Background/Purpose:
Childhood‐onset lupus (cSLE) is a chronic autoimmune disease and its effect on health‐related quality of life (HRQoL) has not been systematically established. The Patient Reported ...Outcomes Measurement Information System (PROMIS™, http://nihpromis.org) is a publicly available system to measure patient reported outcomes that features electronic data collection. Although several validated legacy QoL measures exist for cSLE, each is longer than the PROMIS™ Pediatric Short Forms (Short Forms). The objective of this study was to investigate the feasibility, construct and discriminant validity of the Short Forms in cSLE in a clinical setting.
Methods:
In this ongoing study at two sites, 98 of 100 projected patients completed the Pediatric PROMIS™ Short Forms (Anger, Anxiety, Depressive, Fatigue, Mobility, Upper Extremity, Pain Interference, Peer Relations) and legacy QoL measures (Pediatric Quality of Life Inventory™ Generic Core GC & Rheumatology Modules RM, Simple Measure Of Impact Of Lupus Erythematosus In Youngsters SMILEY, Childhood Health Assessment Questionnaire CHAQ, Child Health Questionnaire CHQ, pain and well‐being visual analog scales VAS). Questionnaire scores were compared and Spearman correlation analysis was performed in support of the construct validity of the Short Forms when used in cSLE. The discriminant validity was supported by analysis of PROMIS™ and legacy QoL score change at sequential visits and relationship of change was made with Spearman correlation analysis (to be shown).
Results:
Participants (78% female; 41% White, 44% Black, 6% Asian) had a mean age of 16 yrs (SD 3) and mean SLEDAI score of 6.07 (SD 5.99). There were no problems with completion of any of the PROMIS™ Short Forms (mean score = 50, clinically relevant difference = 10) which required 5–10 minutes in total (legacy QoL tools >15 min. each). On average, cSLE patients scored importantly worse in the Short Form assessing upper extremity function and mobility than the healthy children, while the other QoL domains were less affected (Table ). This is also supported by the scores of the CHQ–PHS. Concurrent validity of the Short Forms is supported by moderate correlations with the scores of various legacy measures (to be shown).
Comparative scores for HRQoL in cSLE*
PROMIS Short forms
CHAQ
PedsQL‐GC
PedsQL‐RM
SMILEY
CHQ‐P50
Anger
52.0 (4.3)
Emotional
74.1 (21.1)
Worry
70.0 (27.6)
Effect
63.3 (19.9)
Behavior (BE)
83.4 (15.9)
Anxiety
49.6 (3.8)
Treatment
80.8 (15.0)
Burden
60.1 (17.4)
Mental Health (MH)
77.5 (15.9)
Depression
51.9 (3.2)
Self‐esteem (SE)
81.8 (15.5)
Fatigue
53.5 (3.4)
Role/Social Limits‐Physical (RP)
87.5 (25.8)
Mobility
42.8 (3.5)
Walk
0.3 (0.6)
Physical
74.0 (20.3)
Activity
87.0 (17.7)
Limitation
68.2 (17.0)
Physical Function (PF)
78.2 (30.5)
Arise
0.4 (0.6)
Hygiene
0.3 (0.7)
Play
0.7 (0.9)
Upper Extremity Function
46.1 (3.9)
Dress & Groom
0.3 (0.7)
Physical Summary (PHS)
43.0 (13.2)
Eat
0.3 (0.7)
Reach
0.5 (0.8)
Grip
0.4 (0.7)
Pain
52.7 (3.1)
Pain
66.1 (28.2)
Bodily Pain/Discomfort (BP)
65.7 (29.7)
Peer Relations
46.7 (3.5)
Social
82.4 (20.0)
Social
72.8 (18.6)
Social & Emotional Limits (REB)
86.0 (29.3)
General Health Perception (GH)
56.6 (14.9)
School
66.0 (20.3)
Communicate
71.6 (27.6)
Psychosocial Summary (PSS)
52.8 (7.9)
Completed by Child
Completed by Parent
Values are means (standard deviations).
The PROMIS™ short forms use T score distributions (50 represents the average for the US general population, with a standard deviation of 10. High scores represent more of the concept being measured (for negatively‐worded concepts like pain, fatigue, and anxiety, a 60 is one standard deviation worse than average; for positively‐worded concepts like physical or peer relationships, a 60 is one standard deviation better than average).
Conclusion:
This pilot study supports QoL measurement using the PROMIS™ Short Forms as feasible and concurrently valid. PROMIS™ measures can now be utilized by clinicians treating cSLE for a more efficient patient reported health outcomes measure, taking advantage of their easy interpretation of scores and changes in scores, thereby, reducing respondent burden and making QoL assessment feasible in both research and clinical care settings.
Background/Purpose:
Recently, 26 quality indicators (QI) categorized into 9 domains for childhood‐onset systemic lupus erythematous (cSLE) have been developed based on international consensus and ...scientific evidence. QI are defined as minimum standards of medical care in support of optimal disease outcomes. The current level at which these QI are followed has not been well documented. Hence, the objective of this study is to assess the current quality of medical care received by patients with cSLE at tertiary pediatric rheumatology centers.
Methods:
Cross‐sectional data pertaining to the QI were acquired via chart review and analyzed collectively in 290 cSLE patients followed at four tertiary pediatric rheumatology centers—two in the United States and two in Brazil.
Results:
Adherence to the QI differed by QI domain, ranging from 41 to 100%. The QI domain with the highest adherence 100% was Laboratory Testing at the time of diagnosis, and the lowest domain 41% was Medication Management. The recommended kidney biopsies for newly diagnosed lupus nephritis were done in only 67% of the patients and 85% of the patients received at least one of the recommended vaccinations (Table ). Cardiovascular risks factors were discussed in 86% of cases when patient was 13 years or older. Adherence to the QI differences between countries and insurance status will be presented, supporting that the set of current QI are suitable for international use.
Adherence to QI by Domain
Quality Indicators by Domain
Results
Lab testing at diagnosis & screening
Obtained diagnostic/confirmatory labs within first two visits
100%
Obtained lab surveillance of CBC, renal, LFT every 12 months
85%
General prevention
Prescribed influenza and/or encapsulated organisms vaccination, unless contraindicated
85%
Discussed and documented education on sun avoidance at least once in the medical record (e.g., wearing protective clothing, applying sunscreens whenever outdoors, and avoiding sunbathing)
89%
Discussed transition plan to appropriate adult healthcare providers with patient age ≥14 years.
78%
Lupus nephritis (LN) and hypertension management
Kidney biopsy discussed/ordered/performed if developed proteinuria >500 mg/day, or worsening GFR, or urinary sediment
60%
Evaluated by a nephrologist in the last year of care for lupus nephritis (LN) and of hypertension
65%
Evaluated by rheumatologist every 3 months in last year if a patient has known LN regardless of disease activity.
96%
Received kidney biopsy when diagnosed with LN
67%
Class III/IV, treated with immunosuppressive + glucocorticoids within 1 month
92%
Angiotensin receptor blocker (ARB) or, Angiotensin–converting enzyme (ACE) inhibitor were prescribed if ongoing proteinuria >500 mg/day or worsening GFR in last year of care.
96%
Check assessments (Kidney function, urine sediment, and proteinuria) every three months.
99%
Medication Management
If started new meds, discussed risk vs. benefit of therapy
97%
Currently prescribed any antimalarial therapy
92%
Attempted to taper a dose of steroids not acceptable for chronic use.
89%
Attempted to taper and unable to decrease steroid; added/increased steroid sparing agent.
41%
Surveillance for medication safety done at regular intervals.
99%
Bone Health
Received at least one bone mineral density testing DEXA scan
72%
Repeat bone mineral density testing if baseline testing outside normal limits (Z score ≤2)
62%
Prescribed calcium/vitamin D if a patient is on any steroid therapy.
78%
Ophthalmological surveillance
Receives eye exams annually while on anti‐malarial therapy
82%
Receives eye exams annually while on glucocorticoids
85%
Education on cardiovascular risk factors
Education on cardiovascular risk factors (smoking, hypertension, high BMI) every 1 year with patient and parent if patient is 13 years or older.
86%
Discussed lifestyle modifications (smoking cessation, weight control, exercise) every 2 years with parent and patient 13 years or older.
87%
Pregnancy
Anti‐SSA, anti‐SB and anti‐phospholipid antibodies have been assessed during pregnancy
100%
CNS health
Prescribed Immunosuppressive therapy if patient has major neuropsychiatric manifestations in last year of care (Optic neuritis, coma, psychosis … etc.)
94%
Conclusion:
Based on this initial benchmarking effort, the medical care of patients with cSLE at tertiary pediatric rheumatology centers is very good. Systematic planning and documentation of patient education on lifestyle modifications seems warranted and is expected to improve the self‐management skills of cSLE patients. Furthermore, increased focus on bone health, eye exams annually, and adequate vaccination of cSLE patients are areas to continue to monitor.
Objectives
The aims of this study are (1) to characterize factors influencing self-management behaviors and quality of life in adolescent and young adult (AYA) patients with childhood-onset systemic ...lupus erythematosus (cSLE) and (2) to identify barriers and facilitators of treatment adherence via focus groups.
Methods
AYAs with cSLE ages 12–24 years and primary caregivers of the adolescents participated in this study. Recruitment occurred during pediatric rheumatology clinic visits at a Midwestern children's hospital or the hospital's cSLE active clinic registry. Information about disease severity was obtained from patient health records. Pain and fatigue questionnaires were administered. Descriptive statistics were used to analyze data.
Results
Thirty-one AYA patients and caregivers participated in six focus groups. Ten major themes emerged from sessions; four were expressed both by the AYA and caregiver groups: knowledge deficits about cSLE, symptoms limiting daily function, specifically mood and cognition/learning, barriers and facilitators of adherence, and worry about the future. Themes unique to AYA participants included symptoms limiting daily functioning—pain/fatigue, self-care and management, impact on personal relationships, and health care provider communication/relationship. For caregiver groups unique themes included need for school advocacy, disruption of family schedule, and sense of normalcy for their adolescent.
Conclusion
AYAs with cSLE face a lifelong disease characterized by pervasive pain, fatigue, organ damage, isolation—social and/or physical—and psycho-socioeducational challenges. This study confirmed that continued psychosocial support, health information education, adherence interventions, and personalized treatment plans are necessary to increase self-management and autonomy in AYAs with cSLE.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, OILJ, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
The term “duty to recontact” refers to the possible ethical and/or legal obligation of genetics service providers (GSPs) to recontact former patients about advances in research that might be relevant ...to them. Although currently this practice is not part of standard care, some argue that such an obligation may be established in the future. Little information is available, however, on the implications of this requirement, from the point of view of GSPs. To explore the opinions of genetics professionals on this issue, we sent a self-administered questionnaire to 1,000 randomly selected U.S. and Canadian members of the American Society of Human Genetics. We received 252 completed questionnaires. The major categories of respondents were physician geneticist (41%), Ph.D. geneticist (30%), and genetic counselor (18%); 72% of the total stated that they see patients. Respondents indicated that responsibility for staying in contact should be shared between health professionals and patients. Respondents were divided about whether recontacting patients should be the standard of care: 46% answered yes, 43% answered no, and 11% did not know. Those answering yes included 44% of physician geneticists, 53% of Ph.D. geneticists, and 31% of genetic counselors; answers were statistically independent of position or country of practice but were dependent on whether the respondent sees patients (43% answered yes) or not (54% answered yes). There also was a lack of consensus about the possible benefits and burdens of recontacting patients and about various alternative methods of informing patients about research advances. Analysis of qualitative data suggested that most respondents consider recontacting patients an ethically desirable, but not feasible, goal. Points to consider in the future development of guidelines for practice are presented.
Exertional heat illness (EHI) risk is a serious concern among athletes, laborers, and warfighters. US Governing organizations have established various activity modification guidelines (AMGs) and ...other risk mitigation plans to help ensure the health and safety of their workers. The extent of metabolic heat production and heat gain that ensue from their work are the core reasons for EHI in the aforementioned population. Therefore, the major focus of AMGs in all settings is to modulate the work intensity and duration with additional modification in adjustable extrinsic risk factors (e.g., clothing, equipment) and intrinsic risk factors (e.g., heat acclimatization, fitness, hydration status). Future studies should continue to integrate more physiological (e.g., valid body fluid balance, internal body temperature) and biometeorological factors (e.g., cumulative heat stress) to the existing heat risk assessment models to reduce the assumptions and limitations in them. Future interagency collaboration to advance heat mitigation plans among physically active population is desired to maximize the existing resources and data to facilitate advancement in AMGs for environmental heat.
Scope
A better understanding of factors contributing to interindividual variability in biomarkers of vitamin K can enhance the understanding of the equivocal role of vitamin K in cardiovascular ...disease. Based on the known biology of phylloquinone, the major form of vitamin K, it is hypothesized that plasma lipids contribute to the variable response of biomarkers of vitamin K metabolism to phylloquinone supplementation.
Methods and results
The association of plasma lipids and 27 lipid‐related genetic variants with the response of biomarkers of vitamin K metabolism is examined in a secondary analysis of data from a 3‐year phylloquinone supplementation trial in men (n = 66) and women (n = 85). Year 3 plasma triglycerides (TG), but not total cholesterol, LDL‐cholesterol, or HDL‐cholesterol, are associated with the plasma phylloquinone response (men: β = 1.01, p < 0.001, R2 = 0.34; women: β = 0.61, p = 0.008, R2 = 0.11; sex interaction p = 0.077). Four variants and the TG‐weighted genetic risk score are associated with the plasma phylloquinone response in men only. Plasma lipids are not associated with changes in biomarkers of vitamin K function (undercarboxylated osteocalcin and matrix gla protein) in either sex.
Conclusion
Plasma TG are an important determinant of the interindividual response of plasma phylloquinone to phylloquinone supplementation, but changes in biomarkers of vitamin K carboxylation are not influenced by lipids.
Contributions of lipids to interindividual variability in biomarkers of vitamin K were examined in a secondary analysis of data from a three‐year randomized phylloquinone (the major dietary and circulating form of vitamin K) supplementation trial. Plasma triglycerides contributed to the interindividual response of circulating phylloquinone, but interindividual changes in biomarkers of vitamin K carboxylation were not influenced by lipids.
Documenting human impacts on marine mammals is critical for understanding and mitigating harm. Although propeller strike injuries in small marine mammals are often debilitating and fatal, little is ...known about the occurrence or demographics of these types of injuries in pinniped populations. Using data of stranded harbor seals (Phoca vitulina) in the Salish Sea from 2002–19, we identified 27 cases of fatal propeller strikes. Weaned pups were the most frequently affected (64% of cases) with a much higher rate of propeller strikes than expected for the age class. Although they do represent animal welfare concerns, harbor seals in the Salish Sea probably are not threatened by these types of injuries at the population level; nevertheless, propeller strike cases increased significantly over the time of this study period, indicating increased interactions between boats and seals in the region. Continued monitoring and increased efforts to consistently quantify vessel traffic in the area are recommended to create and monitor long-term effectiveness of mitigation measures.
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