Category Archives: Inherited Diseases

Cystic Fibrosis Diet

 Nutritional Needs of Cystic Fibrosis Child:

Generally, kids with Cystic Fibrosis need more calories than other kids in their age group. The amount of additional calories they need will vary according to each child’s lung function, activity level, and illness.

A child’s caloric needs might be even higher during an illness (even a low-grade infection can greatly increase the calories required). A CF dietitian can help you determine how many calories your child needs each day, and track growth and weight gain over time in order to provide a good nutrition plan.

Essential fatty acids. Patients with cystic fibrosis have altered levels of plasma fatty acids. Found in plant oils, safflower oil, and soybean oils, they help in the building of cell membranes and may play a role in lung function. It is suggested that CF patients try to incorporate more omega 3 fatty acids into their diet, which can be done with either food sources or supplements. Salmon, flax seeds, and walnuts are excellent food sources of omega 3 fatty acids. Your CF dietician wll guide you with that.

Calcium. Patients with pancreatic insufficiency have difficulty absorbing calcium and are especially at risk for developing osteoporosis (weak, brittle bones), as are all kids with CF. Dairy products are good sources of calcium (and the full-fat varieties also are good sources of fat and calories). Many juices also are fortified with calcium.

Fat-soluble vitamins (vitamins A, D, E, and K  These vitamins are important for immune function, growth, and healing. They are absorbed along with fat. Most kids with CF have trouble digesting fat, they often have low levels of fat-soluble vitamins and need to take vitamin supplements.

Iron. Children with CF are at risk for developing iron-deficiency anemia, which can cause fatigue and poor resistance to infection. Fortified cereal, meats, dried fruits, and dark green vegetables are good sources of iron.

Salt. Kids with CF lose a lot of salt in their sweat, especially during hot weather and when they exercise. So a CF dietitian may suggest adding salt to an infant’s formula and giving an older child salty snacks. During hot weather and when your child plays sports, he may need sports drinks along.

Zinc.  is found in meats, liver, eggs, and seafood. It is important for growth, healing, and combating infection

Meals at Home and Away

If your child is a choosy eater, pay attention to the foods your child especially likes and adapt the menu as these preferences change. Ask an older child with CF to try one new food a week. It’s also important to model good behavior for kids of all ages. Eat a well-balanced diet with a variety of foods and make sure you try new things yourself.

To make meals a pleasant experience:

  • Try to keep meals to about 20-30 minutes for toddlers and young kids, who can’t sit still for extended periods.
  • Eat in a comfortable environment with few distractions.
  • Don’t give huge portions that might seem irresistible.
  • Offer praise for eating well. Encourage kids to eat as much as they can comfortably.
  • Keep food choices simple, especially for younger kids.
  • Make foods as attractive and appealing as much as possible.

All childcare providers and teachers should know that your child has CF and be aware of his or her nutritional and caloric needs. Work with the staff to plan high-calorie meals and snacks or send food if the menu can’t accommodate your child’s needs. Young kids need help taking their enzymes and supplements, and the staff should understand that they need to be taken before all meals.

As kids with CF grow into teens, they’ll probably begin eating more meals away from home and may feel pressured to eat in certain ways. Work with your teen, emphasizing the positive and monitoring any weight changes that are of concern. If you’re packing lunch, include high-calorie options like ranch dressing and increasing Caloric Intake.

Make sure that kids with CF eat enough fat and calories. One way to increase calories without creating an entirely separate menu is to increase the calories and fat in one part of the meal.

Here are some simple ways to do so:

  • Add extra butter or margarine to sandwiches, sauces, and potatoes.
  • Use dressings on salads or vegetables; add extra oil to the dressing.
  • Prepare meals with gravies and creamy sauces.
  • Add bacon to burgers and chicken.
  • Add dried skim milk powder to sauces and beverages.
  • Add extra cheese to garnish potatoes or macaroni and cheese; order extra cheese on pizza.
  • Top salads and sandwiches with avocados.
  • Add nuts to cookies, cakes, pancakes, and salads.
  • Add extra cheese and deli meats to sandwiches.
  • Grill sandwiches in butter or margarine.
  • Use heavy whipping cream and whole milk when cooking.
  • Make milkshakes.
  • Add instant breakfast mixes to milk-based beverages.
  • Make high-calorie smoothies.
  • Prepare calorie-rich desserts such as pudding and cheesecake.
  • Top hot chocolate, pudding, and other desserts with whipped cream.
  • Provide high-calorie snacks like peanut butter crackers or trail mix.
  • Prepare high-calorie versions of popular family recipes.

When you’re shopping, be sure to compare the labels on similar prepared foods and purchase those that have higher fat and calorie content. Avoid diet foods — anything that claims to be nonfat, low fat, reduced calorie, or light/lite.

Look for whole-fat versions of dairy products such as sour cream, cottage cheese, and yogurt. Don’t forget to check baby food labels. You’d be surprised at how the caloric level varies between brands.


Beyond Food: Enzymes and Tube Feeding


A child with pancreatic insufficiency will need to take enzymes with meals and snacks to help digest food properly and to get the nutrition and fat needed to grow and gain weight.

Signs that your child may need enzymes or an enzyme dose adjustment include:

  • failure to gain weight, in spite of a strong appetite
  • frequent, large, greasy, or smelly bowel movements
  • bloating or gas

The CF dietitian or doctor will prescribe enzymes based on weight, growth, and how much a child eats at a time. An increased enzyme dose doesn’t necessarily mean that a child is doing poorly. It may mean that he or she has gained weight and now requires more enzymes.

Enzymes need to be taken with every meal and most snacks. They come in capsules, full of tiny beads, that can be broken open for kids who are too young to swallow entire capsules. They should only be mixed with foods that are acidic, like applesauce. They should not be chewed or crushed. Never change the dose of enzymes without first consulting your child’s dietitian or doctor.


Cystic Fibrosis Genes

Cystic Fibrosis Facts

What is Cystic Fibrosis?

  • Cystic Fibrosis (CF) is one of the  most common life-threatening inherited diseases.
  • It is caused by a defective gene that controls the movement of salt and water in and out of the cells within the body.
  • Cystic Fibrosis affects the internal organs, especially the lungs and digestive system, by blocking them with thick sticky mucus. This makes it hard to breathe and digest food.
  • Cystic fibrosis (CF) is a genetic disorder that particularly affects the lungs and digestive system and makes kids who have it more susceptible to recurring lung infections.
  • Due to high-tech medical advances in drug therapy and genetics, children born with CF can live longer and more comfortable lives. In the last 10 years, research into all aspects of Cystic Fibrosis has helped doctors to understand the illness better and to develop new therapies. Ongoing research may lead to a cure in near future.
  • The inherited CF gene directs the body’s epithelial cells to produce a defective form of a protein called CFTR (or cystic fibrosis transmembrane conductance regulator) found in cells that form the lining of the lungs, digestive tract, sweat glands, and genitourinary system. When the CFTR protein is defective, epithelial cells can’t regulate the way chloride to pass across cell membranes. This disrupts the essential balance of salt and water needed to maintain a normal thin coating of fluid and mucus inside the lungs, pancreas, and passageways in other organs. The mucus becomes thick, sticky, and hard to move.
  • Normally, mucus in the lungs traps germs, which are then cleared out of the lungs. But in CF, the thick, sticky mucus and the germs it has trapped remain in the lungs, which become infected.
  • In the pancreas, thick mucus blocks the pathways that would normally carry important enzymes to the intestines to digest foods. When this happens, the body can’t process or absorb nutrients properly, especially fats. Kids with CF have problems gaining weight, even with a normal diet and a good appetite.
  • For a baby to be born with Cystic Fibrosis, both parents must be carriers of the defective CF gene. The diagram shows how CF is inherited. Where both parents carry the faulty gene, each child has a one in four chance of having CF, a two in four chance of being a carrier and a one in four chance of not having any CF genes.

    Cystic Fibrosis Genes

  • Cystic Fibrosis affects more than 30,000 kids and young adults in the United States.
  • Each week, five babies are born with Cystic Fibrosis.
  • Each week, two young lives are lost to Cystic Fibrosis.
  • Presently half of the CF population will live past 41 years of age, and improvements in treatments mean a baby born today is expected to live even longer.

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Cystic Fibrosis Tests

How is Cystic Fibrosis diagnosed?

Cystic Fibrosis is increasingly being diagnosed through screening, but some babies and older children (and even adults) are diagnosed following unexplained illness.

There are three types of screening for Cystic Fibrosis: newborn screening, carrier testing and antenatal testing.

 Newborn Testing:

The test is a heel-prick to sample blood as part of the normal Guthrie test carried out on all children. The sooner cystic fibrosis is diagnosed, the sooner appropriate treatment can begin.

cystic fibrosis tests

heelprick test Continue reading


Cystic Fibrosis Symptoms

What are the Symptoms of Cystic Fibrosis?

Cystic Fibrosis causes the body to produce thick secretions that particularly affect the lungs and digestive tract.

  • Lungs
  • Symptoms include a persistent cough, repeated chest infections, prolonged diarrhea and poor weight gain. These symptoms are not unique to Cystic Fibrosis.
  • Digestive system

Cystic Fibrosis affects the pancreas, which makes it difficult for people with CF to digest food. This can cause malnutrition, which can lead to poor growth and delayed puberty. In older patients, insulin production can become deficient due to increasing pancreatic tissue damage. Some develop CF related diabetes mellitus and their blood sugar levels are no longer controlled. This rarely happens to children with Cystic Fibrosis.

  • Common symptoms of diabetes include thirst, hunger, weight loss and excessive need to urinate.
  • Other Affected Organs
  • People with CF develop thin, brittle bone due to the nutritional and other problems involved with the disease. Bone disease in adults is because of the adverse effects of steroids taken to control lung disease.
  • In most men with CF, the tubes that carry sperm are blocked, which causes infertility. Because underweight women are more likely to have irregular menstrual cycles, the nutritional problems associated with CF may affect fertility. Women with CF do produce healthy, fertile eggs so effective contraception is necessary. Cystic Fibrosis does not cause sexual impotency.
  • Cystic Fibrosiscan cause the blockage of small ducts in the liver. This only happens to approximately 8% of people who have CF, but it is a serious health risk and may require liver transplantation. Continue reading