Ouch It really hurts

1
Ouch! It really hurts

• Managing crises in Sickle cell disease
• A primary care perspective
• Chris Manasseh, MD
• 12-04-01

2
Sickle Cell Crisis - Resources

• Web based
• sicklecelldisease.org
• nih.gov
• ahcpr.gov
• ampainsoc.org
• guidelines.gov
• uptodateonline.com
• medscape.com
• aafp.org
• partnersagainstpain.com

• Community based
• Sickle cell centers
• Joint center for sickle and thalassemic disorders
• Pain management centers

3
ObjectivesSickle Cell Crisis

• What is a crisis?
• Why crises occur?
• When to make the diagnosis?
• How to manage patients in crisis?
• What are some preventive strategies?

4
Sickle Cell Disease(SCD)At a Glance

• gt70 of patients receive transfusion by 20 years
  of age.
• gt50 of patients reach 5th decade of life
• Only 5 of patients have more than 3 episodes of
  crisis in a year.
• Only 3 of patients with SCD are addicted to
  opioid analgesics.

5
A Sickle cell crisis is

• Painful Episode - Infection vs Infarction
• Bone crisis
• Joint crisis
• Abdomen crisis
• Pain and beyond
• Other crises
• Other Clinical conditions
• Ominous predictor

6
Other crises in SCD

• Hematologic crisis
• Splenic sequestration crisis
• Aplastic crisis
• Hepatic sequestration crisis
• Infectious crisis
• Functional Asplenia
• Dysfunctional Immunity

7
One such other Crisis...
8
Another clinical condition...
9
Crises due to Vaso-occlusive phenomenon

• Cascade of events
• Contributors/Triggers
• Alcohol/Acidosis/Altitude
• Bacteria/Infection
• Cold exposure
• Dehydration
• Exercise/Emotional stress

10
Case Presentation

• A 22 y/o male with known sickle cell disease and
  beta thalassemia
• presents with c/o severe lower back and bilateral
  hip pain for 3 days., which is the usual pattern
  of his sickle cell pain.
• Patient took his home medications, Motrin and
  percocet with minimal
• relief. He rates his pain on the 0-10 numerical
  pain intensity scale as a 10.
• The next step in his assessment should include
• Appropriate history
• A detailed pain evaluation
• A thorough physical exam
• A comprehensive laboratory exam
• A rapid action plan

11
When to make the diagnosis

• Evaluating pain
• Subjective Self report
• Objective Simple scale
• Examining the patient
• Symptoms Suspect infection
• Search for Triggers
• Signs Look, listen and feel

12
Red flags when diagnosing sickle cell crisis

• Abnormal vitals
• Fever greater than 101 F
• Tachycardia/Tachypnea/Dyspnea
• Hypotension
• Abnormal sights
• Pallor/Jaundice/Erythema
• Edema
• Lethargy
• Abnormal sounds
• Rales/Rhonchi
• Bowel sounds

13
Making the diagnosisWatch out when blood tests-

• CBC
• Hemoglobin/MCV
• White count
• Platelets
• Electrolytes
• Sodium
• Liver enzymes
• Bilirubin

14
Making the diagnosisTime to get...

• ABGs CXR
• Bone studies X-rays and scans
• Culture from everywhere
• Urine
• Blood
• Sputum
• Joint aspirate

15
Managing the sickle cell crisisAs in construction

• Foundation - Analgesia
• Building blocks
• Antibiotics/Administer oxygen
• Blood transfusion
• Correcting volume deficits
• Hydroxyurea
• Mortar - Empathy and Education

16
Managing the crisisOne building block is fluids

• Route of administration
• Amount to be given
• Type of fluid
• Monitor
• Is and Os
• Daily weights
• Volume overload

17
Another building block is transfusion
18
Managing the crisisFoundation is Analgesics
The ABCs for pain management by AHCPR A - Ask
about pain regularly. Assess pain systematically.
B - Believe patient/family in their reports of
pain what relieves it C - Choose pain control
options appropriate for the patient, family,
and setting. D -Deliver interventions
in a timely, logical,coordinated fashion E -
Empower patients and their families . Enable
patients to control their course to the greatest
extent
possible.
19
WHO Pain Ladder

• Nonopioids
• NSAIDS
• Tramodol
• Toradol
• Adjuvants
• TCAs
• Anticonvulsants
• Opioids
• Supplemental
• Cognitive
• Physical modalities
• TENS

20
Using the OpioidsIts decision time!

• Oral vs. Parenteral
• Morphine vs. Meperidine
• Fixed time schedule vs. PRN
• Patient control (PCA) vs. Physician control
• Outpatient vs. Inpatient
• Tolerance vs. Dependence

21
Worries about Opioid side effects ?

• Constipation
• Nausea
• Sedation
• Pruritis
• Respiratory Depression
• Urinary retention

22
Clinical Scenario
A 25 y/o patient well known to the Family
Medicine Inpatient service is admitted for the
third time in 4 months with a painful sickle cell
crisis. She is on prn percocet at home with NKDA.
After starting a fever workup you begin IV fluid
hydration as she is unable to tolerate anything
PO. Which of the following narcotic regimens
would you choose? A. IM Demerol 100 mg q 3 hours
prn B. IV Morphine 2-4 mg q 3 hours scheduled C.
IV Morphine 4 mg q4 PCA with 0.5 mg rescue q1
hour D. IM Toradol 30 mg q6 hours with IV
morphine 1-2 mg q 3 hours prn
23
Strategies to prevent Sickle Cell Crisis - Its
about roles

• By the health care provider
• Continuity
• Coordination
• By the patient
• Keeping the pain diary and the f/u
• Carrying the card
• Calling the doctor/going to the hospital
• By others
• Healthcare facility

24
One Last thing..think ofHBSS PAIN CRISIS

• H-Hemolysis
• B-Bone marrow infarction
• S-Stroke
• S-Skin ulcers
• P-Painful episodes,Priapism
• A-Aplastic crisis,AVN
• I-Infection
• N-Nocturia, Hyposthenuria

• C-Cholelithiasis,CHF
• R-Renal failure
• I-Infarction
• S-Sequestration
• I-Increase fetal loss
• S-Sepsis