Download Free Medical About Cystic Fibrosis PowerPoint Presentation

About Cystic Fibrosis PowerPoint Presentation

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About This Presentation

Description : Check out this medical PowerPoint presentation titled "Cystic fibrosis" by Tyra Bradbury, MPH,RD,CSP.

This medical PowerPoint presentation is about cystic fibrosis (CF), a genetic disorder that affects the respiratory, digestive, and reproductive systems. It is caused by a mutation in the CFTR (cystic fibrosis transmembrane conductance regulator) gene, which results in the production of thick, sticky mucus in the body.

The thick mucus can block the airways in the lungs, making it difficult to breathe and leading to lung infections and damage over time. It can also block the ducts in the pancreas, preventing digestive enzymes from reaching the small intestine and causing malnutrition. Additionally, CF can affect the liver, sweat glands, and reproductive system.

CF is typically diagnosed through newborn screening or genetic testing. Treatment involves managing the symptoms and complications of the disease, including antibiotics to treat lung infections, bronchodilators to open airways, and enzymes to aid digestion. Lung transplantation may also be an option for some individuals with severe lung damage.

There is no cure for CF, but advances in medical care have improved the prognosis and quality of life for individuals with the disease. Early diagnosis and aggressive treatment can help manage symptoms and prolong life expectancy.

Tags : Cystic fibrosis, genetic disorder, mutation in the CFTR gene, cystic fibrosis transmembrane conductance regulator gene, lung infections, Pediatrics, Pediatrics PowerPoint presentation

Published on : Jun 23, 2023 | Views : 1869 | Downloads : 4

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Slide 1-

CYSTIC FIBROSIS Tyra Bradbury, MPH,RD,CSP Neonatal/Pediatric Dietitian
Slide 2-

Outline Arizona Newborn Screening Definition Diagnosis Nutrition Estimated calories and protein needs Salt Enzymes Types and dosing CF related issues
Slide 3-

Outline Resources Families Websites CF centers Healthcare providers Websites CF centers Explanation of the project currently being worked on Community/rural education Nutrition practice guidelines
Slide 4-

Arizona Newborn Screening All babies born in Arizona Blood spot screening – 28 disorders Hearing screen CF screening in Arizona began in November of 2007 46 mutations www.AZNewborn.com
Slide 5-

Cystic Fibrosis - Definition Genetic disease affecting the mucus glands in the liver, lungs, pancreas and intestines Defective gene produces a protein that causes thick mucus to accumulate Affects 1 in every 3000 Caucasian babies born 30,000 people in the US (70,000 worldwide) www.cff.org
Slide 6-

Cystic Fibrosis - Diagnosis Newborn screen Sweat test Measures the amount of salt in the sweat Positive test - >60 mEq/L Negative - <40 mEq/L Genetic testing Used to confirm diagnosis Also used to test for carriers More than 1000 mutations
Slide 7-

Cystic Fibrosis - Nutrition Calories 1.2-2 times the DRI for age Goal is sustained weight gain Tips: Infants – higher calorie breast milk/formulas Children and adults Butter or margarine added to foods Add oils, cheese, heavy cream Whole milk High calorie snacks – supplements, flavored syrups on ice cream Cystic Fibrosis Nutrition 101 (2009)
Slide 8-

Cystic Fibrosis - Nutrition Protein 1.5-2 times the DRI for age Fat 35-40% of calories Hyperlipidemia generally not a risk Cystic Fibrosis Nutrition 101 (2009)
Slide 9-

Cystic Fibrosis - Nutrition Vitamins Fat soluble vitamin needs increased Require water miscible forms Serum levels assessed annually Sodium Salt lost through sweat Infants with CF 2-4 mEq/day = 1/8-1/4 teaspoon daily added to breast milk/formula Cystic Fibrosis Nutrition 101 (2009)
Slide 10-

Cystic Fibrosis – Enzyme Replacement 85-90% pancreatic insufficient Usually in capsule form with small beads inside (enteric coating) Swallow capsules whole or beads mixed in acidic foods Take before (and sometimes during) meal, not after Never take generic enzymes Cystic Fibrosis Nutrition 101 (2009)
Slide 11-

Cystic Fibrosis – Enzyme Dosing Infants 1000 Units lipase/kg/meal 2000-4000 Units lipase/120ml breast milk or formula Children 1000 Units lipase/kg/meal 500 Units lipase/kg/snack Older Children and Adults Start with 500 Units lipase/kg/meal Maximum dose 2500 Units lipase/kg/meal 10,000 Units lipase/kg/day Cystic Fibrosis Nutrition 101 (2009)
Slide 12-

Cystic Fibrosis - Enzymes Creon (second smallest microsphere) Creon 5 = 5000 units lipase/capsule Creon 10 = 10,000 units lipase/capsule Creon 20 = 20,000 units lipase/capsule Pancrease MT 4, 10, 16 and 20 (number x 1000 = units lipase/capsule) Ultrase 4500 units/capsule Ultrase MT 12, 18 and 20 (number x 1000 = units lipase/capsule)
Slide 13-

Cystic Fibrosis - Enzymes What if patient is at max dose and still symptomatic? Acid blockers Switch brand of enzyme ? non-adherence (with snacks/drinks/etc) What else causing loose stools? Refer to GI Cystic Fibrosis Nutrition 101 (2009)
Slide 14-

Cystic Fibrosis – Related Issues Meconium ileus Ileum completely obstructed No stool at 24-48hrs old, emesis, abdominal distention Enemas vs surgery CFRD Usually diagnosed after 18 years old (using OGTT) Insulin treatment common Do not follow usual dietary advice Cystic Fibrosis Nutrition 101 (2009)
Slide 15-

Cystic Fibrosis – Related Issues Bone disease Low body weight, vitamin D deficiency, delayed puberty, lung disease Tube feedings ~50% of needs 8-10 hours overnight Intact formulas Meal enzyme dose before and after Cystic Fibrosis Nutrition 101 (2009)
Slide 16-

Cystic Fibrosis – Related Issues Pregnancy Lung function may suffer Vitamin A is teratogenic at high doses (>8000 IU/day) Transplant Good nutrition status key before transplant CFRD common secondary to steroids Pancreatic disease persists Cystic Fibrosis Nutrition 101 (2009)
Slide 17-

Cystic Fibrosis - Resources Cystic Fibrosis Foundation www.cff.org Cystic Fibrosis Nutrition 101 Amanda Leonard, MPH, RD, CDE – The Johns Hopkins Children’s Center Terri Schindler, MS, RD – Rainbow Babies and Children’s Hospital University of Arizona Pediatric Pulmonary Center (PPC)