John B. Gordon, Sarah P. Brown, Jerie Beth Karkos, and Lynn A. D’Andrea
In 1997, the Maternal Child Health Bureau adopted the title Children with Special Health Care Needs (CSHCN) to identify children who have, or are at risk of having, a chronic physical, developmental, behavioral, or emotional condition that requires health and related services of a type or amount beyond that required by children generally.1 Applying this definition to data collected in the 1994 National Health Interview on Disability, 18% of the pediatric population is identified as having special needs. In three subsequent survey reports, the National CSHCN Screener in 2001, the National Survey of CSHCN in 2003, and the 2001–2004 Medical Expenditures Panel Surveys, the prevalence of CSHCN has ranged from 12.8% to 19.3%.2 Multiple studies using data from these surveys have shown that CSHCN use more resources, have greater unmet health care needs (both primary and specialty care), higher costs, and inadequate insurance. Children with the highest functional disability were 50% less likely to have adequate insurance as are minority and poor families. In addition, there has been an increase in health care costs borne by families, particularly when privately insured.3-5
A small, but growing, subset of CSHCN can be described as “medically complex and fragile” with chronic conditions requiring technology assistance. In 1987, the former US Office of Technology Assistance defined the medically fragile, technology-dependent child as “one who needs both a medical device to compensate for the loss of a vital body function and substantial ongoing nursing care to avert death or further disability.”6 The continued growth of medical knowledge and technology has led to an increase in the number of technology-dependent children living longer and living at home with technological assistance. This fact, coupled with the lack of home nursing care and the increased expertise of parents in providing health care, has made the dual requirement of technology and nursing an inadequate definition of medical fragility. However, no new standard definition of medical complexity and fragility has been established.7,8 Common characteristics of medically complex and fragile CSHCN include chronic conditions requiring multiple medical and nonmedical services, the use of technological supports, and the need for effective care coordination. The level of and time involved in care coordination depend both on their medical complexity and fragility and a variety of nonmedical factors that impact access to needed services (Table 124-1). This chapter presents an approach to care coordination and describes some of the common technological supports required to sustain life and optimize function in medically complex and fragile children with chronic conditions.
Medical complexity and fragility caused by chronic disease affect the children themselves, families, communities, the health care system, and society at large. Caregivers face constant stress as they try to balance work and family life with the needs of their sick child (see Chapter 123). The child’s tenuous health often means multiple health care visits and what amounts to an intensive care unit (ICU) environment at home. Identifying and negotiating the maze of community and hospital-based services, obtaining insurance coverage, and accessing social entitlement programs is a Herculean task. Families complain that they are often excluded from decision making and lack the information needed to care for their children at home.9Community services are often uncoordinated, episodic, fragmented, or do not exist at all.10 This is particularly true both in small rural communities and inner cities, where inadequate funding and the lack of trained personnel make it difficult to deliver federally—or state—mandated services. The children require multiple medical services and frequent admissions, but care is often partitioned with limited communication among providers contributing to duplicative or unnecessary investigations and therapies, the potential for costly medical errors, and frustration for families. Society at large is impacted by the disproportionately high health care costs of medically complex and fragile CSHCN7,9,11 and by the loss of productivity because of parents taking time off from work and siblings missing school.
Table 124-1. Factors Influencing the Need for Care Coordination
Families, providers, and organizations seeking to improve health care have identified poor care coordination as a major problem in the care of medically complex and fragile CSHCN. The American Academy of Pediatrics and others advocate the Medical Home, a concept of accessible, continuous, comprehensive, family-centered, coordinated, compassionate, and culturally effective primary care delivered by a primary care physician (PCP), to meet the needs of CSHCN.12 However, few PCPs can manage these children’s unusual diagnoses, treatments, and technologies as well as coordinate their many nonmedical services (eg, education, out-of-home care, family support, insurance) by themselves. Nor do most PCPs have the time or expertise to manage long and frequent hospitalizations or arbitrate among the tertiary center specialists who may espouse competing diagnoses and treatments.
Many tertiary centers have developed disease- or condition-specific programs that provide care coordination for medically complex and fragile CSHCN (eg, diabetes programs, tracheostomy/ventilator programs). More recently, disease- or condition-independent care coordination programs have been developed to address the needs of medically complex and fragile CSHCN whose disorders cross multiple specialty areas. Some provide care for specific populations (eg, Title V recipients). Others offer predominantly ambulatory care or hospitalist- based inpatient “Complex Care Services.”13 Finally, a few programs offer integrated inpatient and outpatient services, some in collaboration with local primary care physicians.7 Virtually all of these models of care coordination have shown a decrease in hospital days, increase in family satisfaction, and decrease in costs after enrollment in their program, but the lack of standardized definitions of the populations served make comparisons among these models difficult.8
Regardless of the model, a team approach that incorporates the concepts of the medical home is key to meeting the needs of medically complex and fragile CSHCN. In one model shown in eFigure 124.1 , the family and child are at the core of the TEAM flanked by a PCP and a tertiary center program that has assumed responsibility for care coordination. The PCP ensures routine and acute care of the child, particularly if the child lives far away from the tertiary hospital center. The tertiary center program providing care coordination may be disease or condition specific or not. It may be a formal care coordination program or an ad hoc group of providers who have come together to serve a particular child. Ideally, it will include an invested physician, nurse, and social worker as well as other providers and advocates if available. Other care coordination team members play a vital, but potentially intermittent, role. Close collaboration and good communication ensure the expertise needed to access resources and benefits while minimizing the burden on each team member.14 A leader of the team who can serve as the point person for the family should be identified. The leader is unlikely to be expert in all domains of the child’s care, but must know how to access (and be accessible to) the others. Initially, the tertiary center program physician or nurse care coordinator may take the leadership role. As the child stabilizes and the disease trajectory becomes more defined, the primary care physician may take on the role. Over time, the family often achieves such a level of expertise in both the child’s medical management and coordination that they take the lead.
CARE COORDINATION NEEDS ASSESSMENT
There is ample literature describing the differences in perceptions, needs, and goals of families and providers.15 eTable 124.1 summarizes the components of a care coordination needs assessment. Thus, the first step in identifying care coordination needs is to identify the family’s goals. Listening as the family tells the child’s story and describes their hopes and dreams for the child generally provides an appreciation for their medical sophistication, understanding of the medical condition(s), cultural and religious values, and general approach to the challenges that they will likely encounter. It is important to learn what, if any, limits to invasive interventions the family wishes to put on the child’s care and to understand why they feel as they do. After learning of the family’s goals, it is important to determine what resources exist and are needed to meet the goals. What is truly known of the child’s diagnoses and prognosis? Are other studies or consultations needed? This information is crucial to appropriately advising the family about invasive interventions or alternative code status. Care coordinators must also recognize how their own values may influence their opinions and should seek input from others who may have differing views.
INITIAL CARE COORDINATION SERVICES
Tertiary center programs providing care coordination generally have more resources than do primary care physicians. The tertiary center program personnel can assist the family in identifying and obtaining the insurance, social benefits, and community services needed. Recreational programs, community-based therapies, and parent-to-parent organizations can be identified. Because the stress of a newly diagnosed medically complex and fragile CSHCN can be overwhelming, it is helpful if the tertiary center program providing care coordination actually makes the initial connections for the family (eg, calls the local rehabilitation or developmental service coordinator, helps fill out insurance forms) rather than simply providing a list of names and numbers to the family.
The tertiary care program is also well placed to assist in developing a comprehensive care coordination summary. Several styles exist on line (eg, http://www.medicalhomeinfo.org or http://www.medicalhomeimprovement.org). Regardless of the format chosen, the summary should include relevant medical and nonmedical information that will assist the family and providers meet the goals established for the child as shown in eTable 124.2 . One very useful purpose of the summary is to provide a synopsis of the child’s medical history and plan of care. Careful documentation of active diagnoses and past problems as well as relevant testing (particularly when a diagnosis is uncertain) enhances communication among providers, improves consultations, minimizes unnecessary testing, and provides the primary care physician with a comprehensive overview. Synthesizing information may identify conflicts between plans proposed by different specialists and enhance a unified approach to the child. Sharing the summary with the family will identify missing or erroneous information and help focus the plan on their goals. It also serves to educate the family and enhance their ability to participate in care and develop skill as advocates and coordinators.
A well-prepared and updated care coordination summary can speed and ease subsequent emergency room visits and hospital admissions. The description of a child’s diagnoses, a list of providers and medications, a baseline exam, and an overview of the child’s care as well as an emergency plan may also enhance the child’s safety. However, parents and other providers must advise the coordinators of medication and other therapeutic changes that may have occurred. Moreover, summaries can become quite unwieldy if used to document all care coordination issues. Several Web-based databases that allow the family or provider to generate situation specific reports are under development.
ASSESSING TECHNOLOGY NEEDS
The care coordination needs assessment and summary often lead to consideration of assistive technology. The family is integral to deciding whether a particular technology support is consistent with their overall goals for their child. Simply because a particular intervention or technology is available rarely means it must be performed or acquired. This is particularly true when it will not significantly alter the course of a child’s ability to meet a goal. If assistive technology is desired, the choice of support or device requires input from the family and multiple members of the coordination team. The primary care physician and tertiary center program providing care coordination may be able to advise the family on the merits and limitations of a certain technology support, particularly if they have specialized expertise (eg, a tracheostomy and ventilator program). More often, other specialists, therapists, teachers, vendors, durable medical equipment companies, and insurers are needed to ensure that the technology is appropriate. It is particularly important that the right device be prescribed the first time, because many insurers limit the number of replacements that will be covered over a given period.
All technology should maximize the child’s participation in developmentally appropriate physical, cognitive, adaptive, and social activities. Thus, it is important to consider child-centered factors (size, growth, anticipated medical, physical, and developmental needs, play) when deciding on the type of support. It is also critical to consider caregiver factors (physical and cognitive) and the environment in which the device will be used (school, home, community), because it will not be used if the family, teachers, and therapists find it unacceptable. Durability, repair services, and expenses must also be considered, particularly when considering high-technology electronic assistive devices. Letters of medical necessity justifying prescription of a technology support should document the medical condition, anticipated medical and functional course, the patient goals, and why less-expensive alternatives are not acceptable. Letters of medical necessity may need to be submitted well in advance of the anticipated need for some items, particularly some of the higher technology and expensive equipment. Should denials of coverage occur, the prescriber must be prepared to write additional letters, place phone calls to the insurer’s medical director, and otherwise advocate for reconsideration of authorization. Specialists and therapists experienced with the equipment can be invaluable in this process.
INVOLVING SPECIALIZED THERAPISTS
Because of their role in coordinating care, physicians (whether in the tertiary center or primary care setting) must clearly understand the roles and functions of physical and occupational therapists and speech language pathologists who often play a critical role in the care of these children. Physical therapy addresses problems associated with neuromuscular dysfunction and gross motor delay as they influence the child’s ability to be mobile within the environment. Occupational therapy addresses problems with neuromuscular dysfunction, sensory perception, psychosocial competence, and fine motor delay as they influence the child’s ability to participate in self-care, play, and school activities. In practice, there is often considerable overlap in the methods and techniques used by the two disciplines, especially with very young children. In general, children should be referred for physical therapy evaluation when there is a delay in gross motor development or concern about the child’s quality of movement. Children should be referred for occupational therapy evaluation when there is reason to suspect impairment in the performance of age-appropriate daily tasks or routines, including self-care, play, social interaction, or in the execution of school-related activities that have a perceptual-motor component. Referral should include a complete diagnosis, information about relevant precautions (eg, allergies, weight-bearing status, exercise tolerance), and the reason for referral. In addition, copies of previous evaluations help the therapist avoid repeating unnecessary interviews pertaining to the child’s medical background or developmental history.
Speech-language pathologists are professionals who are educated in the study of human communication, including normal development and communication disorders. Speech-language pathologists are also trained to recognize neurologic and upper aerodigestive disorders that affect speech and swallowing. By evaluating the speech, language, cognitive-communication, and swallowing skills of children with disabilities, the speech-language pathologist can determine specific deficits, the severity of the disorder (often based on age level norms), and possible etiologic and contributing factors. This information forms the basis of a treatment plan for the child. The recommendations may include formal therapy or parent training and consultation with periodic rechecks. Depending on the type of disorder, therapy may include exercises to improve oralmotor skills, exercises to develop appropriate articulation placement, or activities to develop functional language skills. Some children lack the basic prerequisites for verbal communication for a variety of causes (ie, tracheostomy, significant hearing loss, neuromuscular disorder). For those children, augmentative or alternative communication systems are developed. Regardless of the type of therapy, parent involvement is a key component of the treatment process. Therefore, parents are encouraged to observe the therapy sessions whenever possible and are given instructions for working with the child at home. It is important to note that early intervention is critical for the best long-term prognosis.
SELECTED TECHNOLOGY SUPPORTS
NUTRITION AND INTRAVENOUS ACCESS
Medically complex and fragile CSHCN with swallowing disorders and other gastrointestinal problems often require supplemental or complete enteral or parenteral feeding. It is important to recognize that they may have very different nutritional needs than healthy children of the same age or size. For example, an oxygen-dependent child with chronic lung disease may have increased caloric needs due to increased work of breathing and those caloric needs may suddenly decrease if chronic ventilator support is initiated. Monitoring body weight and length and head circumference is necessary in deciding on the individualized nutritional plan. Periodic assessment of trace elements, minerals, and electrolyte balance is also important, particularly when caloric needs are low enough that standard enteral formulas must be diluted. A nutritionist or gastroenterologist is often essential in guiding nutritional management. For a detailed discussion of specialized nutritional support refer to Chapter 33.
Indications for chronic central venous access in medically complex and fragile CSHCN include continuous or intermittent intravenous nutrition, chronic intravenous medications or immunotherapy, and assured venous access in precarious children with poor peripheral access. Chronic central venous access can be obtained with a peripherally inserted central catheter (PICC), a tunneled central venous catheter (CVC), or a subcutaneously implanted reservoir and catheter (PORT). Each option is available as either a single lumen or a double lumen device and CVCs are available with three lumens. PICCs tend to be used when relatively short periods of access are needed. They pose little danger of pneumothorax or bleeding, but their length and small diameter make clotting of the catheter more frequent and can limit the infusion rate. PICCs are not tunneled under the skin before entering the vein so there is a greater potential for infection and accidental removal. CVCs are often chosen when frequent or continuous long-term access is needed. Although the collar of a tunneled CVC provides some protection, CVCs may be damaged or dislodged by pulling on the catheter. PORTs are used when intermittent, but secure, chronic central access is required. The reservoir is relatively large and must be accessed with a needle through the skin, making it less attractive for young children. Most providers allow swimming in patients with PORTs, but discourage it in patients with CVCs despite a lack of evidence that swimming increases infections. Problems with PORTs include subcutaneous infiltration when the needle is dislodged and surgical removal when infected.
Several problems can arise with chronic central venous access (Table 124-2). Evidence-based recommendations for preventing infections were published in 2002 by the Centers for Disease Control and Prevention.16 Good hand-washing, skin antisepsis, and catheter site dressing are essential. Specialized “central access teams” reduce catheter-related infections, complications, and costs and these teams are helpful in teaching providers and families how to maintain and troubleshoot problems with the catheter. Antibiotic “lock” or “flush” techniques in which a vancomycin–heparin solution (eg, vancomycin 25 mcg/mL and heparin 10 units/mL) is instilled in the catheter are also effective.17 The “lock” (ie, allowing the solution to dwell in the catheter) was more effective than simply flushing the solution through the catheter, but concern about vancomycin-resistant organisms has led many to reserve the use of “locks” for patients with repeated infections. Other approaches to infection in chronic central venous access include the use of antiseptic or antimicrobial-impregnated lines.
Clotting is another major complication of central catheters. Prophylaxis against clots within the catheter is provided by heparin-containing flushes when accessed frequently or by instilling 5 mL of a 100 unit/mL heparin solution when deaccessing a subcutaneously implanted reservoir and catheter and monthly thereafter. If blood cannot be withdrawn and a clot is suspected, tissue plasminogen activator (tPA 0.5 mg in 2 mL) may be effective. The high frequency of large vein thromboses associated with central lines is of concern, particularly in children who are dependent on long-term central venous access for survival. Thus, several groups recommend the use of low doses of warfarin or low-molecular-weight heparin, although support for this practice is limited.
Table 124-2. Apnea Monitor Settings
Many medically complex and fragile CSHCN receive supplemental oxygen or ventilator support at home and may benefit from pulse oximetry to maintain safety and provide guidance in adjusting the level of support. The pulse oximeter alarms should be set at levels that alert the caretaker to assess the child and potentially intervene. Setting limits too close to the child’s baseline range will result in multiple alarms and decrease use of the monitor. Instructions for the caregivers should note when supplemental oxygen should be initiated (eg, when SpO2 is less than 90%) and when their physician should be called.
Apnea monitors are generally used when medically complex and fragile CSHCN with a tracheostomy or mechanical ventilator are asleep or not directly observed by a caregiver. The monitor settings are typically based on the child’s age (see Table 124-2). Although positive-pressure home ventilators are equipped with high- and low-inspiratory pressure alarms, an apnea alarm can be an important adjunct safety device. For example, the ventilator’s low-pressure alarm is designed to alert caregivers to ventilator circuit disconnection, leakage within the ventilator circuit, or tracheostomy tube decannulation. However, the low-pressure alarm may not be triggered after decannulation because of the high resistance of small tracheostomy tubes18 or after circuit disconnection because the tubing may be obstructed by clothing or bedding.
Suctioning and Cough Assist Devices
Suctioning is required intermittently in children with tracheostomy tubes and other medically complex and fragile CSHCN with copious secretions and decreased airway clearance. Portable suction machines are typically used for travel and stationary machines are used in the home. A mechanical cough assist device (insufflator-exsufflator) is used to stimulate a cough in individuals with neuromuscular weakness. The cough assist device gradually applies a positive pressure to the airway via a mask or mouthpiece, then rapidly shifts to a negative pressure. A recent study showed that the in-exsufflator was safe, well tolerated, and effective in preventing pulmonary complications in 90% of children followed in a pediatric muscular dystrophy clinic.19
High-Frequency Chest Compression
High-frequency chest compression (HFCC) is indicated for assistance with airway clearance in patients with cystic fibrosis and is increasingly used for other medically complex and fragile CSHCN with difficulty mobilizing pulmonary secretions leading to recurrent pneumonia or atelectasis.20 The HFCC device consists of two components: an air delivery device with a motor-driven valve and an inflatable vest. The air delivery device creates oscillating air pressure that is delivered to the vest via hoses. This action produces high-frequency chest compressions that create an oscillatory effect within the airways to help mobilize bronchial secretions. Generally, the frequency and pressure of the HFCC are set at 10 to 15 Hz and 4 to 6 cm of water, respectively, for 20 minutes 2 to 3 times per day.
Supplemental oxygen is commonly prescribed for home use in medically complex and fragile CSHCN with chronic lung disease, congenital heart disease, neuromuscular weakness, or chronic respiratory insufficiency (with or without mechanical ventilation). A general guideline is that the child must require less than or equal to 0.35 FIO2 to be discharged from the hospital, and caregivers should contact the physician when a higher FIO2 is required to maintain saturations at a predefined acceptable level at home. In the hospital, blenders and mechanical ventilators are capable of delivering a specific FIO2. At home, 100% oxygen is generally delivered and the desired FIO2 is achieved by varying the flow rate via nasal cannula, mask, tracheostomy collar, or ventilator. The amount of air entrained with each breath determines the actual FIO2 received by the child. As a general rule, the FIO2 likely exceeds 0.35 when oxygen flow through a nasal cannula exceeds 2 liters per minute in infants or 3 to 4 liters per minute in older children, when Venturi-type valves calibrated for greater than 35% O2 are used, when nonrebreather masks are used, and when flow exceeds 4 liters per minute on a home ventilator. It may be helpful to measure the relationship between flow and FIO2 on a home ventilator prior to discharging the child from hospital.
Oxygen is supplied in liquid form, as a compressed gas, or from an oxygen concentrator. Liquid oxygen is more portable, and lightweight liquid oxygen tanks can be refilled from a large reservoir tank kept at home. However, liquid oxygen may cost more and may not last as long as compressed oxygen because it evaporates. Moreover, liquid oxygen is cold and can burn if it comes into contact with the skin. Compressed oxygen is available in cylinders, ranging in size from small portable tanks to large stationary tanks for home use. An oxygen concentrator is an electric machine that can deliver approximately 4 liters per minute of 100% oxygen. The percentage decreases at higher flow rates. It is recommended that families using a concentrator have a backup cylinder of compressed oxygen in case of a power failure. All families should be reminded never to smoke or allow others to smoke when oxygen is being used.
Children require tracheostomy tubes for several reasons (Table 124-3). The tracheostomy tube is placed surgically, after which the child is generally monitored in the intensive care unit until the first tracheostomy tube change in 5 to 7 days. “Stay sutures” are placed on each side of the tracheal incision to facilitate reinsertion of the tube if it becomes dislodged before the stoma has matured. Complications in the early postoperative period include accidental decannulation, obstruction of the tube, infection, hemorrhage, and creation of a false passage if the tube is improperly replaced. In general, 25% to 50% of children with tracheostomy tubes will eventually have complications (typically infections and obstruction), but death from a complication is rare.
Table 124-3. Indications for Tracheostomy
FIGURE 124-1. Illustration of pediatric Shiley and Bivona tracheostomy tubes. A: Shiley pediatric (left) and neonatal tracheostomy tubes. Although the neonatal tube has a larger internal diameter, it has a shorter cannula and smaller neck flange compared to the pediatric tube. B: Bivona pediatric uncuffed (left) and cuffed (middle, right) tracheostomy tubes. The cuff can be deflated (middle) or inflated as necessary to decrease the leak.
The selection of the tracheostomy tube is based on several factors. The tube should be at least 1 to 2 cm above the carina and the diameter should be selected to avoid pressure on the tracheal wall, minimize work of breathing, and, if possible, promote translaryngeal airflow in order to facilitate vocalization. Pediatric tracheostomy tubes are generally composed of silicone, which is quite flexible, or polyvinyl chloride, which may be either flexible or rigid. They are characterized by internal diameter, outer diameter, and cannula length, but are usually referred to by their internal diameter. A neonatal tracheostomy tube has a shorter cannula and neck flange (see Figure 124-1). The tracheostomy tube may have an inflatable cuff to reduce the risk of aspiration and/or help reduce the “leak” when requiring ventilator support. Depending on the manufacturer, the cuff should be inflated with either air or sterile water.
Adequate humidification of the trachea to minimize thick secretions in the tracheostomy tube is accomplished by using a tracheostomy collar with humidified oxygen or air when sleeping and when ill. During travel and other activities, the tracheostomy tube can be covered with a heat and moisture exchanger (HME), speaking valve, or cap. The speaking valve is a one-way valve that allows air to pass into the tube on inspiration, but forcing air back through the vocal cords on expiration. Children with tracheostomies generally require suctioning at least three to four times per day and more frequently during times of respiratory illness. Signs that a child needs suctioning include rattling mucus sounds, tachypnea, secretions pooling at the opening of the tracheostomy tube, or signs of respiratory distress. Normal saline drops instilled prior to suctioning help loosen the thick secretions or elicit a cough. The size of the suction catheter depends on the size of the tracheostomy tube. Shallow suctioning removes secretions at the opening of the tracheostomy. Suctioning just past the tip of the tracheostomy tube allows complete clearance. The desired depth of the suction catheter can be calculated as tracheostomy length + adapter length + up to 5 mm as detailed in eTable 124.3 . Suctioning until the catheter meets resistance from the carina should generally be avoided as this may injure the lining of the airway. Applying suction pressure both on insertion and withdrawal of the suction catheter facilitates removal of the secretions. Following suctioning, many children with tracheostomy tubes may benefit from a few manual ventilation breaths to re-recruit the lung.
The child with a tracheostomy tube must be attended at all times by a trained adult caregiver who knows emergency tracheostomy management. A “to go” bag containing a suction machine with suction catheters, extra tracheostomy tubes (one of the same size and one a size smaller), ties, gloves, saline vials, a water-soluble lubricant, an extra HME, a manual resuscitation device, and any inhaled respiratory medications must accompany the child when outside the home.21 In 2000, the Pediatric Assembly of the American Thoracic Society published a consensus statement to serve as a standard of care for children with a chronic tracheostomy tube that provides detailed guidelines with supporting evidence.22
Noninvasive Mechanical Ventilation
Noninvasive mechanical ventilation is accomplished without an endotracheal airway using either positive- or negative-pressure support. Improved pediatric-appropriate masks and portable ventilators designed for home use have helped make intermittent noninvasive positive-pressure mechanical ventilation a reasonable therapeutic option for an increasing number of medically complex and fragile CSHCN with chronic upper airway obstruction or progressive respiratory insufficiency. Cognitively intact, medically complex CSHCN can be managed with continuous noninvasive mechanical ventilation using a combination of mask and positive-pressure ventilation at night and a positive-pressure ventilator attached to a small mouthpiece that the child can intermittently trigger to receive a full ventilator breath (“sip and puff”) during the day. The use of noninvasive mechanical ventilation has been shown to reduce pneumonia or atelectasis, improve gas exchange, reduce hospitalizations, and improve sleep quality.23
Invasive Mechanical Ventilation
Long-term invasive ventilation refers to the application of positive-pressure ventilation through a tracheostomy tube. There are no specific guidelines for inducing invasive mechanical ventilation. In general, this mode is chosen when noninvasive interfaces are not accepted or tolerated, when the need for ventilatory support exceeds a major part of the day and the child cannot cooperate with the “sip and puff” device, or when bulbar function is impaired and a tracheostomy tube is required for airway hygiene. Medically complex and fragile children who require invasive mechanical ventilation can be safely cared for at home and successfully reintegrated into the community. Caregivers (including nurses and respite caregivers) should be formally trained in how to provide care for these children. In general, medical cost comparisons between hospital and home care reveal that for most children, care at home was less expensive than in a hospital, although this is somewhat dependent on the complexity of the child’s care, the amount of nursing care required at home, the number and duration of readmissions to the hospital, and the ability of parents to provide unpaid care.
FUNCTIONALITY AND MOBILITY
Chronic illness and significant injury in childhood usually result in impairment, defined by the World Health Organization (WHO) as any loss or abnormality of psychological, physical, or anatomic structure or function. Disability is the limitation in activity caused by impairment. Handicap exists when an impairment or disability limits or prevents participation in a role that is normal for age and gender, within the social and cultural milieu. Goals of management of pediatric chronic disease and disability include minimizing the impairment and maximizing activity and participation in age-appropriate life roles (school, play, work). The approach to care is often interdisciplinary and should be coordinated, comprehensive, and family-centered.
The major objective in disability management is to facilitate independent function in the particular areas, referred to as domains, that are affected. Function is promoted in mobility, self-care, communication, cognition, and/or psychosocial domains. In each area, efforts are initially directed toward assisting the child to accomplish skills independently. This is accomplished through treatment strategies that enhance the functional capacity either of the affected system, when skills can be restored or developed, or through compensatory strategies using systems unaffected by the pathologic condition. Secondary disability should be prevented to the extent possible. When necessary, prescription of equipment or modifications to the physical or social environment may provide the child with greater independence. Psychological and educational techniques may also enhance patient performance. Prescriptions for therapy programs, adaptive equipment, orthoses, and prostheses should be age-appropriate and include consideration of the child’s ongoing growth and development.
Orthoses can be defined as custom-fitted devices applied to or around a body segment that are designed to meet specific musculoskeletal goals, such as (1) prevention of movement because of abnormal tone or that are involuntary; (2) maintenance of joint alignment to facilitate body mechanics; and (3) stabilization of a joint.24 Acronyms used to describe orthoses generally refer to the body part (eg, AFO is the acronym for ankle-foot orthosis). The family and child should meet with a specialist skilled in functionality (eg, rehabilitation, neurodevelopmental pediatrics, orthopedics) and the child’s therapists to establish the goals and appropriate use and care of an orthosis before it is prescribed. Orthoses should be simple, durable, strong, easy to use, lightweight, and cosmetically pleasing. They must fit well and be used as prescribed to be effective. The child’s condition and the purpose of the orthosis should be indicated on the prescription and consultation with an orthotist considered. Shoes for use with orthotics should have a wide toe box with an upper of soft leather, canvas, synthetic, or nylon fabric and a tongue that goes far down into the toe box. Specialty shoes are expensive, but insurers may reimburse the family if a prescription and letter of medical necessity are presented. Skin breakdown is the most common complication of orthoses. Skin should be checked daily, and if an area of redness lasts longer than 20 to 30 minutes, the orthotist or physician should be notified. The goals, fit, and use of the orthoses should be reviewed at least every 6 months until growth stabilizes and each time the child experiences therapist transitions (eg, when starting school).
Wheeled Mobility and Seating
A child’s inability to master the environment independently may lead to decreased socialization, learned helplessness, and delayed development. Therefore, wheeled mobility should be considered as early as necessary to facilitate developmentally appropriate independence and functional activity. Because poor positioning and restriction of movement can result in pressure areas and musculoskeletal deformity, mobility devices are often combined with customized positioning or seating systems when the child needs assistance to maintain appropriate position and use of the trunk, head, or limbs. Multiple physical impairments may lead to the need for wheeled mobility (eg, weakness, low endurance, movement disorders, abnormal tone, pain syndromes). The type of wheeled mobility should be based on the child’s immediate and anticipated needs and goals (eg, duration of need, ability to self propel, other assistive technology, surgery, ability to partially ambulate), family issues (eg, transportation, access, lifting restrictions), and the proposed environment in which the device will be used (Table 124-4). Although studies have shown that 12- to 18-month-old children with normal cognition can learn to use powered mobility safely, evaluation for motorized options requires detailed assessment of sensory, motor, cognitive, and behavioral status as well as consideration of accessibility.25,26
Car Restraints and Transportation
Most medically complex and fragile CSHCN will be safest when positioned facing rearward in the back seat of the car for as long as possible. Others will require alternative restraints.27,28 Resources that may be helpful in choosing restraints include the National Highway Traffic Safety Administration (http://www.nhtsa.dot.gov/people/injury/childps/contacts/index/cfm), Riley Hospital Automotive Safety Program (http://www.preventinjury.org), the AAP Car Shopping Guide for Children (http://www.aap.org/family/carseatguide.htm), and the Transport Canada road safety site (http://www.tc.gc.ca/roadsafety). Commercially available car beds are designed to accommodate infants in the 1.8 to 9 kg range. Car beds are indicated for low-birth-weight infants (less than 2.3 kg) who failed conventional car seat testing prior to discharge and infants with other selected conditions (Table 124-5). Padding must not be placed between the infant and the car bed or the harness system because it may interfere with the effectiveness of the restraint system. Safety vests are designed for children at least 2 years old, weighing between 9 and 76 kg. These children cannot sit safely in a conventional car seat or wear appropriate lap belt or shoulder harness because of motor, behavioral, or positioning problems. The safety vest is applied prior to placing a child in the car and then it is anchored to the vehicle by a tether strap and seatbelt. Specialized car seats are designed for children between 10 and 48 kg who require more physical support than is provided by conventional seats. The Federal Motor Vehicle Safety Standard status of the particular model should be verified before prescribing or advising a family to purchase one. Parents should be trained in appropriate application of necessary tethers and harness fit since problems with restraints usually arise from inappropriate application and fit of tether and harness.
Additional consideration in the use of car restraints applies to selected populations. Children with tracheostomies should avoid restraint systems that may impair ventilation or risk impact to the tracheostomy tube in an accident. They should be kept facing the rear in a convertible, well-fitting car seat. When the seat is turned forward, one must ensure that the chin does not cover the tracheostomy. Pulse oximetry can be used to verify appropriate saturations while seated in the car seat. Children with seizure disorders should be in restraints that will provide appropriate support of airway during and after a seizure. A specialized car seat restraint may be necessary for a child weighing more than 20 kg who has frequent seizures, because this is the usual upper weight limit for commercial car seats with harness straps. Children with hypotonia and/or risk for high cervical subluxation (eg, Down syndrome, achondroplasia) or fractures (eg, osteogenesis imperfecta) should use a rear-facing car seat as long as possible. A standard convertible child safety seat allows children weighing up to 15 kg to face rearward.
Table 124-4. Wheeled Mobility
Table 124-5. Vehicle Restraints
Children who use wheelchairs should ride in a properly fitting car seat rather than their wheelchair whenever possible. If transport in the wheelchair is necessary, it should be secured in a forward-facing position with 4-point tie-downs for the chair and a separate 3-point belt restraint for the child. Trays must be removed and head support is strongly advised. Other medical equipment should be anchored to the floor if possible. There are no tethers made specifically for medical equipment in a private vehicle. Thus, options include placing the equipment under the vehicle seat or wedging it in place with pillows, foam, or blankets. Unused seat belts may also be used to ensure that the equipment will remain secured in the event of a crash. Batteries for equipment should be dry cell or gel cell to limit flammability.
Transfer Aids and Lifts
Many medically fragile and complex CSHCN will attain close to adult size and weight. Some are able to bear weight, but need assistance to move from one surface to another. Safe and efficient transfers require skill and practice. Therapists can instruct caregivers in appropriate techniques. A full-lift transfer is required if the child bears no weight when being transferred. Transfer aids should be considered for any child who weighs more than 25 kg and cannot be safely transferred by one caregiver alone. Aids should also be considered for children with inconsistent ability to assist in transfers (eg, frequent seizures, behavioral issues, variable endurance) or when caregivers cannot safely lift a child. There is no single transfer aid that accomplishes full-lift transfers in all settings from all surfaces (see eTable 124.4 ). If possible, the family should try the equipment before buying it; if the mechanical aid is not easy and efficient, they will likely revert to lifting the child manually. Some insurers consider transfer aids medically necessary only for transfers between bed and chair, wheelchair, or commode. Reimbursement for toilet lifts and ceiling lifts mounted on tracks will often require strong advocacy.
Bathing a baby is relatively easy compared with transferring and positioning a slippery child, particularly when the child has poor head control or severely affected muscle tone. When children have episodic emergencies (eg, seizures or mucous plugs in a tracheostomy), bathing equipment that allows the caretaker use of both hands is essential. Bathing equipment should provide comfortable restraint and positioning while ensuring safety during medical emergencies. Anticipated growth, changes in medical condition, limitations of the bathroom space, and the need to accommodate other family members should all be considered when evaluating bathroom equipment. As with much of durable medical equipment, it is often best to test equipment prior to purchase or purchase from a vendor that allows exchanges in case it does not meet family and child goals. Low-technology options for the child with good head and trunk control, but who has poor balance, include waterproof upright corner chairs with anterior trunk supports and pelvic belts. Medium-technology systems restrain the child in a semireclining position and are made for the child who cannot sit or maintain head control. Features include adjustable seat and back, trunk and pelvic harness, leg straps, and adjustable frame height for easier transfers. High-technology systems include a battery-operated, push-button lift for individuals age 3 to adult.
ACCESS TO AND IN THE HOME
Remodeling should be considered when access to the home or living spaces limits caregiving, the child’s anticipated acquisition of independence skills, or the safety of child and care-giver. Home adaptations can also improve the child’s ability to perform activities of daily living (eating, dressing, hygiene) and participate in social and avocational activities within both the family unit and the community.29 Input from developmental and rehabilitation specialists and therapists can help families develop realistic goals and plan environmental modifications. Leveling thresholds and investing in automatic power door openers can improve access through the front door. External doorways should be widened to 36 in if possible. The majority of homes have at least one or two steps leading up to the external door. Building codes require that wheelchair ramps be 12 in long for every 1 in rise. Ramps should be a minimum of 36 in wide, have enclosed handrails, and have a 5-ft-square level area for maneuvering on the landing. Vertical platform lifts consist of a platform attached to a tower that houses an electric motor and drive mechanism. They have a lifting height of 12 ft and require 30 sq ft of space. Porch lifts are similar to vertical platform lifts, but have a maximum lifting height of about 6 ft. They are especially useful when space is limited. Chair lifts enable the user to be carried up the stairs and can be used both inside and outside and on straight or curved stairways, but are not appropriate for children who use a wheelchair full time. All renovations are relatively expensive and may not be reimbursed by insurance companies. Families should be aware that many state programs offer financial assistance for needed home remodeling if the home is owned, but often not for rental homes. Alternative solutions for moving the child during an emergency must be considered.
Bathrooms are often the smallest rooms in the house, with a narrow doorway and turning radius that limits easy access to the toilet, sink and bath and shower. Low-tech solutions include installing a hand shower in the bathtub, using single-lever faucets, and modifying the toilet by raising or lowering the toilet seat height. The use of a bath or shower chair and a commode with needed supports is often helpful. Minor modifications to the bathroom include using nonskid tile and removing counter cabinets to increase wheelchair accessibility. Call buttons and intercoms can be installed for emergency assistance. High-technology solutions include ceiling-mounted lift, power bathtub lift, electronic faucets, self-flushing toilets, and walk-in baths or showers.
Access to the bedroom is also essential. Low-technology modifications include simply adjusting the bed height for ease of transfer; changing from swinging to sliding doors to increase space and visibility within closets; and lowering closet shelves so that a child, short adult, or seated person can choose clothing. A bedside compact refrigerator is often useful for fluids or medications and an emergency button or intercom to request assistance is essential. Higher-tech solutions include power beds. Similar accommodations throughout the home (eg, kitchen and living room) can make it possible for the child to reach different rooms and participate to the best of his or her abilities.
CARE COORDINATION SERVICES AT DISCHARGE AND BEYOND
Ensuring a seamless transition from hospital to home for a medically complex and fragile CSHCN requires involvement of the care coordination team and enormous attention to detail. This is particularly true when the child is going home for the first time after a diagnosis of a chronic condition has been made, but the principles remain the same with each discharge (Table 124-6). Prior to discharge, the child’s medical and nonmedical needs must have been addressed; caregiver education must have been completed; and needed home renovations, transportation, and community services put in place. Insurance and social benefits must have been identified and accessed as much as possible. An emergency plan, often a simple algorithm for dealing with nonacute, urgent, and emergent problems should have been developed. In small communities or in the case of a child with unusual or unstable conditions, it may be helpful to provide the local emergency medical services and emergency room with details of the child and his or her common problems and usual therapies. Most important, the primary care physician must be involved in the discharge planning. Ideally, the primary care physician should participate in a discharge planning conference in person or by telephone and, at a minimum, the care coordination summary should be provided to the primary care physician. Prior to discharge, the family, primary care physician, and tertiary center program should agree on their respective care coordination roles and responsibilities and a list of contact numbers should be provided to the family and primary care physician.
Table 124-6. Preparing the Medically Complex and Fragile Child for Home
Initially after discharge, it may be helpful to families and primary care physicians if the tertiary center program providing care coordination acts as a single point of contact for all tertiary center services. This is particularly true when the child’s conditions cross multiple specialty boundaries, because the tertiary center program is often better able to identify and locate the appropriate consultant. As the family and primary care physician become more familiar with the child and his or her problems they will be better able to decide when the primary care physician will see the child for an acute illness or whether a particular specialist at the tertiary center should be contacted. Indeed, families often develop sufficient expertise to direct the child’s care coordination needs themselves. Electronic communication between the primary care physician and specialists and families may be a helpful and efficient use of time.
Over time, the tertiary center program providing care coordination remains a useful backup to the family and primary care physician and other members of the care coordination team. Changes in disease trajectory, the particular expertise of the tertiary program, or admission to a hospital often necessitate the input of the tertiary center program providing care coordination. Such a program knows the child, family, and primary care physician and thus often reduces hospital stays, decreases unnecessary studies or failed therapies, and facilitates introduction of new resources (eg, at times of transition, when traveling). Finally, maintaining an extended-care coordination team can facilitate assessment of and documentation for needed technology supports.
REIMBURSEMENT OF CARE COORDINATION SERVICES
Care coordination is time consuming and no discussion would be complete without at least brief mention of reimbursement. Time-based common procedural terminology codes can be used to charge for services if more than 50% of the time is spent in counseling and coordination. Private and public insurers generally reimburse office and hospital visits billed accordingly. Prolonged face-to-face service is also reimbursed, although this is relatively limited in care coordination. Services other than face-to-face occupy more time and are less frequently reimbursed. It is therefore essential when providing care coordination to enlist the help of the most expert team members to provide services efficiently and to document carefully all of these services in the hope that insurers will recognize their importance and reimbursement will improve in the future.