21 Introduction to Disease Management
Respiratory and gastrointestinal infections are the most common illnesses seen in pediatric practice settings and may present as minor or life-threatening acute illnesses (Smith, 2011). Noninfectious diseases can also present as acute or chronic conditions, such as those seen with inflammatory or allergic responses, trauma, malignancies, or autoimmune diseases. This chapter provides an overview of the care of the child with acute or chronic diseases, and moves into a general discussion on assessment, management, and educational approaches of all diseases. One of the major roles for the primary care provider is to arrive at a diagnosis and management plan that is consistent with pediatric standards of practice; this may involve telephone triaging. Two areas also deserve special consideration in pediatrics—fever and pain—because these problems can be a part of the clinical presentation. Pain is addressed in Chapter 22; fever and its management are addressed in this chapter.
Acute Diseases in Children
The health care provider begins management of an acute illness by getting a clear understanding of the presenting complaint and taking a complete history. The provider must always remember that these two elements, plus an accurate assessment, diagnosis, and successful management plan are contingent on the following six factors:
1. Careful observation of the child
2. Attention to pertinent positive and negative historical and physical findings
3. Knowledge of physiological functions and developmental considerations that vary by age
4. Consideration of the trajectory of the problem over time
5. Use of shared decision making (SDM) in the evaluation and management of the child. This concept is crucial for a successful interaction between the provider and family or child. It depends on input and feedback from the parents or caretakers and, if appropriate, the child. This resulting partnership takes into consideration culture, patient choices, social milieu, and specific patient needs, and, as a result, improves adherence to a management plan (Butz et al, 2007; Fiks et al, 2010) (see Shared Decision-Making).
6. Use of health literacy concepts. It should not be assumed that any medical term used by the parent means the same to providers. For example, a parent’s definition of fever may be any temperature greater than 99° F (37.2° C), or wheezing to a parent may in fact be rhonchi. A parent who is told that the management needed for the child’s problem is some “tincture of time” may go to the local pharmacy looking for that “medication.”
Feedback from the family and child needs to be obtained in order to ensure that there is understanding and agreement about the management plan and what was told to the parent about the diagnosis and etiology of the problem if known. When satisfied that these six parameters have been given adequate attention, an action or management plan is formed that is acceptable to the parent or guardian, child, and provider.
Shared Decision-Making
The shared decision-making partnership varies with different situations and requires different levels of involvement from the provider; the goal is to jointly make decisions consistent with the patient’s wishes (Kon, 2010). Many parents are interested in helping to make decisions and want support and information in the evaluation of options (Jackson et al, 2008). Other families prefer provider-driven decisions. It is not always possible to have parent or patient input into decisions (e.g., emergency intervention after a life-threatening accident), but some providers assume a paternalistic approach to patients and families in all of their interactions. This approach is one in which the provider tells the family and child about the plan without giving them options. At the other end of the spectrum, all options are presented and discussed with the family or child, and the decision is left up to the family members without any provider input. The shared decision-making approach is somewhere in the middle; the provider, family, and child jointly decide the course of action. A good example of SDM might involve the decision to order diagnostic testing by a primary care provider. The decision needs to consider family, child, and provider preferences because there are many courses of action that can lead to the same end. Parents of a 6-year-old may pressure the provider to order blood work and imaging studies when the diagnosis is clearly primary enuresis and not related to a kidney abnormality. In this situation ordering extensive laboratory and other diagnostic tests would not be the best course of action for the child. Similarly, if the diagnosis appears to be systemic lupus erythematosus, the child would be better served by being referred to a pediatric rheumatologist for confirmation of the initial diagnosis, ordering of laboratory tests, and treatment.
The use of SDM is supported by research findings that show improved health outcomes when the family agrees to the management plan (Hirsch et al, 2010; Merenstein et al, 2005). And yet Cox and colleagues’ study (2007) showed that passive involvement in decision-making by parents and children is more the norm, occurring in 65% of visits. A recent Cochrane review failed to come to any firm conclusion on how best to encourage professionals to adapt SDM into their practice (Légaré et al, 2010). Shared decision-making is a key point in the management of both acute and chronic illness.
Patient Education and Prevention
Primary care providers’ effectiveness is enhanced by their ability to educate children and their families about the prevention of disease and the management of common acute illnesses. The patient-parent educational component of the management plan must be individualized and include the following essential points:
• Information about the length of time it can take before the child improves and symptoms wane; description of what the course of the disease or illness is likely to be and signs of improvement
• Written information about specific signs and symptoms that indicate worsening of the illness, the need for immediate medical attention or for a return visit sooner than planned (e.g., a newborn with a fever of 100.4° F [38° C]; a child with severe lethargy, tender abdomen, labored breathing, stiff neck, bluish lips, purple “dots” on the skin, severe pain, inability to walk, or fever greater than 104° F [40° C])
• Specific, written instructions about when to return for any necessary follow-up or when to be available for a scheduled telephone conference. With electronic medical records, a recall system should be set up to ensure successful follow-up is completed. If the electronic medical record does not allow for a recall, a clinic tickler file is particularly useful for tracking patients whose diagnostic studies or follow-up appointments are crucial to successful management or treatment. This tickler file can be a simple card file, divided by months. For example, a card would list the patient’s name, date, clinic number, medical problem, and contact information and be placed in the file in the month when follow-up is needed.
• Written instructions using key concepts of health literacy to make sure that families truly understand special treatment or therapy, how to use adaptive devices, and how to perform home monitoring tests (see Health Literacy later in this chapter)
• Careful instructions about the proper dosing of medication, the potential for the need to switch medications during treatment, and the side effects of both prescription and over-the-counter (OTC) drugs (see Appendix A and Pharmacological Agents and Patient Education later in this chapter)
• Issues related to administration of medications at school. All appropriate forms must be completed and school personnel instructed on key issues related to pharmacological therapy.
• Specific information about any dietary needs or changes, special hydration needs (i.e., electrolyte solutions or increase in fluid intake), plus any changes in eating patterns that can be expected
• The rationale for and procedures involved with diagnostic testing, including laboratory (e.g., blood, urine, or cerebrospinal fluid [CSF] cultures; skin testing; antibody titers; or rapid antigen testing for preliminary diagnosis), radiographic, or imaging tests and the meaning of results
• Estimations of the length of time or time frame before laboratory or imaging results are available, especially when there will be long waiting periods (these are particularly frustrating for parents)
• Information about the cause, if known, and epidemiology of infectious or noninfectious illnesses or medical conditions, communicability issues, and prevention guidelines, if applicable
• Information about prevention and recurrence risk
• Determination of impediments that prevent the parent or child from complying with the management plan (e.g., limited financial resources, inability to read, dysfunctional family, transportation problems) and discussion about steps to correct these difficulties
• Recognition and discussion of cultural practices and beliefs about illnesses. Discuss the potential benefit or harm from specific folk medicine or complementary and alternative medicine (CAM) practices (including herbal, dietary supplements, or botanical preparations) if used either alone or concurrently with prescribed or OTC medications.
• Information for the working parent about resources for sick care in the community that are convenient (accessible) and affordable
When discussing the management plan with parent(s) and/or child, sit down and make eye contact with them. It is a sign of respect and should be a standard of care. The parents’ or caregivers’ understanding of instructions should always be assessed by asking them to repeat what they have been told. By doing this any misunderstandings can be addressed. One of the ways to obtain this feedback is by using the three questions in the “Ask me three” plan (Abrams et al, 2009; Partnership for Clear Health Communication, 2010):
It is important to allow time for the body’s natural defense system to fight disease. Premature and excessive pharmacological therapy can result in needless iatrogenic disease and resistance to antimicrobial agents and often confuses the clinical picture. The drug of first choice—the one that is least harmful—should be given time to work. Prematurely changing to a new drug, adding additional drugs, and using more toxic drugs are dangerous practices and can decrease confidence in the provider (Ledford et al, 2010).
For the most part, parents are alert to subtle changes in their children, so it is important to listen attentively when parents voice their concerns. A sick child who is medically considered high risk due to physical or social problems merits closer observation and follow-up than does the average thriving child who becomes ill. If the child returns and is not significantly improved or is more symptomatic, the initial evaluation and diagnosis should be revisited by carefully analyzing the symptoms, investigating problems related to compliance or adherence, repeating the physical examination, reviewing likely differential diagnoses, and confirming the diagnosis before deciding on another management plan. Be sure to have the family’s current or contact telephone number in case a telephone contact needs to be made regarding the results of diagnostic tests that come back or to monitor the course of the child’s condition.
The number of infants and young children in group daycare is expanding as the number of women in the workforce increases. This phenomenon creates several issues:
• The disease pattern in this cohort of children is often related to group exposure to illnesses.
• The issue of multiple caregivers can complicate history taking. It is critical to get as much information as possible from as many sources. When the person bringing the child is not the caregiver, the provider should use the phone to communicate with the actual caregiver.
• Sometimes parents express feelings of guilt because they must work and their child is exposed to various communicable illnesses at daycare. Simply explaining that children do get sick during childhood may help relieve stress for parents.
With the diversity of dialects spoken in the United States, language issues can be barriers to providing optimal health care. If a practice setting does not have access to an interpreter or native speaker, interpreter services can sometimes be obtained from local telephone services. It is important that both the health care provider and the parent or caretaker can communicate with and understand each other (see Chapter 3).
Chronic Diseases in Children
The types and characteristics of chronic diseases in children are varied and include a spectrum of rare conditions and genetic or prenatal conditions. Some chronic conditions are not permanent, serious, or obvious, whereas others are irreversible, involve acute exacerbations and remissions, and are readily apparent. The number of children with chronic conditions also varies depending on the definition and methods used to classify a chronic condition (Allen, 2010).
Children with special health care needs (CSHCN) are defined as “those who have or are at risk for a chronic physical, developmental, behavioral, or emotional condition and who also require health and related services of a type or amount beyond that required by children generally” (McPherson et al, 1998, p 138). This definition remains the guiding principle to identify children eligible for federal and state assistance because of their chronic health condition. The need for services, rather than medical diagnosis, is the key factor or criterion that labels a child as having special needs. Using this criterion, about 18% of the pediatric population younger than the age of 18 years has a physical, developmental, behavioral, or emotional condition that requires additional services beyond that required by children generally (Levine, 2011). These children make up nearly 25% of pediatric outpatient visits (Hing et al, 2010).
The number of children reaching young adulthood with severe long-term illness continues to grow with advances in medical and surgical technology, requiring more coordinated care (Stille, 2009), and the percentage of children with certain chronic conditions, such as asthma and obesity, is escalating (Wise, 2007). If the definition of a chronic condition is limited to those with conditions lasting or expected to last longer than 3 months and that limit social functioning or recreational activities, then approximately 6% of children have chronic illnesses. These figures do not include children with speech defects, visual and hearing disabilities, chronic ear infections, chronic skin conditions, and dental decay. Approximately 6% to 7% of children experience illness or disability that interferes with the child’s usual daily activities (Wise, 2007).
There can be great variability in the presentation and the course of illness among children with special needs. They and their families often face a range of problems that are as diverse as the conditions that cause these difficulties. A variety of genetic, congenital, and acquired conditions can lead to permanent or persistent problems that have a significant effect on the child’s and family’s lifestyle. Health care providers must remember that family members are the ones who bear the major daily burden of care. There are several key points to keep in mind when working with these children and their families:
• Integrate clinical practice guidelines as part of the care for patients with special health care needs. These are available at www.guidelines.gov and include guidelines published by the National Association of Pediatric Nurse Practitioners (NAPNAP) and the American Academy of Pediatrics (AAP).
• Prevention of special health problems is a primary goal of care and includes the following:
• Early intervention from the time of birth and afterward to prevent secondary psychosocial difficulties is crucial; the developmental aspects of long-term illness must be addressed.
• Counseling may be needed for the child and family to handle psychosocial and behavioral problems or to discuss their emotions and feelings.
• The child, the family, and school personnel must be consulted to ensure that the child is able to attain realistic developmental milestones.
• Appropriate educational support in school is a right.
Public Law 94-142, the Education of All Handicapped Children Act of 1975, mandates an appropriate education for all school-age children with developmental disabilities in the least restrictive environment.
Public Law 99-457 (1986) provides states with the opportunity to extend benefits of Public Law 94-142 to children from birth to 2 years old.
Prevention of discrimination is a right and is mandated under legislation related to individuals with disabilities. The Americans with Disabilities Act (1990) is a law that provides federal protection in the areas of employment, transportation, public accommodations, and communication for individuals with disabilities. The scope of protection covers both private and public sectors. The Individuals with Disabilities Educational Improvement Act (IDEIA) has been amended several times since the original act was passed to bridge the gap between what children with disabilities learn and what is required in a regular curriculum (IDEA-Public Law 105-17, 1997). It provides for the least restrictive environment for educating all children with disabilities. This act was reauthorized and amended in 2004 and 2008.
Section 504 of the Rehabilitation Act of 1973 for students with disabilities in regular education/inclusive settings prevents discrimination and provides safeguards and support for reasonable accommodations in the school settings, such as ramps, use of assistive technology, special seating arrangement, and permission to hand in assignments late due to illness (Sadof and Nazarian, 2007).• Each state has programs (Title V) to assist CSHCN with medical care and to provide links to social services, state vocational rehabilitation programs, and state school-to-work projects.
• Social service support is essential to help determine financial eligibility for Supplemental Security Income (SSI) or state program benefits (e.g., Medicaid) for individuals with physical, mental, and developmental disabilities, or specific chronic diseases.
• Advocacy for children with chronic conditions and their families includes assisting them to secure coordinated and comprehensive health care and community-based services as needed.
• Provision of primary care services—regular health maintenance supervision and anticipatory guidance—must not be overlooked.
Emotional Support
Helping parents and children more effectively handle the emotional stress associated with a chronic illness and condition is a major focus of care. Key points to be cognizant of include:
• The time of diagnosis and periods of exacerbations of illness are viewed as times of crisis and added stress. Chronic sorrow is a phenomenon that involves feelings of sadness, anger, guilt, or failure. Parents of a child with a chronic condition may experience these feelings at various times during their child’s life. The term was coined by Olshansky in the 1960s to describe cyclical, recurring feelings of sadness during one’s lifetime that are of differing degrees of intensity. It involves grieving without finality. It is not pathological and does not occur uniformly within families (Hobdell, 2004; Roos, 2002; Shepard and Mahon, 2002).
• Developing a trusting relationship with these children and their families involves being respectful and accepting of their varied emotional needs.
• Engaging parents and their children in the treatment plan is a major and essential task (see Shared Decision-Making earlier). Self-management of their disease whenever possible empowers the child, parents, or both.
• Research has demonstrated that more paternal involvement in illness-related support is associated with better family and maternal outcomes in families of children with chronic illness; hence in a two-parent household, participation by both parents in their child’s care and health care visits should be encouraged (Gavin and Wysocki, 2006).
• Partial or poor adherence to complex treatment regimens should be addressed. Motivational interviewing techniques can be a useful tool. Nonadherence issues should be dealt with in a collaborative, “blame-free” problem-solving approach.
• Parents of children with chronic diseases are more likely to think about using, or are using, CAM practices. Respecting their reaching out for additional treatments is important. However, it is not common for parents to reveal this to their conventional pediatric providers, so it is important that the health care provider ask about such practices. Some of these treatments may be harmful or ineffective. (See Chapter 42 for a full discussion on engaging the parents in a discussion about CAM, for being an advocate for the patient, and for becoming a collaborative agent with the family.)
Family-Centered Care
Family-centered care is a key concept that should be used to empower the family. Parents who have infants and young children with chronic conditions should be viewed as therapeutic partners in the management plan. Communications with parents should be open and honest. They should be treated with respect and dignity and allowed to vent their emotions and to use coping mechanisms that work for them. Likewise as the older child and adolescent mature, their partnership role emerges. Relapses in adherence behavior are problematic but not unusual in situations involving complex treatment plans. Problems of adherence to the management plan can lead to serious medical complications, increased rates of hospitalization, greater length of hospital stay, and increased health care costs. Therefore, the provider should explore with parents and children what can help them become more adherent using motivational interviewing (Schwartz, 2010; Suarez and Mullins, 2008). Box 21-1 outlines categories and key factors to consider when addressing concerns about adherence. Training in motivational interviewing, in which the interviewer seeks to ascertain the individual’s level of readiness to change, is a promising technique to use in situations of less than optimal adherence. The key tenets of motivational interviewing are to establish and express empathy; to provide the choice to change or not; to work with patients and families to identify their own personal treatment goals; to work with resistance; to assist in the removal of barriers to change; to provide feedback; and to advocate for the development of patient self-efficacy (Fielding and Duff, 2006) (see Chapter 9 for more information).
BOX 21-1 Key Factors That Affect Treatment Adherence in Children and Adolescents With Acute or Chronic Diseases
Health Care Provider and Environment
• Communication style of health care providers with child, family, and other health care providers; belief in patient empowerment
• Organization of clinic or office setting to be child-, teen-, and family-friendly; need for adaptive modifications in their environment
• Number of health care providers involved in the child’s care; team member collaboration and partnership among themselves and with the family
• Open and “blame-free” approach when adherence issues arise
Family support groups are often beneficial; they offer an opportunity to interact with others who have experienced many of the same challenges, difficulties, sorrows, and triumphs. The provider must address sibling issues and feelings, such as anger or embarrassment; a sense of being overwhelmed with added responsibilities; or believing they need to be the protector for their brother or sister. These groups can be face-to-face or Internet based depending on patient preferences.
Establishing a Medical Home
Children with chronic diseases have unique health and psychosocial needs. The health care provider may give care to a child with a rare disease or disorder or be involved with the management of a child with a much more common chronic condition, such as asthma or cerebral palsy. Of note, an increasing number of primary care providers are involved in the specialty care of children with chronic conditions. Each child with a chronic illness deserves a medical home where they, as unique children and families, can receive comprehensive, culturally effective, community-based, family-centered coordinated health care (McAllister et al, 2009).
The critical issue in health promotion and disease management for children with special health needs is to ensure an organized and coordinated approach to provide appropriate treatment for the child’s specific chronic disease or condition and to ensure that the child’s primary health care needs are met. The goal of the medical home should be to: (1) provide family-centered care; (2) provide clear, unbiased information about medical care, management, and community resources; (3) provide all-encompassing primary care that is available on an in-patient and outpatient basis 24 hours a day throughout the year; (4) provide care over an extended period of time that allows for transitions to adult care; (5) provide appropriate referrals to subspecialists and care coordination with the team; (6) maintain a record of pertinent information; (7) interact with educational systems including early intervention programs; and (8) provide developmentally appropriate and culturally competent counseling to ensure optimal outcomes (Medical Home Initiatives for Children With Special Needs Project Advisory Committee, 2008).
Health care management for children with special health needs includes: (1) assessing their needs; (2) planning comprehensive health care to provide for physical and psychosocial needs; (3) facilitating and coordinating services; (4) following up and monitoring services given and the child’s progress; and (5) empowering the child and family through education, counseling, and support. Addressing issues up front about quality of life should always be part of the assessment process in chronic pediatric illness management. Child and parent perceptions about quality of life issues, such as physical and emotional pain and discomfort, may not be the same as those held by the health care provider. Child, parent, and health care provider may each have different perceptions. It is vitally important to determine how the child and the parent feel—physically, emotionally, and socially—by listening to them and asking for their input, rather than assuming that all is going well based on outward appearances. Health care management of children with chronic disease is about empowering them to live their lives to the fullest potential.
Providing quality care requires screening patients with chronic conditions who are at risk for other medical problems. For example, patients with Down syndrome have a 3% to 5% risk for hypothyroidism and should receive yearly screening for thyroid disease (AAP and Committee on Genetics, 2001). Prevention of further disability by ensuring that screening and clinical practice guidelines for chronic disease management are meticulously adhered to is a key component of the medical home.
The level or type of involvement in the treatment and management of a child with a specific chronic disease may vary depending on the unique situation of the child and family and the health care provider’s subspecialty training and education. Strategies related to fostering the child’s psychosocial development should be addressed at each health care encounter. A holistic approach to care is a major tenet of the medical home model. Certain situations may require additional advocacy when children with special needs and their families are in a particularly vulnerable position (e.g., if the parent of a child with special needs loses his or her job or suffers significant illness or injury and cannot adequately provide for the child). Children with chronic conditions do well when family functioning is high and there is positive family adaptation. The philosophy underlying the medical home model is to provide for open communication between parents and providers and to advocate for effectively and efficiently coordinated health care services.
Although chronic illnesses are diverse in their severity and effect on the child, certain issues are often common concerns for children with chronic conditions and their families and their health care management. By using a medical home model, members of the health care team can communicate about a variety of issues (Stille, 2009). They include the following:
• The high cost of treatment—the potential need for financial assistance
• Lack of, or difficulties and barriers in, acquiring health care insurance
• Family lifestyle alterations that may be required of parents, siblings, or both, in caring for the child
• The need to overcome system barriers that families may face navigating through the maze of agency paperwork
• The need for supervised care by multiple health care providers and the frequent lack of coordination of services in providing continuity of care
• Unpredictability of the condition and the potential for complications, frequent medical visits, hospitalizations, and death
• The desire to be kept informed of their child’s condition and progress
• Treatments or procedures that may be embarrassing, painful, or time consuming
• The developmental effect that chronic disease can have on a child, especially during adolescence and early adulthood (periods of increased vulnerability)
• Longevity concerns—ability to live and function independently as an adult, including the need for career and vocational counseling
• The level of knowledge parents need about the pharmacological management of pain and the disease process or other therapeutic treatments, including nutritional support for the at-home care of the child
• The effect of stress on emotional and psychological well-being of the child and family members—parents or caregivers, siblings, and possibly the extended family support network
• Parental striving to successfully normalize their child’s life by acknowledging the child’s condition and its effect on family lifestyle while actively engaging in accommodations to focus on the child and not the condition
• Dealing with feelings (e.g., anger, sorrow) while attempting to cope with chronic illness
• Developing advocacy skills for these children to access services through schools, state and community agencies, or special federally sponsored programs
• Securing special illness-related equipment (e.g., movement and mobility aids, such as walkers, wheelchairs, or braces) or acquiring communication aids, such as hearing aids or special computers with voices
• Finding respite care or transitional care for the dependent adult child
• Legal conservatory issues and the concern about who will care for the child as an adult when parents are no longer capable of providing physical care or are deceased
Pediatric health care settings should work to establish a medical home with a multidisciplinary team model and care coordination. These teams offer the expertise of many individuals in a united approach. In ideal situations the involvement of a clinical social worker, a community health nurse, or a nurse case manager is important to secure essential community resources for the child and family. Parents or guardians are a crucial part of the team. All team members must remember to respect the knowledge that parents or caregivers have about their child, their child’s condition, and how the child is likely to respond physically and emotionally to new therapeutic interventions or treatments, situational changes, or exacerbations of illnesses. Other principles coming out of the medical home model are to:
• Develop a database for CSHCN who require additional contact time outside of the typical scheduled time frame for either sick or well visits and be sure to flag their charts. An electronic medical record system may provide a function for this. This will help in scheduling additional visit time and alert the office staff when scheduling visits.
• Designate a care coordinator for each special care needs patient and train the staff about the medical home concept.
Assessment
History and Physical Examination
Chapter 2 discusses the complete history and physical examination of children from infancy through adolescence. In addition, each of the pediatric disease management chapters in this unit focuses on key questions to ask in history taking and highlights significant findings to be alert to if found on physical examination. Careful attention must be given when analyzing the signs and symptoms of a child’s illness, including the presentation of clinical findings, the course of the disease process, and its associated manifestations. A clear history of the illness is essential and requires a comprehensive description of any symptom or sign of illness.
To help assess the severity of illness in infants and young children, careful attention must be given to judging key indicators during the history and the physical examination (Box 21-2). These indicators are an important part of the assessment and judgment when determining management, and include level of consciousness, hydration, color, respiratory status reaction to stimulation, sleep-to-awake or awake-to-sleep state, and response to social cues. The child should be noted to have either a normal (NL), moderately impaired (MI), or severely impaired (SI) response in each of the key areas (McCarthy, 2007; National Institute for Health and Clinical Excellence, 2007).
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