PURPOSE: Although technological cardiopulmonary therapies, such as noninvasive ventilation (NIV), have increased life span for patients (pts) with Duchenne muscular dystrophy (DMD), the disease remains fatal. Pts with end-stage DMD who forgo “do not resuscitate” directives may be partially resuscitated after near-fatal events, exposing them to the risk of brain damage. PURPOSE: To assess the frequency of attempted resuscitation among pts with DMD in our clinic who experienced cardiopulmonary arrest, and to describe their outcomes.
METHODS: Retrospective chart review.
RESULTS: Ten of our pts with DMD died during the time period 9/1/03-4/1/07. Mean age at death +/- SD: 20.7 +/- 2.7 years. Three of the 10 pts had unmeasurable vital capacities and were dependent on NIV 24 hrs/day. Mean vital capacity of the remaining 7 pts: 0.57 +/- 0.53 liters. All pts were prescribed daily NIV but 4 pts used NIV infrequently. All pts had cardiomyopathy. Four pts were in congestive heart failure. Two pts had cardiac defibrillators. After cardiopulmonary arrest, five of the 10 pts underwent resuscitation attempts. No pt was successfully resuscitated. Four of the 5 pts died immediately. One pt was partially resuscitated, experienced brain damage, and died after being taken off the ventilator by his family.
CONCLUSION: A significant proportion of our pts with end-stage DMD who experienced cardiopulmonary arrest underwent attempted resuscitation. None of the resuscitation attempts was successful. Lack of “do not resuscitate” advance directives exposed these pts to the risk of partial resuscitation and brain damage.
CLINICAL IMPLICATIONS: Pts with advanced DMD are increasingly cared for by cardiologists, pulmonologists, and intensivists, whose focus may be technologically oriented. We speculate that use of technological therapies like assisted ventilation and defibrillators among such pts may produce an unrealistically optimistic view of their prognosis, causing resistance to advance “do not resuscitate” directives, even among pts with end-stage disease. Studies are needed regarding how to reconcile palliative and technological therapies to optimize end-of-life care among pts with severe DMD.
DISCLOSURE: Garey Noritz, No Financial Disclosure Information; No Product/Research Disclosure Information