The overall objective of this talk is to show how palliative care can be of help to patients with sickle cell and cystic fibrosis. It is our hope that by continuing to build a strong inpatient team and increase our resources we can extend our services to a full outpatient service. Overview of Palliative Care ServicesOverview of Sickle Cell DiseaseOverview of Cystic FibrosisThe needA possible solution
The primary mission of palliative care is to improve quality of life for patients and families. This is done using a multidisciplinary team often including physicians, nurse practitioners, nurses, social workers and spiritual counselors who seeks to address the needs of patients and families on corporeal, emotional and spiritual levels. Teams assist with identifying and offering support services to help the patient and family cope as they navigate illness
The unique multidisciplinary functionality of these teams allow them to be expert at eliciting patient & family goals of care, initiating advance care planning and providing continuity of care.Quality of life has been demonstrated to be further improved through expert management of pain and other physical symptoms.
What areas of Cystic Fibrosis care, do you think that palliative care can positively impact?
Cystic Fibrosis is the most common multi-organ genetic disease in the Caucasian population and leads to devastating chronic lung disease, recurrent infection, bronchiectasis and malnutrition due to pancreatic insufficiency. Advances in therapy for CF have dramatically increased the average life span, so that current birth cohorts are expected to live into their 50s. There has not, however, been concurrent, significant progress in improving quality of life. Achieving meaningful gains in quality of life for patients with CF has been shown to be important not only for providers but also for these patients and their families.
Literature demonstrates that, across the spectrum of disease, the most prevalent symptoms from which patients with cystic fibrosis suffer are dyspnea, fatigue, anorexia, anxiety, pain and cough. Despite this symptom burden, little attention is paid to methods of palliative care for patients with cystic fibrosis in the medical literature unless it relates to end-of-life or end-stage disease; in fact, even in these circumstances, palliative care referrals are woefully limited. A recent study examining the end-of-life circumstances of children with CF showed that, palliative care should be instituted well in advance, with ongoing attention to quality of life management and EOL planning.
The patients who do receive palliative care derive clear benefit. Unfortunately, the data is presently limited to the terminal phase of disease where the primary need is medical management of symptoms, such as dyspnea, nausea, pain, anxiety, and confusion. Palliative care teams are able to rapidly alleviate these symptoms using aggressive pharmacotherapeutics and thus improve the patient’s quality of life.
What about transplant? Among patients and families, the goal of treatment is to reach lung transplant.We as health care providers know that there is still significant mortality after transplant. Yet, transplantation has the potential to produce profound effects on survival and health-related quality of life (HRQL). Traditionally, the assessment of outcomes in lung transplantation has focused on patient survival. Lung transplantation has lower long-term survival compared with many other solid-organ transplants.1 Graft failure, chronic rejection, or broncholitisobliterans syndrome (BOS) and infections.This study looked at patient’s perspectives on their health related quality of life after transplant as part of the assessment. The authors found that cystic fibrosis patients, single-lung transplants, and older patients showed significantly lower HRQL. After lung transplantation, emotional health and pain continue to be negatively affected
Despite this demonstrated success, initiating palliative care involvement only after disease-directed treatment options have been exhausted and the patient is dying, deprives the patient and her family of the opportunity to have improved quality of life throughout the disease course. Best practice would be to include patients who are still receiving standard cystic fibrosis care – who are actively pursuing potentially curative treatment; this would ensure access to palliative care for all patients who can benefit.
Do you think about palliative care and patient’s with sickle cell?What areas of sickle cell care, do you think that palliative care can positively impact?
Sickle cell disease is among the most common life-limiting genetic disease in ethnic minorities characterized by diverse co-morbidities related to nearly every organ system.Similarly Medical advancement in this disease process has dramatically increased the life span for patients so that the vast majorities are living well into adulthood.However, the intensive daily treatments, frequent hospitalizations, social isolation, and long-term endurance of troublesome symptoms of chronic illness that have been necessary consequences of these advances can detract significantly from quality of life.
One of the most profound and devastating factors affecting patients’ quality of life is the unpredictable episodes of pain. A hallmark symptom by which the sickle cell disease is defined, pain is a common experience in children beginning as early as 4-6 months of age, with dactylitis as an early prognostic indicator for increased complications.Yet despite the advances leading to life prolongation in these diseases, pain is still undertreated, and in some cases not fully understood. Hemoglobin SS is the most severe form of SCD and carries an increased risk for stroke and severe pain crisis. Although the initial treatment for stroke and pain is an exchange transfusion, the treatment plan is rigorous and time consuming, but leads to increased survival.However, simply surviving is not adequate; the quality of survival has emerged as an essential focus of comprehensive health care. The presence of physical symptoms, combined with disease management routines (transfusions, repeated clinic visits and multiple hospitalizations) disrupts the daily life of these children and significantly diminishes their quality of life.
Further, a large body of research demonstrates that this patient population also suffers from multiple disease related psychosocial symptoms including depression, poor coping skills and others that further diminish their quality of life.Those patients who do not currently suffer from these symptoms are at increased risk for development and need to be monitored. In the biopsychosocial model describing the pathogenesis of psychiatric conditions, the development of psychiatric disorders is related to the impact of the following sequence of events: genetic predisposition, early life events, and current stressful life events.Sickle cell disease affect the latter two factors. This means that the chances of patients with these hemoglobinopathies developing serious psychiatric conditions or mood disorders, beyond the anxiety, guilt, shame, or depression that may accompany any normal stressful situation, are increased.
Patients with severe hemoglobinopathies are seen repeatedly in emergency rooms and the inpatient setting for long periods of time, seeking relief from their symptoms. Furthermore, it has been shown that patients with hemoglobinopathies and a comorbid history of mental health diagnosis experience even longer hospitalization days.
The relationship between patients with SCD and health care providers has been noted to be adversarial.Indeed, attitudes of the caregiver toward patients with SCD reportedly contribute to the under treatment of sickle cell–related pain.This creates mistrust between patients and health care providers and contributes to patients with SCD experiencing emotional marginalization, amplification in depressive moods, and expressed helplessness and hopelessness.Deficiencies in school readiness have been noted in children with SCD that do not seem to be caused by the underlying medical pathology. These factors can culminate in adolescents/Young adults as social anxiety and manifest as anger and low expectations; feelings of disfranchisement will result in limited productivity in the adult workforce with resultant dwindling expectations from medical and lay communities.
Within these two disease populations, we have patients that push the traditional boundaries palliative care, yet are of clear need.How can palliative care be useful?
There are overarching themes that apply to both populations, but let us first address the marginalization of patients with sickle cell disease.Using palliative care philosophies in the management of patients with SCD could change the public and medical view of this population’s illness and likely have a tremendous impact on the health care system as well as patients and their families. Indeed, it is predicted that utilization of emergency department services alone would plummet if caregivers fostered better psychosocial adjustment and acceptance of this population’s dilemma.
Overall, the core ideals of integrated palliative care are open communication, timely access to care, intensive symptom management, flexibility in the implementation of interventions, ethical decision making, and attention to the quality of life of the patient, the family, parents, siblings and significant others. The interventions are patient focused and family oriented and can be modeled to fit “any place, any time,” in the hospital or the home, with home-care and other support.Palliative Care is able to accomplish this patient centered care by using a multidisciplinary team often including physicians, nurse practitioners, nurses, social workers and counselors who seek to address the needs of patients and families on physical, and emotional levels. In short, our goal is to meet the patient where they are.
Often time, when we meet the patients, we are meeting them in the hospital, towards the end of their health trajectory. Improving quality of life can be done with a hospital consult, and can shorten hospitalization stays. Goals of care conversations happen, and complex, challenging symptoms are addressed. But we can do better for patients like these. Patients with chronic illness, who face tremendous treatment burden, a large psychosocial component, and increasing symptomatology as their disease progresses.
How can this be done? Part of the solution is to strengthen the inpatient presence by seeing these patients earlier in the course of their hospitalization, to strengthen the trust and build a connection. The second part is the need for a strong outpatient component. Out patient palliative care is a natural extension of “whole patient care” and helps us reach the goal of meeting the patients where they are earlier the course of their disease.To fully utilize the components of our team and help manage symptoms well, we need work with the medical homes of these patients to establish that initial connection and trust. Members of our teams can work with the primary team to help patients manage their symptoms on an outpatient basis. This helps provide more consistent involvement across disease trajectory whether there is a cure or not.
Outpatient palliative care is a great extension of the care that is provided on the inpatient basis and will be a great benefit for patient with cystic fibrosis and SCD. One of our colleagues did an assessment of the different palliative care models that exist in San Diego. Despite the different models one consistent barriers is limited resources and over extension of restricted services. It is important to have enough team members to adequately staff both the inpatient and outpatient component. In doing so, palliative care can better address the complex, and multidimensional symptoms seen in this patient population.
In patients with Sickle Cell Disease and Cystic fibrosis, we have a patient population that paradoxically breaks the mold of what people think of, when regarding palliative care patients, yet, exemplify the very patients that can benefit from our services and care. We are dedicated to working with teams to help improve the quality of life of these patients by providing patient centered care that focuses on expert symptom management, eliciting patient & family goals of care, initiating advance care planning and providing continuity of care. Our resources may be limited, but by continuing to increase our referral basis, we will extend our resources.
Outpatient palliative Care for patients with Cystic Fibrosis and Sickle Cell
Toluwalase Ajayi MD
Palliative care can be of help to patients
with sickle cell and cystic fibrosis.
By continuing to build a strong inpatient
team and increase resources we can
extend our services to a full outpatient
Palliative Care Services
Improve quality of
Support services to
Palliative Care Services
Experts at eliciting
patient & family
goals of care
Experts at symptom
Most common multiorgan genetic
disease in the
Low quality of life
Tuchman et al. 2010
anxiety, pain and
Little to no palliative
care till end stage
Palliative care needs
to be instituted early
Dellon EP al. 2010
Urquhart DS et al. 2013
Patients who receive
palliative care derive
Rapid alleviation of
Robinson W. 2000
Yankaskas JR et al. 2004
Stenekes SJ et al. 2009
Life after transplant
Oreans JB et al 2009
opportunities for all
Sickle Cell Disease
Increased life span
Low quality of life
Mehran et al 2012
Dale et al 2009
Strickland et al 2001
Musaliam et al 2008
Sickle Cell Disease
episodes of pain
Begins in early
Rigorous and time
Disruption of quality
McCish et al 2005
Haines et al 2013
Quinn et al 2008
Stegenga e t al 2004
Sickle Cell Disease
Early life events
Current stressful life
Giuseppina et al 2008
Association et al 2000
Sickle Cell Disease
Repeated ER visits
Myvik et al 2013
Sickle Cell Disease
pt and medical team
Under treatment of
McClain et al 2007
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Labbe et al. 20006
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