Personal Details

PERSONAL DETAILS

Hospital

Name

Date of birth

Telephone contact

Unit number:

If found please contact

NORTH WEST MIDLANDS CYSTIC FIBROSIS CENTRE Children’s Outpatients, Ground Floor, Royal Stoke University Hospital, Newcastle Road, Stoke on Trent ST4 6QG

E-mail: [email protected]

THE NORTH WEST MIDLANDS CF TEAM

Medical Team Professor Warren Lenney - Consultant Paediatrician Dr Fran Gilchrist - Consultant Paediatrician Dr Ang Ho Dr Martyn Rees - Consultant Paediatrician Dr Senthil Kumar Sethuraman Admin Team Erin Hodgetts - Co-ordinator Angela Black - Secretary Rachel Davies - Secretary Carol Binsley - Secretary Specialist Nurses Siobhan Davies Kelly Bakewell Belinda Gower Physiotherapists Zoe Rushton Christine Law Dieticians Sue Bell Hannah Joscelyne Clinical psychologists Dr Ruth Fishwick Dr Clair May Welfare Advisor Joanne Frain Community nurses Angela Malkin Heather Parr Rachel Lugg Tash Grocott Clinical Care Advisor (CF Trust) Dominic Kavanagh

NORTH WEST MIDLANDS CYSTIC FIBROSIS CENTRE Children’s Outpatients, Ground Floor, Royal Stoke University Hospital, Newcastle Road, Stoke on Trent ST4 6QG E-mail: [email protected] Contact Information

ALL TEAM MEMBERS IN STOKE ON TRENT CAN BE CONTACTED ON: 01782 675105 Between 8.30am – 4.30pm, Monday to Friday. If there is no answer, leave a message on the answer machine. If it is urgent, contact switchboard (01782 715444) and ask the operator to put your number on the bleep to save you holding on. (There may be occasions when the team member will not be able to respond immediately to your bleep, but will do so at the earliest opportunity). Out of hours and weekends, contact children’s ward.

ADMIN TEAM Erin Hodgetts Co-ordinator Ange Black Secretary CHILDREN’S TEAM Professor Warren Lenney Consultant Paediatrician Dr Fran Gilchrist Consultant Paediatrician Dr Ang Ho Consultant Paediatrician Siobhan Davies Clinical Nurse Specialist Kelly Bakewell Support Nurse Sue Bell Advanced Specialist Dietician Zoe Rushton Advanced Specialist Physiotherapist Dr Ruth Fishwick Psychologist 01782 408354 Ward 216 01782 6(75216) 01782 6(76216) Community Team 01283 504 867 (patients in Burton area) SUPPORT AND ADVICE Joanne Frain Welfare Advisor for Cystic Fibrosis Dominic Kavanagh Clinical Care Advisor (CF Trust) 02084647211 PHARMACY Dispensary 01782 6(74597) Manufacturing 01782 6(74579) Lloyds 01782 6(74591)

ALL TEAM MEMBERS IN SHROPSHIRE CAN BE CONTACTED ON: SHREWSBURY (COMMUNITY) 01743 450855 TELFORD 01952 565985 Between 8.30am – 4.30pm, Monday to Friday. If there is no answer, leave a message on the answer machine. If it is urgent, contact switchboard (01952 641222) and ask the operator to put your number on the bleep to save you holding on. (There may be occasions when the team member will not be able to respond immediately to your bleep, but will do so at the earliest opportunity). Out of hours and weekends, contact children’s ward 01952 565919.

ADMIN TEAM Rachel Davies 01952 565985 Carol Binsley 01952 641222 Ext:4183 CHILDREN’S TEAM Dr Martyn Rees Consultant Paediatrician Dr Senthil Kumar Sethuraman Consultant Paediatrician Rachel Lugg Community Nurse Tash Grocott Community Nurse Belinda Gower Specialist Nurse Christine Law Physiotherapist Hannah Joscelyne Dietician Clair May Clinical Psychologist SUPPORT AND ADVICE Joanne Frain Welfare Advisor for Cystic Fibrosis 01782 675105 Dominic Kavanagh Clinical Care Advisor (CF Trust) 02084647211 PHARMACY Hayley Atkin Pharmacist

Personal Hand Held Record

This is your own personal record which contains information about you and your cystic fibrosis. The record can be used by you, your parents, carers and members of your cystic fibrosis team.

You decide what information you want to keep in it and who you would like to read it and who you would like to write in it.

The record has been designed to hold all the information that you should hopefully need.

You may find the record useful if you are seeing your own doctor as he/she can see what treatments you are taking and the doctor can write down any changes needed. You may wish to take this record on holiday with you. Should you become ill, you will then have an up-to-date record of your cystic fibrosis confirming all your medications and other treatments.

You and your family are now under the care of The North West Midlands Cystic Fibrosis (CF) Centre which is a designated Centre providing a full service for patients with CF. The centre covers a wide area of Staffordshire and Shropshire. (Map over leaf). A small number of patients from Wales and Cheshire also use the centre. Services include in-patient, out-patient and support from community and hospital at home teams. The CF team is based at the Royal Stoke University Hospital with a supporting team based in Shropshire. There are community teams in Shropshire, Stoke on Trent, Stafford and Burton who also support the teams. We are fortunate here in Stoke on Trent that our adult CF team service is based on the same site as the Children’s and therefore young persons from the age of 11 years will be introduced to the adult team and seen regularly until they transfer to the adult team at an age of their choice between the age of 16 years and 18 years. The team will support you through this period and will provide you with the transition delivery document. For children living in Shropshire, the adult team come to Shrewsbury on the 1st Thursday of every month and so children/teenagers living in the area will meet the team there. All patients with CF in the North West Midlands will be offered one of these records so that if any of you are attending appointments in any hospital or general practice there will be consistency of information available. Additional information will be given along the way and will include transition information for the adult service.

Map of the area the North West Midlands CF Team cover

Cheshire Royal Stoke University Hospital Staffordshire

Queens Hospital

Royal Shrewsbury Hospital Princess Royal Hospital Shropshire

Contents

Acknowledgments How to use your Personal Hand Held Record Section One - Information Sharing Day to day diary Information to and from your team Appointments Section Two - Medication List of medicines I am taking Drug allergy and reactions Oral antibiotics Intravenous antibiotics Vaccinations Section Three - Personal Planning Ages and Stages Personal Plan Care Pathway Plan from 0-18 years (including plan around transition to adult care) Section Four - Information Section Information about my tests and my investigations I have had. Section Five - Growth and Nutrition Growth and Nutrition Section Six - Respiratory Physiotherapy, Lung Function Results, Equipment including nebulisers Loaned equipment Section Seven - General Information General Information Holidays, travel insurance Schools, University Housing Benefits Other supporting groups Section Eight - Glossary Glossary

Acknowledgments

Thanks to the children and parents who have provided us with a list of what they would like to see in their personal record and for using the record over a 6 month period providing us with really helpful comments and suggestions.

Thanks also to the Cystic Fibrosis Trust for giving permission to use their material.

A big thank you to the Bloxwich Golf Club whose members have raised the money to fund the record (The Lady Captain at the time was Alveen, grandmother of Evie Pritchard)

The North West Midlands Cystic Fibrosis (CF) Centre is a designated Centre providing a full service for patients with CF living in and nearby North Staffordshire (including Stoke-on-Trent, Burton, Stone and Stafford) and in Shropshire. Services include in-patient, out-patient and services in the community setting. A small number of patients from Wales and Cheshire also use the Centre’s services. All patients with CF in the North West Midlands will be offered one of these records so that if any of you are attending appointments in any hospital or general practice there will be consistency of information available.

How to use your personal record

These pages provide you with general information, results of investigations, charts showing your height and weight. Information also about your CF, its management, its treatment and a list of equipment you may need.

If you don’t feel certain pages are useful, they can be easily removed. They can be re-inserted if needed at a later date.

Section One

Section One - Information Sharing

• Day to day diary

This section allows you and those people caring for you, to record about how you are feeling and how your CF is affecting you.

You are able to choose who writes in these pages and who you show them to. They are for you to keep your thoughts and feelings and will help people understand how you are between visits.

• Information to and from your team. This section is for you to list questions for members of the team and for them to record their answers.

• Appointments Here you can list your appointments with the team.

Diary

Date Signature Diary

Date Signature Things to ask my CF Team Answers and Plans Things to ask my CF Team Answers and Plans Clinic Appointments

Type of Clinic Dates Location Clinicians Name

Section Two - Medication Section Two

• This section includes details of your medicines and when you should take them. It will also tell you when you started each treatment and when or if changes are made. You will also be able to insert details of the medicine and devices you may have with instructions on how to use them.

• You can also keep a record of courses of intravenous antibiotics and record any problems you have.

• In this section you can keep a record of any drug allergy.

• You can keep a record of any vaccinations you receive.

I am allergic to these medications

Date Medicine Comments and side effects I am allergic to these medications

Date Medicine Comments and side effects

CYSTIC FIBROSIS ORAL ANTIBIOTIC PREVENTION DOSAGES

0 6 6 24 Weeks Months Months

AUGMENTIN DUO 0.3 ml/kg Suspension: 400/57mg/5ml OD

AZITHROMYCIN 10mg/kg Capsules: 250mg. Three times per week Suspension: 200mg/5ml

FLUCLOXACILLIN 125mg Syrup: 125mg/5ml and 250mg/5ml BD

TRIMETHOPRIM 2mg/kg 25mg 50mg Suspension: 50mg/5ml BD BD BD

OD – once daily BD – twice daily

Intravenous (IV) antibiotics regimes

Times No. of Start date Antibiotic name Dose Comments daily days Intravenous (IV) antibiotics regimes

Times No. of Start date Antibiotic name Dose Comments daily days Vaccinations

Yearly Flu Pneumococcal

*Infuenza(Flu) – every year Pneumococcal (Pneumovac) every 5 -7years

Section Three - Ages and Stages

• Guide for your visits • Ages and stages The following pages are a guide for families how to check if they have all the help and information they need to bring up happy and healthy children with cystic fibrosis. Section Three

• Onwards and upwards

From the age of 11 years, you will be provided with a transition document very similar to your Ages and Stages sheets you have been using already. The adult team will also be introduced to you and attend some of your clinic visits. The transition document Ready steady Go will help you prepare for the future and plan your transition into the adult service. The paediatric team will support you in this process as they have done all through your childhood.

General CF Guide

Guide for your visits

AGES AND STAGES:

Living and Growing up with Cystic Fibrosis

Developmental Education for use with Parents and Patients living with CF.

Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

Initial Diagnosis Initial Diagnosis

 Discuss Newborn screening process

 Understanding the genetics of Cystic Fibrosis

 Basic review of Cystic Fibrosis

 Other test results (e.g.faecalelastase)

 CF Clinic visits – frequency of visits and purpose

 Team approach to CF

 Getting information – tips for ‘safe’ websites (e.g. Cystic Fibrosis Trust)

 How to contact your CF Centre

 Sharing the diagnosis with family members

Comments/Questions Parents/Guardians Professionals

Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

First follow up visit after diagnosis First follow up visit after diagnosis

 Fitting CF into everyday life

 Enjoying your baby’s normal growth and development

 Understanding the respiratory system - Introducing physiotherapy

 Understanding the digestive system - Creon

 Aims of the outpatient management

 How to give your baby the best start - Good infection control - Smoking

Comments/Questions Parents/Guardians Professionals

Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

SecondSecond followfollow-up- upvisit visit

 Opportunity for you to ask questions

 Monitor growth and development

 Review physiotherapy

 Creon and nutrition

 Fitting CF into everyday – balancing the needs of a new baby, CF and your family

 Port CF database consent and discussion

 First point of call

Comments/Questions Parents/Guardians Professionals

Good infection control – hand washing, hand gels, avoid ill people Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

Up to 2 years of age visits Up to 2 years of age visits

 Feeling confident - Physiotherapy - Nutrition and Creon - Medication

 Balancing CF and family life

 How a diagnosis of CF affects you and other family members

 Annual review discussion, routine and tests. Tests to be done 2 months prior to the annual review appointment.

 Positive parenting

 Sharing information about treatment with family members

Comments/Questions Parents/Guardians Professionals

Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

2 to2 to 4 4 years of ageof age

 Discuss the importance of continuing physiotherapy - Identifying respiratory symptoms - Nutrition and Creon - Medication

 Nutrition - Mealtime routines - Creon doses

- Identifying malabsorption

 Involving your child in his/her CF care

- Teaching your child the basics about all treatments. - Introducing child’s participation in airway clearance

 Child care concerns

 Helping to set limits and boundaries with your child

 Getting prepared for school

Comments/Questions Parents/Guardians Professionals

Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

5 to5 to7 7 yearsyears of ageof age

 Child’s understanding and independent use of physiotherapy treatment: - Airway clearance - Respiratory symptoms - Pulmonary function tests

 Child’s understanding and independence of nutrition: - Creon - Malabsorption

 Child can talk about how he/she feels when asked and and can discuss with CF health professionals

 Child to actively take part in sputum clearance and airway clearance e.g. incentive spirometer, bubble games.

 Child and parents learning about CF medicines and what they do.

 Setting limits and boundaries with your child.

 School issues - Telling teachers and friends about CF - Taking Creon at school

- Preparing for school lunch and snacks

- School trips - Toilet needs - Water and salt needs at school

Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

Comments/Questions Parents/Guardians Professionals

Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

8 to8 to 12 12 yearsyears of ageof - ageCHILD CHILD

 Begin taking part more actively in clinic visits, answering independently.  Explaining CF to friends.  Continue taking Creon without help from others

 Understanding why some people have CF and others do not  Basic nutrition: - Continue to help child understand nutrition and malabsorption - Respiratory symptoms  Encourage child to talk about symptoms without being asked.

CHILD AND PARENT

 Knowing the names of the medicines and the reasons for taking them.  Discuss options for airway clearance.

 Exercise and sports including salt and fluids for physical activity.  Importance of lung health.  Coping with a lifelong chronic illness – identifying anxiety and depression.  School issues: - Friends - Meals/snacks - Toilet use - Activities

Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

Comments/Questions Parents/Guardians Professionals

Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

13 years13 years of of age age and and upwards upwards

From the age of 13 years, you will be provided with a transition document very similar to your Ages and Stages sheets you have been using already.

The transition document will help you prepare for the future and plan your transition into the adult service. The paediatric team will support you in this process as they have done all through your childhood.

Adapted from Kecia Nelson - Ages and Stages University of Missouri CF centre Amended by the North West Midlands Cystic Fibrosis Centre 07/08/2012

Onwards and Upwards!

The teenage years are a time to become more independent and responsible for the things you do. Looking after your health is just one of these things. To do this you will need information about your CF and the chance take over some of the care you rely on your parents or the CF team for. Around your 11th birthday we will give you some information that will help you prepare, plan and move from the children’s services to the adult services. Members of the adult team will also attend some of your clinic appointments which means you will get to know them which will prepare you for when you move across between your 16th and 18th birthday. We are fortunate the adult CF service is in the same building and work very closely with the children’s team. When you are ready to move across the children’s team will still be there to support you and your family e.g. will attend some of your clinic appointments, visit you if you were to become an inpatient and remember we are only a phone call away.

Section Four - Investigations you may need

• Please do ask a member of the CF team if you need more information. • Keep record of which investigations that you have had. Section Four

Tests

Below is brief information about some tests/investigations which may be needed. If you would like more information please ask your CF nurse for a handout which can be added into your record.

Newborn screening Needs a few drops of blood from baby’s heel on day 6 or7. A number of diseases are screened for including cystic fibrosis.

Sweat Test This measures the amount of salt (usually measured as chloride) in baby’s sweat. A very small amount of sweat is collected from the arm. In cystic fibrosis the salt level is high.

Measuring height and weight and blood oxygen levels.

Every time you come to hospital you will have your height and weight measured and often your blood oxygen levels will be recorded.

Chest x ray

The first usually take place around 6 months of age then routinely every year as part of an annual review. If your child becomes unwell he/she may require an extra chest x ray.

A Chest CT scan

This gives more information about the structure of the lungs than an x-ray.

Bronchoscopy

This investigation is to see deep into the airways of the lungs. A flexible tube connected to a camera is passed down into the large airways. The investigation is done with your child fully anaesthetised. On waking he/she will remember nothing. It is painless and is undertaken as a day patient

Bone scan (dexascan)

From the age of 10 years we arrange a painless scan of your bones to make sure they remain healthy. The scan is usually repeated every 2-3 years

Every year around your birthday we will take some of you blood to check your vitamin levels, measure your liver function and look for any signs of infection. If you are unwell we sometimes need to take some blood at other times as well.

Abdominal ultrasound

From the age of 10 and yearly afterwards we will undertake an abdominal ultra-sound to check your liver, kidneys and gall bladder to make sure that they are working well.

Glucose Tolerance Test

Some children develop CF related diabetes. This test is also checked yearly from your 10th birthday onwards to find out whether you could be becoming diabetic. It is important to screen for CF related diabetes as early treatment can protect against weight loss, deterioration in lung function and long-term complications

Investigation record

Date

Chest x ray

Blood tests

Blood Glucose test

Bronchoscopy

Dexa scan

Abdominal Ultrasound

Chest CT Scan

Insertion of Portacath Investigation record

Date

Chest x ray

Blood tests

Blood Glucose test

Bronchoscopy

Dexa scan

Abdominal Ultrasound

Chest CT Scan

Insertion of Portacath Section Five - Growth and Nutrition

In this section you will receive information about:

• Growth (growth chart enclosed) • Pancreatic enzymes if you need them • Advice on feeding • Cystic fibrosis related diabetes (CFRD) • Blood sugar monitoring Section Five

Height and Weight (back to back and enough boxes that would fill page)

Date Weight kg Height cm

Date Weight kg HeightHeight cm and Weight (back to back and enough boxes that would fill

Date page) Weight kg Height cm

Date Weight kg Height cm

Height and Weight (back to back and enough boxes that would fill page)

Date Weight kg Height cm

Date Weight kg HeightHeight cm and Weight (back to back and enough boxes that would fill

Date page) Weight kg Height cm

Date Weight kg Height cm

Food in Cystic Fibrosis

A high energy high protein diet is needed in cystic fibrosis. This is to : 1 Allow normal growth height and weight 2 Fight chest infections and improve lung function 3 Replace energy lost in the stools (if on enzymes ) 4 Building up a store if unwell There are no foods that need avoiding. Try to have foods from each of the food groups below: If extra energy is needed the following ideas can be used: 1 Spreads e.g.: butter polyunsaturated margarine 2 Oils e.g.: olive oil, rapeseed oil 3 Use Full fat foods 4 Snacks between meals Your dietician will help you plan your diet Ideas for family meals with extra energy Here are a few suggestions for family meals: Pasta for everybody Children who need extra calories – add 1 teaspoon of olive oil butter Bolognaise sauce Children who need extra energy, add a 1 teaspoon of oil, butter or margarine to mince and extra cheese! Salad - tomato cucumber lettuce add a teaspoon dressing mayonnaise Desserts Thick and creamy full fat yogurt

Don’t forget to explain why your child needs extra energy you can say ‘to keep healthy to grow strong and play’

How many calories does your child need? This will be individual to your child below is an idea of how many calories they may need per day: Age/yrs Energy /Calories kcal per Day Male Female 1-3 1230 -1450 1165 -1400 4-6 1715-2000 1545 -1850 7-10 1970-2350 1740- 2100 11-14 2200-2600 1845 - 2200 15-18 2755-3300 2110 -2500 19-50 2550-3000 1940 -2300 Table of Energy and Fat content of common foods Meat and alternative Kcal Average Fat/g 1 oz mince/1tbsp 70 5 3 chicken nuggets 150 5 2 fish fingers 100 5 1 thin sausage 60 5 1 boiled egg 75 5 5” pizza 200 5 4 oz/100g chicken breast 160 5 1 rasher bacon 120 10 1 fried egg 120 10 2oz beef burger 120 10 1 thick sausage 120 10 1 burger in bun 250 10 1 oz cheese 120 10 5 oz fried fish 250 15 1 sausage roll large 300 20 1 scotch egg 300 20 5 oz meat/cheese pie 400 25 1 large pizza 800 30 2 tbsp baked beans 50 0 Bread/Cereals Kcal Average Fat/g 2 Weetabix and 150ml milk 220 5 1 slice bread and butter 130 5 2 slices sandwich and filling 300-400 15-20 2 oatcakes and cheese 400 20

Potato/rice/pasta Kcal Average Fat/g 10 chips large/1 waffle 100 5 2 tbsp pasta/rice/2 potatoes 100 0 1 packet regular fries 250 10 Puddings 1 apple/pear 50 0 1 bowl jelly 80 0 1 bowl milk pudding 150 5 1 full fat yoghurt 150 5 1 small Fromage Frais 90 5 1 scoop ice cream 100 5 1 chocolate mousse 120 5 1 small cake 200 5 Snacks 1 rich tea biscuit 35 1 1 plain digestive biscuit 70 3 1 packet crisps 150 10 50g chocolate bar 250 10 30g/1oz peanuts 170 15 1 doughnut 250 10 Milky drinks, 200ml fortisip/ ensure plus /paediasure plus 300 10 1/2pt Milk full fat 200 10 1 scandishake/1/2pt milk 600 30 Takeaway meals Indian/Chinese meals 800 >30 Big Mac 500 20 Macdonalds milk shake 400 10 EXTRA Calories 1 tbsp/15ml Double cream 65 7 1 tsp/10g margarine/butter 70 8 1 tbsp/15ml oil 125 14 1 tsp/10g peanut butter/chocolate spread 70 8 1 tbsp Oil Dressing oil 75-100 7 1 tbsp cream cheese 70 5 1 tsp jam/honey /sugar 20 -

ENZYMES

Pancreatic enzymes are natural chemicals that help the body to break down and digest protein, fat, and carbohydrates in food. The enzymes are contained in the digestive juices produced by the pancreas.

However in most people with CF, the pancreas is prevented from functioning normally, which is known as pancreatic insufficiency or pancreatic failure. This is seen in 85–95% of those with Cystic Fibrosis.

This can cause malnutrition, which can lead to poor growth and physical weakness.

Most babies with Cystic Fibrosis will therefore need pancreatic enzyme supplements to replace those not produced by the pancreas. The most commonly-used pancreatic enzyme supplement for children in the UK is Creon.

The enzymes are administered in small capsules or granules called microspheres. They are usually very effective at digesting food. The dose of pancreatic enzyme supplements will be prescribed at the hospital clinic, and will vary from baby to baby. (Cystic Fibrosis Trust 2010)

How many enzymes your child has will depend on the following: • How much of your child’s natural enzyme reaches the small intestine • How much food or milk your child takes • What kind of foods especially how much fat and protein the food contains

Use below as a guide for malabsorption Home gastrointestinal symptom tracker Check the boxes that apply to your symptoms each day. See opposite side for symptom examples.

GI Symptom Tracker Day 1 Day 2 Day 3 Day 4 Day 5 Day 6 Day 7

Loose and/or large bowel movements

Foul smelling bowel movements

Constipation

Diarrhea

Mucus or oil in the bowel movements

Gas and/or cramping

Stomach pain

Bloating or distention

Difﬁculty gaining weight

Appetite: Less, More, Baseline

Other:

Total number of bowel movements/day

Comments: Adapted from the University of Cincinnati, Adult Cystic Fibrosis Center. Signs as to when the enzyme dose needs to be changed: • Enzymes may need to be adjusted when the meal or snack contains more fat than the usual amount. • Signs of malabsorption - (fatty stools / bloating /wind /tummy aches) when taking the usual amount of enzymes - this is when the pancreas has fewer natural enzymes to digest food. • Major changes in your child’s diet

TABLE FOR RECORDING NUMBER OF ENZYMES FOR MILK/MEALS AND SNACKS Number of Number of Number of Max enzymes Creon per Date enzymes enzymes enzymes with Snacks/ g Fat with Milk with Meals per day puddings

Comments Comments Cystic fibrosis-related diabetes (CFRD)

Cystic fibrosis-related diabetes (CFRD) is common in adults and adolescents with cystic fibrosis (CF). It occurs in approximately 20% of adolescents and 40 to 50% of adults with cystic fibrosis. It is less common in children.

What is diabetes? Diabetes is a condition where the amount of glucose (sugar) in the blood is too high. Normally a hormone called insulin controls the amount of glucose (sugar) in our blood. Insulin is needed to move glucose from the bloodstream into the cells. In diabetes the pancreas does not produce enough insulin and sometimes the insulin produced does not work properly called insulin resistance.

Why is cystic fibrosis associated with diabetes? In most individuals with cystic fibrosis the pancreas does not work properly. This can results in the pancreas not producing enough digestive enzymes, which break down foods such as carbohydrate, proteins and fats. The pancreas also is the organ where insulin is produced. The pancreas can reduce its production of insulin and this can result in CF-related diabetes. A fact sheet on CF related diabetes from the CF trust will be given to you at the time.

Section Six

Section Six - Respiratory

• This this section includes a record sheet for you to record your lung function. You will also receive information about: • Physiotherapy, (including techniques) devices, • Inhalers • Equipment including nebulisers

• Loaned equipment/service date record sheet. Some of your equipment will need to be serviced once a year. (date is on your nebuliser and it is possible to arrange for your nebuliser to be serviced while you are in clinic).

Equipment Loaned or Given

Date Device Instructions Service Date

Comments Comments Comments

Section Seven Section Seven - General Information

General Information

• Holidays This section will provide a check list that you should think of taking with you. Useful numbers for holiday insurance • Housing • Benefits • Prescription charges • Welfare grants • Other supporting agencies • Schools/university

Holidays

• We would recommend that you take your personnel record with you as it contains important information about the care and management about your cystic fibrosis. • The hospital will provide you with a letter stating that you are fit to travel and also providing a list of medications you are required to take with you and any other information which is applicable to you e.g. if you have a port a cath insitu/if you are a diabetic. • Discuss with the CF team what medications would be advisable to take with you. • Carry any medications with your hand luggage. • Remember to drink plenty of fluids • Maintain good salt levels (Salt and water advice leaflet enclosed) • Ensure you have good sun protection in terms of clothing. Hats and most important high factor sun cream. • Remember to take out travel insurance. • Dream holidays – contact your CF team who can refer your family for a Dream holiday (UK only). • List of known insurance companies (If you have experience and good deals for travel insurance let your CF team know and we can recommend to others). Abletotravel 01892 839501 Barclays 0845 600 8090 Norwich Union Direct travel insurance 0800 559 3201 Columbus Insurance 0845 3308578 Freedom Insurance Cambridge 0870 7743760 Worldwide Insurance, com 0870 1128100 Liverpool Victoria 08000223919 Ace Travel 08000282396 Flexicover 08000939495 Direct Travel Insurance services 08456052500

Housing

If you have concerns about your home e.g. too small, damp etc., we would advise you to contact your welfare officer Jo Frain on 01782 657105 who will advise and support you through the process and involve members of the CF team as needed. Cystic Fibrosis Trust leaflet available (ask a member of the team to provide you with one) Benefits The benefit system is quite complex if you require help with completing these forms or advice on what your child is entitled to contact Jo Frain on 01782 675105 Welfare grants These grants are available through the Cystic Fibrosis trust contact a member of the Cystic Fibrosis team to see if you would be eligible and guidance will be given to complete this application. Education When you are planning to start your child in nursery, primary school and senior school members of the CF team will arrange a visit with yourselves and the staff who are going to be responsible for your child. This will give them an insight to Cystic Fibrosis and a care plan provided. When you are looking at Further education as in 6th form college or University again members of the CF team are there to support you including the welfare officer who can help with grant applications, equipment etc.

Comments Comments Section Eight - Glossary

• Glossary This section will provide you with explanations of words/tests.

If you have any questions or tests you would like information then make a note and talk to a member of the team. Section Eight

Glossary

BM Measures the sugar level in the blood Bronchus (Bronchi) The larger airway(s) in the lung. DNA The abbreviation for deoxyribonucleic acid, the principal molecule carrying genetic information in all living organisms. Enzymes Chemical which in CF digestive enzymes which digest food so it can be absorbed and used by the body. FEV1 The maximal amount of air you forcefully breathe out in 1 second. FVC The amount of air which can be forcibly breathe out, from the lungs after Taking the deepest breath possible Genetic Testing The method of detecting certain genes which in CF explains if a person is a carrier for CF or actually has the disease. Haemophilus Influenzae Bacteria which are a common cause of respiratory infection in Cystic Fibrosis. Haemoptysis Coughing up blood. Intravenous Sometimes antibiotics or other medicines are given into a vein rather than by mouth. To make it easier, a small plastic cannula (tube) can be left in the vein so that the medicine can be put through it rather than needing an injection each time. There is a cream which numbs the skin before the cannula is inserted.

Meconium Ileus An obstruction of the small intestine at Birth which occasionally occurs in CF

Nebuliser A small machine which converts liquid medication into a fine mist which can be breathed directly into the lungs.

Pancreas An organ which lies below the stomach andmakes digestive juices or enzymes and insulin.

Physiotherapy Part of the treatment for Cystic Fibrosis

Polyps A small growth of mucous membrane that can grow on the lining of the nose.

Pseudomonas aeruginosa A bacterial infection which affects the lungs.

Sats Recording the oxygen level

Sinusitis Inflammation of the membrane lining the sinuses (the air-filled cavities in the bones surrounding the nose).

Sputum Material produced by the cells lining the respiratory tract.

Staphylococcus aureus A bacterial infection that can affect the lungs..

Sweat Test A test used to diagnose Cystic Fibrosis alongside testing for CF genes.