- Collaborative generalist and subspecialist comanagement is the Medical Home model most likely to be successful for the care of children requiring chronic invasive ventilation.
- Standardized hospital discharge criteria are suggested.
- An awake, trained caregiver should be present at all times, and at least two family caregivers should be trained specifically for the child’s care.
- Home equipment requires proper maintenance of all equipment and regular review of ventilator settings.
Summary of Recommendations
- Recommendation 1: For children requiring chronic home invasive ventilation, we suggest a comprehensive Medical Home comanaged by the generalist and respiratory subspecialist.
(conditional recommendation; very low quality evidence)
- The Medical Home model can provide family-centered care for children with special health care needs, including children on home invasive ventilation. This recommendation places a high value on the possible medical and social benefits to this intervention and places low value on the potential risks, which may include increased provider time.
- Recommendation 2: For children requiring chronic invasive ventilation, we suggest the use of standardized discharge criteria to objectively assess readiness for care in the home.
(conditional recommendation; very low quality evidence)
- The Workgroup believed that comprehensive standardized discharge criteria would encourage a complete review of each patient’s medical stability and home situation to facilitate safe discharge. The goal is to identify and eliminate important barriers to care in the home before discharge and consider alternate care arrangements if obstacles cannot be eliminated. Weight given to each component of the proposed criteria would vary from patient to patient. The recommendation places high value on the potential benefits of considering all facets of a child’s care in the home before discharge and low value on the increased provider time and resource use that may be required.
- Recommendation 3a: We recommend that an awake and attentive trained caregiver be in the home of a child requiring chronic invasive ventilation at all times. (strong recommendation; very low quality evidence).
- Despite very low quality evidence supporting this recommendation, the Workgroup was confident that in this case the desirable consequences would clearly outweigh the undesirable consequences of following this recommendation. Lack of an awake and attentive trained caregiver would place the child in a life-threatening situation. Training of caregivers is irrelevant if one is not available to respond to an emergent situation. For most families this requires the support of a professional appropriately trained in-home caregiver to allow family caregivers time to sleep, work, and maintain a life balance. This recommendation places a high value on the safety of the patient, and low value is placed on avoiding the increased use of resources and the possible disruption to families who may need to accommodate a professional caregiver in their home.
- Recommendation 3b: For children requiring chronic invasive ventilation, we suggest that at least two specifically trained family caregivers are prepared to care for the child in the home.
(conditional recommendation; very low quality evidence)
- The experience of the Workgroup and available data indicate that a lone trained family caregiver would rarely be capable of shouldering the entire burden of care for a child using invasive ventilation in the home. This recommendation places high value on the safety of the patient and quality of life of caregivers and low value on increased resource use for training more than one caregiver.
- Recommendation 3c: We suggest that ongoing education to acquire, reinforce, and augment skills required for patient care be provided to both the family and professional caregivers of children requiring chronic home invasive ventilation. (conditional recommendation; very low quality evidence)
- The Workgroup believed, based on clinical experience, that practitioners and professional personnel agencies must strive to provide ongoing education to family and professional caregivers. Continuing education would help reinforce learned skills and allow training on new technologies and protocols. This recommendation places a high value on safety and on the potential clinical benefits to the patient and a low value on increased cost and resource use.
- Recommendation 4a: For children requiring chronic home invasive ventilation, we suggest monitoring, especially when the child is asleep or unobserved, with a pulse oximeter rather than use of a cardiorespiratory monitor or sole use of the ventilator alarms. (conditional recommendation; very low quality evidence)
- Small indirect studies and the experience of the Workgroup suggest that ventilator alarms may not always function correctly. Furthermore, hypoxemia is most likely to be the first indicator of a serious issue in a child with respiratory disease. The workgroup believes pulse oximetry is the preferred method for monitoring patients on home mechanical ventilation. This recommendation places high value on the safety of the child and low value on possible increase in caregiver burden secondary to false alarms.
- Recommendation 4b: For children requiring chronic home invasive ventilation, we recommend regular maintenance of home ventilators and all associated equipment as outlined by the manufacturer.
- Although states have differing regulatory requirements for DME providers, and the data supporting the value of equipment maintenance are lacking, the Workgroup believed strongly that maintenance of all home equipment by appropriately trained DME employees as recommended by the manufacturer should be standard of care. Care should be taken to assure that the actual ventilator settings as seen on the control panel match the prescribed settings. Twenty-four hour a day service and phone support must be available. This recommendation places a high value on the likely clinical benefits of properly functioning equipment programmed with the correct patient settings and low value on increased resource use.
- Recommendation 4c: We suggest the following pieces of equipment for use in the home when caring for a patient on home mechanical ventilation: the ventilator, a back-up ventilator, batteries, a self-inflating bag and mask, suctioning equipment (portable), heated humidifier, supplemental oxygen for emergency use, nebulizer, and a pulse oximeter (nonrecording). (conditional recommendation; very low quality evidence)
- On the basis of experience, the Workgroup believed the presence of specific pieces of equipment could prevent the development of life-threatening situations and/or reduce their severity. This recommendation places high value on the potential to avoid emergent situations due to the presence of important reserve and emergency equipment and low value on increased resource use and increased equipment costs.
- Recommendation 4d: We suggest that a mechanical insufflation–exsufflation device be used to help maintain airway patency in patients requiring home mechanical ventilation with ineffective cough, including, but not limited to, those with neuromuscular disease with poor respiratory muscle strength.
(conditional recommendation; very low quality evidence)
- Equipment to facilitate airway clearance is essential in reducing the risk of acute airway obstruction in patients with ineffective cough. This recommendation places high value on the potential to avoid emergent airway plugging and low value on increased costs and resource use.
Table 1. Features of a Comanaged Medical Home for Children Requiring Chronic Invasive Ventilation in the Home
The care would be family/patient centered.
- Recognition of patient/family preferences, social services availability, barriers to communication or medical provision is necessary.
- Keeping in mind that many children requiring mechanical ventilation are equally dependent on subspecialists, such as neurologists, gastroenterologists, physical therapists, etc., this collaborative partnership depends on the situation and will require oversight from a speciﬁc provider most accessible to the family and identiﬁed as primarily accountable. This provider is frequently but not necessarily the primary care provider.
- In practice, a written summary of responsibilities will be provided to the team and family by a social worker or nurse or primary care provider outlining expectations and order of communication channels.
The generalist would be responsible for all aspects of primary care.
The comanagement collaborative would decide and delineate responsibility for comprehensive assessment, coordination, and management of all other aspects of care.
- This includes access to medical care, transportation, family care and respite, access to nutritional needs, community resources, etc. A social worker or local public health resource may be crucial to assist the primary care provider. Pulmonologists, medical specialists, and their teams will be required to assist in coordination of complex care requirements and appointments to lessen the burden on the family. Much care can be delegated to the local primary caregivers with communication from the specialist teams.
- Ideally, access to the same electronic medical record system for communication between the primary care provider, specialists, nursing staff, and social workers would allow sharing of family communications, expected appointments, and ongoing medical issues. If this electronic medical record system is not available to all providers, communications should be outlined in writing to the comanagement collaborative.
Table 2. Proposed Standardized Criteria for Discharge of an Invasively Ventilated Child to Home
1. The child must be medically stable for discharge.
- No signiﬁcant change to ventilator settings or oxygen requirement for at least several days and preferably several weeks before discharge.
- No acute decompensation events (e.g., PICU transfers) within the few days to weeks before discharge.
- Ventilator and oxygen requirements compatible with long-term medical stability and equipment available for home setting.
- Home respiratory equipment trialed and tolerated in the hospital for at least 24–48 h before discharge.
- Must tolerate the transport to and from hospital.
2. Family caregivers must demonstrate the willingness and ability to care for the patient.
- Caregivers must demonstrate competency in delivering all prescribed therapies (e.g., medication administration, feeding, respiratory care, CPR, home ventilator use, responding to monitors).
- Caregivers must demonstrate competency in the care and replacement of their child’s tracheostomy, and caregiver education must include recognizing and responding to urgent issues such as tube obstruction, decannulation, and bleeding from tracheostomy.
- At least two family caregivers must be fully trained in all aspects of the child’s care.
- Caregivers must understand the importance of the continual presence of an alert caregiver who can respond to alarms and emergencies.
- Caregivers must agree to care for their child in situations when additional services (such as in-home nursing) are not available even for extended periods of time.
- Caregivers should complete an independent stay before hospital discharge during which they are responsible for all aspects of the child’s care (including responding to simulated emergencies).
- Routine hand washing is essential; its importance cannot be overemphasized.
- Caregivers must be able to safely transport the child in both routine and urgent situations (a “Go Bag” with all necessary travel items, including an extra tracheostomy tube and obturator, a size smaller tracheostomy tube, suction catheters, scissors, tracheostomy tube ties, and lubricant, will remain with the child at all times. Disability parking privileges should be considered).
- Family caregivers should understand that if the child improves and no longer requires the same amount of professional caregiver support, they will be required to assume increasing responsibility for the child’s care.
- Family caregivers must be instructed not to engage in cigarette smoking near the child and respiratory equipment. Smoking cessation should be encouraged.
3. A DME company must be available and able to provide the required equipment and technical support.
- The DME (or trained personnel from discharge facility) must perform a home inspection to conﬁrm that the home environment and electrical systems are adequate for the necessary medical equipment.
- The DME company must provide 24-h availability as a resource and to service the equipment, including same-day replacement of malfunctioning equipment.
- DME respiratory clinicians should visit patients at least monthly and more often as needed.
4. Professional in-home caregivers (e.g., nurses) as required to support the family must be arranged before discharge.
- Home professional caregivers must maintain infant/child CPR certiﬁcation.
- Professional caregivers must be required to achieve the competencies expected of the child’s family-based caregivers.
- Each professional caregiver must complete ventilator training involving the speciﬁc type of ventilator used in the child’s home.
- Professional caregivers must be available to meet the child at home on the day of discharge.
- An accredited agency must provide professional caregivers with experience in home mechanical ventilation and will maintain training to ensure maintenance of skills.
- Professional caregivers must be instructed not engage in cigarette smoking while on duty.
5. The home and community environment must be safe and allow access to routine and urgent care as needed.
- Primary care, pulmonary subspecialty care, and care coordination must be provided in a collaborative manner consistent with the family-centered care and Medical Home models.
- A formal safety plan should be posted near the patient to include: emergency contact numbers (EMS, primary care provider, specialty providers, DME contact, nursing agency) and any medical information essential to the child’s care (allergies, medications, ventilator settings, speciﬁc instructions).
- A functioning phone must remain with the patient in case of emergency.
- The home should be safe and free from ﬁre/health/safety hazards and provide easy access to the child at all times.
- The home must have a functional ﬁre extinguisher that home occupants are able to operate. A home ﬁre escape plan that includes the patient and minimal equipment needed for life support should be in place.
- The ambient temperature in the home should remain within the range recommended by the ventilator’s manufacturer.
- Irritants (e.g., cigarette smoke, incense burning, molds) should not be present.
- Local EMS should be made aware of the patient and the patient’s condition. On the basis of distance from emergency services, consideration should be given to additional back-up equipment in home.
- Letters requesting that services be restored quickly in an outage should be sent to the telephone and utility companies.
Table 3. Suggested Educational Objectives for Family and Professional Caregivers of Children Requiring Home Mechanical Ventilationa
Pulmonary care and assessmentCaregivers should be able to:
- Obtain basic vital signs, including pulse rate, breath rate, and oxygen saturation
- Recognize and state the child’s usual breath rate, pulse rate, oxygen saturation, respiratory effort, and color
- Identify type of ventilator used in home and ventilator settings
- Identify type of tracheostomy tube and whether it is cuffed or uncuffed and, if cuffed, instructions for inﬂation of the cuff (manufacturer recommendation for cuff inﬂation [air vs. water], amount of air/water used to inﬂate cuff, hours per day spent with cuff inﬂated)
- Identify type of “back-up” tracheostomy tube to be used if primary tube cannot be replaced
- Know that an additional tracheostomy tube, the back-up tracheostomy tube, and the supplies needed to change the tracheostomy should be with the child at all times (the “Go Bag”)
- Identify signs of respiratory distress and describe proper intervention. (Signs of respiratory distress to review may include: increased tracheal secretions or change in color of secretions, retractions, increased work of breathing, cough, color changes, nasal ﬂaring, increased or decreased heart rate, increased or decreased respiratory rate, desaturation, anxiety, abnormal breath sounds.)
- Guidelines for tracheostomy education are provided in Sherman JM et al. Am J Respir Crit Care Med 2000;161:297–308.
Emergency responseCaregivers should be able to:
- Verbalize criteria for calling emergency services (such as 911 in the United States)
- Be certiﬁed in CPR
- Be able to access a list of numbers for emergencies or problems not requiring emergency services (physicians, DME, etc.) and know whom to contact on the basis of the type of emergency
- Demonstrate use of self-inﬂating bag and mask in routine and emergency care procedures
- List signs of tracheostomy obstruction
- Demonstrate appropriate suctioning techniques to remove tracheostomy obstruction
- Demonstrate an emergency tracheostomy tube change (change done by one caregiver without assistance)
- Demonstrate knowledge of emergency medications (if applicable)
- Verbalize plans for loss of electricity, ﬁre, tornado, or other natural disaster
Ventilator trainingCaregivers should be able to:
- Identify electrical power sources
- Assemble ventilator circuit and humidiﬁcation system
- Describe routine cleaning of equipment
- Add oxygen to circuit if indicated
- Verbalize that ventilator alarms must be audible throughout the home
- Demonstrate how to properly turn the ventilator on, test the ventilator before use, and view and verify settings
- Demonstrate an understanding of ventilator alarms and how to troubleshoot the alarms
- Demonstrate the appropriate technique for draining tubing in the ventilator circuit, down and away from child
- Demonstrate the ability to keep battery-operated back-up equipment charged and ready for use
- Demonstrate how to charge batteries for ventilators
- Demonstrate how to connect and use the external battery for the ventilator if applicable
- Verbalize understanding of the approximate battery life for each piece of equipment
Infection control practicesCaregivers should be able to:
- Demonstrate infection control practices as they relate to the plan of care
- Demonstrate proper hand-washing technique
- Demonstrate proper disposal of contaminated material
- Demonstrate how to clean and disinfect reusable medical supplies
MedicationsCaregivers should be able to:
- Identify the dosage and frequency of all medications required by the child
- Explain the indications and side effects of medications
- Demonstrate the ability to prepare and administer medications correctly
OxygenCaregivers should be able to:
- Demonstrate the proper care and use of home oxygen delivery equipment
(e.g., cylinders, concentrators)
- Verbalize the safety issues related to use of oxygen in the home (risk of ﬁre with smoking, open ﬂames, ﬂammable products near the oxygen, or close proximity to heat sources)
- Demonstrate understanding of when and how oxygen should be used for the patient
Oximetry monitoringCaregivers should be able to:
- Demonstrate the proper and secure placement of the oximeter probe
- Demonstrate the ability to differentiate true from false oximeter readings
- Verbalize steps for responding to an oximeter alarm
- Verbalize an understanding of normal oxygen saturations and a plan for responding to saturations that fall below the normal range
Suctioning equipmentCaregivers should be able to:
- Demonstrate the correct catheter size to use for the patient
- Demonstrate the correct suction pressure and catheter depth to use to clear tracheostomy
- Demonstrate how to test for suction pressure
- Demonstrate a clean suction technique
Nebulizer/metered dose inhaler (if ordered)Caregivers should be able to:
- Verbalize when the prescribed inhaled medications are required
- Demonstrate how to deliver inhaled medications in ventilator circuit if applicable
- a Further instruction and competency assessment will be required if patient requires equipment/therapy beyond those listed above (e.g., pulmonary clearance therapies, cardiorespiratory monitors, end-tidal CO2 monitors, etc.). The goal should be to ensure that all caregivers are fully versed in all aspects of the child’s care.