Cystic Fibrosis

Immune | January 19, 2018 | Author: Naturopath

Immune

Cystic Fibrosis

In Australia, one in 25,000 babies are born with Cystic fibrosis (CF)—making it the most common life-threatening genetic disorder among Caucasians. CF primarily affects the respiratory, digestive and reproductive systems. The glands produce a thick, sticky substance which make it difficult to digest food and in the lungs, it causes repeated infections and irreversible damage.

The is no cure for CF but treatment aims to slow the progression of the disease. In natural therapies there are certain dietary interventions as well as supplements, digestive enzymes and lifestyle habits which can help to manage the symptoms.

Symptoms of cystic fibrosis

  • A persistent cough with thick mucus
  • Wheezing and shortness of breath
  • Fatigue
  • Exercise intolerance
  • Recurrent lung and nasal infections
  • Poor weight gain and growth
  • Foul smelling, greasy stools
  • Abdominal pain, bloating and gas
  • Severe constipation or diarrhoea
  • Unintentional weight loss

How Cystic fibrosis is inherited

How Cystic fibrosis is inheritedOn average 1 in 25 people carry the CF gene, most of whom are unaware that they are carriers because they have no symptoms. For a child to have CF they would need to inherit one of these genes from each of their parents. If they inherit only one copy, they won’t develop the disease but instead they might pass this gene onto their children.
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Screening for Cystic fibrosis

Since 1986, newborns undergo a heel prick test to screen for a range of conditions including CF. Unfortunately, this test does not detect CF in every infant and there is another test available called the sweat test. People with CF have very high levels of salt in their perspiration.

If there are obvious symptoms of CF in young children such as an intestinal blockage or failure to thrive –this may lead doctors to identify the disease.

Causes of Cystic fibrosis

The genetic defect changes a protein that controls the movement of salt in and out of cells. This results in an increase in salt in sweat which can then deplete salt in the body and lead to muscle cramps and dehydration. It also affects the cells that produce mucus and digestive juices. Instead of a thin and slippery mucus being produced, this defect results in secretions that are sticky and thick. These thick secretions block tubes, ducts and passageways, especially in the airways and pancreas.

Risk factors include family history and race. Although cystic fibrosis can occur in all races, it is most common in white people of northern European ancestry.

There are many different defects that can occur in the same gene. Different types of gene mutations are associated with varying levels of disease severity.

Managing the symptoms of cystic fibrosis

Dietary interventions

Due to a decrease in nutrient absorption and an increase in salt excretion it is imperative that the diet is high in calories and nutrients such as salt, healthy fats, complex carbohydrates, antioxidants and fat-soluble vitamins. This is likely to result in a stronger metabolism, immune system and digestive function. While processed foods are high in fat and calories they are usually low in nutrients and are high in omega-6 which promotes inflammation. 

Dietary interventionsCF fibrosis increases the risk of diabetes, so although increased calories are required they should not be obtained from high GI foods such as lollies, soft drinks or biscuits.
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A higher sodium intake is required, especially if exercising, and this can be achieved by adding rock salt to meals.
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Examples of good quality foods to eat include fresh vegetables and fruit, coconut oil and water, fresh fish, nuts and seeds, eggs, avocado, wholemeal grains, natural yoghurt and grass-fed meats.

Keeping hydrated is important to thin mucous, prevent dehydration and regulate bowels.

Fat-soluble nutrients

In CF there is a greater risk of experiencing a deficiency in fat-soluble nutrients because of decreased pancreatic function. Our fat-soluble vitamins are essential nutrients and include vitamins A, D, E, and K.

Fat-soluble nutrientsIn the body they act as antioxidants, support healthy mucus membrane and lung function, reduce the risk of infection and are required for strong bones. These can be found in a variety of foods including green leafy veggies, eggs, organ meats, wheatgerm, almonds, pumpkin, mackerel, salmon and sunflower seeds. Often these nutrients need to be supplemented to avoid deficiencies and can be found individually or combined.

Digestive enzymes

There are a variety of different enzymes the body requires to break down foods for absorption in the small intestine. Vegetarian digestive enzymes facilitate the breakdown of carbohydrates, fats, proteins, dairy and fibrous parts of plants. Pancreatic enzymes are also commonly given to help digest foods and calories more effectively. Ideally, they should be taken with each meal or as directed by your health care provider.
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Other beneficial nutrients with proven efficacy in cystic fibrosis include:Cystic fibrosis

  • Beta-carotene
  • Zinc
  • Magnesium
  • Coenzyme Q10
  • Fish oil
  • Curcumin
  • Garlic
  • Probiotics, especially Lactobacillus reuteri and Lactobacillus rhamnosus LGG

Regular exercise and physical therapy

Engaging in regular gentle physical exercise helps to loosen mucus, improve mood and self-esteem, reduce disease complications and strengthen the heart. Anything that increases the heart rate is beneficial and this can include walking, team sports and cycling. Other helpful therapies include breathing exercises and cupping therapy.

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References

http://www.cysticfibrosis.org.au/

https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/cystic-fibrosis-cf

https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700

Braga SFAlmgren MM. Complementary therapies in cystic fibrosis: nutritional supplements and herbal products. J Pharm Pract. 2013 Feb;26(1):14-7

https://www.ncbi.nlm.nih.gov/pubmed/23178411

Santi M, et al. Magnesium in cystic fibrosis--Systematic review of the literature. Pediatr Pulmonol. 2016 Feb;51(2):196-202

https://www.ncbi.nlm.nih.gov/pubmed/26663706

Papas KA, et al. A pilot study on the safety and efficacy of a novel antioxidant rich formulation in patients with cystic fibrosis. J Cyst Fibros. 2008 Jan;7(1):60-7

https://www.ncbi.nlm.nih.gov/pubmed/17569601

del Campo R, et al. Improvement of digestive health and reduction in proteobacterial populations in the gut microbiota of cystic fibrosis patients using a Lactobacillus reuteri probiotic preparation: a double blind prospective study. J Cyst Fibros. 2014 Dec;13(6):716-22

https://www.ncbi.nlm.nih.gov/pubmed/24636808

Bruzzese E, et al. Disrupted intestinal microbiota and intestinal inflammation in children with cystic fibrosis and its restoration with Lactobacillus GG: a randomised clinical trial. PLoS One. 2014 Feb 19;9(2):e87796

https://www.ncbi.nlm.nih.gov/pubmed/24586292

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