Cystic fibrosis service — Canterbury

If you need to stay in hospital, you will be admitted to Ward 25 — the adult respiratory ward.

Our infection control policy requires each person with cystic fibrosis to be cared for in a single room. If you do not have a room with a bathroom, you will be allocated a toilet and shower to use during your stay. This is to prevent cross infection between CF patients.

What is provided

To help you feel at home the Cystic Fibrosis Association has provided:

• TV, video/DVD and fridge units
• laptop computer (available on request)
• free Wi-Fi internet access
• a telephone and separate extension number
• access to a DVD library (as well as a wide range of movies, we have DVDs of cystic fibrosis conference talks available).

Meals

The Catering Associates provide you meal options. As a cystic fibrosis patient with specific nutritional needs, you should get a form for ordering extras. If you do not get this, ask to see the dietitian.

Laundry

There is a washing machine and clothes dryer available. These are mainly for patients staying from out of town or who have limited social supports. Due to hospital infection control policy, you can only do hot washes (95°C).

Keeping fit

There is an exercycle in the corridor that you can use. You can also arrange to visit the hospital gym — ask your nurse or the physiotherapist.

Other facilities at Christchurch Hospital (internal link)

By the time you are an adult with CF, you will have heard many times about the need to maintain a good body weight.

Diet definitely does matter. Achieving and maintaining a good weight helps to maintain good lung function, which can result in less time in hospital.

A healthy body mass index (BMI) for someone with CF is between 22 and 25.

Dietary requirements

A person with CF may need 120% to 150% of the calories needed by someone without CF. This is due to things like:

• difficulty absorbing the nutrients from food
• increased energy needed to fight infection
• reduced appetite when you are sick
• increased energy needed to breathe if you have lower lung function
• losing fat in your stools, especially if you do not take enough enzymes.

If you are having trouble gaining or maintaining weight, our dietitian can:

• give you tips on maximising calories in your diet
• review your enzyme use so you get the most out of the food you are eating
• advise you on high energy supplement drinks.

If you are still unable to reach an ideal weight, you can have a gastrostomy tube (PEG) inserted to supplement your food intake.

A PEG can be used during the day or for overnight feeding while you sleep. It is used to supplement your food intake, not to replace what you usually eat.

How PEG tubes are put in

PEG tube

PEG tubes are usually put in by a specialist team in the Gastroscopy Day Unit. While you are sedated, the tube is placed into your stomach through the abdominal wall. You will have a longer tube in place until the incision site heals — usually about 6 to 8 weeks.

The long tube is then replaced with a low profile “button” as shown in the picture. Using a connecting tube, supplement drinks or feeds can go straight into your stomach.

Most people will use their PEG tube on a daily basis, or several times a week. When you have achieved your target weight, you may only use it as a back up if you get sick or lose weight.

If you think a PEG may be helpful for you, or are interested in finding out more, ask one of the CF team for more information.

Probiotics for cystic fibrosis

Probiotics may be helpful for people with antibiotic associated diarrhoea.

There are many different types of probiotics — some may be more helpful than others. Speak to us before starting them. They are generally safe and well tolerated but are not recommended in people who are immunocompromised, for example CF patients following lung transplant.

Currently in Canterbury the Cystic Fibrosis Association will fund short term probiotics if requested by the CF team. It is best to start probiotics at the same time as antibiotics.

Physiotherapists can help you manage your CF. It is not just about airway clearance, although that is an important factor.

Airway clearance

People with CF should do self-administrated clearance techniques every day. Your physio can help teach you the technique that is best suited to you and your lifestyle. This may include using a device to help with clearance.

Postural drainage and percussion

You will be placed in different positions to encourage mucus to drain from different parts of the lung. Chest clapping, or percussion, is added, along with deep breathing exercises, huffing and coughing to help clear mucus from the airway. Usually someone else needs to help you with this treatment.

Active cycle of breathing technique (ACBT)

This is a cycle of:

• breathing control
• chest expansion exercises
• huffing.

Breathing control and chest expansion exercises allow air to travel deep into the lungs and get behind thick mucus, which can then be coughed up by huffing.

Positive expiratory pressure (PEP)

PEP involves breathing out against resistance through a face mask or mouth piece. The resistance causes pressure to build up inside the lungs which stops the small breathing tubes collapsing. This allows air to get behind any mucus plugs and push them into a large airway where they can be coughed up. There are different types of PEP devices.

Exercise

Regular exercise is important to keep yourself well. There is evidence that decreased aerobic fitness and decreased muscle power, strength and endurance affects lung function.

Musculo-skeletal issues

Some people with CF can develop aches and pains due to poor posture and decreased flexibility. Your physiotherapist can help you learn to correct your posture and teach you how to maintain a good posture when coughing.

Pelvic floor issues

Advice on pelvic floor function is also an important area for CF, due to the repeated force with coughing. A weak pelvic floor means you can get leaking of urine from the bladder when you cough. The physiotherapist can assess and educate on bladder or bowel dysfunction.

Making informed choices about your sexual and reproductive health is important for everyone. There are some extra things that need to be considered when you have CF.

Men

Although 98% of males with CF are infertile, it is possible to have your own children. Infertility happens because the small tubes (called the vas deferens) that let sperm mix into the seminal fluid (ejaculate) become blocked by thick mucus, so sperm cannot pass out of the testicles.

This has usually happened by the time male babies with CF are born. However, with advances in fertility treatment, men with CF can have their own biological children through sperm aspiration and IVF.

Being infertile does not affect normal sexual function, it just means there will not be any sperm in your semen. If you are thinking about starting a family, having a semen analysis is the first step in the process. Talk to the CF team if you would like to find out more about starting a family.

Although you may be "shooting blanks" you should still practice safe sex (using a condom) to prevent catching sexually transmitted infections (STIs).

Women

Women with CF may have reduced fertility but are often able to conceive a baby naturally. Causes for reduced fertility can include:

• not ovulating (releasing an egg) or getting your period due to being underweight or sick
• thick cervical mucus can make it difficult for sperm to reach fallopian tubes.

If you do not want to get pregnant, you should use contraception. It can be a bit more complicated choosing what sort of contraception to use if you have CF. Talk to your CF team if you have questions about which contraception method is best for you.

Starting a family

The decision to start a family is a big one. There are lots of things to consider including:

• your overall health
• the supports you have available
• your partner's CF carrier status — this can be checked with a blood test. The CF team will refer you to a genetic counsellor to talk about this
• your lung function — pregnancy is not recommended if your lung function is below 50%
• reviewing your medication to make sure the meds you are taking are safe during pregnancy.

If you are unable to conceive a baby naturally, the New Zealand government offers funding for up to 2 cycles of IVF for eligible couples. Talk to the team if you have any questions about becoming a parent.

Fertility — Cystic Fibrosis Trust UK (external link)

Travelling with a medical condition like CF means that you may need to do some extra preparation and planning before heading away.

Some travel destinations present unique challenges for people with CF, for example:

• countries with hot climates
• some tropical regions.

Discuss your travel ideas with the CF team before booking your trip.

Travel insurance

It can be difficult and expensive getting travel insurance that covers CF. You may want to consider travelling to countries that offer reciprocal health care to New Zealanders, such as Australia and the UK.

Medication and equipment

We recommend you keep a couple of days' worth of medication in your hand luggage in case your bags go astray. If you take nutritional supplements, speak to the CF dietitian about the best way to arrange a supply of these while overseas.

We can provide a letter to show customs and security about any medication or equipment you will have with you, including syringes, needles or nebulisers.

Oxygen for the plane

If your oxygen saturation at sea level is 94% or below, it is likely you will need oxygen on the plane.

If you do need oxygen, the airline you are travelling with will require a medical certificate from the CF team, stating that you are fit to fly and how much oxygen you need. Some airlines provide oxygen (at a cost) and most will allow you to carry an airline approved portable oxygen concentrator that runs off a battery to deliver oxygen while in flight.

It is really important to talk to the CF team before booking your flights to see whether you may need to fly with oxygen. Sometimes the cheapest airline ticket may not end up so cheap if the airline charges a lot for oxygen.

• Official NZ travel information — Safe Travel (external link)
• Travel for people with CF — Cystic Fibrosis New Zealand (external link)