OUTPATIENT PALLIATIVE CARE WITH SICKLE CELL DISEASE AND CYSTIC

FIBROSIS

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 multi-

organ 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. 2004Stenekes 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, social isolations

Low quality of life

Mehran et al 2012Dale et al 2009Strickland et al 2001Musaliam 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 2005Haines et al 2013Quinn et al 2008Stegenga e t al 2004

Sickle Cell Disease Psychosocial

Symptoms Early life events Current stressful life

events Increased risk

Giuseppina et al 2008American 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 2007Ross-Lee et al 1994Labbe et al. 20006Laurence 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

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