When the Trust began in 1964, people with cystic fibrosis would be lucky to live past five years old. Now, according to the UK Cystic Fibrosis Registry Annual Report for 2016, half of those with the condition in the UK will live to celebrate their 47th birthday, and a baby born with the condition today can expect to live even longer.
As the result of significant improvements in treatment, standards of care and basic understanding of cystic fibrosis, there are now more adults than children with the condition. We believe that we can reach the day when everyone born with cystic fibrosis can live a life unlimited by their condition.
However, as people with cystic fibrosis live longer into adulthood, they are encountering new challenges and issues. Some of the issues you develop as you get older are different from those you may have faced in your earlier years.
Over time the burden of care increases, and you are likely to be taking more medicines and receiving more treatments. This can also have an impact on partners or family members as they may take on more caring roles.
You may experience a range of emotional and social issues as well, some of which may differ from your peers. Managing your CF while coping with work and relationships can prove to be an additional challenge.
Both men and women who have CF can enjoy a normal sex life, but around 98% of men are infertile because the tube that carries sperm from the testis to the penis (the vas deferens), does not develop properly. Men with fertility problems can try a specialised form of IVF called intracytoplasmic sperm injection (ICSI), where a sperm is collected and injected into your partner’s egg.
In women, the onset of puberty is often delayed by one or two years. Most girls with CF have normal menstrual cycles after this, but there is a higher chance of missed or irregular periods and amenorrhoea (the absence of menstrual periods in a woman of reproductive age). This is most likely to happen if you have a reduced percentage of body fat. The majority of women who have cystic fibrosis can become pregnant without any difficulty. Female fertility problems in cystic fibrosis are tackled in the same way as for other women. If you are a woman with CF and considering trying for a baby you should consult your CF specialist, who will arrange for genetic and obstetric advice, and check that your prescribed treatment is safe in pregnancy.
Other health issues
Eating well is key to living healthier with cystic fibrosis. There is an apparent link between higher bodyweight and good lung function. Some people with CF may struggle at times to keep up adequate energy intake and growth, despite a high-fat diet and dietary supplements. During these times it may be necessary to take extra nutrition either via a tube through the nose, or directly into the stomach. Read more on adult nutrition and cystic fibrosis.
Changing medication or treatment
As the symptoms and impact of your condition change over time, you may need to take more and/or different medication. As the form of your condition changes, and as other complications arise you may have to alter your physiotherapy regime and other treatments. For example, if osteoporosis develops, percussion may not be suitable as the bones are too vulnerable.
Part of growing up with CF is the transition from paediatric care to an adult CF service. In becoming more responsible for your own care and treatments, you will learn the skills you need to manage adult life and get the most from activities such as employment, going away to college, or living with a partner.
Being an adult with CF can involve a number of distinct challenges for you and your family. Aside from physical concerns, some of the areas to think about include:
Emotional and social issues
Planning how to provide for yourself and your family in the event of prolonged illness and absence from work can be daunting. Take a look at our benefits page for some basic information.
As people with CF live longer, pensions are becoming more of a concern for many. Some older people may not have considered starting a pension when they were younger, but are now becoming aware of the need to plan for the future. Visit the Department of Work and Pensions website for more information. If you have any questions you can also ask your hospital if there is a social worker you can consult.
Coping with bereavement
Cystic fibrosis is still a life-shortening illness, and facing the loss of a friend or loved one can be hard. If you would like to talk to someone about this you can call our helpline number 0300 373 1000 or 020 3795 2184, where you can chat to someone in confidence. Alternatively, you can share your thoughts and feelings with our online community via Facebook.
Other emotional and social issues
Balancing a 'normal' life with managing a chronic condition can be challenging. As an adult with CF you may encounter a very different range of emotional and social experiences to your peers, such as coping with work or relationships and cystic fibrosis.
Some people with CF may feel barriers to forming relationships, such as a lack of independence or embarrassment because of symptoms. There are a large number of further health complications that come with age in CF, as well as the condition itself becoming more severe. In these circumstances adult relationships may be affected as partners or family members take on more caring roles.
If you require help and support with these or any other issues, speak to your specialist CF team.
If you would like to talk to someone about any of these issues, you can call our helpline on 0300 373 1000, where you can chat to someone in confidence. You can also share your thoughts and feelings with our online community on our forum and via Facebook
If you need more information, or want to share tips, support and advice, check out our Forum. You will need to sign up to access the Forum.