Cystic Fibrosis in Children

What is Cystic Fibrosis? What causes Cystic Fibrosis?

Cystic Fibrosis (CF) is a genetic disorder that causes irregularities in the cells of many organs in the body, usually affecting the lungs, intestines, sweat glands and pancreas.

The cell irregularities can cause secretions to become thick, and this may inhibit the absorption of nutrients and fat during the digestive process.

These secretions can also hinder the movement of cilia in the lungs. Babies with CF are therefore at higher risk for respiratory problems, poor weight gain and pancreatic problems.

The average life expectancy in 2006 of someone suffering from Cystic Fibrosis was predicted to be 37 years, but there are many varying degrees of severity. 

Cystic Fibrosis Symptoms and Signs may Include

•    The baby tastes salty when you kiss him/her. This is because babies with CF have higher levels of salt in their sweat. 

•    Signs of Cystic Fibrosis may include a baby’s stools turning greasy, frothy or very bad smelling when solid foods are introduced.  

•    A blockage of the intestines called “Meconium ileus". Surgery is needed in this instance.

•    One of the very common symptoms of Cystic Fibrosis, is when baby develops a chronic cough.

Diagnosis of Cystic Fibrosis in Children / Babies

Cystic Fibrosis diagnosis is done with an easy, non-invasive sweat test. A chemical solution is placed on the baby’s skin. The sweat is then collected and sent away to a laboratory for testing. Persons with CF have a higher than normal salt content in their blood. 

Cystic Fibrosis and Breastfeeding

Cystic Fibrosis babies may not have any feeding difficulties, but may seem hungry most of the time, due to the fact that the nutrients and fats are not being absorbed as they should. Slow weight gain may follow.

Should Mom Continue to Breastfeed?

What are the advantages of continued breastfeeding for CF babies?

•    Some CF babies only show symptoms of CF after the mother has weaned them. Formula fed babies usually show symptoms much sooner. 

•    Breastfed CF babies grow better and are heavier and taller than formula fed CF babies. 

•    Breastfed CF babies have a much lower risk of respiratory infections. 

•    CF babies that are breastfed exclusively for 6 months have been found to need IV antibiotics much less than their formula fed counterparts. 

•    Breast milk makes it easier for a CF baby, to absorb fats. Breast milk contains the enzyme Lipase, which CF babies do not produce; Lipase makes it easier for the baby to absorb the fat. 

Breastfeeding Advice

•    Try to breastfeed your baby for as long as possible. Breast milk will protect your baby for as long as he/she breastfeeds. 

•    Make sure that your baby drains the breast at each feeding, ensuring that he/she gets the hindmilk (milk towards the end of a feeding), which is more calorie dense. 

•    Breast compressions while breastfeeding, can help increase the fat content of milk that the baby receives at a feeding. 

•    The mother can pump more milk after each feed, keeping the hindmilk for extra supplementation. 

•    Calorie supplements can be added to expressed breast milk. 

Other Cystic Fibrosis Treatment Options

•    Pancreatic enzyme supplements are often given. These enzymes can help the baby gain weight faster and will help the baby absorb more nutrients. 

•    Keep the airways clean with percussion ( smacking baby’s back, to loosen any secretions). 

•    A chest vest can be used to keep the chest compressed to help remove mucous.

•    Aerosal therapy, Bronchodilators, Inhaled Corticosteroids and antibiotics can help.

•    Hydrolyzed formula may be recommended because they are easily digested. The mother should know that breast milk is still more easily digested than these formulas. If the mother cannot supply her own breast milk, Donor breast milk is the next best choice and then lastly Hydrolyzed formula.

•    A baby with Cystic Fibrosis may need salt supplements.

•    Vitamin supplements.

cystic fibrosis image, cystic fibrosis photo

Tracy Behr, CBC, CLD (CBI)

Reference: Breastfeeding counselor course through Child birth international on the Physiology of breastfeeding / Health problems / Cystic Fibrosis.

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