Living with cystic fibrosis
Every person living with cystic fibrosis will have a different experience. At times life might mean isolating and spending days, or even weeks, indoors to avoid exposure to infections. Other times, life will include social activities, sport, parties…even mountain climbing!
While there is currently no cure for cystic fibrosis, the focus of ongoing research is dedicated to improving quality of life for people living with CF, as well as advancing treatments to reduce symptoms and the affect the condition has on the body.
While the treatments regimes vary dramatically from person to person, living with CF is all about routine.
This routine often consists of taking one or two nebulisers every morning and every night with physiotherapy sessions in between, and depending on co-conditions, can mean taking up to 50 tablets a day, including antibiotics some containing enzymes that aid food metabolism.
Medications and treatments aside, it’s essential for people with CF to incorporate exercise into each day to keep lungs active, and to ensure a healthy food routine, including essential nutrients and healthy fats to maintain a healthy body weight and avoid malnutrition.
Due to advancements in medical research, many people with CF can receive treatments at home, which can certainly make sticking to a routine easier, and can increase quality of life.
Rest is another essential part of living with CF. It’s important to stay well rested and to reduce anxiety, stress and strain on the body and lungs, to avoid ending up in hospital.
Hospitalisations can consist of at least two weeks at a time, and can including procedures such as intense physiotherapy and strong antibiotics administered through an IV drip. People with CF will have, on average, two hospital stays a year but some will have as many as six.
Outside of hospital there are specialists and CF-specific clinics where people with CF can regularly go to be tested and have their health monitored.
People with CF will generally attend at least three monthly outside of hospital clinical reviews with a specialist CF multidisciplinary team, which may include all or some of the following health providers and professionals:
- CF Physician
- Social Worker
Newborns and children
Learning that your baby has cystic fibrosis can be extremely difficult, emotional and all consuming news, it can also be a shock if you are unaware of any family history. We want you to know that we understand how challenging this time is, and you are not alone. CFSA was established to help you and your family though life with CF.
In the past, most people were diagnosed with CF after presenting with symptoms. Because of genetic screening programs over the past 20 years, there has been a dramatic increase in the number of CF cases identified at birth, and also prior to birth. This means we are in a more fortunate position now to get ahead with treatment early.
Prior to the availability of newborn screening, infants and children were typically diagnosed with CF at around one year, after presenting with early symptoms, including respiratory symptoms, digestive and bowel problems or nutritional problems including weight loss or inability to gain weight at the expected rate.
By ensuring a diagnosis at birth, early treatment can help to prevent or lessen the impact of these symptoms.
Establishing a treatment plan
The key to your child’s health and happiness will be to ensure you establish a treatment plan immediately. This is something your CF team will help you to do.
Because of the complexities of the condition, treating cystic fibrosis requires a range of therapies, and a team of specialists who can help you and your child to address needs in different parts of the body, particularly the lungs and the digestive system.
This will most likely involve a form of airway clearance to help loosen thick mucus that can build up in the lungs, including physiotherapy, and inhaled medications to clear airways. As fighting lung infection is essential, these medicines may also include antibiotics.
In addition to these daily treatments and specialist care, children with CF must learn early on the importance of staying healthy, which includes regular exercise, good nutrition including a high-fat, high-calorie diet rich in vitamins and minerals, and keeping things as clean as possible to avoid picking up viruses and infections.
Some simple ways you can help your child and family to avoid contact with nasty germs and bacteria include:
- Regular hand cleaning
- Covering coughs
- Cleaning and disinfecting nebulizers
- Not sharing personal items
- Avoiding contact with dirty or unwashed public spaces and surfaces
Life outside of treatment
In spite of daily treatment needs and periods of illness that can lead to hospitalisation, most people with cystic fibrosis can live a normal, happy and fulfilled life.
The life expectancy for people living with CF is increasing and the majority of children with CF will grow up and go to school, get involved in sport, play music, have hobbies, make friends, establish relationships and have careers and families of their own.
Our motto at CFSA is life, right now, because we’re all about helping you ad your child live the best life possible now.
Young people and adults
Whether you’re an adult, or a teenager transitioning to adulthood, it’s important to know that just like life without CF, life with CF will have amazingly fulfilling moments but it will also have its challenges.
That doesn’t mean that life with CF can’t be full of experiences and opportunities, it just means that CF will be there with you along the way.
Just like any adult, people with CF participate in hobbies, study, and work, they have friendships and relationships, and start their own families, all while balancing CF symptoms, treatment and management. It can certainly be tricky sometimes, but it’s not unachievable.
As life gets busier, it can be hard to maintain treatment regimes, which is something adults with CF need to be aware of. In fact, recent studies have shown that around 50% of people with CF don’t follow their doctors treatment recommendations completely, which can sometime land them in hospital.
Just as in childhood, it’s important to stick with a clear and consistent treatment regime, and surround yourself with trusted medical and allied health partners who know you and your condition well.
Managing your routine
Here are some of the key things you can do as an adult with CF to stay on top of your condition and reduce your risk of hospitalisation.
- Surround yourself with a team of trusted medical and health experts you can call on.
- Make a treatment plan with your care team and stick to it.
- Manage your daily schedule to ensure you have enough time to implement your treatment regimes.
- Say no to social or work commitments that don’t allow you to stick to your routine, don’t push yourself and make sure you forgive yourself for doing less.
- If you’re struggling physically or emotionally seek help from peers and professionals as soon as you can.
- Surround yourself with a support network that includes people who have CF or understand what life is like with CF.
Raising a family
In addition to your own health and happiness, as an adult or young person with CF it’s important to think about how your condition may impact relationships and plans for starting a family in the future.
Planning for men
As men with CF can experience fertility issues, and women with CF are at a higher risk of experiencing complications during pregnancy and birth, it is important to talk openly with life partners about your condition and your position on carrier screening.
Planning for women
If you are a woman with CF and decide to have children, it is advised that you ensure the following as part of your pregnancy management plan:
- Stable lung function prior to conception and carefully monitor function during pregnancy
- Seek genetic counselling regarding the risk of passing on the condition, carrier testing, and prenatal diagnosis options.
- Close monitoring of maternal nutrition and weight stability
- Early screening for gestational diabetes in pregnancy
Getting a diagnosis later in life
As carrier screening and diagnosis at birth has only been common practice for the past 20 years, some people may only present with CF symptoms later in life.
For people diagnosed in adulthood symptoms can be quite different, even mild. For example, adults are more likely to be diagnosed upon presenting with gastrointestinal symptoms, diabetes, and infertility.
Adults with CF are also less likely to be living with pancreatic insufficiency, and often return less certain results on sweat tests than children with CF.
Life with a transplant
The treatment of CF related lung disease is experiencing a rapid period of advancement, with exciting new clinical trials improving our understanding of the genetic causes and symptomatic outcomes of the condition.
Fortunately, these discoveries and the implementation of new treatments has also led to significant improvements in survival for people living with CF and those who have lung transplants.
What is involved in a lung transplant?
Most lung transplants for patients with CF require both lungs to be replaced, this prevents any risk of cross-infection from a CF sufferers lung, to a newly implanted lung.
The key risks involved in lung transplantation surgery are – as with any surgical procedure – complications during the surgery itself, and rejection of the new lungs.
Rejection is when the bodies immune system identifies the newly implanted lungs as foreign to the body, and therefore attempt to fight it. Unfortunately this something that will need to be monitored not only after surgery, but throughout life with a transplant.
In order to prevent transplant rejection damaging the new lungs, a person who had received a transplant will need to take a variety of immunosuppressive medications that prevent an immune response which would lead to rejection.
These drugs can have other side effects relating to having a suppressed immune system, however they are essential to keeping lung transplant survivors healthy.
Ongoing medical management
There are a number of other management considerations, of which your treatment team will advise you prior to, and following, a transplant.
This will include specific information on how to take care of your new lungs, including:
- What medicines to take, why take them, how many, and at what time?
- What signs or symptoms to look out for, which may indicate problems with the transplant.
- How to monitor your health at home, this may include keeping a diary or symptom log.
It’s also important to consider the emotional toll having a transplant can take. It can be a very overwhelming time for you and your family, so keep in mind you’re not alone.
Remember CFSA is there to help you with counselling and connecting you to other people with CF who have experienced lung disease and transplants. Head to our services page to find out more, or contact us to have a chat.