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Amyotrophic Lateral Sclerosis (ALS) – Reading & Sharing

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.

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Kubler-Ross Model – The 5 Stages of Grieving

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.

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Making the Right Decision – Ethical and Legal Considerations (Reading & Sharing re: End-of-life)

Scope and Standards of Practice – End of life Care

  1. Structure and processes of care: the composition and qualifications of the interdisciplinary team and define how the team should collaborate with patients and families.
  2. Physical aspects of care: management of physical symptoms such as pain, fatigue, anxiety, and others.
  3. 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.
  4. Social aspects of care: focus on leveraging family strengths and social support mechanisms to alleviate family stress.
  5. 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.
  6. Cultural aspects of care: describes cultural competence and defines processes for the provision of culturally sensitive care.
  7. 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.
  8. 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.
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Mechanical Ventilation (Reading & Sharing)

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.

The two most common types of ventilations:

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Death Rattle and Anticholinergic Medications

Death rattle – as patients near death, they are unable to cough to clear secretions that begin to pool in the oropharynx and bronchi, resulting in rales (“death rattles”). Because the sound is often distressing to family members, an anticholinergic (glycopyrrolate or atropine) may be given subcutaneously to relieve respiratory distress. A hyoscine hydrobromide transdermal patch is also available, but action is slower, 12 hours compared to 1 minute for injections. Risks associated with anticholinergics include xerostomia (dry mouth), increased sedation, and increased delirium. Elevating the head of the bed or turning the patient to the side may also relieve rattling. Patients normally stop taking fluids as they near death, resulting in dehydration and drying of the mucous membranes of the mouth. The death rattle also begins to lessen.

Palliative care is a form of specialized medical care which aims to optimize the quality of life and alleviate the suffering of patients through early identification and treatment of new symptoms along with management of those that prove refractory.

Excessive secretions can cause the frequently noted ” death rattle” in patients that are actively dying. This is caused by relaxation of the oropharyngeal muscles leading to a pooling of secretion in the throat. While it is typically not distressing for the patient, it does often make family members and other visitors uncomfortable. Anticholinergic agents, especially sublingual atropine drops, can be administered to assist in secretion reduction. Anticholinergic agents have multiple side effects, including decreased/ absent bowel sounds, decreased sweating, hot skin, and mydriatic pupils (dilated pupils).