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Outpatient palliative Care for patients with Cystic Fibrosis and Sickle Cell

Outpatient palliative Care for patients with Cystic Fibrosis and Sickle Cell






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  • 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 Outpatient palliative Care for patients with Cystic Fibrosis and Sickle Cell Presentation Transcript

  • Toluwalase Ajayi MD
  • Objective  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 service.
  • Palliative Care Services    Improve quality of life Multidisciplinary team Support services to families
  • Palliative Care Services    Experts at eliciting patient & family goals of care Initiating advance care planning Experts at symptom management
  • Cystic Fibrosis
  • Cystic Fibrosis     Most common multiorgan genetic disease in the Caucasian population Devastating chronic lung disease Medical advances Low quality of life Tuchman et al. 2010
  • Cystic Fibrosis    Prevalent symptoms: dyspnea, fatigue, anorexia, anxiety, pain and cough Little to no palliative care till end stage Palliative care needs to be instituted early Dellon EP al. 2010 Urquhart DS et al. 2013
  • Cystic Fibrosis    Patients who receive palliative care derive clear benefit Rapid alleviation of symptoms Aggressive use medications Robinson W. 2000 Yankaskas JR et al. 2004 Stenekes SJ et al. 2009
  • Cystic Fibrosis    Transplants and HRQL Long-term survival Life after transplant Oreans JB et al 2009
  • Cystic Fibrosis    Deprived opportunities Best practices Increased opportunities for all patients
  • Sickle Cell Disease
  • Sickle Cell Disease     Common life-limiting genetic disease Increased life span Frequent hospitalizations, soci al isolations Low quality of life Mehran et al 2012 Dale et al 2009 Strickland et al 2001 Musaliam et al 2008
  • Sickle Cell Disease     Unpredictable episodes of pain Begins in early childhood Rigorous and time consuming treatments Disruption of quality of life McCish et al 2005 Haines et al 2013 Quinn et al 2008 Stegenga e t al 2004
  • Sickle Cell Disease     Psychosocial Symptoms Early life events Current stressful life events Increased risk Giuseppina et al 2008 American Psychiatric Association et al 2000
  • Sickle Cell Disease   Repeated ER visits Prolonged hospitalization stays Myvik et al 2013
  • Sickle Cell Disease     Adversarial relationship between pt and medical team Under treatment of pain Mistrust/ Marginalization Decreased societal function McClain et al 2007 Ross-Lee et al 1994 Labbe et al. 20006 Laurence B et al 2006
  • Big Problems/Big Solutions
  • Possible Solutions     Overarching themes Expanding the medical view Decreased ED utilization Attention to psychosocial dilemma Brown RT et al. 2006
  • Possible Solutions     Core ideals Patient and family focused Utilizing many disciplines Meeting them where they are Anghelescu DL et al. 2006
  • Possible Solutions     Shorten hospitalization Goals of care Complex symptoms Improved care
  • Possible Solutions   Strengthen inpatient presence Build trust
  • Barriers to Solutions    Great benefit Limited resources Importance of full staff
  • Conclusion
  • Work Cited        Tuchman, LK, Schwartz, LA et al. Cystic fibrosis and transition to adult medical care. Pediatrics 2010; 125;566. Dellon EP, Shores MD, Nelson KI, Wolfe J, et al. Family caregiver perspectives on symptoms and treatments for patients dying from complications of cystic fibrosis. J Pain Symptom Manage 2010; 40 (6):829-37. Urquhart DS, Thia LP, Francis J, et al. Deaths in children with cystic fibrosis: 10-year analysis from two London specialist centres. Arch Dis Child 2013; 98:123-127. Robinson W. Palliative care in cystic fibrosis. J. Palliative Med. 2000 Summer; 3(2) 187-92 Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest 2004;125(1Suppl):1S–39S. Stenekes SJ, Hughes A, Grégoire MC, Frager G, Robinson WM, McGrath PJ. Frequency and self-management of pain, dyspnea, and cough in cystic fibrosis. J Pain Symptom Manage 2009;38(6):837–48. Bradley JM, BLume SW et al. Quality of Life and Healthcare utilization in cystic fibrosis: a multicenter study. Eur Respir J. 2013 Mar;41(3): 571-7
  • Work Cited          Mehran Karimi, Sezaneh Haghpanah, Alit T. Taher, Maria Domenica Cappellini. "Beta Thalassemia: New Theraputic Modalities, Genetics, Complications, and Quality of LIfe." Anemia (2012): 1. Juanita Conkin Dale, Cindy J. Cochran, Lonnie Roy, Ethel Jerigan, Geroge R. Buchanan. "Healthrelated Quality of LIfe in Children and Adolescents with Sickle Cell Disease." Journal of Pediatric Health Care 25.4 (2009): 208-15. Ora Lea Strickland, Gerogia Jackson, Maggie Gilead, Deborah B. McGuire, Shirely Quarles. "Use of Foucs Groups for Pain and Quality of LIfe Asessment in Adults with Sickle Cell Disease." Journal of National Black Nurses Association 12.2 (2001): 36-43. Khaled Musallam, Maria D. Cappellini, Ali Taher. "Challenges Associated with Prolonged Survival of Patients with Thalassemia: Transitioning from Childhood to Adulthood." Pediatrics 121.5 (2008). Donna K McClish, Lynne T Penberthy, Viktor E Bovbjerg, John D Roberts, Imoigele P Aisiku, James L Levenson, Susan D Roseff, Wally R Smith. "Health related quality of life in sickle cell patients: The PiSCES project." Health and Quality of LIfe Outcomes 3.50 (2005). Dru Haines, Marie Martin, Susan Carson, Olivia Oliveros, Sage Greet et al. "Pain in thalassaeimia: The effects of age on pain frequency and severity." British Journal of Haematology 160 (2013): 680687. Miller ST, Sleeper LA, Pegelow CH, Enos LE, Wang WC, Weiner SJ, et al. "Predictions of adverse outcomes in children with sickle cell disease." New England Journal of Medicine 342.2 (2000): 83-9. Quinn CT, Lee NG, Shull EP, Ahmad N, Rogers ZR, Buchanan GR. "Predictions of adverse outcomes in children with sickle cell anemia: A study of the Dallas Newborn Cohort." Blood 111.2 (2008): 544-8. Kristin A. Stegenga, Peggy Ward-Smith, Pamela S. Hinds, Julie A. Routhieaux, Gerald M. Woods. "Quality of Life Among Children WIth Sickle Cell Disease Receiving Chronic Thransfuison Therapy." Association of Pediatric Oncology Nurses 21.4 (2004): 207-213.
  • Work Cited       Giuseppina Messina, Elisa Colombo, Elena Cassinerio, Francesca Ferri, Rita Curti et al. "Psychosocial aspects and psychiatric disorders in young adult with thalassemia major." Intern Merg Med 3 (2008): 33943. American Psychiatric Association. Diagnostic and Statistical manual of Mental Disorders 4th edition . Washington : American Psychiatric Association , 2000. Myvik MP, Burks lM, Hoffman RG, Dassgupta M, Panepinto JA. "Mental health disorders influence admission rates for pain in children with sickle cell disease." Pediatric Blood and Cancer 60.7 (2013): 1211-4. Ross-Lee B, Kiss LE, Weiser MA. "Should health care reform be "colorblind"? Addressing the barriers to improving minority health." Journal of American Osteopath Association 94 (1994): 664-671. Labbe E, Herbert D, Haynes J. "Physicians’ attitude and practices in sickle cell disease pain management ." Journal of Palliative Care 22.1 (2006): 64. Laurence B, George D, Woods D. "Association between elevated depressive symptoms and clinical disease severity in African- American adults with sickle cell disease." Journal National Medical Association 98 (2006): 365-369.
  • Work Cited Orens JB, Garrity ER. General overview of lung transplantation and review of organ allocation. Proc Am Thorac Soc. 2009;6(1):13–19  Santana MJ, Feeny D, Ghosh S, Lien DC. Patientreported outcome 2 years after lung transplantation: does the underlying diagnosis matter? Patient Relat Outcome Meas. 2012;3:79-84. doi: 10.2147/PROM.S32399. Epub 2012 Nov 19  Brown RT, Connelly M, Rittle C, Clouse B. "A longitudinal exam- ination predicting emergency room use in children with sickle cell disease and their caregivers." Journal of Pediatric Psychology 31 (2006): 163-173  Anghelescu DL, Oakes L, Hinds PS. "Palliative care and pediatrics." Anesthesiology Clin N Am 24.1 (2006): 145161 