Broadening Your Health Care Team Meredith Wiltsie MN ANP FNP Nurse Practitioner adult Cystic Fibrosis program Cystic Fibrosis Education Day March 11 th 2017 No disclosures If you are a CF Center patient why do you need a Primary Care Physician ID: 614452
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Slide1
Beyond Cystic Fibrosis: Broadening Your Health Care Team
Meredith Wiltsie, MN, ANP, FNP
Nurse Practitioner, adult Cystic Fibrosis program
Cystic Fibrosis Education Day
March 11
th
, 2017Slide2
No disclosuresSlide3
If you are a CF Center patient, why do you need a Primary Care Physician? Slide4
You are more than CF
Cystic Fibrosis
Living FoundationSlide5
Cystic Fibrosis Foundation Patient Registry, Annual Data Report. 2015
Patients with CF are living longerSlide6
Evolving therapies
Research breakthroughs
Effectiveness of the multi-disciplinary care model
Hard work by CF patients
Patients with CF are living longer
Smyth AR et al, J. Cyst.
Fibros
. 2014
Farrell PM et al,
J
Pediatr
.
2008Cohen-Cymberknoh M et al, Am J Resp Crit Car Med. 2011Slide7
Patients with CF are living longer: Evolving Therapies
Elborn
JS,
Euro
Resp
Review. 2013Slide8
CF health needs
CF Center
Age related health needs
PCP
CF aging needs CF Center + PCP
(in collaboration)
Patients with CF are living longer: Optimal Health TeamSlide9
CF Center vs Primary CareTHE DIFFERENCES AND WHY YOU NEED BOTHSlide10
Large body of literature shows:
Improved outcomes
Improved survival
Elborn
JS et al.
Thorax. 1991
Phelan P et al. Arch Dis Child. 1984Littlewood
JM et al.
CF Congress Sydney.
1988
Walter S et al.
Thorax.
1994
CF Center vs Primary Care:
Specialized CentersSlide11
CF Center vs Primary Care:
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. 2010Slide12
CF Center vs Primary Care
Airway Obstruction
Malabsorption
DIOS
Ear Infection
ABPA
Pancreatic Insufficiency
GERD
Cirrhosis
Bronchiectasis
Hypertension
Broken Bone
Anemia
Headaches
Increased Sputum
Hemoptysis
CFRDM
Thyroid disease
Back pain
High cholesterol
Immunizations
Birth
C
ontrol
Vision Screening
STDs
Acne
Fibromyalgia
Pseudomonas
IGT
Cancer screeningsSlide13
CF Center vs Primary Care: CF Center
“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.”
CF Foundation mission:
Cystic Fibrosis Foundation Patient Registry
, Annual Data Report. 2015Slide14
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 teamNetwork of specialists with CF trainingEndocrinology
ENT, GI, Psychiatry, Liver Disease
CF Center vs Primary Care:
CF Center
Elborn
JS et al,
Euro
Resp
Jour. 2016Slide15
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 DensityBlood SugarsMental Health
CF Center vs Primary Care:
CF CenterSlide16
Airway Obstruction
Malabsorption
DIOS
Ear Infection
ABPA
Pancreatic Insufficiency
GERD
Cirrhosis
Bronchiectasis
Hypertension
Broken Bone
Anemia
Headaches
Increased Sputum
Hemoptysis
CFRDM
Thyroid disease
Back pain
High cholesterol
Immunizations
Birth
C
ontrol
Vision Screening
STDs
Acne
Fibromyalgia
Pseudomonas
IGT
I should contact the
CF Center
CF Center vs Primary Care:
CF CenterSlide17
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 CareSlide18
Day to day health:
Treat day-to-day problems, “sick visits”
Manage blood pressure
Muscle and joint pain/injuries
CF Center vs Primary Care
: Primary Care
Example 2: Travel abroad ---> specific immunizations
Example 1: Fever, sore throat ---> Strep testSlide19
Individualized risks and prevention strategies:
Pharmacogenetics
Genetic risks: screening and predispositions
CF Center vs Primary Care
: Primary Care
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
. 2001Slide20
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 agingCounseling: depression, mental health, LGBT, genetic
Immunizations
CF Center vs Primary Care
: Primary Care
What happens during an annual Primary Care visit? Slide21
CF Center vs Primary Care: Primary Care
EXAMPLE: CDC 2017 Immunizations recommended by age
CDC,
Adult Immunization
Schedule. 2017Slide22
CF Center vs Primary Care: Primary Care
Airway Obstruction
Malabsorption
DIOS
Ear Infection
ABPA
Pancreatic Insufficiency
GERD
Cirrhosis
Bronchiectasis
Hypertension
Broken Bone
Anemia
Headaches
Increased Sputum
Hemoptysis
CFRDM
Thyroid disease
Back pain
High cholesterol
Immunizations
Birth
C
ontrol
Vision Screening
STDs
Acne
Fibromyalgia
Pseudomonas
IGT
I should contact my
PCP
Cancer screeningsSlide23
Living with CF:
Pulmonary health (PFTs, sputum cultures)
Pancreatic insufficiency
Bowel health Sinus disease
Bone health
Nutrition/weight management
Mental health
CF Center vs Primary Care
: Together
Comprehensive health care includes:
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 healthSlide24
Increased Risks of Aging with CF
and how primary care can helpSlide25
Chronic kidney disease (CKD) Aminoglycoside-related kidney and hearing dysfunction
Osteoporosis
GI tract malignancies
Cardiovascular diseases
Sleep related diseases
Increased risks of aging with CFSlide26
Increased risks of aging with CF
:
Kidney Disease
1-
Quon
BS et al, Am J Resp Crit
Car Med. 20112- Schechter MS et al, Am J Resp
Crit
Car Med.
2011
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.
2Slide27
Increased risks of aging with CF
:
Kidney Disease
Risk from Aminoglycosides (Tobramycin, Amikacin,
etc
)
1- Al
Aloul
M et al,
Pedia
Pulm
.
20052 - Florescu M et al, Hemodial
Int.
2012
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
2Slide28
Aris RM et al, Ann Int Med.
1998
Increased risks of aging with CF:
Osteoporosis
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
1998 study of 70 CF patients showedSlide29
Increased risks of aging with CF:
GI Tract Cancers
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
A large study in 28,511 CF patients from 1985 to 1992 found:
Neglia
JP et al,
NEJM.
1995.
Billings JL et al,
J
Clin
Gastroenterol
.
2014Slide30
Increased risks of aging with CF:
GI Tract Cancers
1- Maisonneuve P et al,
J Natl Cancer Inst.
2003
2- Gory I et al,
Scand Jour Gastro. 2014
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
10 fold increased risk of GI tract cancer in CF patients
2
A smaller, case control study of 50 CF patients 2007-2012 showed: Slide31
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 ScreeningSlide32
Working together for youCF CENTER and primary careSlide33
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
Benefits of having both CF Center and Primary CareSlide34
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 PFTsSlide35
- Anabel
Stenzel
Working together for you: Patient’s 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.’”
Stenzel
A,
Prim Care
Prog
.
2012Slide36
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.20138Aris 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
Clin
Gastroenterol
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: 10.1183/09059180.00008112Elborn 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
ReferencesSlide37
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
. 1988Lopes-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.
ReferencesSlide38
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/95.5.381MacKenzie 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.
ReferencesSlide39
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/16/199Walters 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.
ReferencesSlide40
THANK YOU!