Malabsorption of nutrients in cystic fibrosis (CF) has a multifactorial origin. The factors involved in malabsorption include malfunction of the exocrine pancreas and liver, bile acid metabolism, and disordered intestinal resorptive processes. Therapeutic measures presently employed are only partially effective.
What causes malabsorption in cystic fibrosis (CF)?
In people with CF, malabsorption can be caused in two ways: Thick mucus stops the pancreas from sending enzymes into the intestines, which are needed for the body to absorb nutrients in food. A defect in the intestines prevents nutrients from passing into the bloodstream.
What is malabsorption of fats?
Fats are calorie-rich and full of fat-soluble vitamins that the body needs for growth and to stay healthy. When a body does not process nutrients properly, the condition is called malabsorption. About 80-90% people with cystic fibrosis (CF) have pancreatic insufficiency, which causes malabsorption. 1
Why do people with cystic fibrosis need a high-fat diet?
Because the malabsorption that comes with CF affects fats more than any other nutrient, CF patients often need high-fat diets to boost their calorie intake and allow for the absorption of fat-soluble vitamins.
How does cystic fibrosis affect the pancreas?
Poor growth and low weight gain tend to occur in people with CF because the thick mucus generated by CF clogs the ducts (tubes) leading in and out of the pancreas, liver, and intestines. In the pancreas, the mucus created by CF prevents the release of enzymes into the small intestines.
How does cystic fibrosis affect absorption of nutrients?
How Does Cystic Fibrosis Affect Nutrition? The sticky mucus from cystic fibrosis can block normal absorption of key nutrients and fat in the intestines, causing: poor digestion. slow growth.
How does cystic fibrosis affect nutrition?
Cystic fibrosis is a disease that causes thick, sticky mucus to build up in your lungs and digestive tract. You need to eat high-calorie, high-protein foods throughout the day to maintain a normal weight because your disease makes it hard to get enough calories from the foods you eat.
How does CF affect malabsorption of nutrients in a teenager?
CF often causes a condition called pancreatic insufficiency. This means the enzymes made in the pancreas that digest fat, carbohydrates, and protein don't pass into the intestines as they should. So the body can't digest food normally. Teens with pancreatic insufficiency might have problems with growth and weight gain.
What are the most common nutritional consequences of cystic fibrosis?
Complications of CF including liver disease and CF-related diabetes pose further challenges. In addition, deficiency of specific nutrients including fat soluble vitamins (particularly A, E and K) essential fatty acids and occasionally minerals occur for a variety of reasons. Osteopenia is common and poorly understood.
How does cystic fibrosis cause malabsorption?
In people with CF, malabsorption can be caused in two ways: Thick mucus stops the pancreas from sending enzymes into the intestines, which are needed for the body to absorb nutrients in food. A defect in the intestines prevents nutrients from passing into the bloodstream.
Does cystic fibrosis cause fat malabsorption?
Most cystic fibrosis (CF) patients malabsorb dietary fats because of pancreatic insufficiency, which leads to impaired lipolysis (5, 6).
How does cystic fibrosis affect the digestive system?
In CF, the mucus is often thick and sticky. that blocks ducts in the pancreas and prevents enzymes from reaching the small intestine to digest food. Undigested food in the intestines can cause pain, cramping, gas and either loose, greasy, floating stools or constipation. and blockages.
How does cystic fibrosis affect digestive enzymes?
„„In cystic fibrosis (CF) sticky mucus blocks the passages from the pancreas to the small intestine which stops the enzymes working, so the food cannot be digested or absorbed by the body. This is called malabsorption and causes loose or oily stools, wind, stomach ache and poor weight gain.
What vitamin deficiency is common in cystic fibrosis?
An insufficiency. For example, people with CF often have a vitamin D deficiency. , which can impair growth.
Why are cystic fibrosis patients underweight?
What are the causes of poor growth/low weight? Poor growth and low weight gain tend to occur in people with CF because the thick mucus generated by CF clogs the ducts (tubes) leading in and out of the pancreas, liver, and intestines.
What nutrients are important for a child with cystic fibrosis?
Like other children, those with CF should eat a balanced diet that includes whole grains, fruits and vegetables, dairy products, and protein. High-fat, high-calorie foods and snacks; foods with extra salt and low in sugar, can benefit children and teens with CF.
Do people with cystic fibrosis need to eat more?
People with cystic fibrosis need extra calories for several reasons. Maintaining a healthy weight -- and sometimes increasing it -- is key to fighting infection and keeping your lungs and body strong.
How does cystic fibrosis affect the digestive system?
In CF, the mucus is often thick and sticky. that blocks ducts in the pancreas and prevents enzymes from reaching the small intestine to digest food. Undigested food in the intestines can cause pain, cramping, gas and either loose, greasy, floating stools or constipation. and blockages.
What nutrients are important for a child with cystic fibrosis?
Like other children, those with CF should eat a balanced diet that includes whole grains, fruits and vegetables, dairy products, and protein. High-fat, high-calorie foods and snacks; foods with extra salt and low in sugar, can benefit children and teens with CF.
How does cystic fibrosis affect the body?
CF causes thick mucus that clogs certain organs, such as the lungs, pancreas, and intestines. This may cause malnutrition, poor growth, frequent respiratory infections, breathing problems, and chronic lung disease.
What causes a CF to be malabsorption?
The factors involved in malabsorption include malfunction of the exocrine pancreas and liver, bile acid metabolism, and disordered intestinal resorptive processes.
What is the best way to improve fat malabsorption?
Improvement of fat malabsorption is attained by using a pancreatic enzyme supplement consisting of pH-sensitive, enteric-coated microspheres ( microsphere preparations) that prevent enzyme degradation in the stomach and travel with the chyme to the small intestine.
Does microsphere help with bile salt?
Microsphere preparations, however, do not improve bile salt deficiency. The detergent Tween-80, given orally to simulate bile salt activity, does not improve fat absorption. The mucus viscosity is probably enhanced in the intestinal epithelium of CF patients and can be decreased by N-acetylcysteine, which breaks down sulfide bonds.
Why do people with cystic fibrosis burn more calories?
In addition to malabsorption, people with cystic fibrosis often burn more calories due to the extra energy it takes to breathe and fight infections . In fact, people with CF need up to twice the daily recommended calories compared to other people of the same age and weight. 1-3.
What are the symptoms of malabsorption?
Other symptoms of malabsorption include greasy, bulky stools, stomachache, and excessive gas. 1-4
What are the causes of poor growth/low weight?
Poor growth and low weight gain tend to occur in people with CF because the thick mucus generated by CF clogs the ducts (tubes) leading in and out of the pancreas, liver, and intestines.
What is PERT in CF?
Pancreatic enzyme replacements (PERT) First, pancreatic enzyme replacements (PERT) help the person with CF process their foods more efficiently so they can absorb the nutrients in the food they eat. This allows them to retain more energy from the foods they consume.
What is the side effect of cystic fibrosis?
Poor growth in children and trouble keeping weight on during adulthood are symptoms of malabsorption, a common side effect of cystic fibrosis.
Can cystic fibrosis cause fatigue?
Eating fatigue is a common complaint for people with CF. Even if a person with cystic fibrosis feels hungry and eats large amounts of food, it may still not be enough to combat malabsorption.
Why is it so hard to gain weight with CF?
For these reasons, many young men and women with CF who historically have had difficulty gaining weight can lag behind others their age in physical growth and development.
Why is CF bad for teens?
Teens with CF are at risk for poor bone health, which increases their risk for osteoporosis, osteopenia and broken bones later in life. Getting enough calcium and vitamin D in their diet builds bone mass in children and teens and helps keep adult bones healthy.
How to get CF calories for teens?
Many teens get calories through a feeding tube with a pump all night in private, and then have normal daytime meals and activities. A CF care provider can give the teen a video on the proper use of a feeding tube. Exercise. Exercise is important for teens with CF when done safely and correctly.
How much calcium is needed for CF?
Plus, the body needs more calcium when bones are growing. CF malabsorption means less vitamin D, and therefore less calcium, is absorbed, so 1,300 mg/d of calcium daily may be required. Foods high in zinc, such as meats, seafood, beans and nuts, help immune function, growth and wound healing.
What do teens with cystic fibrosis do?
However, it is crucial for a growing teen with CF to pay attention to high nutritional needs and take care of their lungs.
Why does puberty slow down?
Low body weight can lead to low bone density and fractures. Poor nutrition and lung disease can also slow puberty. A lot of bone is made during puberty, so slowed puberty could mean less bone being made, setting the stage for future bone disease.
What percentage of CF patients have constipation?
A recent investigation of 214 pediatric CF patients revealed that 47 percent of the CF children studied have constipation. Furthermore, 106 abdominal radiographs were independently assessed utilizing the Barr and Leech scores to determine the test's diagnostic value. The study concluded that constipation is a significant medical issue in CF and was associated with low total fat absorption and a history of meconium ileus; and that abdominal radiography seems of little value in the regular follow-up of CF patients.
How to help CF teens?
Teens with CF can lead active healthy lives.Paying attention to nutrition and eating a high calorie diet, taking prescribed vitamins and enzymes, and monitoring for diabetes can help teens stay healthy and reach their goals!
How can I get the nutrition I need when I have a busy lifestyle?
Teens are busy people. In addition, teens with CF need to have time to do all of their respiratory treatments and still have time to lead an active lifestyle.Getting enough calories during the day can be a challenge, but it can be done with some planning.
Can CF be fun for teens?
Teens with Cystic Fibrosis - Nutrition. Teenage years can be both fun and challenging for anyone, but this is especially so for teens with CF. During the teen years many changes take place.This is the time when puberty happens and growth really takes off. There appears to be a connection between higher body weights and lung function.
Is cystic fibrosis related to diabetes?
CF Related Diabetes. Some adults and teens with CF have cystic fibrosis related diabetes. This type of diabetes is different than the types of diabetes that occur in people without CF.