physiotherapy for patients with cystic fibrosis

29. October 2021

ask ck physiotherapy about cystic fibrosis

Cystic fibrosis is one of the most inherited genetic disorders.

Currently, only symptomatic treatments are available. But progress in treatment has increased life expectancy from 5 years in the ’60s to 40 years to date. Source Rare Diseases | Findacure

There’s still much to be learnt and explored by doctors, physiotherapists, and patients to formulate effective management and treatment of the disease to increase life expectancy even further.

Whether you’re suffering from cystic fibrosis yourself or inquiring for somebody in your care. This blog will outline the following:

What cystic fibrosis is, plus symptoms presented in adults and children
Physiotherapist advice on treatment and specific exercises
Daily regimes that can be followed
The role of a physiotherapist in alleviating symptoms and the approaches taken

Our aim at CK Physio is to provide comprehensive advice, educational literature, and applicable methods.

With the hope, for those (and for their families) living with cystic fibrosis to live life to the fullest and keep symptoms at bay or manageable.

What is Cystic Fibrosis, its Symptoms, and Tests?

Cystic fibrosis is a severe disease that affects internal organs, especially the lungs and digestive system.

The condition causes a build-up of mucus in the lungs and stomach, which can lead to life-threatening infections, complications with digestion and other respiratory conditions.

Cystic Fibrosis is inherited from parents who are carriers of the defective gene. Usually, it’s diagnosed in the first few weeks of being born through routine screening, yet some diagnoses can be affirmed later in childhood or adulthood.

The symptoms of cystic fibrosis vary from person to person depending on the severity of their CF. Some people will show no symptoms or may experience a few; while others may experience breathing difficulties early on in their lives.

Early diagnosis is key to minimising the impact of the disease, managing symptoms, and maintaining the quality of life.

Cystic Fibrosis symptoms include:

Frequent lung infections: such as pneumonia, which can lead to serious respiratory conditions or even death
Excessive sweating: that may reveal salty skin or electrolyte imbalances
Cough: that often produces phlegm due to chronic infection and inflammation
Repeated sinus congestion and chest infection: caused by chronic inflammation and mucus build-up
Digestive problems: disruption to the digestive system causing meconium ileus (bowel obstruction), distal intestinal obstruction syndrome (DIOS) and gastro-oesophageal reflux

Source: Symptoms of cystic fibrosis (CF) Adults, Cystic Fibrosis: Signs, Symptoms, and Complications (verywellhealth.com) children

Cystic Fibrosis testing includes:

Sweat test: sweat chloride test
Genetic test: ACMG/AGOG targeted mutation panel test
Blood test: IRT (Immunoreactive trypsinogen)
Screening: carrier screening

Source How Cystic Fibrosis Is Diagnosed (verywellhealth.com)

Physiotherapist Advice on Treating Cystic Fibrosis

Physiotherapy for people with cystic fibrosis has many benefits, including improved movement and function, better breathing, and improved general well-being.

As an essential part of treatment, upon diagnosis, a physiotherapist will advise physiotherapy to start as soon as possible.

Although physiotherapy is not a cure for cystic fibrosis, daily exercise and physiotherapy assist in managing symptoms.

Plus, it allows for airways to be cleared and get much-needed oxygen to circulate around the body to reach other organs and maintain healthy muscles, bones, and joints.

Physiotherapy Exercises for Cystic Fibrosis

General exercise is a great way to help clear airways and improve muscle strength and lung function (or delay decline).

Yet physiotherapy led exercise is also crucial to improve physical ability, musculoskeletal function, and cardiovascular performance.

A physiotherapist will assign particular exercises and routines tailored to your age, ability and health.

Studies have documented that a daily regime of physiotherapy treatment actively helps to control cystic fibrosis symptoms and improve quality of life.

Cystic fibrosis physiotherapy exercise areas include:

Airway clearance: ABCT (Active Cycle Breathing), postural drainage, AD (Autogenic Drainage)
Exercise and movement: walking, swimming, cycling, and aerobics
Inhaled treatments: inhalers and nebulisers
Improve posture: good sitting/standing posture, spine stretches, straightening exercises
Incontinence management: pelvic floor exercises
Improve strength and fitness: weight training, dancing, running, yoga, pilates

Source: Physiotherapy resources (cysticfibrosis.org.uk)

Within adults and children, clearing airways is a fundamental part of cystic fibrosis management. Yet suggested and administered exercises can slightly differ. View cystic fibrosis infant physiotherapy on the Great Ormand Street Hospital site for more details.

Physiotherapy exercises may deliver short-term effects but are most effective when consistently repeated over a long-term basis.

Through assessment, your physiotherapist will work out your current fitness level to ensure all prescribed exercises or regimes are suitable.

The Role of a Physiotherapist in Managing Cystic Fibrosis

A physiotherapist is part of a highly trained multidisciplinary team and is there to work with you in alleviating the symptoms, effects, and challenges of cystic fibrosis.

Your physiotherapist will:

Design a fitness program for your daily routine
Provide ways for you to maintain good posture, manage pain and where necessary build/regain muscle strength and endurance
Give advice and demonstrations to learn Airway Clearing Techniques (ACTs)

Your physiotherapist will also help find your fitness level and baseline exercise ability. Through tracked progress, goals will be set to maintain and improve these levels over time.

There are many ways for your physiotherapist to help you with cystic fibrosis. They can work on breathing exercises, to improve your respiratory function, and help with mobility problems.

In addition, your physiotherapist will suggest stretches, ways to make sure you stay hydrated, exercise safely and take adequate rest.

For general well-being and mental health, your physiotherapist may also be able to discuss diet, rest and sleep patterns.