Cystic Fibrosis Service

This site has been designed to give you information about Canterbury Adult Cystic Fibrosis Services. There is also lots of information about CF, CF issues, and links to other useful websites.

If there is anything you can’t find and would like to know please email us and ask.

  • Cystic fibrosis (CF) is an inherited condition that affects more than 500 people in New Zealand.
  • The CF gene is carried by 1 in 25 New Zealanders.
  • Having one CF gene alone does not cause any disease. In order to have CF a person must inherit a CF gene from both parents.
  • Most people with CF are diagnosed as babies, however some people with a milder form of the disease may not be diagnosed until they are older, or even as adults.

  • CF affects different parts of the body, but mainly the lungs and pancreas, clogging them with thick sticky mucus.
  • In the lungs the sticky mucus is an ideal place for bacteria to live. The bacteria can then cause repeated chest infections which damage the lungs
  • In the pancreas, the mucus blocks the tubes that release enzymes used to digest food in the gut. This can make it difficult for people with CF to put on weight and get good nutrition from their food.

Treatment is aimed at reducing the impact of CF on the body by treating chest infections, chest physiotherapy to help people cough up the mucus in their lungs, exercise to keep fit, and nutritional advice and support to keep as healthy as possible.

The Canterbury Adult CF Service sees all adults with CF from around the South Island. We are the second largest adult CF service in New Zealand. Our team consists of: Physicians, Clinical Nurse Specialist, Pharmacist, Clinical Psychologist, Social Worker, Dietitian, Physiotherapist, Community Physiotherapist. View the profiles below to find out their role in your care.

Mission Statement

In partnership with our patients, the Canterbury adult cystic fibrosis service works to optimise the health and quality of life of adults with CF by providing high quality evidenced-based care.  

Cystic Fibrosis Physicians

Dr Richard Laing

As the lead physician responsible for the Christchurch Adult CF Programme, Richard Laing supervises the overall management of individuals with CF. He coordinates both inpatient and outpatient care and forms part of the multidisciplinary team.

Richard’s aim is to offer thorough but individualised approach to CF care with close cooperation with members of the multidisciplinary team and other professionals as indicated.

Dr Bronwen Rhodes

Bronwen Rhodes is a respiratory physician with an interest in cystic fibrosis. She completed a one year CF fellowship in Toronto in 2008. Bronwen is involved in both inpatient CF care and in the CF outpatient clinics.

Dr Greg Frazer

Greg Frazer has a background in Respiratory Medicine and has worked with people with CF in a large transplant service in Australia. Greg attends the CF outpatient clinics, and also looks after the care of CF inpatients on a rostered basis along with Richard and Bronwen.

Cystic Fibrosis Clinical Nurse Specialist (CNS)

Robyn Baird

Robyn is the CF Clinical Nurse Specialist (CNS). You will see her at your CF outpatient clinics and if you have an admission to hospital. Robyn is available to talk with you, can provide education on a variety of topics and supply you with any equipment you may need for your CF care. She also does home visits to support patients when they are managing IV treatment at home. If you have any concerns about your CF or feel unwell between appointments, please contact Robyn as detailed below.

Robyn works from 7.30am – 4.00pm Monday to Friday, and can be contacted by phone or text on 027 213 8087 or by email: robyn.baird@cdhb.health.nz

Pharmacist

Connie Takawira

The CF pharmacist is available to discuss medication-related issues with you during hospital admissions. Topics you may wish to discuss include:

  • The reasons behind using different therapies

  • Any concerns you have (for example, side effects or if you do not think our medications are adequately controlling your symptoms)

  • Prescription fees

The hospital provides your medications during admissions. However, please bring in 48 hours supply to allow time for supplies to be dispensed from the pharmacy. You will also need to bring in the equipment you use for nebulising any medication.

Many patients choose to self-medicate while in hospital. However, if you are very unwell, or your medications are being adjusted rapidly, it may be more appropriate for the nurses to manage your tablets for you.

Physiotherapist

Trish Goulter

Physiotherapy is aimed at maximising your physical function by providing ongoing assessment, treatment and rehabilitation.
Physiotherapy can help with

  • Sputum clearance

  • Breathing pattern disorders e.g. hyperventilation

  • Exercise testing and advice

  • Musculoskeletal concerns/posture

  • Pelvic floor dysfunction

Treatments may include:

  • Breathing exercises

  • Manual chest clearance techniques (percussions & vibrations)

  • Exercise/walking/gym programmes

  • Providing respiratory devices (acapella, PEP)

  • Relaxation

The physiotherapists on the respiratory ward will see you every day during an admission. We have a large team at Christchurch Hospital; you may meet more than on physiotherapist during your stay. Trish will see you at your clinic appointments and is available to provide advice and education in any of the areas listed above.

Dietitian

Tory Crowder

Tory will see you at outpatient clinics, when you are admitted to Ward 25 and can arrange to see you at home. Tory will discuss and arrange an appropriate diet with you, and give you practical recommendations on how to achieve and maintain an ideal body weight.

She is available to discuss the following topics with you:

  • Enzyme therapy

  • Symptom control such as poor appetite or nausea

  • Dietary intake

  • Bone Health

  • CF related Diabetes

  • Gastro-oesophageal reflux

  • Nutrition support (supplement drinks or gastrostomy feeding)

  • Vitamins and minerals

  • Hydration

  • Dietary considerations when travelling

Tory can be contacted at Nutrition Services, Christchurch Hospital – ph:027 775 6033.

Social Worker

Gary Sword

The social worker is available to see you during hospital admissions to Ward 25, by appointment in their office or at an arranged place in the community. Their role is to assess the impact medical needs or regimes may be having on you, your family or friends. They can assist people with CF in being supported practically and emotionally so they remain independent and reach their potential goals.

They are available from 8am – 4.30pm Monday to Friday (there is an after hours social worker available for urgent matters). Please feel free to contact the social worker on phone 03 364 0420.

Psychologist

Sandie Young

Sandie is part of the CF Team. When people have a physical condition like CF, difficulties can occur in doing everyday things and in relationships with family and friends. Sometimes your battery can run flat and you can feel frustrated, unmotivated or down. Occasionally this can lead to more problems such as depression.

Psychologists use “talking therapy” to support you and help you find ways to manage and build coping strategies. They can also act as a sounding board or a safe place to vent outside of your usual support network. They can also help identify any symptoms of depression and anxiety and explore ways with you to best manage these.

Sandie can visit you on Ward 25 or more commonly she will see you at an outpatient appointment. If you would like to see the psychologist you can ask any of the CF team to contact Sandie.

Community Physiotherapist

Hilaire O’Dea

Community physio aims to maximize lung function by providing ongoing advice and treatment in the home environment. Often CF physio care needs to be adapted to fit home and lifestyle requirements. Treatments can include airway clearance, exercise and gym programmes.

Hilaire can visit all those in Christchurch and surrounding areas and can be contacted on 021 133 5194.

Outpatient clinics are held on Thursday afternoons in the Hagley outpatient building indicated as “New Outpatients” below. [outpatients.jpg]

Outpatients living in Canterbury

If you live in the Canterbury area, we will try to see you every 3 months. Frequency of your appointments will depend on how your health is.

Your Cystic Fibrosis clinic appointment is multidisciplinary – this means you won’t just see a Doctor, but also the Cystic Fibrosis nurse specialist, dietitian, and physiotherapist.

Sometimes there may also be medical or nursing students at the clinic too.

You will also be asked to do a breathing test before each clinic appointment.

Breathing tests are done in the Respiratory Laboratory on the 4th floor of the Riverside building (see map below).

[Resp lab location.jpg]

Your annual review

Once a year, an annual review appointment will be scheduled in place of a regular clinic appointment. This is your yearly “warrant of fitness,” and will take longer than a routine clinic appointment. You will get an annual review pack in the mail when your review is due which will have extra information in it regarding this appointment.

If you live outside of Canterbury

Your Cystic Fibrosis care will be shared between our service and a local doctor/hospital. We may be able to arrange to see you via a video link, but we also like to see you in person in Christchurch at least once a year.

If you live more than 300km away, funding for you and a support person to travel to Christchurch is paid for by the Ministry of Health.

You need to contact your local hospital to have this arranged. We will try to get your clinic appointment letter out to you at least 4 weeks in advance so you can book flights/arrange your transportation for your clinic visit.

Being admitted into Ward 25

If you need an admission to hospital, you will be admitted to ward 25 which is the adult respiratory ward.

  • Ward 25 has 27 beds, including 5 single rooms.

Our infection control policy requires each person with Cystic Fibrosis to be cared for in a single room. If you don’t have a room with an ensuite 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)
  • Internet access via free wireless internet
  • Each single room has a telephone and separate extension number
  • Access to 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 an additional sheet may be provided for ordering extras, etc.  If this has not been provided, please ask to see the dietitian.

Approximate meal times are breakfast 7.25am, lunch 12.45pm and dinner 5.25pm.

Laundry

There is a washing machine and clothes dryer available to use. These are primarily provided for patients staying from out of town or for those with limited social supports. Please be aware that due to hospital infection control policy, only hot washes (95oC) are permitted in the washing machine.

Keeping fit

An exer-cycle is available for your use which sites in the corridor. You can also arrange to visit the hospital gym. please ask your nurse or the physiotherapist.

Christchurch Hospital Information Booklets are available. If you don’t get a copy, please ask for one. This booklet explains other hospital facilities, e.g. hairdresser, bank, café, etc.

Probiotics for Cystic Fibrosis

  • Probiotics may be helpful for people with antibiotic associated diarrhoea.
  • Probiotics are thought to alter the gut bacteria mainly by reducing the growth of bad bacteria. This can improve the immune response and gut function as the bad bacteria are stopped from invading the gut lining.
  • Probiotics come in many different strains and names and have different ability to colonise the gut as well as resistances to bile and stomach acid.
  • Probiotics are generally safe and well tolerated but are not recommended in patients who are immunocompromised e.g. 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 recommended that probiotics are started at the same time as the antibiotics.

Maintaining a good body weight

By the time you get to be an adult with CF, the need to maintain a good body weight is something you will have heard over and over again.

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 22-25.

Dietary requirements

A person with CF may need up to 120%-150% of the calories needed by someone without CF. This is due to an increased energy expenditure and a number of different reasons.

  • They may have difficulty absorbing the nutrients from food and so need to eat more
  • When infection is present the amount of energy someone with CF needs increases even more 
  • When you are sick you may not feel like eating or have a reduced appetite, and will be more likely to loose weight
  • The lower a persons lung function is, the higher their body’s energy requirements become due to the increased work of breathing

High calorie diet

Why can a high calorie diet be hard to achieve? 

  • Many people with CF experience reduced appetite, especially during infection
  • Forgetting or taking too few enzymes will result in some fat (energy) being wasted  in your stools. 
  •  Even if you are taking your enzymes appropriately you will continue to lose some fat in your stools and this contributes to your increased energy requirements.

In the case of maintaining a good body weight, the answer is very simple. If you want to stay well for longer and you want to stay out of hospital, then work at keeping up your weight so that your body can fight off the infections that are bound to hit you from time to time.

Gastrostomy Tube

What is a PEG?

A gastrostomy tube (PEG) is used by people with CF (PWCF) to supplement their food intake. This can help to maintain or improve your weight and achieve good nutrition.  

Maintaining or gaining weight to reach a healthy BMI.

Some people find it difficult to maintain or gain weight to reach a healthy BMI. If this is the case, meeting with the dietitian can:

  • Give you tips on maximising the amount of 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

Reaching an ideal weight

If you are still unable to reach an ideal weight then having a PEG inserted can take the pressure off and help you take in the extra energy you need. A PEG might be a good idea if:

  • You are unable to gain weight by improving your diet and using oral nutritional supplements

  • You find your appetite is low

  • You often vomit from coughing

  • You are unable to regain weight that was lost when you were sick

  • You are trying to put on weight for lung transplant

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 are PEG tubes put in? 

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. Initially you will have a longer tube in place until the incision site (stoma/tract) has healed completely (6-8 weeks).

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

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

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

More information on CF and nutrition

If you would like to find out more about CF and nutrition, the websites below have some useful information.

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

Airway clearance

People with CF should do self-administrated clearance techniques every day, and your physio can help with teaching the technique that is best suited to you and your lifestyle. This may include using a device to help with clearance such as PEP or an Acapella™. However, manual techniques are also useful when you have an infection and the physiotherapist will be involved in these situations.

Some of the common types of airway clearance are listed below

Postural drainage & percussion

The person is placed in a variety of positions which encourages 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 is needed to help you with this treatment.

Active cycle of breathing technique (ACBT)

This is a cycle of breathing control, chest expansion exercises, and 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. The ones we use most commonly are shown below.

[image]

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.

Your physiotherapist is here to help all of this by assessing and giving appropriate advice. Exercise testing, such as the 6 Minute Walk Test, is a tool we use for determining exercise capacity or limitation.

Musculo-skeletal issues

postures.jpgSome people with CF can develop aches and pains due to poor posture and decreased flexibility. Back, shoulder, neck pain and headaches can develop because of poor posture related to coughing. Your physiotherapist can help you learn to correct your posture, and teach you how to maintain a good posture when coughing.

[image]

Other 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.

Fertility and CF

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. Below is some general information about fertility, sexual health and CF.

There are also some links to some other sites that have helpful information about CF, sexuality and relationships.

Men

Male-Symbol.jpgDid you know that while 98% of males with CF are infertile, they can still have their 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 can’t pass out of the testicles.

Vas deferens.jpg

This has usually happened by the time male babies with CF are born. The good news is that with advances in fertility treatment it is possible for men with CF to have their own biological children through sperm aspiration and IVF.

Being infertile doesn’t affect normal sexual function; it just means there won’t be any sperm in your semen. If you would like a semen analysis to check your fertility, please ask the CF team to arrange this for you.

If you are thinking about starting a family, having a semen analysis is the first step in the process. Please talk to the CF team if you would like to find out more about starting a family.

It is important to remember that although you may be “shooting blanks” you should still practice safe sex (using a condom) to prevent catching sexually transmitted infections (STIs).

STIs include: Chlamydia, HIV, Hepatitis, Thrush, Gonorrhoea, Syphilis, Genital Warts.

Women

Female symbol.jpgWomen with CF may have reduced fertility, but are often able to conceive a baby naturally. Causes for reduced fertility can include

Not ovulating (getting periods) due to being underweight or if sick
Thick cervical mucus can make it difficult for sperm to reach fallopian tubes
Because natural fertility is possible in most women with CF contraception should be used. It can be a bit more complicated choosing what sort of contraception to use for women with CF because of frequent antibiotic use and other problems. The table below gives an overview of some of the contraceptive options available in New Zealand, their pros and cons. Please talk to your CF team if you have questions about which contraception method is best for you.

Contraception Method Effectiveness Pros Cons
The Pill 96 – 98%
  • Easy to take
  • Can improve PMS
  • 12 hour window if you forget
  • not effective if on antibiotics
  • not suitable if you have liver disease
  • not suitable for severe CFRD

Mini Pill

(cerazette)

98%
  • OK with broad spectrum Abs
  • Suitable for CFRD
  • 12 hour window
  • Cerazette not funded. Cost $50-90 for 6 months

    Other mini pills (noraday, microlut) are funded/part funded butmust be taken at same time each day (3hr window)

Depo provera (injection) 99%
  • No pills to take
  • May reduce or stop periods
  • Not affected by ABs
  • May reduce bone density therefore not recommended
  • Can take up to 2 yrs to regain fertility
Implant 98%
  • Not affected by ABs
  • Not thought to affect bone density
  • Lasts for 3-5  years
  • Small procedure under local anaesthetic to be fitted and removed
  • may get irregular bleeding
IUD (mirena) 99%
  • Not affected by ABs
  • Can last up to 5 years
  • May stop periods
  • Can be uncomfortable when first placed
  • may get irregular bleeding
Condoms (male or female) 85-98%
  • Some protection against STIs
  • Higher failure rates than other methods

Starting a family

The decision to start a family is a big one. There are lots of things to consider including your overall health, and the supports you have available. Your partner should be checked for their CF carrier status (a blood test) to determine if there is a risk of having a baby with CF. The CF team will refer you to a genetic counsellor to talk about this.

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.

Please talk to the team if you have any questions about becoming a parent.

CF and pregnancy

It is very important to speak to the CF team before considering pregnancy. For women whose lung function is greater than 50% predicted, there is no reason you won’t be able to have a safe and health pregnancy.

Pregnancy is not recommended if your lung function is below 50%. This is because of the risks to your health that could occur by being pregnant.

There are a number of “boxes to tick” before trying for a baby including reviewing your medication to make sure the meds you are taking are safe during pregnancy.

Useful information

Below are some links to more information on sexual health and fertility:

Canterbury Cystic Fibrosis (CCF) Adult Patient Advisory Group

The aim of CCF Adult Advisory Group is to:

  • act as an advisory resource to the Canterbury CF Adult Service
  • collaboratively work with the CF team to actively participate in the development of new programs and review recommendations
  • shape a service that is patient centred, efficient and cost effective

CF patient members of this group are available to be contacted at any time if you wish to have a chat, raise some areas of concern that you want us to bring to the CF team’s attention or feed ideas/thoughts of how things could work better for you. Your input is important in helping us all work together to make a great service even better. The Chairperson of the Patient Advisory Group is Lisa Borkus. Lisa can be contacted via email on lborkus@gmail.com

Cystic Fibrosis Association of New Zealand

The Cystic Fibrosis Association of New Zealand provide support and information for people with Cystic Fibrosis and their families. Please follow the ink below to go th their site.
CF Association of NZ

Planning your holiday

Travelling overseas is an exciting prospect – it’s a big world out there with lots to see! Travelling with a medical condition like CF means that you may need to do some extra preparation and planning before heading away on that trip, to make sure you have a fun, hassle-free time away.

Some travel destinations present unique challenges for people with CF (PWCF) such as travelling in countries with hot climates, and some tropical regions, so please discuss your travel ideas with the CF team before booking your trip.

Travel Insurance

There can be some difficulties for PWCF obtaining travel insurance that covers CF. If you are able to get cover for CF related problems as well as general travel insurance it can be reasonably expensive.

For this reason, you may want to consider travelling to countries that offer reciprocal health care to New Zealanders. At present these countries are Australia and the UK. There is information on what is covered by these agreements on the Ministry of Health website.

Medication and Nutrition Supplements

When travelling, we recommend you keep a couple of days worth of medication in your hand luggage in case your bags go astray! That way you will have medication available and time to either get more meds, or for your bags to turn up without running out. If you take nutritional supplements, speak to the CF dietitian about the best way to arrange a supply of these while overseas. Travel can be demanding and tiring and keeping ahead with your nutrition can help you stay energised and well during your holiday.

Your CF team can provide you with a letter to show customs & security regarding the medication you will have with you. This is especially important if you have syringes/needles, or equipment such as nebulisers with you.

Travel Vaccinations

Depending on where you are travelling, it may be recommended that you have vaccinations prior to travelling. Some vaccines need to be given well in advance of your travel, so it pays to get onto this early. Your GP can advise you what vaccinations are recommended for the countries you are travelling to. Travel vaccinations are not funded.

Might you need Oxygen when flying?

When flying on aircraft, there is less oxygen available in the air in the cabin – 15% oxygen compared with 21% oxygen when on the ground. For most people with normal oxygen saturations, this presents no difficulty. If your oxygen saturation at sea level are 94% or below, it is likely you will need oxygen while on the aircraft at altitude.

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!

Useful Sites

Official NZ Travel Information
CFNZ Website – Travel for people with CF

Page last updated: 22 October 2018

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