Single-Center Experience Providing Palliative Care to Pediatric Patients with End-Stage Renal Disease

Abstract

Background:

End-stage renal disease (ESRD) affects nearly 1400 new children each year in the United States. Morbidity and mortality rates remain high for pediatric patients with ESRD, including those that have received a renal transplant.

Objective:

To better understand ESRD patients referred to palliative care, including their physical symptoms, topics discussed, and themes emerging during initial palliative care consultation.

Design/Subjects:

This study is a retrospective chart review of pediatric ESRD patients who received a palliative care consult. Physical symptoms, core topics, and themes were identified by the interprofessional study team.

Results:

The study team found 35 patients met inclusion criteria during the study period. The most common standard palliative care metric noted was “complex or time-intensive communication and interdisciplinary social support.” Pain was the most common physical symptom addressed with goals of care and communication the most common topics discussed. Themes emerging described the emotional distress of patients and parents as well as prognostic discussions.

Conclusions:

This study demonstrates one institution's experience with pediatric ESRD patients undergoing consultation with the pediatric palliative care service. More research is necessary in this population to better describe the best focus for palliative care teams.

Introduction

End-stage renal disease (ESRD) affects approximately 1400 new children each year in the United States, with a prevalence of 9721.¹ Although curative therapy does not exist at this time, modalities such as hemodialysis (HD), peritoneal dialysis (PD), and transplant offer life-prolonging options. During the first year of therapy, more than one-third of pediatric patients receive a transplant. However, despite drastic improvements in care in the past 20 years, median transplant graft survival is only 12.3 years. In addition, mortality rate for all ESRD patients remains high, at 11 deaths per 1000 patient years,¹ increasing as patients live with their disease and develop complications of therapy.

Distress in the ESRD patient population is common, with nearly two-thirds of caregivers describing dialysis initiation as “traumatic.”² Subsets of these caregivers (22%) as well as many of the patients (12%) go on to meet diagnostic criteria for medical traumatic stress.² Measured healthcare-related quality of life (HRQOL) is significantly lower in patients on HD and PD than in children who undergo renal transplantation,³ and all modalities of dialysis or transplant have lower HRQOL than those in healthy children.⁴ Pediatric nephrology guidelines include assessing and implementing strategies to improve HRQOL.⁵ Access to palliative care, which has been defined as “an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness,”⁶ provides one such strategy to support children with ESRD. In addition, transplant medicine recommendations suggest discussing palliative care early in the course given the inherent risks of undergoing and waiting for transplant.⁷ Most recently, summary recommendations for dialysis patients by the Renal Physicians Association advocate to “develop a palliative care plan for all pediatric patients with ESRD from the time of diagnosis and for children with acute kidney injury who forgo dialysis. The development of a palliative care plan is a continuation of the process of advance care planning and should be family-centered.”^8–10

Although palliative care seems to be a natural fit for patients with ESRD, little information exists on how this type of care can be provided to pediatric patients. In the adult literature, this concept is better described,^6,11–13 although standard practice has yet to be well defined.

The primary objective of our study was to describe palliative care consultation in a single institution pediatric ESRD population, including demographics, categorizations of palliative care team recommendations, primary symptoms described, and topics addressed in the consultation.

Methods

This exploratory retrospective study reviewed pediatric palliative care consultations for children with ESRD from January 2006 (when the Palliative Care Program began) through December 2012 at C.S. Mott Children's Hospital. Charts were identified through electronic medical record search for billing diagnosis of “end-stage renal disease,” “encounter for palliative care,” and referral for palliative care consultation. Patients were excluded if they did not meet age criteria (0–18 years at time of consultation) or if there was not a completed initial consultation note. Each palliative care consultation was a coordinated evaluation with 1–4 members of the Pediatric Palliative Care team, consisting of physicians, nurses, a social worker, and a spiritual care provider. Demographic information and standard metrics were collected on the patients identified, and then consults were extracted and deidentified by a research assistant. Standard metrics are five classifications used by this institution, which were defined by the program at its inception to describe the five basic tenets of palliative work. These are used in a consistent manner across consultations for patients of any clinical background and include the following: complex communication and interdisciplinary social support, education regarding palliative care and hospice philosophies, clarification of goals and difficult decision making, resources, and symptom management. After deidentification, a larger clinically focused study team analyzed each initial consultation documentation for symptoms and topics addressed. The study team then met in three separate iterations to establish consensus between all analyzing team members on the content of each individual consultation and its highlighted topics. Although the topics were initially meant to be coded in a binary manner, certain topics did not fit uniformly into this strategy. Thus, the study team opted to do a modified thematic content analysis of several areas by coding these into themes.

Analyzing study team members included a pediatric nephrologist, pediatric dialysis nurses, a pediatric dialysis social worker, pediatric palliative care providers, and a pediatric palliative care social worker.

Descriptive statistics were analyzed in Microsoft Excel.

Results

During the study period, 43 charts were identified: 35 met study criteria. Exclusions were for age criteria and incomplete consultation (refusal or unable to schedule). Patients were aged 35 days to 18 years. Table 1 shows study characteristics of the patient population. Although only the initial consultation was analyzed, the range of visits with the palliative care team was 1–46, with a median of 3 visits. Twelve patients (34%) were deceased at time of chart review. Of those who were deceased at chart review, an average of 486 days elapsed between the initial consultation and the patient's death.

Table 1.

Study of Patient Characteristics, n = 35

	n (%)
Age mean: 7.5 ± 7.2
<1 year	10 (28.6)
1–10 years	11 (31.4)
>10 years	14 (40)
Gender
Female	14 (40)
Male	21 (60)
Type of dialysis
Acute hemodialysis only	6 (17.1)
Chronic hemodialysis	141 (31.4)
Peritoneal dialysis	9 (25.7)
Dialysis not yet initiated	6 (17.1)
Acute and chronic hemodialysis	3 (8.6)
Patient location at consultation
Inpatient	14 (40)
Outpatient	21 (60)
Referral service
Nephrology	16 (45.7)
Nonnephrology	19 (54.3)

The two most common palliative care domains were complex communication and interdisciplinary support (65.7%), followed by education regarding palliative care and hospice philosophies (57.1%). Other metrics noted were clarification of goals and difficult decision making (40%), resources (40%), and symptom management (37.1%). Code status was also assessed. In 34 of 35 consults, code status was either “full code” or not addressed, which would default to “full code” at our institution.

Physical symptom discussions were noted for each consultation, as shown in Figure 1, with pain being the most prominent of symptoms (57%). Aside from physical symptoms, the remainder of the topics evaluated can be broken down into communication, consultation and counseling, or “other,” with frequencies shown in Figure 2. Iterative review of the consultations led to further thematic analysis of specific areas as is noted in Table 2.

FIG. 1.

Percentage of initial consultations with pediatric palliative care in which specific physical symptoms were described by ESRD patients. ESRD, end-stage renal disease.

FIG. 2.

Percentage of consultations with specific discussion focus during initial consultations between ESRD patients and pediatric palliative care team.

Table 2.

Themes Emerging from the Initial Consultations

Theme	Examples
Parents express emotion and worry regarding the current care and ultimate outcome for their child.	• “(Parent) was repeatedly tearful as she shared feeling as though patient's pain ‘was questioned’ by healthcare providers in the past”
	• “(Parent) reports being quite emotionally overwhelmed by the demands on her time and energy, such that she is not able to meet her own needs.”
	• “(Parent states) she would commit suicide because she ‘cannot live’ without patient…”
	• “(Parents) ‘break down’ occasionally when they are faced with the reality of patient's condition and the likelihood that she may not get to experience many important milestones of childhood and adolescence.”
	• “(Parent) tearfully states that she can't believe she is having to experience the serious illness and eventual death of another child.”
Patient's negative emotions focus on care already provided.	• “(Patient) longs to return home and remains frustrated with the restrictions placed on his diet and his ability to leave the hospital.”
	• “(Patient) states that when she was previously on dialysis, she felt that it ‘tore her up’ and completely depleted her both physically and emotionally.”
	• “(Parent) describes patient as both fearful and anxious about dialysis appointments such that he has difficulty sleeping the night before.”
	• “(Parents) feel that he is very angry about all that has happened to him”
Patients and parents engaged in rich discussion about prognosis.	• “(Patient) said he was not afraid to die and was aware that if his renal disease progressed he would likely die in the coming months to years.”
	• “(Patient) expressed understanding that he had ‘bad kidneys’ and without a transplant he could die. He also expressed that even after he gets a kidney transplant it is likely that those kidneys will only last 10–13 years if he takes good care of them.”
	• “(Parent) is struggling with the patient's illness. She hopes for her ‘to be happy as long as she's here,’ but senses that the patient will likely die within the next few years despite optimal medical therapies.”
	• “(Parent) knows her disease is not curable, but thinks that she has months rather than weeks, should she not suffer another complication.”
	• “(Patient) and his parents have come to a perspective of his very poor prognosis and likely premature death.”

Although the sample size was small, there was a trend that inpatients were more likely to discuss goals of care.

Discussion

This study demonstrates a single institution cohort of pediatric ESRD patients who underwent palliative care consultation. Emotional symptoms were prevalent, especially parental emotional distress. No single physical symptom characterized all patients. Topics addressed were consistent with those addressed in other pediatric palliative care populations^14–16 with goals of care being most prominent, although communication issues, especially communication with healthcare providers, were also important. To our knowledge, this study provides the only look at palliative care specific to the pediatric ESRD patient.

Although national guidelines support palliative care in patients with ESRD, little diagnosis-specific information exists regarding what these conversations might entail. In this sample, advanced care planning and addressing current goals of care were focuses of many of the conversations. In addition, physical symptom burden was common, suggesting a focused role for the pediatric palliative care practitioner.

The qualitative data collected, shown in Table 3, highlight the intense and emotionally draining nature of caring for the patient with ESRD on a regular basis. Parental distress was noted to be much more descriptive, perhaps because of the initial consultations focusing more on parental insights. Prognostic conversation topics also suggest that patients and parents recognize that this disease is life limiting, despite the “full code” status of most patients.

As with any study of this type, many limitations exist. The sample size was very small given this is a single institution study and there is a low prevalence of ESRD in the general pediatric population. Owing to the limited number of centers that provide ESRD care in pediatrics, the search reflects the regional patients at a minimum. Many patients with ESRD also have multiple comorbid diseases, and patient complexity was also not specifically accounted for, which may confound the evaluation that these symptoms and themes are specific to the ESRD population. The analysis is also limited by the retrospective nature of the study reflecting documentation by the palliative care team, not allowing for longitudinal analysis, or outcomes data. The most significant limitation, however, is the inherent selection bias in this population, given the analyzed sample included only patients who had been referred to palliative care service without a review of other ESRD patients who may have had similar disease processes but without referral. At varying times during the study period, there were initiatives to increase palliative care consultation in the ESRD population that may have improved this bias; however, none of the initiatives was successful at completely standardizing a partnership in this population.

Although only addressing a small sample of the larger pediatric ESRD population, this study is an opening to assist both palliative care providers and nephrologists in assessing and developing tools for intervention in this challenging patient population. Future direction should focus on better understanding the larger population of pediatric ESRD patients and how to best address symptoms and topics found in this study population with recommendations or interventions that may lead to improved health-related QOL.

Footnotes

Acknowledgments

The authors thank the following individuals for their support and assistance with this article: Kenneth Pituch, David Kershaw, Deborah Gipson, Riley Frenak, Juyoung Samuel Inn, Cecilia Trudeau, and Maureen Giacomazza.

Author Disclosure Statement

No competing financial interests exist.

References

United States Renal Data System. 2015 USRDS Annual Data Report: Epidemiology of Kidney Disease in the United States. Chapter 8: Pediatric ESRD. National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD, 2015.

Neul

: Medical traumatic stress symptoms in pediatric patients on dialysis and their caregivers: A pilot study. Nephrol Nurs J, 2012; 39:483–488.

Varni

, Limbers

, Burwinkle

: Impaired health-related quality of life in children and adolescents with chronic conditions: A comparative analysis of 10 disease clusters and 33 disease categories/severities utilizing the PedsQL 4.0 Generic Core Scales. Health Qual Life Outcomes, 2007; 5:43.

Goldstein

, Graham

, Burwinkle

, et al.: Health-related quality of life in pediatric patients with ESRD. Pediatr Nephrol, 2006; 21:846–850.

Tjaden

, Grootenhuis

, Noordzij

, Groothoff

: Health-related quality of life in patients with pediatric onset of end-stage renal disease: State of the art and recommendations for clinical practice. Pediatr Nephrol, 2016; 31:1579–1591.

Musso

, Trigka

, Dousdampanis

, Jauregui

: Therapeutic alternatives and palliative care for advanced renal disease in the very elderly: A review of the literature. Int Urol Nephrol, 2015; 47:647–654.

Fowler

, Freiberger

, Moonan

: Palliative and end-of-life care in pediatric solid organ transplantation. Pediatr Transplant, 2015; 19:11–17.

Moss

: Revised dialysis clinical practice guideline promotes more informed decision-making. Clin J Am Soc Nephrol, 2010; 5:2380–2383.

National Guideline Clearinghouse (NGC). Guideline summary: Guideline recommendations and their rationales for the treatment of adult patients. In: NGC: Shared Decision-Making in the Appropriate Initiation of and Withdrawal from Dialysis, 2nd ed. Rockville, MD: Agency for Healthcare Research and Quality, 2010. Available at: www.guideline.gov (Last accessed November 24, 2016).

10.

Dionne

, d'Agincourt-Canning

: Sustaining life or prolonging dying? Appropriate choice of conservative care for children in end-stage renal disease: An ethical framework. Pediatr Nephrol Germany, 2015; 30:1761–1769.

11.

Kane

, Vinen

, Murtagh

: Palliative care for advanced renal disease: A summary of the evidence and future direction. Palliat Med, 2013; 27:817–821.

12.

Kwok

, Yuen

S-K

, Yong

, Tse

: The symptoms prevalence, medical interventions, and health care service needs for patients with end-stage renal disease in a renal palliative care program. Am J Hosp Palliat Care, 2016; 33:952–958.

13.

Feely

, Swetz

, Zavaleta

, et al.: Reengineering dialysis: The role of palliative medicine. J Palliat Med, 2016; 19:652–655.

14.

Leong Marc-Aurele

, Nelesen

: A five-year review of referrals for perinatal palliative care. J Palliat Med, 2013; 16:1232–1236.

15.

Moody

, Siegel

, Scharbach

, et al.: Pediatric palliative care. Prim Care, 2011; 38:327–361, ix.

16.

Weaver

, Heinze

, Kelly

, et al.: Palliative care as a standard of care in pediatric oncology. Pediatr Blood Cancer, 2015; 62(Suppl 5):S829–S833.