Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. Motor neurons are affected in certain patterns, such as cervical, thoracic, lumbar, and bulbar (facial) regions. Although initial presentations can vary, eventually upper and lower motor neurons are lost in the two types of ALS: familial and sporadic.
About 6000 people in the U.S. are diagnosed with ALS yearly. A French physician, Jean Charcot, identified ALS in 1869; initially, the disease was called “Charcot Disease.” However, in 1939, when the famous New York Yankees baseball player Lou Gehrig was diagnosed with ALS, it became known as “Lou Gehrig’s Disease”. (Gehrig died from ALS in 1941 at age 37.) It is estimated 300,000 Americans live with ALS in 2018. The median age of onset is 55, and disease indicence peaks between ages 70-75. More males are affected than females. Approximately 90% of ALS cases are determined to be sporadic, or accquired, while the remainder are considered familial, or hereditary.
Elisabeth Kubler-Ross Model describes five stages in which the dying patient moves through denial, anger, bargaining, depression, and acceptance.
The first stage of the process of grieving and preparing for death is denial. This may initially manifest as shock or speechlessness. It is common to believe a mistake in the prognosis has been made due to inaccurate test results, having not attempted the correct treatment, or deficits in knowledge of their provider.
Scope and Standards of Practice – End of life Care
Structure and processes of care: the composition and qualifications of the interdisciplinary team and define how the team should collaborate with patients and families.
Physical aspects of care: management of physical symptoms such as pain, fatigue, anxiety, and others.
Psychological and psychiatric aspects of care: assessment of the psychosocial needs of patients and families. the requirement for bereavement support is included in this domain.
Social aspects of care: focus on leveraging family strengths and social support mechanisms to alleviate family stress.
Spiritual, religious, and existential aspects of care: emphasizes the roles of the members of the interdisciplinary team, especially the chaplain, in recognizing and addressing spiritual and existential distress. Specifically, the competency of all team members in understanding and supporting the religious practice preferences of patients and families is stressed.
Cultural aspects of care: describes cultural competence and defines processes for the provision of culturally sensitive care.
Care of the patient at the end of life: Highlights the importance of providing multidimensional interdisciplinary end-of-life care for patients and their families, which includes educating them and building them through the dying process.
Ethical and legal aspects of care: addresses advance care planning, ethics, and legal aspects of care. The role of the interdisciplinary team in broaching end-of-life conversations and documenting patients’ preferences is stressed. Consultation with ethics committees and legal counsel is also emphasized.
The clinical applications of ventilation and the use of specific modes during the acute stage of illness focus on protecting the lung and improving the patient outcomes.
Acute respiratory distress syndrome (ARDS), the most severe presentation of acute lung injury, results from an acute insult to the body that may be direct or indirect (pneumonia vs sepsis). The release of mediators and a host of other toxic substances affect the alveolar- capillary permeability adversely and result in a noncardiac pulmonary edema. Pathology includes decreased compliance, shunting, and refractory hypoxemia. No definitive treatment, but the therapy focus on managing the underlying condition and on supportive mechanical ventilation.
Chronic obstructive pulmonary disease (COPD) is the third leading cause of death in the United States.
Conventional COPD treatments:
Inhaled bronchodilator therapy: metered-dose inhalers and spacers, dry powder inhalers, or wet nebulizers
Inhaler technique should be assessed to ensure that a patient is able to use a device effectively.
The combination of a long-acting anticholinergic with an as-needed short-acting beta-agonist is a standard initial approach.
For many patients with advanced COPD, the addition of a combination inhaler containing a long-acting beta-agonist and an inhaled corticosteroid to a long-acting anticholinergic agent provides benefit in terms of symptom management and quality of life.