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

The Face of Sickle Cell Disease

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

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

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 10-20 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

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

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

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.

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)

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

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

Now think of our children

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

Living with SCD Spoken Word Kristyl J. L. Smith Los Angeles San Diego University https://www.youtube.com/watch?v=2u5iCvmi-Sc