Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension.

Children with severe Group 1 pulmonary arterial hypertension (PAH) have an unpredictable response to subcutaneous treprostinil (TRE) therapy, which may be influenced by age, disease severity, or other unknown variables at time of initiation. In this retrospective single-center cohort study, we hypothesized that younger age at TRE initiation, early hemodynamic response (a decrease in pulmonary vascular resistance by ≥30% at follow-up catheterization), and less severe baseline hemodynamics (Rp:Rs < 1.1) would each be associated with better clinical outcomes. In 40 pediatric patients with Group I PAH aged 17 days-18 years treated with subcutaneous TRE, younger age (cut-off of 6-years of age, AUC 0.824) at TRE initiation was associated with superior 5-year freedom from adverse events (94% vs. 39%, p = 0.002), better WHO functional class (I or II: 88% vs. 39% p = 0.003), and better echocardiographic indices of right ventricular function at most recent follow-up. Neither early hemodynamic response nor less severe baseline hemodynamics were associated with better outcomes. Patients who did not have a significant early hemodynamic response to TRE by first follow-up catheterization were unlikely to show subsequent improvement in PVRi (1/8, 13%). These findings may help clinicians counsel families and guide clinical decision making regarding the timing of advanced therapies.

Right Atrial Lines as Primary Access for Postoperative Pediatric Cardiac Patients.

To characterize the use of right atrial lines (RALs) as primary access in the postoperative care of neonatal and pediatric patients after cardiothoracic surgery and to identify risk factors associated with RAL complications. Observational retrospective cohort study in pediatric cardiac patients who underwent RAL placement in a tertiary children's hospital from January 2011 through June 2018. A total of 692 children with congenital heart disease underwent 815 RAL placements during the same or subsequent cardiothoracic surgeries during the study period. Median age and weight were 22 days (IQR 7-134) and 3.6 kg (IQR 3.1-5.3), respectively. Neonates accounted for 53.5% of patients and those with single-ventricle physiology were 35.4%. Palliation surgery (shunts, cavo-pulmonary connections, hybrid procedures, and pulmonary artery bandings) accounted for 38%. Survival to hospital discharge was 95.5%. Median RAL duration was 11 days (IQR 7-19) with a median RAL removal to hospital discharge time of 0 days (IQR 0-3). Thrombosis and migration were the most prevalent complications (1.7% each), followed by malfunction (1.4%) and infection (0.7%). Adverse events associated with complications were seen in 12 (1.4%) of these RAL placements: decrease in hemoglobin (n = 1), tamponade requiring pericardiocentesis (n = 3), pleural effusion requiring chest tube (n = 2), and need for antimicrobials (n = 6). Multivariable logistic regression showed that RAL duration (OR 1.01, p = 0.006) and palliation surgery (OR 2.38, p = 0.015) were significant and independent factors for complications. The use of RALs as primary access in postoperative pediatric cardiac patients seems to be feasible and safe. Our overall incidence of complications from prolonged use of RALs remained similar or lower to that reported with short-term use of these lines. While RAL duration and palliation surgeries seemed to be associated with complications, severity of illness could be a confounding factor. A prospective assessment of RAL complications may improve outcomes in this medically complex population.

Membrane Stabilizer Medications in the Treatment of Chronic Neuropathic Pain: a Comprehensive Review.

PURPOSE OF REVIEW: Neuropathic pain is often debilitating, severely limiting the daily lives of patients who are affected. Typically, neuropathic pain is difficult to manage and, as a result, leads to progression into a chronic condition that is, in many instances, refractory to medical management. RECENT FINDINGS: Gabapentinoids, belonging to the calcium channel blocking class of drugs, have shown good efficacy in the management of chronic pain and are thus commonly utilized as first-line therapy. Various sodium channel blocking drugs, belonging to the categories of anticonvulsants and local anesthetics, have demonstrated varying degrees of efficacy in the in the treatment of neurogenic pain. Though there is limited medical literature as to efficacy of any one drug, individualized multimodal therapy can provide significant analgesia to patients with chronic neuropathic pain.

An Update of Current Cannabis-Based Pharmaceuticals in Pain Medicine.

Cannabis users have long reported therapeutic properties of the plant for a variety of conditions, some of which include nausea, emesis, seizures, cancer, neurogenic diseases and pain control. Research has elucidated many cannabinoid pharmacodynamic and pharmacokinetic properties, expanding the potential use of cannabinoids as a medical therapy. Due to the inconsistent delivery and control of the active components involved with smoking, pharmaceutical companies are investigating and prioritizing routes other than smoke inhalation for therapeutic use of cannabinoids. In this relatively new field of pharmaceutical development, ongoing drug development promises great benefit from targeted endocannabinoid receptor agonism. Available in Canada and Europe, nabiximols, a specific extract from the Cannabis plant, has demonstrated great benefit in the treatment of pain related to spasticity in multiple sclerosis, cancer and otherwise chronic pain conditions. The cannabidiol oral solution Epidiolex®, which is available in the USA, is indicated for management of refractory epilepsy but may offer therapeutic relief to chronic pain conditions as well. Current investigative drugs, such as those developed by Cara Therapeutics and Zynerba Pharmaceuticals, are synthetic cannabinoids which show promise to specifically target neuropsychiatric conditions and chronic pain symptoms such as neuropathy and allodynia. The objective of this review is to provide clinicians with an update of currently available and promising developmental cannabis pharmaceutical derivatives which may stand to greatly benefit patients with otherwise difficult-to-treat chronic conditions.

Improving Patient Satisfaction in a Midsize Pediatric Hematology-Oncology Outpatient Clinic.

PURPOSE: The study of patient satisfaction is a rapidly emerging area of importance within health care. High levels of patient satisfaction are associated with exceptional physician-patient communication, superior patient compliance, reduced risk of medical malpractice, and economic benefit in the value-based purchasing era. To our knowledge, no previous reports have evaluated methods to improve the patient experience within the pediatric hematology-oncology (PHO) outpatient clinic. METHODS: Patient satisfaction was measured using returned Press-Ganey surveys at Blank Children's Hospital PHO outpatient clinic (UnityPoint Health). The aim of this study was to raise the overall patient satisfaction score to the 75th percentile and raise the care provider score (CP) to the 90th percentile nationally. After analyzing data from 2013, interventions were implemented in January 2014, including weekly review of returned surveys, review of goals and progress at monthly staff meetings, distribution of written materials addressing deficiencies, score transparency among providers, provider use of Web-based patient satisfaction training modules, devotion of additional efforts to address less satisfied demographics (new patient consultations), and more liberal use of service recovery techniques. RESULTS: In the PHO outpatient clinic, overall patient satisfaction improved from the 56th to 97th percentile. Care provider scores improved from the 70th to 99 th percentile. For new patients, overall satisfaction improved from the 27th to 92 nd percentile, and care provider scores improved from the 29th to 98 th percentile. CONCLUSION: Patient satisfaction was improved in a midsize PHO clinic by implementing provider- and staff-driven initiatives. A combination of minor behavioral changes among care providers and staff in conjunction with systems-related modifications drove improvement.