Everything about Cystic Fibrosis totally explained
Cystic fibrosis (also known as
CF,
mucoviscoidosis, or
mucoviscidosis) is a
hereditary disease that affects the exocrine (mucus) glands of the lungs, liver, pancreas, and intestines, causing progressive disability due to multisystem failure.
Thick mucus production, as well as a less competent
immune system, results in frequent
lung infections. Diminished secretion of pancreatic enzymes is the main cause of
poor growth,
fatty diarrhea and deficiency in fat-soluble vitamins. Males can be
infertile due to the condition
congenital bilateral absence of the vas deferens. Often, symptoms of CF appear in infancy and childhood.
Meconium ileus is a typical finding in newborn babies with CF.
Individuals with cystic fibrosis can be diagnosed prior to birth by
genetic testing.
Newborn screening tests are increasingly common and effective. The diagnosis of CF may be confirmed if high levels of salt are found during a
sweat test, although some false positives may occur.
There is no cure for CF, and most individuals with cystic fibrosis die young: many in their 20s and 30s from lung failure. However, with the continuous introduction of many new treatments, the life expectancy of a person with CF is increasing to ages as high as 40 or 50.
Lung transplantation is often necessary as CF worsens.
Cystic fibrosis is one of the most common life-shortening, childhood-onset inherited diseases. In the United States, 1 in 3900 children is born with CF. It is most common among
Europeans and
Ashkenazi Jews; one in twenty-two people of European descent are
carriers of one gene for CF, making it the most common genetic disease in these populations. Ireland has the highest rate of CF carriers in the world (1 in 19).
CF is caused by a
mutation in a
gene called the
cystic fibrosis transmembrane conductance regulator (CFTR). The
product of this gene is a chloride ion channel important in creating sweat,
digestive juices, and
mucus. Although most people without CF have two working copies (alleles) of the CFTR gene, only one is needed to prevent cystic fibrosis. CF develops when neither allele can produce a functional CFTR protein. Therefore, CF is considered an
autosomal recessive disease.
Symptomatic diseases
Lung and sinus disease
Lung disease results from clogging the airways due to mucus buildup and resulting inflammation.
Inflammation and infection cause injury to the lungs and structural changes that lead to a variety of symptoms. In the early stages, incessant coughing, copious
phlegm production, and decreased ability to exercise are common. Many of these symptoms occur when
bacteria that normally inhabit the thick mucus grow out of control and cause pneumonia. In later stages of CF, changes in the architecture of the lung further exacerbate chronic difficulties in breathing. Other symptoms include coughing up blood (
hemoptysis), changes in the major airways in the lungs (
bronchiectasis), high
blood pressure in the lung (
pulmonary hypertension),
heart failure, difficulties getting enough
oxygen to the body (
hypoxia), and respiratory failure requiring support with breathing masks such as
bilevel positive airway pressure machines or
ventilators. In addition to typical bacterial infections, people with CF more commonly develop other types of lung disease. Among these is
allergic bronchopulmonary aspergillosis, in which the body's response to the common
fungus Aspergillus fumigatus causes worsening of breathing problems. Another is infection with
mycobacterium avium complex (MAC), a group of bacteria related to
tuberculosis, which can cause further lung damage and doesn't respond to common antibiotics.
Mucus in the
paranasal sinuses is equally thick and may also cause blockage of the sinus passages, leading to infection. This may cause facial pain, fever, nasal drainage, and
headaches. Individuals with CF may develop overgrowth of the nasal tissue (
nasal polyps) due to inflammation from chronic sinus infections. These polyps can block the nasal passages and increase breathing difficulties.
Gastrointestinal, liver and pancreatic disease
Prior to prenatal and
newborn screening, cystic fibrosis was often diagnosed when a newborn infant failed to pass faeces (
meconium). Meconium may completely block the
intestines and cause serious illness. This condition, called
meconium ileus, occurs in 10% of newborns with CF. In addition, protrusion of internal
rectal membranes (
rectal prolapse) is more common in CF because of increased fecal volume, malnutrition, and
increased intra–abdominal pressure due to coughing.
The thick mucus seen in the lungs has its counterpart in thickened secretions from the pancreas, an organ responsible for providing
digestive juices which help break down food. These secretions block the movement of the digestive enzymes into the
duodenum and result in irreversible damage to the pancreas, often with painful inflammation (
pancreatitis). The lack of digestive enzymes leads to difficulty absorbing nutrients with their subsequent excretion in the faeces, a disorder known as
malabsorption. Malabsorption leads to
malnutrition and poor growth and development because of calorie loss. Individuals with CF also have difficulties absorbing the fat-soluble vitamins
A,
D,
E, and
K. In addition to the pancreas problems, people with cystic fibrosis experience more
heartburn, intestinal blockage by
intussusception, and
constipation. Older individuals with CF may also develop
distal intestinal obstruction syndrome when thickened faeces cause intestinal blockage.
Thickened secretions also may cause liver problems in patients with CF.
Bile secreted by the liver to aid in digestion may block the
bile ducts, leading to liver damage. Over time, this can lead to
cirrhosis, in which the liver fails to rid the blood of toxins and doesn't make important
proteins such as those responsible for
blood clotting.
Endocrine disease and growth
The pancreas contains the
islets of Langerhans, which are responsible for making insulin, a hormone that helps regulate blood
glucose. Damage of the pancreas can lead to loss of the islet
cells, leading to diabetes that's unique to those with the disease. Cystic Fibrosis Related Diabetes (CFRD), as it's known as, shares characteristics that can be found in
Type 1 and
Type 2 diabetics and is one of the principal non-pulmonary complications of CF. Vitamin D is involved in
calcium and
phosphorus regulation. Poor uptake of vitamin D from the diet because of malabsorption leads to the bone disease
osteoporosis in which weakened bones are more susceptible to
fractures. In addition, people with CF often develop
clubbing of their fingers and toes due to the effects of chronic illness and
low oxygen on their tissues.
Poor growth is a hallmark of CF. Children with CF typically don't gain weight or height at the same rate as their peers, and occasionally are not diagnosed until investigation is initiated for poor growth. The causes of growth failure are multi–factorial and include chronic lung infection, poor absorption of nutrients through the gastrointestinal tract, and increased metabolic demand due to chronic illness.
Infertility
Infertility affects both men and women. At least 97 percent of men with cystic fibrosis are sterile. These men make normal
sperm but are missing the tube (
vas deferens), which connects the
testes to the
ejaculatory ducts of the
penis. Many men found to have
congenital absence of the vas deferens during evaluation for infertility have a mild, previously undiagnosed form of CF. Some women have fertility difficulties due to thickened cervical mucus or malnutrition. In severe cases, malnutrition disrupts
ovulation and causes
amenorrhea.
Diagnosis and monitoring
Cystic fibrosis may be diagnosed by many different categories of testing including those such as,
newborn screening,
sweat testing, or
genetic testing. As of 2006 in the United States, 10 percent of cases are diagnosed shortly after birth as part of newborn screening programs. The newborn screen initially measures for raised blood concentration of
immunoreactive trypsinogen. However, most states and countries don't screen for CF routinely at birth. Therefore, most individuals are diagnosed after symptoms prompt an evaluation for cystic fibrosis. The most commonly-used form of testing is the sweat test. Sweat-testing involves application of a medication that stimulates sweating (
pilocarpine) to one
electrode of an apparatus and running
electric current to a separate electrode on the skin. This process, called
iontophoresis, causes sweating; the sweat is then collected on filter paper or in a capillary tube and analyzed for abnormal amounts of
sodium and
chloride. People with CF have increased amounts of sodium and chloride in their sweat. CF can also be diagnosed by identification of mutations in the CFTR gene.
A multitude of tests is used to identify complications of CF and to monitor disease progression.
X-rays and
CAT scans are used to examine the lungs for signs of damage or infection.
Examination of the sputum under a
microscope is used to identify which bacteria are causing infection so that
effective antibiotics can be given.
Pulmonary function tests measure how well the lungs are functioning, and are used to measure the need for and response to antibiotic therapy.
Blood tests can identify liver problems,
vitamin deficiencies, and the onset of diabetes.
DEXA scans can
screen for osteoporosis and testing for
fecal elastase can help diagnose insufficient digestive enzymes.
Prenatal diagnosis
Couples who are pregnant or who are planning a pregnancy can themselves be tested for CFTR gene mutations to determine the likelihood that their child will be born with cystic fibrosis. Testing is typically performed first on one or both parents and, if the risk of CF is found to be high, testing on the
fetus can then be performed. Cystic fibrosis testing is offered to many couples in the US. The
American College of Obstetricians and Gynecologists (ACOG) recommends testing for couples who have a personal or close family history. Additionally, ACOG recommends that carrier testing be offered to all Caucasian couples and be made available to couples of other ethnic backgrounds.
Because development of CF in the fetus requires each parent to pass on a mutated copy of the CFTR gene and because CF testing is expensive, testing is often performed on just one parent initially. If that parent is found to be a carrier of a CFTR gene mutation, the other parent is then tested to calculate the risk that their children will have CF. CF can result from more than a thousand different mutations and, as of 2006, it isn't possible to test for each one. Testing analyzes the blood for the most common mutations such as ΔF508 — most commercially available tests look for 32 or fewer different mutations. If a family has a known uncommon mutation, specific screening for that mutation can be performed. Because not all known mutations are found on current tests, a negative screen doesn't guarantee that a child won't have CF. In addition, because the mutations tested are necessarily those most common in the highest risk groups, testing in lower risk ethnicities is less successful because the mutations commonly seen in these groups are less common in the general population. These couples may therefore consider testing through labs that offer CF screens with a high number of mutations tested.
Couples who are at high risk for having a child with CF will often opt to perform further testing before or during pregnancy.
In vitro fertilization with
preimplantation genetic diagnosis offers the possibility to examine the
embryo prior to its placement into the uterus. The test, performed 3 days after
fertilization, looks for the presence of abnormal CF genes. If two mutated CFTR genes are identified, the embryo isn't used for
embryo transfer and an embryo with at least one normal gene is implanted.
During pregnancy, testing can be performed on the
placenta (
chorionic villus sampling) or the fluid around the fetus (
amniocentesis). However,
chorionic villus sampling has a risk of fetal death of 1 in 100 and amniocentesis of 1 in 200, so the benefits must be determined to outweigh these risks prior to going forward with testing. Alternatively, some couples choose to undergo
third party reproduction with
egg or
sperm donors.
Pathophysiology
Cystic fibrosis occurs when there's a mutation in the CFTR gene. The protein created by this gene is anchored to the
outer membrane of
cells in the
sweat glands, lungs, pancreas, and other affected
organs. The protein spans this membrane and acts as a
channel connecting the inner part of the cell (
cytoplasm) to the
surrounding fluid. In the airway this channel is primarily responsible for controlling the movement of chloride from inside to outside of the cell, however in the sweat ducts it facilitates the movement of chloride from the sweat into the cytoplasm. When the CFTR protein doesn't work, chloride is trapped inside the cells in the airway and outside in the skin. Because chloride is
negatively charged, positively charged
ions also can't cross into the cell because they're affected by the
electrical attraction of the chloride ions. Sodium is the most common ion in the extracellular space and the combination of sodium and chloride creates the
salt, which is lost in high amounts in the sweat of individuals with CF. This lost salt forms the basis for the sweat test.
Over time, both the types of bacteria and their individual characteristics change in individuals with CF. In the initial stage, common bacteria such as
Staphylococcus aureus and
Hemophilus influenzae colonize and infect the lungs. Eventually, however,
Pseudomonas aeruginosa (and sometimes
Burkholderia cepacia) dominates. Once within the lungs, these bacteria adapt to the environment and develop
resistance to commonly used antibiotics.
Pseudomonas can develop special characteristics that allow the formation of large colonies, known as "mucoid"
Pseudomonas and rarely seen in people that don't have CF. In the past, people with CF often participated in summer "CF Camps" and other recreational gatherings. Hospitals grouped patients with CF into common areas and routine equipment (such as
nebulizers) wasn't sterilized between individual patients. This led to transmission of more dangerous strains of bacteria among groups of patients. As a result, individuals with CF are routinely isolated from one another in the healthcare setting and healthcare providers are encouraged to wear gowns and gloves when examining patients with CF in order to limit the spread of virulent bacterial strains. Often, patients with particularly damaging bacteria will attend clinics on different days and in different buildings than those without these infections.
Molecular biology
The
CFTR gene is found at the q31.2
locus of
chromosome 7, is 230 000
base pairs long, and creates a protein that's 1,480
amino acids long. The most common mutation,
ΔF508 is a deletion (Δ) of three nucleotides that results in a loss of the amino acid
phenylalanine (F) at the 508th (508) position on the protein. This mutation accounts for seventy percent of CF worldwide and 90 percent of cases in the
United States. There are over 1,400 other mutations that can produce CF, however. In
Caucasian populations, the frequency of mutations is as follows:
! Mutation
! Frequency
worldwide
|
-
| ΔF508
| 66.0%
|-
| G542X
| 2.4%
|
-
| G551D
| 1.6%
|-
| N1303K
| 1.3%
|
-
| W1282X
| 1.2%
|}
There are several mechanisms by which these mutations cause problems with the CFTR protein. ΔF508, for instance, creates a protein that doesn't
fold normally and is degraded by the cell. Several mutations, which are common in the Ashkenazi Jewish population, result in proteins that are too short because
production is ended prematurely. Less common mutations produce proteins that don't use energy normally, don't allow chloride to cross the membrane appropriately, or are degraded at a faster rate than normal. Mutations may also lead to fewer copies of the CFTR protein being produced.
Treatment
The cornerstones of management are proactive treatment of
airway infection, and encouragement of good nutrition and an active lifestyle. The treatment for cystic fibrosis continues throughout a patient's life, and is aimed at maximizing organ function, and therefore quality of life. At best, current treatments delay the decline in organ function. Treatment typically occurs at specialist multidisciplinary centres, and is tailored to the individual, because of the wide variation in disease symptoms. Targets for therapy are the
lungs,
gastrointestinal tract (including
insulin treatment), the
reproductive organs (including
Assisted Reproductive Technology (ART)) and psychological support. In addition, therapies such as
transplantation and
gene therapy aim to cure some of the effects of cystic fibrosis.
The most consistent aspect of therapy in cystic fibrosis is limiting and treating the lung damage caused by thick mucus and infection with the goal of maintaining
quality of life.
Intravenous,
inhaled, and oral antibiotics are used to treat chronic and acute infections. Mechanical devices and inhalation medications are used to alter and clear the thickened mucus.
Antibiotics to treat lung disease
Antibiotics are given whenever pneumonia is suspected or there has been a decline in lung function. Antibiotics are often chosen based on information about prior infections. Many bacteria common in cystic fibrosis are resistant to multiple antibiotics and require weeks of treatment with intravenous antibiotics such as
vancomycin,
tobramycin,
meropenem,
ciprofloxacin, and
piperacillin. This prolonged therapy often necessitates hospitalization and insertion of a more permanent
IV such as a
PICC line or
Port-a-Cath. Inhaled therapy with antibiotics such as tobramycin and
colistin is often given for months at a time in order to improve lung function by impeding the growth of colonized bacteria. Oral antibiotics such as ciprofloxacin or
azithromycin are sometimes given to help prevent infection or to control ongoing infection. Some individuals spend years between hospitalizations for antibiotics, whereas others require several antibiotic treatments each year.
Several common antibiotics such as tobramycin and vancomycin can cause
hearing loss or
kidney problems with long-term use. In order to prevent these
side-effects, the amount of antibiotics in the blood are routinely measured and adjusted accordingly.
Other methods to treat lung disease
Several mechanical techniques are used to dislodge sputum and encourage its expectoration. In the hospital setting, physical therapy is utilized; a therapist pounds an individual's chest with his or her hands several times a day. Devices that recreate this percussive therapy include the
ThAIRapy Vest and the
intrapulmonary percussive ventilator (IPV). Newer methods such as
Biphasic Cuirass Ventilation, and associated clearance mode available in such devices, now integrate a cough assistance phase, as well as a vibration phase for dislodging secretions.
Biphasic Cuirass Ventilation is also shown to provide a bridge to transplantation. These are portable and adapted for home use.
Aerobic exercise is of great benefit to people with cystic fibrosis. Not only does exercise increase sputum clearance but it also improves cardiovascular and overall health.
Aerosolized medications that help loosen secretions include
dornase alfa and
hypertonic saline. Dornase is a
recombinant human
deoxyribonuclease, which breaks down DNA in the
sputum, thus decreasing its
viscosity.
N-Acetylcysteine may also decrease sputum viscosity, but research and experience have shown its benefits to be minimal.
Albuterol and
ipratropium bromide are inhaled to increase the size of the small airways by relaxing the surrounding muscles.
As lung disease worsens, breathing support from machines may become necessary. Individuals with CF may need to wear special masks at night that help push air into their lungs. These machines, known as
bilevel positive airway pressure (BiPAP) ventilators, help prevent low blood oxygen levels during sleep. BiPAP may also be used during physical therapy to improve sputum clearance. During severe illness, people with CF may need to have a
tube placed in their throats and their breathing supported by a ventilator.
Treatment of other aspects of CF
Newborns with meconium ileus typically require surgery, whereas adults with
distal intestinal obstruction syndrome typically do not. Treatment of pancreatic insufficiency by replacement of missing digestive enzymes allows the duodenum to properly absorb nutrients and vitamins that would otherwise be lost in the faeces. Even so, most individuals with CF take additional amounts of vitamins
A,
D,
E, and
K and eat high-calorie meals. It should be noted, however, that nutritional advice given to patients is, at best, mixed: Often, literature encourages the eating of high-fat foods without differentiating between
saturated and
unsaturated fats/
trans-fats; this lack of clear information runs counter to health advice given to the general population, and creates the risk of further serious health problems for people with cystic fibrosis as they grow older. So far, no large-scale research involving the incidence of
atherosclerosis and
coronary heart disease in adults with cystic fibrosis has been conducted.
The
diabetes common to many CF patients is typically treated with
insulin injections or an
insulin pump. Development of osteoporosis can be prevented by increased intake of vitamin D and
calcium, and can be treated by
bisphosphonates. Poor growth may be avoided by insertion of a
feeding tube for increasing
calories through supplemental feeds or by administration of injected
growth hormone.
Sinus infections are treated by prolonged courses of antibiotics. The development of nasal polyps or other chronic changes within the nasal passages may severely limit airflow through the nose. Sinus surgery is often used to alleviate nasal obstruction and to limit further infections. Nasal steroids such as
fluticasone are used to decrease nasal inflammation. Female infertility may be overcome by
assisted reproduction technology, particularly
embryo transfer techniques. Male infertility may be overcome with
intracytoplasmic sperm injection.
Third party reproduction is also a possibility for women with CF.
Transplantation and gene therapy
Lung transplantation often becomes necessary for individuals with cystic fibrosis as lung function and
exercise tolerance declines. Although single lung transplantation is possible in other diseases, individuals with CF must have both lungs replaced because the remaining lung would contain bacteria that could infect the transplanted lung. A pancreatic or liver transplant may be performed at the same time in order to alleviate liver disease and/or diabetes. Lung transplantation is considered when lung function approaches a point where it threatens survival or requires assistance from mechanical devices.
Gene therapy holds promise as a potential avenue to cure cystic fibrosis. Gene therapy attempts to place a normal copy of the CFTR gene into affected cells. Studies have shown that to prevent the lung manifestations of cystic fibrosis, only 5–10% the normal amount of CFTR
gene expression is needed. Many approaches have been theorized and several clinical trials have been initiated but, as of 2006, many hurdles still exist before gene therapy can be successful.
Prognosis
In most cases, CF causes an early death. Average life expectancy is around 36.8 years, although improvements in treatments mean a baby born today could expect to live longer.
Cystic fibrosis is diagnosed in males and females equally. For unclear reasons, males tend to have a longer
life expectancy than females. Life expectancy for people with CF depends largely upon access to health care. In 1959, the median age of survival of children with cystic fibrosis was six months. In the United States, the life expectancy for infants born in 2006 with CF is 36.8 years, based upon data compiled by the
Cystic Fibrosis Foundation.
The Cystic Fibrosis Foundation also compiles lifestyle information about American adults with CF. In 2004, the foundation reported that 91% had graduated
high school and 54% had at least some college education. Employment data revealed 12.6% of adults were disabled and 9.9% were unemployed. Marital information showed that 59% of adults were single and 36% were married or living with a partner. In 2004, 191 American women with CF were pregnant.
Theories about the prevalence of CF
The
ΔF508 mutation is estimated to be up to 52,000 years old. Numerous hypotheses have been advanced as to why such a lethal mutation has persisted and spread in the human population. Other common autosomal recessive diseases such as
sickle-cell anemia have been found to protect carriers from other diseases, a concept known as
heterozygote advantage. Resistance to the following have all been proposed as possible sources of
heterozygote advantage:
- Cholera: With the discovery that cholera toxin requires normal host CFTR proteins to function properly, it was hypothesized that carriers of mutant CFTR genes benefited from resistance to cholera and other causes of diarrhea. Further studies have not confirmed this hypothesis.
- Typhoid: Normal CFTR proteins are also essential for the entry of Salmonella typhi into cells, suggesting that carriers of mutant CFTR genes might be resistant to typhoid fever. No in vivo study has yet confirmed this. In both cases, the low level of cystic fibrosis outside of Europe, in places where both cholera and typhoid fever are endemic, isn't immediately explicable.
- Diarrhoea: It has also been hypothesized that the prevalence of CF in Europe might be connected with the development of cattle domestication. In this hypothesis, carriers of a single mutant CFTR chromosome had some protection from diarrhoea caused by lactose intolerance, prior to the appearance of the mutations that created lactose tolerance.
- Tuberculosis: Poolman and Galvani from Yale University have added another possible explanation - that carriers of the gene have some resistance to TB.
History
The name
cystic fibrosis refers to the characteristic 'fibrosis' (tissue scarring) of the biliary tract ("cystic" being a generic term for all that's related to the
biliary vesicle and/or the bladder), first recognized in the 1930s. Formerly known as cystic fibrosis of the
pancreas, this entity has increasingly been labeled simply
cystic fibrosis. Although the entire clinical spectrum of CF wasn't recognized until the 1930s, certain aspects of CF were identified much earlier. Indeed, literature from Germany and Switzerland in the 1700s warned "Wehe dem Kind, das beim Kuß auf die Stirn salzig schmekt, es ist verhext und muss bald sterben," which translates to "Woe is the child kissed on the brow who tastes salty, for he's cursed and soon must die," recognizing the association between the salt loss in CF and illness.
Carl von Rokitansky described a case of fetal death with
meconium peritonitis, complication of meconium ileus associated with cystic fibrosis. Meconium ileus was first described in 1905 by
Karl Landsteiner. In 1936,
Guido Fanconi published a paper describing a connection between celiac disease, cystic fibrosis of the pancreas, and
bronchiectasis.
In 1938,
Dorothy Hansine Andersen published an article titled "
Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathological study" in the American Journal of Diseases of Children. In her paper, she described the characteristic cystic fibrosis of the pancreas correlated it with the lung and intestinal disease prominent in CF.
In 1988, the first mutation for CF,
ΔF508, was discovered by
Francis Collins,
Lap-Chee Tsui and
John R. Riordan on the seventh chromosome. Research has subsequently found over 1000 different mutations that cause CF. Lap-Chee Tsui led a team of researchers at the
Hospital for Sick Children in
Toronto that discovered the gene responsible for CF in 1989. Cystic fibrosis represents the first genetic disorder elucidated strictly by the process of
reverse genetics. Because mutations in the CFTR gene are typically small,
classical genetics techniques were not able to accurately pinpoint the mutated gene. Using protein markers,
gene linkage studies were able to map the mutation to chromosome 7.
Chromosome walking and
jumping techniques were then used to identify and
sequence the gene.
Public Awareness
Some children with cystic fibrosis in the United States call their disease
65 Roses because the words are easier to pronounce. This trademarked phrase has been popularized by the
Cystic Fibrosis Foundation. The phrase came into being when it was used by a young boy who had overheard his mother, a volunteer for the Foundation, speaking of his illness. He later informed her that he knew she was working to help with "sixty-five roses" The term has since been used as a symbol by organizations and families of cystic fibrosis victims.
The fight against cystic fibrosis has been a news story in
France, where on
April 30,
2007, the rising pop singer
Grégory Lemarchal died from the illness at the age of 23. Grégory won the fourth round of
Star Academy (equivalent of Pop Idol) in 2004 with a voting score of 80% at the grand final - a percentage unmatched in the history of the show. On
May 4, a special television programme was broadcast on
TF1 to commemorate his life, and its 10.5 million viewers were asked to donate money to help progress research into finding a cure. More than 7.5 million euros have been raised. Following his death, his family started
Association Grégory Lemarchal, an advocacy organization supporting people with cystic fibrosis.
Former Real World San Diego Castmate
Frankie Abernathy died of cystic fibrosis
June 9,
2007.
In January 2008, a documentary called "A Boy Called Alex" was screened on
Channel Four about Alexander Stobbs, a gifted music scholar at
Eton College. It showed his inspirational determination to conduct
Bach's
magnificat, despite interruptions due to life-threatening illness. The documentary has received excellent reviews and rais
Orla
Tinsley, a CF sufferer, has written many articles about CF, and the plight it causes, in the Irish Times, and is a frequent campaigner for CF patient rights.
The main character of the play 'John Lennon and Me' by Cherie Bennet has CF and resides at a pediatric hospital.
Further Information
Get more info on 'Cystic Fibrosis'.
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