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Cystic Fibrosis

Respiratory System

Abigail Kluck and Lindsey Faraon

Respiratory System

Respiratory System

  • The respiratory tract is divided into upper (organ outside thorax - nose, pharynx, and larynx) and lower respiratory tract (organ within thorax - trachea, bronchi, bronchioles, alveolar duct, and alveoli).

Functions

  • The primary function of the system; a process that provides body cells with oxygen and removes waste product carbon dioxide; includes four separate processes:
  • Pulmonary ventilation (often shortened to ventilation) – a movement of air in and out of the lungs
  • Pulmonary gas exchange – the movement of gases between lungs and blood
  • Gas transport – a movement of gases through blood
  • Tissue gas exchange – the movement of gases between blood and tissues
  • Serve to maintain homeostasis

What is it?

What is it?

Cystic fibrosis (commonly known as CF) is an inherited disorder that causes severe damage to the lungs, digestive system, and other organ system in the body.

Continued

Continued

CF causes malfunction in the electrolyte transport system causing cells to absorb too much sodium and water. This causes the normal thin secretions in the lungs to become very thick and hard to move around which leads to these thick secretions increasing the risk for frequent respiratory infections. Recurring respiratory infections will lead to extensive damage of the lungs and eventually the death of cells in the lungs.

Occurrence, Morbidity/Mortality, and Life Expectancy

Occurrence, morbidity/mortality, and life expectancy

There are more than 30,000 children and adults in the US suffering with cystic fibrosis. Due to improved medical treatments and research many patients diagnosed with CF can expect to live well into their 30’s, 40’s and beyond. According to the Cystic Fibrosis Foundation’s patient registry data supports people living longer, healthier lives with CF. However, the median age of death is still only 32 years old. Those living with the disease still face many challenges including hospitalization, complications, and even depression.

Treatment

Treatment

CF is a genetic disease and the only way to prevent or cure it would be with gene therapy at an early age. Ideally, gene therapy could repair or replace the defective gene. Another option for treatment would be to give a person with CF the active form of the protein product that is scarce or missing. For patients whose disease is very advanced, lung transplantation may be an option.

Hannah Goetz

Story

Hannah has always had cystic fibrosis her entire life. In July of 2019, Hannah came down with the flu that quickly worsen to pneumonia. The doctors decided since the medication wasn't working the only option left was to do a double lung transplant. It took about a week to find a set but when they did, they took her into surgery late July 25 and finished up on the 26th. According to the doctor everything went well but wanted to wait to take her off the vent to give her body time to heal. About after a week of getting off the vents, she started therapy to strength her body and eventually was able to leave the hospital about a month after the transplant.

Pictures

Picture

Hannah

Hannah

Hannah

Quick Overview Video

Video

Sources

  • “About Cystic Fibrosis.” Cystic Fibrosis Foundation, www.cff.org/intro-cf/about-cystic-fibrosis.
  • Cystic Fibrosis Fact Sheet - Centers for Disease Control ... www.cdc.gov/scienceambassador/documents/cystic-fibrosis-fact-sheet.pdf.
  • “Cystic Fibrosis.” Johns Hopkins Medicine, www.hopkinsmedicine.org/health/conditions-and-diseases/cystic-fibrosis.
  • “Cystic Fibrosis.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 23 Nov. 2021, www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700.
  • Erin, Amerman. “Chapter 21.” Pearson EText for Human Anatomy & Physiology.
  • MacDemystMed, director. YouTube, YouTube, 24 Nov. 2016, www.youtube.com/watch?v=k4U5wyPzxJE. Accessed 29 Mar. 2022.

Sources

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