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
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.”
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.
Patient Education and Prevention
• 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
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 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
• 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.
• 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
• 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).
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
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.
• 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
• 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).
BOX 21-2 Indicators for Assessing Severity of Illness in Pediatric Patients and a Scoring Guide
1. Level of consciousness or quality of cry
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