Sense of mastery and attitude towards illness: Examining longitudinal benefits of a medical specialty camp for youth with sickle cell disease.

Medical specialty camps can play an important role in the positive development of psychosocial outcomes for children and youth with sickle cell disease (SCD). This study examined how sense of mastery and attitude towards illness outcomes changed over 6 months for 100 campers aged 8-16 years with SCD. The outcomes were measured twice before and twice after camp. Latent growth curve modeling was used to analyze data. Results showed no changes in the outcomes for this study population. Implications for future research designs, populations, and outcomes are discussed, as are implications for communications about camp, and policy and practice.

SNAP: Supportive noninvasive ventilation for acute chest syndrome prevention in children with sickle cell disease.

BACKGROUND: Acute chest syndrome (ACS) is a leading cause of morbidity and mortality among children with sickle cell disease (SCD). Preventing hypoxemia by optimizing lung aeration during sleep remains a challenge. OBJECTIVES: To explore safety, feasibility, and tolerability of noninvasive, bi-level positive airway pressure ventilation (BiPAP) as preventative, supportive care for hospitalized, medically stable children with SCD on a general pediatric inpatient unit. METHODS: Retrospective chart review of patients ≤22 years of age with SCD admitted to the general pediatric inpatient unit from February 1, 2017 to March 1, 2020 for whom BiPAP was recommended as supportive care. Hospitalizations were excluded if patients were admitted to the pediatric intensive care unit (PICU), required BiPAP for respiratory failure, or used BiPAP at home for obstructive sleep apnea. RESULTS: Twenty-three patients had 53 hospitalizations in which BiPAP was recommended. Fifty-two (98%) hospitalizations included acute SCD pain. Indications for BiPAP included prior ACS (94%), chest or back pain (79%), and/or oxygen desaturation (66%). On 17 occasions, patients already had mild to moderate ACS but were stable when BiPAP was recommended. BiPAP was used successfully during 75% of hospitalizations for a median of two nights. There were no adverse effects associated with BiPAP. PICU transfer for respiratory support occurred during three hospitalizations. In 26 hospitalizations of children at risk for ACS who tolerated BiPAP, 23 (88%) did not develop ACS. CONCLUSIONS: BiPAP is safe, feasible, and well tolerated as supportive care for hospitalized children with SCD. Next steps include an intervention trial to further assess the efficacy of BiPAP on ACS prevention.

Social Determinants of Health and the Role of Routine Pediatric Care in a Medically Complex Toddler.

CASE: Late on a Friday afternoon, a new family presents to your practice for urgent care. They come with their youngest child Mai, a 2-year-old girl, who, although born in the United States at 36 weeks gestation, has resided in Laos with her grandparents for the past 16 months. Your triage nurse tells you that she has a fever and was found to have profound anemia while at the WIC (Special Supplemental Nutrition Program for Women, Infants, and Children) office earlier today.On walking into the room, you describe Mai as "listless" and "sickly." Her vitals were notable for fever (102°F), tachycardia (140 beats per minute), and tachypnea (35 breaths per minute). On physical examination, she was grunting with a systolic ejection murmur and without hepatosplenomegaly. Laboratory test results revealed hemoglobin of 2.2 g/dL, hematocrit of 12%, mean corpuscular volume of 50 fL, red cell distribution width of 27%, reticulocyte count of 3%, ferritin of <2 ng/mL, iron of 15 µg/dL, total iron binding count of 420 µg/dL, white blood cell count of 13.5 K/µL, and platelets of 605 K/µL. Her evaluation was consistent with severe iron deficiency anemia (IDA), which was further supported by reported restrictive diet and excessive cow milk intake of 35 ounces daily. She was admitted to the Pediatric Intensive Care Unit in high-output cardiac failure and was slowly transfused with 15 mL/kg of packed red blood cells over 2 days with careful monitoring. Once stabilized, she was transferred to the inpatient floor for further nutritional evaluation and supplementation. Additional workup, including hemoglobin electrophoresis, fecal occult blood test, celiac studies, and stool parasite testing were normal. The clinical picture was consistent with a viral infection in the setting of profound IDA and malnutrition.Although her clinical status had improved, she remained inpatient for nutritional optimization. Her height was at the 54th percentile (z-score: 0.11), weight was at the first percentile (z-score: -2.25), and body mass index was below the first percentile (z-score: -3.18), diagnostic of severe protein-calorie malnutrition. She was evaluated by an interdisciplinary growth and nutrition team, received multivitamin and mineral supplements, and was monitored for refeeding syndrome. She was noted to be "difficult to engage," "resistant to new faces," and made little progress on expanding her dietary choices. Concerns about a possible diagnosis of autism spectrum disorder were raised by her treating team. What would you do next? REFERENCE: 1. Bouma S. Diagnosing pediatric malnutrition: paradigm shifts of etiology-related definitions and appraisal of the indicators. Nutr Clin Pract. 2017;32:52-67.

Food Habits, Stressors, and Use of Complementary Medicine Therapies Among Pediatric Patients Who Attend an Integrative Medicine Pediatric Pain Clinic.

Purpose: Chronic pain is a growing problem among children and adolescents, and is more prevalent in low-income families. This observational study was conducted to describe the demographics and various descriptors, complementary medicine therapy (CMT) use, and lifestyle factors (i.e., food habits and supplement use) of pediatric patients with chronic pain. Methods: Boston Medical Center's Interdisciplinary Pediatric Pain Clinic provides patient education and unique treatment plans, tailored with medical and nonpharmacologic interventions for managing complex and chronic pain. Patient data were obtained through electronic medical chart review and self-reported surveys, and were completed by participants and parents at enrollment. Results: Of the 83 participants, the average age was 15.4 ± 4.3, and 80% were female. Referrals to the clinic were due to abdominal pain (52%), headache (23%), and musculoskeletal or other pain (25%). Thirty-one percent were on food assistance programs, with only 24% of patients currently using CMTs and 85% using pain medications. More than half of all participants (63%) missed up to 5 days of school in the past year, while 26% missed more than 6 days. School or academics (77%) were the most frequently self-reported stressor, followed by social/peer issues (39%), bullying (18%), and parental stress (18%). A very small proportion of patients had sufficient intake of dairy (12%), water (23%), vegetables (1%), and fruits (22%). Conclusion: Chronic pain can significantly affect lifestyle factors, stress, and child development. Patients evaluated at the clinic had poor diets and report having stressors regarding school, social/peer issues, parental stress, and bullying.

A cross-sectional review of the prevalence of integrative medicine in pediatric pain clinics across the United States.

PURPOSE: This project assesses the prevalence of integrative medicine (IM) in pediatric pain clinics (PPCs) across the United States. METHODS: PPCs were identified through the American Pain Society and cross referenced through the International Association for the Study of Pain (IASP). A cross-sectional review using each PPC's website was then utilized for further information. We collected data regarding each program's target population, non-profit status (where non-profits were designated as hospitals that do not operate for-profit purpose, and private as institutions receiving private funding), location, services provided and participating providers. Descriptive statistics were used for data analysis. RESULTS: Of the 53 PPCs identified, 43 (81%) were part of a non-profit healthcare organization, and 10 (19%) were within a private hospital; 85% were located in urban settings, 15% in rural settings; 83% were located in free-standing children's hospitals. Thirty-two (60%) PPCs utilized IM, including acupuncture (38%), mind-body (21%), massage (21%), aromatherapy (19%), nutrition counseling (17%) and/or art/music therapy (11%). The most prevalent providers within PPCs offering IM were yoga instructors (84%), nutritionists (56%) and mind-body specialists (44%). IM was offered in 63% of programs in non-profit organizations and 50% in private hospitals; 58% of urban sites and 75% of rural sites. Within each region, 91% (n = 10) of PPCs in the West offer IM, 53% of PPCs in the Midwest (n = 10) and Northeast (n = 8) offer IM and 50% (n = 4) of PPCs in the South offer IM compared to PPCs who do not. CONCLUSIONS: Of 53 current identified PPCs, over half offer IM services. While children in the US are more likely to find a PPC offering IM services, access to do so is more limited in rural and southern regions.

Provider Perspective on Integrative Medicine for Pediatric Sickle Cell Disease-related Pain.

OBJECTIVE: Integrative medicine (IM) approaches are sometimes used to manage sickle cell disease (SCD)-related pain. The purpose of this research is to (1) understand provider perspective towards the use of IM for pain in children with SCD at a large urban children's hospital and (2) provide recommendations on how to better promote IM for children and adolescents with SCD. METHODS: After approval from the institutional review board, a qualitative case study approach was used with criterion-type purposeful sampling to select providers from the division of hematology to adequately inform the study. Semistructured interviews were completed using audiotape to facilitate transcription. NVivo 10 analytic software (QSR International Pty Ltd, Doncaster, Victoria, Australia) was used to organize data into themes to answer the study questions. RESULTS: Ten provider interviews were completed. Attitudes were generally positive, and most providers felt that IM is generally helpful. All providers reported that they do not optimally use integrative therapies for children with SCD. The barriers uncovered focused on lack of process for integration of IM, specifically that IM resources seem transient and based on short-term funding. Provider attitude towards CAM is generally positive, but provider comfort level is highly variable. No providers are completely comfortable with their knowledge base about IM, and increased knowledge is desired. CONCLUSIONS: Creation of protocols and processes to incorporate IM into management plans for patients with SCD could help to promote its use. Education of providers about utility and efficacy of IM for SCD-related pain and about existing resources would aid in promotion of IM for children with SCD.

Low-dose ketamine as a potential adjuvant therapy for painful vaso-occlusive crises in sickle cell disease.

The hallmark of sickle cell disease (SCD) is the acute painful vaso-occlusive crisis (VOC). Among SCD patients, vaso-occlusive pain episodes vary in frequency and severity. Some patients rarely have painful crises, while others are admitted to the hospital multiple times in a year for parenteral analgesics. Opioids are the mainstay of therapy for SCD-related pain. However, a subset of patients report continued pain despite escalating doses of opioids. Tolerance and opioid-induced hyperalgesia (OIH) have been considered as possible explanations for this phenomenon. The activation of the N-methyl-d-aspartate (NMDA) receptor has been implicated in both tolerance and OIH. As a NMDA receptor agonist, ketamine has been shown to modulate opioid tolerance and OIH in animal models and clinical settings. Low-dose ketamine, by virtue of its NMDA receptor agonist activity, could be a useful adjuvant to opioid therapy in patients with refractory SCD-related pain. Based on limited studies of adjuvant ketamine use for pain management, low-dose ketamine continuous infusion appears safe. Further clinical investigations are warranted to fully support the use of low-dose ketamine infusion in patients with SCD-related pain.