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Psychosocial Aspects of Living with Gaucher Disease Wendy Packman, JD, PhD Professor Director, Pediatric Psychology and JD-PhD Program in Psychology and.

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Presentation on theme: "Psychosocial Aspects of Living with Gaucher Disease Wendy Packman, JD, PhD Professor Director, Pediatric Psychology and JD-PhD Program in Psychology and."— Presentation transcript:

1 Psychosocial Aspects of Living with Gaucher Disease Wendy Packman, JD, PhD Professor Director, Pediatric Psychology and JD-PhD Program in Psychology and Law Pacific Graduate School of Psychology at Palo Alto University Gaucher Patient Meeting: 2014

2 Outline   Psychosocial issues in inborn errors (IEM): general concepts   Research: Gaucher disease (GD)   Quantitative findings   Qualitative findings   Coping strategies   Psychosocial and neurocognitive issues in GD: Pediatric patients

3 Psychosocial Issues   IEM are chronic diseases   Infancy through adulthood   Affect family system   Clinical presentations of IEM are variable:   Metabolic decompensation   Neurological symptoms   Multi-system organ involvement   Developmental delays   Behavioral problems

4 Sources of Psychological Stress   For Patients:   Secondary to disease pathology and toxicity   From stressors inherent in living with chronic illness   For Parents and Families:   Having a loved one with an inborn error (Emotional & financial stress, loss of friends, lack of freedom, medication management) From: Weber S, Segal S, & Packman W (2012), Molec Genet Metab 105:

5 Focus of Our Research   What are the psychosocial stressors in a given inborn error?   What are the reactions to the stressors in a given disorder?

6 Disorders Studied in Our Research   MSUD   Galactosemia   PKU   Hunter syndrome [MPS II]   Niemann-Pick B   Fabry disease   Gaucher disease

7 Psychological Complications of Patients with Gaucher Disease W. Packman, T. Crosbie, A. Riesner, C. Fairley, & S. Packman J Inherit Metab Dis 2006; 29(1):

8 Psychological Aspects of Patients with GD: Research Questions 1. Is there a typical psychological profile associated with GD? 2. How does this compare with profiles of other patients with chronic illnesses? 3. Are psychological profiles different for individuals related to severity of symptoms and length of time on ERT? 4. What is the psychological impact of GD on psychosocial functioning?

9 Psychological Aspects of Patients with GD   Demographic questionnaire   Minnesota Multiphasic Personality Inventory (MMPI-2 )   567 true-false items to assess personality features   Used in chronic illness and chronic pain populations to assess psychological functioning   Semi-structured interview   Initial reaction to diagnosis of GD   Daily hardships as a result of their condition   Perceived quality of life   Relationships with family and friends, stressors

10 GD symptoms Percent Enlarged spleen 82 Enlarged liver 71 Anemia (low blood count)71 Bone pain 71 Fatigue61 Nose bleeds/bleeding problems 57 Frequent bruising 57 Enlarged abdomen 43 Fractures25 Liver problems 18 Symptom Characteristics of GD Patients (n = 28)

11 MMPI-2 Inventory

12 MMPI: Validity Scales The validity scales include:   Lie scale (L), which measures the tendency to present oneself in a positive light   Infrequency (F) scale, which detects unusual or atypical responses to the test questions   Correction (K) scale, which measures test defensiveness and problem denial

13 MMPI: Clinical Scales 1 1 (Hs) Hypochondriasis 2 2 (D) Depression 3 3 (Hy) Hysteria 4 4 (Pd)Psychopathic deviate 5 5 (Mf)Masculinity-femininity 6 6 (Pa)Paranoia 7 7 (Pt)Psychasthenia 8 8 (Sc)Schizophrenia 9 9 (Ma)Hypomania 0(Si) Social introversion

14 Findings from MMPI-2

15 Comparison of Psychological Profiles Between GD Participants and Normative Sample  Patients with GD scored significantly higher than MMPI-2 normative sample on MMPI-2 scales  Validity (K)  Hypochondriasis (Hs)  Depression (D)  Hysteria (Hy)  Psychasthenia (Pt)  Schizophrenia (Sc)

16 MMPI-2 Findings   Clinical scales (Hs, D, Hy) suggest reactions that are common among chronically ill individuals: Somatic concerns, sadness Emotional distress, tension Under stressful situations -- increase in physical symptoms and suffering. Comparable to scores of patients with other IEM and chronic disorders

17 Percentage of Participants with GD Scoring in the Normal, Moderately or Markedly Elevated Range on MMPI-2

18 MMPI-2 Findings   Clinical scales (Hs, D, Hy) suggest reactions that are common among chronically ill individuals:   Somatic concerns, sadness   Emotional distress, tension   Under stressful situations -- increase in physical symptoms and suffering   Comparable to scores of patients with other IEM and chronic disorders

19 Findings from Interviews From: Packman, Crosbie, Behnken, Eudy, & Packman (2010). Am J Medical Genetics 152A,

20 Initial Reaction to Diagnosis of GD (N = 28) Which of the reactions was the strongest for you? Feelings Other Shock Concern Sadness Relief Disappointment Guilt Anger Fear Percent

21 Coping with the Diagnosis   Feelings of relief -- the naming of the illness finally put an end to the frightening and ambiguous questions that surrounded their physical health. Several participants stated: “it was good to have an answer.’’   The diagnosis of GD also helped to rule out potentially more fatal illnesses such as leukemia and lymphoma.   Concern or shock were also prominent reactions.

22 “What is your average amount of physical pain attributed to GD?” Several individuals emphasized problems juggling the demands of work with a chronic ‘‘full-time illness.’’ Bone crises forced one man to leave work and go on disability. For many, GD kept people from moving forward in their career or dictated career choice because of physical limitations. Several individuals emphasized problems juggling the demands of work with a chronic ‘‘full-time illness.’’ Bone crises forced one man to leave work and go on disability. For many, GD kept people from moving forward in their career or dictated career choice because of physical limitations. No pain 54% Little/Some 21 % Moderate 18 % Considerable 4 % Extreme pain 4 %

23 “ How has GD affected your career decisions?” “GD has affected me hugely. My first choice was to be a dancer. Second choice was pediatrician. I wanted to be a physician from the time I was little. I was always told I would not be able to do it physically.” year- old woman “GD has affected me hugely. My first choice was to be a dancer. Second choice was pediatrician. I wanted to be a physician from the time I was little. I was always told I would not be able to do it physically.” year- old woman No effect 46% Little/Some14% Moderate11% Considerable14% Extreme effect 14 %

24 “How has GD affected your ability to participate in recreational activities?” “My greatest hobby was sports. So, it essentially destroyed it. The activity which gave me the most satisfaction in life was movement and I can’t move very well. Not well enough to participate in sports, can’t hike anymore, or walk significant distances.” year-old man “My greatest hobby was sports. So, it essentially destroyed it. The activity which gave me the most satisfaction in life was movement and I can’t move very well. Not well enough to participate in sports, can’t hike anymore, or walk significant distances.” year-old man No effect 29% Little/Some18% Moderate18% Considerable29% Extreme effect 7 %

25 Psychological and Social Support Most Important Source of Support Source of Support Friends Religious group Children Extended family Spouse Percent

26 “How has GD affected your relationships with your immediate family?” Participants indicated that having GD deepened their family relationships, improved communication between family members, and that the family felt protective. Participants also described friends as supportive and understanding. No effect54% Little/Some21% Moderate 4% Considerable18% Extreme effect 4%

27 Quality of Life   Currently on ERT: 82% (range 1 to 13 ys)   65% on ERT for 9+ ys   Quality of life since beginning ERT   Extreme improvement 35%   Considerable26%   Moderate 9%   Some17%   No improvement 13%

28 “How does your outlook on life compare with that of other people you know?” “I’m very optimistic. I think I appreciate life more than other people because of my experience with illness.” –20 year-old man “I’m very optimistic. I think I appreciate life more than other people because of my experience with illness.” –20 year-old man 7% Much better outlook 26% A little better 19% About the same 48% Quite a bit worse

29 Suggestions: Improving the Care of Individuals with GD   “Listen, feel like I’m being listened to. I think it would be helpful if the health profession didn’t project what they think they know about the disease.” –55 year-old woman   “I struggle with how do you have healthcare providers help with the frustrations of dealing with chronic illness? I guess I’m looking for empathy and acknowledgement of how frustrating it is to be sick so often.” – 59 year-old woman

30 Stressors   Major stressors include psychological turmoil:   Coping with uncertainties of a chronic illness   Coping with insurance   Impact of GD on career and recreational activities   Finances

31 Reactions   Anxiety, worries, increased stress (35%)   Sadness and depression (25%)   Somatic concerns as noted on MMPI-2   Pain and Physical injury   Existential fears

32 Stress-related Growth   GD had a positive influence on lives (70%)   Strong relationships with family and friends   Positive outlook on life   More empathic and compassionate   Optimistic   Able to see the bigger picture more than others   Happy with their own life

33 Coping Strategies  You are not your illness –What gives your life meaning?   Self-nurturing   Exercise   Stress-reducing Practices (Meditation, hypnosis)   Changing Thoughts and Feelings   Therapies (psychotherapy, complementary medicine [acupuncture])   Support Groups

34 You Are Not Your Illness   Tendency to become so identified with the disease and its treatment that we forget who we are.   We are not out illnesses – we are still ourselves.   Separate yourself from GD -- begin to have control over it instead of being totally controlled by it.

35 Coping Strategies   Self-nurturing   Exercise   Stress-reducing Practices (Meditation, Hypnosis)   Changing Thoughts and Feelings   Therapies (psychotherapy, complementary medicine [acupuncture])   Support Groups   You are not your illness

36 Self Nurturing Rest -- Give yourself permission to rest Pleasant Activities – Engage in experiences and relationships that you value. “Sit down next to your joy. Find the people that bring out the best in you and listen to their talk and watch their walk. They live quietly among us, in our own families and circles of friends.” (Rumi)

37 Exercise within your limits Activity, Rest, and Pacing Pain leads to reduced mobility which leads to reduced fitness Find a personal balance Choose an activity you want to do and construct a realistic schedule (walking, cycling, chores)

38 Stress-reducing Practices Imagery, Meditation, Self-Hypnosis   Change from passive treatment approach to an active process of taking control and being in charge   Use as diversion, to lower stress, filter hurt out of pain

39 Changing Thoughts and Feelings- ABCD Model Activating Event or Stressor Beliefs (thoughts & attitudes about stressor ) Consequent Emotion Disputing (challenging negative thinking) Back pain while driving the car “Oh no, my back is going out again. I will have to pull over and will never get where I’ m going. I’ll be laid up now for weeks.” Fear, anxiety, depressed “It will be o.k.- I have managed this situation before and I can manage it again. I may have to take a break and be late. Last time this happened, I rested for a couple days and I was fine.” Boss yelled at me“How could I be so stupid. He probably wants to fire me. I can’t take much more of this. “ Shame, anger, crying, heart racing “I feel bad that he yelled at me. He’s probably having a really bad day. I’ll talk to him when we are both less stressed. Tonight, I will go to a movie with a friend.”

40 Therapies and Support Groups Psychotherapy   Cognitive-Behavioral Therapy (CBT) – emphasizes the role of thinking in how we feel and what we do   Psychodynamic or insight-oriented therapy: focuses on unconscious processes as they are manifested in a person’s present behavior Complementary medicine   Acupuncture Support Groups

41 The Psychosocial and Neurocognitive Impact of Gaucher Disease, Type 1 on Pediatric Patients and their Families Andrea Alioto and Wendy Packman, JD, PhD Palo Alto University

42 Psychosocial and Neurocognitive Impact of Gaucher Disease, Type 1 on Pediatric Patients and their Families Previous Research:   Hayes et al. (1998)   Health Related Quality of Life in 16 GD, Type 1 patients (8-67 ys)   Symptoms of bone pain and chronic fatigue interfered with academic, occupational, and social activities.   Caregivers gave similar responses about impact of symptoms and ERT on functioning   No study has independently examined psychosocial or cognitive effects in children.   No study has examined psychological well- being of parents or implications on family unit.

43 Study Goals:  To examine:  Health-Related quality of life  Psychological and neurocognitive functioning  Effect of ERT on symptoms and functioning  Psychological well-being of parent  Impact on family unit  Develop recommendations to improve quality of care.

44 Participants Children ages 5-18 diagnosed with GD, Type 1 and their parent(s). Children ages 5-18 diagnosed with GD, Type 1 and their parent(s). Recruitment: Recruitment: 2015 National Gaucher Conference National Gaucher Care Foundation Gaucher’s Association (UK)

45 Assessment Measures   Pediatric Quality of Life Inventory   PedsQL Pediatric Pain Questionnaire   Behavior Assessment System for Children   Behavior Rating Inventory of Executive Function   PedsQL Cognitive Functioning Scale   Profile of Mood States- 2 nd Edition   PedsQL Family Impact Module   Pediatric Inventory for Parents  Self-report measures (in person, mail, via Skype)


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