All the Cystic Fibrosis information you need to live with the condition.

If you are wondering ‘what exactly is cystic fibrosis?’ and are looking for reliable cystic fibrosis information, Complete Care Shop are here to help. This guide will explore cystic fibrosis symptoms, treatments for cystic fibrosis, what causes cystic fibrosis, and types of cystic fibrosis. We hope you find this guide useful, and we hope it raises awareness of the signs of cystic fibrosis to look out for.

What is Cystic Fibrosis

Caused by one’s genetics, people with cystic fibrosis are born with the condition. Cystic fibrosis causes sticky mucus to build up in a person’s organs, including the lungs, pancreas and liver, which means the organs cannot function properly. There are many other potential symptoms of cystic fibrosis, and each person with the condition will be affected differently.

There are around 11,000 people in the UK with cystic fibrosis, and it affects both males and females of all ethnic groups, although it is most common in white people. Each day, around 5 babies are born with cystic fibrosis in the UK. There are lots of treatments for cystic fibrosis, which can help relieve symptoms and help a person have a good quality of life. Many people with cystic fibrosis live well into adulthood and are able to go to school, college, work, get married and do the things that people without the condition do

Many people now even live to the age of 50.

Read more about cystic fibrosis.

What causes cystic fibrosis?

Cystic fibrosis is an inherited genetic disorder, wherein the defective (mutated) genes responsible for the condition are transmitted from parents to their unborn child. Interestingly, even though the parents themselves may not exhibit any symptoms, they can both carry the faulty gene.

The inheritance patterns can vary. In some cases, one parent may have cystic fibrosis while the other carries the faulty gene. Alternatively, both parents might be carriers of the faulty gene without having the condition themselves. Each of these scenarios presents different probabilities for the child to develop cystic fibrosis.

The genes that cause cystic fibrosis are responsible for making proteins that create cells within the body function. The particular cystic fibrosis gene mutations affect the CFTR protein that makes salt and water move into and out of cells, and this is what affects the body’s over-production of mucus. There are lots of different types of cystic fibrosis mutation, which present as different cystic fibrosis symptoms.

Read more about what causes cystic fibrosis and the importance of genes.

Did you know?

Cystic fibrosis is a life-limiting, genetic, inherited condition that a person is born with.

Cystic fibrosis symptoms

Cystic fibrosis symptoms may differ from person to person because there are so many variations of the genetic mutations that are affecting the body, which produce different symptoms in different people.

The main symptom of cystic fibrosis which is fairly common amongst those living with the condition, and which is most often associated with the condition, is related to the lungs. Cystic fibrosis affects the respiratory tract because a person with the condition produces thicker mucus in their lungs than is normal in a healthy body.

In individuals with cystic fibrosis, persistent coughs, wheezing, and compromised lung function can be common, leading to difficulty in breathing. The excess mucus in the airways contributes to frequent bacterial infections. Over time, the accumulation of mucus can cause damage to the delicate cilia, which are essential structures within the lungs.

Read more about the effects of cystic fibrosis on the lungs.

Individuals with cystic fibrosis face an ongoing risk as they are more susceptible to catching viruses, which can lead to severe infections. For this reason, it is strongly recommended that people with cystic fibrosis avoid physical contact with one another. This precaution is essential because each person may carry bacteria that could be especially harmful to another individual with the same condition. Maintaining distance between affected individuals helps minimise the risk of cross-infection and enhances overall well-being.

Read more about the risks of cross-infection.

Other symptoms of cystic fibrosis may include:

• Liver problems, caused by bile in the liver becoming sticky and causing inflammation
• Digestive system problems, often involving the pancreas, which can be affected by mucus blockages
• Diabetes, caused by problems with the pancreas being unable to produce insulin properly
• Sinusitis
• Nasal polyps
• Late puberty
• Fertility problems
• Difficulty gaining weight / growing problems
• Problems with bones e.g. arthritis, osteoporosis, etc
• Swollen fingers
• Excessive sweating

Whilst an extensive list of symptoms, every person with cystic fibrosis is affected differently and may only experience a selection of these symptoms, dependent on the type of cystic fibrosis they have. Find out more about how cystic fibrosis affects the body.

Diagnosis of cystic fibrosis

Some time ago, a cystic fibrosis diagnosis would have been made later in a person’s life, when symptoms were noticed. However, in the UK nowadays, newborn babies undergo a screening test in the first week of their life, which explores whether they have a set of diseases, one of which is cystic fibrosis. The test involves a ‘heel prick’ to obtain a blood sample which is then tested. This test may indicate that a baby has cystic fibrosis genes and further testing is then carried out to confirm if the child has the condition. A sample of the baby’s sweat is obtained and tested for levels of salt within the sweat, which is an indicator of cystic fibrosis. Read more about diagnosis of cystic fibrosis in babies.

Some adults who were born before newborn screening was implemented, or who were born in the 80s/early 90s before testing was accurate, may be diagnosed in adulthood or may have been misdiagnosed as having other health conditions. If you are concerned you may have symptoms of cystic fibrosis, visit your GP to discuss these.

Someone with a cystic fibrosis diagnosis, and their family, may undergo genetic testing to confirm the particular genetic mutation they have, and also to confirm who carries the cystic fibrosis gene. Read more about genetic testing for cystic fibrosis.

Types of cystic fibrosis

There are different categories of cystic fibrosis which are determined by the type of genetic mutations a person has. The mutations that occur within a gene mean that the gene will make the body’s proteins work in a certain way, and this will cause different cystic fibrosis symptoms. There are thousands of possible mutations and these are categorised broadly into 5 groups. Read more about the genetic mutations that cause cystic fibrosis.

Did you know?

Cystic fibrosis causes mucus to build up in some of the body’s organs including the lungs and the pancreas.

Treatments for cystic fibrosis

Cystic fibrosis is a hereditary genetic condition, making it not possible to cure. Consequently, the primary goal of treatment revolves around symptom management and minimising the impact of the disease on the body and vital organs. There are many different types of cystic fibrosis treatment, depending on how the disease is affecting a person, because symptoms may vary a lot. Symptoms are determined by the particular genetic mutations a person has, and therefore cystic fibrosis causes different issues for each person with the condition.

Below, we will explore the medication, surgical and therapeutic cystic fibrosis treatments that may be available to a child or adult with cystic fibrosis:

• Antibiotics used to treat infections of the lungs
• Steroids used to calm inflamed airways of the lungs
• Drugs that reduce lung mucus and open airways to make breathing easier
• Daily physiotherapy that clears lung mucus
• Enzymes to help with food digestion if the pancreas is affected
• Nasal medications and rinsing the nose can help if the nose is affected by thick mucus

In some instances, cystic fibrosis can inflict such damage on a person's lungs, that their lungs can no longer function properly which can have a huge impact on their daily life and health. When this occurs, a potential solution may involve considering a lung transplant. During this procedure, both of the damaged lungs are replaced with healthy donor lungs to improve the individual's respiratory function and quality of life. 

This is a serious undertaking and there are lots of issues to consider, such as being on a transplant list for some time, the risks and aftereffects of such major surgery, and the fact that living with transplanted lungs is likely to result in regular, life-long medical checks. Not only this but if a person’s liver is also damaged by cystic fibrosis, a liver transplant may also be recommended. Read more information on organ transplants as a treatment for cystic fibrosis.

Did you know?

Cystic fibrosis symptoms may include coughing, frequent lung infections, progressive lung damage, digestive problems, fertility problems (in men), osteoporosis and diabetes.

Living with cystic fibrosis

Likely to affect day-to-day life in several ways, cystic fibrosis is a chronic condition. Most people living with cystic fibrosis will require treatment which is likely to impact daily life.

Impact on daily living

Cystic fibrosis affects people’s daily life in a range of ways, including:

Symptoms

Managing cystic fibrosis symptoms can be challenging, as they often lead to persistent problems related to breathing and digestion. Additionally, the burden of living with these symptoms can have psychological effects on some individuals, resulting in feelings of low mood or even depression.

However, many people are able to use treatments that reduce the impact that symptoms have on their life. There are lots of cystic fibrosis support options that give people the chance to connect with each other or talk to knowledgeable advisors if symptoms are worsening or impacting daily life. Sharing experiences may help some people with the condition and their families to find ways to cope with their experiences.

Treatment

Cystic fibrosis treatment focuses on symptom management, but it demands a significant time commitment each day. For instance, individuals with the condition often require daily physiotherapy to help loosen mucus in the chest. In the case of a child with cystic fibrosis, parents are likely to learn these physiotherapy techniques to effectively alleviate mucus build-up and relieve symptoms. Other treatments like using a nebuliser may also require time each day. Certain medications can, unfortunately, come with side effects. For people who need to have transplant surgery, this is an intense challenge that is likely to have a huge impact on daily life.

Lifestyle

Individuals with cystic fibrosis are encouraged to adopt a healthy lifestyle, which includes regular exercise and maintaining a nutritious diet to ensure they receive sufficient nutrition. If you're interested in learning more about the cystic fibrosis diet, you can find further information later.

Cross-infection

It is very strongly recommended for individuals with cystic fibrosis not to meet one another face to face, and completely avoid being in close proximity to each other (i.e. in the same room or area). This is because they both risk becoming infected with bacteria that they individually hold in their lungs, which is likely to differ from person to person. Passing on one person’s bacteria to another is very risky and could make the person being infected very ill. This may be difficult for some people who wish to develop friendships and connections with others who have cystic fibrosis, but there are lots of ways this can still be achieved without ever meeting in person, such as using social media, forums, and via telephone/text message/video call. Read more about the risks of cross infection.

Prognosis and survival

Coping with a chronic and life-limiting health condition naturally leads to concerns about the future and its uncertainties. Cystic fibrosis development varies for each person, making it difficult to predict. As a result, most individuals approach each day as it comes, tackling challenges as they arise. However, it's worth noting that ongoing research into new treatments offers hope and is continuously improving the quality of life for people with cystic fibrosis, making their journey more manageable.

Most people living with cystic fibrosis are able to live their life in much the same way that those without the condition do, such as going to school, college, university, getting a job, having a family and so on. Read more about how cystic fibrosis affects young people’s lives.

Cystic fibrosis products

Daily living aids can be useful products for people who have chronic health conditions or disabilities. At Complete Care Shop, we are experts in daily living aids, and our specialist team of Occupational Therapists can advise on suitable products that could help with various daily tasks.

If you are unsure what cystic fibrosis products may help you, or need any advice, Contact Us or get expert advice from our team of occupational therapists.

PLEASE NOTE: Our Expert Advice Service can only advise about equipment and products which may help you to live more independently. They cannot give any advice on medications or treatments for symptoms of this condition.

Cystic fibrosis diet

When managing cystic fibrosis, the importance of considering one’s diet should not be underplayed. 

Cystic fibrosis often affects a person’s digestive system, specifically the function of the pancreas. The pancreas is responsible for releasing enzymes into the gut to help break food down and also regulate blood sugar levels.

The pancreas of a person with cystic fibrosis becomes blocked with excess mucus and can become scarred, leading to the two key functions of the organ being affected. This means that food may not be broken down properly and that some foods are particularly difficult to digest, such as fats. 

Some people with cystic fibrosis may also develop diabetes because the pancreas is unable to produce enough insulin to manage blood sugar correctly. Read more about diabetes caused by cystic fibrosis.

Exercise for cystic fibrosis

Cystic fibrosis exercise is really important because physical activity helps improve lung function by moving mucus from the lungs. Exercise also helps improve mood, reduce anxiety, build strength in the muscles, make bones stronger and keep joints flexible.

Parents of children with cystic fibrosis must encourage their children to exercise from an early age, making it a priority throughout childhood. For individuals living with cystic fibrosis, cardiovascular exercise is particularly beneficial, as it gets the heart pumping. This includes activities like team sports, running, swimming, and more. Regular exercise also helps preserve bone strength, which is essential for those with the condition, as they may be at risk of osteoporosis and related issues Cystic fibrosis exercise should also include stretches that help the joints stay flexible- yoga and Pilates are excellent choices.

A physiotherapist may be able to put together a programme of exercise for cystic fibrosis patients.

Read more about being active when you have cystic fibrosis.

Cystic fibrosis and employment

Living with a cystic fibrosis diagnosis does not mean that a person is unable to work – many people with the condition do work and have careers. Cystic fibrosis symptoms may cause some issues at work such as needing time off for medical appointments, but employers are legally obliged to permit these and ensure reasonable adjustments are made to allow a person with the condition to carry out their job safely and comfortably. With health as their main priority, a person living with cystic fibrosis may be unable to do certain jobs - for example, keeping their lungs healthy is really important so they may need to avoid careers where chemicals, dusts, smoke or other potentially lung-damaging inhalants are used.

Read more information on cystic fibrosis and work here.

Did you know?

Cystic fibrosis can be treated with medications and physical therapy, with some people requiring organ transplantation.

Support

We hope this cystic fibrosis explanation has been useful to you, whether you are living with cystic fibrosis yourself or have a child with a cystic fibrosis diagnosis.

Living with, or raising a child with, a genetic condition can be difficult and feel lonely at times, but there are lots of cystic fibrosis support options available. Here, we point you to sources of cystic fibrosis help, including websites that provide further information and advice, and cystic fibrosis support groups that enable people with the condition to connect.

Communities

Cystic Fibrosis Facebook Support Group – a Facebook support group for anyone who has cystic fibrosis, or their families, to share experiences, ask for informal advice and talk freely about the condition

Cystic Fibrosis Trust Forum – an online community for people to share their experiences of living with cystic fibrosis or raising a child with cystic fibrosis

Resources

British Lung Foundation – a charity supporting people with a variety of lung conditions including cystic fibrosis, featuring information on the condition as well as how to care for the lungs generally; a helpline is also available

Cystic Fibrosis Foundation – a US charity that supports people with cystic fibrosis with advice, information and research

Cystic Fibrosis Trust – the leading CF charity, providing information and advice about the condition, including symptoms, treatments, practical matters, research projects, as well as an advice helpline

NHS – source of official medical advice and information about cystic fibrosis symptoms, treatments and living with the condition

Did you know?

Nutrition and exercise are really important for people with cystic fibrosis.

Glossary

While we do our best to present information in a simple and clear manner, there are instances where using accurate medical terminology becomes necessary. Medical terms can be known for their tricky pronunciation, and being difficult to understand. To aid in your understanding of cystic fibrosis, we've compiled a list of terms used on this page, accompanied by brief explanations to ensure the information remains as straightforward as possible.

Antibiotics

Medicine that kills bugs and bacteria within the body to halt infection.

Cilia

Tiny, very fine hairs in the lungs, which help to remove mucus but which can become damaged.

Enzymes

Contained in fluid within the body, and within the pancreas, which are responsible for breaking down fats, proteins and carbohydrates to digest food effectively.

Fats

An essential fatty acid that the body cannot produce, and which must be absorbed from food to help store energy and protect organs.

Faulty Gene

An error within a gene (part of DNA) which results in a medical condition and which may be passed on to offspring.

Liver

A major organ in the human body, responsible for making proteins, detoxifying the body, controlling nutrient storage and producing bile.

Lungs

Major organs within the body, which enable oxygen to be breathed in and carbon dioxide breathed out.

Mucus

A thick substance produced in the body, designed to protect the body and its organs by keeping things lubricated, clean and trapping infection.

Nasal Polyps

Fleshy lumps that develop in the nose or sinuses, which can cause obstruction.

Pancreas

A major organ in the body, which helps to digest foods and produces hormones such as insulin to control blood sugar/energy in the blood

Puberty

The period of life of a teenager where they become physically able to reproduce.

Sinusitis

A common condition causing inflamed sinuses.

Steroids

Medicine designed to reduce inflammation in the body.

Respiratory tract

The mouth, nose, throat and lungs which all work together to enable breathing.

Contact Us

We hope this guide has served you well, providing as much cystic fibrosis information as you need. However, if there is anything else you would like to know, you can head to our useful help and advice section for more guidance.

If you’d like to speak to a member of our team to discuss our living aids, don’t hesitate to contact us.