Not long ago, many people with severe nerve, muscle, spine, or lung disease died when their breathing muscles could no longer keep up with the need to breathe. Adults with amyotrophic lateral sclerosis (ALS), kyphoscoliosis or chronic obstructive pulmonary disease (COPD) died prematurely when their respiratory muscles failed. Many children born with a congenital nerve or muscle disease such as muscular dystrophy, spinal muscular atrophy, or severe cerebral palsy died of respiratory failure before adulthood. For such patients, weak breathing muscles also lead to difficulty swallowing, weak cough, and difficulty clearing mucous. Lung infections and pneumonia were common complications, leading to long hospitalizations and premature death.
This has changed with recent dramatic advances in home respiratory support where machines have been adapted to be used at home. These advances enable many patients with chronic respiratory failure to live safely and thrive at home. When needed, patients wear a face mask attached to a home ventilator the size of a shoe box to assist their breathing while they sleep at night and sometimes also during the day.
The machine assists each breath to allow the respiratory muscles to rest. Through this assisted ventilation, patients sleep better, breathe more comfortably during the day, and can often do more activity while awake. Other machines including cough assist devices and home suction machines can be used to help prevent lung infections or support patients at home when such infections do occur.
The Fishman Program
In 2017, with the support of a major gift from Jay and Randy Fishman and their many friends, Penn Medicine launched a multidisciplinary program devoted specifically to adults who benefit from long-term assisted ventilation. The multidisciplinary Fishman Program team attends to all the specialized needs of patients with chronic respiratory failure so that they can live safe, productive lives at home.
Travel is difficult for most people who need assisted ventilation. To stay closely in touch with patients, the Fishman Program combines home telemedicine and telephone check-ins with in-depth multi-disciplinary office visits.
Conditions We Treat
The Fishman Program Team offers comprehensive respiratory care for people 18 years of age and older with weak breathing muscles caused by common and rare diseases including:
The Penn Difference
The Fishman Program is a national leader in precision monitoring and management of assistive breathing devices. Home care begins with a comprehensive assessment of respiratory symptoms, function, and needs.
An intensive phase of treatment planning and set-up follows. Home ventilators, cough assist devices, suction machines, oxygen concentrators and other respiratory assistive devices are acquired and adjusted to individual needs. The goals are to restore breathing to normal, assure sound uninterrupted sleep, and prevent or minimize respiratory crises that might otherwise require hospitalization. For needs or concerns beyond respiration, consultations are arranged with collaborating specialists in neurology, sleep medicine, physical medicine, and cardiology.
After initial care is optimized, treatment focuses on home monitoring and prevention of respiratory setbacks. Blood oxygen and carbon dioxide are measured at the time of every office visit without the need for arterial puncture using a device that measures both blood gases through the skin. All home ventilators are set up with a telemetry device that allows the treatment team to monitor ventilator usage and function continuously through the internet. Problems are discovered and solved before they cause problems.