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You are the FY1 on a paediatrics ward. Lou Pus is a 10 year old boy who is attending for clinic as he has a long term medical condition. He attends clinic once every 6 months and was hospitalised last year with a respiratory infection. He has come to clinic with his mother to discuss the impact the long term condition is having on his life. Lou’s medications include:

Creon Omeprazole Saline (nebulised) Azithromycin

Please take a history from Lou and perform the appropriate examination. Ensure that you assess his concerns and investigate the impact this condition is having on his life.

You are Lou Pus, a 10 year old boy who enjoys superhero movies, football and cycling with your dad. You also have been to hospital a lot in your life, with what your mum calls a ‘bad chest’. When you were very young, you were told that you have something called ‘Cystic Fibrosis’, which means that you produce a lot more thick mucus, and makes it hard to breath sometimes. This also means that you get lots of infections and have to go to hospital quite frequently. This has meant that you’ve missed a lot of school, which you think is a real pain, since you quite like school.

HPC: You don’t remember a time when you didn’t have CF. Your mum told you that when you were a baby, you didn’t grow quite as well as your older brother, Jake, did. They said that you weren’t keen to eat as much, and didn’t put on weight as you were expected. You were rushed into hospital when you were a few weeks old, with a chest infection. Your mum has told you that you needed to get all kinds of special medicine, but you came through it ok.

As a 10 year old, the CF does still affect you. You have to take a lot of medicines, and you see a special nurse, Barbra, who comes to your home once or twice a week to help you with your breathing. Last year, you were brought into hospital again, for another chest infection. This one was really bad, however, and it didn’t go away very fast. You had a high temperature, a really bad cough, and just felt really yucky at the time. You couldn’t even get out of bed! You remember that your mum was really worried at the time: she always worries, but that time she was extra stressed!

In your day to day life, your body is affected by this condition. You find it difficult to go to the toilet and are constipated often. When you do go, you produce really smelly poo. It’s quite embarrassing! Aside from the infections, you are always coughing up lots of snot, and it sometimes has blood in it. You have a cough on most days, which isn’t usually painful.

ICE: You try your best to have a normal life, and are quite happy despite all the visits to hospital. You worry that it will affect your schoolwork, however, as you had to take a lot of time out of school last year, which meant that you really fell behind. You really like science and missed out on the school science day last year, which really annoyed you! You like to play football with your school team, but when your chest is bad you can’t keep up with the other boys. This is starting to become more and more of a problem. When your classmates are ill, your mum tells you to stay away from them, as you could get sick too. You want to be a doctor one day, so this worries you sometimes: you want to help sick people!

What stresses you out most is how your mum acts sometimes. She is really protective, and you understand that, but it gets on your nerves sometimes! You want to be able to go out and cycle with your friends all the time, but she wont let you go when it’s raining outside. The problem is, it’s always raining!

PMH: You’ve been told you might get diabetes ,like your dad, one day, but you don’t have any other problems, other than the CF.

SH: You live in a house with your mum, dad, brother Jake and your turtle, Fenton. Your mum won’t let you get a dog as she’s worried you might be allergic!

FH: Your dad has diabetes. Your grandmother (mum’s mum) had CF as well, which is what you think has scared your mum so much.

The student should demonstrate a clear history of the impact cystic fibrosis is having on Lou’s life. In terms of examination, a respiratory exam would be appropriate, however an abdominal exam may also be acceptable. The student’s differential diagnosis should sound something like:

My differential diagnosis would include a multisystem genetic cause, more specifically Cystic fibrosis. This is particularly demonstrated by the patient’s failure to thrive as a newborn, recurrent respiratory infections and steatorrhoea. Example for discussion may include:

  • How would you investigate this patient, when they first presented as a newborn?
  • How would you manage this patient with an acute infection?
  • What is Cystic Fibrosis?
  • What are the systemic complications of cystic fibrosis, and when would a patient develop them?
  • What health professionals could be involved in Lou’s care?
  • What prognosis is associated with CF?