Cystic fibrosis workshop

  1. What is the condition of meconium ileus, and what are the dangers associated with it?

    condition that primarily affects new borns with cystic fibrosis. the meconium becomes abnormally thick and obstructs the intestines. Dangers include bowel obstruction, pain and discomfort, and malnutrition.

  2. How can the blockage of a meconium ileus be removed?

    surgery

  3. What is a contrast enema X-ray? Can you interpret the results?

    its a medical imaging procedure used to examine the large intestine. patient lays on x-ray table and a small tube gets inserted into the rectum, barium is inserted through the tube, which lines the colon and makes it visible on xray

    A shows a blockage

  4. How is meconium ileus related to Cystic Fibrosis?

    like CF there is excess, thickened sticky mucus and meconium ileus is a strong indicator of cystic fibrosis.

  5. How common is the delayed passage of meconium in newborns with CF?

    its relatively common and often one of the early signs of CF

  6. How is a sweat test conducted?

    two electrodes are attached to skin, one to stimulate sweat production and the other to collect it. pilocarpine is applied under one of the electrodes to stimulate sweat glands. sweat is collected and conc of chloride is measured

  7. Why is a sweat test conducted?

    Elevated levels of chloride in the sweat are indicative of cystic fibrosis.

  8. What are the test levels inidcative for CF? 

    A diagnosis of CF is usually made if the chloride concentration is above a certain threshold (usually 60 mmol/L) on two separate tests

  9. Why is a stool sample taken?

    Pancreatic Insufficiency

  10. What are levels of elastase are indicative of normal pancreas function?

    a fecal elastase level of 200 micrograms per gram (µg/g) or higher is often considered indicative of normal pancreatic function.

  11. How would you explain the illness to the parents?

  1. lower levels than expected, exocrine pancreatic insufficiency

  2. n

  3. educating family: provide info about CF and treatment, respect cultural and language difference

    sharing info with relatives: protect patient privacy and follow data protection laws, balance potential with potential harm

    identifying CF carrier status in a minor child:

  4. What does "Δ508" in terms of a genetic change. In very general terms what effect does it have and how common is it?

    loss or deletion of a specific amino acid in the CFTR (cystic fibrosis transmembrane conductance regulator) protein which is essential for regulating the movement of salt and fluids in the body's cells causes the production of thick, sticky mucus in various organs, including the lungs and digestive system.

  5. What is a CFTR modulator? What is meant by a ‘potentiator’ and ‘corrector’?

    type of medication designed to target and improve the function of the CFTR protein. Potentiators are drugs that aim to enhance the function of CFTR proteins. Correctors are drugs designed to improve the processing and trafficking of CFTR proteins the protein may not fold or move correctly within the cells, preventing it from reaching the cell surface. Correctors help "correct" this issue by assisting the CFTR protein in folding properly and moving to its intended location on the cell surface.

  6. Before CFTR modulators, what were typical treatments for CF?

    Airway Clearance Techniques: These techniques, such as chest physiotherapy and postural drainage. Mucolytics: Medications like dornase alfa (Pulmozyme) were used to thin the mucus in the airways, making it easier to clear.Bronchodilators: Medications like albuterol were used to open the airways, making it easier to breathe and reducing bronchoconstriction.

    Antibiotics: Antibiotics were prescribed to treat and prevent respiratory infections, which are common in individuals with CF.

  7. What does the wet cough suggest?

    bacterial infection- suggests that there is an excess of mucus in the airways or the lungs

    Clear or White Mucus: Clear or white mucus in a wet cough may indicate a viral respiratory infection, allergies, or exposure to irritants like smoke or dust. It can also be a normal response to cold or dry air.

    Yellow or Green Mucus: Yellow or green mucus can suggest the presence of an infection, such as bronchitis or pneumonia. It is often seen in bacterial respiratory infections when white blood cells are present.

    Rust-Colored Mucus: Rust-colored mucus can be a sign of older blood in the respiratory tract and may indicate bleeding in the airways. It's important to seek medical attention if you notice this.

    Thick, Sticky Mucus: Thick and sticky mucus may be associated with conditions like cystic fibrosis, chronic bronchitis, or bronchiectasis, where mucus is abnormally thick and difficult to clear.

    Frothy or Pink Mucus: Frothy or pink-tinged mucus can be a sign of heart-related issues, such as congestive heart failure, when fluid accumulates in the lungs.

    Excessive Mucus Production: Chronic excessive mucus production may be seen in conditions like chronic obstructive pulmonary disease (COPD), asthma, or chronic sinusitis.

  8. What can a flexible bronchoscopy permit?

    a thin, flexible tube with a light and a camera (bronchoscope) is inserted through the nose or mouth and into the airways to examine the respiratory system

  9. Why are Pseudomonas aeruginosa infections a concern for CF patients?

    could lead to decrease in lung function, its very hard to get rid of once infected. around half of CF patients have it