Technical "Jargon" of Cystic Fibrosis

Term/Condition

Description

1. ABPA (Allergic Bronchopulmonary Aspergillosis)	An allergic reaction to spores in the atmosphere. Causes inflammation and wheezing. Occurs in PWCF and people with Asthma. Usually treated with steroids. More info here!
2. Arthropathy	Occurs in up to 10% of PWCF. Symptoms include joint pain, episodic arthritis, rash and swelling. Episodes tend to settle after 3-4 days and respond well to NSAIDs.
3. Burkholderia cepacia (B. cepacia)	Identified in the 1980’s B. cepacia is one of the 'bugs' PWCF worry about the most. In fact the majority of CF patients who have B. cepacia in their lungs will not suddenly deteriorate clinically. Unfortunately, 20% of CF patients will experience rapid decline. Due to this and its tendency to be multi-resistant, people with B. cepacia are usually isolated from other PWCF.
4. CFRD (Cystic Fibrosis Related Diabetes)	Similar to Type 1 Diabetes, CFRD is usually treated with insulin injections. However, CFRD requires a unique approach in regards to management. Around 15% of PWCF develop CFRD in their lifetime. Average age of onset is 18-21 years. Unique approach is the fact that sugar is not cut out of the diet, due to the need for a high calorie intake for someone with Cystic Fibrosis. Click here for more info!
5. Distal Ileal Obstruction Syndrome (DIOS)	See 'Meconium Ileus Equivalent'.
6.FEV1	This stands for Forced Expiratory Volume; this measures the amount air you blow out in the first second of your lung function test (hence the “1” part of the name). This is the reading that CF clinics usually concentrate on.
7. FVC (Forced vital capacity)	This measures the total amount of air you blow out (forcefully) in your lung function test. The percentages are worked out using your height, age, sex and race to calculate what would be “normal” for you. A “normal” FVC is generally considered to be anything from about 80% - 120%. A drop of around 20% is often an indicator for IV treatment, although other factors such as how you are feeling, and sputum and blood results are taken into consideration.
8. Liver Cirrhosis	Many types of chronic injury to the liver can result in scar tissue. This scarring distorts the normal structure and regrowth of liver cells. The flow of blood through the liver from the intestine is blocked and the work done by the liver, such as processing drugs or producing proteins, is hindered. Click here for more info!
9. Meconium Ileus	An intestinal obstruction in newborn babies, generally requiring surgery. Key indicator of CF, therefore a sweat test usually follows. It is seen in about 10% of PWCF. Find out more here!
10. Meconium Ileus Equivalent (a.k.a. DIOS)	Complete or partial bowel obstruction and a common problem for many PWCF. Symptoms are pain and distension of the stomach. Usual treatment is Gastrographin. Info available here!
11.Methicillin-Resistant Staphylococcus Aureus	A version of the bug, Staphylococcus Aureus, but one which has become resistant to various antibiotics. It is, however, treatable with certain antibiotics. More info can be found here!
12. Nasal Polyps	Occur in around 10% of CF children and up to 40% of CF adults. Usually asymptomatic, but if troublesome, a nasal spray usually helps. Removal via surgery is also an option. Find out more here!
13. NSAIDs (Non-Steroidal Anti-inflammatory Drugs)	Self-explanatory, e.g. Ibuprofen Find out more here!
14. Osteopenia	The presence of less than the normal amount of bone. Osteopenia, if not treated, may result in osteoporosis (see below). Info can be found at here!
15. Osteoporosis	Mal-absorption causing low vitamin D levels and inactivity are both contributing factors to the common occurence of Osteoporosis in PWCF. (Around 10-20% of adults) Causes weakened bones, which can result in multiple breakages. Treatments include calcium supplements, attention to diet, and exercise. National Osteoporosis Foundation site can be found here!
16. Passport	Similar to a portacath.
17. Portacath	An implantable venous access device. Requires surgical implantation so is usually only offered if venous access becomes a problem, or IVs are required very often. Used for the administration of IVs and blood can also be taken from it. It is inserted into a main vein, and the port sits just under the skin. Can be accessed whenever needed, using a special port needle.
18. Pneumothorax	Where air leaks out of the lung into the chest wall cavity, causing the lung to collapse. A small collapse can be treated conservatively (wait for the air to be absorbed and the lung to re-inflate). However, larger ones may require needle aspiration or chest drain insertion. Find out more here, but scroll down to view!
19. Pseudomonas Aeruginosa	One of the most common bugs in PWCF. Once caught, it is very hard to permanently eradicate but can be suppressed with treatments such as nebulised Colistin. Exacerbations are treated with oral or IV antibiotics. Detailed info can be found here!
20. PWCF (People/Person With Cystic Fibrosis)	Pretty self-explanatory. Common acronym in the Cystic Fibrosis community used to refer to someone or a group of people who have CF.
21. Sinusitis	Extremely common in PWCF, often mistaken for "a cold". One study revealed 92% of participants had sinusitis (most were symptomatic). It can clear up on its own, or antibiotics can be used. More info available here!
22. Staphylococcus Aureus	Another very common bug in PWCF. Causes problems for some, but not for others. One common treatment is flucloxacillin.. Exacerbations are treated with oral or IV antibiotics. Just one of the many sources for info can be found here!
23. Steroids	Used to reduce inflammation in the lungs. May be in the form of an inhaler, (e.g. Seratide) or in tablet form (e.g. Prednisolone). They can lower the immune system, so patients should be aware of staying away from people with contagious illnesses like Chicken Pox.
24. Tx	A common abbreviation for transplantation used by many pwcf.

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