Cystic Fibrosis Annual Review

Transcription

1 James Paget University Hospitals NHS Foundation Trust Cystic Fibrosis Annual Review Your Guide

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3 This guide has been put together to help you to understand why your child undergoes an annual review, who your child will see and explains some of the tests and investigations that are carried out. Annual Review The annual review for children with Cystic Fibrosis is carried out each year at the James Paget University Hospital (JPUH). The Cystic Fibrosis (CF) team will see your child in the paediatric outpatients clinic (antenatal clinic signpost). The CF team consists of your Consultant Paediatrician, Physiotherapist, Dietician and Children s Community Nurse. It provides the medical team and yourselves with a detailed assessment of your child s progress. It takes an overall view of your child s well-being and development, looks at the effectiveness of their treatment over the last year and to plan for the next year. Notes to ask CF Team: 3

4 If you have any questions about the review, or the date of the appointment then please contact: Cystic Fibrosis Team/Contact No Children s Community Nurse/CF Nurse Rhona Webster Mob Children s Outpatients JPUH Paediatric Consultant Secretary (Dr Jayalal) Susan Morgan Paediatric Physiotherapist Suzie Webster Paediatric Dietician Patrick Friel Ward 10 (The Children s and Young Person s Unit) When you attend your CF annual review appointment this will be longer than your regular CF clinic as you will be reviewed by all the members of the team. Results of tests and x-rays can be discussed and future treatment plans agreed upon. It is also an ideal opportunity for you to ask questions of the CF Team, discuss new therapies and any concerns you may have. The annual review aims to be a two way process, so that it is as informative for you as it is for the CF team. 4

5 Tests and investigations When your child has an annual review there are various tests and investigations that are carried out. These help the CF team assess how well your child is doing and if any changes need to be made in their treatment. Height and weight This looks at the rate of growth and will be plotted on a growth chart. It shows the overall rate at which your child is growing. The dietician will review your child s growth and will sometimes use other measurements such as mid upper arm circumference and blood results to assess your child s general nutritional state. The dietician will also discuss your child s food intake and you will be asked to complete a food diary before coming to clinic. This information will help the dietician to assess whether your child is eating well and if they need to take more enzymes with their meals and snacks and check that they are taking the correct amount of vitamins. The dietician will be happy to discuss any concerns that you may have concerning your child s diet or feeding. Blood tests will be requested four weeks before your annual review appointment and a food diary/explanation will be sent to you from the dietician. Lung function This test is performed on children over 5 years by the physiotherapist or children s community nurse. Your child will be asked to take a deep breath in before breathing out into a special machine. This machine measures the amount of air your child can breathe out. They will be asked to do this three times to measure their best effort. The physiotherapist will then discuss physiotherapy techniques with you and your child and any problems you may be experiencing. Throat swab/sputum Children with cystic fibrosis have thicker secretions and are more vulnerable to bacterial/fungal infections. A throat swab or collection of sputum will be done in clinic to look for bacteria. This will help in choosing appropriate antibiotics. 5

6 Oxygen saturations This machine monitors the level of oxygen in your child s body. Normal levels are between % Nebulisers/infusion pumps If your child uses a nebuliser (Pariboy or e-flow) or you have/have had IV (intravenous) therapy at home using an infusion pump (Grasby etc) then the community nurse will discuss these with you to see if anything requires servicing or repair by the hospital. The community nurse will also discuss with you any issues with home IV therapy if you have received it over the previous year. Chest x-ray Your child will have a picture called an x-ray taken of their chest. This is usually done before your child s annual review appointment. This looks at the shape and size of your child s lungs. The picture of the lungs are then divided into five zones. These are right lung upper, middle and lower lobes; left lung upper and lower lobes. Abdominal ultrasound An ultrasound of your child s abdomen may be performed if there is a concern with some of your child s blood results, in particular liver function. This is a painless procedure using high frequency sound waves to create a picture of your child s liver and surrounding organs. It will show the size and shape of your child s liver and how well the blood flows into and out of their liver. This will be discussed with you by your consultant. 6

7 Blood tests Cystic Fibrosis affects many parts of the body. Your child will have blood taken from a vein in their arm or in a baby, their foot as sometimes the veins are very small. Special cream is used first to numb the area where the blood test will be done. Bloods are taken to check your child s liver function, kidney function, full blood count, clotting time, glucose and vitamin levels. Other tests may be necessary and discussed with you at the clinic appointment. These tests help the team determine your child s overall general health. You will be asked to take your child for their blood tests four weeks before the annual review appointment. Please contact the ward to arrange your child s blood to be done (ask the ward clerks to arrange). Phlebotomy clinics for children are on: Monday - under 2 years pm onwards at half hourly slots (6 slots) Wednesday - over 2 years - morning 9am, afternoon 12 noon If your child has a portacath in situ, then the community nurse can arrange to take the blood tests at home or on ward 10 six weeks before the annual review appointment. Please contact Ward 10 to arrange for your child's blood tests to be done. Please use this space to write down a few questions or topics you would like to discuss with the CF team: 7

8 Terminology ACBT active cycle of breathing technique a physiotherapy therapy technique Abdomen part of the body which contains the stomach, liver, spleen, kidneys and pancreas. ABPA (allergic bronchopulmonary aspergillus) allergic reaction to aspergillus fungus which causes asthma type symptoms. Treated with steroids and antifungal antibiotics Aspergillus Pptns a blood test looking for the aspergillus fungus AST an enzyme found in the red blood cells, liver and heart Asymptomatic without any symptoms Bronchiectasis abnormal stretching and enlarging of the airways caused by the build up of mucus Centile measurement of weight and height recorded on a chart divided into one hundred parts Chronic colonisation long term infections Creps noises heard in the chest Delta F508 one of the CF genes Diabetes Mellitus sugar diabetes Diffusely enlarged bigger all over Endoscopy telescope to look inside the body Exacerbation an increase in the severity of disease of or its symptoms Faecal fat estimation fat contents of stools Ferritin the form in which iron is stored in the body FET forced expiratory technique used in physiotherapy FEV1 lung function test showing how much air you can force out in one second FVC lung function test showing how much air you can force out Glucosuria sugar in urine H2 antagonists drugs to reduce stomach acidity 8

9 Haematemesis vomiting blood Hepatosplenomegaly enlarged liver and spleen Heterozygote two different CF gene types Homozygote pair of CF genes which are the same type IgE a blood test that looks for an allergic response Mané latin word for in the morning Nocte latin word for at night Nasal polyps part of the lining of the nose becomes swollen which can block the nose making breathing difficult and impair the sense of smell Peri bronchial thickening airways become thicker/swollen Phenotype outward physical parts of cells, structures, tissues, organs etc Portal hypertension increased pressure in the veins of the liver caused by obstruction in the liver Prophylaxis preventative Pseudomonas aeruginosa bacterial infection often found in people with CF R533X one of the CF genes RASTS a blood test to check for allergies Spirometry lung function testing Splenomegaly enlarged spleen Staphylococcus aureus bacterial infection often found in people with CF Ursodeoxycholic acid (URSO) medication for liver disease Wheezing a sound that is most pronounced on the exhalation of air Yeasts single cell fungal infection common in people with CF Bile a yellow green fluid made in the liver and stored in the gall bladder which breaks down fat Clotting time length of time it takes for blood to clot 9

10 Constipation delay or difficulty passing a hard very firm bowel motion/poo Coryzal symptoms viral illness usually a cold or upper respiratory infection Vitamin A Fat soluble vitamin important for growth and vision in dim light. Found in milk, eggs, liver and some vegetables, cabbage and carrots Vitamin D Fat soluble vitamin that helps with the absorption of calcium. Found in dairy products, milk, cheese, yoghurt. Vitamin E Fat soluble vitamin that is important for normal cell development. Found in vegetable oils, eggs, butter and wholemeal cereals. Vitamin K Fat soluble vitamin important for normal clotting of the blood. Found in green leaf vegetables and meat. Creatinine A substance in blood and urine. Haemoptysis Coughing up blood from the lungs, usually streaks, caused by possible infection or trauma from coughing hard Immunoglobulins A group of proteins that act as antibodies found in blood and body fluids Liver enzymes A substance produced by the liver and released into the blood stream. Pancreatic enzymes creon/pancrease A special protein that helps break down starches, proteins and fats found in food and milk PEP (positive expiratory pressure) mask and flutter valve Hand held devices that older children can use as an alternative method of physiotherapy Pneumonia Infection of the lungs. Signs include; fever, tiredness, cough and chest pain Upper respiratory tract infection A viral infection of the nose, throat and upper airways 10

11 Blood Test Range Levels Full blood count Range level HB WBC Lymphocytes Neutrophils Platelets HbA1c 4-7mmols Electrolytes kidney function Range level Sodium Potassium Urea Creatinine Calcium Phosphate Liver function Range level Bilirubin 0-20 Albumin Alk Phosphate ALT 0-31 Clotting Range level INR APTT ratio Magnesium Vitamin Levels A D E K Range level umol/l (1-6 years) umol/l (7-12 years) umol/l (13-18 years) > 30ug/l (ideal summer) > 25ug/l (ideal winter) 5-19 umol/l (< 1 year) 7-12 umol/l (1-6 years) umol/l (7-12 years) umol/l (13-18 years) Vitamin K ug/l

12 please let us know. The hospital is able to arrange for an interpreter to assist you in communicating effectively with staff during your stay through INTRAN. If you need an interpreter or a person to sign, If you require a large print version of this booklet, please contact PALS on Photos from NHS Photo Library Author: Rhona Webster Children's Community Nurse (CF Nurse) December 2007 Revised January 2010, July 2011 James Paget University Hospitals NHS Foundation Trust Review Date: July 2014 CH 33 version 3