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Cystic Fibrosis: A Deadly Disease Affecting 30,000 Children 
 
by Valencia P. Higuera September 07, 2005

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.


 




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