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CF
Former Member
Posts: 1,876,323 The Mix Honorary Guru
this isn't a problem that i've got, it's just some questions i need to ask about it. first of all, does anybody here have cystic fibrosis. i would prefer answers from people who have it so that i know that i'm getting the right info. i'm doing a presentation at college on monday and need to find out what type of treatment is available for CF sufferers and what type of support you and your family get and who you get the support from.
i'm grateful for any information any body can give me.
i've tried searching the net but can't find exactly what i need.
oh yes and one last question, how did you cope with it when you were younger.
thanks!
i'm grateful for any information any body can give me.
i've tried searching the net but can't find exactly what i need.
oh yes and one last question, how did you cope with it when you were younger.
thanks!
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i don't want you to actually answer the question for me, i just want some basic info that i can do more research on
Genetic background:
Cystic fibrosis is the most common autosomal recessive disorder in Europe. Incidence is ~1 in 2000 live births, and 1 in 22 of us are carriers of the defective gene. It is lethal, but life expectancy has increased markedly in recent years to approx 40 years now, although CF affects sufferers to different degrees.
Clinical features:
Affected organs and organ systems include the exocrine (as opposed to the endocrine) pancreas, GI tract, bile ducts, airways (trachea, bronchi etc), sweat glands, and gonadal function. Some sufferers will have particular problems with their lungs, other may be more affected by gut function.
What's wrong?:
The genetic mutation affects the CFTR gene which disrupts the carrier protein responsible for chloride ion transfer across cell membranes. This disruption causes excess sodium chloride release in sweat --> salty skin, and this is diagnostic.
Internally, the chloride channel dysfunction causes the build up of viscous mucus in the airways and GI tract. The build up of the mucus in the airways in particular leads to infection since the airways are not cleared effectively. Chronic respiratory infection leads to degradation of lung tissue and ulimately leads to decreased lung function. Continual use of antibiotics leads to increased likelihood of an antiobiotic-resistant infection. CF patients are a source of such bacteria, and are often barrier-nursed in hospital (pschycological effects of segregation from 'normal' patients etc etc).
Obstruction of the bile ducts within the liver also disrupt digestion and liver function. Likewise, the thick mucus blocks the pancreatic duct and prevents the effective release of digestive enzymes into the GIT.
What does this mean?:
As I've said, repeated infections in the lung lead to irriversible damage, and lung function deteriorates slowly over many years.
Blockages in the pancreatic duct affect absorption of fats and therefore fat-soluble vitamins. Vitamin A, D and K deficiency diseases are most common.
Blockage of the vas deferens in male sufferers often leads to infertility. Fertility in females is also reduced due to build up of a thick mucus within the vagina, which restricts entry of sperm. However, some CF sufferers are fertile.
Treatment:
CF sufferers are given pancreatic extract to replace enzymes normally released by the pancreas (lipase, amylase & protease). They are also given large doses of vitamins A, D and K.
The recommended diet is high in calories, fats and proteins, with vitamin supplementation and liberal use of salt. The aim is to keep body mass index within the normal ranges (20-25).
Respiratory infections are treated with antibiotics. Sufferers, and their parents, are taught physiotherapy exercises which are usually done twice daily and last approx 30 mins. The aim is to aid drainage of the bronchial secretions by:
postural drainage
percussion and vibration
forced expiration ("huffing")
controlled breathing
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
I'm not sure what an "early years practitioner" is. Perhaps that is the new PC name for a paediatrician. Most likely the GP will be the first to pick the disease up following continual episodes of chest infection and/or diarrhoea.
I suppose the main role of the "early years practitioner" is education. They must teach the parent(s) how to perform the physio and how to administer the dietary supplements. The patient may occasionally attend O/P physio appts but only to be taught new exercises or if their condition deteriorates suddenly. Hospital admissions can be regular for some sufferers, but are rarely frequent.
Life expectancy ~40 years.
Sorry it's so long - I hope some of it is useful... <IMG SRC="smile.gif" border="0" ALT="icon">
[Edited to add sources and useful links]
Sources:
Concise Oxford Textbook of Medicine Ledingham, J (ed) Warrell, D (ed)
ABC of Clinical Genetics Kingston, H
Principles of Anatomy and Physiology Tortora, G Grabowski, S
Links:
Cystic Fibrosis Trust homepage
CF & You (CF Trust)
CF: the facts (CF Trust)
Antibiotic treatment (CF Trust)
[ 14-05-2002: Message edited by: Kentish ]
Nothing to do with this thread of course <IMG SRC="wink.gif" border="0" ALT="icon"> <IMG SRC="rolleyes.gif" border="0" ALT="icon">
There's no maximum, but they CF puts extra strain on the body so they do die relatively young. It all depends on how severely affected they are.
I'm glad at least someone read my post <IMG SRC="smile.gif" border="0" ALT="icon">