Cystic Fibrosis: Suffering and Disease of Body, Mind, and Spirit
Mary Dell, MD, DMin, Nationwide Children's Hospital
Cystic fibrosis (CF) is the most common autosomal recessive disease among Caucasians in the United States. One in twenty-five is a CF carrier, and one individual in 2,300 to 3,000 live births has CF disease. Other populations are affected, with carrier frequency and incidence varying by race and ethnicity. Approximately 32,000 adults and children in the United States have been diagnosed with CF, and 70,000 have the disease worldwide. There are approximately five million carriers in the US. The median predicted survival age increased from fourteen years in 1969 to over 36 years in 2005, and is currently forty years. Over half of all current CF patients are eighteen years and older, with the percentage of those over eighteen increasing yearly for the past quarter century.
All organ systems with epithelial tissues are affected. Common disease manifestations include cough, difficulty breathing, and frequent pneumonias requiring antibiotics, oxygen, and hospitalizations. The pancreas, liver, and gastrointestinal tract are involved, leading to malabsorption, malnutrition, and diabetes. Other medical comorbidities include male sterility, metabolic bone disease, growth issues, and the potential of increased cancers as the CF population survives to older ages. The daily care burden can be astounding, with several respiratory treatments and many medications every day. Physical pain increases with age, and includes musculoskeletal chest pain from violent coughing, abdominal pain, neuropathic pain from antibiotic neurotoxicity, and pain from bone fractures.
CF patients suffer emotionally as well, with increased incidences of depression, anxiety, panic, and substance use and dependence compared to medically healthy populations. While psychotropic medications are helpful, the selection of agents can be limited for CF patients due to drug interactions and vulnerability to side effects. Professionals who care for CF patients, encouraged by the Cystic Fibrosis Foundation, are now screening for depression, anxiety, and suicidal ideation on a regular basis.
Until recently, the spiritual care of CF patients and their loved ones had not been a priority, but it is receiving greater attention with the increased life expectancy and medical and psychiatric care burdens. Questions of theodicy, the meaning of life, and the religious and spiritual angst of palliative and end-of-life care are being experienced by increasing numbers of adult CF patients. The roles of spirituality and religious beliefs, rituals, and communities are arising more often as young people with CF pursue education, jobs and careers, marry, and seek to have families, even as they live with the disease. In addition, regardless of the severity of the illness or prognosis, the anguish of parents and caregivers of infants and children is heart-wrenching, with significant implications for their faith. Religion and spirituality can provide resources for coping, though at times contribute to guilt.
This presentation will review the medical and psychiatric contributions to potential pain and suffering for those affected by CF. The implications of CF for religion/spiritual lives with by discussed, including how medical and faith communities can work together to provide care for this group of individuals and their loved ones.
All organ systems with epithelial tissues are affected. Common disease manifestations include cough, difficulty breathing, and frequent pneumonias requiring antibiotics, oxygen, and hospitalizations. The pancreas, liver, and gastrointestinal tract are involved, leading to malabsorption, malnutrition, and diabetes. Other medical comorbidities include male sterility, metabolic bone disease, growth issues, and the potential of increased cancers as the CF population survives to older ages. The daily care burden can be astounding, with several respiratory treatments and many medications every day. Physical pain increases with age, and includes musculoskeletal chest pain from violent coughing, abdominal pain, neuropathic pain from antibiotic neurotoxicity, and pain from bone fractures.
CF patients suffer emotionally as well, with increased incidences of depression, anxiety, panic, and substance use and dependence compared to medically healthy populations. While psychotropic medications are helpful, the selection of agents can be limited for CF patients due to drug interactions and vulnerability to side effects. Professionals who care for CF patients, encouraged by the Cystic Fibrosis Foundation, are now screening for depression, anxiety, and suicidal ideation on a regular basis.
Until recently, the spiritual care of CF patients and their loved ones had not been a priority, but it is receiving greater attention with the increased life expectancy and medical and psychiatric care burdens. Questions of theodicy, the meaning of life, and the religious and spiritual angst of palliative and end-of-life care are being experienced by increasing numbers of adult CF patients. The roles of spirituality and religious beliefs, rituals, and communities are arising more often as young people with CF pursue education, jobs and careers, marry, and seek to have families, even as they live with the disease. In addition, regardless of the severity of the illness or prognosis, the anguish of parents and caregivers of infants and children is heart-wrenching, with significant implications for their faith. Religion and spirituality can provide resources for coping, though at times contribute to guilt.
This presentation will review the medical and psychiatric contributions to potential pain and suffering for those affected by CF. The implications of CF for religion/spiritual lives with by discussed, including how medical and faith communities can work together to provide care for this group of individuals and their loved ones.