Opioid Dependency: Challenges in Managing Cystic Fibrosis Patients with Addiction Angie Payne, MSN, RN, AGCNS-BC Dell Children’s Medical Center - Adult Cystic Fibrosis Program Austin, Texas

Angie Payne, MSN, RN, AGCNS-BC Presenter Disclosure Angie Payne, MSN, RN, AGCNS-BC There are no relationships to disclose related to this presentation.

Patient: 24-year old male with the following medical history: Cystic Fibrosis, genotype delta F508 and G551D, on Kalydeco Bronchiectasis Pancreatic insufficiency Major depressive disorder Generalized anxiety disorder Chronic back pain - on opioid therapy managed by pain specialist

Exam (Jan, 2016): VITAL SIGNS: Wt 66.7 kg, T 98, HR 96, RR 20, BP145/93, 02 sat 96% on 4 liters O2. GENERAL: He is alert and interactive. He appears to be in good spirits. Occasionally coughing. HEENT: Head is normocephalic, atraumatic. Ears normal set. TMs clear and mobile. Nares show no discharge. Oropharynx moist. NECK: Supple, no lymphadenopathy or stridor. CHEST: Shows relatively good air entry bilaterally. There are decreased breath sounds on the right compared to the left. No wheezes heard. Occasional crackles heard bilaterally. CARDIOVASCULAR: Regular rate and rhythm, normal S1, S2 without murmurs, rubs or gallops. ABDOMEN: Soft, bowel sounds normoactive without hepatosplenomegaly, Gtube appears normal. EXTREMITIES: Well perfused x 4, no cyanosis or edema, mild clubbing on digits. SKIN: Shows no rash, eczema or hives. LABORATORY STUDIES: Sputum cultures that have grown small colony variant Staph aureus as well as several strains of Pseudomonas aeruginosa

Port CF FEV1 trends: Hospital admission Rehab admission Most recent admission was 10/4/17 – FEV1 29% predicted….. Improved to 35% predicted after one week

Case History: 2013 Transferred care to our CF center Baseline FEV1 80% predicted, hospitalized once per year 2014 Mom dies – Dementia and Parkinson’s disease 2014 – 2015 Baseline FEV1 60% predicted, hospitalized on average 5 times per year. Shows significant improvement after treatment. Quickly declines after discharge. Requests IV Dilaudid, Fentanyl and Ativan for chest and back pain each admission. Must wean off prior to d/c.

Case History: 2016 Admitted to hospital 7 times for a total of 157 days FEV1 drops to 32% predicted Father dies from metastatic kidney cancer while patient is admitted for treatment of a CF exacerbation. Suicide attempt at home, overdoses on narcotic pain meds

Social History: 2016 Admits to stopping his CF treatments to make himself sick in order to gain admission to hospital to have access to narcotic pain meds Feels RNs and staff are his “family” and they fill a parental void At urging of CF team and hospital staff, admits to inpatient rehab facility Successfully weans off all narcotic pain meds

Current status: Sober for last year, working part-time Leader in his outpatient therapy group Latest FEV1, 41% predicted at his baseline Two hospitalizations needed for IV antibiotics in 2017 Improved weight gain – 69.5 kg in 6/2016 to 88 kg 10/2017 Good adherence to CF therapies Just completing 2 weeks of antibiotic therapy. Still using 4 L supplemental oxygen. Spirits good. Visitor from support group

For Discussion: Is chronic pain an increasing problem in adult CF patients/patients with chronic disease? Is opioid addiction an increasing problem in adult CF patients/patients with chronic disease? Who should be managing chronic pain and opioid usage in CF patients as outpatients and when admitted to the hospital? What is the role for Psychiatry, Palliative Care and Pain Management?

For Discussion: If patients aren’t on pain meds, what should we do for them while inpatient when they complain of severe pain? Do other hospitals have pain management protocols for CF patients? What is the most effective way to confront a patient with concerns for addiction?