Supplemental Tube Feeding Research Articles

Optimization of Exocrine Pancreatic Insufficiency in Pancreatic Adenocarcinoma Patients.

This study explores the optimization of exocrine pancreatic insufficiency (EPI) management in pancreatic adenocarcinoma patients, focusing on the scientific advancements and technological interventions available to improve patient outcomes, including oral pancreatic enzyme replacement therapy (PERT) and immobilized lipase cartridge (RELiZORB®). This was a prospective Institutional Review Board (IRB)-approved study from October 2019 through to August 2021 at the Louisville Medical Center in collaboration with Norton Healthcare and the University of Louisville Division of Surgical Oncology. Patients with a diagnosis of pancreatic adenocarcinoma (Stage 2 or 3) who underwent oncologic surgical resection were included in this study. Patients were contacted at pre-defined intervals (prior to surgery, before hospital discharge, and 2, 4, 6, and 12 weeks after surgery) to complete nutrition evaluation, EPI assessment, and quality of life questionnaires to identify the severity and frequency of gastrointestinal (GI) symptoms. EPI symptoms were reported in 28 of the 35 total patients studied (80%). Jejunostomy tubes were placed during oncologic surgery in 25 of the 35 total patients studied (71%), and 12 of the 25 patients with a jejunostomy tube utilized enzyme cartridges to manage EPI symptoms while on supplemental tube feeding (48%). EPI symptoms were reported in 8 of the 10 patients without a feeding tube (80%), and their EPI symptoms were managed with PERT alone. EPI interventions, both oral PERT and immobilized cartridges, were associated with a decrease in EPI symptoms after surgery and improved quality of life (QOL). Overall, early optimization of EPI is crucial to enhance overall patient care, return to oncology therapy after surgery, and improve quality of life in pancreatic adenocarcinoma patients.

Feeding Variation Among Infants in Acute Care Cardiology Units.

Infants with heart disease are at high risk of feeding difficulties and complications. Feeding practices amongst acute care cardiology units are not standardized. This study aims to describe feeding practices for infants at the time of discharge from a Pediatric Acute Care Cardiology Collaborative (PAC3) center and practice variation between centers. Discharge encounters for infants in the PAC3 registry between February 2019 and October 2021 were included. Nutrition type and feeding route at discharge were summarized with descriptive statistics and a modified bump plot. Center variation was assessed using funnel plots with control limits set at the 99.9% confidence interval from the group mean. A total of 15,414 encounters across 24 PAC3 centers were recorded from 8313 unique patients (median encounters 1, range 1-25). Nutrition at discharge consisted of standard formula in 8368 (54%), human milk in 6300 (41%), and elemental formula in 3230 (21%), either alone or in combination. Feeds were fortified to ≥ 24kcal/oz in 12,359 (80%). Discharge supplemental tube feeding was present in 7353 (48%) encounters with 4643 (63%) receiving continuous feeds, 2144 (29%) bolus feeds, and 566 (8%) a combination. Funnel plots demonstrated variability in nutrition type and feeding route at discharge. Infants with heart disease commonly require high calorie nutrition and supplemental tube feedings at discharge. Feeding strategies at discharge vary widely between PAC3 centers. Collaborative approaches to identify best practices in feeding strategies are needed.

Supplemental tube feeding: qualitative study of patient perspectives in advanced pancreatic cancer

ObjectivesMalnutrition is associated with poor quality of life and survival outcomes for patients with cancer, but is challenging to prevent or treat in pancreatic cancer due to the multifactorial drivers...

Association of in-line digestive enzyme cartridge with enteral feeds on improvement in anthropometrics among pediatric patients with cystic fibrosis.

Approximately 85% of patients with cystic fibrosis (CF) have exocrine pancreatic insufficiency (EPI) with 10% requiring supplemental nighttime enteral tube feedings. Administration of pancreatic enzyme replacement therapy (PERT) with nighttime feedings is fraught with challenges. RELiZORB (Alcresta Therapeutics, Inc), an in-line lipase cartridge, delivers PERT continuously with enteral feedings. Outcomes related to the use of this in-line lipase cartridge are lesser known. This project evaluated anthropometrics related to in-line lipase cartridge use among pediatric patients with CF already receiving oral PERT therapy prior to nighttime enteral feedings. Retrospective chart review was performed on 29 patients with CF and EPI receiving supplemental tube feedings and utilizing in-line lipase cartridge for a continuous 12 month period between 2015 and 2019. Anthropometrics were evaluated 12 months before and after initiation of in-line lipase cartridge. Compared with mean height z score at 6-months pre-in-line lipase cartridge, mean height z score at 6-months post-in-line-lipase cartridge (adjusted mean difference [AMD] = 0.2540; 95% CI = [0.0487, 0.4592]; P = 0.0153) and mean height z score at 12-months post-in-line lipase cartridge (AMD = 0.2684; 95% CI = [0.0203, 0.5166]; P = 0.0340) were significantly higher. Mean weight z score at 12-months post-in-line-lipase-cartridge neared statistical significance compared with 6-months pre-in-line lipase cartridge (AMD = 0.2816; 95% CI = [-0.0003, 0.5634]; P = 0.0502) when excluding seven patients with advanced lung disease (forced expiratory volume in the first second of expiration of 40%). Weight-for-length or body mass index did not significantly differ compared with pre-in-line lipase cartridge. Use of in-line lipase cartridge with enteral feeds improved anthropometrics, especially height, in pediatric patients with CF.

Functional Laryngectomy and Quality of Life in Survivors of Head and Neck Cancer With Intractable Aspiration

Late effects of head and neck cancer (HNC) treatment include profound dysphagia, chronic aspiration, and death. Functional laryngectomy (FL) can improve patient survival and quality of life (QoL); however, removing a failing larynx for a noncancer reason is a difficult decision. Data regarding the ability of FL to improve self-perceptions of voice, swallowing, and QOL in survivors of HNC with intractable aspiration are inconclusive. To investigate the association of FL with changes in self-reported perceptions of voice, swallowing, oral intake, QoL, and mood in survivors of HNC experiencing profound dysphagia and intractable aspiration. This cohort study was conducted at a single academic institution and included survivors of HNC with profound swallowing dysfunction and intractable aspiration who underwent FL from July 2016 through March 2022. Of the initial 22 patients enrolled, 2 patients (15%) died of aspiration pneumonia before receiving FL. Data analyses were performed from July 2016 through March 2023. Self-reported measures of voice using the VHI (30-item Voice Handicap Index), swallowing using the EAT-10 (10-item Eating Assessment Tool), functional oral intake scale using the FOIS (Functional Oral Intake Scale), and quality of life using the FACT-H&N (Functional Assessment of Cancer Therapy-Head & Neck) were assessed before FL and at 1, 3, and 6 months after FL. Mood states were evaluated using the POMS (Profile of Mood States, second edition), before FL and at 6 months after FL. The study analyses included 20 patients (mean [SD] age, 72.4 (7.0) years; 19 [95%] males and 1 [5%] female) who underwent FL and had complete data across all time points. Among these, 12 patients (60%) had received chemoradiation for oropharyngeal, 7 (35%) for laryngeal, or 1 (5%) for nasopharyngeal cancer. The mean (SD) time from completion of oncologic treatment to FL was 15.5 (5.5) years. Mean (SD) score on the EAT-10 improved from 33.2 (7.4) to 23.1 (10.8) at 1 month; 12.1 (9.1) at 3 months; and 8.3 (7.4) at 6 months, with a large effect size (η2 = 0.72; 95% CI, 0.54-0.80). Mean (SD) score on the FOIS improved from 2.0 (1.5) to 2.9 (1.7) at 1 month; 4.8 (2.5) at 3 months; and 5.2 (1.7) at 6 months, with a large effect size (η2 = 0.6; 95% CI, 0.38-0.71). Improvement in oral intake was achieved in 19 patients (95%), and feeding tubes were removed in 10 of 16 patients (63%) who were feeding tube-dependent; 6 patients (27%) continued to require supplemental tube feedings. Mean (SD) score on the VHI improved from 63.6 (34.0) to 86.9 (33.7) at 1 month; 71.3 (36.1) at 3 months; and 39.7 (26.9) at 6 months, with a large effect size (η2 = 0.42; 95% CI, 0.19-0.56). Seventeen patients (85%) were able to use a tracheoesophageal voice prosthesis for alaryngeal communication. Mean (SD) score on the FACT-H&N improved from 86.2 (17.8) to 93.6 (18.4) at 1 month; 109.0 (18.4) at 3 months; and 121.0 (16.8) at 6 months, with a large effect size (η2 = 0.64; 95% CI, 0.42-0.74). Mean (SD) score on the POMS improved from 58.9 (13.2) at baseline to 44.5 (9.9) at 6 months, with a large effect size (Cohen d = 1.04; 95% CI, 0.48-1.57). None of the patients experienced major complications of FL; 1 patient (5%) had a postoperative pharyngocutaneous fistula. The findings of this cohort study indicate that FL was associated with marked improvements in self-perception of voice and swallowing, functional oral intake, QoL, and mood state among survivors of HNC. These findings can serve as a framework for FL counseling among HNC survivors experiencing profound dysphagia and intractable aspiration.

Assessing the Association Between Pre-operative Feeding and the Development of Oral Feeding Skills in Infants with Single Ventricle Heart Disease: An Analysis of the NPC-QIC Dataset.

Pre-operative feeding may improve long-term feeding outcomes in single ventricle patients, including weaning from supplemental tube feedings in infancy. This study examines the association between pre-operative enteral feeding and subsequent long-term feeding outcomes while also assessing the counterbalancing risk of necrotizing enterocolitis (NEC). Secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative database was performed. The association between pre-operative feeding practice and achieving all oral feeds through the first year of life was examined using a multivariable regression model. Similarly, the association between pre-operative oral feeding and NEC was also assessed. Of 944 patients with 1-year feeding outcomes available, 58% were fed preoperatively (41.3% exclusively oral) and 12.3% were not fed per institutional approach. At hospital discharge after Stage 1 palliation, 57% required a feeding tube, while 39% required a feeding tube at their first birthday. In infants who were orally fed, the odds ratio to achieving tube-free feeding at 1year was not significantly increased (1.3, confidence interval 0.8-2.0). Of 1740 infants with pre-operative feeding and Stage 1 there was no statistically significant difference in NEC among patients who were preoperatively fed versus those that were not fed per institutional approach (p = 0.2). Pre-operative feeding of infants with single ventricle heart disease was not associated with early achievement of tube-free feeding in the first year of life. However, pre-operative oral feeding was also not associated with increased risk of NEC, suggesting that it can be safely offered among appropriate patients.

Gastrostomy Tube Placement in Neonates Undergoing Congenital Heart Surgery: A Novel Assessment for Improving Utilization and Timing of Placement.

Neonates undergoing congenital heart defect repair require optimized nutritional support in the perioperative period. Utilization of a gastrostomy tube is not infrequent, yet optimal timing for placement is ill-defined. The objective of this study was to identify characteristics of patients whose postoperative course included gastrostomy tube placement to facilitate supplemental tube feeding following neonatal repair of congenital heart defects. A single-institution, retrospective chart review identified 64 consecutive neonates who underwent cardiac operations from 2012 to 2016. Perioperative variables were evaluated for significance in relation to gastrostomy tube placement. A total of 27 (42%) underwent gastrostomy tube placement. Diagnosis of a genetic syndrome was associated with the likelihood of placement of gastrostomy tube (P = .032), as were patients with single ventricle physiology (P = .0013) compared to those felt to be amenable to eventual biventricular repair. Aortic arch reconstruction (P = .029), as well as the need for delayed sternal closure (P = .05), was associated with increased frequency of gastrostomy tube placement. Postoperative outcomes including the number of days intubated (P = .0026) and the presence of significant dysphagia (P = .0034) were associated with gastrostomy placement. Additionally, genetic syndrome (P = .003), aortic arch reconstruction (P = .01), and postoperative intubation duration (P = .0024) correlated with increased length of stay, where increased length of stay was associated with gastrostomy tube placement (P = .0004). Patient characteristics that were associated with a high likelihood of eventual gastrostomy placement were identified in this study. Early recognition of such characteristics in future patients may allow for reduced time to gastrostomy tube placement, which in turn may improve perioperative growth and outcomes.

Supplemental enteral tube feeding nutrition after hospital discharge of esophageal cancer patients who have undergone esophagectomy

BackgroundAfter undergoing esophagectomy to treat esophageal cancer, there are changes in the normal intake patterns in most patients, with more than half found to have an inadequate oral intake at the time of their hospital discharge. However, the use of home supplemental enteral tube feeding nutrition after hospital discharge in esophagectomy patients has yet to be established. The aim of this study was to evaluate the feasibility of 90-day home supplemental enteral tube feeding nutrition in esophagectomy patients.MethodsThis single-center, prospective, and single-arm study evaluated the feasibility of using supplemental tube feeding nutrition intervention for 90 days in esophageal cancer patients who have undergone esophagectomy.ResultsThis study enrolled 24 post-esophagectomy patients between February 2015 and September 2016. Twenty patients were administered 70% or more of the planned nutrient, with 83% of the patients completing the nutritional intervention procedure. There were no grade 3/4 adverse events observed, with a mean body weight change of − 7.6 ± 6.0%.ConclusionsOur results showed that routine use of 90-day home supplemental enteral tube feeding nutrition after hospital discharge for esophagectomy patients was both feasible and acceptable.Trial registrationUMIN000016286.

Neurodevelopmental Intervention Strategies to Improve Oral Feeding Skills in Infants With Congenital Heart Defects

Purpose Full-term infants with congenital heart defects (CHD) are at high risk for developmental and feeding difficulties secondary to a complex combination of immature neurological structures, early surgical intervention, postsurgical complications, and disruption in typical care. Infants with CHD often present with neurobehavioral immaturity that resembles that of premature infants, resulting in difficulties in achieving stability in the physiological and behavioral subsystems. This results in poor arousal, muscle tone abnormalities, and poor state regulation, which affect their ability to achieve oral feeding readiness and progress in their oral feeding skills. In fact, a significant number of infants with CHD require supplemental tube feeding upon hospital discharge to meet their nutritional needs. However, despite these unique barriers, the feeding experience for infants with CHD is frequently overlooked and the focus tends to be on higher level oral motor and swallowing skills and volume intake. Conclusion Given the growing evidence of neurological immaturity and subsequent neurodevelopmental delays in this population, it is crucial for therapists to recognize neurobehavioral stability as a foundation for a complex neuromotor activity such as oral feeding. This article will discuss how to utilize infant neurobehavioral intervention as part of feeding treatment for children with CHD to help develop appropriate neural pathways for oral feeding.

Sucking patterns are not predictive of further feeding development in healthy preterm infants

Preterm infants are at risk of experiencing difficulties in their feeding development. For a possible early identification of these, we examined the association between sucking patterns, assisted spoon feeding, and chewing skills in 40 healthy preterm infants, and the role of experience in the acquisition of these skills in a prospective longitudinal study. Sucking patterns were evaluated at 34, 37, and 44 weeks postmenstrual age (PMA), assisted spoon feeding was evaluated at 6, 9, and 12 months PMA, and chewing was evaluated at 9, 12, and 24 months PMA. Sucking patterns were rated using the Neonatal Oral Motor Assessment Scale, assisted spoon feeding was rated using the Observation List Spoon Feeding, and chewing was evaluated using the Mastication Observation and Evaluation Instrument.All infants showed progression in their oral motor skills during the study period, but not all demonstrated a stable progression of skills. Quality of sucking patterns was not associated with skill level achievement of assisted spoon feeding or with chewing. Length of experience in sucking was significantly associated with duration of supplemental tube feeding but not with the quality of the sucking pattern. Length of chewing experience was significantly associated with chewing abilities at 9 and 12 months PMA. No clinical characteristics were sufficiently predictive of oral motor skill development. Results show that sucking patterns are not predictive of subsequent feeding development in healthy preterm infants. The food consistency offered and the length of feeding experience influenced the acquisition and quality of oral motor skills differently.

P338 Efficacy of supplemental nocturnal tube feeding in adult cystic fibrosis patients with respiratory failure

P338 Efficacy of supplemental nocturnal tube feeding in adult cystic fibrosis patients with respiratory failure

Safety of Appetite Manipulation in Children with Feeding Disorders Admitted to an Inpatient Feeding Program.

Appetite manipulation can be effective in weaning children off gastrostomy tube feeding dependence but can cause dehydration, hypoglycaemia, and ketone body production, which is anorexigenic. As the safety of this approach has not been described, our aim was to describe adverse events observed when weaning children from G-tube dependence using our appetite manipulation protocol. This was a retrospective study of prospectively collected data of patients who completed our inpatient tube-weaning protocol. Daily safety parameters included twice-daily urine specific gravities and urine ketones and fasting capillary blood glucose. Graded clinical interventions to manage adverse events were collected. A total of 143 children with a mean age of 4.8 ± 2.4 years were seen in the inpatient feeding program of which 74 (51.7%) were male. The children were hospitalized 10.1 ± 2.5 days with the vast majority being discharged between days 11 and 14. Overall, 78.2% of patients experienced at least 1 adverse event: urine specific gravity >1.020 was seen in 60.5%, ketonuria in 48.9%, and hypoglycemia (≤60 mg/dL) in 13.4%. Only 2 children had blood glucose levels <40 mg/dL and these were corrected with oral supplementation. Graded clinical interventions to manage adverse events included oral rehydration in 89.9% of children and supplemental tube feeding in 25.2%. Adverse effects are common when appetite manipulation is used to wean children off G-tube dependence. Anticipating, monitoring, and having a clear intervention plan in a closely monitored setting are necessary to safely use this method.

Enteral tube feeding in adult patients with cystic fibrosis and respiratory failure

To evaluate the efficiency of nocturnal hyperalimentation in adult patients with cystic fibrosis (CF) and respiratory failure. The investigation enrolled 17 patients older than 18 years (mean age, 25.6±4.2 years) diagnosed with very severe CF (forced expiratory volume in one second (FEV1), < 30%; body mass index (BMI), < 18.5 kg/m2); all the patients were on the waiting list for lung transplantation. Nutritional status and pulmonary function parameters, such as body weight, height, BMI, and FEV1, were measured at baseline, before and 6 and 9 months after tube feeding. The study group showed a considerable increase in body weight and BMI after 6 and 9 months. The change in lung function was statistically insignificant. Lung transplantation was successfully conducted in 5 patients; 4 died while on the waiting list; the cause of death was respiratory failure. Supplemental PEG tube feeding improves the nutritional status (BMI, body weight) of patients with very severe CF.

Closer Look at the Nutritional Outcomes of Patients After Primary Repair of Congenital Diaphragmatic Hernia.

We hypothesize that the patients after primary repair of congenital diaphragmatic hernia (CDH) can have poor nutritional outcomes and plan to identify risk factors to further stratify these patients. Retrospective cohort of patients who had primary repair of CDH between 2000 and 2014 and had follow-up at our institution. Z scores (weight for age and weight for length) were calculated using the World Health Organization and Centers for Disease Control and Prevention growth standards. For the 67 patients in the cohort, the median age at the time of repair was 3 days (interquartile range 2-5) and at the time of discharge was 20.5 days (interquartile range 16-30). Fifteen percent of the patients required supplemental tube feeding for inadequate oral intake and 69% required fortified feedings for inadequate growth at discharge (4 patients subsequently needed gastrostomy tube placement). The median z scores at discharge were -1.0 (-2.1 to -0.3) and -1.2 (-2.3 to -0.5) in weight for age and weight for length, respectively. The risk factors for low z scores included open repair and longer periods of postoperative intubation or hospitalization. The z scores were similar at 6 months of age compared to discharge, but then statistically improved at 12 months of age. Patients with primary repair of CDH are at risk for poor nutritional outcomes at the time of hospital discharge and require follow-up to ensure adequate growth. Patients at highest risk are those who had an open repair and had prolonged intubation or hospitalization.

Steven Johnson Syndrome and Toxic Epidermal Necrolysis in a burn unit: A 15-year experience

IntroductionThe diffuse epidermal exfoliation seen in Steven Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) is similar to skin loss in second degree burns, and many of these patients are referred for treatment at burn centers. Treatment can differ markedly from center to center, and mortality can range from 25% to 70%, including a considerable morbidity. However, our experience over a 15-year period from 2000 to 2015 with 40 patients found a mortality rate of only 10% (4/40). The purpose of this paper is to discuss our treatment algorithm as a model for other centers treating SJS/TENs patients. MethodsRecords were reviewed for all patients admitted to the LAC+USC burn unit between 2000 and 2015 and 40 patients were identified with biopsy-proven SJS or TENS. These cases were reviewed for age, gender, initial and greatest TBSA, causative drug, pre-existing medical conditions, and morbidity and mortality. All data were entered into the SPSS statistical software package and all statistical analyses were performed using this program. ResultsOur treatment algorithm focused on early referral to a specialty burn unit, immediate discontinuation of the offending drug, fluid resuscitation, nutritional supplementation, and meticulous wound care. Average time to transfer to a burn unit was 3.36 days. Silver-releasing antimicrobial dressings were applied to the affected skin surface and changed every 3 days. Mupirocin coated petroleum gauze was used for facial involvement. Steroids were tapered and discontinued if initiated at an outside facility (58% of patients), and starting after 2001, all patients received a course of IVIG. All patients received fluid resuscitation and the majority received supplemental tube feedings (69%). Average length of total stay was 17.1 days and length of ICU stay 15.9 days. While 44% were transferred to another facility for further rehabilitative care, 37% of patients discharge to home. In patients discharged home with complete resolution of skin lesions, time to healing was an average of 14 days. DiscussionWith our 10% mortality rate in 40 patients, our study represents a relatively large study population while maintaining a relatively low mortality rate. The demographic data from our study largely aligns with the existing literature, and we therefore feel that our low mortality rate is due to our treatment algorithm, rather than to a less severe pathology in our patient population. This claim is supported by a standard mortality ratio of 1.68. This ratio proves a significantly improved mortality than would be expected based on disease severity on admission.

Considerations on the late-preterm newborn with cardiac problems

Focus on the late preterm birth (LPB) and critical congenital heart defects (CCHD) Data on outcomes of CCHD specifically in late preterm infants are exceedingly scarce. The preterm newborn with associated complex CHD presents many intricate problems to clinicians who take care (neonatologist, cardiologist, cardiothoracic surgeon). Therapies used for the preterm neonate without heart disease frequently need to be altered in the presence of heart disease and those needed for management of heart disease may need to be altered because of prematurity. The population of newborn born preterm is at higher risk for postoperative death and short and long term complications than term newborn with the same CHD and the causes of this are multifactorial. Technical issue related to small cardiac structure, immaturity of other organ systems, decreased nutritional and cardiac reserve, increased risks of bleeding, abnormal chest wall mechanics after sternotomy can all together account for this epidemiologic data. In past years these findings were related in particular to low-birth weight infants and conventional management was oriented to await a threshold weight in order to reduce bypass-related morbidities. From 1990 rates of LPB have risen and now account for 75% of all preterm births. Whether late preterm infants with CHD, who may be only marginally more immature than their term counterparts, remain at risk for adverse outcome in unclear. Some recent papers do demonstrate the independent effect of LPB on mortality and morbidity if a CHD is present and this issue emerge as an important factor for a correct counseling. Natarajan have observed that the weight at surgical intervention was significantly lower and age higher in the late preterm infants compared to those delivered at term. Late preterm infants had significantly higher rates of NEC and seizures, with a greater risk of supplemental oxygen and tube feeding at discharge. Costello have examined mortality and morbidity stratified by gestational age, reporting for LPB group with CHD an hospital mortality rate of 16.4% compared to 2.6-8% in the term group. The exact mechanism of this vulnerability remains unclear although functional immaturity of the lung and the brain are plausible aetiologies. Altered intrinsic cardiac mechanism could play a role in adverse outcome of late preterm with CHD. Gestational age at delivery can be a risk factor over which clinicians might have some control. Before birth the challenge is planning the time and the place for the delivery of a foetus with CHD. After birth the challenge is not waiting for obtain an arbitrary correct weight for surgery but working in a complex team to discuss each case in order to maximize outcome. Domande inerenti la relazione del Dott. Stefano Fiocchi

Risk and Prevalence of Developmental Delay in Young Children With Congenital Heart Disease

Children with congenital heart disease (CHD) are at risk for developmental delay (DD). Changes in cognitive, language, and motor skills in early childhood have not been described. We report the results of a structured approach using longitudinal testing to identify problems and ensure early intervention in accordance with published guidelines. Bayley Scales of Infant Development, Third Edition, were used to assess cognitive, language, and motor skills in 99 children with CHD. Subjects were evaluated 3 to 6 times in the first 3 years of life. DD was defined as scores >1 SD below the population mean. Cardiac anatomy was single ventricle (1V) in 34 subjects and 2 ventricles (2V) in 65. Medical comorbidities were present in 21% and genetic syndromes in 19%. Most subjects (75%) had DD in ≥1 area at ≥1 assessments. Subjects with 1V anatomy had equivalent outcomes to those with 2V. Cognitive and language scores declined in subjects with genetic syndromes but were stable and within the average range for subjects with 1V and 2V. Motor scores improved for subjects with 1V and 2V but remained low for those with genetic syndromes. In addition to age, need for supplemental tube feeding, longer cardiopulmonary bypass time, and shorter time since last hospitalization were significant predictors of developmental outcomes. DDs in young children with CHD are both common and dynamic. Providers should encourage longitudinal surveillance for children with CHD because exposure to risk and prevalence of DD change over time.

Supplemental Tube Feeding Does Not Mitigate Weight Loss in Infants with Shunt-Dependent Single-Ventricle Physiology

Infants with shunt-dependent single-ventricle (SV) physiology are at risk for poor weight gain before superior cavopulmonary connection (SCPC). Lower weight-for-age z-score is a risk factor for prolonged length of stay (LOS) after SCPC. We sought to characterize infant growth and feeding and determine the effect of method of feeding on outcomes. Chart review of infants with shunt-dependent SV physiology born between October 2007 and September 2010 was performed. The cohort was divided into three groups based on feeding method at discharge after initial palliation; 53 in the oral feeding (PO) group, 56 in the nasogastric (NG) tube group, and 26 in the gastrostomy tube (GT) group. Birth weight z-score did not differ among groups (p = 0.39), but infants fed by NG or GT were smaller than PO-fed infants at hospital discharge (p = 0.0001), a difference that persisted through SCPC (p < 0.0001). Predictors of need for GT included Norwood procedure (p = 0.008) and longer LOS after initial palliation (p < 0.001). Interstage mortality and age at SCPC did not differ among groups. Risk factors for longer LOS at SCPC included longer LOS and need for supplemental feeds at discharge from initial palliation as well as lower weight at SCPC. Poor growth is common among infants with shunt-dependent SV physiology. Infants who require GT have lower weight-for-age z-scores at hospital discharge and remain smaller at SCPC than those fed PO. Although GT after initial palliation is associated with longer LOS after SCPC, it is not associated with an increase in interstage morbidity or mortality.

Cystic Fibrosis-Related Diabetes in Adults: Inpatient Management of 121 Patients during 410 Admissions

With improved longevity, cystic fibrosis (CF)-related diabetes (CFRD) has emerged as the most common nonpulmonary complication of CF. Patients with CFRD are frequently admitted to the hospital with infections and deterioration of pulmonary function, during which time glycemic control might have an impact on pulmonary function, recovery from infection, and survival. In an attempt to share our insight into inpatient management of CFRD, this article summarizes the experience of our inpatient glucose management team with hospital management of 121 adult CFRD patients who were hospitalized on 410 occasions at the University of Colorado Hospital between January 2009 and September 2011. This is a retrospective chart review descriptive study of inpatient management of CFRD in our center. Our cohort includes CFRD patients treated with basal and mealtime insulin through multiple daily injections or continuous subcutaneous insulin infusion (CSII), as well as patients receiving steroids or enteral nutrition, which adds complexity to the management of CFRD during hospitalization. Multiple hospitalizations and intensive inpatient management of CF are integral elements of treatment. Inpatient therapy for CFRD requires a customized approach that is uniquely different from that of type 1 or type 2 diabetes. Our experience highlights clinical circumstances such as irregular food intake, high dose steroid therapy, and supplemental tube feeding. For many patients, it is possible to continue CSII therapy during hospitalization through a combination of mutual trust between the patient and hospital staff and oversight provided by the glucose management team.

Peripheral nerve block in a patient with amyotrophic lateral sclerosis

To the Editor: We have used lumbar plexus and sciatic nerve blocks in an ALS patient with a collum femoris fracture. The patient was 55-year-old female who had been followed-up for the past 1.5 years for ALS with bulbar onset and upper and lower motor neuron involvement. On physical examination, jaw opening was limited, the patient was dysphonic, and, because of dysphagia, a nasogastric tube was inserted. Preoperative ALS severity score was 17 (speech, 5: frequent repeating required; swallowing, 4: supplemental tube feeding; lower extremity and walking, 4: able to support weight; upper extremity dressing and hygiene, 4: attendant assists patient) [1]. Her ALSSS score the first month postoperatively was 13. The anatomical reference points were marked for lumbar plexus and sciatic nerve blocks by the posterior approach (Winnie’s technique). An 8-cm, 17-gauge Touhy needle (Arrow, USA) was inserted and attached to a nerve stimulator (Stimuplex HNS 11, BBraun). When twitch of the quadriceps muscle and patella at 0.5 mA current was observed, a 60-cm stimulating peripheral nerve catheter (Stimucath, Arrow, International PA, USA) was placed by the lumbar plexus. A mixture of local anesthetics comprising 15 ml 0.25% levobupivacaine (Chirocaine 0.5%, Abbott, Turkey) ? 10 ml 1% prilocaine (Citanest 2%, Astra Zeneca, Turkey) was administered to the lumbar plexus via the catheter, monitoring the nerve motor response. Then, a 21-gauge, 100 mm long Stimuplex needle (Stimuplex A, BBraun, Germany) was inserted to the sciatic nerve sheet. A 0.5 mA current was given by nerve stimulator (Stimuplex HNS 11, BBraun) until plantar flexion was observed then a mixture of 10 ml 0.25% levobupivacaine and 10 ml 1% prilocaine was injected. At 20 min of the procedure, motor block score using the bromage scale was 3, and at 25 min the sensory level (T10) was determined by pin-prick and cold tests. At the fourth hour postoperatively the motor block resolved. Follow-up control visit at 3 months showed no neurological progression in terms of ALS. In neurological diseases such as ALS, regional techniques are relatively contraindicated because of mechanical trauma induced by needle or catheter, nerve ischemia as a result of supplementation of vasoconstrictor agents, and toxic effects of local anesthetics. However, more recently, researchers argue that regional techniques may be used in patients with neurological disease on the basis of risk– benefit analysis [2]. In ALS, peripheral nerve blocks have advantages over other anesthesia techniques in terms of prevention of respiratory failure due to weakness of the respiratory muscles, protection of the laryngeal reflexes, and maintaining of the hemodynamic stability [3]. Lumbar plexus and sciatic nerve blocks have disadvantages attributable to being technically challenging, late onset of anesthesia compared with neuraxial anesthesia, insufficient anesthesia, and toxicity due to use of more local anesthetic than for neuraxial anesthesia. In conclusion, we suggest that peripheral nerve blocks can be chosen as an alternative technique to neuraxial blocks and general anesthesia in ALS patients undergoing surgery on their extremities. Although we performed peripheral nerve block with a stimulating nerve catheter, ultrasound-guided nerve block with nerve stimulation could be best for regional anesthesia in ALS patients. N. Sertoz (&) S. Karaman Department of Anesthesiology and Reanimation, Ege University, School of Medicine, Izmir 35100, Turkey e-mail: nezihsertoz@mynet.com