Why People with Cystic Fibrosis Need Extra Salt

Why People with Cystic Fibrosis Need Extra Salt

Cystic fibrosis (CF) is an extremely rare genetic disorder that affects about 70,000 people worldwide, with more than 30,000 of them in the United States. Only about 1,000 people are diagnosed with this disease each year in the country.

Due to this rarity, many people are relatively unaware of the symptoms of this life-threatening disease, including its impact on the body and how to manage its effects. For families with children born with this condition, getting familiar with it can be challenging, especially as they have to do so while dealing with the reality of their child’s illness.

What is Cystic Fibrosis?

As mentioned above, CF is a genetic disease and is caused due to a mutation in a gene known as the cystic fibrosis transmembrane conductance regulator (CFTR) gene.  

When mutated, this gene changes a protein that regulates the movement of salt in and out of the body’s cells. This, in turn, cause mucus to build up throughout the body, including in the reproductive, digestive, and respiratory systems. Another side-effect is excessively salty sweat, which can cause its own problems.

CF is an inherited disease, and if you have family members with the illness, you and your children are at a greater risk for it. Additionally, it is most common in white people with Northern European ancestry, though it occurs around the world and in people of all races.

Most people with CF are diagnosed either at birth or in early childhood. In the U.S., all newborns are routinely tested for the disease so that treatment can begin as soon as possible. Testing at birth includes a blood test, a sweat test, and genetic tests if necessary.

If a child isn’t screened at birth but shows symptoms later in life, doctors may suggest testing them for CF. Testing methods include genetic and sweat tests.


Symptoms of Cystic Fibrosis

There are numerous symptoms of CF, each of which may differ in severity from person to person. They include:

  • Blocked lungs due to a mucus build-up in the lungs. Furthermore, the mucus can still trap bacteria, which are then trapped in the lungs alongside it. This can lead to numerous serious infections, including ABPA.
  • Other respiratory difficulties, including damaged airways, nasal polyps, pneumothorax (collapsed lungs), coughing up blood, and even respiratory failure. Respiratory symptoms tend to worsen over time.
  • Digestive issues due to problems with the pancreas. The pancreas produces enzymes to help transport digestive juices to the small intestine, a crucial part of the digestive process. However, in patients with CF, the excess mucus their body produces blocks the path between the pancreas and the intestine. This results in difficulty in digesting food, which can cause malnutrition if their diet is not monitored correctly. Furthermore, as the pancreas also controls blood sugar levels, CF can result in patients developing type-1 diabetes. Other digestive problems, including liver disease, intestinal obstructions, and distal intestinal obstruction syndrome (DIOS).
  • Reproductive issues in both men and women – women experience reduced fertility, while most men with CF are infertile due to mucus blocking parts of their reproductive systems.
  • Salty sweat that causes electrolyte imbalances and dehydration. As mentioned above, the cause of CF is a mutation of the CFTR gene, which regulates the movement of salt through the cells of your body.

For all people, regardless of CF status, salt is carried to the skin when you sweat. However, in healthy people, this salt is reabsorbed into your body. Due to the mutation of the CFTR gene, people with CF cannot reabsorb the salt, and the salt (in the form of chloride) remains on the skin.

Salty sweat is such a hallmark of CF that checking for chloride levels in sweat is one of the best ways of diagnosing CF in infants. In fact, even going back to medieval Europe, salty sweat was considered a hallmark of severe illness in a child. Though CF had yet to be discovered, folk wisdom warned parents, “Woe is the child who tastes salty from a kiss on the brow, for he is cursed, and soon must die.” Texts dating back to 1595 link salty sweat to damage of the pancreas and witchcraft, and infants born with salty sweat were considered hexed.


Salt Intake and Cystic Fibrosis

The extra salt lost through sweat means that CF patients are constantly at risk of unbalanced electrolyte levels, especially during periods of excess sweating, like hot weather, exercise, and illnesses. 

Due to low salt levels, the body is unable to recognize when it needs water. This puts CF patients at risk of dehydration, as well as other conditions, including fatigue and weakness, muscle cramps, vomiting, and heatstroke. 

One of the most effective ways to combat the challenges of CF is by making dietary changes. Your doctor will be able to recommend a diet plan tailored to your specific needs. However, in general, some common tips include:

  • Consuming additional calories.
  • Increasing fluid intake, especially before, during, and after exercise. 
  • Tube feeding for children struggling to meet their calorie goals.
  • Increasing intake of key vitamins and minerals, including zinc, iron, vitamin D, calcium, vitamin A, vitamin K, and vitamin E.
  • Increasing salt intake.

The last point is one of the most important parts of any CF patient’s diet. All CF patients need a high salt diet due to the amount of salt they lose through their sweat glands. The exact amount varies from patient to patient, and your healthcare provider will be able to provide you with an exact calculation.

Your salt intake should be increased further before exercising, during hot weather, and when you fall ill. Additionally, many patients with feeding tubes are provided with formulas that are low on sodium, so you must take special care if you are one of them.

For parents of infants with CF, sodium intake must be monitored carefully. Because young children grow quickly, they need more salt than older children and adults. This salt will need to be added to their formula or baby food.

For older people who need to boost their salt intake, there are numerous options available to you, including:

  • Adding extra salt to your meals
  • Snacking on salty snacks, such as jerky, chips, and popcorn
  • Adding salty broths to your meal-plan
  • Consuming sports drinks high in electrolytes

However, regulating your salt intake along with an increased caloric intake can prove a challenge. For people looking for easier ways to add salt to their diet, supplements like salt tablets and Klaralyte can come in handy.

Klaralyte has been specially formulated to provide a balance of sodium and potassium, ensuring you boost your salt intake while keeping the rest of your electrolytes in balance. Each tablet contains 250mg of sodium and 50mg of potassium and offers an easy way to boost your salt intake without radically changing your diet or forcing yourself to eat more food than you feel like.


Other Sources:

You will not be disappointed!

Additional Information

*Not Evaluated by FDA: These statements have not been evaluated by the Food and Drug Administration. This product is not intended to diagnose, cure, or prevent any disease. Klaralyte LLC manufactures dietary supplements and medical food products that should be used under the direct supervision of a licensed healthcare practitioner.

Designated Medical Food: Klaralyte Salt Capsules are classified as a medical food under 21 U.S.C. 360ee(b)(3), and are intended for specific dietary management based on recognized scientific principles, as evaluated by a physician.

Comparative Advertising: Klaralyte is a registered trademark of Klaralyte LLC. Registered trademarks, brand names, images, or any information that could refer to another brand are used solely for lawful comparative advertising. This follows FTC-defined criteria for objectively comparing brands based on attributes or price, while clearly identifying the alternative brand.