Cystic Fibrosis 

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Bronchiectasis in Cystic Fibrosis. Courtesy Dr Jerry Nick National Jewish Medical and Research Center, Denver

Lung Tissue in Bronchiectasis.  Courtesy Dr Jerry Nick National Jewish Medical and Research Center, Denver

What Is Cystic Fibrosis?

Cystic fibrosis is a genetic disease of the exocrine glands which causes the body to produce abnormally thick and sticky mucus and results in progressive damage to the respiratory system and digestive problems.

Severity of disease depends on the particular genetic mutation of affected individuals.

Q: How does the abnormal mucus damage the lungs and digestive tract?

A: In all cases of cystic fibrosis, sticky mucus build-up in the bronchioles (small breathing passages) of the lungs causes COPD (chronic obstructive pulmonary disease) that results in life-threatening lung infections. 

In the pancreas, the abnormal mucus damages its ability to produce required enzymes to digest food, causing failure to adequately digest fats, carbohydrates and protein which are vital for life.

Other tissues that may be involved are sweat glands, sinuses, and the male reproductive system, causing infertility.

Millions of Americans carry the defective cystic fibrosis gene, but do not have any symptoms. That is because cystic fibrosis requires the inheritance of two defective cystic fibrosis genes — one from each parent who carries it. An estimated 1 in 29 Caucasian Americans have the cystic fibrosis gene.

The disease is the most common, deadly, inherited disorder affecting Caucasians in the United States. It is more common among those of Northern or Central European descent. About 30,000 children and adults in the United States have cystic fibrosis while more than 10 million people carry the gene.1

There is no cure yet for cystic fibrosis, however, treatments have greatly improved in recent years. The goals of treatment include:

  • Preventing and controlling lung infections.
  • Loosening and removing thick, sticky mucus from the lungs.
  • Preventing or treating blockages in the intestines.
  • Providing enough nutrition.
  • Preventing dehydration (a lack of fluid in the body).2

What Is Cystic Fibrosis In Celiac Disease and/or Gluten Sensitivity?

  • Cystic fibrosis is a severe genetic disorder associated with celiac disease and may be a presenting feature of untreated celiac disease.
  • A study by Walkowiak et al. aiming to assess the genetic predisposition to celiac disease and its incidence in patients with cystic fibrosis comparing it to data from the general population found that genetic predisposition to celiac disease in cystic fibrosis patients is similar to that of the general population. However, results suggest that cystic fibrosis is a risk factor for celiac disease development, showing a prevalence of 2.13%.3

How Prevalent Is Cystic Fibrosis In Celiac Disease and/or Gluten Sensitivity?

Cystic fibrosis association with celiac disease is classic.4

Positive anti-endomysium antibodies were found in 2.84% of the 282 studied patients with cystic fibrosis. The incidence of proven celiac disease was 2.13%. The incidence of celiac disease as well as positive serological screening were significantly more frequent in the cystic fibrosis group than in the general population. The frequency of celiac disease-related HLA-DQ alleles in patients with cystic fibrosis and healthy controls did not differ.3

What Are The Symptoms Of Cystic Fibrosis?

Cystic fibrosis is marked by these general symptoms:

  • Fatigue.
  • Muscle weakness.
  • Difficulty breathing complicated by bronchiectasis, bronchitis, pneumonia.
  • Salt depletion through sweat.
  • Malabsorption of fats, proteins, and carbohydrates.
  • Nutritional deficiencies in all ages.
  • Steatorrhea (bulky pale stool) due to fat malabsorption.
  • Intestinal gas.
  • Abdominal distention (swollen belly).
  • Severe constipation.
  • Death is usually the result of respiratory failure.

Below is a breakdown adapted from an article by Dr. Jerry Nick National Jewish Medical and Research Center, Denver.5 

  • Newborns and Infants.
  • Obstruction of the bowel at birth (meconium ilius).
  • Poor growth and weight gain, despite a good appetite.
  • Frequent greasy, bulky stools, or difficulty in bowel movements.
  • Very salty-tasting skin. Cough and other respiratory symptoms.
  •  Children.
  • Persistent coughing, at times productive with sputum.
  • Frequent respiratory infections.
  • Wheezing or shortness of breath.
  • Poor growth and difficulty with weight gain (failure to thrive).
  • Neurologic syndrome of vitamin E deficiency including ataxia with loss of deep tendon reflexes, loss of vibration and position sense, ophthalmoplegia, muscle weakness, ptosis, and dysarthria.  
  • Adolescents and Young Adults.
  • Recurrent or persistent lung infections with Staphylococcus aureus or  Pseudomonas aeruginosa.
  • Chronic sinusitis, sinus infections and nasal polyps.
  • Clubbing of the fingers and toes.
  • Male infertility with an absence of sperm.
  • Delayed puberty.

How Does Cystic Fibrosis Develop In Celiac Disease and/or Gluten Sensitivity?

  • Cystic fibrosis is caused by gene mutation on chromosome 7.

Does Cystic Fibrosis Respond To Gluten-Free Diet?

Yes. Celiac disease-related cystic fibrosis improves on a gluten free diet.6

6 Steps To Improve Cystic Fibrosis In Celiac Disease and/or Gluten Sensitivity:

Treatment. This condition responds to the complete elimination of gluten, which is the required treatment that improves both cystic fibrosis and gut health.

  • Gut health is the foundation to restore ALL health. Restored health will enable you to maintain a strict gluten free diet, just as other life tasks will be easier.
  • A strict gluten free diet means removing 100% of wheat, barley, rye and oats from the diet.
  • Cutting out bread and other obvious sources of gluten is not good enough for recovery. Even 1/8th teaspoon of flour or bread crumb is enough to sustain the inflammation that is damaging your small intestine, causing increased permeability (leaky gut) and allowing undigested gluten to enter your body where it can damage structures and function, and instigate immune inflammatory responses.

Correct Your Individual Nutritional Needs.

Recovery. You should begin to feel better within a week and notice more energy as inflammation subsides and the  absorbing cells that make up the surface lining of your small intestine are better able to function.

  • Intestinal lining cells are replaced every 5 days. The healing process is like sunburn where the damaged surface layer of skin sloughs off and is replaced with new normal cells.
  • Leaky gut normally resolves in two month after starting a gluten free diet and brings about a big improvement in health. Improvement in intestinal permeability precedes morphometric recovery (cell appearance and structure) of the small intestine in celiac disease.7
  • The intestinal lining may take up to a year to heal.
  • 2 Reduce Inflammation. Foods to Eat and Foods Not to Eat:

Because gluten is inflammatory, eliminate OTHER inflammatory foods from your diet to reduce an additive effect to gluten. At the same time, try to eat foods that reduce inflammation (anti-inflammatory).

Here Are Major Inflammatory Food Types That Reduce Healing:

  • Damaging Foods. In susceptible persons, includes corn, dairy (cow), and soy. Lactose, the sugar in any animal milk disrupts intestinal permeability causing leaky gut.8
  • Allergenic Foods. Includes foods that trigger the immune sytem to produce IgE antibodies. Allergy testing is the usual way to discover these offending foods.
  • Shelf Stable Processed Foods. Includes any that contain additives and preservatives. Look for them on the nutrition label of the box or package. Additives and preservatives also disrupt intestinal permeability causing leaky gut.8
  • Fats. Limit deep fried foods, trans-fats, saturated fats (animal fat/butter), and EXCESSIVE omega-6 fatty acid oils like corn oil. Rancid fats, sodium caprate (a medium chain fat), and sucrose monester fatty acid (a food grade surfactant) induce significant disruption of the intestinal barrier that causes leaky gut.8.
  • Excessive Refined White Flours (bran layer removed)Includes products made from them such as cookies, bread, cakes, pies. Bran contains the vitamins and minerals that metabolize grains and slows the otherwise rapid entry of sugar from their digestion into the bloodstream. Also disrupt intestinal permeability causing leaky gut.8
  • Refined Sugars.  Includes white sugar, corn fructose and high fructose corn syrup.
  • Certain Spices. Includes paprika and cayenne pepper which disrupt intestinal permeability causing leaky gut.8
  • Alcohol and Caffeine. Disrupt intestinal permeability causing leaky gut.8

Here Are Important Anti-Inflammatory Food Types to Promote Health:

  • Fruits. Contain ample amounts of vitamins, minerals and phytochemicals which are naturally occuring components in plants that detoxify toxins, carcinogens (reducing the risk by 50%) and mutagens.
  • Non-Starchy Vegetables. Support intestinal integrity and provide ample amounts of vitamins, minerals and phytochemicals. Includes green leafy vegetables such as lettuce and kale, also onion, broccoli, garlic, and others.
  • High Quality Complex Carbohydrates. Provide vitamins, minerals, and fiber while boosting serotonin levels to help you relax and feel calm. Includes whole grains, legumes, and root vegetables such as carrots, parsnips, sweet potatoes, turnips, red beets, and others.
  • Antioxidants. Protect the body from inflammatory oxidant molecules that continually occur and help us handle stress and reduce irritability. Includes vitamin C-containing foods such as lemon, grapefruit, apricot, Brussels sprouts and strawberries, and others. Also, includes vitamin E-containing foods such as nuts, seeds, avocado, olive oil, and others. Cocoa is good, too.
  • Omega-3 Fatty Acids. Balance opposing omega-6 fatty acids and bad fats. Fish sources includes tuna, salmon, cod, and others. Plants sources include flax, chia seeds, canola oil, and others.
  • Probiotics. Supply normal microbes needed for colon health and health of the body such as these fermented foods: yogurt, kefir, and unpasteurized apple cider vinegar.
  • Prebiotics/ High Fiber Foods.  Food with fiber keeps our population of colonic microbes healthy.
  • Protective Herbs and Spices.  See below #6 below for examples.
  • 3 Information Sheet You Can Take to Your Doctor or Other Health Professional:

Click here.

  • 4 Manage Your Medications Safely:

Medicines that are commonly prescribed to help treat or prevent lung infections, reduce swelling and open up the airways, and thin mucus in cystic fibrosis cause nutritional deficiencies that promote complications in celiac disease.

Ask your doctor or pharmacist about this possible adverse effect if you are taking any of the drugs listed below. Do not stop prescribed medications without supervision.

This is not a complete listing.

ANTIBIOTICS to treat infection disrupt intestinal permeability which complicates celiac disease.

ANTI-INFLAMMATORIES disrupt intestinal permeability which complicates celiac disease.

BRONCHODILATORS

  • Inhaled corticosteroid inhalers (Flovent, Pulmicort and others) that are breathed in on a daily basis as a long term therapy to reduce inflammation in airways deplete potassium, calcium, vitamin D, and B vitamins.
  • Albuterol inhalers that are breathed in on a daily basis as a long term therapy and also for quick relief as rescue inhalers to open airways depletes potassium.
  • Theophylline by mouth as a long term therapy to open airways depletes potassium.

  • 5Nutritional Supplements To Help Correct Deficiencies:

The type and quantity of nutritional supplements that may be needed depend on which nutrients are deficient.

  • Multivitamin/mineral combination that provides 100% once a day is useful to improve overall nutrient levels. This is a safe dose, but always check with your doctor to avoid interactions with medications.
  • Oral pancreatic enzymes to help you digest fats and proteins and absorb more vitamins.
  • Supplements of vitamins A, D, E, and K as prescribed following blood test for status to replace the fat-soluble vitamins that your intestines cannot absorb.
  • Calcium citrate as prescribed following blood test for status to promote bone health. Calcium carbonate is a poor choice.
  • Vitamin D3 as prescribed following blood test for status to promote bone health.
  • Chelated magnesium  as prescribed but do not take at same time as calcium because they compete for absorption.
  • Potassium must not be taken as a supplement except as prescribed by a doctor based on blood testing for status.

Storage NoteStore container tightly sealed, away from heat, moisture and direct light to avoid loss of potency. That is, in a safe kitchen cabinet – not in the bathroom or on the kitchen table.

  • 6Manage Natural Remedies: 

Hydration:

  • Eight glasses of water are recommended per day unless there is a contraindication such as kidney or heart disease. The Institute of Medicine recommends approximately 2.7 liters (91 ounces) of total water, from all beverages and foods, each day for women and 3.7 liters (125 ounces) daily of total water for men.
  • If you are thirsty, drink water. Add fresh, squeezed lemon to water. Lemon is anti-inflammatory, alkalizing and provides vitamin C.
  • Hydration Test: Urine should be pale yellow. Fingertips should be plump, without pruning but this may not be reliable when fingers are swollen with edema. Lips should be plump, without puckering. The feeling of thirst can be unreliable.
  • What is wrong with soda, coffee, tea, and alcohol? These drinks are dehydrating, increase acid, and deplete nutrients.

Carminatives. The following  anti-inflammatory plant sources called carminitives help heal the digestive tract. They also tone the digestive muscles which improves peristalsis, thus aiding in the expulsion of gas from the stomach and intestine to relieve digestive colic and gastric discomfort.

Carminative Food Remedies:

  • Raspberry.
  • Carrot is also a cleansing digestive tonic.
  • Grape is also bile stimulating and a cleansing remedy for sluggish digestion and laxative.
  • Redbeets also stimulate and improve digestion and are easily digested.
  • Cabbage also stimulates and improves digestion and is also a liver decongestant.
  • Lettuce also stimulates and improves digestion and is also an alterative, meaning it improves the function of organs involved with the digestion and excretion of waste products to bring about a gradual change.
  • Potatoes are antispasmodic (due to atropine like properties) and a liver remedy.

Carminative Herb Remedies:

  • Sage is also a digestive, astringent, bile stimulant and energy tonic that heals the mucosa.  Drink as tea or use in cooking.
  • Chamomile, lemon balm, and fennel, (as a tea) also help relieve nervous tension.
  • Parsley also relieves indigestion.
  • Rosemary as a tea and in cooking also is a nervous system tonic for stress and fatigue, bile stimulant, and can relieve headaches and indigestion.
  • Thyme is also soothing remedy useful for stimulating digestion of rich, fatty foods.

Carminative Spice Remedies:

  • Cloves are also antispasmodic.
  • Nutmeg is also useful for indigestion.
  • Ginger.

Exercise Helps:

Exercise improves circulation and rids the body of toxins.

  • Walking is aerobic exercise that reconditions the whole body to improve stamina.
  • Aerobic exercise that makes you breathe harder can help loosen the mucus in your airways so you can cough it up. However, cystic fibrosis causes sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. Thus, your doctor may recommend a high-salt diet or salt supplements to maintain the balance of minerals in your blood.9.Read more about Exercise and Fitness.
  • Weight training builds muscle. Read more about Exercise and Fitness.
  • Stretching improves flexibilty. Read more about Exercise and Fitness.

Note: Exercise is important, but the amount and type of exercise undertaken depends on your health. Your first priority is to heal.

What Do Medical Research Studies Tell About Cystic Fibrosis n Celiac Disease?

RESEARCH STUDY SUMMARIES

Cystic fibrosis is a risk factor for celiac disease.” This study aimed to assess the genetic predisposition to celiac disease and its incidence in patients with cystic fibrosis comparing it to data from the general population found that genetic predisposition to celiac disease in cystic fibrosis patients is similar to that of the general population. However, results suggest that cystic fibrosis is a risk factor for celiac disease development.

Two hundred eighty-two with cystic fibrosis were enrolled in the study. In 230 with cystic fibrosis the genetic predisposition to celiac disease (the presence of HLA-DQ2/ DQ8) was assessed. In all patients with cystic fibrosis, serological screening for celiac disease was conducted. In patients with positive antiendomysial antibodies (EMA) gastroduenoscopy was offered. Intestinal histology was classified according to modified Marsh criteria. The results of serological celiac disease screening in 3235 Polish schoolchildren and HLA-DQ typing in 200 healthy subjects were used for comparison.

RESULTS: Positive EMA was found in 2.84% of the studied patients with cystic fibrosis. The incidence of proven celiac disease was 2.13%. The incidence of celiac disease as well as positive serological screening were significantly more frequent in the cystic fibrosis group than in the general population. The frequency of celiac disease-related HLA-DQ alleles in patients with cystic fibrosis and healthy controls did not differ.10

CASE REPORT SUMMARIES

“Coagulopathy as initial manifestation of concomitant celiac disease and cystic fibrosis: a case report.” This case report describes diagnosis of a five-year-old boy of Turkish ethnicity with both celiac disease and cystic fibrosis, who presented initially with a skin hemorrhage. The diagnosis of celiac disease was made with a positive serum anti-tissue transglutaminase antibody test and the presence of HLA-DQ2 heterodimer, and confirmed on histology with small intestinal villous atrophy. A positive sweat test confirmed the diagnosis of associated cystic fibrosis. The clinical significance of this case is the consideration of malabsorption with both celiac disease and cystic fibrosis in patients who present with unexplained coagulopathy.11

“Diagnosis of cystic fibrosis and celiac disease in an adult: one patient, two diseases, and three reminders.” This case report describes the uncommon occurrence of celiac disease and cystic fibrosis in an obese adult patient. Apart from its rarity, the case serves to highlight the elusive nature of these two disease when presenting with atypical features in an adult.12

“Celiac disease, cystic fibrosis and dilated cardiomyopathy.” This case report describes the investigation and subsequent diagnosis of celiac disease and cystic fibrosis in an 8 year old girl with a history of steatorrhea, abdominal distention, and abundant and fetid stools with frequent cough and respiratory infections, referred to cardiology for congestive cardiac insufficiency. Cystic fibrosis is a common cause of cardiopathy but this patient had idiopathic cardiomyopathy associated with celiac disease, which is the reason why this situation must be investigated through anti-endomysium and anti-tranglutaminase antibodies and intestinal biopsy.4

  1. www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001167/ []
  2. National Heart, Blood, and Lung Institute []
  3. Walkowiak J, Blask-Osipa A, Lisowska A, Oralewska B, Pogorzelski A, Cichy W, Sapiejka E, Kowalska M, Korzon M, Szaflarska-Popławska A. Cystic fibrosis is a risk factor for celiac disease. Acta Biochim Pol. 2010;57(1):115-8. Epub 2010 Mar 20. [] []
  4. Lima Rm, Rocha C, Alvares S, Rocha A, Senra V, Rocha H. Celiac disease, cystic fibrosis and dilated cardiomyopathy. Anales de Pediatria : Publacacion Oficial de la Asociacion Espanola de Pediatria. Aug 2004;61(2)193-4. [] []
  5. http://health-fts.blogspot.com/2012/01/cystic-fibrosis.html []
  6. Lima Rm, Rocha C, Alvares S, Rocha A, Senra V, Rocha H. Celiac disease, cystic fibrosis and dilated cardiomyopathy. Anales de Pediatria : Publacacion Oficial de la Asociacion Espanola de Pediatria. Aug 2004;61(2)193-4. []
  7. Cummins AG, Thompson FM, Butler RN, et al. Improvement in intestinal permeability precedes morphometric recovery of the small intestine in coeliac disease. Clinical Science. Apr 2001;100(4):379-86. []
  8. Farhadi A, Banan A, Fields J, Keshavarzian A. Intestinal barrier: an interface between health and disease. Journal of Gastroenterology and Hepatology. 2003;18:479-91. [] [] [] [] [] []
  9. National Heart, Lung, and Blood Institute []
  10. Walkowiak J, Blask-Osipa A, Lisowska A, Oralewska B, Pogorzelski A, Cichy W, Sapiejka E, Kowalska M, Korzon M, Szaflarska-Popławska A. Cystic fibrosis is a risk factor for celiac disease. Acta Biochim Pol. 2010;57(1):115-8. []
  11. Kostovski A1, Zdraveska N. Coagulopathy as initial manifestation of concomitant celiac disease and cystic fibrosis: a case report. J Med Case Rep. 2011 Mar 24;5:116. doi: 10.1186/1752-1947-5-116. []
  12. Rabinowitz I. Diagnosis of cystic fibrosis and celiac disease in an adult: one patient, two diseases, and three reminders. Respiratory Care. May 2005;50(5):644-5. []

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