Cystic Fibrosis is a life threatening, genetic disease that causes persistent lung infections, and progressively limits the ability to breathe (Cystic Fibrosis, n.d.).Pathophysiology and History of

Cystic Fibrosis is a life threatening, genetic disease that causes persistent lung infections, and progressively limits the ability to breathe (Cystic Fibrosis, n.d.).Pathophysiology and
History of
the Disease
Cystic Fibrosis is caused by defects in
the cystic fibrosis gene.
Cystic Fibrosis is a life threatening, genetic disease that causes persistent lung infections, and progressively limits the ability to breathe (Cystic Fibrosis, n.d.).Pathophysiology and
History of
the Disease
Cystic Fibrosis is caused by defects in
the cystic fibrosis gene.
Cystic Fibrosis was recognized over 400
years ago in Germany.
There is no cure for Cystic Fibrosis.Impact on Adults to the Health
Care System and Nursing Practice
Increased heath care costs
Over crowed clinics
Individuals received less personal
attention
Limited number of hospital beds
Nursing educationImpact of the Disease on Childhood Altered body image
Impaired relationship with peers
Impact of living with a chronic diseaseImpact of the Disease on Adults
Burden of care increases
Taking more medicines and receiving
more treatments.
Lung damage is greater in adulthood
than earlier in life.
Fertility issues (Heintz, 2010, para. 6)Reasons People with CF are
living into Adulthood
GENE THERAPY
ANTIMICROBIAL THERAPY
THE MODERN CF CARE
CENTER
MUCOLYTICS
MUCOCILIARY CLEARANCEImpacts this Disease will have
on the Adult Client.
Mentally and emotionally
Physically
Sexually
Economically, specifically as it relates to the ability to access health insurance and health care
Susceptibility to engage in substance abuse
Prenatal care and childbearing (see additional slide)
Occupational considerations and hazards (see additional slide) Ability to cope with stress (see additional slide)Pregnancy
Pregnancy
is now
possible
with cystic
fibrosisPregnancy
Importance of Prenatal Care
Pregnancy Statistics
Focus on Family PlanningInfertility causes
The majority of men
with cystic fibrosis
have associated
congenital bilateral
absence of the vas
deferens ( mutations,
1995). Female infertility is
less clear. There is
a theory that the
vaginal mucosa is to
thick for sperm
passage. Other
reasons are thought
to be low BMI, poor
nutrition, and
chronic infection
(Hodges, 2008).Choosing a career
Cystic Fibrosis should not
prevent one from pursuing the
career they seek
Some careers pose greater
health risks for Cystic Fibrosis
patients
(Careers, 2013)Learning to cope
The key is to limit stress by:
Knowledge: ask, watch, study and read
Support: support groups, get in touch with other families that know the ropes.
Planning: getting through daily life will be overwhelming, write a daily plan.
Strength: do not listen to well meaning people with
negative advise listen to your healthcare providers
(About, 2014).Outcomes for the Adult Patient
Adequate airway clearance
Reduction of risk factors associated with
respiratory infections

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