondary outcomes included decreases in arterial pulse wave velocity and carotid artery echodensity and increases in skeletal rigidity and sensorineural hearing within patient subgroups. All patients improved in one or more of these outcomes. Results from this clinical treatment trial for children with HGPS provide preliminary evidence that lonafarnib may improve vascular stiffness, bone structure, and audiological status.

In this paper, we present the design of a thumb exoskeleton for pediatric at-home rehabilitation. Pediatric disorders, such as cerebral palsy (CP) and stroke, can result in thumb in palm deformity greatly limiting hand function. This not only limits children's ability to perform activities of daily living but also limits important motor skill development. Specifically, the device, dubbed IOTA (Isolated Orthosis for Thumb Actuation) is a 2-DOF thumb exoskeleton that can actuate the carpometacarpal (CMC) and metacarpalphalangeal (MCP) joints through ranges of motion required for activities of daily living. The device consists of a lightweight hand-mounted mechanism that can be custom secured and aligned to the wearer. The mechanism is actuated via flexible cables that connect to a portable control box. Embedded encoders and bend sensors monitor the two degrees of freedom of the thumb and flexion/extension of the wrist. Using this platform, a number of control modes can be implemented that will enable the device to be intuitively controlled by a patient to assist with opposition grasp, fine motor control, and ultimately facilitate motor recovery. We envision this at-home device augmenting the current in-clinic therapy and enabling tele-rehabilitation where a clinician can remotely monitor a patient's usage and performance.

Purpose – Pediatric disorders, such as cerebral palsy and stroke, can result in thumb-in-palm deformity greatly limiting hand function. This not only limits children's ability to perform activities of daily living but also limits important motor skill development. Specifically, the isolated orthosis for thumb actuation (IOTA) is 2 degrees of freedom (DOF) thumb exoskeleton that can actuate the carpometacarpal (CMC) and metacarpophalangeal (MCP) joints through ranges of motion required for activities of daily living. The paper aims to discuss these issues. Design/methodology/approach – IOTA consists of a lightweight hand-mounted mechanism that can be secured and aligned to individual wearers. The mechanism is actuated via flexible cables that connect to a portable control box. Embedded encoders and bend sensors monitor the 2 DOF of the thumb and flexion/extension of the wrist. A linear force characterization was performed to test the mechanical efficiency of the cable-drive transmission and the output torque at the exoskeletal CMC and MCP joints was measured. Findings – Using this platform, a number of control modes can be implemented that will enable the device to be controlled by a patient to assist with opposition grasp and fine motor control. Linear force and torque studies showed a maximum efficiency of 44 percent, resulting in a torque of 2.39±1.06 in.-lbf and 0.69±0.31 in.-lbf at the CMC and MCP joints, respectively. Practical implications – The authors envision this at-home device augmenting the current in-clinic and at-home therapy, enabling telerehabilitation protocols. Originality/value – This paper presents the design and characterization of a novel device specifically designed for pediatric grasp telerehabilitation to facilitate improved functionality and somatosensory learning.

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Objective The purpose of this case report is to describe the challenges that COVID-19 presented for therapists in a pediatric hospital and the response to these challenges. Methods (Case Description) The case report setting is a physical therapy and occupational therapy department (department) of an academic pediatric medical center that provides a range of health care services for children and youth. Challenges that COVID-19 presented to the Department included: (1) managing safety concerns for patients, their families, and staff; (2) continuing to provide high-quality therapy services within state mandated restrictions; (3) triaging patients; and (4) keeping clinicians employed and working productively. Results The Department therapists responded to these challenges by: (1) increasing communication huddles, (2) developing procedures for staffing and triaging of patients, (3) developing procedures for telehealth therapy services, and (4) designing a remote work program for all department employees. The number of patients and staff on site were reduced by initiating telehealth services, triaging patients, and developing a remote work plan. Communication huddles, department meetings, and supervision meetings were converted to virtual meetings. Staffing rates, patient-care productivity, and department project work were maintained. Conclusion In response to COVID-19, the department developed new protocols provided information about the protocols, which might be helpful for other pediatric hospitals or outpatient settings when planning for future pandemics or other issues that challenge the ability to provide usual care. Increasing the frequency of verbal and written communication on operational topics is recommended. Primary sources of information from national organizations (eg, the American Physical Therapy Association and the American Occupational Therapy Association) can assist with determining the scope of practice and code of conduct during a pandemic. Impact COVID-19 posed challenges to operations and delivery of patient care. Although this case report is specific to COVID-19, principles applied and lessons learned from this experience can be applied to other emergency situations.