Cystic Fibrosis in Children - Symptoms, Diagnosis and Prevention
Health Education

Cystic Fibrosis in Children

Dr.Surya Rao Poodipeddi profile Authored by Dr.Surya Rao Poodipeddi on 28 Jul 2014 - 18:08.

Cystic-Fibrosis

Cystic fibrosis (CF) is a life threatening genetic disorder affecting infants and children.It affects about one child in every 2,500 if the baby is a Caucasian. In African heritage the incidence is low affecting one in 15,000 children. In Asians the incidence is as low as one in 30,000 babies.

The root cause that triggers this possibility is a gene named CF gene. Babies get this disease if they inherit one defective cystic fibrosis gene from the mother and one from the father and the disorder is almost always diagnosed sometimes in the first three years of life.

The production of heavy and sticky mucous is the main cause which clots and blocks the passageways in the lungs and the pancreas causing labored breathing and digestive problems. The mucous also acts as an ideal vehicle for bacteria to grow and cause serious infections.

Some of the main symptoms include: The inability to put on weight, pungent stools, a salty tasting skin and a persistent cough with a wheeze. Most of the babies with this disorder are born with their intestines obstructed by the meconium, a green/black substance that comes out in the baby’s first stool after birth.

It is very important for the mothers to notice any of these main symptoms and immediately consult a pediatrician. Though CF cannot be cured, the encouraging aspect of this condition is that there are new treatments that not only prolong a child’s life but also make life near normal. The rule of thumb is the earlier the condition is diagnosed the more effective these new therapies will be. Therefore, immediate attention to these symptoms can help the pediatrician address them effectively.

The diagnosis is done by a test called “sweat test”. One of the signs of this disease is salty-tasting skin which is significance from the “sweat test” The test is quick and painless. A drug called pilocarpine stimulates a spot on the arm to sweat. A filter paper placed on the sweat absorbs it which is subjected for testing sodium and chloride content. Higher than the normal level of sodium strongly suggest CF. Family history of CF and other tests like chest X-ray will affirm the results of the sweat test.  Once the preliminary tests point towards Cystic Fibrosis, a confirmative blood test is done for a genetic study. The results available in 3 to 4 days will settle the diagnosis.

Treatment depends on the severity of the disease. There are hundreds of mutations of the CF gene each causing a different kind of disease and the symptoms have no consistency in their appearance. They may be severe or slow down. They may get aggravated or sometimes fade away. A baby with a bad case may need prolonged stay in the hospital for treatment. The one with a milder attack may need treatment only for few times in a year.

The important point to remember is to try and drain out the mucous. Thumping the chest with a cupped hand is done to loosen the mucous. Putting the baby on a tilted table with his head down helps drain out the mucous. Doctors treating the child will teach these methods to the mothers and inmates so that time is not lost in draining out the thick mucous. If the problem increases, the child has to be shifted to hospital where expert methods like suction apparatus etc. are used to remove the mucous.

Medication :Doctors have been developing new drugs that not only relieve the dogged symptoms of CF but also lengthen their life span. Pulmozyne is a new drug which reduces the number of lung infections making breathing more easier.

TOBI (TOBramycin Solution for Inhalation) approved in 1997 improves lung function and reduces number of hospital stays. The prospects keep getting better and better. In the earlier days a child born with CF did not live beyond 2nd year of life. Now, with the advent of newer techniques and newer drugs the life span of a child born with CF in 1998 has an expected life span of 40 years.

Educated mothers and mothers with access to internet can get lot of latest information on CF from Cystic Fibrosis Foundation, a central clearing house for information, advocacy and support.

Most of the babies get preventive shots of vaccine against Hib and Pneumonia. Majority of the babies with CF receive bronchodilators which will clear the lungs for a better and easy breathing. Corticosteroids and bronchodilators are the two common drugs used in most of the babies. Some doctors use Ibuprofen which is claimed to be an effective agent to open up the airways. Whatever drug is given, it is important for the mothers to carefully follow the doctor’s instructions.

Because the disease gums up the pancreas with the sticky mucous digestion becomes a nagging problem, Pancreatic enzymes are essential for breaking down the food especially fats. Lack of these enzymes in CF may lead to undernourishment and hunger in the baby. The best choice in such situations is to encourage the babies to eat more frequently and also add supplements of enzymatic preparations. A healthy and well-nourished baby with CF will respond better to the treatment schedules than a malnourished baby. If we set aside the bad part of this problematic disorder there is some good news also.

The good news is that, in many cases, babies with CF can grow up to lead nearly normal lives. Like any other children they can go to school, play with other children, and continue to be kids despite the fact that the adults are watchful and vigilant.

*Disclaimer This is not medical advice. The content is for educational purposes only. Please contact your doctor for any health care issues.