Living with cystic fibrosis
Learning to manage life around the needs of a child with cystic fibrosis may seem overwhelming at first. But most parents soon establish a routine that allows them to enjoy time with their child and live their lives.
Consistent routine and discipline can be a good way to ensure that your child feels secure. Whether for medications, physiotherapy or having treats, routine and discipline help teach your child self-control and, eventually, self-management of their condition.
Your child’s cystic fibrosis hospital clinic team will provide the best medical care available, while social workers and psychologists also help look after parents’ wellbeing.
Cystic Fibrosis Queensland provides support programs for the entire cystic fibrosis community. Parents receive newly-diagnosed packs and we’re also happy to speak to you about any concerns you may have. Please contact firstname.lastname@example.org or 07 3359 8000 to talk confidentially with our Membership and Services Coordinator.
It is more difficult for children with cystic fibrosis to absorb energy and nutrients from food, so their diet should compensate for this by being higher in kilojoules, fats and nutrients.
Digestive enzymes made by the pancreas help the body digest food by breaking down fats, proteins and carbohydrates. For many people with cystic fibrosis, mucus blocks the ducts that carry the enzymes from the pancreas to the digestive system.
People with this symptom need to take enzyme medications (such as Creon) to assist their digestion. For an infant, you can mix Creon granules in with purees and other soft food.
Physiotherapy exercises help clear the thick, sticky mucus that the body produces from the lungs and airways.
A cystic fibrosis physiotherapist will develop a physio treatment plan to suit your child. Many parents say that engaging their child in play or games is a great way to get them to do their physio and airway clearance exercises, for example bouncing on a trampoline or blowing bubbles.
Being physically active is also beneficial for maintaining lung function so encourage your child to take part in physical games, sports and other aerobic activities.
Child care and preschool
It’s natural to have concerns when your child starts preschool, a good approach to communicating these concerns can be to meet with staff from your child’s centre or preschool. These meetings are a useful opportunity for the staff to learn about cystic fibrosis and what it means for your child. You can also discuss ways that the staff can work together with you to help manage your child’s cystic fibrosis while they are attending the centre.
Teachers and child care staff can learn more about cystic fibrosis here.
Avoiding infection is one of the main parental concerns. Illnesses like coughs and colds are unfortunately common in child care and preschool. Your child, like all of their classmates, is likely to pick up some of the bugs and illnesses that go around. The risk can be lessened through simple but effective infection control procedures, such as hand washing, using hand sanitising gels, opening windows where possible, and asking staff to keep other children with coughs and colds separate from your child.
As well as the risk of illness, there is the chance that the environment and activities at the centre could expose your child to bacteria and fungi. The centre should take care to ensure that only fresh, clean water is used in water play, and that stagnant water is not left to accumulate in play equipment. You also may want to talk with the centre staff about whether you are comfortable for your child to participate in activities such as digging in soil, playing in sand pits and playing with mud.
Even though your child may be at ease with taking Creon, the centre’s staff may feel anxious about giving it to them if they haven’t been used to giving children medication. Talk with your centre’s staff about how to manage giving Creon, and don’t forget to talk them through different scenarios, such as what to do when your child has been given a dose of Creon but then doesn’t want to eat their food.
As your child moves through primary school he or she may become more independent; however, it is still useful for teachers to understand about cystic fibrosis and how it affects your child.
Teachers and child care staff can learn more about cystic fibrosis here.
The school/teacher’s responsibilities are to:
Ensure your child is taking the required medication and food/snacks.
Ensure quick and easy access to the toilet is provided if needed.
Provide catch up work if your child is absent.
Be aware of any changes e.g. symptoms of dehydration, tired, on the toilet more often.
Promote good hygiene and infection control practices.
Your responsibilities are to:
Provide the teacher with the medications required by your child to be taken at school.
Provide the teacher with clear information about medications (how, when, storage, any side effects).
Inform the teacher of any additional medications required during the year.
Inform the school/teacher if your child has to go to hospital, clinic appointments or other absences.
Inform the teacher if there are any changes to your child’s health.
The cystic fibrosis diet
Schools will support a high fat cystic fibrosis diet once they understand why it’s required. Ask the teacher to make reference to the cystic fibrosis diet if the class is learning about healthy eating (as this can sometimes cause confusion for children with cystic fibrosis on a high calorie diet).
Remind the teacher that your child will need regular water (or cordial/salty drinks in summer) and may need to take salt tablets or liquid while at school. The teacher and teaching assistants need to be aware of the signs of dehydration.
Ask the school what infection control practices they have, for example, providing:
paper towel and liquid soap
access to hand gel
regular and ongoing education for the students about good hand washing practices
a school policy on unwell children coming to school
It’s a good idea to make the school aware of cross infection issues in case there is another child at the school who has cystic fibrosis. This is rare but it does happen.
It is not advisable for children with cystic fibrosis to be in the same class as each other, but if they are in different year groups the risk can be managed.
Things to discuss with your child’s teacher:
Symptoms that might impact your child while at school e.g. coughing, tiredness, wind, toilet habits, becoming dehydrated easily, anxiety.
Enzymes – where will they be stored e.g. in your child’s lunchbox, with the teacher? What sort of system will you use to communicate with the teacher about how many are needed for snacks and lunch?
By the time someone with cystic fibrosis reaches adolescence, they will hopefully have settled into a routine when it comes to managing their condition. Many of the differences at this age are related to your budding independence.
For parents, you want to ensure that your child is empowered and well equipped to face the challenges of the teenage years, and then into adulthood. For young people with cystic fibrosis , you are about to take part or full responsibility for your treatment, while at the same time you are emerging as your own person with your own ideas and ambitions.
During your teenage years, your cystic fibrosis clinic team will begin the process to move your cystic fibrosis care from a children’s hospital to an adult hospital. It’s a gradual and well-planned journey, with your health and well-being being the most important things.
Getting a job
In most cases, cystic fibrosis shouldn’t limit your choices when it comes to finding a part-time or full-time job. Some things to think about when looking for a job are:
Is it easy to get from your home and your cystic fibrosis clinic to your workplace?
What is the physical environment like at the workplace? Are there any obvious health risk issues such as bad air quality or dusty conditions?
What are the facilities like? Is there a kitchen with a fridge, well-maintained bathrooms and a rest-room or first-aid room? Would you have to climb stairs to get to work or is there a lift?
Does the employer offer flexible working conditions such as work-from-home or flexible hours?
What is the sick leave allowance, and (if you need it) would they let you have extra unpaid days off?
You have no obligation to disclose your cystic fibrosis when you have a job interview, and anti-discrimination laws make it illegal for employers to ask about any medical conditions. In fact, if you do not wish to disclose your cystic fibrosis at all – even after you get a job – then you don’t have to.
Having a family
Fertility for women with cystic fibrosis
Women with cystic fibrosis have anatomically normal reproductive tracts and normal hormonal function. The ovaries, the fallopian tubes and the uterus are all expected to be normal.
Women with cystic fibrosis usually have normal menstrual cycles and are able to become pregnant. Increasing numbers of adult women with cystic fibrosis are becoming pregnant and giving birth to healthy babies, with minimal impact on their health. If you are healthy, with stable lung function, pregnancy is safe. However, pregnancy puts additional strain on your body, and can be a risk, particularly if you are unwell or have poor lung function. It is recommended that you discuss your plans with your cystic fibrosis team, and that you are as healthy as possible before conceiving.
Fertility for men with cystic fibrosis
Men with cystic fibrosis produce sperm normally and their testes produce normal levels of testosterone. Testosterone is absorbed directly into the blood and is responsible for gains in muscle mass and sexual feelings. Men with cystic fibrosis have normal levels of testosterone and therefore normal sexual developmental and sexual function.
Approximately 98% of men with cystic fibrosis have problems with the development of the vas deferens, the duct that leads sperm from where it is produced in the testes to where it is stored prior to ejaculation. Therefore the ejaculate produced does not contain sperm. This means that most men with cystic fibrosis cannot conceive a child through sexual intercourse. Men with cystic fibrosis are still able to have biological children using assisted reproductive technology.
As well as sperm, semen contains fluid made by glands called seminal vesicles. In men with cystic fibrosis, these glands don’t function normally and are sometimes absent. For men with cystic fibrosis, the ejaculate will contain fluid produced in other parts of the reproductive tract. Even though the volume of fluid may be reduced, sexual function is not affected. Sex will feel the same as it does for men who don’t have cystic fibrosis; it will also feel the same for their partners.
It is possible to find out if you are fertile or not through a test called semen analysis. You can ask your cystic fibrosis team or your GP if you are interested in having this test. There is no right time to have semen analysis. Some people want to know if they are fertile in adolescence, and other people may choose to wait until they are thinking about having children.
Cystic fibrosis is a progressive condition so there may come a time when your lung function declines substantially. A lung transplant replaces diseased lungs with healthy donor lungs and is a treatment, not a cure. The main goal of lung transplants for people with cystic fibrosis is to extend life expectancy and improve your quality of life.
Australia has some of the best transplant results in the world and survival after a lung transplant is higher in Australia than anywhere else. Most patients report returning to a relatively normal life after their lung transplant.
Good mental health is an important part of treating cystic fibrosis, both for people living with cystic fibrosis and the people around them. Mental health impacts on physical health, and conversely physical health also affects mental health. You can’t treat one in isolation from the other.
Find out more about looking after your mental health here.
If your feel that you need to explore options for professional help to work through intense emotions and overcome obstacles, visit Beyond Blue.