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Cystic Fibrosis

Everything you need to know about living well with cystic fibrosis

  • Introduction
  • Symptoms
  • Treatments
  • Daily Living
  • Support
  • Glossary

Introduction

If you or someone you know has been given a cystic fibrosis diagnosis, this guide may help you understand more about the condition.

You may be wondering, what is cystic fibrosis? NRS Healthcare are here to help explain this, as well as 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.

Introduction

What is cystic fibrosis?

Cystic fibrosis is a condition caused by genetics, meaning a person is born with it. 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 live to the age of 50.

Read more about cystic fibrosis.

What causes cystic fibrosis?

Cystic fibrosis is a genetic condition, which means the faulty (mutated) genes that are responsible for cystic fibrosis are passed on from parents to their unborn child. Some parents will both have a faulty gene even though they themselves do not have the condition.

Sometimes, one parent may have cystic fibrosis, and the other just carries the faulty gene, and sometimes both parents may simply be carriers of the faulty gene. In each of these cases, there are different chances of the child having 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

Here we describe the most common symptoms of cystic fibrosis.

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.

Introduction

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. Some people with cystic fibrosis may also experience constant coughs, wheezing, and reduced lung function that makes breathing difficult. The mucus clogs the airways and bacterial infections may become common. The lungs can become damaged due to the build-up of mucus, which affect delicate parts of the lung called the cilia.

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

There is a constant risk for people with cystic fibrosis, as they may be more likely to catch viruses and these may cause a serious infection. People with cystic fibrosis are strongly advised not to be in physical contact with one another, because they each may harbour bacteria that would be particularly harmful for someone else with the condition.

Read more about the risks of cross infection.

Other cystic fibrosis symptoms 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

This is an extensive list, and includes most cystic fibrosis symptoms, but every person with the condition is an individual and may experience only a selection of these symptoms depending 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

Here we run through cystic fibrosis treatments.

Cystic fibrosis is a genetic, inherited condition and so it is not possible to cure the disease. Treatment for cystic fibrosis therefore focuses on managing symptoms and attempting to reduce the amount of damage the disease does to the body and its major 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.

Treatments

Below, we 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

Sometimes, a person’s lungs can become so damaged by the condition that they are no longer functioning properly, which may have a huge impact on their daily life and health. In such cases, a person may be considered for a lung transplant, where both of the lungs are replaced by donor lungs. 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 after effects of such major surgery, and the fact that living with transplanted lungs is likely to result in regular, life-long medical checks. Similarly, if a person’s liver is damaged by cystic fibrosis, they may be recommended to have a liver transplant. Read more information on organ transplants as 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

Here, we explore the impact that cystic fibrosis has on daily life.

Cystic fibrosis is a chronic condition and is likely to affect daily life in lots of ways. Most people living with cystic fibrosis will require treatment which is likely to impact daily life.

Introduction

Impact on daily living

Cystic fibrosis affects day-to-day life in many ways, including:

Symptoms – cystic fibrosis symptoms may be difficult to deal with, causing constant issues with breathing or digestion, and some people experience psychological symptoms due to the pressures of living with symptoms, such as feelings of low mood or 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 helps manage symptoms but it takes time each day, For example, most people with the condition need daily physiotherapy to loosen mucus in the chest. Having a child with cystic fibrosis means a parent is likely to need to learn the physiotherapy techniques that can help shift this mucus build up to relieve symptoms. Other treatments such as using a nebuliser may require time each day. Some medications may have 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 – people with cystic fibrosis are likely to need to live a healthy lifestyle, exercising regularly and eating a healthy diet to ensure they are getting adequate nutrition. You can read more about cystic fibrosis diet later

Cross-infection – people with cystic fibrosis are strongly advised not to meet each other face to face, and to avoid being in close proximity to each other (i.e. in the same room or area). This is because they each have a risk of becoming infected with the bacteria that they individually hold in the 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 – living with a chronic and life-limiting health condition inevitably brings anxiety about the future and what this will hold. It is impossible to say how cystic fibrosis will develop for each individual with the condition, and most people take each day as it comes, dealing with each set of challenges as they arise. There is a huge amount of research being undertaken into new treatments, which are making life easier for people with the condition

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 NRS Healthcare, 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.

Daily living aids are designed to help people who have mobility problems or who are recovering from surgery. They are great for helping with certain everyday tasks that may become difficult due to cystic fibrosis symptoms. For example, if lung problems make breathing difficult in advanced stages of the condition, this can cause fatigue and mobility difficulties for some people, who may find that getting around or standing for long periods becomes tiring. A rollator or a perching stool may help address these issues. If a person needs help to manage medication, there are lots of pill reminders and storage devices to assist, and if a person is experiencing incontinence there are many solutions available to help relieve the distress this may cause.

We also provide a range of medical equipment such as bronchial suction units that help to clear a person’s airways.

If you are unsure what cystic fibrosis products may help you, contact our friendly and knowledgeable Occupational Therapist Product Advice team for free by telephoning 0345 121 8111 or emailing productadvice@nrshealthcare.co.uk

Cystic fibrosis diet

Diet for cystic fibrosis is really important for helping to manage the condition.

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 to regulate blood sugar levels.

In a person with cystic fibrosis, the pancreas becomes blocked with excess mucus and can become scarred, resulting in these two key functions of the organ being affected. This can lead to food not being broken down properly and some foods being 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.

Some children and adults with cystic fibrosis find that they are underweight, which may be due to the condition causing them to burn more energy than they take in from food. Lung infections and persistent coughing means more calories are needed to give the body energy to fight the infection and physically cough. Digestive issues can also mean that calories and nutrients are not absorbed correctly. Cystic fibrosis symptoms may affect a person’s appetite, which can lead to not eating enough.

A cystic fibrosis diet therefore needs to ensure a healthy balance of nutrients but also that ample calories are consumed, which may mean increasing the amount of fat eaten.

Research shows that people with cystic fibrosis who eat a well-balanced diet and who are a healthy weight, actually have better lung function than those who are underweight, so diet for cystic fibrosis is an important factor in managing and treating the condition.

Read more about nutrition and diet for 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.

It is important for parents of a child with cystic fibrosis to instil physical activity from a young age and make it a priority during childhood. Exercise for those living with cystic fibrosis should involve cardiovascular exercise that gets the heart pumping, such as team sports, running, swimming and so on. Exercise helps maintain bone strength, which is important for people with the condition because 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 for Cystic Fibrosis

Remember, you are not alone!

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.

Introduction

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

Unsure what something means? Checkout our Glossary section below

Although we always try to explain things as simply and as clearly as possible, sometimes it’s necessary to use the correct medical terminology. Medical terms are often known for being tricky to pronounce and if you’re not an expert in the subject, they can also be a little difficult to understand. Below, we’ve put together a list of terms used on this page along with a brief explanation of what they mean to help make your understanding of cystic fibrosis as straightforward as possible.

Introduction

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 in the 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


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