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Fdnm 1003 - Disease and Disability - Nutrition Management in Cystic Fibrosis

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Nutrition management in Cystic Fibrosis

FDNM1003- Disease and disability

Cystic fibrosis is an autosomal recessive genetic disorder which is life threatening. It is a disease of exocrine glands which causes abnormal sodium, chloride transport. This leads to elevation in electrolyte levels within the sweat glands, also leading to dysfunction of other exocrine glands, producing some viscous secretions which is low in water content. This eventually results in insufficiency of pancreas leading to malabsorption and these individuals fail to weight gain, also the airway gets obstructed which increases the chances and episodes of recurring bronchial infection which leads to lung damage and can also cause respiratory failure.

Major clinical abnormalities of cystic fibrosis include gastrointestinal tract involvement caused because of pancreatic insufficiency which leads to malabsorption causing malnutrition eventually. Respiratory tract infection caused because of obstructive pulmonary disease and also causes recurrent infections. Salt loss through sweat also leads to severe dehydration due to hyponatremia. Pancreatic disturbance starts prenatally and causes intestinal obstruction in new born infants with cystic fibrosis.

Like pancreatic insufficiency, lung disorders are not always present at the time of birth. However, infections and obstructions are inevitably seen. The onset of respiratory tract disorders is not known. Research proves that 80 to 90% of cystic fibrosis patients suffer from pancreatic insufficiency. This also makes them more susceptible for developing insulin insufficiency and diabetes because the islet cells of Langerhans are also affected due to pancreatic inefficiency causing lack of insulin thus triggering the person towards developing diabetes or impaired glucose tolerance. These individuals usually require supplemental insulin or exogenous insulin to manage their blood sugars. Cystic fibrosis is a condition which causes development of other chronic diseases.

Causes for cystic fibrosis-it occurs because of mutation in gene named CFTR which controls the transport of fluids and sodium in cells. the causes also include infectious pathogens like staphylococcus aureus, pseudomonas aeruginosa, it also occurs due to cystic fibrosis transmembrane gene expression.

• Nutrition management for improving survival rate- nutritional management for these patients include controlling the effects of malabsorption and maldigestion. Protein energy malnutrition is very common in cystic fibrosis patients. These patients usually have low circulating concentration of any given nutrient, but they do not exhibit any symptoms. Severe nutrient deficiency can lead to serious complications like growth retardation, metabolic abnormalities, structural and functional disorders. Malnutrition is most commonly seen during the period or growth and development when the protein and energy needs are high. There multiple reasons of malnutrition but CF patients three causes are primary, increased loses of energy and nutrients, increased energy expenditure, and decrease food intake which causes low energy and nutrient availability. Other factors also cause energy loses like diabetes can cause energy loss because of excessive glycosuria if not controlled.

• The food intake of these patients is limited because respiratory exacerbations which are a major reason for anorexia, and chest infection worsens the condition by causing vomiting and nausea. Elevated levels of tumour necrosis factor also contribute to anorexia. Also, apart from respiratory complications, gastro-intestinal disturbances also cause decrease in food intake because of rapid episode of gastroesophageal reflux, esophagitis can all reduce the food intake. Also, intermittent bowel disturbances, cramping of stomach can all contribute to wasting of energy and reduced caloric intake.

• Various deficiencies – these patients also suffer from deficiency of Vitamin A, E, K and D out of which vitamin A and vitamin E are of greatest concern. As liver is a storage house for these vitamins, their stores are depleted because of fibrosis of liver cells. Vitamin A level is low due to low levels of retinol binding protein which required for the absorption of vitamin a. Vitamin e is evident by low alpha-tocopherol levels. Vitamin k levels are only low in individuals who have severe cholesteric liver disease.

• Macro minerals concentration should be considered for patients suffering from cystic fibrosis because minerals like sodium is abnormally lost via sweat. This can also lead to a hyponatremia shock. Sodium supplements are required only for patients that have excessive sodium loss.

• Essential fatty acids deficiency in these patients with cystic fibrosis can cause serious damage to new born because it can growth retardation, mental disabilities, poor wound healing, thrombocytopenia.

• Malabsorption and malnutrition in CF- Malnutrition is the common problem associated with cystic fibrosis patients. Protein should be adequately supplied because of malabsorption and increased requirements. However, protein needs are met if energy requirements



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