Understanding Primary Ciliary Dyskinesia

What causes Primary Ciliary Dyskinesia?

Primary Ciliary Dyskinesia (PCD) is a rare birth defect. The cilia in patients with PCD malfunction. Cilia are the tiny hair-like structures whose job it is to move mucus out of respiratory passages. When cilia don't work correctly, the mucus gets trapped and blocks your respiratory tract. 

How does Primary Ciliary Dyskinesia affect Your Body?

  • PCD affects your respiratory system and other systems that depend on cilia to function properly, including the middle ear and reproductive organs.
  • About half the people with PCD have a condition called Kartegener's Syndrome. All the organs in their chest cavity and abdomen are the mirror image of where they should be situated.
  • When the cilia do not move mucus out of the respiratory tract, the lungs and airways become very inflamed and become perfect breeding ground for bacteria as the mucus sits.
  • People with PCD have frequent infections of the lungs, ears, throat, and sinuses.
  • The build-up of mucus also can cause bronchiectasis, which causes bronchial tubes to weaken and stretch out, and often leads to pneumonia. 

How Serious is Primary Ciliary Dyskinesia?

  • PCD worsens over time, and there is no cure.
  • Cases of PCD can vary greatly in how severe they are.
  • People can maintain stable lung function for many years with aggressive therapy and close monitoring.