Signs and Symptoms
CF is often accompanied by the following signs and symptoms:
- Thick, viscous mucus secretions in the lungs
- Changes in color and amount of material coughed up from the lungs called sputum
- Chronic cough, possibly with blood streaking
- Chronic sinusitis
- Nasal polyps (fleshy growths inside the nose)
- Weight loss, failure to thrive in infants, abdominal swelling
- Excessive salt in sweat, dehydration
- Failure of newborn to pass stool
- Abdominal pain, flatulence
- Greasy, smelly feces
- Failure to thrive
Conditions such as late onset of puberty, intestinal obstruction, inflammation of the pancreas, cirrhosis (a liver condition), and infertility may also be signs of CF.
What Causes It?
CF is caused by a defective gene that tells the body to produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and the pancreas, the organ that helps break down and absorb food. This collection of sticky mucus results in life-threatening lung infections and serious digestion problems. The disease may also affect the sweat glands and a man's reproductive system.
Who's Most At Risk?
CF is caused by inherited genes. To have CF, a child must inherit two abnormal genes -- one from each parent. People who have lower muscle mass, shorter stature, and a low body mass index are more likely to die from the disease prematurely.
What to Expect at Your Provider's Office
A baby born with the CF gene usually has symptoms during its first year, although signs of the disease may not appear until adolescence or even later.
Your child's health care provider can help make a diagnosis and guide you in determining which treatment or combination of therapies will best alleviate symptoms of the disease. Your provider will perform a physical exam and run laboratory tests, including a sweat test, which checks for higher than normal amounts of sodium and chloride in the sweat. Other tests include a sputum test, genetic screening, and a stool analysis. Imaging techniques may help reveal lung conditions and abdominal obstruction.
Currently, it is not possible to prevent CF.
The hope for the future is that gene therapy could repair or replace the defective CF gene. A person with CF might be given the active form of the protein product that is scarce or missing. For now though, the best health care providers can do is ease the symptoms of CF or slow the progression of the disease to improve patients' quality of life.
CF patients suffer from frequent lung infections caused by obstructed breathing. So, the mainstays of treatment are physical therapy, exercise, and medications for reducing the mucus blocking the lung's airways.
Medications are often inhaled and include the following:
- Bronchodilators. Which widen the breathing tubes.
- Mucolytics. Which thin the mucus.
- Decongestants. Which reduce swelling of the membranes of the breathing tubes.
- An enzyme that thins the mucus. By digesting the cellular material trapped in it.
- Antibiotics. To fight lung infections.
- Nonsteroidal anti-inflammatory drugs. To improve weight gain and reduce declines in lung function.
- Corticosteroids. To reduce inflammation in the airway.
Digestive problems caused by CF are managed with these medications:
- Pancreatic enzymes which help digestion
- Enemas and mucolytic agents to treat intestinal obstructions
Surgical and Other Procedures
CF patients with respiratory failure may need a lung transplant. Patients experiencing gastrointestinal obstruction may also require surgery.
Complementary and Alternative Therapies
A comprehensive treatment plan for CF may include a range of complementary and alternative therapies (CAM). Some supplements and herbs can interfere with certain medications. New research is emerging daily. Always work with knowledgeable providers and make sure to inform your physicians about any and all supplements you are considering using.
Nutrition and Supplements
Following these nutritional tips may help reduce symptoms:
- Eliminate potential food allergens and foods that increase mucous production, including dairy (milk, cheese, sour cream, and ice cream), wheat (gluten), soy, corn, potatoes, cabbage, bananas, sugar, preservatives, food additives, and excessive salt and meats. Your health care provider may want to test for food sensitivities.
- Eat more foods that decrease mucous production, including garlic, onions, watercress, horseradish, mustard, parsley, celery, rose hips tea, pickles, lemon, and anti-inflammatory oils (nuts, seeds, and cold-water fish).
- Eat more foods containing digestive enzymes, such as papaya and pineapple.
- Avoid refined foods, such as white breads, pastas, and sugar.
- Eat fewer red meats and more lean meats, cold-water fish, tofu (soy, if no allergy), or beans for protein.
- Use healthy oils, such as coconut oil.
- Reduce or eliminate trans fatty acids, found in commercially-baked goods, such as cookies, crackers, cakes, French fries, onion rings, donuts, processed foods, and margarine.
- Avoid tobacco.
- Drink 6 to 8 glasses of filtered water daily.
- Exercise moderately, for 30 minutes daily, 5 days a week.
You may address nutritional deficiencies with the following supplements:
- Omega-3 fatty acids. Such as fish oil, to reduce inflammation and improve immunity. Fish oils may increase bleeding in sensitive individuals, such as those taking blood-thinning medications (including aspirin).
- A multivitamin daily. Containing the antioxidant vitamins A, C, D, E, K, the B-vitamins and trace minerals, such as magnesium, calcium, zinc, and selenium.
- Digestive enzymes, with meals. Your physician may already be prescribing a digestive enzyme, or something similar, for you -- make sure to speak with your physician before taking any digestive enzymes.
- Coenzyme Q10 (CoQ10). For antioxidant and immune activity. Speak with your physician, particularly if you are on blood pressure medication or blood-thinning medication, such as CoQ10 can potentially interfere with its actions.
- N-acetyl cysteine (NAC). For antioxidant effects. Alternative health care practitioners may use higher dosages. Check with your health care provider. Your doctor may already be prescribing something like NAC for you. NAC can interact with nitroglycerin, and potentially other medications.
- Probiotic supplement (containing Lactobacillus acidophilus). For maintenance of gastrointestinal and immune health. Some probiotic supplements may need refrigeration. Check the label carefully. People who are severely immunocompromised should check with their physicians before taking probiotics.
- Methylsulfonylmethane (MSM). To help reduce inflammation.
- Whey protein. For support of immunity and weight gain, when needed. Whey protein can interact with some medications, particularly Levodopa. People with dairy intolerance should substitute with other protein supplements.
Herbs are a way to strengthen and tone the body's systems. As with any therapy, you should speak with your provider before starting any treatment. You may use herbs as dried extracts (capsules, powders, or teas), glycerites (glycerine extracts), or tinctures (alcohol extracts). Unless otherwise indicated, make teas with 1 tsp. (5 g) herb per cup of hot water. Steep covered 5 to 10 minutes for leaf or flowers, and 10 to 20 minutes for roots. Drink 2 to 4 cups per day. You may use tinctures alone or in combination as noted.
- Green tea (Camellia sinensis). Standardized extract, for antioxidant and immune effects. You may also prepare teas from the leaf of this herb.
- Cat's claw (Uncaria tomentosa). Standardized extract, for inflammation, immune and antibacterial or antifungal activity. Cat's claw may interact with certain medications, including blood pressure medications. Cat's claw may worsen autoimmune disorders and Leukemia.
- Milk thistle (Silybum marianum). Seed standardized extract, for detoxification support. Milk thistle may have an estrogen-like effect, so people who have a history of hormone-related cancers should use milk thistle with caution. Milk thistle is in the same family as ragweed and may cause allergic reactions in people who are sensitive to ragweed. Since milk thistle works on the liver, it may interact with medications. Speak with your physician.
- Bromelain (Ananus comosus). Standardized extract, for pain and inflammation. Bromelain may increase bleeding in sensitive individuals, such as those taking blood-thinning medications, including aspirin. Bromelain may also impact how your body metabolizes antibiotics. Your physician may already be prescribing something like bromelain, so check with your provider before taking a supplement.
- Ground Ivy (Hedera helix). Standardized extract, to reduce mucous production and to loosen phlegm. Ground ivy can be particularly toxic to the liver and kidneys. People who have a history of seizure disorders should avoid ground ivy. You should only take ground ivy under the supervision of a trained herbalist who is working with your physician.
Although few studies have examined the effectiveness of specific homeopathic therapies, professional homeopaths may consider the following treatments to alleviate respiratory symptoms (such as those experienced from CF) based on their knowledge and experience. Before prescribing a remedy, homeopaths take into account a person's constitutional type, includes your physical, emotional, and psychological makeup. An experienced homeopath assesses all of these factors when determining the most appropriate treatment for each individual.
Use the following treatments under the guidance of a licensed, certified homeopath, in addition to standard medical care provided by a medical doctor:
- Antimonium tartaricum. For wet, rattling cough (although the cough is usually too weak to bring up mucus material from the lungs) that is accompanied by extreme fatigue and breathing problems. Symptoms usually worsen when the person is lying down.
- Carbo vegetabilis. For shortness of breath with anxiety, chills, and bluish skin discoloration.
Acupuncture may alleviate symptoms of cystic fibrosis. Acupuncture may help enhance immune function, normalize digestion, and strengthen respiratory function.
Therapeutic massage can help drain mucus from the lungs.
Respiratory problems are the most common complication from CF.
CF patients receive pulmonary function tests every 3 to 6 months. They also receive chest x-rays every 2 to 4 years, or more often if needed.
Adde FV, Rodrizues JC, Cardoso AL. Nutritional follow-up of cystic fibrosis patients: the role of nutrition education. J Pediatr (Rio J). 2004;80(6):475-482.
Andoh-Duku A, Rafeq S. Cystic fibrosis. In: Ferri FF, ed. Ferri's Clinical Advisior 2017. 1st ed. Philadelphia, PA: Elsevier; 2017:339-340.e2.
Beckles Willson N, Elliot TM, Everard ML. Omega-3 fatty acids (from fish oils) for cystic fibrosis. Cochrane Database Syst Rev. 2002;(3):CD002201.
Bruzzese E, Raia V, Gaudiello G, et al. Intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration. Aliment Pharmacol Ther. 2004;20(7):813-819.
Cabrera C, Artacho R, Gimenez R. Beneficial effects of green tea -- a review. J Am Coll Nutr. 2006;25(2):79-99.
Caramia G, Cocchi M, Garliardini R, et al. Fatty acids composition of plasma phospholipids and triglycerides in children with cystic fibrosis. The effect of dietary supplementation with an olive and soybean oils mixture. Pediatr Med Chir. 2003;25(1):42-49.
Chin J. Intestinal microflora: negotiating health outcomes with the warring community within us. Asia Pac J Clin Nutr. 2004;13(Suppl):S24-S25.
Cvetnic Z, Vladimir-Knezevic S. Antimicrobial activity of grapefruit seed and pulp ethanolic extract. Acta Pharm. 2004;54(3):243-250.
Doron S, Gorbach SL. Probiotics: their role in the treatment and prevention of disease. Expert Rev Anti Infect Ther. 2006;4(2):261-275.
Egan ME, Green DM, Voynow VA. Cystic fibrosis. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 403.
Farrell P, Rosenstein B, White T, et al. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. Journal of Pediatrics. 2008;153(2):S4-S14.
Fogarty AW, Britton J, Clayton A, Smyth AR. Are measures of body habitus associated with mortality in cystic fibrosis? Chest. 2012;142(3):712-717.
Galli F, Battistoni A, Gambari R, et al. Oxidative stress and antioxidant therapy in cystic fibrosis. Biochim Biophys Acta. 2012;1822(5):690-713.
Giusti R. Cystic fibrosis. In: Bope ET, Kellerman RD, eds. Conn's Current Therapy 2016. 1st ed. Philadelphia, PA: Elsevier; 2016:392-395.
Gonclaves C, Dinis T, Batista MT. Antioxidant properties of proanthocyanidins of Uncaria tomentosa bark decoction: a mechanism for anti-inflammatory activity. Phytochemistry. 2005;66(1):89-98.
Grey V, Mohammed SR, Smountas AA, et al. Improved glutathione status in young adult patients with cystic fibrosis supplemented with whey protein. J Cyst Fibros. 2003;2(4):195-198.
Gruber W, Orenstein DM, Braumann KM, Paul K, Huls G. Effects of an exercise program in children with cystic fibrosis: are there differences between females and males? J Pediatr. 2011;158(1):71-76.
Guo R, Pittler MH, Ernst E. Herbal medicines for the treatment of COPD: a systematic review. Eur Respir J. 2006;28(2):330-338.
Hale LP, Greer PK, Trinh CT, James CL. Proteinase activity and stability of natural bromelain preparations. Int Immunopharmacol. 2005;5(4):783-793.
Heggers JP, Cottingham J, Gussman J, et al. The effectiveness of processed grapefruit-seed extract as an antibacterial agent: II. Mechanism of action and in vitro toxicity. J Altern Complement Med. 2002;8(3):333-340.
Huang SH, Schall JI, Zemel BS, Stallings VA. Vitamin E status in children with cystic fibrosis and pancreatic insufficiency. J Pediatr. 2006;148(4):556-559.
Infante P, Redecillas F, Torrent V, et al. Improvement of intestinal function in cystic fibrosis patients using probiotics. An Pediatr. 2008;69(6):501-505.
Irons JY, Petocz P, Kenny DT, Chang AB. Singing as an adjunct therapy for children and adults with cystic fibrosis. Cochrane Database Syst Rev. 2016;9:CD008036.
Jagannath VA, Fedorowicz Z, Thaker V, Chang AB. Vitamin K supplementation for cystic fibrosis. Cochrane Database Syst Rev. 2015;1:CD008482.
Jonsdottir B, Bergsteinsson H, Baldursson O. Cystic fibrosis -- review. Laeknabladid. 2008;94(12):831-837.
Kormosh N, Laktionov K, Antoshechkina M. Effect of a combination of extract from several plants on cell-mediated and humoral immunity of patients with advanced ovarian cancer. Phytother Res. 2006;20(5):424-425.
Lobo J, Rojas-Balcazar J, Noone P. Recent advances in cystic fibrosis. Clin Chest Med. 2012;33(2):307-328.
McCabe H. Riboflavin deficiency in cystic fibrosis: three case reports. J Hum Nutr Diet. 2001;14(5):365-370.
Mizejewski GJ, Pass KA. Fatty acids, alpha-fetoprotein, and cystic fibrosis. Pediatrics. 2001;108(6):1370-1373.
Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Sys Rev. 2013;4:CD002769.
Murray KL, Lee CK, Mogayzel PJ Jr, Zeitlin PL, Rosenstein BJ. Dietary supplement use in pediatric patients with cystic fibrosis. Am J Health Syst Pharm. 2008;65(6):562-565.
Nick JA, Chacon CS, Brayshaw SJ, et al. Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosis. Am J respir Crit Care Med. 2010;182(5):614-626.
O'Connor OJ, Vandeleur M, McGarrigle AM, et al. Development of low-dose protocols for thin-section CT assessment of cystic fibrosis in pediatric patients. Radiology. 2010;257(3):820-829.
Olveira G, Olveira C. Nutrition, cystic fibrosis and the digestive tract. Nutr Hosp. 2008;23(2):71-86.
Proesmans M, Vermeulen F, De Boeck K. What's new in cystic fibrosis? From treating symptoms to correction of the basic defect. Eur J Pediatr. 2008;167(8):839-849.
Radtke T, Nolan SJ, Hebestreit H, Kriemler S. Physical exercise training for cystic fibrosis. Cochrane Database Syst Rev. 2015; (6):CD002768.
Rubin BK. The pharmacologic approach to airway clearance: Mucoactive agents. Paediatr Respir Rev. 2006;7(Suppl 1):S215-S219.
Simopoulos AP. Omega-3 fatty acids in inflammation and autoimmune diseases. J Am Coll Nutr. 2002;21(6):495-505.
Thornton J, Rangaraj S. Anti-inflammatory drugs and analgesics for managing symptoms in people with cystic fibrosis-related arthritis. Cochrane Database Syst Rev. 2016;1:CD006838.
Tonelli AR, Fernandez-Bussy S, Lodhi S, et al. Prevalence of pulmonary hypertension in end-stage cystic fibrosis and correlation with survival. J Heart Lung Transplant. 2010;29(8):865-872.
Yoon JH, Baek SJ. Molecular targets of dietary polyphenols with anti-inflammatory properties. Yonsei Med J. 2005;46(5):585-596.