Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes.

Slides:

Advertisements

Similar presentations

By: Ryan M. and Anthony L..

Advertisements

Cystic Fibrosis Julianne Tamoney. Objectives Brief discussion of pathophysiology Understand medical and nursing interventions Discussion of clinical relevance.

CF week. Anatomy of the LRT  Airway branchings & LMB vs RMB  Name levels from trachea to alveoli  Changes as we descend resp tract?  Pleural cavity.

Cystic Fibrosis (Mucoviscidosis)

 By: Nneoma Ajiwe and Angie Olutimehin Cystic fibrosis is caused by a defective genes which causes an over-production of mucus. It is a recessive trait,

Cystic Fibrosis Fred Hill, MA, RRT. Overview Cystic fibrosis is the most common fatal, inherited disease in the U. S. Cystic fibrosis is the most common.

Cystic Fibrosis Gina Brandl, RN BSN Nursing Instructor, Pediatrics.

Pulmonary Function Testing in Cystic Fibrosis

CF Education Day March 15, Agenda - Morning Keynote - Bob Beall, President CFF Newborn Screening - Jackie Zirbes New Treatments: VX770 - Daya Upadhyay.

Cleaning Equipment 101 CF Education Day 2009

Cystic Fibrosis and Physiotherapy Linda Lapointe P.T. CHEO March 3 rd, 2015.

CYSTIC FIBROSIS FANOURAKI MARIA CHARALAMPIDOU ALEXANDRA

Cystic Fibrosis By Kristy Sandman. Physiology Genes found in nucleus of each cell Genes made up of nucleotides Genes can be rearranged or mutated Genes.

1 RETROSPECTIVE EVALUATION OF THE PATIENTS WITH CYSTIC FIBROSIS DR.LALE PULAT SEREN ZEYNEP KAMİL MATERNITY AND CHILDREN’S TRAINING AND RESEARCH HOSPITAL.

Cystic Fibrosis Stacey Simon. Statistics  Most common lethal, hereditary disorder among Caucasians  1 in 1,000 live births  Prevalence: 30,000 children.

Cystic Fibrosis Sharon George PD.3. Cystic Fibrosis is inherited when the child inherits two mutated CFTR genes, one from each parent. The presence of.

Cystic Fibrosis Theresa Brady January 9, Cystic Fibrosis What is it? What is it? How do you get it? How do you get it? How can you be tested for.

Author: Richard H. Simon, M.D., License: Unless otherwise noted, this material is made available under the terms of the the Creative Commons.

By Taliyah and Selina. Cystic Fibrosis CF Mucoviscidosis.

The Child with Cystic Fibrosis Lydia Burland. Learning Outcomes By the end of the session you should;  Know the basic physiology underlying CF  Recognise.

Symptoms In newborns: – Delayed growth – Failure to gain weight normally during childhood – No bowel movements in first 24 to 48 hours of life – Salty-tasting.

Patient: Lily Johnson Case study by Alexa Angelo

High-Throughput Screening Speeding Up CF Drug Discovery >10,000 Primary assays/day High-throughput screening CFTR Modulator Drug SAR based Medicinal Chemistry.

Cystic Fibrosis Kayla Barber. What is it? Cystic Fibrosis is a hereditary disease that a person gets when BOTH parents are carriers. It causes abnormally.

Sweat Test. A sweat test measures the amount of salt chemicals (sodium and chloride) in sweat. Sodium and chloride are part of your body’s electrolyte.

Approach to bronchiectasis

Cystic Fibrosis By: Morgan. Definition Cystic fibrosis is a thick mucus that clogs the air ways and tends to cause lung diseases. A diseases common among.

Cystic Fibrosis: A Review for Healthcare Providers

Cystic Fibrosis By: Olivia DeCoste.

-Cystic Fibrosis (CF) is one of the UK's most common life-threatening inherited diseases. -Cystic Fibrosis affects over 8,500 people in the UK. -Over.

Cystic fibrosis is an inherited disease that causes thick, sticky mucus to build up in the lungs and digestive tract.

Cystic Fibrosis Afshaun Haniff, PharmD Candidate

Objectives Review the causes of cystic fibrosis (CF) Describe the symptoms and laboratory findings in CF Review current and emerging CF treatments Review.

A GENETIC, CHRONIC, AND LIFE-THREATENING DISEASE THAT CAUSES THICK, STICKY MUCUS TO BUILD UP IN THE LUNGS, DIGESTIVE TRACT, AND OTHER AREAS OF THE BODY.

CYSTIC FIBROSIS AND CELL COMMUNICATION. CFTR Cystic Fibrosis Transmembrane Conductance Regulator ( Or CFTR)  Is a transport protein for Chloride across.

Cystic Fibrosis.

Cystic Fibrosis By: Jenna Mahoney and Jake Tuyls.

Gene Therapy Cystic Fibrosis. Key ideas Understand that cystic fibrosis is a genetic disease that results in the build-up of salt inside of cells. Understand.

Update on Cystic Fibrosis – treatments and new products Dr Caroline Pao, Consultant Respiratory Paediatrician, Royal London Hospital.

LAUREN BURNETT NUR3215 EBP CYSTIC FIBROSIS. OBJECTIVES By completion of this presentation, learners should be able to: Describe the effects Cystic Fibrosis.

Cystic Fibrosis By: Lexi Luft. What is Cystic Fibrosis? How can one get it? Are there any treatments?

New Developments in Cystic Fibrosis

Cystic Fibrosis Overview

Cystic Fibrosis and Termination of Pregnancy Jalandria Gurley, MS, FNP-BC Texas Woman’s University.

BY: TERESA KRASZEWSKI CYSTIC FIBROSIS. BACKGROUND AND HISTORY Late 16th century babies who had “salty skin” when kissed were likely to die 1938 Dr. Dorothy.

Cystic fibrosis. Etiology and epidemiology Cystic fibrosis (CF) is an autosomal recessive disorder that is the most common life limiting genetic disease.

Cystic fibrosis.

Cystic Fibrosis Chris Kingsnorth.

Cystic Fibrosis By: Tamaryn Cortes.

Addition of Inhaled Tobramycin to Ciprofloxacin for Acute Exacerbations of Pseudomonas aeruginosa Infection in Adult Bronchiectasis* Diana Bilton, MD;

Nuts and Bolts: Pulmonary Overview Starla G. Martinez, M. D

Cystic Fibrosis Tutoring

Site, Sponsor, CRO: Understanding Roles & Expectations Creating a Team Approach Alan Genatossio, BSN, RN Seattle Children’s, Seattle, WA Colleen.

Improving the Number of Quantity Not Sufficient [QNS] Specimens in Pediatric Cystic Fibrosis [CF] Patients’ Sweat Chloride Testing Christi Dodd, BSN,

A Simple Glance at Cystic Fibrosis

Cystic Fibrosis And Treatment

Pseudomonas Infection in Cystic Fibrosis

Cystic Fibrosis Learning Objective

Cystic Fibrosis Richard H. Simon Pulmonary and Critical Care Medicine Department of Internal Medicine Fall 2008.

Cystic Fibrosis By: Sharan Kaur.

Algorithm for the diagnosis of CF starting with the CFTR DNA test.

Evaluating Effects of CFTR Modulation in Cystic Fibrosis

New Horizons in Cystic Fibrosis Clinical Developments From Fall 2014

Addressing Treatment Challenges in Cystic Fibrosis

The predictive potential of the sweat chloride test in cystic fibrosis patients with the G551D mutation Verena I. Seliger, David Rodman, Fredrick Van.

Inhaled Antibiotics for Patients With CF in the Era of CFTR Modulators

Cystic Fibrosis An Overview

Design of adult and paediatric studies investigating the efficacy and tolerability of ivacaftor [16, 17]. Design of adult and paediatric studies investigating.

Correlation of sweat chloride levels with cystic fibrosis transmembrane conductance regulator (CFTR) protein expression and function. Correlation of sweat.

Presentation transcript:

Cystic Fibrosis "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and soon must die." This European adage accurately describes the fate of an individual diagnosed with cystic fibrosis during ancient times. Part 2

Diagnosis Patients present with persistent pulmonary infections, pancreatic insufficiency, and elevated sweat chloride levels Adults are more likely to present with GI symptoms, diabetes, and infertility As of 2010 CF newborn screening is required in all states

Diagnostic Criteria BOTH of the following criteria must be met to diagnose CF: 1.Clinical symptoms consistent with CF in at least one organ system, AND 2.Evidence of CFTR dysfunction (any of the following): Elevated sweat chloride ≥60 mmol/L (on two occasions) Presence of two disease-causing mutations in CFTR from each parental allele Abnormal nasal potential difference

Sweat Chloride Test Remains the primary test for the diagnosis of CF. Intermediate results of sweat testing should be clarified by DNA analysis using a CFTR multi-mutation method, and sweat chloride testing should be repeated.

Treatment and Survival Although CF shortens lifespan, survival has dramatically increased from the 1950’s –Between 1985 and 2007 the median lifespan increased from 25 to 37 years 45% of CF patients are >18 years old 10% of CF patients are >40 years old In 2011 the median predicted survival for patient in the United States was 36.8 years

Cornerstones of Chronic Cystic Fibrosis Therapy Airway clearance –Manual percussion, oscillating vest, flutter valve Mucolytic dornase alfa Nebulized antibiotics –Tobramycin –Colistimethate, aztreonam Oral azithromycin Inhaled hypertonic saline Aggressive antibiotic therapy for exacerbations Nutritional support –High-caloric, high salt diet Replacement of fat- soluble vitamins ADEK Exercise

Pulmonary Treatments

Ivacaftor (Kalydeco®) Only for patients with G551D mutation 150 mg by mouth twice daily with high fat meal Improves chloride transport by potentiating CFTR Decreases sweat chloride, increases FEV1, increases weight, decreases exacerbations, increases quality of life

Dornase Alfa (Pulmozyme®) Reduces mucous viscosity Dosed at 2.5 mg daily or twice daily Administered via nebulizer and should not be mixed with other drugs Improves FEV1 and decreases exacerbations

Hypertonic Saline Improves airway clearance by hydrating the airway surface liquid 3% or 7% saline twice daily to four times daily Administered via nebulizer Improved FEV1 and decreased exacerbations

Inhaled Tobramycin (TOBI) Antipseudomonal aminogylcoside antibiotic Dosed at 300 mg inhaled every 12 hours for 28 days and then not taken for 28 days Must be used with PARI-LC PLUS handheld nebulizer Administer TOBI last and do not mix with other inhaled medications Improves FEV1 and decreases exacerbations

Aztreonam (Cayston®) Antipseudomonal antibiotic Dosed at 75 mg inhaled every 8 hours for 28 days and then off for 28 days Utilizes Alteral Nebulizer System. Do not mix with other inhaled medications Improves FEV1 and decreases exacerbations

Azithromycin (Zithromax®) Antibiotic that does not have antipseudomonal activity also exhibits some anti-inflammatory properties Dosed at 250 or 500 mg orally three times a week Decreases exacerbations