Goals Of Pediatric Palliative CareCurrent PerceptionsPatient Population Expanding our Patient BaseConclusion
What are the goals of Pediatric Palliative Care?Palliative care for children aims to improve the quality of life for the patient and the family. This is done through expert management of pain and other physical symptoms such as shortness of breath, nausea, vomiting and anxiety.Its is also done through using a multidisciplinary team to address the emotional, spiritual and other support services to help the patient and family cope as they navigate through the illness at hand and they determine what their goals of care are.
When is pediatric palliative care appropriate for the child?Palliative care ideally beings when the family first receive the news that their child has a life limiting illness. It can and should be provided along with efforts to cure or prolong life as long as it is appropriate and desired by the patient and family.The American Academy of Pediatrics supports an integrated model of palliative care “in which the components of palliative care are offered at diagnosis and continued throughout the course of illness, whether the outcome ends in cure or death”. Waiting to initiate palliative care for children who have exhausted every curative treatment and are already dying means that these children missed out on the opportunity to have improved quality of life while struggling with their disease. Including children who have life threatening illness or condition but are still receiving curative treatment ensures that all children who can benefit from palliative are have access to it. This isn’t news for pediatricians who practice palliative medicine, what about the general pediatricians in the community?
In 2009 a study was designed to investigate how pediatricians define palliative care, pediatricians were divided in their definitions. 41.9% defined palliative care as hospice care, 26.2% did not know how to define it and 31.9% had alternative definitions. From that population, 28.7% saw it as the type of care one gives a patient when there is no cure. 29.4% saw it as supportive care and 28.7% saw it as Disease and symptom management. And these results were taken from areas in which the majority ( 61.5%) had pediatric and or hospice services in their area. So, despite the many published articles stating what pediatric palliative care is, there still doesn’t seem to be a consensus on the definition amongst the general pediatrician population
This study also looked at 13 diseases with varying prognosis and asked the pediatricians to select the disease they thought warranted palliative care referrals. For ever needing a referral, cancer ranked highest at 96.9%, followed by cystic fibrosis (94.3%) and metabolic/storage diseases (94.1%). These are diseases that benefit from palliative care at the time of diagnosis…
Yet, In regards to the timing of referrals; early referrals were extremely rare. When you looked at all 13 diseases, the mean percentage for early referrals was 14.3%; 36.1% the middle course of the disease and 35.8 when curative therapy was no longer the goal, at the end of life.So, we must ask ourselves, what are the barriers to more timely referrals?
This study showed that barriers to referrals, especially referrals during the early course of the disease on the part of the pediatricians can be explained by a lack of understanding what pediatric palliative care means and what the goals of this specialty are. Another study cited family perception as a major reason why pediatricians don’t refer until the end of life. This study’s primary aim was to determine the barriers to pediatric palliative care as perceived by pediatricians. Barriers were related to the following five domains: access, reimbursement, family perceptions, personal or professional perceptions, and the health system.This paper showed that the primary reason why they didn’t refer was that families viewed such a referral as a sign that the medical team was giving up.So, what is it about our service that gives this perception? Do the population of kids we see as palliative care providers or the services we provide align with these perceptions?What are the demographics of the children that pediatric palliative care team see?Factors associated with perceived barriers to pediatric palliative care: a survey of pediatricians in Florida and California Palliat Med 2012; 26:3 268-274
In 2011 A multicenter study was published that looked at the demographics of patients that were actually referred to pediatric palliative care Teams as well as the type of services we provide.There data was collected between Jan 1 2008 and March 31st 2008. As you can see; This study showed that for the children we were consulted on; the overall main goals of were symptom management, facilitating communication, decision-making and assisting with logistics or coordination of care. Least common were for do-not-resuscitate (DNR) orders and peri-death recommendations (9.1%)
So as you have seen, there is still a large disconnect between the reality of what our scope of practice is and encompasses, and what our professional colleagues and patient’s families perceive. What can we do to change this perception?How can we change,or rather build and improve upon our current practice so the general and professional population perception meets what we, the palliative care providers have stated and defined pediatric palliative care?
I propose we do this by broadening our patient population base. The study I cited earlier showed that the majority of patient that we see, have than one diagnosis. The three most prevalent were children with genetic or congenital disorders ( 40.8%). Children with Neuromuscular disease and cancers. On the other end of the spectrum were children with respiratory , GI, other diagnosis.If we were to include more patients with respiratory, GI and other diagnosis, we might be able to better close the gap between perception and reality.
There are two pediatric populations I feel exemplify this concept. Not only do patients with Cystic Fibrosis and Sickle Cell have complex with disease process that involve nearly the entire body; They are also examples of pediatric disease that bridge the pediatric and adult population. These patients are often in the greatest need of palliative care, yet rarely get referred to us, and if they do, it isn’t usually until they are near the end of life.
I want to start first with patients with Cystic Fibrosis.I remember when I was in medical school and we were taught that the median life expectancy for kids with cystic fibrosis was in the mid to early to mid twenties.Now, it is projected that children with CF born in the 1990s will live into their 40s and that current birth cohorts will live into their 50sYet maintaining quality of life and improving life-span duration are continuing challenges for patients, their families and CF care providers. We know that most of this population spend a significant amount of time being hospitalized particularly during adolescents and young adult when lung function by be declining and treatment burden and complications of multisystem organ failure are increasing. We can help with that.
One of the few studies I could find investigating how factors relating to quality of life affects disease outcome, showed that a negative perception of one’s quality of life correlated with poor performance on pulmonary function tests.The adolescents in this study reported moderate to strong relationships between their pulmonary function and self reports of general health, pain, and limitations in activities at school and with friends because of physical problems.
During our fellowship, my fellow pediatric colleague and I did a chart review on three patients with cystic fibrosis who were referred to hospice to receive home based palliative care. These patients were referred to us because it was felt due either to their pulmonary function tests, or because of the increased frequency of hospitalizations, they were nearing the end of life.During their time with us, they were maintained on the same therapeutic regimen as they had been prior to the referral. They continued to follow with their pulmonologists, and our team saw the patient’s at home as well as in the hospital. We co-managed patient’s symptoms, provided counseling, home nursing. We found that when palliative care was provided in conjunction with the standard medical treatment the number of hospitalizations for these patients were decreased dramatically. Patient 1-9 months on hospice before graduated Patient 2-10 months on hospice before being graduatedPatient 3- 6 months while data was collected, she is still on hospice and is actually worse
In regards to their pulmonary function tests, we found that with the addition of palliative care to their current care, these patient’s had general improvements of their FEV1. The data were collected with these specific time marks plus or minus 25 days from date of referral. You see that patient number two did have an acute decrease in her PFT at the nine month mark. What this chart doesn’t show simply due the time cut offs is that 20 days later, her PFT increased back up to 67%, which is still higher than it was at time of referral while she was still enrolled in hospice. The reasons why there is no data for 12 months for patient’s one and two is because they were discharged from hospice due to extended prognosis and improvement.It is because of patients like these that pulmonary team at Rady’s Children Hospital have now started a home based palliative care program for the cystic fibrosis patients who do not meet the admission criteria for hospice. Especially looking at Patient number two. It is hard to argue that with PFTs in the mid 55s at the time of referral, she would meet eligibility hospice, but truly needed and benefited from palliative care interventions.
An adult cystic fibrosis center in the UK developed an integrated model of palliative care where by most care is delivered by the CF team using palliative guidelines and pathways with additional support from the palliative care team.We can and should start this process in our pediatric patients and publish our results. The chart review that my colleague did had a small number, but shows large promise when done prospectively and on a larger scale. Adding this type of data and clinical research to the growing literature on pediatric palliative care adds power behind our words and broaden our spectrum of care.An integrated model of provision of palliative care to patients with cystic fibrosis.Bourke SJ, Doe SJ, Gascoigne AD, Heslop K, Fields M, Reynolds D, Mannix K.
What about children with Sickle Cell disease?The life span for children with SCD has increased dramatically in the past 50 years. This is in large part due to penicillin prophylaxis, pneumococcal vaccination, broader spectrum antibiotics have been used to prevent and treat infections. Many children at high risk of stroke can be identified by using transcrainialdoppler screening and managed with regularly blood transfusions. We are aware that because of the chronic hemolysis that is the nature of SCD, we screen for gal stones. Together with newborn screening and these interventions and many more; The median life expectancy for SCD has increased from Less than 15 years in the 1970s to greater than 40 years in the 1990s. Pain is the hallmark symptom by which the sickle cell disease is defined. It is a common experience in children beginning as early as 4-6 months; dactylitis is an early prognostic indicator for increased risk of complications in children. and though some of these patients can be managed with a pain team consult alone; there exists a large subset of patients who are seen repeatedly in the patient service with uncontrolled pain. Uncontrolled pain has a universal and profound negative effect on the quality of life in all ages and with all types of painThe Greatest harm of all may well be decreased quality of life.Failure to address the pain early can have lifelong implications for their health, generating a vicious cycle of fear, avoidance, pain and poor coping strategies.Bope and Kellerman: Conn's Current Therapy 2012, 1st edMiller SC, Sleeper LA, Pegelow CH, et al. Predictions of adverse outcomes in children with sickle cell disease. N Engl J Med. 2000; 342: 83-89
Not only that lead to negative consequences such as missed days of school, other life activities or fear/mistrust of health care providers, but it can also can lead to amplified responses to subsequent pain experiences and sensitivity to pain later in life.Some become profoundly disabled from chronic pain and require substantial social and healthcare resources that are not consistently available. Particularly in this patient population.McClain B, Zain ZN. Pediatric Palliative care: a novel approach to children with sickle cell disease. Pediatrics. 2007; 119:612-614Ely B, Tasa JCI. Pain in children-early pain experiences can impact future health. The Pain Community News. 2008; 8:5. www.painfoundation.org/Publications/PCN08winter.pdf
The integrative approach of palliative care emphasizes pain management and attention to psychological, social, and spiritual issues.This needs to be introduced to patients with SCD early in childhood to prevent the negative cascade of events previously mentioned. It has been proposed that doing so could improve the public and medical views of this patient populationAs well as tremendously impact the health care system, patients and their families.We in the palliative care field are in the great position of providing the empiric data to show this to be true. One of my goals as a pediatric palliative care physician is to improve access for these patients and provide the clinical data that shows we can and do improve the quality of life for these patients. Beider S. An ethical argument for integrated palliative care. Evid Based ComplementAlternat Med.2005;2:227– 231
Pediatric palliative care exists to improve the quality of life for the patient and the family. We do this through expert management of pain, shortness of breath, nausea, vomiting and other such symptoms. We utilize a multidisciplinary team to address the emotional, spiritual and other support services to help the patient and family cope as they navigate through the illness at hand as they determine their goals of care. Despite the many published works indicating that this is who we are and what we do; the majority of our professional colleagues patients still see us as an end of life service. I propose that by expanding our patient base to include these two large, high need population we can help change this perception. When we take care of these patients we need to publish the emperic data that shows how we improved their quality of life and thus their disease processes.
An integrated model of provision of palliative care to patients with cystic fibrosis.Bourke SJ, Doe SJ, Gascoigne AD, Heslop K, Fields M, Reynolds D, Mannix K.
1. ToluwalaseAjayi, MD
Palliative Care Fellow
San Diego Hospice and Institutive of Palliative Care
Expanding our patient base can broaden
the spectrum of pediatric palliative care
as perceived by our professional
3. Pediatric Palliative Care
4. Pediatric Palliative Care
INTEGRATED PALLIATIVE CARE
5. Current Perceptions
Lindsay A et al.
6. Current Perceptions
Lindsay A et al.
7. Current Perceptions
End of life
Lindsay A et al.
8. Current Perceptions
Family reluctance to accept hospice & 4.8 95.2
There is a perception by families that
hospice & palliative care indicates health
professionals are giving up.
Knapp C. et al.
9. Patient Population
Feudtner C. et al.
10. Patient Population
11. Broadening The Spectrum
Genetics, NM, Cancer
Respiratory, GI, Other.
Feudtner C. et al.
13. Cystic Fibrosis
Cystic Fibrosis Foundation
Elborn, JS et al.
Tuchman et al.
14. Cystic Fibrosis
Powers. P et al.
15. Cystic Fibrosis
# of hospitalizations before and after hospice
16. Cystic Fibrosis
FEV1 at time of Referral and after
17. Cystic Fibrosis
Integrated model of Palliative Care.
Bourke S. et al.
18. Sickle Cell
Conn’s Current Therapy
Miller S. et al.
• Poor coping
19. Sickle Cell
Decreased quality of
McClain B. et al.
Ely B. et al.
20. Sickle Cell
Beider S. et al.
22. Work Cited
Thompson LA, Knapp C, et al. Pediatricians’ Perceptions of and
Preferred Timing for Pediatric Palliative Care. Pediatrics 2009; 123;
e777. DOI: 10.1542/peds.2008-2721.
Knapp C, Thompson LA. Factors associated with perceived barriers
to pediatric palliative care: a survey of pediatricians in Florida and
California Palliat Med 2012; 26:3 268-274.
Feudtner C, Kang T, et al. Pediatric Palliative Care Patients: A
Prospective Multicenter Cohort Study. Pediatrics 2011;127;1094.
Cystic Fibrosis Foundation. Cystic Fibrosis. Foundation Annual
Patient Registry Report. Bethesda, MD: Cystic Fibrosis Foundation;
Elborn JS, Shale DJ, Britton JR. Cystic Fibrosis: current survival
and population estimates to the year 2000 [published correction
appears in Thorax. 1992;47(2):139]. Thorax. 1991;46(12):881– 885.
Tuchman LK, Schwartz LA, et al. Cystic Fibrosis and Transition to
Adult Medical Care. Pediatrics 2010;125;566. DOI:
23. Work Cited
Powers PM, Grestle R, et al. Adolescents With Cystic Fibrosis:
Family Reports of Adolescent Health-Related Quality of Life and
Forced Expiratory Volume in One Second. Pediatrics 2001;107;e70.
Bourke SJ, Doe SJ, et al. An Integrated Model of Provision of
Palliative Care to Patients with Cystic Fibrosis. Palliat Med 2009
Bope and Kellerman: Conn's Current Therapy 2012, 1st ed.
Miller SC, Sleeper LA, Pegelow CH, et al. Predictions of adverse
outcomes in children with sickle cell disease. N Engl J Med. 2000;
McClain B, Zain ZN. Pediatric Palliative care: a novel approach to
children with sickle cell disease. Pediatrics. 2007; 119:612-614
Ely B, Tasa JCI. Pain in children-early pain experiences can impact
future health. The Pain Community News. 2008; 8:5.
Beider S. An ethical argument for integrated palliative care. Evid
Based ComplementAlternat Med.2005;2:227– 231