ALBERT

Description: Nontuberculous mycobacteria (NTM) are ubiquitous free living soil and water– borne organisms that cause numerous clinical syndromes including lymphadenitis, skin, soft tissue and pulmonary infections, however disseminated infection is almost exclusively in patient with severe immunocompromise (i.e:HIV, Hematological malignancy, and bone marrow transplant). Mycobacterium avium complex (MAC) is hard to diagnose as it is considered slow grower NTM. We describe a case of disseminated Mycobacterium avium-intracellulare complex infection in teenager with sickle hemoglobinopathy with unique presentation mimicking pRBCs transfusion reaction. Patient presented on three different occasions with tachycardia, hypotension and fever within 2-24 hours following pRBCs pheresis, all three episodes were investigated and were negative for transfusion reactions. Patient had central venous catheter (CVC), frequent admissions for vaso-occlusive painful episode, on hydrocortisone for adrenal insufficiency and off Hydroxyurea for two months. Diagnosis of mycobacterium avium complex bacteremia was confirmed by two positive blood cultures, whole body CT scan showed liver nodules, spleen nodules and lung nodules. Pulmonary dissemination was confirmed by Biopsy and culture, Lymphocyte markers showed severe lymphopenia with absolute CD4 count of 64. Patient underwent treatment with three month of four antibiotics followed by 9 months of three antibiotics with removal of the central line, follow up scan showed decrease size and numbers of nodules, patient started tolerating pheresis within one month of the antibiotics initiation. NTM infection should be added to the list of pathogens in sickle cell patients with CVCs and fever and should be considered in frequent pRBC transfusion like reaction with negative workup. Routine blood culture can identify rapid growing NTM but specific mycobacterial blood culture is required in case of other NTM species (slow grower). As dissemination almost always occurs in those with impaired cellular immunity, HIV testing and lymphocyte markers should be performed Removal of involved CVCs is essential for the treatment as well as appropriate antimicrobial medications. Disclosures No relevant conflicts of interest to declare.

Description: Introduction: Living with sickle cell disease (SCD) and its associated complications and treatment can impact adolescent coping and adjustment, including peer relationships, participating in physical activities, and academic performance. Understanding the adolescent perspective on SCD and its impact on life activities will enable healthcare professionals to develop focused interventions to improve quality of life for adolescents and young adults (AYA) living with SCD. Objectives: To examine adolescent understanding of SCD and its impact on psychosocial functioning using exploratory qualitative research methodology. Methods: Twenty-one participants (ages 12-21 years old, median: 17 years, 12 female) with a diagnosis of sickle cell anemia (any variant) participated in IRB approved semi-structured interviews. Participants were recruited at routine sickle cell clinic visits and were on various treatment regimens such as chronic transfusions or Hydroxyurea. The interview protocol was piloted with AYA patients for content, understandability, and face validity. All interviews were recorded and transcribed by a single interviewer. All interview transcripts were independently coded by two researchers (In Vivo coding: codes are direct quotes) and required 100% agreement for a code to be included in the final codebook. Using the constant comparative method, codes were placed into categories and themes. A final construct of codes, categories, and themes was agreed upon by three researchers with expertise in psychology and SCD. Interviews did not continue past 21 participants since no new categories or themes were identified during the final interviews. Results: The overarching theme that emerged was the impact of SCD on psychosocial functioning of AYA as it related to coping and adjustment strategies. Three main subthemes relating to coping and adjustment were noted: social support, pain, and transition to adult care. Participants (20/21) reported sharing information about SCD with peers with and without SCD, with several expressing benefits to disclosure; “felt good to talk about it and get it off my chest,” “[friends] they help me out too. They get me water and take care of me.” All participants described benefits of developing relationships with other AYA with SCD and expressed a desire to meet peers with SCD; “it helps because they really know how you feel and what you go through,” “it would be helpful to hear about other people’s experiences and tips for dealing with things.” Participants (18/21) described their disease in terms of pain. Unpredictable pain events negatively impacted relationships with peers; “if I’m out and start feeling bad…it just reminds me that I’m not like everybody else.” Participants (2/21) described balancing taking medication to relieve pain and avoiding becoming dependent on pain medication. Primary concerns about transitioning to adult care included being viewed as drug-seeking by adult emergency department personnel and trusting an unfamiliar doctor’s judgment about their care and willingness to learn how their disease affected them as an individual. Surprisingly, 7/21 participants expressed some positive experiences from living with SCD including feeling “special,” “stronger and better,” and having a different perspective on life than peers without SCD. Conclusion: During adolescence, peer interactions play a major role in social and emotional development. Our findings strongly support the need for AYA with SCD to have opportunities to interact with healthy peers as well as those experiencing similar struggles related to SCD (SCD summer camps, social networking, teen support groups or mentoring). Participants identified pain as a defining characteristic of living with SCD and commented only on pharmacologic pain management. Participants expressed anxiety related to transitioning to adult care with particular focus on inadequate pain management at adult institutions. Our results suggest: 1) medical centers should focus on educating teens about pain and developing pain protocols that highlight teaching non-pharmacologic pain management strategies 2) improved pain management will positively impact quality of life and reduce anxiety about transition. Additionally, participants identified benefits to living with SCD, and future research should identify strategies to promote benefit finding behaviors in AYA with SCD. Disclosures No relevant conflicts of interest to declare.

Description: Introduction Patients with sickle cell disease (SCD) experience acute painful events; some patients develop chronic pain requiring daily short acting opioids, and frequent emergency department (ED) visits, and inpatient hospitalization. From the Cooperative Study of Sickle Disease (Platt, NEJM 1991), only 1% of patients with SCD experience more than 6 pain events per year. Methadone is a synthetic opioid used in chronic pain, but there is a paucity of data on its use and effectiveness in the pediatric SCD population. We hypothesized that methadone can be used safely in pediatric patients with SCD with severe chronic pain and would reduce ED visits and hospitalizations. Methods We conducted a retrospective cohort study (IRB approved) of 16 pediatric patients with SCD who received methadone for chronic pain management, indicating having more than 5 pain events per year. These patients were among 1100 total patients with SCD at our center. Primary SCD providers escalated doses to maximal effect and when clinically stable, doses were weaned. Follow-up started at time of methadone initiation and ended when a patient was weaned or transitioned to adult care; the 1 year period before treatment initiation was defined as the baseline period. We calculated descriptive statistics to characterize the study population. We compared clinical outcomes (ED visits or hospitalization for pain) for the baseline period and 1 year post methadone initiation using paired T test. To evaluate methadone dosing and dependency, we identified the mean dose, percentage of time at highest dose, and wean of methadone. We assessed withdrawal and safety by reviewing outpatient encounters, EKG screening for QTc 〉 450, eGFR by cystatin C to determine either a decrease in eGFR by 20% or an eGFR