The purpose of this article is to provide a brief overview of cystic fibrosis, a potentially deadly disease that affects approximately 30,000 children.
Childhood illnesses are common and affect millions of children each year. By
and large, any illness that plagues children can potentially become serious.
Sicknesses such as chicken pox, measles, and mumps were once popular illnesses,
but now only affect a small number of children. Although complications may
arise from these illnesses, they are relatively minor when compared to other
children ailments such as cancer, diabetes, and asthma. In most cases, children
develop illnesses as they become older. However, a considerable number of
children are born each year with a rare, but serious condition. This condition
is known as cystic fibrosis, and it affects approximately 30,000 children and
3,000 newborns each year in the United States.
What is Cystic Fibrosis?
Cystic fibrosis or CF is a genetic or inherited disease that affects the
lungs and digestive tract. Children and adults living with this condition
experience a continual buildup of mucus in their lungs. Everyone produces
mucus, especially when experiencing a cold or infection. However, individuals
diagnosed with cystic fibrosis produce excessive amounts of thick, dry mucus
that clogs the lungs. This problem often leads to severe breathing difficulty,
and makes a person more susceptible to lung diseases and infections. There are
instances when mucus clogs the pancreas which prevents this vital organ from
functioning properly. A damaged pancreas may result in growth and developmental
problems. Sadly, cystic fibrosis is known to shorten a person's life expectancy.
Years ago, those who suffered from CF did not live pass the age of 27. Today,
the life expectancy of CF sufferers has increased to 32 years.
Symptoms of Cystic Fibrosis
The symptoms of cystic fibrosis vary. Those who have CF are born with the
condition. In most cases, physicians will be able to detect signs of cystic
fibrosis within days of birth. However, there have been cases when children
were not diagnosed with the condition until their teenage years. They likely
exhibited mild symptoms during their early years that did not trigger red
flags. Symptoms of cystic fibrosis include:
Blockage of small
intestines: Babies born with CF generally do not have a bowel
movement within the first 48 hours of life
Salty tasting skin
Recurrent respiratory
problems
Weight loss during
infancy
Slow growth rate
Wheezing
Chronic cough
Failure to gain
weight after birth
Poor appetite
Initially, parents may not perceive the signs of cystic fibrosis. Babies
develop differently. They are also exposed to bacteria that may cause numerous
infections. However, during routine check-ups, pediatricians are generally able
to identify any abnormalities that point to CF. Moreover, parents should report
any signs of cystic fibrosis to their infant's doctor. Additional signs include
a sudden worsening of cough or wheezing, sudden onset of breathing
difficulties, and foul-smelling stools. Parents who suspect CF should not
automatically conclude the worse. Cystic fibrosis is a rare illness, and only
affects approximately 3,000 newborns a year. The root of symptoms could be one
of many less serious, treatable conditions. Nonetheless, ignoring mild symptoms
may cause more harm to a baby's health.
Causes of Cystic Fibrosis
Cystic fibrosis is caused by a defected gene. This defected gene is inherited
from parents who are carriers. Those who are carriers of the gene that causes
CF do not have the disease. In the majority of cases, parents are unaware that
they carry the gene. In order for a baby to be born with CF, both parents must
be a carrier of the gene. In cases where only one parent is a carrier, the
child will not have CF. Additionally, both parents being a carrier of CF does
not guarantee that their baby will have the disease – but it greatly increases
the odds. Thus, it is possible for two people who carry the gene to have
several healthy babies, and only one baby with CF. Cystic fibrosis largely
affects Caucasians in the United States.
However, the disease is also common in Europe. It is
estimated that less than 10% of the Caucasian population have the defected CF
gene, nonetheless, 1 in 3,600 are affected with cystic fibrosis. Although CF is
more common for individuals of this particular race, CF also affects 1 in
17,000 African Americans and 1 in 90,000 Asians.
Long-Term Effects of Cystic Fibrosis
Because CF affects the sweat glands, children diagnosed with this condition
may have skin that taste salty. Parents and others who kiss these children will
likely detect the salty taste. Additionally, CF children also lose a
considerable amount of their body's salt when they sweat. Cystic fibrosis also
affects the pancreas, thus children experience difficulty digesting foods
properly. The thick mucus that the body produces results in many infections,
congestion, and sinus problems. Asthma-like symptoms such as wheezing and
coughing are common. When the lungs and body fills with thick mucus, CF
sufferers may cough fitfully for several minutes in order to remove mucus and
restore normal breathing. The mucus, accompanied with continual infections hinders
breathing, causing sufferers to feel winded, or struggle to take in air.
Diagnosis and Treatment
When diagnosing cystic fibrosis in infants and children, a sweat
test is preformed. This test involves collecting samples of a child's
sweat over an hour period. Afterwards the sample is examined to check the sweat
chlorine level. For accuracy, two sweat tests are routine. If the sweat
chlorine level is over 60, a diagnosis of CF is made. Additional standard test
for cystic fibrosis include:
Chest X-ray
Blood test
Stool test
Pulmonary functioning
test
Unfortunately, there is no cure for cystic fibrosis. Because CF sufferers
have a short life expectancy, a diagnosis is disheartening for many. However,
researchers and doctors across the country devote much time to developing
medications that will prolong life. The primary function of medication
available is to treat lung infections and control the body's production of
mucus. Antibiotics are effective for treating recurrent infections, whereas a
mucus-thinning drug prevents the thickening of mucus.
Preventing Cystic Fibrosis
Unlike birth defects, there is no way to prevent CF. A few parents
experience guilt due to thinking that the situation could have been prevented.
However, CF is the result of a defected gene, thus it is not related to
insufficient prenatal care, lack of vitamins, or exercise. Nonetheless, couples
who are planning on starting a family may request a genetic screening test to
determine whether they are carriers of the CF gene. Of course, both parties
being a carrier does not mean that their offspring will unquestionably develop
cystic fibrosis.