Event 3: Clinical Encounters

Consultation with Inpatient Pharmacist: Learning Objectives

Upon completion of this segment the learner will be able to:

  • List potential non-pharmacologic pain management interventions
  • Identify common opioid counseling points
  • Describe the goals for pain management interventions

Sharee with Inpatient Pharmacist

Discharge Medication


chemical composition of morphine
Oxycodone chemical composition

10 mg. every 4 hours as needed for pain

  • Used as an analgesic
  • Is an agonist at the mu opioid receptor
  • Good oral bioavailability
  • Metabolized by hepatic cytochrome P450 enzymes into a number of active metabolites
  • Some of its metabolites, such as oxymorphone, are more potent than oxycodone
  • Excreted by renal elimination
  • May cause respiratory depression and death
  • May lead to tolerance, dependence, and addiction
  • May cause constipation and urinary retention
  • Discontinuation may lead to withdrawal symptoms
  • DEA Schedule II drug

 Medication Chart

  • Patient should use a chart to track times of medication administration

Dosage Overview

  • Higher dose at discharge
  • Primary Care visit in 3 days to re-evaluate
  • Goal: Reduce dosage
  • Side Effects: Dizziness, stomach ache, constipation
  • Serious potential side effect: Slowed breathing (fatigue is early sign)
    • If breaths <8-10/min call 911

Timing of Effect of Oral Opioid Dose

  • Initial effect: 30 minutes
  • Maximum effect: 60 minutes
  • Total duration: 4 hours

Adjunctive Therapies that May Be Effective to Avoid Use of Opioids

  • Meditating
  • Breathing
  • Sitting listening to music
  • Relaxing

Interventions are Additive Goals of Intervention/Medication:

  • Good function
  • Activity level
  • Comfort
  • Sleep

Medication Storage

  • Pill case
  • Keep inaccessible to others

Consultation with Hematologist: Learning Objectives

Upon conclusion of this segment the learner will be able to:

  • Understand some of the concerns with opioid use in sickle cell disease
  • Be familiar with the transition needs of young adults with sickle cell disease

Sharee and the Hematologist


  • What brought you to the ED?
  • Transition to college

Sharee's Usual Home Care

  • Usual Trigger: Cold
  • Home management routine:
    • Ibuprofen
    • Hydrated
    • Pain medication
    • Relaxation
    • Music
    • Lights off
    • Heat

ED Management

  • O2
  • Fluids
  • Morphine ineffective, changed to hydromorphone (Dilaudid®)
    • Sharee is concerned about addiction


Chemical composition of hydromorphone
  • Synthesized from morphine
  • Higher lipid solubility than morphine and crosses the blood-brain barrier more easily than morphine
  • Oral bioavailability is very low. Usually used in hospital setting where it is giving by i.v. administration
  • Not metabolized by cytochrome P450 enzymes. Metabolized by glucuronidation to hydromorphone-3-glucoronide
  • Excreted by renal elimination
  • May cause respiratory depression and death
  • May lead to tolerance, dependence, and addiction
  • May cause constipation and urinary retention
  • Discontinuation may lead to withdrawal symptoms
  • DEA Schedule II drug

Tolerance/Dependence vs. Addiction

  • Tolerance: Body becomes tolerant and requires increased dose or rotation to another medication
  • Dependence: Body becomes physiologically dependent; withdrawal symptoms if discontinued too quickly
  • Addiction: Craving; focus on getting more medication

Tolerance/Dependence can occur with chronic use of an opioid even if taken as prescribed, but does not by itself constitute addiction.

How do I manage my medications on my own?

  • Pharmacist will discuss tapering to prevent withdrawal

Other Therapies

  • Physical Therapy
    • Inpatient and outpatient benefits
  • Acupuncture


  • New providers
  • How will they know about me?

Contact University Health Services

  • PCP
  • Hematologist

Information Transfer to New Providers

  • Type of Sickle Cell Disease
  • Medications
  • Complications
  • Pain Management Protocol

Consultation with Outpatient Pharmacist: Learning Objectives

Upon completion of this segment the learner will be able to:

  • Describe the purpose of folic acid treatment in patients with sickle cell disease.
  • Understand the purpose and common side effects of hydroxyurea treatment in patients with sickle cell disease.
  • Recall frequency of monitoring with hydroxyurea

Sharee with Outpatient Pharmacist


  • Folic Acid
  • Hydroxyurea

Folic Acid Purpose and Instructions

  • Patient is advised to take 1 mg (1 tablet) by mouth once daily
  • Helps prevent anemia
  • Patients with sickle cell disease make red blood cells quicker than other people
  • Folic acid helps the body build red blood cells
    • If patients are feeling tired or weak they should contact their doctor

Folic Acid

  • Image
    Chemical composition of folic acid
    Chemical composition of folic acid
    In patients with Sickle Cell Disease (SCD), the RBC count is lower than normal because sickled cells usually die after 10 to 20 days, in contrast to 120 days for normal RBCs. Because of high cell turnover, folate stores are often depleted.
  • Folic acid replenishes the depleted folate stores necessary for erythropoiesis. Folic acid supplementation is well established in the treatment of chronic hemolytic anemia.


  • Replacement needed due to accelerated cell destruction
    • Recommended for adults with SCD, women considering pregnancy & patients with SCD + chronic hemolysis
    • 1 mg by mouth once daily

Kennedy, Fung, Kawachak, et al. Red blood cell folate and serum B12 status in children with sickle cell disease. J Pediatr Hematolo Oncol 2001; 23:165-69.

Hydroxyurea's Purpose

  • Who's a candidate for treatment with hydroxyurea*?
  • Patient is advised to take full dose 2000 mg by mouth once daily
    • Dose is based on patient's weight
  • Hydroxyurea reduces the frequency that patients experience sickle cell crises, such as:
    • Pain crises or vaso-occlusive crisis (VOC)
    • Acute chest syndrome
    • Priapism (pain prolonged erection in male patients)
  • Hydroxyurea has also been shown to decrease hospitalizations

*Who is a candidate for Hydroxyurea therapy?

In adults with sickle cell anemia (SCA) who have greater than or equal to 3 moderate to severe pain crises associated with sickle cell disease (SCD) during a 12 month period, initiate treatment with hydroxyurea.

In adults with SCA who have sickle cell-associated pain that interferes with daily activities and quality of life, initiate treatment with hydroxyurea.

In adults with SCA who have a history of severe or recurrent acute chest syndrome (ACS), initiate treatment with hydroxyurea.

In adults with SCA who have severe symptomatic chronic anemia that interferes with daily activities or quality of life, initiate treatment with hydroxyurea.

In infants 9 months of age or older, in children, and in adolescents with SCA, offer treatment with hydroxyurea regardless of clinical severity to reduce complications (e.g., pain, dactylitis, ACS, anemia) related to SCA.

In adults and children with SCD who have chronic kidney disease and are taking erythropoietin, add hydroxyurea therapy to improve anemia.

In persons with HbSß+ thalassemia or HbSC who have recurrent SCD-associated pain that interferes with daily activities or quality of life, consult with an SCD expert for consideration of hydroxyurea therapy.


Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, Jordan L, Lanzkron SM, Lottenberg R, Savage WJ, Tanabe PJ, Ware RE, Murad MH, Goldsmith JC, Ortiz E, Fulwood R, Horton A, John-Sowah J. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014 Sep 10;312(10):1033-48.

Hydroxyurea Common Side Effects

  • Gastrointestinal (GI) side effects:
    • Upset stomach
    • Nausea
  • Mouth sores possible (practice good oral hygiene)

Skin Effects of Hydroxyurea

  • effects (less common): Patients should use sunscreen and wear sunglasses
    • Hair loss
    • Lightening

Hydroxyurea Mehcanism of Action

Chemical composition of hydroxyurea
Chemical composition of hydroxyurea
  • Prevents clumping of red blood cells by reducing deformity
  • Mechanism of action: increase presence of fetal hemoglobin and slow down cell destruction
  • Onset: 4-12 weeks
    • Response seen in lab parameters (HgF and MCV) @ 8 weeks
    • Peak response & benefits beginning @ 40 weeks
  • Precautions: teratogenic (causes birth defects)
    • Contraception recommended for both male and female patients during and after treatment
  • Administer w/ folic acid
    • folate deficiency may be masked by hydroxyurea
  • Hydroxyurea is an oral medicine that has been shown to reduce or prevent several SCD complications.
  • This medicine was studied in patients with SCD because it was known to increase the amount of fetal hemoglobin (hemoglobin F) in the blood. Increased hemoglobin F provides some protection against the effects of hemoglobin S.
  • Hydroxyurea was later found to have several other benefits for a person with SCD, such as decreasing inflammation.



Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, Jordan L, Lanzkron SM, Lottenberg R, Savage WJ, Tanabe PJ, Ware RE, Murad MH, Goldsmith JC, Ortiz E, Fulwood R, Horton A, John-Sowah J. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014 Sep 10;312(10):1033-48.

Hydroxyurea Monitoring

Monitoring (approximate time frame):

  • Before starting hydroxyurea
  • When hydroxyurea is started
    • Every month
  • With changes in dose
    • Decrease: every week
    • Increase: every 2 months
  • Once stable dose is reached (no changes in dose or labs)
    • Every 2-3 months

Hydroxyurea and Contraception

  1. Both men and women should have safe sex practices while on hydroxyurea
    • Men should wear latex condoms (if not allergic)
    • Women should use a consistent method of birth control (oral contraceptives, implants, etc.)
  2. Patients taking hydroxyurea should plan ahead before trying to have children as it can cause harm to an unborn child.
    • Contact your doctor about planning a pregnancy as the medication should be safely discontinued

Pain Medication

Head and shoulders shot of Sharee, an African American young adult with shoulder length hair.
  • Oxycodone IR (Immediate Release
  • OTC ibuprofen
    • Need for medication life-long
    • Dosages will vary
    • Specific medications will also vary

Checklist for Transition

  • Contact campus health service
    • Inform them of your SCD
  • Keep current list of medications
  • Monitor side effects/symptoms

Consultation with Psychologist: Learning Objectives

Upon completion of this segment, the learner will be able to:

  • Identify how engagement with behavioral health can promote positive psychosocial adjustment for individuals with sickle cell disease.
  • Describe how the biopsychosocial and cognitive-behavioral frameworks can promote effective pain management and healthy medical coping and acceptance.
  • Discuss aspects of transition from pediatric to adult medical care.

Sharee with Psychologist


  • Academic: High School to College
  • Medical: Pediatric to Adult Self-Care World

Role of Psychology for Individuals with Sickle Cell Disease

  • Non-Threatening Description of Role
    "I look for pain patterns and triggers, and ways to help you develop coping skills"
  • Empathy
    "Life is hard enough, let alone living with a pain-related chronic medical condition"
  • Normalization
    "This would be hard for anyone in your shoes"
  • Validation
    "Your pain is real"
  • Psychoeducation
    "Pain is multifactorial" / Biopsychosocial Model / Gate Theory of Pain
  • Skill Building
    "There are evidence-based, non-pharmacological practices and interventions to help you live a more engaged, value-based life"
  • Positive / Corrective Emotional Experience
    To promote engagement and adherence

Engagement Model

  • Goals
    • Enhance life engagement, despite pain
    • Increase independent functioning
  • Assumptions
    • Limit pain, not eliminate pain
    • Pain profile may or may not improve
  • Signs of progress
    • Increased functioning
    • Enhanced coping
    • Improved self-efficacy

The Biopsychosocial Model


  • Sleep
  • Diet
  • Exercise
  • Hydration
  • Exercise


  • Physical health
  • Sex
  • Pain modulation
  • Pubertal development


  • Individual beliefs
  • Mood/affect
  • Anxiety/fear
  • Coping


  • Culture
  • Socioeconomic status
  • School/work environment
  • Social/peer interactions
  • Parental/family factors

Linking Biopsychosocial Model and Lifestyle Hygiene

  • "I always have to plan in my head"
    (for lifestyle needs)
  • "Like not wearing a jacket and being super cold"
    (landed me in the hospital)
  • "One moment of not caring"
    (can cascade into a negative health cycle)

Cognitive Triangle

Illustration shows thoughts linked with behavior, feelings, and the body.

Cognitive Behavioral Therapy (CBT)

  • Intervention to promote healthy coping, engagement, and functioning
  • Consider referral when:
    • Difficulty accepting or adapting to living with sickle cell disease
    • Limited range of tools for pain / stress management
    • Lifestyle changes have not been effective or patient has had difficulty implementing changes
    • Adherence challenges with medical regimen
    • Avoidance of engagement with life activities (e.g., school, work, family, social)
    • Family / friends are enabling maladaptive behavior
CBT: Core Characteristics
  • Thoughts, feelings, behaviors = interconnected
    • Make changes to thoughts and behavior that can effect positive mood and overall change
  • Assumption that most emotional and behavioral reactions are learned
    • Therapy goal = help patients unlearn unwanted reactions and learn new ways of responding
  • Emphasis on current behavior
  • Based on "rational thought"
    • Facts, not assumptions
  • Targeted and time-limited
    • Care can be episodic
  • Structured and directive
  • Homework is a core feature

Control What You Can Control

"Keep warm and hydrated" (lifestyle)
"Take medication" (adherence)
"Trust medical team" (support)
"get rest" (lifestyle)
Remembering I am more than sickle cell" (identity)

Self-Identity (beyond medical condition and pain)

  1. "You are more than just your sickle cell disease"
  2. "You are more than just your pain"
    • Pain is pain, but suffering is optional
    • Pain is real, but the amount of suffering is adjustable

Coping with Pain and Stress in the Past

  • "Hold on and push through" pain and stress
  • "Don\\\'t really think about what you\\\'re doing"
  • Reliance on parental coaching / support
  • Reliance on pediatric medical team to follow up

Coping with Pain and Stress in the Future

  • "It is just me. I have to come up with a way not to rely on anyone else but myself"
  • Self-reliance includes using available supports

Thoughts and Emotions Regarding Transition

What are your thoughts as you go off to college?

  • I am ready and I am nervous
  • Ambivalence is normal

Supports for Transition

Have you found your new...

  • PCP?
  • Sickle Cell Specialist?
  • Psychologist or Therapist?

What If you do not connect to resources when you go off to college?

  • "Where will you go for care?"
  • "The ED, which is not a good idea"

College Resources

  • University Health Service
    • Primary Care
    • Specialty Care
    • Psychotherapy

Consultation with Physical Therapist: Learning Objectives

Upon completion of this segment the learner will be able to:

  • Describe the role of the physical therapist, as part of an interdisciplinary team, in treating individuals with chronic pain.
  • Describe examples of exercises that may be prescribed to target multisystem involvement, specifically cardiopulmonary or musculoskeletal, resulting from SCD.
  • Explain the importance of diaphragmatic breathing during exercise and times of stress.

Sharee with the Physical Therapist

What is a physical therapist?

  • A movement specialist that treats people of all abilities to improve their function and quality of life across their entire lifespan.

Exercises for a patient with SCD who has:

  1. Avascular necrosis
    • Stationary bike with arm component
    • Light resistive exercises
  2. Acute Chest Syndrome
    • Postural and breathing exercises to relieve lung restriction

Avascular Necrosis

  • Caused by repeated disruptions in blood flow to the joint
  • Usually found in adolescents and adults, not children
  • Sickling in the bones of the hip, shoulder joints, knees, and ankles
  • Decreased oxygen flow results in severe damage
  • Symptoms:
    • Pain and problems with walking and joint movement


Acute Chest Syndrome

  • Caused by occlusion of blood vessels in the lungs
  • Lungs can be deprived of oxygen causing tissue damage
  • Often starts after a painful crisis
  • Symptoms:
    • Pain
    • Fever
    • Shortness of breath
    • Cough


Practicing Diaphragmatic Breathing with Pursed-Lip Exhalation

  1. Hand on abdomen
  2. Feel hand on abdomen push out with inhalation
  3. Hands on sides to monitor movement for inhalation and exhalation

CBT: Behavioral Techniques

  • Evidence-based coping strategies that can decrease pain / stress experience
  • Choose two or more coping strategies – practice daily and use as needed:
    • Diaphragmatic breathing
    • Progressive muscle relaxation
    • Mindfulness
    • Meditation

CBT: Behavioral Techniques – Diaphragmatic Breathing (DB)

  • AKA deep breathing or belly breathing
  • Basics
    1. Lie down (or sit comfortably) with knees bent and shoulders, head, and neck relaxed.
    2. Place one hand on your upper chest and the other hand just below your rib cage. This will allow you to feel your diaphragm move as you breathe.
    3. Breathe in slowly through your nose so that your stomach moves out against your hand. The hand on your chest should remain as still as possible.
    4. Exhale through pursed lips by tightening your stomach muscles and letting them fall inward as you exhale. The hand on your chest should remain as still as possible.
    5. Practice 5 minutes at a time, starting with 1-2 practice sessions per day.

Stacked Breathing

  • 3 puffs (short breaths)
  • Exhale with pursed lips for a count of 4

Diaphragmatic Breathing

  • Expands lungs
  • Changes the mechanics of breathing

Diaphragmatic breathing can help relaxation at times of stress

Practicing Postural Exercises

  • Sitting up, opening up rib cage
  • Squeezing shoulder blades together and down
  • Improving posture improves breathing

Sharee's Perspective

"I've been dealing with chronic pain all my life"

"I stray away from doing any exercises… As I get older... because I'm afraid of hurting myself or causing a crisis"

A physical therapist as part of the team can:

  • Help reduce fear through teaching and monitoring appropriate exercise
  • Be a motivator to take an active role in one's own care

Consultation with Acupuncturist: Learning Objectives

Upon conclusion of this segment the learner will be able to:

  • Explain how an acupuncturist would assess and characterize sickle cell disease
  • Be familiar with the current evidence base relating to acupuncture and sickle cell disease
  • Describe the specific risks and adverse events associated with acupuncture

Sharee with the Acupuncturist

Agenda of Visit

  • Pain experience
  • Diagnostic technique (pulses, tongue)

Pain Experience

  • Pain occurs any joint anytime
  • Most severe pain is in knee because of avascular necrosis
  • Some pain-free days
  • Pain is brought on by dehydration, cold, stress
  • 4-5 severe crises/year
  • Pain can be caused by relatively minor environmental stressors

Characterization of Pain Episode

  • Excruciating
  • Being cut from the inside/out
  • Beaten by a 2 x 4
  • Dull, throbbing
  • Will not go away

Acute Chest Syndrome

  • Affects breathing
  • Pain in chest, ribs
  • Less frequent, but more severe, than many other SCD crises

Elements of the Physical Exam

  • Pulses
  • Tongue
    • Coating
    • Coloration

Examination of the Pulses

  • Blood flow in various body regions
  • Overall constitution/state of health
  • Her pulses
    • String-like
    • Healthy or associated with pain
    • May signal body is working hard

Examination of the Tongue

  • Thick coating
    • Internal dampness
    • Concentration of fluids
  • Redness, small speckles
    • Heat in the upper body
  • Scalloped

Can Acupuncture help?

  • Yes, during crises
  • Ongoing intervention might reduce:
    • Frequency of crises
    • Severity of pain
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