If you are a hospice nurse, a caregiver, or a family member of a terminally ill patient, you may have heard of the terms adrenal insufficiency and adrenal crisis. But what do they mean, and why are they important to know? This article will explain what adrenal insufficiency and adrenal crisis are, what causes them, and what consequences they can have for your patient. We will also describe how common and risky they are for terminally ill hospice patients and what you can do to prevent and treat them. We aim to help you better understand and manage these conditions and improve your patient’s quality of life and care.
What are adrenal insufficiency and adrenal crisis?
The adrenal glands are two small organs on top of the kidneys. They produce hormones that help the body cope with stress, regulate blood pressure and blood sugar, and maintain fluid and electrolyte balance. Adrenal insufficiency is when the adrenal glands do not produce enough of these hormones, especially cortisol. Cortisol is the main stress hormone that helps the body respond to physical, emotional, or environmental challenges. When the body faces severe stress, such as an infection, an injury, surgery, or a trauma, it needs more cortisol than usual. The body can enter an adrenal crisis if the adrenal glands cannot produce enough cortisol to meet this demand. An adrenal crisis is a life-threatening emergency that can cause low blood pressure, shock, coma, and death.
How common and how risky are adrenal insufficiency and adrenal crisis for terminally ill hospice patients?
Adrenal insufficiency and adrenal crisis are not very common in the general population, but they are more frequent and more dangerous for terminally ill hospice patients. This is because terminally ill patients often have multiple chronic diseases, such as cancer, heart failure, kidney failure, or liver failure, which can damage the adrenal glands or interfere with their function. They also often receive medications, such as opioids, steroids, or chemotherapy, which can suppress the adrenal glands or affect their stress response. Furthermore, terminally ill patients are more likely to experience severe stresses, such as infections, bleeding, dehydration, or pain, which can trigger an adrenal crisis. Therefore, it is estimated that up to 10% of terminally ill hospice patients may have adrenal insufficiency, and up to 5% may develop an adrenal crisis.
What are the main objectives and scope of this article?
The main objectives of this article are to help you:
Recognize the signs and symptoms of adrenal insufficiency and adrenal crisis and how to differentiate them from other conditions
Diagnose adrenal insufficiency and adrenal crisis and understand their limitations and challenges
Prevent adrenal insufficiency and adrenal crisis by following the recommendations and guidelines for steroid replacement therapy, stress dosing, and patient education.
Treat adrenal crisis by following the steps and principles of emergency management, such as immediate administration of hydrocortisone, rehydration, and electrolyte correction.
Consider the palliative care and quality of life aspects of adrenal insufficiency and adrenal crisis, such as the physical, psychological, social, and spiritual impact, as well as the ethical and legal issues and dilemmas.
The scope of this article is limited to the management of adrenal insufficiency and adrenal crisis in terminally ill hospice patients. It does not cover the management of other endocrine disorders, such as diabetes, thyroid disease, or pituitary disease. It also does not cover the management of adrenal insufficiency and adrenal crisis in other settings, such as the hospital, the clinic, or the home.
We hope this article will provide useful and practical information and empower you to care for your patient with compassion and confidence.
Diagnosis and Prevention of Adrenal Insufficiency and Adrenal Crisis
Suppose you are caring for a terminally ill patient. In that case, you need to be aware of the possibility of adrenal insufficiency and adrenal crisis, two serious conditions affecting your patient’s quality of life and survival. This section will explain how to recognize, diagnose, and prevent these conditions.
What are the signs and symptoms of adrenal insufficiency and adrenal crisis?
Adrenal insufficiency and adrenal crisis are caused by a lack of cortisol. This hormone helps the body cope with stress, regulate blood pressure and blood sugar, and maintain fluid and electrolyte balance. Adrenal insufficiency is a chronic condition that develops gradually, while the adrenal crisis is an acute emergency that occurs suddenly.
The signs and symptoms of adrenal insufficiency and adrenal crisis may vary from person to person, but they often include:
Darkening of the skin, especially in areas exposed to friction
Mood changes, such as irritability, anxiety, and depression
Loss of body hair or sexual function in women
Some of these symptoms may also occur in other conditions, such as dehydration, infection, bleeding, or pain. Therefore, monitoring your patient closely and reporting any changes to your healthcare provider is important.
An adrenal crisis is a life-threatening situation that requires immediate treatment. It can be triggered by severe stress, such as an infection, injury, surgery, or trauma. It can also occur if the patient misses or reduces the dose of steroid medication or if the medication is not absorbed properly due to vomiting or diarrhea. The signs and symptoms of adrenal crisis may include:
Severe abdominal pain, back pain, or leg pain
Severe vomiting and diarrhea leading to dehydration
If you suspect that your patient is having an adrenal crisis, you should call 911 or seek emergency medical attention right away. Do not wait for the test results or try to treat the patient at home.
How are adrenal insufficiency and adrenal crisis diagnosed?
Diagnosing adrenal insufficiency and adrenal crisis depends on measuring cortisol and other hormones in the blood. However, these tests are not always available or reliable and may not reflect the patient’s condition. Therefore, the diagnosis also depends on the clinical judgment of the health care provider, based on the patient’s history, symptoms, and response to treatment.
Some of the tests that may be used to diagnose adrenal insufficiency and adrenal crisis are:
Blood test. This test can measure the levels of cortisol, adrenocorticotropic hormone (ACTH), sodium, potassium, and glucose in the blood. ACTH is a hormone that stimulates the adrenal glands to produce cortisol. Low cortisol and high ACTH levels indicate primary adrenal insufficiency, which means the problem is in the adrenal glands. Normal or low ACTH levels indicate secondary or tertiary adrenal insufficiency, which means the problem is in the pituitary gland or the hypothalamus, the brain’s parts controlling the adrenal glands. Low sodium, high potassium, and low glucose levels may also indicate adrenal insufficiency or crisis.
ACTH stimulation test. This test involves injecting a synthetic form of ACTH into the vein or muscle and measuring the cortisol level in the blood before and after the injection. The cortisol level should rise after the injection, indicating that the adrenal glands are working properly. If the cortisol level does not rise, the adrenal glands are not responding to ACTH, and the patient has adrenal insufficiency. This test can also help differentiate between primary and secondary or tertiary adrenal insufficiency. In primary adrenal insufficiency, the cortisol level does not rise, while in secondary or tertiary adrenal insufficiency, the cortisol level may rise slightly or after a delay.
Insulin-induced hypoglycemia test. This test involves injecting insulin into the vein and measuring the cortisol and glucose levels in the blood at regular intervals. Insulin lowers the blood sugar level, which normally triggers the release of cortisol from the adrenal glands. If the cortisol level does not rise, the adrenal glands are not responding to stress, and the patient has adrenal insufficiency. This test is used to diagnose secondary or tertiary adrenal insufficiency, as it also measures the function of the pituitary gland and the hypothalamus. However, this test is risky and should only be done under close supervision by an endocrinologist, a specialist in hormone disorders.
Imaging tests. These tests involve taking pictures of the adrenal glands, the pituitary gland, or the hypothalamus using techniques such as computed tomography (CT) scan, magnetic resonance imaging (MRI) scan, or ultrasound. These tests can help detect abnormalities, such as tumors, infections, bleeding, or inflammation, that may cause adrenal insufficiency or crisis.
These tests have some limitations and challenges, such as:
They may not be available or accessible in some settings, especially in emergencies.
Depending on the results’ timing, method, and interpretation, they may not be accurate or reliable.
They may not reflect the patient’s true condition, as cortisol levels can vary depending on the time of the day, the stress level, the type and dose of medication, and other factors.
They may not be sufficient to confirm or exclude the diagnosis, as some patients may have normal or borderline results but still have symptoms of adrenal insufficiency or crisis.
Therefore, the diagnosis of adrenal insufficiency and adrenal crisis should not rely solely on the test results but also on the healthcare provider’s clinical judgment based on the patient’s history, symptoms, and response to treatment.
How can adrenal insufficiency and adrenal crisis be prevented?
Preventing adrenal insufficiency and adrenal crisis depends on the underlying cause and the type of adrenal insufficiency. In general, the prevention strategies include:
Taking steroid medication as prescribed by the health care provider and not missing or reducing the dose without medical advice.
Increasing the dose of steroid medication during times of stress, such as illness, injury, surgery, or trauma, according to the instructions of the health care provider, is called stress dosing. It helps prevent an adrenal crisis by providing the extra cortisol needed to cope with stress.
Carrying a medical alert card or bracelet that indicates the diagnosis of adrenal insufficiency, the type and dose of medication, and the contact information of the health care provider. This can help in an emergency, as it can alert the medical staff to the need for immediate treatment with steroids.
Having an emergency kit that contains injectable hydrocortisone, syringes, needles, alcohol wipes, and instructions on how to use them. This can help in an adrenal crisis, providing life-saving treatment before reaching the hospital or getting medical attention.
Educating oneself and others about the signs and symptoms of adrenal insufficiency and adrenal crisis and the steps to take in an emergency can help in early recognition and prompt treatment of these conditions and reduce the risk of complications and death.
Seeking regular follow-up care from the health care provider and having periodic blood tests to monitor the hormone levels and the response to treatment. This can help adjust the dose of medication, detect any problems or complications, and provide guidance and support.
By following these prevention strategies, you can help your patient manage adrenal insufficiency and adrenal crisis effectively and improve the quality of life and care for your patient.
Emergency Management of Adrenal Crisis
An adrenal crisis is a life-threatening condition that occurs when the adrenal glands do not produce enough cortisol, a hormone that helps the body cope with stress. Cortisol is essential for maintaining blood pressure, blood sugar, and immune function. Without enough cortisol, the body can go into shock and organ failure.
An adrenal crisis can be triggered by numerous factors, such as infection, injury, surgery, or emotional stress. It can also happen if a person with adrenal insufficiency (a chronic condition where the adrenal glands do not produce enough cortisol) does not take enough steroid medication or misses a dose during a stressful event.
An adrenal crisis is a medical emergency that requires immediate treatment. The following steps and principles should be followed:
Inject 100 mg of hydrocortisone intravenously (IV) or intramuscularly (IM) immediately. Hydrocortisone is a synthetic form of cortisol that can restore the hormone level and prevent shock. Do not wait for laboratory tests or other confirmations to start treatment.
Give fluids and electrolytes IV to correct dehydration and electrolyte imbalance. Use normal saline (0.9% sodium chloride) or a similar solution. Monitor the blood pressure, heart rate, and urine output closely.
Continue giving hydrocortisone at a dose of 200 mg per 24 hours, either as a continuous IV infusion or as 50 mg every 6 hours IV or IM. Adjust the dose according to the clinical response and the severity of the stress. Maintain this treatment until the person recovers and can resume oral steroid medication.
Identify and treat the underlying cause of the adrenal crisis, such as infection, bleeding, or trauma. As needed, give antibiotics, blood transfusions, or other interventions.
Educate the person and their family or caregivers about the signs and symptoms of adrenal crisis and how to prevent and manage it. Please provide them with a steroid emergency card, a medical alert bracelet, and an emergency injection kit. Teach them how to inject hydrocortisone IM in an emergency and when to seek medical help.
Review the evidence and best practices for the emergency management of adrenal crisis and highlight the potential complications and pitfalls.
The evidence and best practices for the emergency management of adrenal crisis are based on clinical guidelines and expert consensus from various professional organizations, such as the Society for Endocrinology, the Royal College of Physicians, and the Endocrine Society. These guidelines are supported by observational studies, case reports, and clinical experience. However, there is a lack of randomized controlled trials and high-quality evidence on some aspects of the diagnosis and treatment of adrenal crisis.
Some of the potential complications and pitfalls of adrenal crisis include:
Delayed diagnosis and treatment can increase the risk of death and organ damage. Adrenal crisis can be mistaken for other conditions, such as sepsis, diabetic ketoacidosis, or cardiac arrest. Therefore, having a high suspicion index and starting treatment promptly based on clinical signs and symptoms is important rather than waiting for laboratory tests or imaging results.
Inadequate or excessive steroid replacement can cause adverse effects or insufficient response. The dose and duration of hydrocortisone treatment should be tailored to the individual’s needs and the type and severity of the stress. Too much hydrocortisone can cause fluid retention, high blood pressure, high blood sugar, and an increased risk of infection. Too little hydrocortisone can cause persistent symptoms, recurrent crises, or adrenal insufficiency.
Lack of patient and caregiver education and awareness can lead to poor adherence, missed doses, or inappropriate dose adjustment. Patients and caregivers should be informed about the causes, symptoms, and prevention of adrenal crisis, as well as how to use the steroid emergency card, the medical alert bracelet, and the emergency injection kit. They should also be advised to contact their healthcare provider or seek emergency care if they experience any signs of adrenal crisis or if they have any questions or concerns about their steroid medication.
An adrenal crisis is a serious and potentially fatal condition that requires immediate recognition and treatment. Delayed or inadequate treatment can result in irreversible organ damage, coma, or death. Therefore, hospice nurses, caregivers, and family members must be aware of the risk factors, signs and symptoms, and management of the adrenal crisis and act quickly and appropriately in case of an emergency.
Hospice nurses, caregivers, and family members play a vital role in preventing and treating adrenal crises. They can:
Monitor the person’s condition and vital signs regularly and report any changes or concerns to the healthcare provider.
Administer the steroid medication as prescribed and adjust the dose according to the stress level and the healthcare provider’s instructions.
Ensure that the person has enough supply of steroid medication and store it properly and safely.
Help the person cope with the physical and emotional stress of the illness and provide end-of-life care, comfort, and support.
Recognize the signs and symptoms of adrenal crisis, and inject hydrocortisone IM as soon as possible if the person is unable to take oral medication or is unconscious.
If the patient is full code, call 911 or the local emergency number and inform the operator that the person has adrenal insufficiency and is having an adrenal crisis.
Stay with the person until help arrives, and continue to monitor and reassure the person.
Follow the instructions of the emergency medical personnel and provide them with the steroid emergency card and the person’s medical history.
If the patient is in hospice, call the hospice provider; if you are the hospice nurse reading this article, follow the orders of the patient’s provider or medical director.
By following these steps and principles, hospice nurses, caregivers, and family members can help prevent, recognize, and treat adrenal crises and improve the quality of life and the comfort of the person with adrenal insufficiency.
Palliative Care and Quality of Life Considerations
Palliative care is a type of care that focuses on improving the quality of life of patients with serious or life-limiting illnesses and their families. It aims to relieve physical, emotional, social, and spiritual suffering and respect the values and preferences of patients and families. Palliative care can be provided along with curative or life-prolonging treatments or as the main focus of care when such treatments are no longer desired or beneficial.
Adrenal insufficiency and adrenal crisis are conditions that affect the production and function of cortisol, a hormone that helps the body cope with stress and maintain vital functions. Adrenal insufficiency is a chronic condition that requires lifelong steroid replacement therapy. In contrast, an adrenal crisis is a life-threatening emergency that requires immediate treatment with high-dose steroids and fluids. Both conditions can significantly impact the quality of life of patients and their families, especially in palliative care.
Some of the ways that adrenal insufficiency and adrenal crisis can affect the physical, psychological, social, and spiritual aspects of palliative care and quality of life are:
Physical: Adrenal insufficiency and adrenal crisis can cause various symptoms, such as fatigue, weakness, nausea, vomiting, abdominal pain, low blood pressure, dehydration, electrolyte imbalance, and hypoglycemia. These symptoms can interfere with the patient’s ability to perform daily activities, enjoy hobbies, and participate in social events. They can also increase the risk of infections, bleeding, and organ damage. Adrenal insufficiency and adrenal crisis can also affect the response to other treatments, such as chemotherapy, radiation, or pain medications, and may require dose adjustments or additional medications. Adrenal insufficiency and adrenal crisis can also complicate the management of other symptoms, such as breathlessness, anxiety, or depression, and may require specialized interventions.
Psychological: Adrenal insufficiency and adrenal crisis can cause emotional distress, such as fear, anger, sadness, guilt, or hopelessness. Patients may feel overwhelmed by the complexity and uncertainty of their condition. They may worry about the possibility of having an adrenal crisis, especially in situations where they may not have access to adequate medical care. Patients may also experience changes in their self-image, self-esteem, and sense of control and may struggle with coping with their illness and its impact on their lives. Patients may also have concerns about the burden and impact of their condition on their family and caregivers and may feel isolated or misunderstood by others.
Social: Adrenal insufficiency and adrenal crisis can affect the patient’s relationships with family, friends, and other social supports. Patients may need more assistance and care from their families and caregivers and may feel dependent or indebted to them. Patients may also feel less able to fulfill their roles and responsibilities, such as spouse, parent, child, sibling, friend, or worker. They may feel guilty or resentful about their situation. Patients may also have difficulties communicating their needs and preferences and may encounter stigma or discrimination from others who do not understand their condition. Patients may also have fewer opportunities or motivation to engage in social activities and feel lonely or isolated.
Spiritual: Adrenal insufficiency and adrenal crisis can affect the patient’s spiritual beliefs, values, and practices. Patients may question the meaning and purpose of their life and illness and seek answers or explanations from their faith or spirituality. Patients may also experience spiritual distress, such as feeling abandoned, betrayed, or punished by a higher power or feeling angry, doubtful, or hopeless about their faith or spirituality. Patients may also have unmet spiritual needs, such as forgiveness, reconciliation, gratitude, or peace. Patients may also have spiritual resources, such as prayer, meditation, rituals, or sacred texts, which can provide comfort, strength, and hope.
Ethical and Legal Issues and Dilemmas
Ethical and legal issues and dilemmas are common in palliative care, especially when dealing with complex and sensitive decisions about the management of adrenal insufficiency and adrenal crisis in terminally ill hospice patients. Some of the ethical and legal issues and dilemmas that may arise are:
Informed consent: Informed consent is obtaining the patient’s voluntary and informed agreement to a proposed treatment or intervention after providing adequate information about the benefits, risks, alternatives, and consequences of the treatment or intervention. Informed consent is based on respect for autonomy, beneficence, non-maleficence, and justice. Informed consent is also a legal requirement in most jurisdictions and may be written or verbal, depending on the situation. Informed consent may be challenging in the context of adrenal insufficiency and adrenal crisis because patients may have impaired decision-making capacity due to their illness, medications, or symptoms and may not be able to understand or retain the information provided. Patients may also have cognitive or emotional biases, such as denial, wishful thinking, or fear, affecting their judgment and preferences. Patients may also have conflicting or changing values and goals and may be unable to anticipate their future needs and wishes. Therefore, assessing the patient’s decision-making capacity is important, providing clear and accurate information, eliciting and respecting the patient’s values and goals, and involving the patient’s family and caregivers, as appropriate, in the informed consent process.
Advance directives: Advance directives are documents that allow the patient to express their preferences and instructions for their future medical care if they cannot make or communicate their own decisions. Advance directives may include a living will, which states the patient’s wishes for specific treatments or interventions, such as resuscitation, artificial nutrition and hydration, or palliative sedation, and a durable power of attorney for health care, which appoints a person to act as the patient’s surrogate decision-maker, by the patient’s wishes and best interests. Advance directives are based on respect for autonomy, beneficence, non-maleficence, and justice. Advance directives are also legally binding in most jurisdictions and may require certain formalities, such as signatures, witnesses, or notarization, depending on the situation. Advance directives may be helpful in the context of adrenal insufficiency and adrenal crisis because they can provide guidance and direction for the health care team, the patient’s family and caregivers, and the patient’s surrogate decision-maker if they cannot make or communicate their own decisions. Advance directives can also reduce uncertainty, conflict, and stress and enhance the quality of care and the quality of life of the patient and their family and caregivers. Therefore, it is important to encourage and assist the patient in completing and updating their advance directives and reviewing and respecting them, as appropriate, in managing adrenal insufficiency and adrenal crisis.
Withholding or withdrawing treatment: Withholding or withdrawing treatment is the decision not to start or to stop treatment or intervention that is considered futile, ineffective, burdensome, or unwanted by the patient, to respect the patient’s wishes and best interests, and to allow a natural death. Withholding or withdrawing treatment is based on respect for autonomy, beneficence, non-maleficence, and justice. Withholding or withdrawing treatment is also legally permissible in most jurisdictions if it is consistent with the patient’s informed consent, advance directives, or surrogate decision-maker’s authorization, depending on the situation. Withholding or withdrawing treatment may be relevant in the context of adrenal insufficiency and adrenal crisis because patients may have preferences and instructions for specific treatments or interventions, such as steroid replacement therapy, fluids, and electrolytes, antibiotics, blood transfusions, or other interventions, which may affect their comfort, dignity, and quality of life at the end of life. Patients may also have preferences and instructions for the level of aggressiveness and invasiveness of the treatment, such as whether to seek hospitalization or to stay at home, whether to receive palliative or curative care, or whether to pursue or decline life-prolonging measures. Therefore, it is important to discuss and document the patient’s preferences and instructions for withholding or withdrawing treatment and to follow and respect their preferences and instructions, as appropriate, in managing adrenal insufficiency and adrenal crisis.
Practical Tips and Resources
Practical tips and resources are useful for hospice nurses, caregivers, and family members to support and care for patients with adrenal insufficiency and adrenal crisis. Some of the practical tips and resources are:
Educate yourself and others about adrenal insufficiency and adrenal crisis, including the causes, symptoms, prevention, and treatment. Learn how to recognize and respond to an adrenal crisis and how to use the steroid emergency card, the medical alert bracelet, and the emergency injection kit. Seek reliable and credible sources of information, such as the National Institute of Diabetes and Digestive and Kidney Diseases, the Society for Endocrinology, the Endocrine Society, and the Addison’s Disease Self-Help Group.
Communicate effectively and respectfully with the patient, the health care team, and other family members and caregivers. Share your concerns, questions, and feelings, and listen to theirs. Express your appreciation and gratitude and ask for help when you need it. Use clear and simple language and avoid medical jargon. Use open-ended questions and avoid assumptions or judgments. Use active listening skills, such as paraphrasing, reflecting, and summarizing. Use non-verbal communication skills, such as eye contact, nodding, and smiling. Use empathic statements, such as “I understand how you feel” or “I’m sorry you’re going through this.”
Provide the patient with physical, emotional, social, and spiritual comfort and support. Help the patient with daily activities, such as bathing, dressing, eating, and moving.
Hospice Care
Hospice care is a type of palliative care that is specifically designed for patients who have a terminal illness and a life expectancy of six months or less. Hospice care focuses on providing comfort and dignity to patients and their families rather than curing or prolonging life. Hospice care can be provided in the patient’s home, a hospice facility, or other settings, such as hospitals, nursing homes, or assisted living facilities.
Hospice care is provided by a team of professionals and volunteers who work together to address the patient’s and their family’s physical, emotional, social, and spiritual needs. The hospice team may include doctors, nurses, social workers, chaplains, home health aides, and trained volunteers. The hospice team works with the patient, the family, and the patient’s primary doctor to create a personalized plan of care that reflects the patient’s wishes and goals.
Hospice care offers various services and support, such as:
Pain and symptom management to relieve suffering and improve quality of life
Medications, equipment, and supplies related to the terminal illness
Emotional and spiritual counseling to cope with grief, loss, and existential issues
Bereavement support to help the family and caregivers deal with the death of the patient
Respite care, to give the family and caregivers a break from caregiving
Education and information to help the patient and the family understand the illness and the care options
Hospice care is covered by Medicare, Medicaid, and most private insurance plans for eligible patients. It is not a one-time decision; the patient and the family can change their minds and stop anytime. Hospice care does not give up hope but rather accepts the reality of the illness and chooses to live as fully and comfortably as possible. It is a compassionate and holistic approach to end-of-life care that respects the patient’s and their family’s dignity and autonomy.
Conclusion
Adrenal insufficiency affects the production of cortisol, a hormone that helps the body cope with stress and maintain vital functions. Adrenal crisis is a life-threatening complication of adrenal insufficiency that requires immediate treatment with steroids and fluids. Both conditions can affect the quality of life of patients and their families, especially in hospice care.
Hospice care is a type of palliative care that focuses on improving the quality of life of patients with terminal illnesses and their families. It aims to relieve physical, emotional, social, and spiritual suffering and respect the values and preferences of patients and families. Hospice care can be provided in different settings, such as the patient’s home, a hospice facility, or a hospital.
The management of adrenal insufficiency and adrenal crisis in terminally ill hospice patients involves various clinical, ethical, and legal issues and dilemmas, such as informed consent, advance directives, and withholding or withdrawing treatment. These issues and dilemmas require careful assessment, communication, and decision-making involving the patient, the health care team, and the family and caregivers, as appropriate. Managing adrenal insufficiency and adrenal crisis also requires practical tips and resources, such as education, support, and emergency kits.
