If I told you there’s a disease that about 12 million Americans currently carry the gene for and that this same disease affects more than 30,000 children and young adults in the U.S., you’d want to know about it, right? Of course you would, and that’s why you need to know about cystic fibrosis.
Cystic fibrosis (CF) is an inherited disorder that disrupts normal functions of epithelial cells, the cells that line the passageways of many of our most important organs — including the lungs and respiratory system, liver, kidneys, skin, and reproductive system. People with CF have a certain defective gene that impairs epithelial cell functions causing them to experience a buildup of sticky mucus inside the body, often leading to lung damage and chronic coughs. This defect in turn affects how CF patients breath and filter air, digest food, and absorb nutrients in the digestive tract. (1)
Because epithelial cells have many important roles inside the lungs, sweat glands of the skin and the digestive system, CF symptoms can occur throughout the body and become severe. CF is a serious, lifelong disease that needs to be properly managed throughout a patient’s lifetime.
According to the Cystic Fibrosis Foundation, currently CF is considered to be incurable, however certain dietary interventions, supplements, digestive enzymes and lifestyle habits can help manage symptoms. (2) Fortunately, over the past several decades cystic fibrosis treatment approaches have come a long way. Most people with cystic fibrosis now live into their 20s and 30s — and some even longer, into their 40s or 50s. Research shows early diagnoses of cystic fibrosis can help prevent complications and certain developmental problems, helping improve a patient’s quality of life.
Natural Ways to Help Manage Cystic Fibrosis
1. Early Diagnoses and Care
CF is diagnosed most often in unborn babies, newborns or young children by performing genetic tests. Doctors can now test for CF during pregnancy, which is especially common in children who are at a high risk. In newborns or children, a “sweat test” is now the most common means of making a CF diagnosis, which is conducted by testing a sample of sweat from the skin for levels of chloride. Aside from a sweat test, chest X-rays, blood tests, nutritional status tests, bacterial tests and pulmonary function tests to measure breathing can also be performed. (3)
Early diagnoses of cystic fibrosis can help manage symptoms and complications before they worsen, such as dehydration and diarrhea, which can contribute to weight loss. Doctors can help clear the lungs of mucus, helping prevent infections, and get the patient started on a diet that will prevent nutrient deficiencies. Digestive enzymes and supplements can also help with healthy development and weight gain.
2. Dietary Intervention to Prevent Deficiencies
Young CF patients who begin a diet with high-nutrient foods, supplements and digestive enzymes are more likely to have stronger metabolisms, gain weight normally and avoid deadly infections. Diet plays an important role in cystic fibrosis management because lung infections and diseases progress more slowly among children who are growing well and getting enough vitamins, minerals, probiotics and antioxidants.
Many doctors/nutritionists working with CF patients recommend a high-fat, high-calorie diet to reduce the risk for malnutrition, although enzymes are often needed to boost absorption of fat and fat-soluble vitamins. Junk foods and processed foods might be high in calories and fat (especially trans fats or omega-6 fatty acids found in refined vegetable oils, such as safflower, sunflower, corn and sesame), but they’re usually not helpful for lowering inflammation and preventing lung damage, therefore they’re not recommended for people with CF.
Some of the beneficial nutrients people with cystic fibrosis are most likely to be low in include: (4)
- vitamin K
- vitamin D
- vitamin A
- vitamin E
- some people with CF might also need to add more salt to their diets to prevent dehydration
- Omega-3 fatty acids can be beneficial for lowering inflammation (found in wild-caught fish like Alaskan salmon, sardines and halibut)
- pancreatic enzymes are also commonly given to help digest food and calories better
- high-quality multivitamins and antioxidants can help prevent inflammation, susceptibility to infections and lung damage
- probiotic supplements are also helpful for improving digestive functions
Eating anti-inflammatory fats, including monounsaturated fats like extra virgin olive oil, avocado and virgin coconut oil, is a good way to increase calorie intake. Nuts and seeds, such as almonds, chia seeds, flaxseeds, walnuts and hemp seeds, are also good choices for this purpose, as are naturally fattier meats like grass-red beef and lamb. Unrefined carbohydrates can help provide dense calories and energy, especially when combined with fat, but it’s best to eat unprocessed, low glycemic kinds such as ancient grains, starchy veggies and fruit.
3. Help with Proper Lung Function & Breathing
Cystic fibrosis treatments today are generally aimed at improving quality of life, helping boost nutrient intake/absorption, and improving breathing and lung capabilities. The use of non-medication pulmonary therapy treatments to maintain lung function is very common and can be life-saving. Devices such as a bronchodilator can help relax the muscles in the airways of the lungs, help cough up mucus and prevent inflammation from worsening.
Using certain inhalants or bronchodilators can help restore breathing and keep infections away, as can use of devices called oscillatory positive expiratory pressure, which increase airflow to the lungs through use of vibrations that help break mucus. (5, 6) Research shows that approximately 50 percent of patients with CF have some degree of bronchial lability (asthma), which a bronchodilator can improve by increasing clearance of secretions from the chest. Bronchodilators are sometimes used in combination with other therapies, like physical therapy, or anti-mucus medications and salt solutions to expel phlegm and offer even more relief.
4. Physical Therapy and Exercise
Other natural approaches to helping CF patients breath more easily include breathing exercises, physical therapy and cupping therapy. These techniques require no medications or antibiotics and help dislodge mucus in the chest, airways and elsewhere, which limit breathing and decrease quality of life.
Throughout the years, there have been mixed opinions on whether or not physical therapy can be effective for lowering CF symptoms. One meta-analysis published in the American Journal of Respiratory and Critical Care Medicine looked at several different leading approaches of clearing bronchial secretions in the treatment of patients with cystic fibrosis. These approaches included positive expiratory pressure mask, forced expiratory technique, exercise, autogenic drainage, and “standard physical therapy” using vibrations, drainage and percussion. They found that physical therapy worked very well in the majority of patients, especially when used in combination with other approaches like gentle, appropriate exercise.
Over 65 relevant trials and eight review articles were included in the meta-analyses comparing the independent techniques, and it was found that standard physical therapy resulted in a significantly greater sputum expectoration than no treatment or other single treatment. Physical therapy in combination with exercise suited for the patient was associated with a statistically significant increase in quality of life and lower CF symptoms. (7)
Facts About Cystic Fibrosis
- CF affects more than 30,000 children and young adults in the U.S. (8)
- This disease is most common among Caucasians, while Asian Americans are the least likely to have CF. In the U.S. about one of every 3,600 Caucasian children is born with CF (compared to one of every 17,000 African Americans and only one of every 90,000 Asian Americans)
- The defected gene responsible for causing CF is found on “chromosome number 7” of a person’s DNA. It takes two copies of a CF gene (one inherited from each parent) for someone to develop CF. When two CF carriers have a child, there is a one in four chance that the child will have CF.
- CF can also be carried in people who don’t develop the disease if they inherit only one CF gene. The carrier parent can then pass on the disease to their child, and estimates show that about 12 million Americans are currently CF carriers.
- 85 percent of people with CF are diagnosed as infants. Today, earlier detection, including during pregnancy, is helping doctors manage the disease as early as possible.
How Cystic Fibrosis Develops
CF affects the how certain proteins work inside epithelial cells of the mucus glands. Most people think of epithelial cells in terms of the skin — since they do, in fact, help us sweat through so we can regulate our body temperatures. But these important cells also have the responsibility of controlling the way that chloride (part of natural salts) passes across cell membranes in other parts of the body too. Epithelial cells balance the ratio of salt to water, forming important fluids, digestive juices and mucus that help us break down food and prevent viruses or infections. (9)
With CF, thick mucus begins to accumulate and can start to block the airways in the lungs, contributing to reoccurring infections since germs are more likely to get trapped inside the body. In addition to this mucus damaging the lungs and lowering immunity, it’s also capable of affecting the digestive system, especially the pancreas, kidneys, intestines and liver. Mucus can start to slow down production of important digestive enzymes and “juices” that are normally secreted by the pancreas, which leads to trouble properly absorbing nutrients inside the intestines and sometimes unhealthy weight loss.
Cystic Fibrosis Symptoms
People with cystic fibrosis suffer from symptoms including: (10)
- nasal congestion, lung damage, chronic coughs and asthma-like symptoms; coughs that produce thick, heavy, discolored mucus
- repeated sinus and lung infections (such as bronchitis and pneumonia, which is one of the leading causes of a shortened life span)
- trouble breathing or wheezing, even when at rest
- nutrient deficiencies, especially of fat-soluble vitamins (vitamins A, D, E, and K)
- developmental problems in infants and children
- unintentional, sometimes dangerous weight loss
- diarrhea, dehydration and electrolyte imbalance
- oily, bulky stools (caused by undigested fat and protein accumulating in stool)
- digestive problems, such as intestinal gas, swollen belly, chronic diarrhea and abdominal pain
- salty “frosting” on the skin (caused from epithelial cells not being able to balance salt and water)
- chronic fatigue
- long-term complications and illnesses that can develop from CF include: sinusitis, bronchiectasis, pancreatitis, intestinal blockages, nasal blockages, collapsed lungs (pneumothorax), rectal prolapse, gallstones, diabetes and low bone density
Since many cases of cystic fibrosis are diagnosed in infants or very young children, developmental problems and life-threatening infections are some of the biggest concerns. Many newborns born with CF don’t gain weight as expected since mucus prevents pancreatic digestive juices from entering the intestines and breaking down fats and proteins properly. The lungs are especially susceptible to reoccurring infections, such as bronchitis or pneumonia, sometimes which babies or young children aren’t capable of fighting off.
Causes of Cystic Fibrosis
Cystic fibrosis is an inherited disorder, caused by a faulty gene that directs how the body produces a type of protein known as cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is found in the body’s epithelial cells, which are most abundant in the lungs, digestive tract, sweat glands and genitourinary system (the organ system of the reproductive organs and the urinary system).
CFTR proteins help epithelial cells regulate levels of chloride found in salt (sodium chloride), so when they aren’t working normally, the consistency of mucus and other fluid levels becomes altered in a dangerous way. The delicate balance of salt and water that’s needed to maintain a thin coating of mucus inside the organs is shifted, and as a result, a thicker-than-usual mucus forms that heavily coats the inside of the lungs and passageways.
Mucus inside the lungs has the important role of trapping and helping remove germs, such as bacteria, viruses and fungus. In people without cystic fibrosis, mucus is normally thin and fluid-like, which allows it to be easily cleared from the respiratory system, carrying with it germs and waste, which are then cleared out of the lungs. However, when someone has CF, “sticky” mucus stays lodged in the passageways of the lungs, unable to remove potentially dangerous germs.
CF causes the most damage to the lungs but also impairs normal digestion. In the pancreas and intestines, thick mucus can block the way that important enzymes work, changing how food is digested and nutrients absorbed. Many people with CF have a hard time eating and digesting food normally, especially children who are susceptible to slow development, nutrient deficiencies and low weight gain. Even if cystic fibrosis doesn’t change someone’s appetite and food intake, it can still block some nutrients from being absorbed.
Future Care for Cystic Fibrosis
Instead of simply treating the symptoms of CF, new approaches are looking to target the underlying genetic causes. (11) Several new treatments are being developed for CF that offer a lot of hope for the future.
One treatment that has already been used successfully in over 100 CF patients in the U.S. is an inhaled spray containing normal copies of the defective CF gene, which can correct the faulty copy in the lungs and improve how mucus is made. Another similar approach is protein repair therapy, which also corrects how the defective CFTR protein works and lowers accumulation of sticky mucus in the digestive tract and airways.
Cystic Fibrosis Takeaways
- Cystic fibrosis affects more than 30,000 children and young adults in the U.S.
- When two CF carriers have a child, there is a one in four chance that the child will have CF. CF can also be carried in people who don’t develop the disease if they inherit only one CF gene. The carrier parent can then pass on the disease to their child, and estimates show that about 12 million Americans are currently CF carriers.
- Most people with cystic fibrosis now live into their 20s and 30s — and some even longer, into their 40s or 50s. Research shows early diagnoses of cystic fibrosis can help prevent complications and certain developmental problems, helping improve a patient’s quality of life.
- The best ways to naturally manage cystic fibrosis include early diagnosis and care, dietary intervention to prevent deficiencies, help with proper lung function and breathing, and physical therapy and exercise.
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