3.1 Recognizing children who need LTV and screening for sleep-disordered breathing

Recognizing children who need LTV in LRS where access to PSG is severely limited, requires a composite assessment of a patient's risk, clinical criteria, and alternative screening tests. Clinical and lung function surveillance can inform who would best benefit from the scarce resource of PSG and/or empiric LTV. The first step is to identify the child at risk; then undertake relevant clinical and screening tests to inform who requires confirmatory testing as summarized in Table 2.

Overnight oximetry is a very useful screening test for severe SDB, with a good positive predictive value.^21-23 It is relatively widely available in LRS and should be advocated for as an important diagnostic tool. Respiratory criteria for sleep oxygenation have been recommended for initiation of LTV (minimum oxygen saturation SpO₂ ≤ 90%, >2% recording time SpO₂ ≤ 90%, 3% oxygen desaturation index >1.4 events/hour, apnea hypoxia index (AHI) > 10 event/hour).⁵ This is particularly helpful for children with variable upper airway obstruction as a cause of their respiratory failure. Other available screening tool useful in LRS is to get a home video of the symptoms when child asleep. This can be done as video or audio on a mobile phone.

For children at risk of hypoventilation carbon dioxide (CO₂) should be monitored. If neither PSG, capnography nor transcutaneous CO₂ monitoring are available, daytime arterial blood gas (done with appropriate care: age-appropriate explanation to child, topical anesthetic, skilled phlebotomist) is useful. For hypoventilation due to NMD lung function with spirometry, maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and/or cough peak flow, should be monitored at least annually. Children with FVC < 50% or daytime CO₂ ≥ 45 mmHg, symptoms of nocturnal hypoventilation, or recurrent respiratory infections, should have overnight oximetry.²⁴ NIV during sleep is indicated for children with chronic respiratory failure (daytime pCO₂ > 6.7 kPa/50 mmHg) with or without symptomatic nocturnal hypoventilation.

3.2 Empowering and preparing families/caregivers for LTV and patient-centered care approach

In contrast to most HIC supporting families with LTV at home through professional home-based nursing care is rarely possible in LRS, unless privately funded. Although unsupported by evidence, strong recommendations by the American Thoracic Society that an awake and attentive trained caregiver be in the home of a child requiring LTV at all times are equally valid in LRS.⁴ In addition to this, one of the most important prerequisites for considering invasive LTV at home in LRS settings is that the caregiver(s) and/or extended family must be able and willing to provide full-time care on a 24-h basis. Ironically, the lack of trained professionals for home care is one of the most common reasons cited in HIC for delays in discharging children from hospital to home. Delays ranging between 2 months (United States), 6 months (Canada), and up to 10 months (UK) have been directly attributed to a lack of home nursing care.^25-27 In contrast, median time to home discharge in South Africa for invasively ventilated children, a setting without home-based nursing care, was 3 months (range 8–991 days).⁸ Equipping caregivers with the technical skills and confidence to provide full-time care of their technology-dependent child may add a significant burden to already stressful social and financial circumstances, but if appropriate selection criteria are applied for eligibility of LTV, it can be empowering to caregivers, regardless of socioeconomic circumstances or education.⁸

3.3 Selecting patients and families eligible for LTV at home in LRS

The indications and eligibility for LTV at home in LRS will vary considerably depending on local expertise and available resources. In broad terms, they can be categorized as patient-related, caregiver and home environment-related, and program-related (Box 1). In conditions where resources are very constrained, selecting children with more favorable outcomes and who have adequate home environments, over children with less favorable outcomes, inadequate or unsafe home environments will be inevitable. Difficult decisions need to be made by multidisciplinary clinical teams, which may lead to the withdrawal of life-supporting care and active palliation. Neuromuscular conditions presenting beyond infancy, either congenital or acquired, represent the largest group with the most favorable outcomes and should be prioritized for LTV in LRS. Unlike HIC, bronchopulmonary dysplasia in preterm infants is an uncommon indication for invasive LTV in LRS, where the morbidity and mortality in preterm infants is high due to limited access to neonatal intensive care.²⁸

Compared with NIV, invasive LTV in the home setting is more expensive in terms of equipment and consumables (i.e., ventilators, humidifiers, and circuits), requires accompanying tracheostomy care and suction devices, and additional caregiver training in emergency measures. Social determinants are often important barriers to invasive LTV. Factors such as uninterrupted electricity supply and whether the caregiver(s) are able to provide full-time 24-h care at home are important factors when considering the feasibility of invasive LTV in LRS. Caregivers who are employed may be forced to resign from their positions which adds significant financial burden on families, more so in LRS where they may be the main breadwinners in the household and social assistance programs are less supportive.

3.4 Solution-focused approach and preparing caregivers for LTV at home

Once the indication and need for invasive LTV is identified in child, a stepwise process that begins to engage and prepare caregivers and their extended family is required to establish feasibility and potential barriers for invasive LTV within the family's unique circumstances and resources. Understanding the caregiver and family's insight of their child's condition and medical needs is important, especially in LRS, where levels of basic literacy, education, and medical knowledge may vary widely. Allowing the caregiver and family to express their personal experience and journey with their sick child facilitates open, honest discussion about the future care of their child and the impact it will have on their family. Including the family at the outset of the LTV journey empowers them to face their difficult new reality, and in turn, identify skills, strengths, personal and familial qualities, and beliefs, that will guide the family in finding what capacity they have to cope with LTV at home. With appropriate preparation and guidance in committed families, it is unusual that LTV at home cannot be accomplished, even with very basic home conditions (Figure 1).⁸ “De-medicalizing” the lives of children and families requires more than technical training. Caregivers need to gain the confidence to take on the challenge of LTV, and mobilize their own family and community resources with the assurance of ongoing support of a multidisciplinary LTV team.

3.5 Tracheostomy care

Tracheostomy care practice and protocols vary widely across the world. A recent multicenter survey of the International Pediatric Otolaryngology Group (IPOG) in North America and Europe reported responses from members in four areas: in-patient teaching, homecare, evaluation, and management of tracheitis, and surveillance endoscopies.²⁹ The conclusion of this survey was that practices and opinions varied widely despite published guidelines. Factors that often influence practices are health insurance requirements and family resources. For example, the recommended frequency of tracheostomy changes reported in the IPOG survey was highly variable: 25% recommend changes twice per month, 25% recommend changes at least every 1–2 months; 8% recommend weekly changes, while others based frequency on type of tracheostomy or proximity to the hospital.²⁹ Examples of innovative and successful tracheostomy care practices do exist in LRS and provide evidence that a tracheostomy should not present a barrier to LTV at home in LRS. A long-standing tracheostomy home care program in South Africa adopted unique practices which include training caregivers to do daily tracheostomy tube changes and re-use two tracheostomy tubes on a rotational basis; simple cleaning techniques with soap, pipe-cleaners, and toothbrush; and simple taping techniques to prevent dislodgement (Figure 2). Daily tracheostomy tube changes equip caregivers with confidence and competency to change the tracheostomy tube in all situations and have the added advantage of preventing sudden life-threatening tracheostomy occlusion or tracheitis that can result from the accumulation of biofilm material in the tracheostomy lumen.³⁰ A study from this South African center of 157 (16% receiving LTV) children discharged home with tracheostomies between 2008 and 2012 reported low mortality (1.2%) and increased risk or unplanned admissions within 1 year in families with a history of substance abuse and household cigarette smoke exposure.³¹ An example of an innovative manual for caregivers on information and training on tracheostomy and LTV can be downloaded at this website (chrome-extension://efaidnbmnnnibpcajpcglclefindmkaj/http://breatheasyprogramme.org/uploads/6ba5c67c361fb06ba9c67c3f/1442588335920/Home-Care-Book-final-email.pdf).

3.6 Home LTV equipment and consumables

The technology requirements for LTV will depend on patient-specific needs, the underlying diagnosis and time required per day on the ventilator.² Modern home ventilators for either NIV or invasive LTV range from simple, less expensive bi-level pressure-cycled devices to more expensive sophisticated multi-modal pressure and volume-cycled ventilators designed for longer or continuous LTV. Consumables include single-limb circuits with expiration outlets, appropriate patient interface connections, and humidifiers with accompanying automated temperature regulation systems. Humidification of the airway is essential with invasive LTV as it prevents dehydration of the airway mucosa and secretions, which may cause life-threatening complications such as obstruction and infections. Heated humification systems have better outcomes compared to heat and moisture exchangers but are more expensive, require electricity, and are more complex to operate.³²

Modern home ventilators have a lifespan of 5–7 years if maintained well but the ongoing expenses of consumables such as air filters, circuits, and humidification must be factored into the overall costs. Although not recommended by the manufacturers, using less expensive bi-level devices for invasive LTV is feasible in some circumstances, such as delivery of CPAP in children with severe tracheomalacia or LTV in young children with severe muscle weakness disorders where chest wall compliance is very high.¹⁴ However, bi-level devices generally do not have built-in batteries and, therefore, will not operate if there is an interrupted or unreliable power supply. Ensuring uninterrupted power supply in LRS is one of the more difficult challenges and barriers in providing LTV at home in LRS. The inclusion of manual resuscitator bags is mandatory in most LRS due to the high risk of unpredictable interruption of power supply. Similarly, providing manual suction devices is equally important. Modern re-chargeable external backup batteries can provide up to 8-h of power during electricity outages but add significantly to the overall costs. The COVID-19 pandemic sparked renewed interest in designing low-cost (USD 700) basic home ventilators in countries like India.³³ Although data and experience are lacking, children needing LTV in LRS could stand to benefit in the future from similar advances in technology.

3.7 Transitioning safely from hospital to home

It is essential that, before LTV being initiated, the family undergo an in-depth interview detailing the family composition and broader support. This helps the healthcare worker to learn about the child's family and community and what the family understands about what the doctors have told them regarding their child's condition. This process is key to the eventual safe transition home. Caregiver counseling is ongoing and an important part of the preparation and training. The primary carer should be with their child in the hospital while the LTV is successfully established so they can be adequately trained, learning about the equipment, the multidisciplinary care, and troubleshooting. This ensures too that there is time to identify the various community networks that will replicate the hospital care team once they are home. The caregiver training must include training in emergency care and response; it is essential that discharge planning includes printed emergency numbers and action plans to keep easily at hand. To adequately plan for safe discharge, a home visit is recommended. This will assist the family through the logistics of how home ventilation will work. For example, a guaranteed power supply is essential and may require batteries and/or a generator, certain necessary home modifications may be identified for action. Transition planning must include considerations of transport, carer support, multidisciplinary community care support, schooling, and communication with local health facilities. A checklist inclusive of equipment (considering specific household needs like electrical outlets and extension cords, portability, humidification, oximeter, and alarm settings), training needs, essential carer competencies, and discharge planning steps should be completed before discharge; an example is included in supplementary material. This ensures that no important steps are missed and that all communication has been clear. Completing a detailed patient-specific care plan (text and pictures) that includes an explanation of the child's condition, details of the ventilation support, how to manage complications, and clear emergency plans should be completed and be given in paper and electronic form to the primary carer. It can, in addition, be shared with the community care teams and school. Follow-up appointments are important for monitoring family adherence and patients' quality of life. Community follow-up may be needed if there are complicated intercurrent issues or missed appointments. Careful and consistent case management of a child on LTV managed at home is essential for safety and success. Any homecare ventilation program must identify who in the team is responsible for coordinating care; and updating and communicating multidisciplinary plans between the healthcare team, family, and community networks.

3.8 Social determinants impacting quality of life

Without professional help in the home setting, families in LRS must often make difficult lifestyle and financial decisions and adjustments to their lives to care for their children on LTV. There is good evidence from both HIC and LMIC settings that Health-related Quality of Life is reduced in caregivers and children on LTV.^{3, 34-36} In addition, increased mental health and sleep disturbances are reported in caregivers with children on LTV.^37-39 The high demands related to the care of children on LTV children therefore pose a significant risk factor for triggering poor mental health outcomes in caregivers and their families. Careful evaluation and screening for potential stressors in the family and social environment is necessary as part of the decision-making to establish the feasibility of LTV, especially if there are pre-existing mental health or substance abuse disorders 3.9 Training care providers and building professional capacity for home LTV programs.

Apart from the expense of LTV equipment and consumables, a lack of professional expertise and experience is a major obstacle to advancing pediatric LTV at home in LRS. Specialized pediatric nursing and Advanced Nurse Practitioners (ANP) are more established professions in HIC but are frequently poorly implemented or unsupported by regulation in LMIC.⁴⁰ Evidence for higher patient satisfaction, improved chronic disease outcomes, and cost-effectiveness with ANP compared to medical practitioner-led care exist in primary, secondary, and specialist care settings involving both adult and pediatric populations.⁴¹ In the absence of ANP or specialized LTV nursing career pathways, multi-disciplinary teams caring for children needing LTV can build expertise and capacity through short courses or training modules such as this example in South Africa: (https://health.uct.ac.za/childrensnursingunit/teaching/short-courses/nursing-children-tracheostomy).