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What is the Effect of Cystic Fibrosis on the Body & Importance of Diet and Nutrition in Cystic Fibrosis ?

Cystic fibrosis is a life-threatening and progressive disorder that causes damage to the lungs and the digestive system. Cystic fibrosis is a genetic disorder that leads to persistent lung infections and restricts the ability of a person to breathe properly over a period of time. If you are suffering from cystic fibrosis, then understanding the importance of good nutrition is absolutely critical for you. After all, good nutrition is necessary for maintaining your overall health and growth, especially when you are unwell.

There are many challenges that a person with cystic fibrosis has to live with, including nutrient intake, fat absorption, appetite management, and other problems. If you suffer from cystic fibrosis, then there are many ways in which you can ensure that you are getting the nutrition that you need. Proactive habits and a nutritious diet will help you deal with your symptoms of cystic fibrosis better. Today we take a look at what a cystic fibrosis-friendly diet consists of and whether or not it works for cystic fibrosis patients?

What is the Effect of Cystic Fibrosis on the Body?

Before discussing nutrition, it is important to first understand how cystic fibrosis affects the body. Cystic fibrosis is a rare genetic disorder that has no known cure as of today. You have a 25 percent chance of being born with cystic fibrosis if both your parents are carriers of the cystic fibrosis gene mutation.

Cystic fibrosis negatively affects the epithelial cells in the body. The epithelial cells are responsible for regulating the balance of water and salt in the body and are found in the pancreas, lungs, and sweat glands. These places are where mucus, sweat, and digestive juices get manufactured. People who have cystic fibrosis, end up having their body’s fluids including mucus become thick and sticky instead of staying thin and slippery ⁽¹⁾. A thickening of the fluids in the body leads to significant problems while breathing, with digestion, and also with nutrient absorption.

Sometimes, people with cystic fibrosis are also known to develop a type of exocrine pancreatic insufficiency (EPI), which causes more challenges with digestion due to the presence of excess mucus in the pancreas ⁽²⁾. This mucus starts blocking the pancreatic enzymes from traveling to the intestines, where, under normal circumstances, they would support and boost the digestive process. Without having these enzymes present in the right place, the absorption of fat, proteins, carbohydrates, and other vitamins and minerals cannot take place. Without these nutrients, the body is unable to function properly as these are required for growth and other vital functions in the body.

The Importance of Diet and Nutritional Requirements in Cystic Fibrosis

Yes, a cystic fibrosis-friendly diet does work and is beneficial for cystic fibrosis. When your body does not get sufficient protein, fat, vitamins, and other nutrients, it becomes difficult to provide protection from the common cold and other types of infections. It also becomes more difficult for the body to keep the lungs free from fluid and healthy. Cystic fibrosis can also cause you to become underweight.

It is necessary for you to consume a balanced diet that is high in protein, fat, dairy, vegetables, and fruits. While everyone needs to eat a healthy and balanced diet, consuming a healthy diet becomes even more important if you are suffering from cystic fibrosis. You need to increase the amount of each macro- and micronutrient in your diet to make sure that your body is able to still absorb a sufficient amount of these nutrients in spite of the absorption difficulties created by your disorder.

The Recipe for a Cystic Fibrosis-Friendly Diet

You can follow a cystic fibrosis-friendly diet by adding the following foods to your diet:

Salt- People having cystic fibrosis tend to have saltier sweat ^{(3, 4)}. This can cause a potential imbalance in the level of electrolytes in the body along with dehydration. Salty snacks, sports drinks and pickles can all help replace the lost electrolytes and salt.

Protein- People suffering from cystic fibrosis need to consume foods that are high in protein, including chicken, eggs, beef, fish and soy. Protein rich foods are important for preventing the loss of muscle mass and are a must include in cystic fibrosis-friendly diet.

Zinc- Cystic fibrosis patients need to include foods that are rich in zinc, such as kidney beans, spinach, beef, eggs, liver and seafood. Zinc is very much important for cystic fibrosis patients, as it helps in fighting off infections.

Iron- Iron is also important to cystic fibrosis patients, as it helps fight off infections and also increases the production of oxygen-carrying red blood cells. Foods that are rich in iron include lamb, seafood, eggs, liver, turkey and beef and should be included for a cystic fibrosis-friendly diet.

Calcium- Foods rich in calcium include dairy products, such as whole milk, high-fat cheese, and high-fat yogurt and are an important constituent for a cystic fibrosis-friendly diet. Such calcium-rich foods are required in cystic fibrosis to lower the risk of developing osteoporosis, which is a particular concern with patients.

Fiber- Doctors typically recommend consuming high-fiber foods in order to lower the risk of developing intestinal blockages when suffering from cystic fibrosis. Starting the day with high-fiber breakfast cereals is a good suggestion, as they are also rich in protein.

Antioxidants- Fruits and vegetables are one of the best sources of antioxidants and are also important to stave off bacterial infections; especially when suffering from cystic fibrosis. Patients of cystic fibrosis often experience a high level of airway inflammation in the lungs, which makes them susceptible to bacterial infections. This can be very dangerous to your health if left untreated.

How to Boost your Calorie Intake in Cystic Fibrosis?

People who have cystic fibrosis have a requirement for more calories and more fat than people who do not suffer from cystic fibrosis ⁽⁵⁾. A cystic fibrosis-friendly diet consists of meals that would generally be considered too high in fat for people who do not have this condition; for example, cheeseburgers along with French fries are generally considered to be okay choices for people having cystic fibrosis. You can increase your calorie consumption in the following ways:

Start buying whole milk instead of skimmed milk.
Opt for high-fat ground beef.
When you have eggs, fry them with extra butter.
Sprinkle bacon and cheese on eggs, salads and any other prepared dishes as per taste.
When making a sandwich, spread an extra layer of mayonnaise on the bread.
Make smoothies that are enriched with whole milk, bananas and peanut butter.
Eat pasta with olive oil and add lots of freshly-grated parmesan cheese. You can also add bacon or Alfredo sauce to increase the calories.
Instead of regular chocolate, switch to having high-quality dark chocolate.

Apart from these tips, you should also try to avoid anything that is labeled as: low-fat, diet, reduced calories and no-fat or zero fat.

Increasing your Protein Intake for a Cystic Fibrosis-Friendly Diet

Foods that are high in protein help everyone build muscles, repair tissues and keep your bones strong and healthy. For cystic fibrosis patients, it is important to get good amounts of protein every day. Apart from eating protein-rich meals, here are some other ways in which you can include protein in your diet:

Add cheese and meat to your morning breakfast of scrambled eggs. Salami, baloney, bacon, and sausage are also good options to include in a cystic fibrosis-friendly diet.
Drink high-protein shakes for a snack or even you don’t feel like having a large meal.
Opt for using quinoa instead of white rice. Quinoa is a protein-packed food that contains all the nine essential amino acids that the body needs.
Instead of eating regular yogurt, changeover to high-fat Greek yogurt.
Have almonds for a snack and you can opt for sprinkling cut almonds over cottage cheese as well for a higher nutritional boost.
Add skim milk powder to your juices, breakfast cereal, shakes, pancake mix, and malted milk. This is because skim milk powder is rich in casein, which is a type of protein that gets digested slowly by the body.

How to Ensure You Are Getting an Adequate Nutrient Intake in Cystic Fibrosis?

Apart from following a cystic fibrosis-friendly diet and the above-mentioned tips, your doctor or dietitian may also recommend you to take fat-soluble vitamins, including vitamins A, E, D, and K. These vitamins are necessary for ensuring proper growth and they are also required by the body to heal properly. Fat-soluble vitamins are critical for the body as it helps fat to get absorbed by the body. If you are not getting enough fat from your food, then you might be missing out this key nutrient and when you have cystic fibrosis, you need to increase your intake of fatty foods. You can discuss with your doctor how you can best fit these vitamins into your cystic fibrosis-friendly diet plan.

Apart from these, you may also need to take supplements that contain protein, B vitamins, and other nutrients. You will have to take these supplements with each meal and they are prescribed by your doctor looking at your individual dietary requirements and your overall health.

Some people suffering from cystic fibrosis also benefit from adding extra calories and nutrients through temporary tube feeding. This, however, is only for patients who are unable to maintain their proper nutrition by eating normally. Tube feeding helps provide you with up to 2,000 calories as you sleep. The temporary feeding tube is put into the stomach and requires a small surgical process to be carried out.

If you have exocrine pancreatic insufficiency (EPI), then you will also need to take extra pancreatic enzymes with your meals and snacks in order to make up for the enzymes that your pancreas is unable to supply.

Conclusion

Following a cystic fibrosis-friendly diet is definitely beneficial and definitely works in patients suffering from this condition. Maintaining a balanced diet when you have cystic fibrosis is difficult and needs a lot of planning and extra work. However, it is important that you meet your nutritional needs as suffering from cystic fibrosis is going to zap you of your energy levels and make you feel even sicker if your nutritional needs are not met. Have your doctor and your healthcare team or dietitian help you decide what foods are good for your condition and what foods you should avoid.

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What is the Effect of Cystic Fibrosis on the Body & Importance of Diet and Nutrition in Cystic Fibrosis ?