An official website of the United States government
Official websites use .gov
A
.gov
website belongs to an official government organization in the United States.
Secure .gov websites use HTTPS
A lock
()
or
https://
means you’ve safely connected to the .gov website. Share sensitive information only on official, secure websites.
SHARE:
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
Oxycodone
Image
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
Agenda
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
Hydromorphone
Image
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
Transition
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
Review
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
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.
Reference
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
Image
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
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.)
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
Image
Sharee
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
Transitions
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
Lifestyle
Sleep
Diet
Exercise
Hydration
Exercise
Biological
Physical health
Sex
Pain modulation
Pubertal development
Psychological
Individual beliefs
Mood/affect
Anxiety/fear
Coping
Social
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
Image
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)
"You are more than just your sickle cell disease"
"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:
Avascular necrosis
Stationary bike with arm component
Light resistive exercises
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
Lie down (or sit comfortably) with knees bent and shoulders, head, and neck relaxed.
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.
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.
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.
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
