The life expectancy of patients with cystic fibrosis has improved over the last 50 years
The life expectancy of patients with cystic fibrosis has improved over the last 50 years

The life expectancy of patients with cystic fibrosis has improved over the last 50 years. The median survival age is the age at which the survival chances are 50%. In 1959, half of the patients with cystic fibrosis lived only till 6 months, and in 2008, they could live till 27 years of age. If you are born in 2000, you have a 50% chance of living till 50 years of age. The improved chances of survival are due to advances in early diagnosis, supportive care, nutritional care, and infection control.

Based on the 2018 Registry data, if your child with cystic fibrosis is born between 2014 and 2018, you can expect them to live at least till 44 years. If your child is born in 2018, they have 50% chances to live till 47 years or more.

Finally, how long you can live with cystic fibrosis depends on how severe your disease is, or how you respond to different cystic fibrosis treatments.

What is end-stage cystic fibrosis?

End-stage cystic fibrosis is characterized by fluid-filled sacs (cysts), pus-filled pockets (abscesses), and hardening (fibrosis) of lungs and airways. At this stage, you have to rely on external oxygen support to breathe adequately. Lung transplantation can offer a ray of hope in this condition to improve your chances of survival and quality of life. There are 50% chances that you will survive for at least five years after the transplant.

What are the main treatments for cystic fibrosis?

There is no cure for cystic fibrosis, but a range of treatments can help ease the symptoms and help you live a longer life with improved quality. The main treatments include:

  • Airway clearance: Certain airway clearance techniques, also known as chest physical therapy, can help loosen thick, sticky mucous and help you to cough up easier.
  • Medication: Your doctor may prescribe many medications to prevent or fight infections (antibiotics), help your lungs clear sticky mucus (inhaled bronchodilators), lessen the swelling in your lungs (anti-inflammatory drugs), and for some people, help correct the underlying cause of the disease (medicines that target genetic mutation).
  • Fitness plan: This is a part of pulmonary rehabilitation and includes physical exercises that improve your muscle strength, cardiovascular and respiratory fitness, as well as your mood.
  • Nutritional therapies: These include a high-calorie, high-protein, and energy-dense unrestricted diets to replenish the lost nutrients due to poor absorption by the digestive system. You may have to take pancreatic enzymes supplements to improve your digestive capacity and nutritional intake.

Apart from this, you should:

  • Vaccinate yourself regularly (such as taking annual flu vaccine)
  • Maintain hygiene (by washing your hands frequently)
  • Quit smoking
  • Take time to learn about cystic fibrosis

Cystic fibrosis can impact your daily life considerably. Hence, to deal with the stress, you can:

  • Spend time with your family and friends
  • Find a cystic fibrosis support group

You can also ask your doctor if there are clinical trials of therapies to treat cystic fibrosis. You can participate in one if you are looking for better treatment options.

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Medically Reviewed on 9/16/2020
References
Cystic fibrosis. Available at: https://emedicine.medscape.com/article/1001602-overview

Palliative care for patients with cystic fibrosis. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3654821/

Understanding Changes in Life Expectancy. Available at: https://www.cff.org/

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