Cystic Fibrosis 1

Running head: CYSTIC FIBROSIS

Epidemiological Study of Cystic Fibrosis

Dan Abrahamsson

Hawai’i Pacific University

NUR 4960

Frances Spohn, MS, MPH, RN, CHES

April 8, 2023

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Abstract

Cystic fibrosis (CF) is the most common inherited disease in the United States. There is no cure

and the complications of CF are fatal. Most patients die during early adulthood. Treatment

opportunities focus on secondary and tertiary prevention. The only options to prevent the passing

on of the defective genes to offspring are abstention from sexual intercourse, contraception, or

abortion. Medical care is uninterrupted and costly. Patients and families are subject to constant

stressors. Quality and access to care for rural and low income families are inadequate. Proficient

case management and the collaboration of physicians, physiotherapist and community health

nurses can improve the quality of care and quality of life for CF patients and their families.

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Table of Contents

Introduction…………………………..……………………………………………………………5

I. Overview of the Problem………………….……………………………………………….….5

A. Definition…………………………………………………………………………………5

B. Impact……………………………………………………………………………………..6

1. On client…….……..…………………………………….……………………………6

2. On family……………………………………………………….………………..……7

3. On society………….………………………………………………………………….9

C. Epidemiology Triad…………………….………………………………………………..10

1. Agent………………………………….……………………………………………..10

2. Host……………………………………..…………………………………………....11

3. Environment……………………………………..…………………………………..12

II. Literature Review/Statistical Evidence………………………………………………………14

A. International……………………………………………………….……………………..14

B. National………………………………………………………………………………….16

C. State/Local……………………………………………………….………………………16

III. Cultural/Ethnic Considerations……………………………..………………………………..17

IV. Community Resources……………………………………………………………………….18

A. National…………………………………………………….……………………………19

B. State/Local……………………………………………………………….………………20

V. Healthy People 2010…….…….……………..……..………………………………………..22

A. Objective 1-6…...………………………………………………………………………..22

B. Objective 6-2...…………………………………………………………………………..23

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Table of Contents (Continued)

VI. Roles of Community Health Nurses……………………………...………………………….23

A. Primary Prevention………………………………………………………………………24

1. Public Health Nurse..…………………………………….…………………………..24

2. Parish Nurse…......…………………………………………….……………………..25

B. Secondary Prevention……………………………………………………………………25

1. Home Health Nurse..…………………………………………………….…………..26

2. School Nurse………………………………………………………………….……..26

C. Tertiary Prevention………………………………………………………………………27

1. Home Health Nurse..………………………………………………………….……..27

2. Hospice Nurse..……………………….………….………………………………….28

VII. Conclusion…………………………………..……………………………………………28

VIII. References……………………………………………………………………………….30

IX. Appendixes………………………………………………………….………………………..35

A. Epidemiology Triangle for Cystic Fibrosis...……………………………………………36

B. Number of Patients by State in the CF Patient Registry.…………………………….…..37

C. Summary of the CF Foundation’s Patient Registry Data 2006……………..…………...38

D. Population and Prevalence of Patients with CF in EU Countries…...………………...…39

E. Association Between Incidence of Death and Median Household Income…………...…40

F. List of State and National Resources for CF Patients…………………………………...41

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Epidemiology Study of Cystic Fibrosis

Cystic Fibrosis (CF) was discovered as a distinct disease in 1938 (Davis, 2006, p. 475).

Prior to 1938, the symptoms of CF were considered to be part of celiac disease. Autopsies of

deceased and emaciated infants revealed sticky mucus which congested the pancreatic ducts and

other glands throughout the body. The clogged ducts of the pancreas prevented the excretion of

pancreatic enzymes to the small intestine. As a result, the characteristics of the disease were

malabsorption of nutrients and excess fat in the feces (steatorrhea). Initially the malnourishment

was believed to cause lung infections, which lead to lung damaged. Cysts and scar tissue

(fibrosis) developed in the lungs that ultimately caused death to these infants. During a heat wave

in the summer of 1948 Paul di San’t Agnese, a New Yorker pediatrician, noticed a remarkable

increase of sodium and chloride in the sweat of children with CF. This lead to a simple “sweat

test” method (pilocarpine iontophoresis) to diagnose the disease. The defective CF gene was

discovered in the epithelial cells of sweat ducts in 1989 (Davis, 2006, p. 475).

Overview of the problem

Definition

Cystic Fibrosis is an autosomal recessive disease caused by the presence of two recessive

mutant genes on the transmembrane conductance regulator (CFTR). The CFTR is a protein

involved in the transport of chloride ions across cell membranes. The genetic defects of the

CFTR result in abnormal transport and exchange of electrolytes and water into and out of

epithelial cells that produce mucous and secretion in the body. The sweat glands produce

unusually salty sweat and the mucous becomes dense and sticky (National Heart and Blood

Institute [NHLBI], 2008). These alterations lead to common complications in the lungs and the

gastrointestinal tract. The lungs cannot clear the airways of pathogens and become more

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susceptible to respiratory infections. The ducts in the pancreas and the liver become clogged and

the pancreatic digestive enzymes and bile from the gallbladder cannot reach the small intestine to

digest food. The abnormal mucous in the lining of the intestines impedes the absorption of

nutrients. The poor absorption of nutrients causes malnutrition and the failure to thrive (NHLBI,

2008). The CFTR defect is also present in other glands and ducts in the body, such as in the

salivary glands and genitalia. There is no cure and the complications of CF are fatal. Most

patients die during early adulthood. Treatment methods focus on secondary and tertiary

prevention. The only options that the disease carriers have for primary prevention are birth

control to prevent conception or abortion. The CF patients need uninterrupted and costly medical

care throughout their lives. Both the CF patient and their primary care givers, who are usually

their family members, are additionally subject to the impact of countless physical and

psychological stressors. A qualitative investigation of nine families, by Rinaldi Carpenter and

Narsavage (2004), concluded that being faced with the diagnosis of CF for a family member was

a devastating experience that required continuous adjustments to, and confrontation with,

unfamiliar stressors (p. 29).

Impact

On client. The psychosocial impact on the client, in most cases the child diagnosed with

CF, is overwhelming, particularly if the disease is diagnosed later during the child’s

development. When a CF screening is done at birth, specific care for the child can be initiated

early. The child will grow up knowing that he or she has CF and will be more prepared to cope

with the many issues that develop from the disease. Concerns related to self-esteem, autonomy,

and self-reliance will emerge throughout the child’s life-span (James & Ashwill, 2007, p. 309).

Failure to thrive caused by malabsorption may result in growth failure and can also change

Cystic Fibrosis 7

physical appearance (thin extremities and a barrel-chest), which can be embarrassing for the

child. The negative body image and mocking behavior of peers will impact the child’s self-

esteem. Socialization opportunities and freedom are limited because of frequent therapies,

treatment modalities, and hospitalization. Recurring absences from school during times of

disease exacerbations can lead to delayed cognitive development. The exacerbations of the

disease and the risk for infections will isolate the child from their peers. The child’s reduced

activity-level related to undernourishment is another barrier to integration and socialization

(James & Ashwill, 2007, p. 309). The adolescent is often confronted with the mortality of the

disease. The search for self-identity may be disturbed as a result of this. The client might feel

misunderstood and insignificant. The fear of dying may become crushing. Strong emotions and

hopelessness can lead to irritation with the family and support system, and to noncompliance and

upheaval with the medical therapy. The young adult client might feel like they are a burden to

the rest of the family and support system and become even more alienated from society and

friends. The client may experience difficulties in finding a spouse as a result of the fatal course of

the disease and the risk of passing CF on to the offspring. All these issues can lead to interrupted

family processes and depression (James & Ashwill, 2007, p. 319).

On family. The family is exposed to many stressors because of the ongoing treatments

which require constant monitoring and adjustment. It is necessary for the family to allocate all of

their resources, including financial, time, and energy, to the care of the ill family member. The

financial burden of co-payments to the health insurer for treatments and drugs, prescribed annual

cost deductibles, and cost exclusions for pre-existing illness, could force the family into poverty.

The family needs to review their health insurance plan for home healthcare and drug coverage

limits, payment conditions, benefit caps, and premium costs (CFF, 2006a, pp. 1-3). There is

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rarely any “time off” for any family member and the entire family is at risk for social isolation.

This can lead to a situational crisis where the normal coping abilities of the family are

insufficient. Prioritizing and balancing the needs of the family will be difficult. The focus should

not be the care of the CF family member; rather the needs of the CF family member should be

integrated into the activities of the family. The risk for neglect of other family members is

increased by overprotection of the family member with CF (James & Ashwill, 2007, p. 307). The

immense burden of responsibilities in the parenting role can become overwhelming if it is not

shared between both parents. Since the condition and the needs of the ill family member

suddenly can drastically change, all members within the family system have to learn to be

flexible in their expectations and roles (James & Ashwill, 2007, p. 307). Rinaldi Carpenter and

Narsavage (2004) described three theme clusters for the reactions and coping strategies for

affected families: Falling Apart, Pulling Together, and Moving Beyond. When the family finds

out that a child has CF there is the feeling of Falling Apart which includes three subthemes: (1)

The Devastation of Diagnosis, (2) An All-Encompassing Sense of Fear and Isolation, and (3) An

Overwhelming Sense of Guilt and Powerlessness (p. 29). The following stage, Pulling Together,

has two subthemes: (1) The need for Perpetual Vigilance and (2) Developing Lifestyle Adaptions

that Bring a Sense of Normalcy. The subtheme of the third stage, Moving Beyond, helps the

family to find new ways to exist with an “Optimal Unfolding of a New Kind of Consciousness”

(Rinaldi Carpenter & Narsavage, 2004, p. 29-30). Mastering a situational crisis by rearranging

the family structures and obligations often strengthens the family. This will be especially

important at the time of death of the ill family member. The family might experience an

emotional void and feelings of total futility after the ill family member has passed away (James

& Ashwill, 2007, pp. 308-09).

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On society. The integration of the CF client into society has psychosocial and varied

economical and financial factors. Families who have lost their health insurance coverage because

of economic hardship and unemployment, or who have no funds left for co-payments for the

drugs and the extensive treatments, ultimately will need the aid of social welfare systems. Some

families might not have had any insurance and will be completely dependent on federal and state

welfare benefits from the beginning of diagnosis and treatment. In 2003, 26.6 percent of the

population in the United States had government-provided free health insurance, such as Medicare

and Medicaid, and 15.6 percent had no insurance at all (U.S. Census Bureau, 2006, p. 1). This

means that 42.2 percent of the U.S. population had no private or employment-based health

insurance in 2003. The U.S. Census Bureau’s (2006) household economic study for 2001

compared health status and health insurance coverage of a representative sample of 281,113

persons (p. 10). The result of the survey showed that people who considered themselves having a

lower health status had the highest rate of government financed coverage and the lowest private

health insurance coverage (U.S. Census Bureau, 2006, p. 10). School age children with CF will

need adjustments in the school environment to enable them to obtain their education and to

prepare them for the future. Public schools and universities with federal funding are required to

accommodate urgent needs of a CF student, provided that the student has an Individual

Education Plan (IEP) under the Individuals with Disabilities Education Act (IDEA) or a 504 plan

under Section 504 of the Rehabilitation Act of 1973. These accommodations could, for example,

include access to a private bathroom, a private dorm room, disabled parking, and tutor services

when the client is hospitalized (CFF, 2006b, pp. 2-5). The educational institution will have extra

expenditures for these adjustments. All these accommodations that will assist the client with CF

to maintain a realistic standard of living and quality of life will not only have an impact on the

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family’s financial assets; it will have a vast financial impact on the society. An appropriate level

of funds collected through taxation is needed to cover all societal-born expenses of the CF

patient.

Epidemiology Triad

In contrast to the natural history of communicable diseases with distinct periods of

prepathogenesis and pathogenesis, levels of prevention, and multiple causations of diseases, the

epidemiologic concepts for hereditary diseases are slightly different. According to Maurer and

Smith (2005), the factors (agent, host, and environment) that are used to analyze the natural

history of a disease in the epidemiologic triangle have the ability to change the balance of health

(p. 155). When using the epidemiologic triangle to analyze CF it is important to keep in mind

that most host factors are secondary effects of the disease. There is no cure for CF. The patient is

not dying because of the genetic defect: the patient is dying from secondary infections that are

associated with CF.

Agent. The agent determinant of the CF is biologic since the defect genes are inherited. It

has not been exactly determined what mutagen causes the defect of the gene that is responsible

for the transcription of the CFTR protein in CF. Mutations could be caused during copying of a

sequence with bases of the DNA in the genetic material during replication. A spontaneous

mutation occurs on average once in every 10,000,000,000 bases. Other causes of mutagenesis are

viruses, exposure to UV or ionizing radiation, chemicals, such as benzene, carbon tetrachloride,

and vinyl chloride (Bettelheim, Brown, & March, 2004, pp. 636-37). In de novo mutation, the

mutation takes place only in the female ovum or in the male gamete (sperm). These kinds of

mutagens could be indirect physical and chemical agent determinants. It can also occur

immediately following impregnation. De novo mutations are thought to be responsible when

Cystic Fibrosis 11

none of the parents is a carrier of the disease although the child is affected with the disease

(Genetics Home Reference [GHR], 2008). The CF gene is located on chromosome 7, band q31.

The first mutation of the CF gene that was discovered was named ∆F508 and lacks 3 base pairs

in exon 10 accountable to code for phenylalanine at the 508th amino acid location of the CFTR

protein (Shulman, 2005, pp. 205). The defect ∆F508 gene is the most frequently observed

mutation in the Caucasian population of Central and Northern European origin. The majority of

the Caucasian carriers is homozygous or compound heterozygous for only eight mutations. Until

today, around 1000 mutations have been discovered; however, only 13 of these mutations occur

in more than one percent of the CF chromosomes. Clinical presentations among the affected

clients with CF are almost as variable as the number of available mutations (Shulman, 2005, pp.

205-06). The American College of Obstetricians and Gynecologists (ACOG) provides a 25-

mutation standard laboratory screening to detect mutations of the CF gene in Caucasian and other

ethnic couples who have a family history of the disease and are planning to have children

(Shulman, 2005, p. 205). Genetic screening and DNA analysis of prospective parents are

required in prenatal diagnoses of CF, since it is not possible to assess the CFTR protein in the

amniotic fluid or the chorionic villi. There is an important limitation to the prenatal screening.

Mutations that are not included in the 25-mutation panel could be present in the client’s cells,

remain undetected, and provide a false negative result for the test. Therefore, counseling the

couple about the inability to completely rule out a CF mutation is very important (Shulman,

2005, p. 205).

Host. The host determinant of the disease is intrinsic since CF is an inborn disease. The

disease is produced only when two copies of the defect gene are present. If only one parent is a

carrier of the defect gene, the child will not have CF but will have a 50% possibility of being a

Cystic Fibrosis 12

carrier of the defective gene. If both parents are carriers of the defect genes there is a 25%

possibility that the child will have CF, and a 50% possibility that the child will be a carrier of the

defect gene. Other determinant factors for the host, such as demographics, level of health, body

defenses, state of immunity and the human behavior act as indirect host factors during the disease

progress. Age acts indirectly as a host determinant since most patient die of the complications at

young age. Gender also plays a significant role in the occurrence of the disease. Most males born

with CF experience degenerative changes similar to those which occur in the pancreatic ducts in

the vasa deferentia, which cause the ducts to disintegrate. This condition is called congenital

bilateral absence of the vas deferens (CBAVD). Until recently men with CF were considered

infertile. Since the introduction of assisted reproduction and intra-cytoplasmic sperm injection

(ICSI), an invasive method to remove sperms from the testicles of a male person with a syringe

to inject them into a female ovum, many CF affected men have the chance to become

reproductive again without being fertile. Affected couples that would like to use assisted

fertilization need counseling about the risk of having a child with CF (Gazvani & Lewis-Jones,

2006, pp. 268-70). Ethnic background is strongly correlated with CF. The disease is more

concentrated in the ethnicities of Caucasians, especially with Ashkenazi Jewish background. The

goal in the treatment of CF is to maintain the level of health and increase body defenses as long

as possible with adequate nutrition, physiotherapy, exercise, stress management, and by reducing

risks for infections, particularly for respiratory infections caused by Pseudomonas aeruginosa

and Burkholderia cepacia complex (CFF, 2006b, p. 11). Human behavior is the most significant

factor in preventing early exacerbations of CF. Adherence to the numerous therapies, treatments

and medications, as well as avoiding aggravating factors (smoking, recreational drugs, alcohol) is

crucial. The patient should have all recommended immunizations to minimize exposure to

Cystic Fibrosis 13

extrinsic factors that can cause disease exacerbation. Since the CF patient is especially

susceptible to pulmonary complications, regular flu and pneumonia vaccinations are necessary.

Yearly immunization with polyvalent conjugate vaccine against Pseudomonas aeruginosa

significantly reduces the number of CF patients contracting infection and additionally delays the

chronic state of this multiantibiotic-resistant strain (Lang et al., 2004, p. 508).

Environment. There is a triad of determinants in environmental factors of CF: biologic,

physical, and socio-economic. The biologic determinant must be another human being of the

opposite gender for reproduction and procreation of offspring with CF defect genes. As discussed

above in the host section, the biologic determinant is more concentrated among Caucasians and

especially within the Ashkenazi Jewish population. Although the physical environment, or

geographical environment, has no direct boundaries anymore, the highest prevalence of CF is

among the above mentioned ethnicities in middle and northern Europe and the United States,

naturally related to the history of immigration to the United States. Food and drugs are other

biologic entities that will also have an impact on socio-economic considerations. CF patients

need increased caloric intake due to increased metabolism. Their recurrent infections need

extended treatments with special antibiotics and the patient needs daily pancreatic enzyme

medications to be able to digest their food intake. These factors generate high costs and

economic burdens which the family, insurance companies and communities have to deal with. As

long as CF has a considerably low general prevalence it will be difficult to increase awareness

and help through public policy. Physical properties that will have a secondary influence on CF

are: air quality (in- and outdoors), seasons (allergens and heating), and climate (cold and

humidity). These properties have a direct influence on the patient’s pulmonary function. A study

correlating exacerbations of CF with ambient air pollution and socioeconomic status revealed

Cystic Fibrosis 14

that patients with no insurance or government-financed insurance, such as Medicare and

Medicaid, were more likely to have exacerbations, pancreatic insufficiency, and to be colonized

with disease specific pathogens (Goss, Newsom, Schildcrout, Sheppard, & Kaufman, 2004, p.

817).

Literature Review/Statistical Evidence

Although the prevalence of CF in the United States is low compared to many other

diseases, it is considered to be the most common fatal inherited autosomal recessive disease

(Farrell et al., 2008, p. S4). There are 30,000 patients living with CF in the United States and

70,000 patients worldwide (Cystic Fibrosis Foundation [CFF], 2008). Research and development

of new therapies and medications have increased the lifespan of the CF patients during recent

decades. According to the Patient Registry Annual Data Report, published by the Cystic Fibrosis

Foundation in 2007, the median predicted age of survival for CF patients in 2006 was 36.9 years

(CFF, 2007, p. front cover). This is a remarkable increase over the period since the foundation’s

data collection started in 1950. At that time the median predicted survival was only one year

(CFF, 2007, p. front cover). Because the prevalence and incidence of CF are low compared to

other diseases, it is difficult to find pharmaceutical companies that would be willing to invest in

research for cures or medications for CF. The Cystic Fibrosis Foundation Therapeutics Inc.

(CFFT), a nonprofit affiliation of the Cystic Fibrosis Foundation, is advocating about the need

for research among pharmaceutical companies and is currently investing in 15 different

promising projects for drugs (CFF, 2007, p. 6).

International

Until recently it was difficult to accumulate data concerning the worldwide prevalence

and incidence of CF. In 2004, the World Health Organization (WHO) reported that mutations of

Cystic Fibrosis 15

the CFTR gene had been increasingly diagnosed in Latin America, the Middle East, India,

Pakistan, and even in persons with pure African descent (WHO, 2004, p. 1). Although the

incidence of CF in these parts of the world are much lower than in Europe and the United States,

this increased occurrence has changed the perception that CF only occurs in the Caucasian

population or in mixed bloodlines with Caucasians. The World Health Organization reported that

the incidence of CF in the African population in South Africa was 1 in 7,056 births, in

Chile/Latin America was 1 in 4,000 births, and in Bahrain/Middle East was 1 in 5,800 births. The

countries with the lowest incidence reported a wide range of occurrence statistics. India reported

1 case of CF in 40,000-100,000 births and Japan reported 1 case in 100,000-350,000 births

(WHO, 2004, p. 15). Farrell’s (2008) recently published case report, The prevalence of cystic

fibrosis in the European Union, compiles the data from 115 references in 33 European countries.

The data was collected for the year 2004 and the outcome shows great variations within the

European countries. In summary, there were 35,806 CF patients in the EU countries which had a

total population of 486,114,000 (Farrell, 2008, p. 451). The mean prevalence was 0.737 CF

patients per 10,000 people, which is very close to the calculated prevalence in the United States,

0.797 per 10,000 (Farrell, 2008, p. 451). Among the European countries Latvia reported the

lowest prevalence with only 0.104 patients per 10,000 persons. The Republic of Ireland had the

highest prevalence of 2.98 per 10,000 people, closely followed by the United Kingdom with 1.37

patients per 10,000 people (Farrell, 2008, p. 451). The reason for the high concentration

especially in these countries may be related to their geographic isolation, since both Ireland and

the United Kingdom are island states. A complete listing of the prevalence and estimated

incidence of CF in Europe, for the year 2004, can be found in the appendix of this paper.

Cystic Fibrosis 16

National

The yearly incidence rate of CF in the United States is about 1 in 3,500 newborn (Farrell

et al., 2008, p. S4). Approximately 1,000 persons are diagnosed yearly with CF in the United

States (Centers for Disease Control and Prevention [CDC], 2004, p. 1). The ranking of the states

with the highest number of patients in 2006 were: California with 1,915 patients, which equaled

8% of all registered CF patients by the foundation, New York (1,561 or 6.6%), Texas (1,370 or

5.8%), Ohio (1,363 or 5.7%), and Florida (1,092 or 4.6%) (CFF, 2006c). These top ranking states

also have the highest concentration of Jewish populations in the United States. The Cystic

Fibrosis Foundation registered 14 CF patients in Hawai’i in 2006, which equaled only 0.1% of

all registered patients. A complete listing of the CFF patient registry with all the states, Virgin

Islands, and Puerto Rico can be found in the appendix. A study including 23,817 CF patients in

the United States, published in 2003, showed a strong monotonic association between local

household income and mortality rate. The incidence rate in mortality for the lowest income

category ( < $ 20,000) was 90.3 per 10,000 person years. The incidence rate for the highest

income category ( > $ 50,000) was 62.6 per 10,000 person years. This showed that there was a

44% higher incidence risk of death in the lowest income category (O’Connor et al., 2003, p.

e336).

State/Local

The majority of the 31 registered cases of CF in the state of Hawai’i are Caucasian

(77%); only 7 individuals (23%) had Japanese, African American, Hawai’ian, Asian, or mixed

ancestry. (Hawai’i Cystic Fibrosis Newborn Screening Task Force, 2006, p. 14). The total

population estimate of Hawai’i for year 2007 is 1,283,388 (U.S. Census Bureau, 2008). This

means that the prevalence rate of CF in the state of Hawai’i is very low, only 1 in 42,780

Cystic Fibrosis 17

citizens. The yearly incidence rate in Hawai’i is statistically insignificant because of the ethnic

diversity in Hawai’i. Only 26.3% of the total population is Caucasian and consequently belong to

the risk factor group of genetic carriers (U.S. Census Bureau, 2008). Nevertheless, the State

Department of Health decided to include CF in the mandatory State Newborn Screening Panel

(NBS) program effective September 1, 2008 (Hawai’i State Department of Health, 2008a).

Cultural/Ethnic Consideration

Cystic Fibrosis can affect patients of both genders and any ethnic group. The disease is,

however, more prevalent among Caucasians with northern European and Ashkenazi Jewish

ascendants. Throughout the 20th century there have been many controversial debates about the

connection between race and predisposition to specific diseases. Many researchers have opined

that social categories of race have played a minor role in the prediction of certain diseases.

Today’s genomic research suggests anew that social categories of race do include significant

genetic differences (Osagie, 2008, p. 491). There is a significant risk for stigmatization since CF

mainly affects the Caucasian population to a very limited extent, yet significantly affects the

Ashkenazi Jewish population. From this point of view, some authorities may not find it

unimportant to invest in research for a cure or the prevention of CF. On the other hand; the

perception can arise that there is an extraordinary threat with CF in this ethnic group, and that

defective genes will be passed on increasingly faster to the overall population. Altogether, with

CF, there are nine genetic disorders that have a markedly elevated prevalence in the Ashkenazi

Jewish population. One in seven persons is a carrier of defective genes for at least one of these

nine disorders (Strom, 2004, p. 17). The estimated carrier rate of CF among the Ashkenazi Jews

is 1:25 (Weinstein, 2007, P. 50). Since their religion does not allow termination of pregnancies,

the Jewish authorities had to find another solution for this dilemma. Dor Yeshorim, which is an

Cystic Fibrosis 18

organization responsible for genetic testing that follows the Jewish law, was established. Blood

tests from carrier screenings are kept on file confidentially and prospective couples can call the

organization to ask if they are “compatible” for a marriage. The confidentiality of the test results

prevents the individual from being stigmatized; however, the marriages are arranged solely on

the basis of negative test results (Strom, 2004, p. 12). New York, Miami, Chicago, Los Angeles,

and Baltimore are cities with large Jewish populations that perform frequent publicized screening

tests in Jewish community centers synagogues, on college campuses, and even during the Israel

Independence Day celebrations (Weinstein, 2007, pp. 58-59). It is important to keep in mind that

the cause of the high prevalence of CF genetic codes and disease in the Ashkenazi Jewish

population is related to the laws and the religious dogmas that forbid matrimony outside the

congregation and is not because of Jewish ethnicity. Cystic fibrosis is not a Jewish disease.

Some people may have a false sense of security that CF will remain segregated within the Jewish

population. Sensitivity and responsibility are key factors in any discussion about CF’s genetic

relevance to race, as they are in equal protection jurisprudence for everyone who considers racial

characteristics in a medical context (Osagie, 2008, p. 496).

Community Resources

The Internet has rapidly become the primary overall tool for inquiries about information

and resources of any kind. The use of search engines provides consumers with a myriad of web

links to federal, state, and organizational health care resources that can be widely dispersed and

extremely confusing. The websites of the U.S Department of Health and Human Services (HHS,

2008) and the Health Resources and Services Administration (HRSA, 2008) include the most

complete information about governmental grants, medical service delivery, health system

concerns, medical data and statistics, assistance availability, eligibility for benefits, clinical

Cystic Fibrosis 19

practice, and reimbursement policies. Below are a few examples of nationwide and statewide

community resources specifically for CF. A more comprehensive listing of resources including

degrees of prevention can be found in the appendix.

National

The two major national resources that provide primary, secondary, and tertiary

prevention, by means of information, assistance, and affiliated direct care for CF patients, are the

Cystic Fibrosis Foundation (CFF) (www.cff.org) and the Cystic Fibrosis Research, Inc. (CFRI)

(www.cfri.org). Both organizations fund research, provide educational and personal support, and

are the main national institutions in advocating and spreading awareness of cystic fibrosis. The

CFF was founded in 1955 and its mission is to assure “the development of the means to cure and

control cystic fibrosis and to improve the quality of life for those with the disease” (CFF, 2007,

p. 4). CFF is the principal institute dedicated to CF in the United States. The CFF’s consolidated

statement of financial position shows total assets of $301,903,423 in 2007. The total revenue in

2007 of $246,591,350 was mainly achieved by support from the public, special events, pharmacy

services, and investment income (CFF, 2007, pp.39-40). The organization finances 80 chapters

and branch offices, 95 adult CF care programs, and 50 affiliated CF programs, and accredits 115

Care Centers located all over the United States mainland (CFF, 2007, p. 15). In 1988 CFF

established the CF Services Pharmacy (CFS) that specializes in CF drugs and also assists patients

in receiving maximum reimbursements from their health insurance drug plans. The CF Services

Pharmacy provides financial support to patients who cannot afford medications and also sponsors

the Care Centers and chapters of the foundation (CFF, 2007, p.17). The foundation’s Advocacy

Website gives information about policy decisions that affect the CF community. The foundation

advocates and pursues a legislative agenda to increase funding for research toward a cure and

Cystic Fibrosis 20

also to ensure that CF patients have access to care and the latest therapies. The foundation

advocated for the reauthorization of the State Children’s Health Insurance Program (SCHIP),

since an increasing number of CF patients have difficulties covering the costs of care. CFF offers

a legal information hotline that assists CF patients and caregivers with for example; issues

relating to coverage of medications and government benefits (CFF, 2007, pp. 27-29).

The CFRI was founded in 1975 and is an independent, nonprofit volunteer organization

involved in all three degrees of prevention. The organization recruits research participants for

clinical research projects and provides referrals to CF treatment centers on the U.S. mainland. At

annual conferences the organization informs physicians, scientists, health care professionals, CF

patients, and their caregivers about news and recent achievements in the research projects. CFRI

arranges special CF Teen and Adult Retreats, offers support groups, and creates educational

material for the community, such as the Cystic Fibrosis Website Guide and the Cystic Fibrosis

Classroom. These websites include a data base of knowledge for the patients and their

caregivers. CFRI is a strong advocate for the promotion and legislation of newborn screening

programs. Since its establishment in 1975 the organization has raised more than 7.5 million

dollars through personal mail solicitations, grants, and special fundraising events. The above

mentioned resources fully meet the needs for education and support in primary prevention. The

115 CFF Care Centers complete the needs for secondary and tertiary prevention. The financial

support for quality treatments and medications remains the biggest issue and cannot be

guaranteed with the current system of reimbursement.

State/Local

The specialized services for CF patients in Hawai’i are poor. The national CF resources

listed above and in the appendix must suffice for the needs of the 31 CF patients in Hawai'i.

Cystic Fibrosis 21

Tripler Army Medical Center is a CFF accredited Care Center; however it is available only for

families of military personal. There is no other CFF funded Care Center in Hawai'i since the

accreditation criteria require a minimum of 50 patients (Hawai’i Cystic Fibrosis Newborn

Screening Task Force, 2006, p. 16). The health care agencies that are caring for CF patients in

Hawai'i are Tripler Army Medical Center, Kapiolani Medical Center, and Kaiser Permanente

Hospital/Health Care System. The agencies are all on Oahu. The outer islands have no

standardized service centers. All three Oahu agencies have the diagnostic sweat test for CF

available. The management and follow-up of care for CF patients at Kaiser Permanente and

Kapiolani Medical Center are organized by independent visits to each agency’s specialist and

evaluation within each agency. Kaiser Permanente has a genetic counselor available for couples

and pregnant women. Genetic counseling in Kapiolani Medical Center is provided by a

pulmonologist. Tripler Hospital uses the Tripler Genetics Consultation office and a

pulmonologist. Prenatal screening and care is provided by all three agencies plus Queen’s

Medical Center and the Fetal Diagnostic Institute of the Pacific (Hawai’i Cystic Fibrosis

Newborn Screening Task Force, 2006, p. 14). Because the health agencies just listed are not

specialized CF Care Centers, they cannot provide the same quality of care as the specialized care

of the CFF Care Centers at Tripler Hospital or located on the U.S. mainland. Although all three

categories of preventions are provided by the Oahu agencies, they are not as optimal as the care

given by CFF accredited Care Centers. It would be advantageous for CF patients in Hawai'i if the

local health agencies could collaborate to form a collective Hawai’ian CF Care Center.

The Family Health Services Division of the State Department of Health offers a special

program that assists families with coordinating and obtaining services for children who have

chronic health conditions and require specialized medical care. The program named Children

Cystic Fibrosis 22

with Special Health Needs coordinates health and other services, organizes follow-up with

family, physicians, specialists, and other providers, and serves as a resource for information

about community services. Additionally, the organization provides social work and nursing

services, nutrition consultation, arranges Neighbor Island Clinics, and provides limited financial

assistance for families who meet financial requirements. The broad spectrum of the organizations

services meets all three degrees of preventative health measure. The children have to be less than

21 years of age to the eligible for the program (Hawai’i State Department of Health, 2008b).

Healthy People 2010

The ultimate goals of Healthy People 2010 are to “increase quality and healthy life” and

to “eliminate health disparities” (Healthy People, 2008a). There are 467 objectives in this

national framework of goals designed to reduce preventable threats to health. The two health

objectives stated below are representative for CF.

Objective 1-6

“Reduce the proportion of families that experience difficulties or delays in obtaining

health care or do not receive needed care for one or more family members” (Healthy people,

2008b). The target is to reduce this threat from the baseline data of 12% (in 1969) to 7% for

2010. Twelve percent of the U.S. civilian, noninstitutional population stated, among other

reasons, that they could not afford care, or insurance companies would not approve, cover, or pay

for care. The majority of these were families with an income level below or near the poverty

level. When asked about the health insurance status of their family, 27% stated they were

uninsured, 12% were insured with public insurance, and 7% had private insurance.

Hispanic/Latino, American Indian, and Alaska Natives were ethnicities who experienced the

highest difficulties or delays in receiving health care. The Community Health Nurse (CHN)

Cystic Fibrosis 23

could search for alternative funding resources and apply for donations from charitable

organizations to assist the CF family financially.

Objective 6-2

“Reduce the proportion of children and adolescents with disabilities who are reported to

be sad, unhappy, or depressed” (Healthy People, 2008c). The target is to reduce this threat from

the baseline data of 31% of children aged 4 to 11 years with disabilities (in 1997) to 17% for

2010. Thirty-one percent of the U.S. civilian, noninstitutional population stated, among other

reasons, that they had been unhappy, sad, or depressed, or were limited in some way in activities

because of physical, mental, or emotional problems. The majority of these were families with a

poor or near-poor income level. The data for several ethnic groups were considered unreliable.

The available data showed that 32% of the Hispanic/Latino, 31% of the White, and 30% of the

not Hispanic or Latino disabled children stated that they were sad, unhappy, or depressed. The

CHN could help the families and disabled children by making a thorough assessment of the

feelings and strengths of each member within the family unit. The plan of care should use the

existing strengths to improve and increase coping opportunities. The CHN should also develop

appropriate recreational activity plans that could help to reduce the strain and the stress of

ongoing treatments.

Roles of Community Health Nurses

Nurses generally need to know and understand genetics and genetic testing to be able to

care for families and patients with genetic diseases, such as CF. The community health nurse

(CHN) should be knowledgeable about local services for all kinds of patient care in the

community. One role of the CHN is to be a source for information, referrals, and support for the

patients and their caregivers. Therefore, the CHN needs to be aware about genetic disease

Cystic Fibrosis 24

specific community services such as: genetic testing sites, screening programs, and genetic

counseling services (Weinstein, 2007, p. 59). The CHN needs to stay up-to-date with activities in

the community, awareness campaigns, and community support groups for CF patients and

patients with other genetic disorders. The CHN must teach the families and patients about their

choices and evaluate their understanding of the disease in order that they can make informed

decisions. It is important that the CHN remains non-judgmental, objective, and does not impose

ones own opinion on the client. When the client has made an informed decision the CHN accepts

and supports the client’s choice (Weinstein, 2007, p. 59).

Primary Prevention

Primary prevention avoids the development of a disease. Health promotion activities are

primary precautionary actions. Primary prevention for parents that are carriers of the defective

genes is limited to counseling regarding the likelihood of having a child with CF and instruction

about the use of contraception to prevent pregnancy. The defective genes causing cystic fibrosis

are present in all body cells at the time of conception and consequently later at birth. The genetic

tests available to prospective parents can determine if they are carriers of the defect genes that

cause cystic fibrosis. Although screening generally is considered to be secondary prevention,

genetic screening to detect carriers is considered to be primary prevention, since the conception

of an embryo has not yet occurred.

Public health nurse (PHN). The PHN acts as a researcher, an educator, and an advocate

in primary prevention. As a researcher the PHN works in the emerging field of public health

genetics to discover and collect data about mutagens for CF. The PHN applies advances in

human genetics, genomics, and molecular biotechnology that can lead to improved health and

quality of life for the CF patients, and can also hopefully lead to a cure for the disease. The PHN

Cystic Fibrosis 25

works as an educator by compiling data and informing governmental authorities and the public

about the results of research projects. The PHN surveys and assesses the population of affected

CF patients as a whole and advocates their needs in front of governmental policy makers and

legislators.

Parish nurse (PN). The PN acts as a counselor, role model, and educator in primary

prevention. The PN could be either a Jewish or Catholic nurse who is counseling young adults

about genetic carrier testing, and about the probability of giving birth to a child with CF. Patient

education is crucial in achieving informed consent and rational decision-making. Teaching

clients about test result ranges can help them predict their results and be more prepared for the

outcome (National Guideline Clearinghouse, 2008). The PN uses active listening and therapeutic

communication to help affected persons cope with a positive testing outcome. As a role model,

the PN promotes sexual self-control before marriage, follows other religious protocols, and acts

in accordance with the expectations of the religion. As an educator, the PN explains the natural

history of CF and informs affected carriers about their choices using internal and external

resources consistent with the client ethics and based on the best available information. Although

genetic testing provides important information for family planning, it can also cause great

anxiety and may compel an agonizing decision about a pregnancy (National Guideline

Clearinghouse, 2008).

Secondary Prevention

Secondary prevention actions are intended to foster early disease detection. Early

detection increases the opportunities for intervention to prevent the progression and exacerbation

of CF. Prenatal screening of pregnant women is an act of secondary prevention. It is the earliest

opportunity to predict the disease in an unborn fetus. The sweat test, pilocarpine iontophoresis, is

Cystic Fibrosis 26

used for the actual diagnosis of CF, and cannot be done until the newborn baby is two weeks old.

There are genetic tests that can detect some of the CF gene mutations in the fetus. However, the

defective CF genes undergo spontaneous mutations that prevent the tests from detecting all

variations of the disease (NHLBI, 2008). The prenatal tests available for detecting CF are

amniocentesis and chorionic villus biopsy. In amniocentesis, the cells from the amniotic fluid,

surrounding the fetus in the uterus, are tested for cystic fibrosis genes. In a chorionic villus

biopsy, placental tissue cells are removed and tested for the cystic fibrosis genes (Labtestonline,

2006).

Home health nurse (HHN). In secondary prevention the HHN acts as a care provider,

case manager, and educator. The HHN is the actual link and case manager between the patient,

the family, and the collaborative treatment team. A primary responsibility of the home health

nurse in their role as educator is to inform the family about treatment methods and coping skills,

and to teach the family, by performing direct care, how to care for their ill family member.

Rinaldi Carpenter & Narsavage (2004) pictured the intensity of the care of chronic ill patients in

the statement:

Routine therapies that are required of the CF family are rigorous, complex, and time

consuming. Dealing with complex drug and therapy regimens at home can prove to be

very stressful for parents and other family members. Helping families understand the

purpose and effective implementation of therapies, as well as what to expect when living

with a family member who has a chronic disease, can be effective in helping them cope

with the situation. (p. 26)

School nurse (SN). In secondary prevention the SN acts as a care provider, collaborator,

and educator. As a direct care provider, the SN administers medications to the student and

Cystic Fibrosis 27

educates the student about the importance of adherence to their treatments and therapies during

school hours. As direct care provider, the SN provides a safe retreat in the school nurse’s office

if the student becomes overwhelmed and exhausted. The SN monitors nutritional intake and

educates the student about the importance of adequate caloric intake for growth and

development. The SN collaborates with teachers and the principal to balance the student’s

learning process and physical activities with a state of health and capability. The SN educates

teachers and peers about CF, and about how to intervene in case of complications and other

emergency situations.

Tertiary Prevention

Tertiary preventions reduce the negative impact of an advanced disease. The focus of

tertiary prevention is normally on rehabilitation and reducing disease-related exacerbations. In

the later stages of CF, recurring respiratory infections become more frequent. The patient must

spend an increasing amount of time in the hospital because of acute exacerbations. Ongoing

treatments with antibiotics have made many pathogens resistant and more aggressive

medications must be used to control complications. At latter stages of the disease, only a few

patients are fortunate enough to qualify for lung transplantation, however, this still does not cure

the digestive problems of CF. Thus, lifelong intake of pancreatic enzymes must continue. The

success of lung transplantations and the length of survival have only been moderate. Ultimately,

most patients without a lung transplant will suffer and die from a fatal lung infection.

Home health nurse (HHN). In tertiary prevention the HHN acts as a care provider,

advocate, and leader. The HHN provides direct care as above, teaches the family procedures,

manages and coordinates the team-care. The team usually consists of the physician, the

respiratory therapist, the physiotherapist, the home health aide, and the nutritionist. Because of

Cystic Fibrosis 28

the intensity of care at the end of the tertiary prevention stage, the patient may want to stop the

aggressive treatments. It is the responsibility of the HHN to act as the patient’s advocate and

prevent preventable stress to the patient. The family may not accept the patient’s refuse to care;

the HHN may have to act as a leader to help solve the family conflict and to prevent an

escalation of strong emotions. The HHN may also have to advocate for the family toward home

health agencies and insurance companies in financial and reimbursement issues.

Hospice nurse (HN). In tertiary prevention the HN acts as a care provider, a counselor,

and a role model. At the end of the tertiary stage of prevention, the last sic months of life, the

chronic care of the CF patient changes and becomes palliative, more soothing and comforting.

This is the responsibility for the HN. Instead of aggressive medical treatments intended to cure

infections, the direct care of the HN includes interventions that will alleviate the patient’s

symptoms and suffering, such as analgesic and anxiolytic medication. The HN pays more

attention to the patient’s and the family’s psychological, social, and spiritual needs than to the

direct care. The HN acts as a role model for the family by listening to the concerns, accepting

feelings, showing love, touching and holding the patient’s hand. Already before death of the CF

patient, the HN is responsible for the bereavement care of the family. The HN counsels and

prepares the family for the dying family member’s transition from life to death in collaboration

with the family’s clergyman. The HN educates the family about the physical changes during the

transition, and about how important the expression of emotions during the bereavement period is.

Conclusion

The recent advances in genetic screening and testing have created many ethical and moral

issues. The discussions about how far humanity is allowed to manipulate and influence

procreation are controversial. The new, innovative possibilities in genetic testing bring immense

Cystic Fibrosis 29

advantages to fight not only CF, but many other genetic diseases. Research has revealed that CF

gene defects are being more frequently detected among populations that are not considered to

belong to any ethnic risk group. This eases the bad aftertaste of associating genetic testing with

scientific racism. The tests are very expensive and the costs for the tests are not regularly covered

by health insurance companies. Since the prevalence of CF is low in the general population, the

costs of lifelong care for a CF patient are often not taken into consideration. The current health

care system cannot absorb and capture the devastating financial and psychosocial burden for

most of the affected families. Without private wealth most families are brought to financial ruin.

A universal health care system, such as those that exist in many European countries, could

change the current deficient state of health access for CF patients in the United States and would

secure funds for the vast health care expenses of CF patients and their families. Community

Health Nurses must advocate for equity of access to care and use every chance to influence

governmental policy makers and legislature to excel over the own nations borders; to learn from

other countries with effective health care systems how to solve the problem with our own costly

and fractionated health care system. As long as there is no direct cure for CF, therapies and

treatments will be focusing on minimizing the patients’ exacerbations and improving access to

care and the patient’s quality of life. The most important nursing goals for the quality of care are

to help the CF patient and their family to live with the illness and remain healthy as long as

possible by assuring the effectiveness of care and the patient centered orientation for care.

Proficient case management and the collaboration of a multidisciplinary health care team

consisting of physicians, physiotherapists, psychologists, social workers, and community health

nurses will assist the patient and their family to better cope with the multifaceted hardships of

Cystic Fibrosis.

Cystic Fibrosis 30

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Cystic Fibrosis 33

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Cystic Fibrosis 35

Appendixes

Cystic Fibrosis 36

Appendix A

Epidemiology Triangle for Cystic Fibrosis

Agent

Host

Environment

CysticFibros

is

Biologic determinant: another human being of opposite sex for procreation. Inadequate nutrition and lack of medications or vaccines.Physical determinant: Geographic concentration in Ireland, UK, and USA, air pollution, cold climate, and distance to Care Center, substandard and/or overcrowded housing. Socio-economic determinants: Poverty, Lack of support, inadequate health care system, lack of access to care, lack of awareness by policy makers and legislators about CF., Lack of advocacy for concerns.

InheritedIntrinsic determinant: CF is inherited, acquired at conception, no cure, life expectancy low, fatality at young age. Affects both gender, Male infertility. Ethnicity: affects mainly Caucasian (Northern European) & Ashkenazi Jewish population, Extrinsic determinants: High susceptibility for respiratory infections. Ongoing aggressive treatments with leads to Abx resistance. Malnutrition due to enzyme deficit and malabsorption. Failure to thrive. Activity intolerance d/t hypoxia and metabolic disturbances. Human behavior:Non-adherence to treatments, smoker or recreational drug user

Biologic determinant: defective genes, spontaneous mutation, viruses causing mutationPhysical determinant: exposure to UV or ionizing radiationChemical determination:Maybe benzene, carbon tetrachloride or vinyl chloride

Cystic Fibrosis 37

Appendix B

Number of Patients by State in the CF Patient Registry Data 2006

Cystic Fibrosis 38

Appendix C

Summary of the CF Foundation’s Patient Registry Data 2006

Who are the patients in the CF Foundation’s Patient Registry?

CF Patients 24,487 Median BMI percentile for patients 2-20 years* 47.1%

Newly diagnosed patients in 2006 874 Median BMI for patients over 21 years 21.2%

Patient diagnosed by NBS (%) 21.6% Respiratory cultures positive for:

Median age at diagnosis 6 mos P. aeruginosa 55.0%

Age range in years 0 – 78 B. cepacia complex 2.9%

Total number of deaths 362 S. aureus 51.5%

Predicted median survival in years 36.9 S. maltophilia 12.6%

Patients 18 years and older 44.6% MRSA 18.9%

Race/Ethnicity: Complications:

Caucasian 94.6% Diabetes (CFRD)/Glucose intolerance 19.5%

Hispanic (black or white) 6.8% Bone disease (patients 18 and older) 16.7%

African American 3.9% Liver disease 10.7%

Males 52.0% Nasal polyps requiring surgery 3.7%

Genotyped 85.6% Transplants in numbers:

Home therapy: Lung -

IV antibiotics 20.9% Bilateral 187

Oxygen 5.2% Lobar-cadaveric 4

Supplemental feeding – tube 10.2% Lobar-living related donor 2

Supplemental feeding – oral only 38.0% Liver - 11

Taking pancreatic enzyme supplements 90.7% Therapies:**

Clinical trial participation number 3,037 TOBI® (tobramycin) 61.8%

FEV1% predicted mean 75.4% Pulmozyme® (rhDNase) 67.8%

Ibuprofen 4.5%

Total pregnancies among women aged 13 to 45 209 Zithromax® (azithromycin) 57.3%

Live births per 100 women age 13 to 45 1.6 Hypertonic Saline 23.2%

* The Centers for Disease Control and Prevention have calculators for Body Mass Index (BMI). The national goal for children with CF ages 2-20 years is 50th BMI percentile. For adults with CF the national goal for weight is a BMI of 23 for males and 22 for females.

** This is the percentage of patients who are eligible for a therapy and had it prescribed at least once in 2006.

Cystic Fibrosis 39

Appendix D

Population and Prevalence of Patients with CF in EU Countries

Cystic Fibrosis 40

Appendix E

Association between Incidence of Death and Median Household Income

Deaths

10095 90.

39085 81.

580 76.

473.6

757065 62.

6605550454035302520151050

<$20K $20-30K

$30-40K

$40-50K

>$50K

Median Household Income

Adjusted incidence of death in 2003 among white CF patients in the United States (per 10,000 person-years of follow-up)

Cystic Fibrosis 41

Appendix F

List of State & National Resources for CF patients

Organization: Prevention: Address Phone/email Website

Hawaii State Department of Health

Primary 1250 Punchbowl Street, · Honolulu, HI 96813

808 586-4400 http://hawaii.gov/health/

Children with Special Health Needs Program

Primary,Secondary,Tertiary

741 Sunset AvenueHonolulu, HI 96816

808 733-9055 http:Hawaii.gov/health/family-child-health/cshsn/programsandprojects.html

Kaiser Medical Center

Primary,Secondary,Tertiary

3288 Moanalua Rd. Honolulu, HI 96816

808 432-0000 http://members.kaiserpermanente.org/kpweb/facilitydir/facility.do?id=100434&rop=HAW#anchor1

Tripler Army Medical Center

Primary,Secondary,Tertiary

1 Jarrett White RoadHonolulu, Hawaii  96859-5000

808 433-6661

808 433-9226

http://www.tamc.amedd.army.mil/information/mapsandphones.html

Kapiolani Medical Center for Women and Children

Primary,Secondary,Tertiary

1319 Punahou StreetHonolulu, Hawaii 96826

808 983-6000 http://www.kapiolani.org/women-and-children/default.aspx

CFF - Cystic Fibrosis Foundation national headquarters

Primary,Secondary,Tertiary

6931 Arlington RoadBethesda, Maryland 20814

(800) FIGHT CF

800 344-4823

www.cff.org

CFRI – Cystic Fibrosis Research, Inc.

Primary,Secondary,Tertiary

Bayside Business Plaza2672 Bayshore Parkway Suite 520Mountain View, CA 940

650 404-9975 http://www.cfri.org/home.html

Cystic Fibrosis Services Pharmacy

Secondary,

Tertiary

6931 Arlington Rd, 2nd floor, Bethesda, MD 20814

800 541-4959 www.CFServicesPharmacy.com

HRSA – Health Resources and Services Administration

Primary,Secondary,Tertiary

5600 Fishers LaneRockville, MD 20857

- http://www.hrsa.gov/

HHS - The U.S. Department of Health and Human Services

Primary 200 Independence Avenue, S.W.Washington, D.C. 20201

202 619-0257

Toll Free: 1-877-696-6775

http://www.hhs.gov/

Cystic Fibrosis 42

Government Information by topic:

Health & Nutrition,Grants & Benefits

Health Insurance

Primary Washington, D.C. - http://www.usa.gov/

http://www.usa.gov/Citizen/Topics/Health.shtml