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Cystic fibrosis (CF) is a severe, progressive, multisystemic disease that is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. One of the systems impacted by CF is the gastrointestinal tract: pancreas, liver and intestines. Patients with this disease are prone to malnutrition, growth failure, and vitamin deficiencies. Optimizing nutrition is critical, as higher growth parameters are associated with better pulmonary function and outcomes.

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The pancreas has two roles in the the human anatomy, it is both part of the digestive system and the endocrine system.

The pancreas makes fluid that mixes with the food that we eat, One part of the fluid is a product that neutralises the acid from the stomach, Which is called bicarbonate. Everyone with Cystic fibrosis (including people who don't need enzyme supplements) has a pancreas that does not make enough bicarbonate to neutralise

stomach acid. This can also contribute to pain, cramping, gas, and constipation. Bloating and excessive gas also can be caused by small bowel overgrowth, gastric paresis, and gastroesophageal reflux disease.

In addition to this, the pancreas is important organ in making important hormones including insulin, which helps move glucose ( sugar ) from the blood cells so they can turn food into energy. For people with cystic fibrosis, with the pancreas being affected their body may not let the insulin do its job which can then lead to CF-Related diabetes.

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Management of Pancreas for patients with Cystic Fibrosis 

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Management of Pancreas for patients with Cystic Fibrosis 

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Most people who have Cystic fibrosis will be advised to take pancreatic enzyme supplements, this therapy is known as PERT (pancreatic enzyme replacement therapy) the tablets come in a capsule form. These capsules are coated in special enteric coating, which allows the capsules to be dissolved in the small intestine, to help digest food.

These supplements can help a patient to digest carbohydrates, proteins and fats, gain a healthy weight and absorb essential nutrients such as vitamins and minerals.

These enzymes should be taken at meal times and should be adjusted with what the person has eaten.

Eating a high calorie diet can also be of help to a person with Cystic Fibrosis, Getting regular nutritional assessments with healthcare providers and discussing enzyme care use with the cystic fibrosis care team.

PERT Therapy can also help with Pancreatic cancer: PERT can help manage digestion symptoms and improve how you feel while undergoing chemotherapy or surgery.

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The majority of people with CF experience exocrine pancreatic insufficiency resulting in poor absorption of nutrients and malnutrition. The main foods not digested properly are fatty foods and proteins.

The build up of digestive enzymes in the pancreas lead to inflammation and scarring, breaking down the tissue of the pancreas and the cells that produce insulin - this eventually slows down (eventually stopping) the production of insulin and leads to high blood glucose levels. This is what leads to CF related diabetes.

Diabetes screening is important part of ongoing CF care. Symptoms of this developing are:

-feeling more thirsty than normal

-going to the toilet more frequently

-exhaustion/increased tiredness levels

-weight loss

-more frequent chest infections

Management of CF related diabetes include: diet, insulin, exercise. Developing this can lead to further complications including eye problems and nerve damage.

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Why is it important?

Many people with CF experience malabsorption so the high energy diet helps replace the energy lost.

People with CF may experience an increase work of breathing so they require more energy to facilitate that.

When they have an infection the high energy diet can help them fight the infection.

In cases where the infection has caused low appetite and food intake,nutrients can be taken in by form of a high calorific, energy drink.

The diet can help prevent the body taking nutrients and energy from elsewhere in the body. (ie. Brakedown in muscle and fat)

High Energy foods include:

Full cream dairy products

Olive oil, avocado, nuts and seeds

Meat,fish and egg

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This is used in patients with pancreatic insufficiency and it made out on pigs pancreas. Due to this ethical dilemmas might arise for some religious beliefs.

It contains digestive enzymes that cannot reach the the GI tract as pancreatic duct becomes blocked with CF.

Pancreatin contains a mixture of protease, lipase and amylase which helps breakdown protein, fats and carbohydrates.

They must be taken with food and they can't be chewed due to this they can b e mixed with food as the must be swallowed.

Side effects of this include: Nausea, abdominal discomfort and mucosal irritation.

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The goal of GI Management in Cystic Fibrosis is to support nutrition, maintain digestive function, prevent intestinal blockages and address any liver complications, for instance liver disease. Therefore, getting patients and family members to understand the condition will benefit everyone so the care is consistent.

Patient & Family Education

Self Management - Teaching patients and families about diet and recognising signs and symptoms is critical. Educating helps for quicker intervention.

Overall, GI management in Cystic Fibrosis requires a proactive approach from everyone to support the patients digestion, prevent all complications and maintain the appropriate level of nutrition , which is the requirement for overall health and quality of life.

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CF affects many types of Exocrine glands (Exocrine glands secrete things into cavities within the body)which have epithelial tissue within them including those found lining the pancreas.

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Thick mucus can cause partial or complete blockage of the intestine. This is especially common in newborns, leading to meconium ileus. In older children and adults a similar obstruction known as distal intestinal obstructive syndrome (DIOS) may occur resulting in abdominal pain, bloating and constipation. Medication used to relieve this may be Osmotic laxatives, Metoclopramide, Oral rehydration solutions, Surgical interventions.

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Management of GI Issues in CF

1. Nutritional Support

Enzyme Replacement Therapy: Pancreatic enzyme replacement therapy (PERT) improves digestion and absorption.

High-Calorie, High-Fat Diet: To meet increased energy demands.

Vitamin Supplementation: Fat-soluble vitamins (A, D, E, K) to counter deficiencies.

Salt Supplementation: Due to increased sodium losses in sweat.

2. Managing Intestinal Obstruction

Meconium Ileus: Surgical intervention or osmotic enemas.

DIOS: Oral osmotic agents (e.g., polyethylene glycol), hydration, and stool softeners.

3. Monitoring and Treating Hepatobiliary Disease

Ursodeoxycholic Acid (UDCA): Improves bile flow and reduces liver inflammation.

Regular monitoring of liver function tests and imaging for early detection.

4. Probiotic Therapy

Probiotics may help maintain gut microbiota balance, though evidence is evolving.

5. Managing GERD

Proton pump inhibitors (PPIs) or H2 blockers to reduce gastric acidity.

Lifestyle modifications, such as small, frequent meals and avoiding late-night eating.

6. CFRD Management

Regular screening with oral glucose tolerance tests (OGTT).

Insulin therapy is preferred for glycemic control.

7. Regular GI Monitoring

Assess growth, stool frequency, liver enzymes, and abdominal symptoms.

Early intervention for complications like DIOS or liver disease.

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Cystic Fibrosis Transmembrane regulator(CFTR) is present throughout the small intestine in Epithelial cells, crypt and Brunner's gland (secretes alkaline fluid made up of mucus).

Due to the mutation of the gene it causes a malfunction and results in infection and inflammation in the intestine.

Management would be to keep well hydrated and seek help from GP if discomfort continues. Possible laxatives treatment.

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CF-related liver disease occurs in around 40% of individuals with CF. CF causes secretions and bile to thicken, due to this the ducts leading out of the gallbladder get blocked, thereby causing the backflow of bile into the liver. Over time, this irritates the liver, thereby leading to inflammation in the bile ducts and scarring in the liver, called cirrhosis. The thickened bile also affects the gall bladder by increasing the risk of gallstones.

The liver's role in carbohydrate metabolism is affected which then disrupts the concentration of blood glucose.

Individuals may require transplants later in life as damage occurs to the cells leaving the liver unable to regenerate.

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Cirrhosis of the liver - Symptoms, Diagnosis, Survival Rate and Treatment

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Learn about cirrhosis of the liver. Causes and symptoms of cirrhosis of the liver. Cirrhosis of the liver life expectancy & treatment.