Planning an outing from hospital for ventilator-dependent children.

Returning ventilator-dependent children to the home environment has become a well-accepted occurrence. The success of a home program depends on careful pre-discharge planning in order to ensure the child's medical safety, and adequate preparation to ensure the child's and family's adjustment to an active community life after discharge. To achieve this, involvement in community activities must begin while the child is still in hospital. As part of a complete rehabilitation program, nine ventilator-dependent children were taken on an inpatient outing to Disneyland. The planning and goals of the outing are described.

Hypoventilation and apnea in children during mechanically assisted ventilation.

Uncuffed tracheostomy tubes are used for long-term mechanical ventilation in children. However, upper airway mechanics differ between sleep and wakefulness; this may affect air leak around tracheostomies. We studied 19 children with high cervical spinal cord injury on portable positive pressure ventilators, age range birth to 19 years. Ventilator settings were adjusted while awake to achieve PaCO2 less than 45 mm Hg and PO2 greater than 90 mm Hg. Clinically several children with uncuffed tracheostomies became unstable at night with seizures and sleep disruption. Nine of 11 children on volume controlled systems were found to be inadequately ventilated during sleep. Substitution with a cuffed tracheostomy allowed adequate ventilation both awake and asleep, suggesting that inadequate ventilation during sleep was due to an uncompensated leak around the uncuffed tracheostomy. To avoid cuffed tracheostomies, eight children received pressure controlled ventilation. Gas exchange was adequate throughout the day and night. We conclude that children receiving volume controlled mechanical ventilation via uncuffed tracheostomy tubes can exhibit hypoventilation due to uncompensated air leak. Pressure controlled ventilation improves adequacy of gas exchange during sleep and wakefulness.

Cardiac pacemaker in high spinal cord injury.

Bradycardia followed by cardiac arrest is well documented as a complication of acute injury to the cervical spinal cord. This life-threatening bradycardia is attributed to an imbalance in the autonomic nervous system resulting from dissociation of the parasympathetic from the sympathetic responses during the stage of spinal shock. A patient with a C2 complete quadriplegia secondary to birth trauma who experienced symptomatic bradycardia, continuing for 21 months after injury despite intensive medical management, is reported. Clinical improvement followed insertion of a cardiac pacemaker. Possible etiologies for the continuation of abnormal bradycardia episodes after the resolution of spinal shock are discussed. Cardiac pacemaker implantation is advocated for patients with high cervical spinal cord injuries and continuing symptomatic bradycardia not responding to medical measures.

Decline in respiratory function and experience with long-term assisted ventilation in advanced Duchenne's muscular dystrophy.

We present 17 patients with advanced DMD who required long-term assisted ventilation. Eleven patients used part-time assisted ventilation. Five of the patients received BV and/or M-IPPV or N-IPPV between two and nine years before requiring full-time T-IPPV, while six others initially used part-time T-IPPV. One patient used all three modes before requiring full-time T-IPPV. Mean (+/- SD) FVC and rebreathe PCO2 at the outset of assisted ventilation were 0.62 +/- 0.20 L and 47.4 +/- 7.5 mm Hg, respectively. Clinical features were divided between symptoms suggesting respiratory muscle fatigue and sleep-related disordered breathing. We found that, while useful in early respiratory insufficiency, BV is associated with recurrent aspiration. In our experience, N-IPPV offers the safest and most convenient form of noninvasive ventilation. When the VC has decreased to about 300 ml, most patients will require full-time ventilation; T-IPPV is advised to provide airway access to suction secretions.

Long-term ventilatory support in spinal muscular atrophy.

Before ethical issues regarding prolonging life in patients with degenerative disease can be considered, the quality of life with medical intervention must be delineated. We have followed 15 patients with spinal muscular atrophy who have been treated with mechanical ventilation. They have received assisted ventilation for an average of 8 years 10 months (range 5 months to 23 years 10 months). Three of the patients required full-time ventilator assistance at the time of initiation of ventilation; the remaining 12 used nighttime ventilation for an average of 8 years 7 months. Nine patients continue to receive nighttime ventilation only. Two patients died after 5 years and 14 years of assisted ventilation, respectively. Of the 10 patients more than 18 years of age, three graduated from college, two are in college, three graduated from high school, and two completed eleventh grade. One patient is a mother of a healthy child. Two patients are employed, and two others have found fulfilling volunteer work. Ventilator support has not significantly interfered with these patients' plans and expectations.

Spinal cord insults and heterotopic ossification in the pediatric population.

Fifteen of 152 pediatric patients with spinal cord insults (10%) developed heterotopic ossification (HO) at 19 locations. The average age of the patient was eight and one-half years. The spinal cord levels were 13 thoracic and two cervical. The average time to detection of the HO from spinal insult was six and one-half years. The hip was involved in 15 of 19 HO lesions. Decreased range of motion of the affected extremity was the most common sign of occurrence. Alkaline phosphatase was elevated in five of eight patients at the time of detection. Three patients had some resorption of the HO, and one had nearly complete resorption. Five patients (3.3%) with HO had no other etiologic agent other than the neurologic insult, and their average age at time of injury was 13 and one-half years. The hip was involved in six of seven instances. The average time to diagnose this HO was 14 months after injury. Ten patients had late concurrent etiologic factors such as surgery, decubitus ulcers, late neurogenic hip dislocation, and late acute local trauma influencing HO formation. Pediatric patients who developed HO appeared to have a lower incidence, delayed onset, and fewer associated signs and symptoms compared with their adult counterparts with spinal cord injury. Patterns of ossification about the hip differ from adults. The HO lesion has the potential to resorb. HO may be initiated years after the spinal injury by an incidental insult.

Functional activities in spinal muscular atrophy patients after spinal fusion.

Spinal muscular atrophy patients present with weakness, greater in the proximal muscles, leading to scoliosis and limited upper extremity function. The purpose of this study is to identify unique aspects of these patients and to understand how spinal fusion affects their function. Forty patients underwent Harrington or Luque rod instrumentation with functional evaluations preoperatively and 2 and 5 years postoperatively. Biomechanical assessment of function is important. Flexibility of the spine is functionally advantageous because distal strength is used to align weaker proximal segments. Postoperatively, lack of spinal flexibility resulted in a decline in gross motor function and increased use of UE aids due to a change in the trunk position in the weaker patients. The stronger patients' activities were maintained. Earlier mobilization in patients with Luque procedures did not improve postoperative function.

Patient and family participation in the management of respiratory failure in Duchenne's muscular dystrophy.

Mechanical ventilation is now considered a therapeutic option for respiratory failure associated with Duchenne's muscular dystrophy (DMD). Through the monitoring of forced vital capacity and PCO2, prediction of impending respiratory failure is possible. This knowledge allows the patient to choose institution of mechanical ventilation before acute respiratory failure or no intervention and preparation for a natural death. To assist patients and families in this decision making, a special clinic was established. Thirty adolescent boys with DMD were followed up. Eighteen boys reached the end stage of their disease. Three died of cardiac failure. Prediction of respiratory failure was possible in 14 of the remaining 15; 11 of the 15 were able to make educated choices regarding respirator assistance or a natural death. The clinic has shown that in the majority of cases, when properly educated, patients and families can be active participants in life-and-death decisions.

Gated radionuclide ventriculography in the evaluation of cardiac function in Duchenne's muscular dystrophy.

Left ventricular ejection fractions were determined in 38 patients with Duchenne's muscular dystrophy. No significant correlation between the severity of respiratory dysfunction or age and cardiac function was seen. We suggest that the cardiac status of each patient should be evaluated separately from his respiratory status, particularly when long-term assisted ventilation is being considered.

Neck breathing: a form of voluntary respiration for the spine-injured ventilator-dependent quadriplegic child.

Children with respirator-dependent quadriplegia because of C-2 spinal cord injuries are now surviving the acute stages of their injury. The major cause of mortality and morbidity in the chronic stage is due to respiratory complications. Surveillance, 24 h/d, is the best way to prevent accidental disconnection of respirator equipment from the patient and its inherent catastrophic consequences. The constant risk of disconnection adds tension to the home environment and takes away from the patient any degree of independence or privacy. Because of this, an alternative method of respiration using neck accessory muscles was developed to restore a patient-controlled, voluntary system of respiration. This method, neck breathing, is described in detail in seven children varying in age from 3 years to 16 years 3 months. All seven patients had complete paralysis of the intercostal muscles and the diaphragm. An eighth patient who was unable to learn the technique is also described. Neck breathing is also compared to glossopharygeal breathing, an alternative method of respiration developed during the polio era.