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It has been estimated that almost 55,000 children die each year in the United States. At least 50% of these children die during the newborn period or within the first year of life. Many of these children, particularly those older than 1 year of age, suffer from illnesses that are clearly life-limiting. Thousands more children are diagnosed with life-limiting illnesses, resulting in a chronic condition that may last for many years, even decades. Furthermore, children who are diagnosed with life-threatening illnesses that may be curable, such as cancer, continue to live with the potential of a recurrence of their malignancy for many years. The above populations are those where palliative and end-of-life care could play an important role during the illness of these patients.
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Across the globe the need for pediatric palliative care varies depending on the country. For example, the estimated need for pediatric palliative care ranges from 120 per 10,000 children (Zimbabwe) to 20 per 10,000 children (United Kingdom).
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Although commonly used interchangeably, palliative care and end-of-life care are not synonymous terms. Palliative care aims to prevent, relieve, reduce, or soothe the symptoms produced by potential life-limiting illnesses or their treatments and to maintain the patient’s quality of life along the entire continuum of treatment. Provision of palliative care does not imply imminent death nor does it prohibit aggressive curative treatment modalities. Rather, it acknowledges the uncertainty and potential for suffering inherent in a potentially life-limiting condition such as cancer. Understanding how a family defines quality of life and suffering for their child is imperative and provides a framework for decision making between care provider and the family throughout treatment.
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While a child is doing well with treatment, the primary focus will be on achieving cure or stabilization of the disease. Palliative care goals at this time focus on promoting quality of life in preparation for survivorship in the face of a potentially life-limiting illness. Some of these goals include helping a family come to terms with the diagnosis, addressing issues of treatment-related pain and distress, facilitating reintegration into the social realms of school and community, and promoting as much normalcy in the child’s life as possible. When it becomes clear that the chances for cure are poor or present an unreasonable cost to the child’s quality of life, the goals of palliative care will shift toward end-of-life care. The focus will still be on promoting quality of life but now in preparation for a comfortable and dignified end of life with increasingly less attention given to the treatment or cure of the disease itself.
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Palliative care not only comprises support in the pain and symptom management of the disease but also addresses equally the psychosocial, emotional, and spiritual needs of the patient with a potential life-limiting illness and their family.
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CHILDREN WHO MAY BENEFIT FROM PALLIATIVE CARE INTERVENTIONS
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In a recent review by Himelstein et al, conditions that are appropriate for palliative care were divided into four groups as follows:
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Conditions for which curative treatment is possible but may fail such as advanced or progressive cancer and complex and severe congenital or acquired heart disease
Conditions requiring intensive long-term treatment aimed at maintaining the quality of life such as HIV/AIDS, cystic fibrosis, and muscular dystrophy
Progressive conditions in which treatment is exclusively palliative after diagnosis such as progressive metabolic disorders and certain chromosomal abnormalities
Conditions involving severe, nonprogressive disability, causing extreme vulnerability to health complications such as severe cerebral palsy and anoxic brain injury
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The United States Congress mandated in 2010 that palliative care will be covered concurrently with curative therapies for children with terminal conditions who are receiving Medicaid. Based on the Patient Protection and Affordable Care Act, a voluntary election to receive hospice care for a child does not constitute a waiver of any rights of the child to be provided with, or to have payments made for services that are related to the treatment of the child’s condition. This significant milestone in pediatric palliative care should open the door to concurrent care being covered by private insurance companies in the future.
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PAIN MANAGEMENT IN PEDIATRIC PALLIATIVE CARE
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Optimal pain management is critical when providing pediatric palliative care. (See the section on Pain Management earlier for definitions and guidelines for treatment.) As end of life approaches, dosing of comfort medications may eventually exceed normally prescribed doses. The goal at all times must be to achieve and maintain comfort. When pain management at the end of life is provided with this goal at the forefront and in concert with careful ongoing assessment and documentation of the child’s symptoms, there should be no reason to fear that this action is tantamount to euthanasia which is a conscious action intended to hasten death.
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QUALITY-OF-LIFE ADJUNCTS & SYMPTOM MANAGEMENT IN PEDIATRIC PALLIATIVE CARE
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When offering treatment to children with a life-limiting illness particularly at the end of life, certain nonpain symptoms and signs may develop more quickly in children when compared to the adult population. A thorough and complete history and physical examination should be obtained. It is critical to determine how much distress the symptom causes the child and how much it interferes with child and his/her family’s routine when deciding upon treatment. Areas of management should include drug treatment, nursing care, and psychosocial support. Symptoms that commonly occur during disease progression and at the end of life in children with a life-limiting condition are listed in Table 32–6, with suggestions for management.
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Complementary & Alternative Modalities
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It is not unusual for families seek complementary or alternative modalities (CAM) for their child when mainstream treatment has failed or is unavailable. Children with chronic conditions such as cancer, asthma, sickle cell disease, and epilepsy have a higher incidence of CAM usage compared to the general pediatric population (Post-White, 2009). The use of CAM in children is influenced primarily by parental use and acceptance of CAM. Culturally accepted beliefs and practices also play an important role. In Asia, the use of meditation and prayer as a method to control pain is well supported by the medical community. In Europe, the use of homeopathic remedies is commonplace as children approach end of life, many families opt to try some form of CAM. Most often, these treatments are aimed at improving physical or spiritual quality of life. Sometimes the goal is a desperate hope to find a treatment when other options have failed or an attempt to find something perceived as less toxic than mainstream treatments to induce remission, support the child’s ability to fight the disease, or prolong life. Parents report using CAM gives them a sense of control and hope. The most common modalities reported in pediatrics are prayer/meditation, relaxation techniques, massage, chiropractic care including acupuncture, and nutritional supplements (Post-White et al, 2009).
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Studies of the effectiveness of CAM use in children have been small and the data are often conflicting. There is generally an acceptance for the lack of harm associated with mind-body techniques such as prayer, meditation, touch and sensory modalities, and relaxation. Acupuncture and acupressure are gaining more acceptance in the Western medical community and may be beneficial in some children for relief of pain, nausea, and other symptoms. Touch and sensory modalities such as massage, healing touch, and aromatherapy can induce a relaxation response in some children which can be very helpful. The use of supplements including botanicals and vitamins has been of more concern due to the lack of dosing information for pediatrics, lack of standardization of products, and the potential for serious drug interactions and toxicities. Treatments touted as alternative “cures” are likely not beneficial and can have very dangerous consequences. The cost of CAM, particularly botanicals and alternative medicine treatments, can be prohibitive and the cost is rarely covered by insurance. Hospice providers frequently incorporate relaxation and mind/body/spirit modalities into their programs.
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It is important for the health care provider to ask parents and adolescents about CAM usage and to be open to discussion with the family about modalities they are using or may wish to consider. Parents consistently have reported in studies their desire to inform and discuss CAM with their health care provider, but may be reluctant to do so if they are unsure what response they will get from the provider. Providing families with clear information about the treatment they are considering or using and any contraindications is key. In some cases, recommendation of complementary techniques such as massage, mind/body modalities, and acupuncture/acupressure may be appropriate.
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PSYCHOSOCIAL ASPECTS OF PEDIATRIC PALLIATIVE CARE
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Pediatric palliative care is unique in that caregivers must be familiar with children’s normal emotional and spiritual development. Working with a child at his or her level of development through the use of both oral and expressive communication will allow the child to be more open with respect to hopes, dreams, and fears. A child’s understanding of death will depend also on his or her stage of development. Children understand death as a changed state by 3 years of age, universality by 5–6 years of age, and personal mortality by 8–9 years of age. Table 32–7 gives a broad overview of children’s concepts of death and offers some helpful interventions.
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CHILDREN’S CONCEPT OF DEATH
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As end of life approaches, psychosocial support is invaluable to the child and family. Children may need someone to talk to outside of the family unit who can respond to their questions and concerns openly and honestly. Parents may need guidance and support in initiating discussions with or responding to questions from their child about death and dying. Children and adolescents may have specific tasks they wish to complete before they die. Some want to have input into funeral and memorial service plans and disposition of their body. Parents often need support in making funeral arrangements, handling financial concerns, talking with siblings and other family members, and coping with their own grief.
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It is important to recognize that grief is not an illness but a normal, multidimensional, unique, dynamic process presenting as pervasive distress due to a perceived loss. Once parents have accepted the reality of the loss of the child, they must then complete the other tasks of grief such as experiencing the pain of their loss and adjusting to an environment without their child in order to move on with their lives. Parents who lose a child are at high risk for complicated grief reactions such as absent grief, delayed grief, and prolonged or unresolved grief. Siblings are also at risk for complicated grief and require special attention.
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SPIRITUAL & CULTURAL SUPPORT
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Health care decisions are often intertwined with a family’s culture and belief system. Understanding the influences of a family’s beliefs and culture allows the practitioner to provide sensitive, appropriate care, particularly at the end of life. Interaction with members of the family’s faith and cultural communities can often be instrumental in helping both the care team and the community support of the family. Allowance for specific prayers, rituals, or other activities may help facilitate procedures and discussions.
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Families who speak a foreign language probably suffer from inadequate support the most. Every effort should be made to find and utilize a qualified interpreter, particularly for any discussion that involves delivering difficult news or making critical decisions. Many times, the role of interpreter is imposed upon a bilingual family member or friend who may not understand medical terminology well enough to translate clearly or who may deliberately translate the information inaccurately in an attempt to protect the family.
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WITHDRAWAL OF MEDICAL LIFE SUPPORT
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Medical technology has enabled many children with serious health conditions to enjoy a good quality of life. When technological support no longer enables a child’s quality and enjoyment of life or there are no viable options to restore quality of life to the child, it may be appropriate to discontinue it. Feeding tubes, ventilators, dialysis, parenteral nutrition, and implanted cardiac pacemakers are examples of medical modalities that may need to be re-evaluated when a child’s condition deteriorates or in the case of a catastrophic injury.
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There are five circumstances in which withdrawal of medical support and technology can be considered in children (Tournay, 2000). (See the following table.)
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Helping families identify and define what quality of life means to their child and to the family and what would be an intolerable life for the child is important. It is critical to present in a clear and understandable format the child’s medical condition, test results, and the treatments that have been tried, what the expectations are for the child’s ability to survive or function and interact with his environment, and why it is believed that current or additional interventions will be futile or induce further suffering. These discussions should be conducted with sensitivity and without need for an immediate answer from the parents. It often takes several such discussions for families to come to a decision that they themselves will be able to live with and families should not be rushed into decision making. The family may request additional testing or retesting to assure themselves they are making the right decision for their child. When feasible, these requests should be honored. Spiritual support may be very helpful to families during this process and should be offered.
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Once the family has made the decision to withdraw support, it is helpful to explain what the anticipated course will be following withdrawal, what the child will likely look like during that time and what the plan of care will be to ensure comfort. Create a plan with the family for time and place of withdrawal, who they would like to be there with them, any specific requests for environment such as music, a favorite movie playing, or a book being read, and who they would like to perform the withdrawal. Offering the opportunity for rituals, prayer, or private time prior to or during the withdrawal is appropriate. If death is anticipated to happen quickly after withdrawal, any specific religious requirements for the body after death should be arranged in advance. In all cases of withdrawal, the family should be offered support during the process and after the death occurs.
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ADVANCE CARE PLANNING
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Advance care planning allows patients and families to make known their wishes about what to do in case of serious or life-threatening problems. Himelstein et al describe advance care planning as a four-step process. First, those individuals considered decision makers are identified and included in the process. Second, an assessment of the patient’s and family’s understanding of the illness and prognosis is made and the impending death is described in terms that the child and family can understand. Third, on the basis of their understanding of the illness and prognosis, the goals of care are determined regarding current and future intervention—curative, uncertain, or primarily focused on providing comfort. Finally, shared decisions about the current and future use or abandonment of life-sustaining techniques and aggressive medical interventions are made. In the event of a disagreement between parents or parents and their patient regarding these techniques or interventions, it may be prudent to involve the hospital’s ethics committee in order to resolve these issues.
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Some states permit parents to sign an advanced directive that asserts their decision not to have resuscitative attempts made in the event of a cardiac or respiratory arrest outside of the hospital. When an advanced directive is in place, emergency responders are not required to provide cardiopulmonary resuscitation (CPR) if called to the scene. Some school districts will respect an advanced directive on school property, many will not. If a child with an advanced directive in place wishes to go to school, a discussion between the medical team and school officials should be arranged to determine the best plan should the child have a cardiac or respiratory arrest at school.
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Parents and, occasionally, the child may bring up the possibility of donating organs or body tissues after death. Although the tissues that may be donated by a child may be limited in some instances by the type of disease (eg, cancer), some parents find immense comfort in knowing their child was able to benefit another. If the parents have not discussed donation with the physician by the time of death and donation is possible, the physician should offer the opportunity to the family.
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Autopsy is another subject many physicians find difficult to approach with a family, but it is an important option to discuss. In cases of anticipated death from natural causes, autopsies are generally not mandatory; however, information obtained from an autopsy may be useful for parental peace of mind or medical research. If death at home is to be followed by an autopsy, special arrangements for transporting and receiving the body will need to be made with the mortuary or the coroner.