Why Is She So Distressed?

Illness often raises many questions related to humanity and life meaning both for children and their families, and many lean on spirituality to sustain them in difficult times. When one’s ability to experience and integrate meaning in life becomes disrupted, patients may experience spiritual distress, which can present in physical, psychological, and social ways. The majority of pediatricians acknowledge a positive role for spirituality in healing, in strengthening the therapeutic relationship, and in providing support for patients/families, yet clinicians often feel ill-equipped to address spirituality with families. However, most parents would prefer to be open about their spiritual beliefs and practices with their medical team. In addition to the clinician, chaplains, child life specialists, social workers, and psychologists can support the evolving spiritual needs of children as they grow and develop.

I Just Want to Feel Normal!

Medication nonadherence is common in adolescents and places patients at higher risk of significant health complications. Medication nonadherence should be considered whenever a patient’s medical condition is not under control. Common barriers to treatment adherence include disease frustration, adolescent development, medication regimen issues, and cognitive or mental health concerns. Routinely assessing treatment adherence helps clinicians identify barriers and develop strategies with the patient for addressing those barriers. A collaborative team approach can create a safety net to support the patient’s treatment adherence. This chapter describes the factors associated with adherence, offers strategies and tools for assessing and identifying the barriers to adherence, and outlines a collaborative process for improving adherence.

A Moment Becomes a Memory

Anencephaly is a lethal neural tube defect that results in the absence of skull and scalp with only remnants of brain and brain stem remaining at birth. Although there is a high risk for intrauterine fetal demise and stillbirth, neonates affected by anencephaly can be born alive and may live for hours to days. There is no cure or treatment for anencephaly; however, symptom management, end-of-life care, and family support should be offered. Some families may find it difficult to see the exposed brain; acknowledging this challenge and using bonnets and coverings can help ease this discomfort. Organ donation for transplant is not currently an option for infants with anencephaly, but tissue donation and research donations are potential options in some areas. There is a wide spectrum of severity and survival in other brain abnormalities; each case should be evaluated individually, and palliative programs should strive to align care with family goals.

My Patient Is Twitching; Could It Be Itching?

Pediatric patients receiving palliative care may experience itching as a distressing symptom for a wide variety of reasons. Antihistamine medications are widely used for treatment of this symptom and have effectiveness if histamine is the primary mechanism of itch. Pruritus is a known adverse effect of opioid therapy, occurring in 2–10% of patients. Opioid-induced pruritus is not primarily histamine related and therefore better treated by partial opioid antagonists. Chronic health conditions such as renal disease, cholestasis, and malignancy are all associated with pruritus. This chapter presents a broad differential for the symptom as well as a range of pharmacologic and nonpharmacologic therapies.

No Access and Still in Pain

Pain management for children can be intimidating for providers unfamiliar with pediatric practice. Finding the correct dose can involve weight-based dosing and calculations of suspensions, which are overall more involved than adult dosing strategies. When there is a lack of intravenous access or the inability to use the gastrointestinal tract, finding the correct route of pain medication for children can be challenging. However, following simple strategies can help find the best option for pain relief. The least invasive route of medication administration should be selected first, and nonpharmacologic strategies should be layered in to enhance pain control. Pain should be assessed regularly, and therapy should be escalated when indicated. The pain medication should be matched with the type of pain and anticipated duration of painful stimulus. Skin integrity, weight-based dosing limitations, and prognosis should be considered in the selection of agents. Comfort position, supportive caregiver, and distraction and integrative therapies depending on age should be integrated into any treatment regimen.

Can You Go Change the Family’s Mind?

Parents and legal guardians of seriously ill children are often faced with challenging decisions regarding code status and cardiopulmonary resuscitation. There is commonly a certain amount of discomfort and negative emotions surrounding these conversations. Unfortunately, because of this, when clinicians do initiate these conversations, it is often too late in the course of disease, and opportunities for the children and their families to have their preferred end-of-life wishes may be missed. This chapter reviews the common myths and misconceptions surrounding the code status conversation by addressing the emotions associated with it. In addition, the chapter provides a step-by-step guideline on how to approach this topic.

Tiny Fingers, Tiny Toes

Preterm birth is defined as birth prior to 37 weeks of gestation. Preterm birth can have a significant impact on the infant’s survival and neurodevelopmental outcomes. Extremely preterm infants have the highest risk for mortality and severe disabilities. Medical teams should have honest conversations about outcomes and expectations with parents and elicit their perspectives on quality of life and family goals. Neonatal intensive care unit (NICU) admissions can have phases: the crisis admission phase, the middle marathon phase, and the discharge phase. Parents can experience significant distress during a NICU admission, and they are at higher risk for mental health difficulties. Medical teams should layer in support during the different phases of NICU admission.

Teenage “Bleh”

Fatigue is a common and troubling symptom in children with serious illness. Coping with fatigue impacts and diminishes quality of life in multiple ways. Despite the well-established impact of fatigue, clinicians tend to underrecognize and undertreat this symptom. This chapter examines fatigue as it presents in pediatric patients and suggests best practices for evaluation and treatment. Common causes of fatigue include anemia, medications, pain, disordered sleep, nutritional imbalance, changes in physical activity, emotional and/or spiritual distress, and medical comorbidities. Each case should be evaluated separately, with a focus on treating the underlying cause of the fatigue when possible. An interdisciplinary approach is often required to fully address the factors contributing to a patient’s fatigue. Nonpharmacologic approaches have been shown to be effective and may be used in concert with pharmacologic agents.

Always Putting My Needs Last

Caring for an ailing child can have significant impacts on a caregiver’s physical, spiritual, emotional, and socioeconomic domains of life. Comprehensive care of the whole family includes an assessment and plan for the caregiver’s well-being. Interval evaluation of caregiver well-being can help anticipate future needs such as home care, respite, and counseling. Palliative care interventions should include caregiver assessments and creation of layered support for the whole family. Close attention should be paid to signs of caregiver fatigue, burnout, grief, and neglect of basic needs. Caregiver support can affect downstream care for the ailing child by fortifying the people who are responsible for the medical care.

Stuck on Life Support

Children with medical complexity are living longer with greater technology dependence. However, with each new technology, families face a new decision with new benefits and burdens. Parents in online communities share details of their child’s medical care, which sometimes leads them to inquire about medical interventions they may have learned about from another parent whose child has the same disease. Palliative care teams often help families make decisions with regard to new medical technology. This chapter discusses common decisions families face concerning initiating new medical technology and what families want to hear from their providers before making these difficult decisions.