Transcript Pediatric Procedural Sedation - McMaster Faculty of Health

To Clot Or Not To Clot…
Emergency Care for Coagulation
Disorders/Conditions
Kay Decker Hemophilia Nurse Coordinator
Hamilton Niagara Regional Hemophilia Program
McMaster Children’s Hospital
Current Concept in Pediatrics
October 16th, 2009
Our Program
Responsible for all patients (Adult/Peds)
with hemophilia and other bleeding
disorders such as rare factor deficiencies
and vWD.
Objectives




Hemophilia Basics
Hemophilia Emergency Care
Key Points
Summary
What is Hemophilia?
Hemophilia
 A genetic (inherited) disorder in which a clotting
protein is missing or deficient causing prolonged
bleeding
Hemophilia A
Factor VIII Deficiency
Classic Hemophilia
Hemophilia B
Factor IX Deficiency
Christmas Disease
Incidence
 Factor VIII deficiency (Hemophilia A)
 affects 1 in 10,000 males (www.ahcdc.org : CHR)
 60% have severe disease
 Factor IX deficiency (Hemophilia B)
 affects 1 in 25,000 to 30,000 males (www.ahcdc.org : CHR)
 50% have severe disease
 2,400 affected males across Canada
 Affects all races, all social classes
 X-linked inheritance
 1/3 of FVIII and 1/5 of FIX cases are “spontaneous”
with no family history
Characteristics of Hemophilia
by Severity
Classification
of
Hemophilia
Severe
Moderate
Mild
Level of
Factor VIII/
IX in the
blood*
< 1%
1–5%
5 - 40 %
Expected No. of
Bleeds/Year
(in absences of
prophylaxis)
10-20
1-3
1 or less
Types of bleeding
 Joint and muscle bleeds
 Bleeding can occur
spontaneously and/or from
trauma
 Frequently as a result of
minor trauma
 Bleeding may be similar to
severe Hemophilia with lower
levels
 Bleeding after significant
trauma or with surgical
procedures
 Infrequent bleeding
*clotting factor activity in a normal person is said to be 100%, ranging anywhere from 50-150%
Hemophilia is:
a.
b.
c.
d.
low or absent factor VIII (8) or factor IX (9)
curable
bleed really fast
carried on the y chromosome
Bleeding Manifestations in
Hemophilia
Serious




Joints (hemarthrosis)
Muscle/soft-tissue
Mouth/gums/nose
Hematuria
Life-threatening




CNS
Gastrointestinal
Neck/throat
Severe trauma
Clinical Presentation
Site of Bleeding
Clinical Presentation (signs and symptoms)
Joint
• Pain, decreased joint range of motion, heat/warmth, favouring of
limb, swelling may be present
• Patient may complain of tingling or bubbling in the joint
Muscle
Soft Tissue
Neck/Throat
Gastrointestinal
Head Injury
• Vague ache or pain, heat, swelling, inability/unwillingness to
move muscle, tightness of skin
• Superficial hematomas and bruises
• Pain, swelling, difficulty swallowing/breathing
• Abdominal pain, nausea, coffee-ground emesis, rectal
bleeding
• Severe headache, nausea/vomiting, irritability
• Later symptoms may include rigid neck, slurred speech, visual
changes, confusion, seizures or loss of consciousness
Lacerations and Cuts
 Superficial cuts
 apply pressure
 normal first aid
 Deep lacerations
 factor replacement before sutures and removal of sutures
 Head trauma interventions, if appropriate
Mucous Membrane Bleeding
 Frenulum tears / mouth injuries
 Tooth loss & eruption / dental work
 Epistaxis (nosebleeds)
The assessment of hemarthrosis is difficult in young
children. If you suspect that a child is having a knee
bleed. What would your Immediate assessment include?
a.
b.
c.
d.
X-ray to determine the severity of the bleed
Early factor replacement before investigations
RICE
Attempt to manipulate the joint to improve range
CT of Pelvis
Limb Compartments
 Bleeding into the iliopsoas, anterior calf and forearm
are high risk for compartment syndrome
 As the muscle expands nerves and vessel are
compressed
 Permanent disability can occur if prompt treatment is
not given
How Do We Treat
Hemophilia?
 Factor replacement – Recombinant FVIII or
Recombinant FIX
 DDAVP (for mild only FVIII) – helps increase
endogenous levels of FVIII
 Anti-fibrinolytics (Tranexamic acid)
 RICE (Rest, Ice, Compression, Elevation)
 Comprehensive care – bleed prevention
Treatment Card
Key Points
 FACTOR FIRST, diagnostic investigations later
 Factor is $$$; the entire vial should be used or waste should be
minimized
 NO invasive procedures without first replacing missing factor
 NO IM injection
 NO ASA containing products
 ½ life of VIII is 8-12hours
 ½ life of FIX is 18-24hours
 Allergies to factor concentrate are rare
 Patients are instructed to keep records
 Good healthy veins (Lifeline)
When performing venipuncture on a patient
with hemophilia. What are some nursing
considerations?
a. Hold the site uninterrupted for 5 minutes
b. Dig around the vein if the patient has
difficult venous access to minimize the
amount of pokes
c. Use the smallest gauge needle as possible
d. a & c
The Evaluation of Cases
According to Most Urgency
to Least Urgent
Triage Levels
Ctas
Time To
Medical Care
LEVEL I
Resuscitation
Immediate
LEVEL II
Emergent
15 Minutes
LEVEL III
Urgent
30 Minutes
LEVEL IV
Less Urgent
60 Minutes
LEVEL V
Non Urgent
120 Minutes
Canadian Paediatric Triage and Acuity Scale:
Implementation Guidelines for Emergency Departments
Triage of Bleeding Disorders
HHS clinical guidelines supports:
 “All hemophiliac patients will be triaged as CTAS II”
(statement found on HHS intranet/Emergency
Medicine/Physician Clinical Guidelines)
Ref: M.Bullard et al., CJEM,March 2008; 10(2),
p 132-146; Warren, et al. CJEM, May 2008; 10(3).
p 224-232 )
Case Study
A 10 year old male severe hemophilia B
patient comes into the ER Department
crying with a swollen knee after
playing baseball.
Management: Joint Injury
 Triage Level II (Emergent) seen within 15 minutes
 Is this a life-threatening incident? No! But bleeding into a joint
can cause permanent joint damage even after the 1st bleed
and is very painful (the amount of bleeding into the joint will
determine the amount of damage to the joint).
 Identify type and severity of bleeding disorder
 If and when he last had a treatment
 Treat ASAP
 Diagnostic test later
 Notify the hematologist on call
 Instruct the patient or family to notify the HTC for follow-up
Summary
If a child with hemophilia
states that he has a bleed
… believe.
Summary
85% of treatments are given by
patients or caregivers so when a
patient comes to ER it should be a
warning of something unusual and
potentially dangerous.
Dr. I. Walker…
Summary
Early and appropriate treatment of each bleeding
episode is critical to minimize complications.
Replacement of deficient clotting factor is the
single most important step in
ANY intervention.
SUMMARY
If in doubt…
FACTOR FIRST.
What are some general principles of care?
a.
b.
c.
d.
Prevention of bleeding is the goal
When in doubt treat!!
Acute bleeds should be treated as early as possible
All of the above
Comprehensive Care Team
Our program provides service for Pediatric and Adult population
Team:
 Dr. Chan (Pediatric Director)
 Dr. Walker (Adult Director)
 Dr. Webert (Adult Hematologist)
 Kay Decker (Hemophilia Nurse Coordinator)
 Rebecca Goldsmith (Pediatric Thrombosis/Hemophilia Nurse)
 Theresa Almonte (Program Secretary)
 Cecily Bos (PT)
 Linda Waterhouse (Pediatric SW)
Additional Information
 www.machealth.ca
 www.hemophilia.ca
 www.wfh.org
 www.ahcdc.org