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Beyond Cystic Fibrosis: Beyond Cystic Fibrosis:

Beyond Cystic Fibrosis: - PowerPoint Presentation

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Beyond Cystic Fibrosis: - PPT Presentation

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

primary care fibrosis cystic care primary cystic fibrosis center patients health disease increased risk risks kidney study cancer doi

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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!