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

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.