Risk management protocol for gastrostomy and jejunostomy insertion in ventilator dependent infants

Gastrostomy, gastrojejunostomy and anti-reflux surgery in infants and children who are chronically ventilator dependent are associated with significant risk of morbidity and mortality. We report outcomes of 22 high risk children who underwent these procedures at our centre. Pre-operative investigations included: overnight oxygen and carbon dioxide monitoring and subsequent optimisation of ventilatory support, echocardiography, video fluoroscopy, and assessment of gastroesophageal reflux. We carried out 24 procedures under general anaesthesia. Twenty-one children used ventilatory support pre-operatively. Median age of first surgical procedure was 18 months (range 3–180). Supplementary feeding was commenced in 20 children prior to procedure, median age 9 months (1–31). Median PICU length of stay was 1 (1–8) days. No children died in the post-operative period. Extubation was possible within 24 h in 87% of cases. Complications included; atelectasis (n = 2), ileus (n = 2), abdominal distension (n = 4) and loose stools (n = 1). We conclude that, in this high risk cohort of ventilator dependent children with predominantly neuromuscular disorders, with careful assessment, operative intervention can be carried out under general anaesthesia, with the child being extubated early back onto their routine ventilatory support and aggressive airway clearance. Additionally this protocol can minimise post-operative complications and is associated with a good outcome in the majority.     

The influence of age on heart rate variability during morning wakefulness

Objective  

Early morning wakefulness is associated with a peak in cardiac events. The influence of ageing on cardiac regulation during this time is unknown. This cross-sectional study of healthy men and women (n = 40, 20–30 and >60 years) investigated the effect of age on heart rate variability (HRV) during morning versus evening wakefulness and sleep.     

Sildenafil in pediatric pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a life-threatening disease of varied etiologies. Although PAH has no curative treatment, a greater understanding of pathophysiology, technological advances resulting in early diagnosis, and the availability of several newer drugs have improved the outlook for patients with PAH. Sildenafil is one of the therapeutic agents used extensively in the treatment of PAH in children, as an off-label drug. In 2012, the United States Food and Drug Administration (USFDA) issued a warning regarding the of use high-dose sildenafil in children with PAH. This has led to a peculiar situation where there is a paucity of approved therapies for the management of PAH in children and the use of the most extensively used drug being discouraged by the regulator. This article provides a review of the use of sildenafil in the treatment of PAH in children.KEY WORDS: Child, phosphodiesterase (PDE)-5 inhibitor, Pulmonary hypertension therapy     

Living and dying with respiratory failure: facilitating decision making

Respiratory failure is a predictable cause of death in end stage lung disease including COPD and neuromuscular disorders, and the symptom burden for these individuals in the last six months of life is significant. Palliative care services are less well-developed for patients with chronic disorders compared to those with malignant disease; and communication problems can be compounded by a mismatch in expectations between patients and health care providers on prognosis and other issues. Most patients want more information about their illness to facilitate participation in decision making. Noninvasive ventilation may extend life and palliate symptoms in neuromuscular disorders such as Duchenne muscular dystrophy and motor neurone disease/ALS.     

Evaluating a new strategy for prophylaxis to prevent Pneumocystis carinii pneumonia in HIV-exposed infants in Thailand. Bangkok Collaborative Perinatal HIV Transmission Study Group

OBJECTIVE: To evaluate a strategy for prophylaxis against Pneumocystis carinii pneumonia (PCP) for infants in Thailand. METHODS: HIV-infected women were offered trimethoprim-sulfamethoxazole for PCP prophylaxis for their children at 1-2 months of age. When the children reached 6 months of age, investigators simulated a decision to continue or stop prophylaxis on the basis of clinical criteria, and compared their decisions with results of polymerase chain reaction (PCR) testing for HIV. We calculated the proportions of children who received and completed prophylaxis, and compared the rates of pneumonia and death from pneumonia with rates from an earlier prospective cohort. RESULTS: Of 395 eligible infants, 383 (97%) started prophylaxis. By 6 months of age, 10 (2.6%) were lost to follow-up, three (0.8%) were non-adherent, seven (2%) had stopped because of adverse events, four (1%) had died, and 359 (94%) still received prophylaxis. At 6 months of age, 30 (70%) of 43 HIV-infected children and 16 (5%) of 316 uninfected children met the clinical criteria to continue prophylaxis. The incidence of pneumonia at 1 to 6 months of age was 22% (15/68) in the earlier cohort, and 13% (6/46) in the recent cohort [relative risk (RR) 0.6, 95% confidence interval (CI) 0.3-1.4; P= 0.22]; mortality rates were 9% and 4%, respectively (RR 0.5; 95% CI 0.1-2.3; P = 0.47). CONCLUSION: This PCP prophylaxis strategy appeared to be acceptable and safe, may have reduced morbidity and mortality from pneumonia, and should be considered in developing countries where early laboratory diagnosis of perinatal HIV infection is unavailable.     

Outcome of paediatric domiciliary mask ventilation in neuromuscular and skeletal disease.

Noninvasive positive pressure ventilation delivered by nasal mask or facemask has been used widely in the last decade to manage chronic ventilatory failure in adults with neuromuscular and chest wall disease. However, it has been thought that paediatric patients would not be able to tolerate masks, and previous anecdotal reports on the paediatric application of mask ventilation have not assessed the effects on nocturnal and arterial blood gas control. Domiciliary mask ventilation has been used in 40 children with ventilatory insufficiency due to congenital neuromuscular and skeletal disease aged 9 months-16 yrs. Eighteen patients had symptomatic nocturnal hypoventilation, 17 had diurnal ventilatory failure, three were referred for weaning and two had frequent chest infections associated with sleep-disordered breathing. Thirty eight of the 40 patients tolerated mask ventilatory support long-term. Diurnal mean+/-SD oxygen tension in arterial blood (Pa,O2) increased from 8.5+/-1.8-10.9+/-1.7 kPa (p<0.001) and mean carbon dioxide tension in arterial blood (Pa,CO2) fell from 7.0+/-1.6-5.9+/-0.8 kPa (p=0.01) following initiation of ventilatory support. Mean and minimum nocturnal Pa,O2 and peak transcutaneous carbon dioxide tension (Ptc,CO2) (n=21) improved significantly. Mask ventilation can be used successfully in young children and reverses ventilatory insufficiency due to congenital neuromuscular and skeletal disease.