Archived questions

Here are a selection of some past questions for Ask the Expert - you can browse through the page or go straight to the questions that interest you by clicking on this list.

Feeding a fussy child
Embryo screening
Enzymes with curry and beer
Steroids and behaviour
Risk of infection from river water
Risk of infection from pools and saunas
Risk of infection from horses and animals
Air Quality and location
Growth hormones
Salt tablets
Creon guide
Passive smoking
Risk from pets
Chinese herbs
Pseudomonas in soil
Garden centres
Untreated swimming pools
Risk of infection from sand
Risk from fish tanks
Garlic supplements
Tipping during physiotherapy
Storing Colomycin
Nebuliser servicing
Vitamin K
Scuba diving
Enzyme time of effect
Vitamin E
Arthritis
Liver disease
Curcumin
Carrier Testing
Liver scarring
Creon in stool
Shingles
E.Coli
Gene therapy trials
Treatment of lung lobe collapse
Open fires and dust
Stress fracture of the ribs
Applying for housing
Ibuprofen
CF-related arthropathy
Aspergillus
Condensation
Pseudomonas
CF research in the news
Teeth discolouration
DLA
Safety of air conditioning
Borderline sweat test
Newborn Screening
Bloated tummies
Fluid in hot weather
Carrier symptoms
Stem cell banking
Acapella
Port-a-cath and contact sports
Alternative Therapies
Menstruation
Malabsorption in toddlers

Question: Feeding a fussy child
My son, who has Cystic Fibrosis, is a very active five-year old constantly burning up energy, but his appetite is not at all good. I try to give him all the right foods making them as appetising as possible, but quite often he takes a few bites and eats no more unless I offer him his dessert early. He just gets more and more fussy about what he eats and is quickly losing interest in food generally. Do you have any ideas on how I can encourage my child not to be so faddy?

Answer
In caring for a child with Cystic Fibrosis, a great deal of emphasis is placed on the importance of diet and growth. This can result in understandable parental concern regarding an adequate dietary intake, and children with CF becoming 'at risk' of developing behavioural problems during mealtimes. Unfortunately, food is often used to manipulate parents! Indeed, such behaviour has been found in over 70% of children with Cystic Fibrosis.

Children often learn that refusing to eat gets them lots of attention. To the child, even if you get angry or give them their dessert early, if food is refused, it is seen as giving attention and therefore the bad or undesirable behaviour carries on. Behaviour that is rewarded with attention will continue, while any behaviour that is not rewarded (ignored) will disappear.

Below are a few tips to help minimise the problem:

• Try to have family meals, so your child sits with other children/adults. This will encourage them to learn correct feeding behaviour.

• Avoid other distractions eg having the television on at mealtimes.

• If your child is slow at eating, gentle encouragement will help.

• Make food as attractive as possible.

• Never lose your temper when food is refused. In the child's eyes, any reaction is attention. This will encourage the bad behaviour to continue.

• Encourage good or positive behaviour and give lots of praise for it.

• Limit meal times to 30 minutes. Research has shown that allowing mealtimes to drag on rarely results in any more food being eaten. After 30 minutes remove the food without comment, and wait for the next meal/snack time.

• Never produce a second meal if the first is refused or give dessert if the main course is refused.

• There must be a consistent approach from all carers involved with feeding.

• Parents who are worried about their child's feeding should ask for further advice from the dietitian and/or psychologist in their clinic.

We would also recommend reading the new Eating well with Cystic Fibrosis - A guide for children and parents available in the publications library. This leaflet gives further advice on managing difficult feeding behaviour and nutritious meal and snack ideas to help achieve a good balanced diet.

Question: Embryo screening
We have a lovely three-year old daughter who has Cystic Fibrosis. We would dearly love to have another baby, but are concerned about having another child with Cystic Fibrosis. We know there are tests available in the early months of pregnancy to determine whether or not the baby has CF but we are not happy about managing the decisions of termination or not. Is there any other method of ensuring another child would not have Cystic Fibrosis?

Answer
The Cystic Fibrosis Trust has published a booklet on Genetics, which covers all the issues surrounding carrier testing, tests during pregnancy and for newborn babies. It is available in our publications library or you can order a copy by calling the CF Trust.

The booklet also has the following advice on Pre-implantation Genetic Diagnosis.

The procedure involves in vitro fertilisation (IVF) similar to the technique used for infertile couples. Embryos are created in a test tube from the mothers eggs and the fathers sperm. The embryos are then tested for CF and only embryos free of CF are implanted in the mother's womb.

This is not an easy option, nor is it freely available. It can be time-consuming and disruptive to the family routine as it usually requires travel to one of the few Centres where it is available. Centres offering pre-implantation genetic diagnosis are listed on page 13 of the Genetics booklet.

The mother has to take fertility hormones to ripen a number of her eggs and these have to be collected through a fine tube passed into her lower abdomen. Generally two healthy embryos are put into the mothers womb. The chance of a successful pregnancy is 25%, and there is a higher risk of having twins.

There are only a few centres in the UK where pre-implantation genetic diagnosis is offered. It is also costly, at around £3,000 for each attempt and not all health authorities are prepared to pay for the treatment.

Question: Enzymes with curry and beer
I am a student and often go out for a few lagers on a Saturday night. Sometimes we go for a curry, but on other occasions, we just stagger home. I'm never sure whether to take my enzymes or not. Could you advise, please?

Answer
I remember those days well! There are really two things to think about. Firstly, whether you need to take enzymes with the lager (or alcohol in general) and secondly whether you need enzymes with the curry.

The second question is the easier one to answer. You definitely need to take enzymes with the curry. Also many people forget that curry is often prepared with ghee which is clarified butter, therefore curry tends to be much higher in fat than most people expect. This means you will need more enzymes with a curry than you might expect. It is also important to take into consideration any accompaniments you may have such as popadums, which are also high in fat.

Most people would agree that you do not need enzymes with alcohol as symptoms of malabsorption are most closely linked to fat malabsorption, however a pint of lager contains between 165 to over 300 KCalories dependant on the strength of the lager. If you are drinking a lot of lager then you may have symptoms of loose stools in the morning. Whether these symptoms are related to the effect of alcohol on the digestive system or to symptoms of malabsorption is unclear. If you have loose stools following a "night out" you may benefit from a small dose of enzymes with your alcohol next time you go out. However you should discuss this with your own dietitian and if the enzymes do not resolve your symptoms you should discontinue them.

Question: Steroids and behaviour
My nine year old son who has CF was recently prescribed steroids. For the first few days, there was no problem. But then I noticed his behaviour began to change. He began to misbehave more than is normal for a boy of his age. I had calls from his school, and subsequently he was excluded from lessons on a regular basis and on behavioural report every day. There was no improvement in his behaviour over a period of six weeks.

I advised the school that he was on steroids and that these could induce mood swings, however they were not convinced. The hospital was kind enough to supply a letter of explanation, unfortunately however, by this time the school was extremely unhappy with the situation and advised me that my son would be suspended if his behaviour did not improve.

By this time, I was at the end of my tether and decided to take my son off steroids. A week passed with a slight improvement in behaviour, and by the Thursday of the third week off steroids there had been no bad reports from school for eight days. He was even awarded 'Star of the Week' for much improved performance.

Could this be a coincidence? I realise, of course, that as parents we cannot simply decide to stop and start medication without discussing it with our doctors. Maybe if the problems were highlighted more schools would be better equipped to deal with the situation. My reason for writing this letter is to tell parents experiencing this problem that they are not alone.

Answer
This is a very useful letter. Some children when started on steroids do experience a marked deterioration in behaviour and you should discuss this with your paediatrician or doctor. It is very important that treatment is not stopped without consulting your paediatrician or doctor.

Question: Risk of infection from river water
I have a six year old son who has CF. He is very active and enjoys all types of sport. He does have a port-a-cath and is receiving regular (every 16 weeks) IV antibiotics. Around four months ago he contracted Pseudomonas aeruginosa which has cleared following IV treatment. He has been invited to go on a fishing trip this weekend and I am a bit concerned about him contracting either Pseudomonas or Burkholderia cepacia in this natural environment. What are your thoughts on this matter?

Answer
There is no reason why your son should not go on the proposed fishing trip. The chances of him acquiring either Pseudomonas or B cepacia from the environment are very low indeed. These organisms are not around as spores but usually found in moist places and acquired by direct contact. The spores, which are acquired at times are those of Aspergillus.

The risks of such a trip are very low and outweighed by the enjoyment, so I suggest that your son goes on the trip. In the past, most people who contracted B.cepacia at camps and holidays acquired them from other people with CF who were infected with the organisms.

Question: Risk of infection from pools and saunas
Is it safe for my son, who has CF, to use health club facilities such as a swimming pool, spa, sauna and steam room? I am sure I am correct that it is not wise for him to use the spa pool but I am unsure whether the steam room or sauna is firstly safe for him to use or whether it would be beneficial.

Answer
As you are probably aware, provided the swimming pool is serviced regularly there is no problem for your son to swim. However the Jacuzzi/spa pool is a definite risk for contracting Pseudomonas and should be avoided.

There is no definition information on steam rooms but an experienced CF microbiologist, with whom we discussed this issue, feels this could be a risk due to the fact that steam rooms are damp and Pseudomonas can withstand quite high temperatures - so better avoided.

Saunas on the other hand are more dry heat and there should be no risk of infection.

To summarize, swimming pools and saunas are okay, steam rooms and Jacuzzis are not.

Question: Horse Riding
The Cystic Fibrosis Trust has received several queries from parents about the safety of horse-riding as an activity for their child with Cystic Fibrosis.

Horse-riding itself does not pose any risks as far as contracting infection goes. However it is imperative that a person with CF does not muck out horses' stables. The Cystic Fibrosis Trust's Consensus Document Antibiotic Treatment for Cystic Fibrosis states that people with CF should be 'advised against 'mucking out' stables and visiting other places where there is rotting vegetation likely to be associated with a high spore count'. Stables in particular are a potential source of Aspergillus, a fungal infection that can cause severe problems in the lungs of people with Cystic Fibrosis.

Children with allergies to dogs, cats and other animals should also exercise care around horses which can also trigger allergic reactions in those prone. These reactions can result in breathing difficulty.

Question: Air Quality and location
I am considering moving to a different part of the UK to improve the health of my son, who has Cystic Fibrosis. Could you tell me whether poor air quality has a detrimental impact on those with CF, and if so, where are the best places to live in terms of air quality.

Answer
There is no definite answer, based on research, as to the most suitable climate for someone with Cystic Fibrosis. In fact, there are no publications in over 12,000 on Medline dealing with Cystic Fibrosis and climate.

However, in a book (2004) by Dr David Orenstein, who is a very respected US authority on CF, he writes "People often wonder if there are any areas of the country (USA) that are better than other for children (and adults) with CF. From what is known now, the answer is no. In individual patients it is possible that they might do better in one geographic location than another, but so many factors influence how someone will do and patients are so different from each other that no one place has the perfect combination of factors that make good health for all people with CF...Most physicians believe that ready access to a good CF Centre is important, but other than this criterion, there is little basis for recommending one geographic area over another for someone with CF."

I would add that there is evidence, and it is obvious from clinical experience, that viral infections (colds etc) will often trigger respiratory exacerbations in people with CF and also there is good evidence that it is often at these times that Pseudomonas is isolated for the first time. Therefore, some consider that a warm, dry climate, with fewer winter 'colds' is better for someone with Cystic Fibrosis. If there were a choice I would choose such a climate rather than a temperate climate with cold winters and frequent 'colds', but I'm afraid the hard evidence to support this is not available.

Question: Growth hormones
I am 23 years old, I have CF and attend the gym three or four times a week. I also take protein supplements and have found that this combined with the exercise has increased my weight and improved my health. I'm 5ft 4" and weigh almost 9 stone, but seem to have stopped getting bigger. My arms and legs look thin, and I'd like to bulk them up.

I have been looking on the web at human growth hormone products to increase my muscle fibres. Can you recommend any of these products to help aid muscle growth and increase my weight?

Answer
First of all, it is important to remember that growth may continue into the late teens and early twenties in some male patients with Cystic Fibrosis. It may therefore be a bit early to determine whether growth has stopped.

We and others have used human growth hormone to promote growth in patients with Cystic Fibrosis and although short term growth may be accelerated there is no information on the long term effects of using growth hormone. It is possible that it may even reduce final height.

Also, in our own study we found that although height and weight gain increased, exercise capacity was reduced. Other investigators - Hunter et al (2002) and Schreiber et al (2003) have, however, reported improved lung function and better exercise capacity following treatment with growth hormone.

Despite these encouraging reports, I would be reluctant to advocate the use of growth hormone without proper medical supervision. In particular, I have seen a young man with severe liver dysfunction as a result of taking both growth hormone and steroid supplements in an attempt to improve growth and muscle development, since a high protein intake without adequate calories may inhibit growth and weight gain.

I would suggest you discuss your concerns with your CF specialist who will be able to check your bone age and confirm whether or not you have further growth potential. They will then be able to discuss whether more straightforward intervention such as further nutritional support may be of benefit.

Question: Salt tablets
My son will be seven soon and was diagnosed with Cystic Fibrosis at the age of five months. During summer, his salt intake can become a problem.

When he was younger it was not a problem to get him to drink juice with salt water solution in as it was a small amount. Now he is older and bigger, this is very much a problem. Last year I put the solution in Enlive drinks, which he did drink but as he now has to have between 30-40ml per day in hot weather, it is quite a lot, whereas before it was only two tablets.

I have been trying to teach him to swallow tablets but without success. It would be so much easier not just for taking salt, but for swallowing vitamin E capsules and his creon.

On the salt tablets it says "swallow whole - do not chew". I cut up a tablet to make it smaller therefore easier for my son to swallow but he ended up chewing it, that said, the taste did not bother him.

Have you got any ideas about how to get him to swallow tablets? If he is not bothered by the taste of the salt tablets why cant he chew them? Is there any option other than salt water solution or swallowing tablets?

Answer
We have no particular suggestions on how to get your son to swallow tablets. Trying regularly, encouragement, rewards (bribery!) are all worth trying. I have discussed this with an experienced CF paediatrician and she had no further suggestions. Children do vary greatly in their ability to swallow tablets - your son's problem is not unusual.

There is no reason why your son should not chew the salt tablets. They are coated only to avoid the taste and if he does not mind he can chew them. This sounds like the best solution to the problem.

The option which is used in most CF Centres is sachets of Dioralyte, which can be made up into a pleasant fruit flavoured drink. Each sachet contains 350mg of sodium chloride and some potassium, which would be helpful, and your son would require a supplement of 1 gram of sodium chloride per day. I am sure your doctor would prescribe some dioralyte for him to try.

Question: Creon guide
Is there a chart available to guide us when giving Creon? Our daughter is nearly eight years old, very active with a fantastic appetite, so eats lots of food at each mealtime. It would be great to know if there was a scale available for fat vs Creon to make sure we are getting it right.

Answer
I'm afraid there is no chart relating the amount of Creon to the fat taken, the reason being that people with CF have widely different requirements for pancreatic enzymes. In fact, 5% do not require any Creon at all. This is something you should discuss with your dietitian at the CF Clinic. She/he will have an idea of how many Creon your daughter will require, and is also the person to advise you on how to adjust the dose according to her abdominal symptoms, her stools and the type of food she takes.

Question: Passive smoking
What are the effects of passive smoking and stale cigarette smoke on a child with CF?

Answer
Most reported studies, but not all it should be noted, show that children with CF who live in houses where there is a regular smoker have worse respiratory function tests than those where there are no smokers. Even so, reported surveys show that some 40-50% of CF families do have one smoker.

There is no evidence that any material or substances derived from the smoke on fabric or objects in the environment etc will be harmful to the lungs.

So it would be unacceptable to smoke in the same room as a child with CF, but it would be acceptable for a child with CF to visit a smoker's house, provided there had been no smoking there on the day of the visit before the child with CF visited, and, of course during the visit.

We have discussed this with an experienced CF centre director and he agrees with this advice.

NB Passive smoking can be harmful to all children and adults, not just those with Cystic Fibrosis.

Question: Risk from pets
This may seem an odd question, but is there any risk of catching a chest infection from an animal?

Answer
It is very reasonable for you to wonder if there is any risk of catching a chest infection from an animal.

I'm pleased to say that it is not recorded that chest infections can be caught from domestic pets by people with Cystic Fibrosis. Doctors treating CF do not regard domestic pets as a risk for chest infections.

The water in fish tanks is a potential source of infection, but don't panic and get rid of the fish immediately! To be on the safe side, the person with Cystic Fibrosis should not clean the tank.

Question: Chinese herbs
A lot of my friends swear by the benefits of Chinese herbs in treating the symptoms of colds and coughs. Could these be of benefit for children with Cystic Fibrosis?

Answer
There is no scientific evidence that any complementary medicine can do anything to help a person with CF and it is essential for your health that the conventional treatments your CF clinic recommends are taken in the prescribed way.

However, provided conventional treatments are adhered to, the additions of most complementary treatments should do no harm and some people do report a benefit.

To be sure that what you are taking is safe, always consult your doctor first.

Question: Pseudomonas in soil
My little boy had been gardening, but when I blew his nose, I noticed a couple of dark speckles in his tissue. I am now concerned that this was soil and worried about Pseudomonas as he has already grown this once and is only three years old. I am so un-nerved because I really am a careful mum - unfortunately, I am now a little paranoid. Am I right to feel worried?

Answer
Even if the two dark speckles from your son's nose were soil the chance of their containing Pseudomonas would be very unlikely - so try not to worry. Certainly do not stop him playing in the garden.

It is true that Pseudomonas aeruginosa is present in many damp places and also true that when children grow the germ in their throat swabs, it is usually not known from where they caught it. Fortunately it can be eradicated if recognised early - hence the importance of having regular cultures performed so it can be recognised and treated.

If you have not seen the CF Trust publication Pseudomonas aeruginosa Infection in People with Cystic Fibrosis. Suggestions for Prevention and Control, November 2004, it would be worth reading from our publications library.

Question: Garden centres
As a family we are considering buying a wholesale nursery, propagating and rearing plants etc. We have been told that coming in close contact with some plants and stagnant water is not advisable for people with Cystic Fibrosis. I do wonder whether we should go any further with this venture, as I see thousands of potted plants with fungal type growths on the soil surface plus pools of water laying in various locations, in polytunnels and tractor furrows. Can you advise whether this type of occupation could be dangerous to our son, not that he would be in close contact with the business for several years yet of course. Could dangerous spores be carried into the house on clothing, etc.

Answer
There is no doubt that dampness and vegetation do encourage the growth of spores and other potentially harmful organisms such as Pseudomonas as you have observed. Certainly enclosed places, with increased humidity, stagnant water and rotting vegetation often contain high levels of Aspergillus spores and also other organisms which not uncommonly cause complications in people with Cystic Fibrosis. Stables are a good example.

One has to say that it would not be wise for a child, or indeed anyone, with CF to spend a great deal of time in such places and we would advise against this type of occupation for someone with Cystic Fibrosis.

Question: Untreated swimming pools
We have booked a villa with a private pool in Portugal for our holidays and I am not sure if the pool is chlorinated or not. Will this be a problem for my son who has Cystic Fibrosis?

Answer
If swimming pools are not professionally maintained and checked for bacteria, they pose a risk to people with CF as the water may contain gram negative bacteria such as Pseudomonas aeruginosa. Even when alleged to be chlorinated it is wise to check with the owners that the pool is maintained by a reputable firm. A recent Which survey in the UK showed that some pools were not maintained adequately and contained significant numbers of bacteria. So an untreated pool would be a risk.

If a person were receiving nebulised colistin this would definitely provide some degree of protection as any Pseudomonas bacteria entering the mouth and throat would be killed by the antibiotic.


Question: Risk from sand
Is there any risk to a person with Cystic Fibrosis from playing in the sand on the beach (particularly from Pseudomonas infection) or from paddling in the sea? Also, is there a risk from playing in isolated sand in sand pits (wet or dry) where other children also use it and it may remain wet for a number of days?

Answer
There is no risk from the sea or the sand at the seaside. The salt water is a deterrent to growth of bacteria such as Pseudomonas aeruginosa. Also an ordinary sand pit should not be a risk provided the sand is reasonably clean and not contaminated by pets, etc.

Question: Risk from fish tanks
My younger brother has CF and grows Pseudomonas aeruginosa already. I want to buy a tropical fish tank, but obviously due to the warm water bacteria are bound to grown even if the tank is cleaned regularly. So I just wonder whether or not anything would grow in the water that would affect by brother's condition?

Answer
Fish tanks are not usually a risk to people with CF provided they are clean and maintained properly. It would be better if the person with CF is not the one responsible for cleaning the tank, but from your question it sounds as if you are the one who would be looking after the fish.

Question: Garlic supplements
I was interested to read about research on treating patients with CF with garlic capsules. I have a six-year-old son with CF and we have given him high strength garlic capsules all last winter. This has been his best winter ever, children in his class at school have been knocked down with a dreadful cough/cold 'flu type virus, many children have been absent from school for well over a week. Our son has been the fittest, healthiest pupil in class with perfect attendance all winter long. Can you tell me more about the research and likely outcomes?

Answer
It is encouraging to hear that your son has been doing so well on high strength garlic capsules. To date there have been no publications or studies showing its value in Cystic Fibrosis. However, there are nearly 2,000 references to the use of garlic in a wide variety of other conditions most recently concerning cancer and antioxidants.

The Nottingham CF Centre hopes to start a clinical trial to study the benefits of garlic.

The CF Trust is always interested in new treatments, which may help people with CF and looks forward with interest to the results of clinical trials of garlic. However it should be stressed that it is essential for the future health of those with CF that conventional treatments recommended by the Specialist CF Centres or CF Clinics should continue in the prescribed way.

Question: Tipping during physiotherapy
I have a six month old son with Cystic Fibrosis and I have always regularly given him physiotherapy and it has become a pleasant routine for us morning and evening with extra sessions when he sounds a little "chesty". I have recently heard that it is not recommended that babies should be "tipped" (with head facing down) during physiotherapy as this might be the cause of problems later in life. Can you let me know more about this and whether I should be "tipping" my son during physio' sessions?


Answer:
Traditionally chest physiotherapy has always been given to babies and children with CF in a head down tipped position. However, there is currently a lot of debate about the use of tipping in Cystic Fibrosis. Some studies have suggested that tipping babies head down may increase the risk of reflux (when milk comes back up from the stomach), which in turn can lead to small amounts of aspiration into the airways. It is suggested that this may contribute to chest problems (wheezing and infection). On the other hand, there are also studies that do not support these findings and these contradictory views have led to some confusion regarding this topic.

Further work is definitely needed to determine the exact effects of tipping with chest physiotherapy for patients with Cystic Fibrosis. In the mean time our advice would be that each patient should be carefully assessed by the CF team and an individual physiotherapy programme formulated. If there is any suspicion of an abnormal level of reflux (remember all babies do reflux a little) this should be investigated, treated and the chest physiotherapy programme modified accordingly. On this basis I do not think there is any need for you to stop tipping your son during physio unless you and your CF team think it is causing problems.


Question: Storing Colomycin
My daughter has 2.5 ml of nebulised Colomycin twice daily. She has been on this medication for about 18 months now. I was advised that we could make up a whole box of 10.5 ml vials at a time as they would keep perfectly well in the fridge and this would save us considerable time instead of making up a vial each day. However, a friend whose daughter also has nebulised Colomycin has been advised that Colomycin should only be made up a maximum of 24 hours in advice. Please can you tell me how far in advance I can safely make up the Colomycin as I am rather confused?


Answer:
It is strongly recommended that any Colomycin made in advance is stored in the refrigerator and used within 24 hours. This is also the recommendations made by the manufacturers of Colomycin.

Once made up the solution is chemically stable for 28 days when stored in a fridge. However, from a microbiological point of view, solutions should ideally be used immediately. If this is not possible, solutions can be made and stored in the fridge for no longer than 24 hours. This is because unless the solutions are made in sterile conditions, ie in a hospital pharmacy aseptic unit, there is a risk that bacteria enter the prepared solution, and some may continue to grown and multiply. This would clearly represent a risk to the patient.


Question: Nebuliser servicing
I have had my nebuliser for a long time now. How long should the plastic nebuliser last and does the compressor need servicing at some stage?

Answer:
There is some variation between different hospitals in the frequency that they recommend nebulisers to be serviced. Most hospitals recommend that the nebuliser compressor is serviced on an annual basis, but some recommend every six months. The nebuliser manufacturers typically recommend annual servicing, although one of the major manufacturers has recently introduced a model, which only requires servicing every three years.

The service is usually performed by the hospital engineering department. At the service, the engineer cleans the machine, changes any internal filters and tests the compressor output. If the output of the machine is low then further work such as changing the compressor diaphragm may be required. Hospital engineers report that the output from the nebuliser is very frequently reduced if servicing occurs infrequently. This will result in less drug being delivered to the lungs with reduced clinical benefit. Disposable parts are generally changed every 12 months, but frequently need changing more often than this, particularly if the patient has a habit of chewing the end of the mouthpiece! So keep getting your nebuliser serviced regularly!

There is a small chance of bacteria being transmitted from the nebuliser chamber to the lungs. This is not of any practical importance if the patient just uses their own nebuliser, but do not be tempted to share nebulisers with other patients as this might result in transmission of infection. The hospital should be able to loan you a nebuliser whilst your own nebuliser is being serviced or if it breaks down."


Question: Vitamin K
Specialist CF Centres seem to vary on whether they prescribe regular supplements of vitamin K; also I hear that the vitamin may be related in some way to osteoporosis. Should I be having regular supplements of this vitamin and, if so, is it possible to determine if the supplements are adequate?


Answer:
Vitamin K is one of the fat-soluble vitamins like vitamin A, D, and E. This means that it is absorbed along with the dietary fat. Therefore people with CF are at risk of malabsorption of vitamin K even if they take pancreatic enzyme supplements, e.g. creon. Probably because the exact role of vitamin K was poorly understood until recently, routine supplements have not been given. This may appear slightly illogical as we all prescribe additional vitamin A, D, and E. We have recently learnt that vitamin K may play a role in bone health and the prevention of osteoporosis but this has not yet been fully researched.

What is the present position? Varied. Some units prescribe vitamin K supplements to all their patients. Some units give it only to those patients who show abnormal blood clotting times (vitamin K is used to make the clotting factors in the liver). We are researching into this area. It is likely in my opinion that all patients will be advised to take vitamin K in the future. The problem is that if we don't wait for the research to be done we will never know a) how much to give b) when to give it c) does it make a difference to bone strength.


Question: Scuba diving
My 10 year old son who has Cystic Fibrosis is a keen swimmer and enjoys weekly snorkelling and life saving lessons at his local swimming club. My husband Scuba dives regularly in the UK and abroad. Naturally, my son is beginning to take a keen interest in Scuba diving. Would it be totally unsafe for him to participate in this sport when he is older due to lung pressure or would he be safe to scuba dive to a certain depth?


Answer:
First it is very important that you discuss with your son's CF consultant whether it is safe for him to go scuba diving. The consultant will be familiar with the condition of his chest in terms of infection, damage and associated asthma.

However, we have discussed the general question of SCUBA diving with a CF expert and can provide you with a few facts which may help you make the decision. His reply was along the following lines:

There are two possible problems with people with CF and SCUBA diving.

• A possible infection risk. Breathing equipment is often wet and could become colonised with bacteria such as Pseudomonas. If advice is taken from infection control nurse/team at the hospital this problem could probably be overcome.

• CF "with lung disease" is regarded as a contraindication to diving. This is a statement in the very detailed British Thoracic Societies (BTS) Guidelines on the respiratory fitness for diving. Clearly the question here is what do they mean by "lung disease"? However, many children with CF have very minimal lung involvement these days and it is suggested that the section of these BTS guidelines for diving with asthma and lung function may be useful for children with CF who have little or no lung disease and normal respiratory function. These are summarised as follows:

"Subjects with asthma should be advised not to dive if they have wheeze precipitated by exercise cold or emotion"

"Subjects with asthma may be permitted to dive if, with or without regular inhaled anti-inflammatory agents (this means inhaled steroids), they are free of asthma symptoms, have normal respiratory function tests (FEV1 >80% and FEV!/FVC ratio of >70%) and have a less than 15% fall in FEV1% with exercise."

"Subjects with asthma should monitor their asthma with regular twice daily peak flow measurements and should refrain from diving if they have symptoms requiring relief medication (i.e. Ventolin etc) in the 48 hours preceding the dive, reduced peak flow rate (>10% below their best values) and increased peak flow variability of > than 20%."

Clearly there is always the potential for complications. However, if chest disease is minimal, precautions are taken and any risk is understood, SCUBA diving should be possible in many children with Cystic Fibrosis. In every event we do stress that it is essential you discuss the question with your son's consultant who has all the facts or is a position to obtain them before advising you.

Question: Enzyme time of effect
Sometimes when I make my daughter a meal she refuses to eat it so I put it in the fridge for later. Will the Creon that I've already put in the food work just as well once the food is re-heated? My daughter is 17 months-old so cannot swallow the capsules.

Answer:
The microspheres or the small particles of Creon should not be mixed with the food on the plate; they should be taken with a small amount of liquid or food on a spoon and given at the start of the meal. The coating around the microspheres of Creon is there to protect the digestive enzymes from being inactivated in the acid environment of the stomach. If the food they are mixed with is not acidic, the coating will dissolve and the enzymes will not work. The likelihood of this happening is further increased the longer the enzymes (Creon) are left in the food before it is eaten. I would therefore advise against this practice.

It is quite common for toddlers to go through periods of food refusal. If all of the enzymes are given at the beginning of the meal this can result in giving too many enzymes for the food that has been eaten. If this is a common problem it is best to give half the dose at the beginning and half in the middle. Some toddlers, who have fast transit times, may benefit from giving the enzymes in the middle and at the end of the meal so that the enzymes do not go through the gut ahead of food and result in the child getting a sore bottom. If the food is left it can be put in the fridge but the enzymes should NOT be mixed into the whole feed. They should be given separately as described above.

Question: Vitamin E
I have twins with Cystic Fibrosis. One son is a lot fitter than the other. One takes vitamin E supplements in liquid form, but the other cannot take this as it makes him vomit, no matter how much we try to disguise it. He has not been receiving vitamin E for almost six months. We are still trying to find somewhere that can supply vitamin E in the dose required.

Could you please advise me why vitamin E supplements are so important in Cystic Fibrosis. I have tried to find out from other sources and have had no luck. Also, can vitamin E have anything to do with a six-year-old child not being able to pay attention for any length of time?

Answer:
Vitamin E is one of the fat soluble vitamins that the majority of patients with Cystic Fibrosis require supplementing. From measuring blood levels of vitamin E, we know that because most patients lose a lot of vitamin E in their stools, they need to take additional supplementation at a much higher level than the diet would provide. Therefore liquid, tablet or capsule preparations must be taken. It is important that you know what dose your son requires. Once this is known you could try different preparations eg. crushing tablets or the smaller capsules that have recently become available. Your hospital should be able to tell you the appropriate dose.

In CF, one of the most important roles of vitamin E is that it is a powerful antioxidant. Antioxidants protect cell membranes from damage, including those in the lungs. Vitamin E may therefore be important in controlling the progression of lung disease. Deficiency of vitamin E has also been linked to poor nerve function and a type of anaemia. Vitamin E may also be important for good eyesight. However, all of these deficiency symptoms are rare and I am not aware that vitamin E has been linked with poor attention skills.

I would suggest that your son has his blood vitamin E levels checked to determine the dose that he requires and then tries the different forms of supplement suggested above.

Question: Arthritis
"Arthritis", that's what our doctor told us the last time we took our daughter aged 14 to the CF Clinic and that is the cause of her joint pain. With everything else she has to manage with her CF, she now has the added treatment connected to the discomfort and pain associated with arthritis and joint pain. We want to help her has much as possible. Can you explain why someone with CF is more likely to be affected by arthritis and what treatments are available?

Answer:
Arthritis literally means inflammation in joints. When we use the word, the mind can wrongly jump to 'rheumatoid arthritis' which has nothing to do with Cystic Fibrosis. There is a classical 'CF arthritis', which is definitely not rheumatoid arthritis. It typically occurs in episodes of 10-14 days of severe pain and swelling of large joints like the knee, sometimes it is associated with skin rashes on the legs. It probably occurs because the body makes antibodies against infections, which also happen to be antibodies against the joints.

Most patients with CF with limb pain do not have this arthritis. The majority have some muscle weakness which exposes minor defects in joint mechanics and previously painless joints (especially knees) can become very painful. The obvious answer is to try to build up muscle power as well as take painkillers.

Another cause of pain is irritation of the surface covering of the bone (HPOA). In this there is tenderness along the bone as well as in the joints; this can be temporarily lessened by elevating the limb. Vigorous treatment of the chest may be helpful along with anti-inflammatory tablets. Ciprofloxacin, an antibiotic, can very occasionally produce thickening of tendons around joints which can raise the suspicion of arthritis.

If the family is unfortunate enough to have an arthritic gene running through it, the person with CF may suffer from this even though it is nothing to do with Cystic Fibrosis.

Treatment is not yet satisfactory for the episodic condition. It consists of anti-inflammatory tablets and rest in the exacerbations (with additional painkillers if necessary).

For the other conditions, anti-inflammatory tablets, additional painkillers, the principles of the basics of CF treatment -known so well to readers- and attempts to improve muscle power by exercise are the best that can be currently offered.

If a rheumatoid condition unassociated with CF appears, it can be treated in a conventional way, probably best by collaboration between a rheumatologist and CF expert. Working together with the patient and family they must try to fend off the depression and nihilism that makes all pain worse.

Question: Liver disease
My daughter is 12 years old and has Cystic Fibrosis and CF-related liver disease with an enlarged liver and spleen. She is not on any treatment for her liver but is monitored and being checked regularly for oesophageal varices. I have been advised that at her stage, ursodeoxycholic acid is highly unlikely to be of any benefit and that there is no good evidence that long term it makes any difference to a patient's outcome.

There seems to be conflicting views on its benefits but I have accepted the consultant's judgment and she is not taking it as I do not want her to have to tolerate anything else unless it is likely to benefit her. However, at the back of my mind I am wondering should I try it as I don't want her to miss out on something which may help. Do you have any advice for me?

Answer:
It is true there are no long-term controlled trials on the use of ursodeoxycholic acid (URSO) in people with CF, but there is considerable experience of its use since this was first introduced in 1990.

URSO seems to arrest the progress of liver disease if given at an early stage when there is first any abnormality of the liver function tests. This usually occurs at the time of an annual review when slight abnormality of the liver function blood tests is noted.

In more advanced liver involvement the effect is less clear cut but most CF doctors would put all people with CF who have liver involvement of whatever degree on long term URSO and taurine as it may be of some help. So there may be some benefit - it may well do some good and is unlikely to do any harm.

Question: Curcumin
I have a ten-year-old nephew with CF who from birth has had an operation, been in hospital three or four times needing IVs, due to several chest infections and is, as you can imagine on daily doses of medicines.

I have a friend, whose nephew also has CF, who takes homeopathic medicines including cumin, which the family was told helps to keep the mucus away - it seems to be effective as he has never needed hospital intervention and has only recently needed antibiotics to clear off a chest infection.

I was just wondering what your viewpoint is on homeopathic medicine for CF patients and whether you had heard of using cumin as a treatment?

Answer:
There is no evidence that any homeopathic medicines are of any value in Cystic Fibrosis and we would not advise their use. With regard to curcumin (cumin), this was found to have some value in CF mice but the results could not be repeated and also a small trial in people with CF showed no benefit either in their symptoms or laboratory tests; however, another trial is in progress so it may eventually prove to be of some value. We would certainly not advise taking curcumin at this stage particularly as the first tests in patients showed no benefit

Question: Carrier testing - where and how?
I was wondering how to get tested to see if I'm a carrier of the CF gene. My partner is and has a daughter with CF, we want to start a family but want to make sure I am not a carrier first.

Answer:
As you did not say where you are in the UK, go to the Genetic Interest Group website www.gig.org.uk and click on Genetic Services in the UK. Contact the Regional Genetic centre that covers the area in which you live, they will tell you what you need to do.

Question: Carrier testing - parental consent needed?
A few years ago my cousin was diagnosed with CF and there is a possibility that I myself am a carrier. I am interested in having the screening test done to check whether or not I am. However, at the moment I am 16 and I was wondering if I can have the test done without parental consent and how much the test itself would cost.

Answer:
As your cousin was diagnosed with CF you can be screened without having to pay for testing. I suggest you checkout our Cascade screening factsheet and Genetics booklet on the website www.cftrust.org.uk/aboutcf/publications

If you go to the Genetics Interest Group website (details above) you can find the details for your Regional Genetic Service and give them a ring. I doubt that there is a need for parental consent at 16 as in many areas you are considered to be an adult. Your Regional Genetic centre should be able to answer all your questions.

It seems as if you have a very mature, responsible attitude. As it is your cousin that has CF, the risk of you being a carrier is 1 in 4, as shown in the Cascade screening factsheet.

Question: Liver scarring
My 16-year-old grandson, who has CF, had a scan this morning and the doctor mentioned 'liver scarring'. Can you please explain what is meant by liver scarring, as it is causing us considerable concern?

Answer:
A minority of people with CF have liver problems. The bile (the digestive juice secreted by the liver) is rather sticky (as are the secretions in the chest) and this can cause some blockage in the liver which eventually can cause some degree of fibrosis or scarring - this would be described as "scarring" when seen on a liver scan. The good news is that although the liver may be slightly "scarred" the function of the liver cells themselves remains good and it is unusual for people with CF to develop serious liver problems ¬¬- the situation usually remains stable.

If there is any evidence of liver abnormality, either on blood tests or on scans, it is usual to start the patient on a drug called ursodeoxycholic acid ("URSO" for short) which improves the flow of bile and in many patients can prevent further abnormality provided it is started early.

The liver problems in CF are described in more detail on the following website www.cysticfibrosismedicine.com. Go to Public -> Articles -> Gastrointestinal system -> Liver disease. You may find it a bit complicated but it contains the main facts which have not changed significantly since 2001 when the article was written.

Your daughter should ask the CF consultant for a discussion on the liver findings.

Question: Creon in stool
My three-month-old daughter is having around one¬ or two bowel movements per day. Is it normal to be able to see Creon particles in the stool?

Answer:
Creon particles (microspheres) may appear in a baby's stools and this should cause no concern. The contents of the bowel travel through more rapidly in infants and the appearance of the Creon means that those few particles have not had time to dissolve.

Question: Shingles
We are supposed to be having friends staying this weekend but one of them caught shingles last week. My question is, is this of any concern if we were to let them visit as my son of fifteen is a CF sufferer. He is also starting IV's this Wednesday.

Answer:
If your son with CF has had chickenpox and is not receiving steroid treatment there will be no risk from the visitor with shingles.
If he has not had chickenpox in the past or is at present on steroids there is a risk and the person with shingles should not visit.

Question: E.coli in cough swabs
My son has grown E.coli in his cough swabs recently. I thought that this bacteria was normally found in the gut? Although my son has no symptoms, he is being treated to eradicate the E.coli. Is this normal and should I be concerned that he is growing this bacteria outside of the gut?

Answer
E.coli is not rare in cultures from the upper respiratory tract in children. It is an organism which is a normal inhabitant of the gut. Occasionally in CF it can cause chest infections depending on the particular type. If your child is well, and has no cough, it is not of great concern. However in people with CF, if any potentially harmful organism grows from respiratory cultures, even if there are no symptoms, many doctors would treat with an appropriate antibiotic to eradicate it to ensure that it did not give any trouble.

Question: Gene therapy trials
We have a five-year-old son with Cystic Fibrosis, who is in good health. How do we find out if he could become a subject for the gene therapy trials, whether he is eligible and how to apply? How do we find out about clinical trials in general?

Answer:
The UKCF Gene Therapy Consortium website shows the progress of the clinical trials - www.cfgenetherapy.org.uk In the section entitled 'Clinical Programme', you will see that the Consortium have initially not recruited any children under the age of 12. This is because the regulatory authorities and the Gene Therapy Consortium have agreed on this age as they believe that children of 12 and over are able to decide, along with their parents, whether they want to participate in this trial. The most important thing for your son is that his health stays as good as it is now, so that he can benefit in the future from any advances in new treatments, which we hope will include gene therapy.

Question: Treatment of lung lobe collapse
My two-year-old son has recently been diagnosed with Cystic Fibrosis. He has always suffered from bad bowels and after a year with a persistent cough a specialist started investigating. When he was taken to hospital for having Meningitis C he had a chest X-ray which revealed the lower lobe of his left lung had partially collapsed due to recurrent, untreated infections. This is now being treated with antibiotics. I was wondering if this is common in CF sufferers and what other treatments can be used to reflate the lung?

Answer:
Collapse of a lobe of the lungs is common in children with Cystic Fibrosis, particularly if there has been delay in making the diagnosis.

The treatment usually involves nebulised inhalations of saline or a bronchodilator to loosen the secretions and intensive physiotherapy also to mobilise the secretions blocking the bronchial tubes. Pulmozyme is an inhalation which loosens secretions (a mucolytic) and may also be used. Also intensive antibiotic treatment, usually intravenously, is given to reduce the infection and this also tends to loosen the secretions. If there is not rapid improvement in the chest X-ray, over a week or so, a bronchoscopy is performed to suck out the thick infected secretions from the bronchi and also to ensure that there is no foreign body blocking the bronchial tube to the collapsed lobe. These are the main measures used to treat a lobar collapse in a child with Cystic Fibrosis.

Question: Open fires and dust in house
We live in a house that is heated by an open fire. Will the smoke from this cause a problem with for someone with CF? We also live next to a farm so our house is very dusty.

Answer:
A coal fire should be no problem provided it works properly i.e. the smoke is taken up the chimney efficiently. Farms can be a problem occasionally. It is usually advised that people with CF avoid spending much time in stables where there is damp straw and certainly they should avoid "mucking out" stables and animal houses. The reason is that fungal spores of Aspergillus fumigatus are present in the air in large numbers at such times and can cause an allergic reaction in the lungs of people with CF (so-called "allergic bronchopulmonary aspergillosis"). Although this can be treated, it is better to avoid it.

Question: Stress fracture of the ribs
This may seem like a bizarre question, but can people with CF suffer with stress fractures of the ribs?
My 17-year-old daughter complained of a muscle type pain in her lower left rib area about two weeks ago, that only really hurt when moving or bending. As we were in clinic within days we did a cough swab which came back clear. My daughter then went to a theme park and was generally thrown around on hair raising rides! On coming home the pain is at a specific point and she is in quite a lot of pain when moving. Although she is clear of infection at the moment, she has had a nasty cough through the summer, this being the reason I am querying "stress fractures"

Answer:
Fractures are definitely more common in people with CF than in the general population. Some degree of osteoporosis (thinning of the bones) is relatively common in adults with CF and more likely if they have more severe chest disease. In a study of 107 adults with CF at the Brompton Hospital in London no less than 35% had suffered a fracture at some stage and 9% of these were rib fractures. The Cystic Fibrosis Trust has recently published a factsheet on bone health as well as a more detailed consensus document for health professionals - both documents can be downloaded from the CF Trust website and may be a useful source of further information.

Question: Applying for housing
I am writing in the hope that you might be able to help me. My husband (who has CF) and I are currently renting a property, however we are wishing to move as the house is damp and often suffers with mould. Whilst my husband is well I am concerned that this may become an issue and I would like us to find somewhere more suitable. At the moment we cannot afford to buy our own house so we are applying to the council to go on the housing list. What kind of things do I need to include or consider with regards to my husband's condition?

Answer:
The things you need to consider with regard to CF are the type of property you are in and the type of property you would like to be in. Somewhere free of damp and mould is important. It could be that you need a minimum of two bedrooms, one for treatment and storage. Possibly, you will be looking for a flat on the ground floor without the need to climb stairs. The Cystic Fibrosis Trust Support Service can write letters in support of such applications, and it may be worth asking your CF team if they will also write a letter.
If you have access to a social worker, they should be able to provide you with more information. We also have a series of housing factsheets on our website www.cftrust.org.uk

Questions: Ibuprofen benefits and risks
Is there any benefit to taking regular ibuprofen as part of a treatment regimen and is there any risk?

Answer:
Ibuprofen is a drug which will reduce inflammation in body tissues - a so-called "non-steroidal anti-inflammatory" drug. As inflammation of the airways is an important part of the chest problems in CF and also thought to be one of the damaging factors, the use of long term ibuprofen was tried in people with CF. There have been two main clinical trials; the first was in the USA and published in 1995 (Konstan et al, N Engl J Med 1995; 332:848-854). This was a major study funded by the CF Foundation to determine the benefits of treating inflammation in the CF airways with ibuprofen. Eighty five children and adults received high doses of oral ibuprofen or placebo twice daily for four years Those taking ibuprofen had a marginally slower annual rate of decline of their respiratory function (% FEV1 - 2.17 vs. -3.60); those who took ibuprofen regularly for four years had slower annual decline in FEV1 (-1.48) - they also had better body weight and chest X-ray. These benefits were evident in those less than 13 years. There was no significant difference between the ibuprofen and placebo groups in the frequency of hospitalization. The authors concluded that in patients with CF and "mild" lung disease, high-dose ibuprofen, taken consistently for four years, significantly slows the progression of the lung disease without serious adverse effects.

However, the benefits of ibuprofen in this trial were not convincing to most CF doctors. Subsequently the modest effect and frequent, at times serious, side effects prevented the widespread use of ibuprofen and the treatment never became popular even in the United States, where only some 5% of patients on the CF Foundation's registry are reported to be taking the drug. For example Fennell PB et al (J Cyst Fibros 2007; 6:153-158) reported half their patients stopped ibuprofen because of serious side effects (mainly gastrointestinal pain and bleeding) and the treatment had no effect on either the rate of decline of lung function or hospitalisation rates in those who could tolerate the drug. Yet subsequent reports from Michael Konstan, who led the 1995 trial, remained supportive (Konstan MW et al. Am J Resp Crit Care Med 2007; 176:1084-1089) and a separate study from Canada did show slower decline in forced vital capacity (FVC) but not of FEV1 (Lands LC et al. J Pediatr 2007; 151:249-254). An interesting and unrelated observational report suggested the recurrence of nasal polyps was reduced while patients were receiving ibuprofen (Lindstrom DR et al. J Otolaryngol 2007; 36:309-314).

So, few CF doctors in the UK advise using regular ibuprofen. The benefits are very marginal and frankly unconvincing, blood levels have to be checked regularly and the side effects are frequent and potentially serious. One major UK CF Centre concludes "there is not sufficient evidence for their routine use" and we would agree.

Question: CF-related arthropathy
My partner has just been diagnosed as having CF-related arthropathy. From what we understand, the periodic episodes can be triggered by immune responses. Is there any way, to your knowledge, of preventing or even minimising severe episodes from occurring during periods of illness?

Answer:
Episodic arthritis affects between 2% and 8% of people with Cystic Fibrosis. As you note, it often worsens at times of infection and settles when the chest infection is treated suggesting that the immune response against the chest infection "spills over" to affect the joint/s. The best possible control of the chest infection and perhaps earlier treatment of exacerbations would be helpful. We are not aware of any other preventive measures.

The usual advice, which I'm sure you will have had, is to make sure there are no other causes for the joint pains - for example ciprofloxacin can cause joint pains also other drug and antibiotic allergies. The treatment is given as required with non-steroidal anti-inflammatory drugs such as ibuprofen (Brufen) and if necessary short courses of steroids in a few patients.

Question: Aspergillus
What is the CF Trust's current take on Aspergillus? My child cultured this for years and we were told it was not clinically significant, although their lung function was very poor. We recently changed to another CF Centre where I again raised my concerns and they immediately started treatment with Iatraconozole. Within a few weeks the lung function had shot up and was better than it had ever been; there was also much less sputum, the colour and consistency changed, the chest was much easier to clear and stamina improved.

From reading around this subject I imagine that it is not easy to distinguish between the different manifestations of Aspergillus and the usual symptoms of Cystic Fibrosis. As parents, what signs and symptoms should we look out for? Are there any guidelines for good practice in regard to this?

Answer:
The CF Trust don't have a recent take on Aspergillus - the most recent statement by one of our expert committees being in the "Antibiotic treatment for cystic fibrosis" 2002 in Section 7.5 on page 53 which you will find in the publications section of our website. This is still valid although it is now five years old - the only change being there are a few new antifungal agents such as voriconazole. There is also an Aspergillus website (www.aspergillus.org.uk) and a good section on the website www.cysticfibrosismedicine.com under articles.

There are no clear guidelines on treatment and opinions do vary on how to treat a totally asymptomatic person with CF who has a positive culture. However, the fungus can cause considerable problems particularly when an allergic reaction to it develops in the lung tissue - so-called allergic bronchopulmonary aspergillosis or ABPA.

It is usually considered as causing trouble when new chest symptoms do not respond to the usual antibiotics but do so when one of the antifungal drugs such as itraconazole is tried. As in your case, the favourable response to treatment usually confirms that Aspergillus was a likely cause of the symptoms. So Aspergillus is considered when symptoms (and indeed x-ray changes and respiratory functions tests), do not respond to usual antibiotic treatment or there is a wheezy type cough which doesn't settle.

You are quite right to ask for every result and clinical finding to be fully explained. It is good that your child has responded so well to treatment with itraconazole which suggests that aspergillus was making an important contribution to your child's symptoms.

Aspergillus spores are very common and particularly found in damp houses, water damaged walls and ceilings. They are also found in mouldy hay as found in stables and animal houses, and in building sites where building work is in progress.

Question: Condensation
I have a three-year-old boy with Cystic Fibrosis. I wanted to know if I should be concerned about the really bad condensation on all my windows, as it seems to produce an awful lot of mould. Should I get it looked at, and would if affect my son's health in any way?

Answer:
It is not good for people with CF to live in houses where there is excessive condensation leading to the presence of moulds. One mould in particular - Aspergillus - can give troublesome allergic reactions in the chest (see question above).

We would certainly suggest that you get the condensation seen too, for it could certainly adversely affect your son's health.

Question: Pseudomonas
I have just read the CF Trust consensus document regarding Pseudomonas aeruginosa. My eleven-month-old daughter grew pseudomonas at four months old and has had negative cough swab results since. Does this mean that her life expectancy will be reduced, as this has happened in her first two years of life?

Answer:
Pseudomonas is a common germ in the general environment. It is not uncommon for a single routine throat swab to grow Pseudomonas even in a small infant with CF and then for all future swabs to be negative. The fact that your infant has subsequently had negative swabs almost certainly indicates that the Pseudomonas in no longer present in her airways and certainly the one positive culture does not mean that it will affect her long term outlook.

Question: CF research in the news
There have been several news stories in recent months about possible treatments for Cystic Fibrosis, such as the French research into the drug Miglustat, which received a lot of news coverage last October. The Cystic Fibrosis Trust does not seem to give much coverage to this research - why not?

Answer:
Reporting of medical research news in the media understandably can often raise the hopes of those affected by the condition involved. However much of the research reported is often at very preliminary stages and may have only been tested in vitro or on animal models - in the case of Miglustat, on mice.

It is important to bear in mind that any trials on animals such as mice, however successful, do not necessarily translate into benefits for people, and that trials on mice mean that a drug is a long way from coming to the market - it has to go through a great many more stages after that before it can be made available, which may include several years of clinical trials in humans.

Of course, anything that could help people with Cystic Fibrosis is important and of great interest to us, so we keep our fingers crossed that any potentially beneficial research has a positive outcome. However we prefer to only give coverage to CF research in our magazines and websites when there is something of significant interest or of imminent clinical benefit to report

Question: Teeth discolouration
My eight-year-old granddaughter with CF is just getting her second teeth. Some have yellow blotches. Is this caused by her condition? If so, can it be remedied other than by having them veneered?

Answer:
It is well established that children with CF have more defects in the enamel of their permanent teeth than children who do not have Cystic Fibrosis. However, a number of studies have shown that antibiotics and enzymes may provide some protection against dental caries. Also it is likely that CFTR may itself play a role in enamel formation. Discolouration of the teeth by taking oral tetracyclines was described in the 1960s and these antibiotics are now avoided in young children with Cystic Fibrosis.

A recent publication from a major CF Centre concludes: "Enamel defects particularly enamel opacities which can be disfiguring are more common in CF patients. Early regular dental visits may prevent such defects becoming mentally disabling, and would permit the removal of dental calculus deposits."

It would be wise to discuss the matter with your granddaughter's paediatrician - perhaps there is a dental surgeon who sees the children with CF attending the CF unit?

Question: DLA
Both of my children have Cystic Fibrosis, and neither receives the full disability living allowance (DLA). We know of many other children with CF who have the full benefit. My son is now 15 and I would like him to be able to have a driving licence at 16 but this is not allowed unless he is on full disability allowance. I feel he is losing out twice - can you help?

Not all people with CF on high rate care and mobility are on the right rates. For high rate care, the person should be on overnight feeds or oxygen. For high rate mobility, they must be virtually unable to walk or experience severe difficulties. In this day and age, fewer adults are on the high rate of mobility as they keep so well. You have to demonstrate on application forms that the recipient qualifies. For instance, if a youngster can play a game of football, they are unlikely to be able to demonstrate that they experience severe difficulties when walking a short distance on flat ground.

However, some children do say that they play football when in fact they are just standing in goal, which should of course be reflected in any application.

Question: Safety of air conditioning
Is air conditioning or any other form of air cooling suitable for use in the same room as someone with Cystic Fibrosis? My daughter, who is 25, finds the hot summer weather almost unbearable. She is on oxygen 24/7 and is on the waiting list for a lung transplant.

Answer:
There is no reason why a person with CF should not be in a room with well-maintained air conditioning. In fact, in hot weather it would be beneficial to avoid overheating as people with CF are susceptible to heat exhaustion due to the excessive amounts of salt they lose in their sweat.

Question: Borderline sweat test
My son recently had a sweat test to see if he had Cystic Fibrosis. It was borderline. He seems very well. The CF doctor I saw said he would still like to see him regularly to keep an eye on him. Is this necessary?

Answer:
Yes, it is necessary, because although your son seems very well, the doctor would like to check on him regularly to ensure he is not developing any symptoms of CF and indeed to keep symptoms at bay. Otherwise, he may develop chest infections or other complications of CF, which may well not be treated properly by a general doctor who does not have the necessary expertise in Cystic Fibrosis. Of course, he may stay very well for years and he may never develop symptoms, but the time taken for outpatients appointments with a CF doctor is time well spent. If he does develop symptoms, these will be well controlled and minimised, whereas if he is not cared for by a CF multi-disciplinary team, they could become more serious.

Question: Newborn screening
My 17 week old son has had a persistent cough and wheezing since five weeks old. His screening results at birth came back normal. Is there a chance that he could have Cystic Fibrosis and that it wasn't detected at the screening stage?

Answer:
Most young babies who cough and wheeze, usually after a cold, do not have Cystic Fibrosis. The newborn screening test is very reliable and infants with CF are very rarely missed. However, if an infant or child has persistent signs suggestive of CF even if the infant has been found negative on neonatal screening. This reassures everyone concerned.

Question: Bloated tummies
My six-year-old daughter has CF. Although she is very fit and is tall for her age, ever since we can remember her tummy has been larger than normal. The rest of her body is trim and in proportion, and her weight is normal. The hospital CF team don't think it is a problem, but I am also worried she may get teased if it is like this when she is older.

Answer:
The usual reason for the tummy being prominent in a young person with CF is that the malabsorption of food is not well controlled. This can occur even if there is no complaint of abdominal pain or abnormal stools, and enzymes are being taken.

The first step is to check that there is not an excess of fat in the stools and also that the lower bowel is not overloaded as can occur at times in CF (this is obvious on a plain X-ray of the abdomen).

You should discuss this with the CF team at your Centre and let them know you are worried - they may not appreciate that you are concerned. I'm afraid we cannot, nor would it be appropriate for us to, give specific advice on individual cases.

Question: Fluid intake in hot weather
Does someone with Cystic Fibrosis require more fluids in hot weather than someone without?

Answer:
Those with Cystic Fibrosis may require salt tablets in hot weather as they may lose salt through sweating. The majority of individuals whether they have CF or not, or do not drink enough of the right type of fluid on a daily basis.

Question: Carrier symptoms
I am almost 50 years old and I would like some help understanding my condition. There seems to be very little advice about for CF carriers who also suffer symptoms of CF.

I have had a blood test for CF following an earlier diagnosis of mild brochiectasis following a CT scan. I have been given advice on carrying out postural drainage and chest physiotherapy and have an antibiotic permanently available to use immediately if I get a chest infection. I also was innoculated against pneumonia and receive an annual flu vaccination.

I have suffered with cough, sticky catarrh and been prone to bronchitis all of my life - at least for as long as I can remember - and had pneumonia at age 21. About ten years ago I was also diagnosed with IBS.

The chest consultant told me I was a heterozygote and had the recessive CF gene and was therefore a carrier - I am puzzled as to why I have so many CF symptoms when I understood carriers showed no symptoms.

I also have CBAVD (congenital bilateral absence of the vas deferens), which I understand is also a CF-related symptom.

Answers:
In the past people known to be carriers of a CF gene have usually been regarded as perfectly healthy - in fact, the high frequency of being a CF carrier (1 in 25 of the population) has suggested that over the centuries there may have been some advantage in being a carrier. A greater ability to withstand cholera with less salt and fluid passing into the bowel was one suggestion. On the other hand, more recently, the number of CF carriers amongst people with pancreatitis and chronic sinusitis has been reported to be more than expected, as it has among men investigated for infertility and found to have CBAVD.

So, an increasing number of people are being recognised who have a late onset of milder than average CF. They usually have one of the more frequent CF genes on one chromosome (for example DF508) and a rare "mild" CF gene on the other chromosome which may not be detected if limited blood screening for CF mutations is performed. Such people would have variable symptoms of CF such as repeated chest infections and the men may have BCAVD.

The important practical point in management for such people, who may therefore have mild atypical CF, is to be sure they receive vigorous treatment for their chest infections, as they would if firmly diagnosed with Cystic Fibrosis. Also, it is important that there is referral to a geneticist who will be familiar with the likely possibilities as to there being an additional mutation resulting in Cystic Fibrosis.

It is good practice to offer genetic testing to any blood relatives of a person known to be a CF carrier and this is an entitlement through the NHS.

Questions: Stem cell banking
My CF consultant has suggested that I contact you to see if you know anything about Stem Cell Banking for my baby due in January. Do you know of any companies that specialise in this and have a good reputation? Also, do you think that banking stem cells is worthwhile or is it just clutching at straws? If there is the slightest possibility they could be used to help CF in the future I would like to try. Finally, if the new baby is affected with CF then will it be worth taking stem cells or is it (or could it be in the future) only beneficial to people without CF. We are hoping that the new baby will be a match to her sister who has CF.

I realise that this is a very hard question and we can't tell what will happen with regards to research into helping people with CF but would just like someone else's opinion!

Answer:
At present the CF Trust does not have an official line in the use of stem cells but is funding research into the use of stem cells and would of course welcome any favourable developments in this area which could benefit people with Cystic Fibrosis.

We have been asked about storing cord blood on a number of occasions and recently we consulted an expert in the field who is currently doing research in this area - incidentally funded by the CF Trust. He replied as follows:

"Stem cells are cells which are found abundantly in the umbilical cord and in the bone marrow. These cells have the ability to change into other specialised cells such as white cells, to fight infection and red cells to carry oxygen around the body. There has been recent interest in the ability of these cells to repair damaged organs such as the brain, heart and lung.
Therefore we are carrying out research to see if there is any possibility that they may be useful in CF. However, this is at an extremely early stage and, if successful, it is likely that therapy will only be available many years from now. To date, successful stem cell therapy has been carried out in blood diseases such as leukaemia."

The storage of stem cells is not routinely available on the NHS. There are companies who will do this privately - one such company is Future Health Care which is approved by the Department of Health (www.futurehealthcare.co.uk). This is a useful site which we have checked again before replying to your query and it contains a great deal of up to date information on stem cells including storage, costs, collection etc.

Our expert felt that if one could afford to store the cord blood it would be worth doing as it is without risk and could be very useful in the future. We agree with him.

Question: Acapella
My 12 year old daughter has been given the Acapella for both sessions of physio, in place of percussion and active cycle of breathing. Although this gives her more independence I am concerned that it is not as efficient as the traditional methods that have kept her well. How concerned should I be and should it only be used in sleepovers, holidays etc?

Answer:
The Acapella is a small, handheld device. Breathing out through the Acapella creates small oscillations or "vibrations" which are transmitted to the airways. These oscillations help to loosen and mobilise mucus in the airways. Following a period of breathing through the Acapella, huffing and coughing are used to expectorate (expel) the mucus.
Although there are no formal studies which have specifically looked at the use of Acapella in children with CF, oscillatory PEP devices (such as the Flutter, RC-Cornet and Acapella) have been shown to be as effective as other airway clearance techniques in Cystic Fibrosis. As with all airway clearance techniques, individuals respond in different ways and some techniques suit some people more than others. The physiotherapist will have taken several factors in to consideration when choosing the Acapella for your daughter and I am sure will carefully monitor her response to it. You will also, after a few weeks of her using it, have some idea of how effective you think it is. If your daughter is well, her lung function remains stable and if she seems to clear as much with the Acapella as with her previous physiotherapy, then I think you can be confident that it is just as effective a technique.
It is important that as children approach their teenage years, they take some responsibility for their treatment and are given the opportunity to be a little more independent. This is a difficult transition and both yourself, your daughter and the CF team will work together to ensure this happens without any detriment to your daughter's clinical wellbeing.

Question: Port-a-cath and contact sports
My son is 11 years old and has Cystic Fibrosis. He is very active and plays rugby for school and a local club. We have just had to finish a course of home IVs after only nine days, because he had problems with long lines access and then several canulaes were used, some only lasting a day.

Consequently he suffered a lot of pain including bruised and sore arms, and we have been advised that a port-a-cath might be a good idea, but fear that having a port-a-cath would mean the end of his rugby, which he enjoys so much and which is a big part of his life, as well as in important way for him to keep fit.

His previous IVs were six months ago and before that a year - is there any way a port-a-cath could be used and still allow him to play rugby?

Answer:
We have discussed this with a very important CF physician who says that he would not advise a port-a-cath for anyone playing a contact sport such as rugby. If it were damaged, there could be quite serious complications such as bleeding and displacement of the device.

It does not seem as though your son is requiring very frequent IVs at present and as the rugby is very important to him and will obviously be very beneficial for his CF, it might be best to leave the decision for the time being. You should however discuss the pros and cons with your son's paediatrician who will know all of the details of his case.

Question: Alternative therapies
Is there any evidence that young children with CF would benefit from something like Milk Thistle, which is said to be good for the liver. Could this offset the effect of constantly being on high doses of antibiotics.

We also give our child probiotics, but again are wondering whether there is any evidence of benefits for these.

Answer:
Milk Thistle has been used as a cell protective agent and there are references to its use in liver disease and various forms of cancer therapy on the Medline database, however none linking it to Cystic Fibrosis.

It is suggested that it is non toxic (described as 'relatively safe') but the mechanism of action is not understood. There are studies in progress into the use of Milk Thistle in infection and cancer.

My own view would be not to use it at present as its benefit - if any - in people with CF, can only be very speculative, and there may be unwanted drug interactions with current treatments.

Probiotics are a different matter. There is considerable interest in their possible role in CF as a number of studies have shown that abnormal bacterial infection of the small intestine can occur in a significant proportion of people with CF and this can interfere with absorption. There was recent revival in this problem at the Baltimore conference last year. So although there are no trials of efficacy it would seem quite a reasonable move to use regular probiotics in CF - unlikely to do harm and quite possibly would do good.

I have an open mind on alternative medicine in general and I know that one respected paediatrician is currently doing a trial of garlic in CF. However I do have very strong views on unsubstantiated claims of benefit in a condition such as CF, claims which unfortunately are made by some of the firms producing these products.

Question: Menstruation
Could you tell me whether the menstrual cycle can be affected by Cystic Fibrosis?

Answer:
The normal menstrual cycle is to be expected in girls with Cystic Fibrosis who have a normal weight and good lung function. In a few very sick young women, periods may never start, but a delay would not be considered unusual until after the age of 16.

Irregular or missed periods can occur when a women is ill, especially if she loses weight. They may start when weight is regained, or may stop altogether. When a woman who has had periods misses three or more together, further investigation may be needed. This is not serious in itself, however, but it is a way for the body to conserve more energy, for example to fight infection.

Question: Malabsorption in toddler
My 18 month old child has CF and has been having a lot of problems with her pancreas.

She weighs approximately 11kg and is taking 18 Creon 10,000 per day, but can still have up to seven dirty nappies each day.

At her last CF Clinic a stool test was recommended to see how much fat was in her stools. The results have come back at 11g of fat in her stools, which I have been told is quite high. I'm still waiting for medical advice on what to do next.

Is there anything I can do to help her digestion?

Answer:
The substantial dose of enzymes that your child is taking should be controlling the malabsorption, but obviously is not, as a faecal fat of 11g per day certainly is quite high.

This does occur however, and there are a number of things that can be done to improve matters (which have been described in a paper recently sent to all directors of CF Centres in the UK). Although many doctors will already know the way to deal with such a problem (persisting malabsorption despite large doses of pancreatic enzyme), some perhaps with fewer patients may not.

Briefly, the usual approach recommended is to review the enzyme dose, the method of giving the enzymes, and the timing in relation to meals - children differ in the best time to take enzymes and it is worth trying different timings.

Review by an experienced dietitian is very important - presumably this has been done already.

Also, you should try and tailor the enzyme dose more closely to the fat content of the food, e.g., give larger doses with foods known to be fatty and smaller doses with less fatty foods, rather than the same doses at every meal (your dietitian will help with this).

It is worth checking the expiry date on the enzymes as their activity does decrease over time.

Sometimes, changing to a different enzyme preparation will improve matters considerably, as they do have slightly different ways of releasing their active enzymes in the bowel.

Sometimes, reducing the acid in the stomach by giving a medicine to do this (called proton pump inhibitors) will improve matters.

The important thing is that your daughter is thriving - 11kg is absolutely average for her age. However perhaps some adjustments to her treatment would improve the state of her bowels which are obviously and understandably still a major problem to her and you.