When EMS providers think about endocrine emergencies, patients suffering from Addison’s disease, Cushing’s syndrome and Graves’ disease are not typically what comes to mind. Utilizing real-life EMS cases, this article will highlight why it is import for EMS providers to know about these less common endocrine emergencies.
Endocrine Case #1
Called to a private residence at 1630. Upon arrival you find a 37-year-old male who is unconscious and unresponsive to verbal stimuli.
AMPLE history reveals:
A = No known allergies
M = Solucortef, Fludrocortisone
P = Addison’s disease
L = Lunch around 11:30
E = Wife states the patient was taking a nap before going out to dinner. Wife also states the patient hasn’t been feeling well with flu like symptoms for about four days (nausea, diarrhea and vomiting). Patient was last seen at 1200 prior to nap.
Physical assessment does not reveal any pertinent findings. Patient withdraws to pain appropriately. Pupils are PERL. Skin is cool, pale and diaphoretic. Wife states the patient occasionally experiences issues with low blood sugar which are typically precipitated by stress or poor dietary compliance. Wife states normal dietary intake for today.
Vital signs:
B/P = 78/40
Pulse = 124
Respirations = 32 and regular
Pulse oximetry = 92% on room air
BGL = 41mg/dL
The patient was given 25Gm of 10% IV dextrose. BGL increased to 76mg/dL, but mental status did not improve. 600-700mL of normal saline was administered during transport. The patient was delivered to the hospital still unconscious and unresponsive. Immediately after arrival, the ED physician diagnosed the patient with Addisonian Crisis.
This patient was admitted into the ICU. Three days into the hospital course he developed kidney failure. The patient unfortunately died on day five due to end organ failure.
Addison’s Disease
Addison’s disease is also known as primary adrenal insufficiency. The disease affects approximately three in 100,000 people.3 It arises when the adrenal glands are damaged and cannot produce adequate levels of cortisol and frequently involves low levels of aldosterone. Damage to the adrenal glands is often caused by the body’s own immune system.
Primary functions of cortisol:
- Increases glycogen stores in the liver
- Maintains vascular tone and blood pressure
- Aids in normal metabolism of fat, protein, and carbohydrates
- Suppresses the immune system
Primary functions of aldosterone:
- Increases levels of sodium
- Decreases levels of potassium
- Responsible for increasing blood volume levels
Addison’s disease involves inadequate levels of cortisol and aldosterone. Here are common physiologic implications from suffering with low cortisol and aldosterone levels:
- Difficulty maintaining adequate glucose levels and blood pressure
- Hyponatremia
- Hyperkalemia
- Dehydration
Signs and symptoms for Addison’s disease usually develop gradually and are frequently vague or ambiguous:
- Weight loss
- Muscle weakness
- Fatigue
- Nausea
- Low blood pressure
- May present with hypoglycemia
There is one sign that is specific for Addison’s disease. This sign is darkening of the skin, especially in oral mucosa and areas where the skin folds (gum-line, hands, elbows, knees). The darkening is caused by high levels of accumulated adrenocorticotropic hormone (ACTH). ACTH binds with melanocortin1 receptors on the surface of dermal melanocytes located within these areas of the body.
Prehospital management for Addison’s disease is supportive care. The patient may require IV/oral dextrose for hypoglycemia and/or fluids for hypotension. The replacement of cortisol is generally not required for patients outside of an Addisonian crisis.
Addisonian Crisis
Addisonian crisis is a life-threatening progression of Addison’s disease. Up to 25% of Addison’s patients may initially present in Addisonian crisis.2 It is frequently precipitated by an interrelated problem such as infection, trauma, or stress. Addisonian crisis accounts for up to 15% of deaths in patients with Addison’s disease.2
Signs and symptoms of Addisonian crisis are a little more specific:
- Sudden penetrating pain in large muscle groups of the legs, lower back, or abdomen
- Severe nausea, vomiting, and diarrhea
- Hypotension
- Syncope
- Hypoglycemia
- Seizure
- Fever
- Cardiac dysrhythmias
- Hyperkalemia
Rapid and decisive management of Addisonian crisis is critical for the EMS provider. Management starts with associating that an Addison’s patient has progressed into Addisonian crisis. From there treatment should progress in the following manner:
- Good BLS airway management is critical
- Aggressive IV bolus therapy of normal saline to replace lost fluid and sodium (minimum of 20mL/kg)
- May require multiple doses of dextrose to manage hypoglycemia
- If possible, administer a steroid to replace lost cortisol. Solu-Cortef is the steroid of choice, this is because it possesses the highest level of mineralocorticoid activity when compared to steroids such as Solu-Medrol and Dexamethasone.3 As a side note, Solu-Medrol or Dexamethasone can be administered if Solu-Cortef is unavailable.
- Manage any cardiac dysrhythmias
- If possible, identify and manage hyperkalemia (not covered within this article)
Endocrine Case #2
Called to a private residence at 2030. Upon arrival you find a 54-year-old male lying in a recliner chair complaining of lower back pain.
SAMPLE history reveals:
S = As provided
O = Pain started 1 hour ago after attempting to get out of the recliner.
P = Aggravated by attempting to get out of the recliner, as well as any other movement
Q = Pain is fluctuating between a sharp throb and dull ache
R = Pain radiates down both buttocks.
S = Pain is 7 on a scale of 0-10
T = Pain has been getting worse since it started
A = No known allergies
M = Salmeterol, prednisone, albuterol, hydrochlorothiazide (HCTZ), potassium, metformin, home oxygen @ 3LPM
P = COPD, hypertension, and type 2 diabetes
L = Dinner at approximately 1800
E = Was attempting to get out of the recliner when back pain started
Physical assessment reveals expiratory rhonchi in upper lung fields, multiple purple striates across the abdomen, and increased pain to palpation in the lumbar region of the spine. Normal bilateral CMS of upper and lower extremities. Pupils are PERL. Skin is dry and appears extremely fragile.
Vital signs:
B/P = 148/102
Pulse = 80 and regular
Respirations = 16 and regular
Pulse oximetry = 95% on 3Lpm via nasal cannula
BGL = 156mg/dL
The patient was pivot assisted onto the stretcher with minimal assistance. During transport the patient reported his pain increased to nine. The patient was delivered to the hospital reporting pain at a 10.
This patient suffered a lumbar fracture while attempting to get out of the recliner. The patient later admitted to taking prednisone for 4-6 consecutive months because it made him feel better and breathe easier. He underwent surgery for a lumbar fusion. Weaning off steroids was initiated, and he was released six days after surgery.
Cushing’s Syndrome
Cushing’s syndrome refers to excess cortisol of any etiology. The disease affects approximately six in 100,000 people.3 Cushing’s syndrome is commonly associated with an iatrogenic overuse and/or abuse of steroid medications. Iatrogenic refers to a condition caused by medical treatment. Cushing’s syndrome can also be caused by tumors.
Cushing’s syndrome has multiple effects on the metabolism of bones. Elevated cortisol levels block absorption of calcium, inhibiting bone cell growth. As a result, patients suffering from Cushing’s syndrome may experience lower bone density. This increases the risk of pathologic bone fractures when compared to healthy people.
Common signs and symptoms associated with Cushing’s syndrome:
- Central obesity (Obese torso with excessively thin extremities)
- Thin frail skin
- Purple or red striates on the trunk, buttocks, arms, legs, or breasts
Prehospital considerations in patients with Cushing’s syndrome should revolve around the increased potential for pathologic bone fractures. It is important to note that pathological fractures are rarely unstable, and the outcome illustrated in case study #2 is unusual. None the less, it is prudent to approach any orthopedic related pain in a suspected Cushing’s syndrome patient as a potential fracture. This management should include appropriate splinting.
Endocrine Case #3
Called to a private residence at 0030. Upon arrival you find a 39-year-old female lying semi-fowler in bed complaining of weakness and shortness of breath.
SAMPLE history reveals:
S = Shortness of breath
O = Noticed difficulty breathing about two days ago
P = Physical activity exacerbates the difficulty breathing
Q = Patient describes the difficulty breathing as inability to catch her breath
R = Experiencing intermittent fluttering in her chest
S = Never experienced SOB
T = Has been getting worse over the past day
S = Weakness
O = Weakness started a month ago
P = Post exercise exacerbates the weakness
Q = Patient describes the weakness as a full body “listlessness”
R = Not affecting any other symptoms
S = Never experienced this feeling before
T = Has been getting worse over the past month
A = No known allergies
M = Methotrexate, Enbrel, insulin, on her last day of a prednisone taper
P = Prednisone is for rheumatoid arthritis, Type 1 diabetes
L = Dinner at approximately 1730
E = Was awakened by the difficulty breathing
Physical assessment reveals possible inspiratory rales in the anterior lung fields. The patient is thin, but not emaciated. The patient states she has been unintentionally losing weight. Pupils are PERL. Skin is “clammy” but not diaphoretic.
Vital signs:
B/P = 102/64
Pulse = 140 and irregular
Respirations = 24 regular and shallow
Pulse oximetry = 89% on room air
BGL = 107mg/dL
ECG = Atrial fibrillation, the patient has never been diagnosed with and dysrhythmia
The patient’s pulse oximetry increased to 96% with a nasal cannula oxygen at 4 LPM. Approximately 750 mL of normal saline was infused. The patient reported a slight increase in difficulty breathing during transport. Considered a breathing treatment, but transport time prohibited administration. The patient was delivered to the hospital complaining of increased difficulty breathing.
This patient was ultimately diagnosed with Class III heart failure. In retrospect, this patient did not receive appropriate prehospital care. Unfortunately, the patient’s increased difficulty breathing was most probably due to fluid overload from IV fluid bolus. Fortunately, the patient arrived at the hospital prior receiving a breathing treatment which may have further exacerbated her heart failure. The patient underwent unelective cardioversion due to poor rate control with medications. Cardiac function improved after the rate was controlled. She was expected to improve with a questionable outcome. As a side note, her mother had Graves’ disease.
Graves’ Disease
Graves’ disease is a form of hyperthyroidism. It affects approximately 1 in 00 people.3 Graves’ disease most commonly occurs among women under age 40 but can occur at any age. Occurrence is more common when there is a family history of Graves’ disease. People with other autoimmune disorders are also more likely to develop Graves’ disease. It is the most common cause of hyperthyroidism in the U.S.4
Here are generalized signs and symptoms for Graves’ disease:
- Anxiety and irritability
- Increase in perspiration resulting in clammy skin
- Fine tremor in hands or fingers
- Heat intolerance
- Tiredness or muscle weakness
- Weight loss
- Frequent bowel movements
There are three signs that are classic for Graves’ disease:6
- Rapid and/or irregular heart rhythm
- Goiter
- Graves’ ophthalmopathy which involves
- Bulging of the eyes
- Gritty sensation in eyes
- Pressure or pain in eyes
- Puffy or retracted eyelids
- Light sensitivity
- Vision impairment
If left untreated, Graves’ Disease can lead to the following conditions:
- Atrial fibrillation
- Stroke
- Heart failure
- Vision loss from Graves’ ophthalmopathy
- Pathologic fractures due to osteoporosis
Prehospital management for Graves’ disease starts with identifying key clinical indicators:
- Existing autoimmune disease
- Family history of Graves’
- Unexplained weight loss
- Heat intolerance
- “Clammy” skin
- Tachycardia
- Goiter
- Graves’ ophthalmopathy
If Graves’ disease is suspected, it’s important for the EMS provider to rule in or rule out the following conditions:
- Stroke or other neurologic injuries
- Rate related cardiac dysfunction
- Heart failure
- Orthopedic pain that may be a pathologic fracture
Summary
Although Addison’s disease, Cushing’s syndrome and Graves’ disease are not commonly seen in the prehospital environment. These patients can be identified by their unique physical manifestations and associated histories. Once identified, it is important for the EMS provider consider the clinical implications so the patient may receive the most effective treatment before arriving at the hospital.
References
1. Nieman L, Turner M. Addison’s disease. Clinics in Dermatology. July-August 2006;24(4):276-280.
2. Wouter WW, van der Lely AL. Addisonian crisis and relative adrenal failure. Endocrine & Metabolic Disorders. 2003;4:143-147.
3. Kim SY, Endocrine and metabolic emergencies in children; hypocalcemia, hypoglycemia, adrenal insufficiency, and metabolic acidosis including diabetic ketoacidosis. Annals of Pediatric Endocrinology and Metabolism. December 2015;20(4);179-186,
4. Cooper GS, Stroehla BC. The epidemiology of autoimmune diseases. Autoimmunity Reviews. 2003;2(3):119-125.
5. Ross EJ, Linch DC. Cushing’s syndrome killing disease: discriminatory value of signs and symptoms aiding early diagnosis. The Lancet. 1982;320(8299):646-649.
6. Orth DN. Cushing’s syndrome. The New England Journal of Medicine. 1995;332:791-803.
7. Brent GA. Graves’ disease. The New England Journal of Medicine. 2008;358:2594-2605.
8. Bahn RS. Graves; ophthalmopathy. The New England Journal of Medicine. 2010;362:726-738.