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Section 6
Chapter 18:
Diabetic Emergencies
CWI AEMT Course
Robert S. Cole
Endocrine Glands
 Controls many body functions
› exerts control by releasing special chemical
substances into the blood called hormones
› Hormones affect other endocrine glands or
body systems
 Ductless glands
 Secrete hormones directly into
bloodstream
› Hormones are quickly distributed by
bloodstream throughout the body
Hormones
 Chemicals produced by endocrine
glands
 Act on target organs elsewhere in body
 Control/coordinate widespread
processes:
• Homeostasis
• Reproduction
• Growth & Development
• Metabolism
• Response to stress
• Overlaps with the Sympathetic Nervous System
Hormones
 Hormones are classified as:
› Proteins
› Polypeptides (amino acid derivatives)
› Lipids (fatty acid derivatives or steroids)
Hormones
 Amount of hormone reaching target
tissue directly correlates with
concentration of hormone in blood.
› Constant level hormones
 Thyroid hormones
› Variable level hormones
 Epinephrine (adrenaline) release
 Insulin
› Cyclic level hormones
 Reproductive hormones
The Endocrine System
 Consists of several glands located in
various parts of the body
 Specific Glands
› Hypothalamus
› Pituitary
› Thyroid
› Parathyroid
› Adrenal
› Kidneys
› Pancreatic Islets
› Ovaries
› Testes
Endocrine Disorders
and Emergencies
 Primarily consist of:
› Disorders of the Pancreas
› Disorders of the Thyroid Gland
› Disorders of the Adrenal Glands
The Endocrine System is the control mechanism for the
autonomic functions of the body.
Disorders of the Pancreas
What is the Pancreas?
 The pancreas is a dual purpose gland organ in
the digestive system and endocrine system of
vertebrates.
 It is both an endocrine gland producing several
important hormones, including insulin,
glucagon, somatostatin, and pancreatic
polypeptide, and a digestive organ, secreting
pancreatic juice containing digestive enzymes
that assist the absorption of nutrients and the
digestion in the small intestine.
 These enzymes help to further break down the
carbohydrates, proteins, and lipids in the
chyme.
The Pancreas
Pancreas
 Produces 3 types of cells:
› Alpha (α)
› Beta (β)
› Delta (δ)
Alpha Cells (α)
 Alpha () cells release glucagon to RAISE
blood glucose level and stimulate
glycogenolysis
› When blood glucose levels fall,  cells  the
amount of glucagon in the blood
› The surge of glucagon stimulates liver to release
glucose stores by the breakdown of glycogen
into glucose (glycogenolysis)
› Also, glucagon stimulates the liver to produce
glucose (gluconeogenesis)
Beta Cells (β)
 Stimulate the release of insulin to
Lower the blood glucose level
 Beta Cells () release insulin (antagonistic to
glucagon) to control blood glucose level
› Insulin  the rate at which various body cells take
up glucose  insulin lowers the blood glucose
level
› Promotes glycogenesis - storage of glycogen in
the liver
› Insulin is rapidly broken down by the liver and
must be secreted constantly
Delta Cells (δ)
 Delta Cells () produce somatostatin, which
inhibits both glucagon and insulin
› inhibits insulin and glucagon secretion by the
pancreas
› inhibits digestion by inhibiting secretion of
digestive enzymes
› inhibits gastric motility
› inhibits absorption of glucose in the intestine
The Role of Glucose and Insulin
 Glucose is a major source of energy for
the body.
 Insulin is needed to allow glucose to
enter cells (except for brain cells).
› A “cellular key”
The Role of Glucose and Insulin
The Role of Glucose and Insulin
 When glucose is unavailable, the body
turns to other energy sources.
› Fat is most abundant.
› Using fat for energy results in buildup of
ketones and fatty acids in blood and tissue.
The Role of Glucose and Insulin
 Diabetic ketoacidosis (DKA)
› A form of acidosis seen in uncontrolled
diabetes
› Without insulin, certain acids accumulate.
› More common in type 1 diabetes
› Signs and symptoms:
 Weakness
 Nausea
Diabetes
 Diabetes affects the body’s ability to
use glucose (sugar) for fuel.
 Occurs in about 7% of the population
 Complications include blindness,
cardiovascular disease, and kidney
failure.
The central problem in diabetes is lack, or ineffective
action, or resistance to, of insulin.
Hypoglycemia, the most common diabetic emergency
seen in EMS, is simply the result of over-correction of this
problem, the result of loss of balance in the endocrine
system, but is not the key problem with diabetes.
Diabetes
 As an EMT, you need to know signs and
symptoms of blood glucose that is:
› High (hyperglycemia)
› Low (hypoglycemia)
 Central problem in diabetes is lack, or
ineffective action, of insulin.
Types of Diabetes Mellitus
 Type I
 Type II
 Secondary
 Gestational
The term “Insulin Dependent” and “non-Insulin
Dependent” are no longer accurate definitions of Type I
and Type II Diabetes.
Type I diabetics are now being occasionally prescribed
anti-hyperglycemics as well as insulin; and Type 2
diabetics are (occasionally) being prescribed insulin in
addition to their oral meds.
Type 1 Diabetes Mellitus
 Type 1 patients do not produce insulin at
all.
› Need daily injections of insulin
› Typically develops during childhood
› Patients more likely to have metabolic
problems and organ damage
› Considered an autoimmune problem
Type 1 Diabetes
 More than 90% of children with diabetes
suffer from Type 1 diabetes (previously
known as insulin-dependent diabetes).
 The body loses the ability to make insulin.
This occurs when the immune system
destroys the insulin-producing cells.
 As a result of this attack, these cells stop
making insulin over time.
Type 2 Diabetes Insipidus
 Type 2 patients produce inadequate
amounts of insulin, or normal amount
that does not function effectively.
› Usually appears later in life
› Treatment may be diet, exercise, oral
medications, or insulin.
Key Point:
 Type 1 and type 2 diabetes both:
› Are equally serious
› Affect many tissues and functions
› Require life-long management.
New Onset Diabetes?
 About 25% of new onset Diabetes (Type
I) are discovered due to a DKA type
event.
 Type 2 Diabetes, by contrast is typically
discovered during health screenings or
other routine medical evaluations.
 This DOES NOT IMPLY that Type 2 is less
severe than Type 1.
› Both can be fatal if untreated/unrecognized.
Types of Diabetes
 Secondary Diabetes : Pre-existing
condition affects pancreas and its function
› Pancreatitis
› Trauma
› Shock States
› Cystic Fibrosis
 Occurs during pregnancy
› Usually resolves after delivery
Types of Diabetes
 Definition: Onset of diabetes with pregnancy.
 Most women need two to three times more insulin
when they are pregnant than they usually do.
 In gestational diabetes, there are often no warning
symptoms. All pregnant women need to be tested
for diabetes during the second trimester. This is
especially important for women who are already at
risk.
 After the baby is born, blood glucose levels usually
return to normal. A woman who has had gestational
diabetes is at risk for developing type 2 diabetes
later in life.
 No Pregnant?
› Occurs rarely in non-pregnant women on BCPs
› Increased estrogen, progesterone from BCPs
antagonize endogenous insulin
New Onset Diabetes?
 Classic symptoms of uncontrolled
diabetes (“3 Ps”):
› Polyuria: frequent, plentiful urination
› Polydipsia: frequent drinking to satisfy
continuous thirst
› Polyphagia: excessive eating
 This is why we check a Bgon 90% of IV
starts….
Monitoring Blood Glucose
Source: Accu-Chek® Aviva used with permission of Roche Diagnostics.
Blood glucose monitoring kit
Monitoring Blood Glucose
 Perhaps the single most important factor
in a diabetics health is how well they
monitor their own BG.
 Severity of diabetic complications
depends on patient’s average blood
glucose level.
Glucose Monitors Facts
 Personal use: Calibrated Monthly (rare)
 For EMS use: Calibrated WEEKLY
 Most have a chip which need to be
changed with EVRY BOTTLE OF NEW
STRIPS
 Affected by temp
 Affected by time (samples must be
applied w/in 30 –60 seconds)
Blood Glucose Monitoring
 Should be done daily or more often.
 When done by EMS, should be done in
the opposite ext. as D50 was given.
 Venous blood usually runs about 10
mg/dl higher than capillary blood.
Checking the Blood Glucose
Level (BG)
› Glucometers are commonly found on EMS units.
› Determines the amount of glucose in the blood,
the sample usually coming from a finger stick.
› Glucose is measured in milligrams per deciliter
(mg/dl).
› A normal range is 80-120 mg/dl.
› Hypoglycemia is a BGL <60 mg/dl.
› Hyperglycemia is a BGL >150 mg/dl.
 Typically not significant until greater than 250. Often not
symptomatic until greater than 400 (not always true)
Benefit Of Frequent Testing
Breakfast
100 (5.6)
200 (11)
400 (22)
300 (17)
DinnerLunch Bed
1 test versus 7 tests a day
Testing Frequency
 In a Kaiser study of actual prescription
fulfillment among 44,181 patients with
diabetes:
› 60% of Type 1s were not testing 3-
4 times a day as recommended
by the ADA
› 67% of Type 2s were not testing
once a day as recommended by
the ADA
Diabetes Care 23:477-483, 2000
Why Aren’t People Testing?
 Lack of understanding
 No direct /immediate reward/benefit
 No mechanism for long-term benefit
 No link to cause of BG problems
 Finger-pricking required (NOT FUN)
 No guidance for lowering highs
 No easy way to record other things
$2,000 a year for 7 x a day
testing
LOG BOOKS:
 Can be very useful in reviewing a patients
control
What are the numbers?
 Low: <60mg/dl (<3.0 mmol/L)
 Normal: 60-150mg/dl (3.0-8.0 mmol/L)
› Some recommend 120 mg/dl
 Hyperglycemic: >150 (>8.0 mmol/L)
 DKA usually seen at 250-500mg/dl
mmol/L vs. mg/dl
 What is an MMOL?
 It is a unit of measurement commonly used
in chemistry based on the molecular weight
of the substance it pertains to.
 To convert mmol/l of glucose to mg/dl,
multiply by 18. To convert mg/dl of glucose
to mmol/l, divide by 18 or multiply by 0.055.
Hemoglobin A1c
 Hg A1c is a 3 month Average of blood glucose control
 In the normal 120-day lifespan of the red blood cell, glucose
molecules react with hemoglobin,
forming glycated hemoglobin. In individuals with poorly
controlled diabetes, the quantities of these glycated
hemoglobins are much higher than in healthy people.
 Once a hemoglobin molecule is glycated, it remains that
way. A buildup of glycated hemoglobin within the red cell,
therefore, reflects the average level of glucose to which the
cell has been exposed during its life-cycle. Measuring
glycated hemoglobin assesses the effectiveness of therapy
by monitoring long-term serum glucose regulation. The
HbA1c level is proportional to average blood glucose
concentration over the previous four weeks to three months.
 Some researchers state that the major proportion of its value
is related to a rather shorter period of two to four weeks.
A1c: the Numbers
 In general, the normal range (that found in healthy
persons), is about 4%–5.9%
 The 2010 American Diabetes Association added
the A1c ≥ 6.5% as another criterion for the diagnosis
of diabetes, but this is controversial and has not
been universally adopted.
 Target criteria for DM is typically 6-7%
 In diabetes , higher amounts of glycated
hemoglobin, indicating poorer control of blood
glucose levels, have been associated with
cardiovascular disease, nephropathy,
and retinopathy.
 Monitoring the HbA1c in type-1 diabetic patients
may improve treatment.
Common Diabetic Emergencies
Hyperglycemia and
Hypoglycemia
 Both lead to diabetic emergencies.
 Hyperglycemia: Blood glucose is above
normal.
› Result of lack of insulin
› Untreated, results in DKA
 Hypoglycemia: Blood glucose is below normal.
› Untreated, results in unresponsiveness and
hypoglycemic crisis
 Signs and symptoms of hyperglycemia and
hypoglycemia are similar if your assessment is
shoddy…..
Hypoglycemia
 “Looks Shocky” used to be called Insulin
shock. Pale, diaphoretic, altered mental
tatus. May Vomit.
 BG <60mg/dl
 Reality is this is a hypoglycemic state, not
a shock state.
Hypoglycemia:
Definitions
 “Mild”: Adrenergic (BG<60) (<4mmol)
 “Moderate”: Cognitive (BG<50) (<3mmol)
 “Severe”: Neurologic Unconscious (BG
???)
Hypoglycemia
 More common with Type I diabetics
 Most common complication of diabetes
mellitus seen by EMS
› Most common cause of coma in the diabetic
patient
Common Causes
 Common history is taking insulin:
› And then not eating a meal
› Eating a meal, but having heavier exertion than
normal
› Taking too much insulin and depleting the
glucose levels in the body
 Also caused by Medication Interactions
› Beta Blockers (High Blood Pressure Meds)
 Change in exercise
 Recent illness
Hypoglycemia
Treatment for Hypoglycemia
 Food (simple and Complex Carbs)
 Oral Glucose
 IV Dextrose
 Glucagon
Carbs!!!!!
Oral Glucose
 Class: Simple Carbohydrate
 MOA: A heavy sugar gel that is absorbed across the mucosal
membranes of the mouth and the GI tract, and enters the
blood stream.
› Increases the BGL by providing sugar available for the
brain.
 Indications: Only administer if the following criteria are met
(all three must be met):
 The patient has an altered mental status.
 There is a history of diabetes controlled by medications.
 The patient is responsive enough to swallow and control
their own airway.
 DOSE: 15-45 GM’s PO
IV Dextrose
 IV Dextrose (D50)
12.5-50 GM SIVP
 Consider mixing in a
bag of 250cc and
run in.
 Always administer
slowly….
Glucagon
 MOA: Enzyme that
promotes/stimulates gylcogenisis
 Works opposite of insulin.
› Primary action is to raise the BGL if
it becomes lower than normal
(<60mg/dl).
› Helps the liver to release stored
glycogen back into the
bloodstream, where it is again
restored to the simple sugar
glucose (during a process called
glycogenolysis).
 DOSE: Glucagon 1-5 units IV or IM.
(Uses stores in liver)
› Sometimes patients have their own
glucagon.
 Route: IM or occasionally IV 1-5 units
OK…. their awake, NOW What?
 When awake: f/u with complex
carbohydrates (food)
 Ensure that someone will be with the pt.
 Assess for and treat hypothermia
(common)
 R/O other problems (trauma). Pt must be
fully CA&O.
 Consider T/R if desired after contact with
medical control or per protocol
Releasing the (formerly Hypoglycemic)
Diabetic
The Ada County Protocol:
“APPENDIX V: TREAT AND RELEASE
CHECK SHEET FOR HYPOGLYCEMIC
PATIENTS.”
11 Questions:
 1. Is there a clear reason for the hypoglycemic episode?
 2. Is the patient alert and oriented?
 3. Is the patient’s repeat BG above 80 mg/dl?
 4. Has the patient’s BG been well-controlled prior to this
episode?
 5. Is the patient able to eat a complex carbohydrate meal?
 6. Does the patient have regular, on-going physician care?
 7. Is the patient comfortable with non-transport?
 8. Is the patient/guardian willing to sign a release form?
 9. Is there another responsible person with the patient?
 10. Is the patient’s temperature within normal limits? (95° to
100.4° Fahrenheit)
 11. The patient is free of the influence of alcohol or other CNS-
altering drugs?
KEY POINTS:
 Hypoglycemia is an Altered LOC (ALOC)
emergency until proven to be
hypoglycemia.
› In other words, don’t get surprised by other
causes, i.e. CVA, Alcohol, drugs, etc
 Patent issued
September 14, 1971
for the
Ames Reflectance
Meter
 More physiologic insulin
delivery to mimic the
pancreas
 Basal: steady background
insulin delivery to keep BG
from rising while fasting
 Bolus: spurts of insulin to
cover carbs or lower high
BGs
 Liberalization of diet —
timing & amount (by user)
 Increased control with
exercise (by user)
 Able to work shifts & through
lunch (by user)
 Less hassle with travel and
time zones (by user)
 Aid to weight control (by user)
 Less anxiety in trying to keep on schedule (by
user)
•55.0
29.8
•23.9
•5.1
•13.413.0
7.9
16.4
5.02.5
0
10
20
30
40
50
60
Retinopathy
Progression
Laser Rx1 Micro-
albuminuria2
Albuminuria2
Clinical
Neuropathy3
Conventional
Intensive
76%
Risk Reduction
59%
Risk Reduction
39%
Risk Reduction
54%
Risk Reduction
64%
Risk Reduction
CumulativeIncidence(%)
1. DCCT Research Group, Ophthalmology. 1995;102:647-661
2. DCCT Research Group, Kidney Int. 1995;47:1703-1720
3. DCCT Research Group. Ann Intern Med. 1995;122:561-568.
 Carb boluses
 Accuracy improved with personal
carb factor adjusted for different
times of day
 Personal carb database
 Correction boluses
 Personalized correction factors for different
times of day
 Safer correction of high BGs
 Reports amount of correction bolus used (ie,
over 8% of TDD)
An accurate TDD --> accurate basals & boluses
Each manufacturer provides bolus dose recommendations differently.
 As bolus size
increases,
so too does
duration of
action.
Woodworth et al. Diabetes. 1993;42(Suppl. 1):54A
 Bolus On Board (BOB)
› Discounts bolus for residual
BOB
› Improves accuracy
› Avoids stacking of bolus
insulin
› Acts as guide to whether
carbs or insulin are needed,
ie, BG is 130 mg/dl but
BOB = 5 u
 Requires a blood sugar test, an
accurate duration of insulin
action, and BG targetsPrevents hypoglycemia!
 Reminders (alarms) to
› test glucose following a
bolus
› test glucose after a low
reading
› test glucose after a high
reading
› give a bolus at certain time
of day
› warn when bolus delivery
was not completed, etc.
› change infusion site
108
 Direct glucose entry into database eliminates
data errors and offers optimum use of
glucose/insulin data
• AccuChek Spirit + meter
• CozMore System +
Therasense CoZmonitor
• Soill Diabecare III pump +
meter
• Medtronic 515/715 +
BD Paradigm Link
• Soon: Animas + ? Lifescan
or Glucowatch
 A watch type device worn for about 13
hours/day.
 Uses a disposable pad and minute
bioelectric charge to read glucose
readings as often as every 10 minutes.
 Is effected by sweat (poor readings)
 Is a supplement to (not a replacement for )
normal glucometer readings.
 Downloadable info.
 Real time readings
 Add-on to routine BG
monitoring
 Radio communication
from sensor to monitor
 High and low glucose
alarms
 FDA pending
Caution: Investigational Device.Limited by U.S. Law to Investigational Use
 Silicon microneedles can be used to infuse insulin or allow
glucose measurements in interstitial fluid. This needle array
could replace the AutoSensor in a GlucoWatch for effective
continuous monitoring.
Caution: Investigational Device.Limited by U.S. Law to Investigational Use
Meter replacement
 Open-flow
Microperfusion System
 Inserted into the
subcutaneous adipose
tissue
 Double lumen catheter
 Acquires glucose
readings every 30
minutes
 Goal – subcutaneous
glucose sensing/insulin
delivery system
An intelligent pump would
alert the user when the BG
is likely to cross a selected
threshold value, such as
120 mg/dl (6.7 mmol).
This safe meal delay
reduces glucose exposure,
especially when combined
with a Super Bolus
Max. drop ~4-5 mg/dl per min
 Generally speaking, Insulin used in insulin
pumps should not be used for standard
injection!
 Most insulin pump pt’s only take one
type of insulin (fast acting)
 When encountering a pump in a
hypoglycemic patient, do not try to
adjust it!
› Remove it from the patient.
 Usually presents as either DKA or HHNC
› DKA more common to Type I diabetics
› HHNC more common to Type II diabetics
 Both syndromes have elevated BGL in the
body.
 Altered physiology leads to dehydration
and acidosis.
› Usually a slower onset than that of
hypoglycemic episodes.
 Also known as
“Diabetic Coma”
 DKA usually > 350
mg/dl
(occasionally as
low as 250 mg/dl)
 More common in
type I diabetics
› More common in
the 40’s (can be
seen at all ages)
 The body in an attempt to
compensate for
hyperglycemia, develops an
acidosis state.
 Dehydration
 Vomiting
 Loss of weight
 Kussmaul respirations
 Acetone smell
 Impaired sensorium
 Shock
(hypovolemic/dehydration
)
 Oxygen, Airway Control, Rapid Transport
 ALS Intercept!
› EKG (look out for hyperK)
› IV fluids (may require 4-6 liters over time) Monitor
B/P.
› Recheck BG
 Hospital Care:
› Insulin
› ICU Admission
 Hyperglycemic Hyper-Osmolar Non-ketotic Coma
 Only 10% actually severe enough for coma
 HHNC usually > 600 mg/dl
 More common in type 2 diabetics
 Slightly more common than DKA
 More common in very elderly (can be seen at all
ages)
 Some insulin still being produced is enough to prevent
Lactic metabolism. Thus no Ketones and no acidosis.
 Just profound dehydration.
 Treated with lots of saline as well as glycemic correction
 High chance of severe electrolyte imbalances.
 Mortality 10-20%
One third of patients with HHNC do
not have a prior (known) history of
diabetes.
-Emedicine.com
 Protect the ABC’s
 High Flow O2
 Call ALS
 Rapid Transport
 Look for other causes also.
 All BLS care AND :
› 2 Large Bore IV’s
› Fluid Resuscitation
 Watch for fluid overload though
› Shock management
› Airway Management
 The classical symptoms of
child presenting with (new
onset) Type 1 diabetes:
› increasing thirst
› increased drinking and
› polyuria for some time
(May start having
accidents)
› Weight Loss
› Abd Pain
› Tired and listless
 More Severe complaints:
› Vomiting
› Altered Mental Status
› Kussmal Respirations
› Coma
› Profound dehydration
 25-40% go untreated long enough to develop DKA
 5-10% in diabetic coma from DKA.
 The condition is severe and is still the major cause of
death in children with diabetes.
 Consider abuse and/or neglect.
Hypoglycemia
 “Insulin Shock”
 BG <60
 They Look Shocky!
 Pale, Diaphoretic,
 HR is normal or mildly
increased
 BP typically normal.
 Breathing is shallow
 Sudden Onset
 No real shock
Hyperglycemia: DKA &
HHNC
 “Diabetic Coma”
 Deep Respirations and fruity
Breath (DKA)
 BP decreased
 HR is very increased (120-150 or
more)
 Hypotension and severe
dehydration
 Very Dry skin, Flushed or pale.
 Slower onset.
 Real Shock
 Infection
› Especially on feet and hands
 Atypical Heart Attacks
 Strokes
 Renal Failure
 Hypertension
 Poor recovery from major insults to the
body, prolonged healing.
 After you obtain a good grasp of various
diabetic conditions, you may want to
learn about:
› Graves Disease
› Addison's Disease
› Thyrotoxic Crisis (Thyroid Storm)
› Hypothyroidism
› Cushing's Syndrome
 (not Cushing's response, which is different)
 The endocrine system maintains stability
in the body’s internal environment
(homeostasis).
 Type 1 and type 2 diabetes involve
abnormalities in the body’s ability to use
glucose (sugar) for fuel.
 Polyuria (frequent, plentiful urination),
polydipsia (frequent drinking to satisfy
continuous thirst), and polyphagia
(excessive eating due to cellular
hunger) are common symptoms, or the
“3 Ps,” of uncontrolled diabetes.
 Patients with diabetes have chronic
complications that place them at risk for
other diseases.
 Hyperglycemia is the result of a lack of
insulin, causing high blood glucose
levels.
 Hypoglycemia is a state in which the
blood glucose level is below normal.
Without treatment, permanent brain
damage and death can occur.
 DKA is the buildup of ketones and fatty
acids in the blood and body tissue that
results when the body relies upon fat for
energy.
 Hyperglycemic crisis (diabetic coma) is
a state of unconsciousness resulting
from DKA, hyperglycemia, and/or
dehydration due to excessive urination.
 Hypoglycemic crisis (insulin shock) is
caused by insufficient blood glucose
levels. Treat quickly, by giving oral
glucose (if protocols allow), to avoid
brain damage.
 When assessing diabetic emergencies,
err on the side of giving oral glucose (if
protocols allow). Do not give oral
glucose to patients who are
unconscious or who cannot swallow
properly and protect the airway. In all
cases, provide rapid transport.
 Problems associated with diabetes
include seizures, altered mental status,
“intoxicated” appearance, and loss of
a gag reflex, which affects airway
management.
 Hematology is the study and prevention
of blood-related disorders.
 Sickle cell disease is a blood disorder the
affects the shape of red blood cells.
Symptoms include joint pain, fever,
respiratory distress, and abdominal pain.
 Hemoglobin A is considered normal
hemoglobin. Hemoglobin S is
considered an abnormal type of
hemoglobin and is responsible for sickle
cell crisis.
 Patients with sickle cell disease have
chronic complications that place them
at risk for other diseases, such as heart
attack, stroke, and infection.
 Patients with hemophilia are not able to
control bleeding.
 Emergency care in the prehospital
setting is supportive for patients with
sickle disease or a clotting disorder such
as hemophilia.
2013 aemt chpt 18 Diabetes

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2013 aemt chpt 18 Diabetes

  • 1. Section 6 Chapter 18: Diabetic Emergencies CWI AEMT Course Robert S. Cole
  • 2. Endocrine Glands  Controls many body functions › exerts control by releasing special chemical substances into the blood called hormones › Hormones affect other endocrine glands or body systems  Ductless glands  Secrete hormones directly into bloodstream › Hormones are quickly distributed by bloodstream throughout the body
  • 3. Hormones  Chemicals produced by endocrine glands  Act on target organs elsewhere in body  Control/coordinate widespread processes: • Homeostasis • Reproduction • Growth & Development • Metabolism • Response to stress • Overlaps with the Sympathetic Nervous System
  • 4. Hormones  Hormones are classified as: › Proteins › Polypeptides (amino acid derivatives) › Lipids (fatty acid derivatives or steroids)
  • 5. Hormones  Amount of hormone reaching target tissue directly correlates with concentration of hormone in blood. › Constant level hormones  Thyroid hormones › Variable level hormones  Epinephrine (adrenaline) release  Insulin › Cyclic level hormones  Reproductive hormones
  • 6. The Endocrine System  Consists of several glands located in various parts of the body  Specific Glands › Hypothalamus › Pituitary › Thyroid › Parathyroid › Adrenal › Kidneys › Pancreatic Islets › Ovaries › Testes
  • 7. Endocrine Disorders and Emergencies  Primarily consist of: › Disorders of the Pancreas › Disorders of the Thyroid Gland › Disorders of the Adrenal Glands
  • 8. The Endocrine System is the control mechanism for the autonomic functions of the body.
  • 9. Disorders of the Pancreas
  • 10. What is the Pancreas?  The pancreas is a dual purpose gland organ in the digestive system and endocrine system of vertebrates.  It is both an endocrine gland producing several important hormones, including insulin, glucagon, somatostatin, and pancreatic polypeptide, and a digestive organ, secreting pancreatic juice containing digestive enzymes that assist the absorption of nutrients and the digestion in the small intestine.  These enzymes help to further break down the carbohydrates, proteins, and lipids in the chyme.
  • 11.
  • 12.
  • 14. Pancreas  Produces 3 types of cells: › Alpha (α) › Beta (β) › Delta (δ)
  • 15. Alpha Cells (α)  Alpha () cells release glucagon to RAISE blood glucose level and stimulate glycogenolysis › When blood glucose levels fall,  cells  the amount of glucagon in the blood › The surge of glucagon stimulates liver to release glucose stores by the breakdown of glycogen into glucose (glycogenolysis) › Also, glucagon stimulates the liver to produce glucose (gluconeogenesis)
  • 16. Beta Cells (β)  Stimulate the release of insulin to Lower the blood glucose level  Beta Cells () release insulin (antagonistic to glucagon) to control blood glucose level › Insulin  the rate at which various body cells take up glucose  insulin lowers the blood glucose level › Promotes glycogenesis - storage of glycogen in the liver › Insulin is rapidly broken down by the liver and must be secreted constantly
  • 17. Delta Cells (δ)  Delta Cells () produce somatostatin, which inhibits both glucagon and insulin › inhibits insulin and glucagon secretion by the pancreas › inhibits digestion by inhibiting secretion of digestive enzymes › inhibits gastric motility › inhibits absorption of glucose in the intestine
  • 18. The Role of Glucose and Insulin  Glucose is a major source of energy for the body.  Insulin is needed to allow glucose to enter cells (except for brain cells). › A “cellular key”
  • 19. The Role of Glucose and Insulin
  • 20. The Role of Glucose and Insulin  When glucose is unavailable, the body turns to other energy sources. › Fat is most abundant. › Using fat for energy results in buildup of ketones and fatty acids in blood and tissue.
  • 21. The Role of Glucose and Insulin  Diabetic ketoacidosis (DKA) › A form of acidosis seen in uncontrolled diabetes › Without insulin, certain acids accumulate. › More common in type 1 diabetes › Signs and symptoms:  Weakness  Nausea
  • 22. Diabetes  Diabetes affects the body’s ability to use glucose (sugar) for fuel.  Occurs in about 7% of the population  Complications include blindness, cardiovascular disease, and kidney failure.
  • 23. The central problem in diabetes is lack, or ineffective action, or resistance to, of insulin. Hypoglycemia, the most common diabetic emergency seen in EMS, is simply the result of over-correction of this problem, the result of loss of balance in the endocrine system, but is not the key problem with diabetes.
  • 24. Diabetes  As an EMT, you need to know signs and symptoms of blood glucose that is: › High (hyperglycemia) › Low (hypoglycemia)  Central problem in diabetes is lack, or ineffective action, of insulin.
  • 25. Types of Diabetes Mellitus  Type I  Type II  Secondary  Gestational
  • 26. The term “Insulin Dependent” and “non-Insulin Dependent” are no longer accurate definitions of Type I and Type II Diabetes. Type I diabetics are now being occasionally prescribed anti-hyperglycemics as well as insulin; and Type 2 diabetics are (occasionally) being prescribed insulin in addition to their oral meds.
  • 27. Type 1 Diabetes Mellitus  Type 1 patients do not produce insulin at all. › Need daily injections of insulin › Typically develops during childhood › Patients more likely to have metabolic problems and organ damage › Considered an autoimmune problem
  • 28. Type 1 Diabetes  More than 90% of children with diabetes suffer from Type 1 diabetes (previously known as insulin-dependent diabetes).  The body loses the ability to make insulin. This occurs when the immune system destroys the insulin-producing cells.  As a result of this attack, these cells stop making insulin over time.
  • 29. Type 2 Diabetes Insipidus  Type 2 patients produce inadequate amounts of insulin, or normal amount that does not function effectively. › Usually appears later in life › Treatment may be diet, exercise, oral medications, or insulin.
  • 30. Key Point:  Type 1 and type 2 diabetes both: › Are equally serious › Affect many tissues and functions › Require life-long management.
  • 31. New Onset Diabetes?  About 25% of new onset Diabetes (Type I) are discovered due to a DKA type event.  Type 2 Diabetes, by contrast is typically discovered during health screenings or other routine medical evaluations.  This DOES NOT IMPLY that Type 2 is less severe than Type 1. › Both can be fatal if untreated/unrecognized.
  • 32. Types of Diabetes  Secondary Diabetes : Pre-existing condition affects pancreas and its function › Pancreatitis › Trauma › Shock States › Cystic Fibrosis  Occurs during pregnancy › Usually resolves after delivery
  • 33. Types of Diabetes  Definition: Onset of diabetes with pregnancy.  Most women need two to three times more insulin when they are pregnant than they usually do.  In gestational diabetes, there are often no warning symptoms. All pregnant women need to be tested for diabetes during the second trimester. This is especially important for women who are already at risk.  After the baby is born, blood glucose levels usually return to normal. A woman who has had gestational diabetes is at risk for developing type 2 diabetes later in life.  No Pregnant? › Occurs rarely in non-pregnant women on BCPs › Increased estrogen, progesterone from BCPs antagonize endogenous insulin
  • 34. New Onset Diabetes?  Classic symptoms of uncontrolled diabetes (“3 Ps”): › Polyuria: frequent, plentiful urination › Polydipsia: frequent drinking to satisfy continuous thirst › Polyphagia: excessive eating  This is why we check a Bgon 90% of IV starts….
  • 35. Monitoring Blood Glucose Source: Accu-Chek® Aviva used with permission of Roche Diagnostics. Blood glucose monitoring kit
  • 36. Monitoring Blood Glucose  Perhaps the single most important factor in a diabetics health is how well they monitor their own BG.  Severity of diabetic complications depends on patient’s average blood glucose level.
  • 37. Glucose Monitors Facts  Personal use: Calibrated Monthly (rare)  For EMS use: Calibrated WEEKLY  Most have a chip which need to be changed with EVRY BOTTLE OF NEW STRIPS  Affected by temp  Affected by time (samples must be applied w/in 30 –60 seconds)
  • 38. Blood Glucose Monitoring  Should be done daily or more often.  When done by EMS, should be done in the opposite ext. as D50 was given.  Venous blood usually runs about 10 mg/dl higher than capillary blood.
  • 39. Checking the Blood Glucose Level (BG) › Glucometers are commonly found on EMS units. › Determines the amount of glucose in the blood, the sample usually coming from a finger stick. › Glucose is measured in milligrams per deciliter (mg/dl). › A normal range is 80-120 mg/dl. › Hypoglycemia is a BGL <60 mg/dl. › Hyperglycemia is a BGL >150 mg/dl.  Typically not significant until greater than 250. Often not symptomatic until greater than 400 (not always true)
  • 40. Benefit Of Frequent Testing Breakfast 100 (5.6) 200 (11) 400 (22) 300 (17) DinnerLunch Bed 1 test versus 7 tests a day
  • 41. Testing Frequency  In a Kaiser study of actual prescription fulfillment among 44,181 patients with diabetes: › 60% of Type 1s were not testing 3- 4 times a day as recommended by the ADA › 67% of Type 2s were not testing once a day as recommended by the ADA Diabetes Care 23:477-483, 2000
  • 42. Why Aren’t People Testing?  Lack of understanding  No direct /immediate reward/benefit  No mechanism for long-term benefit  No link to cause of BG problems  Finger-pricking required (NOT FUN)  No guidance for lowering highs  No easy way to record other things $2,000 a year for 7 x a day testing
  • 43. LOG BOOKS:  Can be very useful in reviewing a patients control
  • 44. What are the numbers?  Low: <60mg/dl (<3.0 mmol/L)  Normal: 60-150mg/dl (3.0-8.0 mmol/L) › Some recommend 120 mg/dl  Hyperglycemic: >150 (>8.0 mmol/L)  DKA usually seen at 250-500mg/dl
  • 45. mmol/L vs. mg/dl  What is an MMOL?  It is a unit of measurement commonly used in chemistry based on the molecular weight of the substance it pertains to.  To convert mmol/l of glucose to mg/dl, multiply by 18. To convert mg/dl of glucose to mmol/l, divide by 18 or multiply by 0.055.
  • 46. Hemoglobin A1c  Hg A1c is a 3 month Average of blood glucose control  In the normal 120-day lifespan of the red blood cell, glucose molecules react with hemoglobin, forming glycated hemoglobin. In individuals with poorly controlled diabetes, the quantities of these glycated hemoglobins are much higher than in healthy people.  Once a hemoglobin molecule is glycated, it remains that way. A buildup of glycated hemoglobin within the red cell, therefore, reflects the average level of glucose to which the cell has been exposed during its life-cycle. Measuring glycated hemoglobin assesses the effectiveness of therapy by monitoring long-term serum glucose regulation. The HbA1c level is proportional to average blood glucose concentration over the previous four weeks to three months.  Some researchers state that the major proportion of its value is related to a rather shorter period of two to four weeks.
  • 47. A1c: the Numbers  In general, the normal range (that found in healthy persons), is about 4%–5.9%  The 2010 American Diabetes Association added the A1c ≥ 6.5% as another criterion for the diagnosis of diabetes, but this is controversial and has not been universally adopted.  Target criteria for DM is typically 6-7%  In diabetes , higher amounts of glycated hemoglobin, indicating poorer control of blood glucose levels, have been associated with cardiovascular disease, nephropathy, and retinopathy.  Monitoring the HbA1c in type-1 diabetic patients may improve treatment.
  • 49. Hyperglycemia and Hypoglycemia  Both lead to diabetic emergencies.  Hyperglycemia: Blood glucose is above normal. › Result of lack of insulin › Untreated, results in DKA  Hypoglycemia: Blood glucose is below normal. › Untreated, results in unresponsiveness and hypoglycemic crisis  Signs and symptoms of hyperglycemia and hypoglycemia are similar if your assessment is shoddy…..
  • 50. Hypoglycemia  “Looks Shocky” used to be called Insulin shock. Pale, diaphoretic, altered mental tatus. May Vomit.  BG <60mg/dl  Reality is this is a hypoglycemic state, not a shock state.
  • 51. Hypoglycemia: Definitions  “Mild”: Adrenergic (BG<60) (<4mmol)  “Moderate”: Cognitive (BG<50) (<3mmol)  “Severe”: Neurologic Unconscious (BG ???)
  • 52. Hypoglycemia  More common with Type I diabetics  Most common complication of diabetes mellitus seen by EMS › Most common cause of coma in the diabetic patient
  • 53. Common Causes  Common history is taking insulin: › And then not eating a meal › Eating a meal, but having heavier exertion than normal › Taking too much insulin and depleting the glucose levels in the body  Also caused by Medication Interactions › Beta Blockers (High Blood Pressure Meds)  Change in exercise  Recent illness
  • 55. Treatment for Hypoglycemia  Food (simple and Complex Carbs)  Oral Glucose  IV Dextrose  Glucagon
  • 57.
  • 58. Oral Glucose  Class: Simple Carbohydrate  MOA: A heavy sugar gel that is absorbed across the mucosal membranes of the mouth and the GI tract, and enters the blood stream. › Increases the BGL by providing sugar available for the brain.  Indications: Only administer if the following criteria are met (all three must be met):  The patient has an altered mental status.  There is a history of diabetes controlled by medications.  The patient is responsive enough to swallow and control their own airway.  DOSE: 15-45 GM’s PO
  • 59. IV Dextrose  IV Dextrose (D50) 12.5-50 GM SIVP  Consider mixing in a bag of 250cc and run in.  Always administer slowly….
  • 60. Glucagon  MOA: Enzyme that promotes/stimulates gylcogenisis  Works opposite of insulin. › Primary action is to raise the BGL if it becomes lower than normal (<60mg/dl). › Helps the liver to release stored glycogen back into the bloodstream, where it is again restored to the simple sugar glucose (during a process called glycogenolysis).  DOSE: Glucagon 1-5 units IV or IM. (Uses stores in liver) › Sometimes patients have their own glucagon.  Route: IM or occasionally IV 1-5 units
  • 61. OK…. their awake, NOW What?  When awake: f/u with complex carbohydrates (food)  Ensure that someone will be with the pt.  Assess for and treat hypothermia (common)  R/O other problems (trauma). Pt must be fully CA&O.  Consider T/R if desired after contact with medical control or per protocol
  • 62. Releasing the (formerly Hypoglycemic) Diabetic The Ada County Protocol: “APPENDIX V: TREAT AND RELEASE CHECK SHEET FOR HYPOGLYCEMIC PATIENTS.”
  • 63. 11 Questions:  1. Is there a clear reason for the hypoglycemic episode?  2. Is the patient alert and oriented?  3. Is the patient’s repeat BG above 80 mg/dl?  4. Has the patient’s BG been well-controlled prior to this episode?  5. Is the patient able to eat a complex carbohydrate meal?  6. Does the patient have regular, on-going physician care?  7. Is the patient comfortable with non-transport?  8. Is the patient/guardian willing to sign a release form?  9. Is there another responsible person with the patient?  10. Is the patient’s temperature within normal limits? (95° to 100.4° Fahrenheit)  11. The patient is free of the influence of alcohol or other CNS- altering drugs?
  • 64. KEY POINTS:  Hypoglycemia is an Altered LOC (ALOC) emergency until proven to be hypoglycemia. › In other words, don’t get surprised by other causes, i.e. CVA, Alcohol, drugs, etc
  • 65.
  • 66.  Patent issued September 14, 1971 for the Ames Reflectance Meter
  • 67.  More physiologic insulin delivery to mimic the pancreas  Basal: steady background insulin delivery to keep BG from rising while fasting  Bolus: spurts of insulin to cover carbs or lower high BGs
  • 68.  Liberalization of diet — timing & amount (by user)  Increased control with exercise (by user)  Able to work shifts & through lunch (by user)  Less hassle with travel and time zones (by user)  Aid to weight control (by user)  Less anxiety in trying to keep on schedule (by user)
  • 69. •55.0 29.8 •23.9 •5.1 •13.413.0 7.9 16.4 5.02.5 0 10 20 30 40 50 60 Retinopathy Progression Laser Rx1 Micro- albuminuria2 Albuminuria2 Clinical Neuropathy3 Conventional Intensive 76% Risk Reduction 59% Risk Reduction 39% Risk Reduction 54% Risk Reduction 64% Risk Reduction CumulativeIncidence(%) 1. DCCT Research Group, Ophthalmology. 1995;102:647-661 2. DCCT Research Group, Kidney Int. 1995;47:1703-1720 3. DCCT Research Group. Ann Intern Med. 1995;122:561-568.
  • 70.
  • 71.  Carb boluses  Accuracy improved with personal carb factor adjusted for different times of day  Personal carb database  Correction boluses  Personalized correction factors for different times of day  Safer correction of high BGs  Reports amount of correction bolus used (ie, over 8% of TDD) An accurate TDD --> accurate basals & boluses
  • 72. Each manufacturer provides bolus dose recommendations differently.
  • 73.  As bolus size increases, so too does duration of action. Woodworth et al. Diabetes. 1993;42(Suppl. 1):54A
  • 74.  Bolus On Board (BOB) › Discounts bolus for residual BOB › Improves accuracy › Avoids stacking of bolus insulin › Acts as guide to whether carbs or insulin are needed, ie, BG is 130 mg/dl but BOB = 5 u  Requires a blood sugar test, an accurate duration of insulin action, and BG targetsPrevents hypoglycemia!
  • 75.  Reminders (alarms) to › test glucose following a bolus › test glucose after a low reading › test glucose after a high reading › give a bolus at certain time of day › warn when bolus delivery was not completed, etc. › change infusion site
  • 76. 108
  • 77.  Direct glucose entry into database eliminates data errors and offers optimum use of glucose/insulin data • AccuChek Spirit + meter • CozMore System + Therasense CoZmonitor • Soill Diabecare III pump + meter • Medtronic 515/715 + BD Paradigm Link • Soon: Animas + ? Lifescan or Glucowatch
  • 78.  A watch type device worn for about 13 hours/day.  Uses a disposable pad and minute bioelectric charge to read glucose readings as often as every 10 minutes.  Is effected by sweat (poor readings)  Is a supplement to (not a replacement for ) normal glucometer readings.  Downloadable info.
  • 79.
  • 80.
  • 81.  Real time readings  Add-on to routine BG monitoring  Radio communication from sensor to monitor  High and low glucose alarms  FDA pending Caution: Investigational Device.Limited by U.S. Law to Investigational Use
  • 82.  Silicon microneedles can be used to infuse insulin or allow glucose measurements in interstitial fluid. This needle array could replace the AutoSensor in a GlucoWatch for effective continuous monitoring.
  • 83. Caution: Investigational Device.Limited by U.S. Law to Investigational Use Meter replacement
  • 84.  Open-flow Microperfusion System  Inserted into the subcutaneous adipose tissue  Double lumen catheter  Acquires glucose readings every 30 minutes  Goal – subcutaneous glucose sensing/insulin delivery system
  • 85. An intelligent pump would alert the user when the BG is likely to cross a selected threshold value, such as 120 mg/dl (6.7 mmol). This safe meal delay reduces glucose exposure, especially when combined with a Super Bolus Max. drop ~4-5 mg/dl per min
  • 86.  Generally speaking, Insulin used in insulin pumps should not be used for standard injection!  Most insulin pump pt’s only take one type of insulin (fast acting)  When encountering a pump in a hypoglycemic patient, do not try to adjust it! › Remove it from the patient.
  • 87.
  • 88.
  • 89.  Usually presents as either DKA or HHNC › DKA more common to Type I diabetics › HHNC more common to Type II diabetics  Both syndromes have elevated BGL in the body.  Altered physiology leads to dehydration and acidosis. › Usually a slower onset than that of hypoglycemic episodes.
  • 90.  Also known as “Diabetic Coma”  DKA usually > 350 mg/dl (occasionally as low as 250 mg/dl)  More common in type I diabetics › More common in the 40’s (can be seen at all ages)  The body in an attempt to compensate for hyperglycemia, develops an acidosis state.  Dehydration  Vomiting  Loss of weight  Kussmaul respirations  Acetone smell  Impaired sensorium  Shock (hypovolemic/dehydration )
  • 91.  Oxygen, Airway Control, Rapid Transport  ALS Intercept! › EKG (look out for hyperK) › IV fluids (may require 4-6 liters over time) Monitor B/P. › Recheck BG  Hospital Care: › Insulin › ICU Admission
  • 92.  Hyperglycemic Hyper-Osmolar Non-ketotic Coma  Only 10% actually severe enough for coma  HHNC usually > 600 mg/dl  More common in type 2 diabetics  Slightly more common than DKA  More common in very elderly (can be seen at all ages)  Some insulin still being produced is enough to prevent Lactic metabolism. Thus no Ketones and no acidosis.  Just profound dehydration.  Treated with lots of saline as well as glycemic correction  High chance of severe electrolyte imbalances.  Mortality 10-20%
  • 93. One third of patients with HHNC do not have a prior (known) history of diabetes. -Emedicine.com
  • 94.  Protect the ABC’s  High Flow O2  Call ALS  Rapid Transport  Look for other causes also.
  • 95.  All BLS care AND : › 2 Large Bore IV’s › Fluid Resuscitation  Watch for fluid overload though › Shock management › Airway Management
  • 96.  The classical symptoms of child presenting with (new onset) Type 1 diabetes: › increasing thirst › increased drinking and › polyuria for some time (May start having accidents) › Weight Loss › Abd Pain › Tired and listless
  • 97.  More Severe complaints: › Vomiting › Altered Mental Status › Kussmal Respirations › Coma › Profound dehydration  25-40% go untreated long enough to develop DKA  5-10% in diabetic coma from DKA.  The condition is severe and is still the major cause of death in children with diabetes.  Consider abuse and/or neglect.
  • 98. Hypoglycemia  “Insulin Shock”  BG <60  They Look Shocky!  Pale, Diaphoretic,  HR is normal or mildly increased  BP typically normal.  Breathing is shallow  Sudden Onset  No real shock Hyperglycemia: DKA & HHNC  “Diabetic Coma”  Deep Respirations and fruity Breath (DKA)  BP decreased  HR is very increased (120-150 or more)  Hypotension and severe dehydration  Very Dry skin, Flushed or pale.  Slower onset.  Real Shock
  • 99.  Infection › Especially on feet and hands  Atypical Heart Attacks  Strokes  Renal Failure  Hypertension  Poor recovery from major insults to the body, prolonged healing.
  • 100.  After you obtain a good grasp of various diabetic conditions, you may want to learn about: › Graves Disease › Addison's Disease › Thyrotoxic Crisis (Thyroid Storm) › Hypothyroidism › Cushing's Syndrome  (not Cushing's response, which is different)
  • 101.  The endocrine system maintains stability in the body’s internal environment (homeostasis).  Type 1 and type 2 diabetes involve abnormalities in the body’s ability to use glucose (sugar) for fuel.
  • 102.  Polyuria (frequent, plentiful urination), polydipsia (frequent drinking to satisfy continuous thirst), and polyphagia (excessive eating due to cellular hunger) are common symptoms, or the “3 Ps,” of uncontrolled diabetes.
  • 103.  Patients with diabetes have chronic complications that place them at risk for other diseases.  Hyperglycemia is the result of a lack of insulin, causing high blood glucose levels.
  • 104.  Hypoglycemia is a state in which the blood glucose level is below normal. Without treatment, permanent brain damage and death can occur.  DKA is the buildup of ketones and fatty acids in the blood and body tissue that results when the body relies upon fat for energy.
  • 105.  Hyperglycemic crisis (diabetic coma) is a state of unconsciousness resulting from DKA, hyperglycemia, and/or dehydration due to excessive urination.
  • 106.  Hypoglycemic crisis (insulin shock) is caused by insufficient blood glucose levels. Treat quickly, by giving oral glucose (if protocols allow), to avoid brain damage.
  • 107.  When assessing diabetic emergencies, err on the side of giving oral glucose (if protocols allow). Do not give oral glucose to patients who are unconscious or who cannot swallow properly and protect the airway. In all cases, provide rapid transport.
  • 108.  Problems associated with diabetes include seizures, altered mental status, “intoxicated” appearance, and loss of a gag reflex, which affects airway management.  Hematology is the study and prevention of blood-related disorders.
  • 109.  Sickle cell disease is a blood disorder the affects the shape of red blood cells. Symptoms include joint pain, fever, respiratory distress, and abdominal pain.
  • 110.  Hemoglobin A is considered normal hemoglobin. Hemoglobin S is considered an abnormal type of hemoglobin and is responsible for sickle cell crisis.
  • 111.  Patients with sickle cell disease have chronic complications that place them at risk for other diseases, such as heart attack, stroke, and infection.
  • 112.  Patients with hemophilia are not able to control bleeding.  Emergency care in the prehospital setting is supportive for patients with sickle disease or a clotting disorder such as hemophilia.