Living with Pheochromocytoma: Day-to-Day Tips

Pheochromocytomas are rare adrenal gland tumors estimated to affect no more than eight in one million people annually. They form in the middle of the adrenal gland (called the adrenal medulla) and are made of chromaffin cells, which produce catecholamine hormones, including epinephrine (adrenaline) and norepinephrine (noradrenaline).

When detected early, a multidisciplinary medical team can successfully treat most pheochromocytomas. Managing the day-to-day symptoms and challenges associated with pheochromocytoma requires more than medical intervention. This article aims to provide actionable, practical tips for living with pheochromocytoma, helping patients improve their overall quality of life.

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Understanding Pheochromocytoma: Symptoms and Impact on Daily Life

Adrenaline and noradrenaline are hormones that facilitate the "fight-or-flight" response, helping us react quickly to danger and perceived stressors. When released, they prepare the body for action by mobilizing energy stores, directing blood flow to vital organs, and increasing heart rate, blood pressure, and respiratory rate.

Pheochromocytoma Symptoms

The symptoms of pheochromocytoma reflect a state of catecholamine excess and mirror an exaggerated stress response. People with pheochromocytomas experience episodic attacks, which can vary in intensity and frequency. Some people may be largely asymptomatic, whereas others may experience episodes multiple times a day. (47)

When symptoms occur, they may include:

High blood pressure (hypertension)
Rapid heartbeat (tachycardia and palpitations)
Sweating
Headache
Tremors or shaking
Pale skin
Shortness of breath
Panic or anxiety attacks
Abdominal pain
Constipation or diarrhea
Unintentional weight loss

Impact on Daily Living

Untreated pheochromocytomas can potentially cause serious, life-threatening consequences. This is largely related to chronic hypertension, which damages blood vessels and heart muscle over time and can lead to:

Cardiovascular disease (CVD) is a leading cause of death globally, accounting for 17.9 million deaths annually. Individuals with cardiovascular conditions often experience reduced physical and mental/emotional capacity, directly limiting their ability to perform activities of daily living (ADLs) and negatively impacting quality of life.

Importance of Symptom Awareness

Pheochromocytoma symptoms mimic those attributable to other more common conditions; as such, it can be challenging to diagnose. Recognizing the symptoms, often episodic and unresponsive to other treatments, is the first step toward better symptom management and treatment. (59)

Left untreated, this condition carries a high risk of morbidity and mortality. Detecting and treating pheochromocytoma prevents severe cardiovascular complications and tumor growth and leads to the opportunity for early diagnosis in familial cases, which can prevent further complications in relatives. (41)

Medication Management

Surgery, specifically adrenalectomy (removal of one or both adrenal glands), is the preferred treatment for pheochromocytoma due to its curative potential. The goal of surgery is to remove the tumor and normalize catecholamine levels. Adrenalectomy can often be performed laparoscopically, which is minimally invasive, though larger tumors may require open surgery. (41)

Before surgery, medications are prescribed to avoid hypertensive crises during surgery. Without being properly "blocked" with medication beforehand, anesthesia can cause sudden surges in catecholamine release and dangerously high blood pressure. (59)

Patients are first started on alpha-adrenergic blockers at least 7-14 days before surgery. These medications relax blood vessels and normalize blood pressure. Phenoxybenzamine is typically prescribed, but doxazosin, prazosin, and terazosin are also used. (26)

Beta-blockers are introduced only after alpha-blockade has been established, as using beta-blockers unopposed can actually worsen hypertension. Beta-blockers manage tachycardia and arrhythmias induced by catecholamine surges. Examples include propranolol, atenolol, and metoprolol. (26)

Taking these medications as prescribed is crucial for stabilizing cardiovascular function and preventing surgical complications. Without proper preoperative "blockage," surgical mortality rates are high. However, the operative mortality rate associated with surgical resection of pheochromocytoma in adequately prepared patients is less than 2-3%. (28)

In addition to the preoperative pharmaceutical blockade, patients should avoid taking the following medications known to precipitate a pheochromocytoma attack:

Dopamine D2 receptor antagonists: metoclopramide, sulpiride, amisulpride, tiapride, chlorpromazine, prochlorperazine, droperidol
Sympathomimetics: ephedrine, pseudoephedrine, methylphenidate, and phentermine
Opioid analgesics: morphine, pethidine, tramadol
Tricyclic antidepressants: amitriptyline, imipramine
Monoamine oxidase inhibitors: tranylcypromine, moclobemide, phenelzine
Corticosteroids: dexamethasone, prednisone, hydrocortisone, betamethasone

Stress Management and Relaxation Techniques

Physical and psychological stress can stimulate catecholamine secretion, leading to increased cardiovascular risk in individuals with pheochromocytoma. This underscores the need for effective stress management in these patients.

Various relaxation techniques can mitigate stress and its impact on pheochromocytoma. Yoga, meditation, and mindfulness practices have been shown to lower stress levels and improve overall well-being (52). Research indicates these practices can help reduce cortisol levels, lower blood pressure, and enhance relaxation responses. (42)

Exercise is another important component of stress management; however, it should be approached with caution. While regular physical activity helps alleviate stress, patients with pheochromocytoma should focus on low-intensity and low-impact exercises, such as walking, gentle yoga, or swimming. Strenuous exercise can increase catecholamine release and may precipitate a pheochromocytoma attack.

Diet and Nutrition for Pheochromocytoma

An anti-inflammatory, heart-healthy diet emphasizes whole, nutrient-dense foods such as fruits, vegetables, whole grains, lean proteins, and healthy fats. This diet minimizes processed foods, added sugars, and saturated fats, aiming to reduce inflammation and support cardiovascular health. Examples include the Mediterranean and DASH diets.

If your blood pressure is above normal, these dietary tips may help to keep it in a normal range:

Eat at least five servings of vegetables and two servings of fruit daily (27).
Eat magnesium-rich foods to get at least 500 mg of magnesium daily. Examples include green leafy vegetables, nuts, seeds, and whole grains.
Eat potassium-rich foods to get at least 4,700 mg of potassium daily. Examples include leafy green vegetables, lentils, potatoes, and apricots.
Limit sodium intake to 1,500-2,300 mg daily.*
Eat foods that boost nitric oxide production, such as beets, nuts, seeds, and leafy greens.

*Note that patients preparing for surgery are actually encouraged to eat a high-salt diet to maintain blood volume and prevent hypotension after alpha-blockade (41).

Certain foods are known to stimulate catecholamine release, so these should be avoided (57):

Tyramine-rich foods: aged cheese, beer, red wine, soy, chocolate, smoked meats, fermented foods
Coffee
Tea
Bananas
Citrus fruits
Vanilla

Lifestyle Modifications for Better Symptom Management

A patient-centered approach to pheochromocytoma identifies the unique factors affecting an individual's physical and mental well-being, providing holistic strategies for better symptom management.

Sleep

Poor sleep quality can increase stress levels, exacerbating catecholamine release and worsening pheochromocytoma symptoms.

Strategies for improving sleep include:

Establishing a regular sleep schedule
Creating a relaxing bedtime routine
Avoiding blue light-emitting screens before bedtime
Optimizing the sleep environment by minimizing noise and light

Environmental Adjustments

Identify and modify stress triggers in everyday life to minimize their impact. This could include:

Decluttering your living space
Creating a quiet area at home for relaxation
Discussing a flexible work schedule with your supervisor
Establishing work-life boundaries

Managing Social and Emotional Well-Being

Connecting with support groups or mental health professionals can provide emotional support and help patients cope with the psychological burdens of living with a chronic and rare condition. Sharing experiences with others facing similar challenges can foster a sense of community and belonging. Professional counseling can equip individuals with coping strategies and tools for managing anxiety and stress effectively.

Emergency Preparedness and Safety Tips

Patients with pheochromocytoma must recognize when symptoms escalate to a life-threatening crisis and discuss potential scenarios with their healthcare provider to understand appropriate responses. Carrying medical information, such as a medical alert bracelet, ensures first responders are informed of their condition, while open communication with family, friends, and coworkers fosters a support network for effective crisis recognition and response.

Hypertensive Crisis

A hypertensive crisis is a severe increase in blood pressure that can lead to stroke, heart attack, or organ damage.

Hypertensive Urgency: blood pressure readings exceed 180/120 mmHg without immediate organ damage. Although it does not require emergency treatment, prompt medical attention is necessary to avoid progression to severe complications.
Hypertensive Emergency: elevated blood pressure at 180/120 mm Hg or higher accompanied by signs and symptoms of organ damage. This condition requires immediate medical intervention.

If your blood pressure is elevated at 180/120 mmHg or above, retake it after 1-2 minutes. Contact your doctor if you are experiencing hypertensive urgency (elevated blood pressure in the absence of symptoms). Call 911 immediately if you are experiencing a hypertensive emergency, identified by the presence of these symptoms:

Chest pain
Shortness of breath
Back pain
Vomiting
Numbness
Weakness
Vision changes
Difficulty speaking
Confusion

Diabetic Ketoacidosis

Diabetes can be a primary symptom of pheochromocytoma; glucose intolerance is reported to affect about half of patients with pheochromocytoma.

Individuals with undiagnosed or poorly controlled diabetes may develop diabetic ketoacidosis (DKA), a severe and life-threatening complication of diabetes characterized by high blood glucose levels, the presence of ketones in the blood and urine, and metabolic acidosis.

Call 911 if you experience any of these signs or symptoms of DKA:

Blood sugar above 300 mg/dL
Moderate or high ketones in urine
Increased thirst
Frequent urination
Fast, deep breathing
Fruity-smelling breath
Headache
Fatigue
Nausea and vomiting
Abdominal pain
Confusion

Working With Healthcare Providers

Pheochromocytoma is a complex condition that necessitates a multidisciplinary approach to treatment, involving collaboration among various healthcare specialists, including:

Endocrinologists
Surgeons
Oncologists
Cardiologists
Obstetricians
Primary care physicians

Patients must maintain regular medical monitoring before and after treatment to ensure optimal health outcomes. Pre-treatment diagnosis and monitoring involves measuring catecholamine levels through blood and urine tests, utilizing imaging studies to localize the tumor, and monitoring blood pressure. (31)

After surgical resection, follow-up includes repeat biochemical testing to confirm successful and complete tumor resection. Lifelong annual monitoring is also recommended to detect tumor recurrence or metastasis (tumor spread). (41)

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Key Takeaways

Living well with pheochromocytoma is possible with early detection, proper treatment, and ongoing management. Untreated, the condition can lead to life-threatening complications, but with timely intervention, the prognosis is typically very good.
Patients are encouraged to form a multidisciplinary care team, including specialists like endocrinologists, cardiologists, and surgeons, to receive comprehensive, holistic care tailored to their needs.
To maintain quality of life, patients should develop lifestyle habits to manage symptoms, such as managing stress, getting adequate sleep, eating a heart-healthy diet, and engaging in low-intensity exercise.

Living with Pheochromocytoma: Day-to-Day Tips