Cystic Fibrosis Care Team: Beyond Treatment Strategies

Beyond Cystic Fibrosis: Beyond Cystic Fibrosis: Broadening Your Health Care Team MEREDITH WILTSIE, MN, ANP, FNP MEREDITH WILTSIE, MN, ANP, FNP NURSE PRACTITIONER, ADULT CYSTIC FIBROSIS PROGRAM NURSE PRACTITIONER, ADULT CYSTIC FIBROSIS PROGRAM CYSTIC FIBROSIS EDUCATION DAY CYSTIC FIBROSIS EDUCATION DAY MARCH 11TH TH, 2017 , 2017 MARCH 11

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If you are a CF Center patient, why do you need a Primary Care Physician?

You are more than CF Cystic Fibrosis Living Foundation

Patients with CF are living longer Cystic Fibrosis Foundation Patient Registry, Annual Data Report. 2015

Patients with CF are living longer Evolving therapies Research breakthroughs Effectiveness of the multi- disciplinary care model Hard work by CF patients Smyth AR et al, J. Cyst. Fibros. 2014 Farrell PM et al, J Pediatr. 2008 Cohen-Cymberknoh M et al, Am J Resp Crit Car Med. 2011

Patients with CF are living longer: Evolving Therapies Elborn JS, Euro Resp Review. 2013

Patients with CF are living longer: Optimal Health Team CF health needs CF Center Age related health needs PCP CF aging needs CF Center + PCP (in collaboration)

CF Center vs Primary Care THE DIFFERENCES AND WHY YOU NEED BOTH

CF Center vs Primary Care: Specialized Centers Specialized Centers Large body of literature shows: Improved outcomes Improved survival Elborn JS et al. Thorax. 1991 Phelan P et al. Arch Dis Child. 1984 Littlewood JM et al. CF Congress Sydney. 1988 Walter S et al. Thorax. 1994

CF Center vs Primary Care: Specialized Centers Specialized Centers Advanced survival of children with chronic illness (CF, Sickle Cell Disease, Complex Congenital Heart Disease) Survey: Internist view on barriers to the Primary Care Provider: Felt it would be difficult as PCP could not handle complexity of disease Poor mental health support Lack of reimbursement for chronic illness care Lack of time Okumura MJ et al, Pediatrics. 2010

CF Center vs Primary Care ABPA Thyroid disease Hemoptysis Headaches Cirrhosis IGT Ear Infection STDs CFRDM Bronchiectasis Hypertension Back pain Airway Obstruction Anemia Fibromyalgia High cholesterol Cancer screenings Malabsorption Pseudomonas Broken Bone Pancreatic Insufficiency Immunizations Birth Control DIOS GERD Vision Screening Acne Increased Sputum

CF Center vs Primary Care: CF Center CF Center CF Foundation The mission of the Cystic Fibrosis Foundation is to cure cystic fibrosis and to provide all people with the disease the opportunity to lead full, productive lives by funding research and drug development, promoting individualized treatment and ensuring access to high-quality, specialized care. mission: Cystic Fibrosis Foundation Patient Registry, Annual Data Report. 2015

CF Center vs Primary Care: CF Center CF Center CF Center operates under standards of care and best practice guidelines: Multi disciplinary team focused on Cystic Fibrosis: Pulmonology specialists (MD, NP) CF dietician Social work Respiratory therapy Research team Network of specialists with CF training Endocrinology ENT, GI, Psychiatry, Liver Disease Elborn JS et al, Euro Resp Jour. 2016

CF Center vs Primary Care: CF Center CF Center What happens during a healthy CF visit? (Quarterly Checkups): Status check and optimization of care management of CF-related symptoms: Pulmonary Sinuses GI (Pancreas, GI tract) CF Health Maintenance Bone Density Blood Sugars Mental Health

CF Center vs Primary Care: CF Center CF Center ABPA Thyroid disease Hemoptysis Headaches Cirrhosis IGT Ear Infection I should contact the CF Center STDs CFRDM Bronchiectasis Hypertension Back pain Airway Obstruction Anemia Fibromyalgia High cholesterol Malabsorption Pseudomonas Broken Bone Pancreatic Insufficiency Immunizations Birth Control DIOS GERD Vision Screening Acne Increased Sputum

CF Center vs Primary Care: Primary Care : Primary Care Focus on overall health: Bird s Eye View Health history (including CF) Family history Changes over time Coordination of care (between specialists, including CF Center) Referrals

CF Center vs Primary Care: Primary Care : Primary Care Day to day health: Treat day-to-day problems, sick visits Manage blood pressure Muscle and joint pain/injuries Example 1: Fever, sore throat ---> Strep test Example 2: Travel abroad ---> specific immunizations

CF Center vs Primary Care: Primary Care : Primary Care Individualized risks and prevention strategies: Pharmacogenetics Genetic risks: screening and predispositions Example 1: Family history of breast cancer ---> discussion of genetic evaluation or earlier mammogram Lowton K et al, Brit Jour Gen Prac. 2006 Emery J et al, BMJ. 2001

CF Center vs Primary Care: Primary Care : Primary Care What happens during an annual Primary Care visit? Screenings: Blood pressure and cholesterol BMI, weight-related health risks Vision and hearing Sexual health/STDs/Reproductive health Cancer: pap/breast and testicular exams, colonoscopy, mammogram Drug and alcohol use Cognitive and memory screening, especially with aging Counseling: depression, mental health, LGBT, genetic Immunizations

CF Center vs Primary Care: Primary Care : Primary Care EXAMPLE: CDC 2017 Immunizations recommended by age CDC, Adult Immunization Schedule. 2017

CF Center vs Primary Care: Primary Care Primary Care ABPA Thyroid disease Hemoptysis Headaches Cirrhosis IGT Ear Infection STDs CFRDM Bronchiectasis Airway Obstruction I should contact my PCP Hypertension Back pain Anemia Fibromyalgia High cholesterol Cancer screenings Malabsorption Pseudomonas Broken Bone Pancreatic Insufficiency Immunizations Birth Control DIOS GERD Vision Screening Acne Increased Sputum

CF Center vs Primary Care: Together : Together Comprehensive health care includes: Living with CF: Living with CF: Pulmonary health (PFTs, sputum cultures) Pancreatic insufficiency Bowel health Sinus disease Bone health Nutrition/weight management Mental health Being 20, 30, 40, 50, 60 years old Being 20, 30, 40, 50, 60 years old . . Heart health: Blood pressure, cholesterol Immunizations Gynecology/Men s Health Exposure-related illnesses (travel, etc) Cancer screenings Nutrition/weight management Mental health

Increased Risks of Aging with CF AND HOW PRIMARY CARE CAN HELP

Increased risks of aging with CF Chronic kidney disease (CKD) Aminoglycoside-related kidney and hearing dysfunction Osteoporosis GI tract malignancies Cardiovascular diseases Sleep related diseases

Increased risks of aging with CF: Kidney Disease Kidney Disease Diabetes is a known risk factor for the development of kidney disease CFRDM is not the only risk factor for kidney disease in CF. Study of 11,912 CF patients from 2001-2008 found the prevalence of CKD doubled with every 10 years increase in age. 1 Critique of this study noted it only included patients on insulin, thus possibly missing the role of other potential causes of kidney disease in CF other than diabetes. 2 1- Quon BS et al, Am J Resp Crit Car Med. 2011 2- Schechter MS et al, Am J Resp Crit Car Med. 2011

Increased risks of aging with CF: Kidney Disease Kidney Disease Risk from Aminoglycosides (Tobramycin, Amikacin, Risk from Aminoglycosides (Tobramycin, Amikacin, etc etc) ) Known risks include potential for hearing/balance loss and kidney damage A study measuring kidney function in 80 CF outpatients showed a strong correlation between cumulative IV aminoglycoside use and diminishing renal function 1 A different study following 113 CF patients over 8.5 years found that well managed IV use of Aminoglycosides was not associated with long term diminished renal function 2 1- Al Aloul M et al, Pedia Pulm. 2005 2 - Florescu M et al, Hemodial Int. 2012

Increased risks of aging with CF: Osteoporosis Osteoporosis 1998 study of 70 CF patients showed CF patients have significantly decreased bone density compared to age matched controls. Predictors of decreased bone density included: BMI Cumulative prednisone dose Age at puberty Complications: fracture risk and severe kyphosis Aris RM et al, Ann Int Med. 1998

Increased risks of aging with CF: GI Tract Cancers GI Tract Cancers A large study in 28,511 CF patients from 1985 to 1992 found: Overall risk for most cancers in CF population is similar to that of non-CF patients However, found an increased risk of GI tract cancers Not enough evidence yet to know how early to screen in CF Neglia JP et al, NEJM. 1995. Billings JL et al, J Clin Gastroenterol. 2014

Increased risks of aging with CF: GI Tract Cancers GI Tract Cancers A CF Registry data study of 28,858 patients between 1990-1999: Found increased risk of GI tract cancers in both pre and post transplant CF patients The risk was more pronounced in transplanted patients.1 A smaller, case control study of 50 CF patients 2007-2012 showed: 10 fold increased risk of GI tract cancer in CF patients 2 1- Maisonneuve P et al, J Natl Cancer Inst. 2003 2- Gory I et al, Scand Jour Gastro. 2014

Increased risks of aging with CF The Role of Primary Care Chronic kidney disease (CKD) Aminoglycoside-related Osteoporosis GI tract cancers Blood/Urine Tests Hearing/Blood/Urine Tests Vitamin Testing/Bone Density/Exercise Counseling Cancer Screening

Working together for you CF CENTER AND PRIMARY CARE

Working together for you Benefits of having both CF Center and Primary Care Comprehensive health care, including Bird s eye view General health needs Partner to screen/address the increased risks from aging with CF Coordination of local care

Working together for you Insurance and logistics Some insurance policies insist on labs and x-rays/CTs be done at specific sites (not Stanford) Some don t cover Primary Care Some cover home nursing services to assist when you are on home IV therapy, others don t Example: Home IV coordination of care: Lab draws, Mediport/PICC line care Example: Local PFTs

Working together for you: Patients perspective By offering routine evaluations such as vaccines, pap/pelvic exams, and evaluations for my newest chief complaint sports injuries as a result of new lungs! I have gained more respect and gratitude for primary care s role. At my doctor s office, I have a sense of satisfaction and pride that I am at the doctor for something normal. - Anabel Stenzel Stenzel A, Prim Care Prog. 2012

References Al-Aloul M, Miller M, Alapati S, Stockton PA, Ledson MJ, Walshaw MJ. Renal impairment in Cystic Fibrosis patients due to repeated intravenous aminoglycoside use. Pediatric Pulmonology 2005;39:15-20 DOI:10.1002/ppul.20138 Aris RM, Renner JB, Winders AD, Buell HE, Riggs DB, Lester GE, Ontjes DA. Increased rate of fractures and severe kyphosis: Sequelae of living into adulthood with cystic fibrosis. Annals of Internal Medicine 1998;128(3): 186-193. Billings JL, Dunitz, JM, McAllister S, Herzog T, Bobr A, Khoruts, A. Early colon screening of adult patients with cystic fibrosis reveals high incidence of adenomatous colon polyps. J ClinGastroenterol 2014; 48:e85-e88. Centers for Disease Control (2017) Adult Immunization Schedule, https://www.cdc.gov/vaccines/schedules/downloads/adult/adult- schedule-easy-read.pdf Cohen-Cymberknoh M, Shoseyov D, Kerem E. Managing Cystic Fibrosis: Strategies that increase life expectancy and improve quality of life. American Journal of Respiratory and Critical Care Medicine 2011;183,1463-1471. Cystic Fibrosis Foundation Patient Registry Annual Data Report 2015. https://www.cff.org/Our-Research/CF-Patient-Registry/2015- Patient-Registry-Annual-Data-Report.pdf Elborn JS, European Respiratory Review. 2013; 22(127)3-5; DOI: 10.1183/09059180.00008112 Elborn JS, Bell SC, Madge SL, et al. Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis. European Respiratory Journal 2016;47:420-428. DOI: 10.1183/13993003.00592-2015

References Elborn JS, Shale DJ, Britton JR. Cystic fibrosis: Current survival and population estimates to the year 2000. Thorax 1991;46:881-885. Emery J, Hayflick S. The challenge of integrating genetic medicine into primary care. British Medical Journal 2001; 322:1027-30. Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, Durie PR, Legrys VA, Massie J, Parad RB, Rock MJ, Campbell PW 3rd; Cystic Fibrosis Foundation. J Pediatr. 2008 Aug;153(2):S4-S14. doi: 10.1016/j.jpeds.2008.05.005. Florescu M C, Lyden E, Murphy PJ, Florescu DF, Fillaus J. Long-term effect of chronic intravenous and inhaled nephrotoxic antibiotic treatment on the renal function of patients with cystic fibrosis. Hemodial Int, 2012;16: 414 419. doi:10.1111/j.1542- 4758.2012.00675.x Gory I, Brown G, Wilson J, Kemp W, Eldho P, Roberts SK. Increased risk of colorectal neoplasia in adult patients with cystic fibrosis: A matched case-control study. Scandinavian Journal of Gastroenterology 2014;49(10):1230-1236. DOI: 10.3109/00365521.2014.936035 Littlewood J M, Kelleher J, Rawson I. Comprehensive assessment at a CF centre identifies suboptimal treatment and improves management, symptoms and condition. In Tenth International Cystic Fibrosis Congress, Sydney. 1988 Lopes-Pacheco M. (2016). CFTR Modulators: Shedding Light on Precision Medicine for Cystic Fibrosis. Frontiers in Pharmacology, 2016;7, 275. http://doi.org/10.3389/fphar.2016.00275 Lowton K, Ballard KD. Adult cystic fibrosis patients experiences of primary care consultations: a qualitative study. British Journal of General Practice 2006;56:518-525.

References Maisonneuve P, FitzSimmons SC, Neglia JP, Campbell PW, Lowenfels AB. Cancer Risk in Nontransplanted and Transplanted Cystic Fibrosis Patients: A 10-Year Study. J Natl Cancer Inst 2003; 95 (5): 381-387. DOI: 10.1093/jnci/95.5.381 MacKenzie T, Gifford AH, Sabadosa KA, Quinton HB, Knapp EA, Goss CH, et al. Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: Survival analysis of the cystic fibrosis foundation patient registry. Ann Intern Med. 2014; 161:233-41. Neglia JP, FitzSimmons SC, Maisonneuve P, Schoni MH, Schoni-Affolter F, Corey M, Lowenfels AB, The risk of cancer among patients with cystic fibrosis. New England Journal of Medicine; 1995;332(8)494-499. Okumura MJ, Kerr EA, Cabana MD, Davis MM, Demonner S, Heisler M. Physician views on barriers to primary care for young adults with childhood-onset chronic disease. Pediatrics 2010; 125(4)e748-e754: DOI: 10.1542/peds.2008-3451 Phelan P, Hey E. Cystic fibrosis mortality in England and Wales and in Victoria, Australia 1976-80. Archives of Disease in Childhood 1984;59:71-73. Quon BS, Mayer-Hamblett N, Aitken ML, Smyth AR, Goss CH. (2011) Risk factors for chronic kidney disease in adults with cystic fibrosis. American Journal of Respiratory and Critical Care Medicine 2011;184: 1147-1152. Schechter MS, Stecenko AA. Chronic kidney disease: A new morbidity of cystic fibrosis or an old morbidity of diabetes mellitus? American Journal of Respiratory and Critical Care 2011;184: 1101-1102.

References Smyth AR, Bell, SC, Bojcin S, Bryon M, Duff A, Flume, P, et al. European cystic fibrosis society standards of care: best practice guidelines. J. Cyst. Fibros. 13 (Suppl. 1), S23 S42. DOI: 10.1016/j.jcf.2014.03.010 Stenzel A. Complex patient learns the value of primary care. Primary Care Progress 2012; http://www.primarycareprogress.org/blogs/16/199 Walters S, Britton J, Hodson ME. Hospital care for adults with cystic fibrosis: an overview and comparison between special cystic fibrosis clinics and general clinics using a patient questionnaire. Thorax 1994;49:300-306.

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