1. Moderator The Faces of Sickle Cell Disease
KQ covered more biological basis for pain management. This talks in intended to cover additional resources…to work with medication, particularly with regard to acute pain.
Theopia Jackson, PhD
September 10, 2016
8th Annual Sickle Cell Disease Educational Seminar 2016 1

2. The Face of Sickle Cell Disease

3. Developmental Stages INFANT/TODDLER Parental attunement
Fear of punishment
SCHOOL AGE
Absences
Peer relations
MATURE ADULTS/SENIORS
Chronic pain
$, work limitations
Facing mortality
Infants and Toddlers
Primary caregiver stability, attunement to child’s distress, accuracy as an observer and historian
Anxiety regarding procedures may outweigh anxiety about illness
Belief in illness or procedures as a punishment
Adolescence
School difficulties more pronounced
Cycle of mistrust may develop with providers
Adjustment of educational, employment goals
Transition to adult care
Mature Adults and Seniors
Coping with chronic, as well as acute pain common
Financial, employment limitations
Fears of mortality with each painful episode
ADOLESCENCE
School difficulties
Cycle of mistrust may develop
Goal adjustment
Transition
YOUNG ADULTS
Cumulative illness
effects stress
Prolonged dependence

4. Sickle Cell Disease Morbidity
Stroke or brain damage
Pneumonia or Acute Chest Syndrome
Kidney failure
Increased infections
Pain episodes (VOE)
Splenic sequestration
Delayed puberty, priapism, high risk pregnancy

5. Medical Sequelae/Complications
Anemia
Extreme recurrent pain
Jaundice
Acute and chronic tissue injury
Frequent urination
Nocturnal Enuresis
Increased fluid intake needs
Malnutrition
Iron/protein deficiencies
Opthalmological complications
CVA’s- overt and “silent” strokes
Acute pulmonary events
Life threatening infections
Gallstones
Leg ulcers
Skeletal complications
Bilirubin stones
Risks associated with chronic transfusions
Renal abnormalities
Pica
Priapism
Delayed growth and sexual maturation
Disease carries a host of complications, the severity of which depends on the sickle cell genotype, the existence of comorbid illnesses, adherence to medication, and psychosocial factors
Anemia: most common feature of SCD- sickle cells break up too easily and survive for only days, compared to 120 for normal red blood cells. The rapid destruction of sickle cells leads to anemia, diffiuclt for body to make up new red blood cells fast enough to keep up with the needed supply (may causefatigue, paleness,- sickle
yellowing of the skin and eyes (jaundice), shortness of breath
Pain: Most common symptom, discuss more in a bit
Cerebrovascular Accidents (Stroke) activity reported in 5-10% of individuals before age 20, silent strokes often under-identified
Acute pulmonary events are a major cause of mortality and hospitalization “Acute Chest Syndrome”- life threatening complication, caused by trapped sickle cells or infection in lung
Poor/absent spleen function can prevent clearance of bacterial organisms life-threatening infections
Skeletal complications- include weakened bones that easily fracture and degenerative joint disease, involve chronic pain and disability
Bilirubin stones can occur as result of increased blood cell turnover in SCD, cause pain
Priapism: prolonged painful erections due to sickling within the penile tissues
Retarded growth and delayed puberty

6. The Experience of Pain
VISCERAL REACTION..
THE NAMING OF STRESS….

7. Biopsychosocial Model
Interplay among…
Biological
Physical aspects of pain
Psychological
Mental, emotional, behavioral aspects
Social
Interactions with others (family, providers, peers, culture, etc.)
biopsychosocial is a holistic perspective in that mind and body are seen as automatically intertwined. Many would add soul/spirit.
Ecological factor – environmental factors
APS recommendations
A comprehensive biopsychosocial clinical assessment should be done yearly, and more often for patients with frequent pain
Within a developmental framework, assessment and treatment of pain should be optimized early to provide a foundation for building further constructive pain management interventions as individuals proceed through life
Important to consider the social context for this disease in America…Dr. Vinchinsky’s talk
In this country, a disease that is associated with a marginalized group and in health care training, marginalized attention. This interplay has implications for patient’s disease management.
Disparities in health care
Historical mistrust of providers (Tuskegee) 7

8. Not all Pain is SCD Pain
Chronic pain can be understood as a Biopsychosocial Pain Disorder
Requiring a Biopsychosocial Plan of Care
Comprehensive
Integrative
Multidisciplinary
Vicious Cycles
One unfortunate way in which these elements often interact is to create vicious cycles. For example:
Pain sensations (Sensory) often result in decreased physical activity (Actions).
Decreased physical activities can create feelings of depression (Emotional) and decreased self-worth (Mental).
The depression can lead to decreased motivation and initiative and even less activity (Actions).
Decreased activity, accompanied by withdrawal from other people (Social), leads to even more depression (Emotional), preoccupation with negative thoughts (Mental), and increased awareness of pain (Sensory).
Positive Cycles
Fortunately, the interactions among these elements can work in a positive way as well. Even though it is difficult to directly control your painful physical sensations and negative emotional reactions, you can significantly affect both by altering your thoughts and actions. That is why in our pain self-management program we place primary emphasis on the mental and action components of the Biopsychosocial Model.

9. Biopsychosocioculturalspiritual Approach
Interconnectedness, interrelatedness and/or interrelatedness when considering pain and the developing person within context.
How do these areas contribute to and/or compromise the experience of pain and inform treatment?
Related to the mind body spirit approach
Differs from mind body spirit in the emphasis upon the social and relational emphasis, which includes the spiritual and religious aspects of the the person’s world
Some are proposing an ecobiopsychosocial model.
biopsychosocial is a holistic perspective in that mind and body are seen as automatically intertwined. Many would add soul/spirit.
Ecological factor – environmental factors
APS recommendations
A comprehensive biopsychosocial clinical assessment should be done yearly, and more often for patients with frequent pain
Within a developmental framework, assessment and treatment of pain should be optimized early to provide a foundation for building further constructive pain management interventions as individuals proceed through life
Important to consider the social context for this disease in America…Dr. Vinchinsky’s talk
In this country, a disease that is associated with a marginalized group and in health care training, marginalized attention. This interplay has implications for patient’s disease management.
Disparities in health care
Historical mistrust of providers (Tuskegee)

10. Common Mental Health Issues
Depression or Sadness
Anger in Children
Anxiety or Worries
Pica (eating non-food items)
Attention problems (ADD)
Hyperactivity & Impulsive
Trauma
includes some medical procedures
Anxiety that they are dying
Continued medical trauma
Cumulative impact
Psychological and physiological compromised resilency

11. Family Centered Practice, June 8, 2007
Regional Child Abuse Prevention Councils 2011 11

12. Now think of our children

13. Remember Goff’s research on how Black children are perceived…not as children, bigger than they are, not accorded the benefit of innocence and sense to protect them

14. Living with SCD Spoken Word Kristyl J. L. Smith Los Angeles
San Diego University