QUESTIONS AND ANSWERS

Click on the plus to read the answers to the questions

Borderline CF

Is it possible to be a “borderline CF” and what does that mean?
There is no such thing as borderline Cystic Fibrosis. One either has Cystic Fibrosis or not. However, it is well documented that CF may present in milder forms, often misdiagnosed and thought to be just mild bronchitis. These patients often have the unusual mutations and not the common Delta F508. Usually these milder forms of CF patients do not have enzyme deficiency and do not therefore need to take Creon.

What may be borderline in these CF patients is their sweat test results as their chloride channels in their cell walls are working to a certain degree so as to lessen the symptoms.

Should you require further information on these atypical forms of CF I suggest you Google some of the reputable CF sites.

Bronchodilators and Saline solutions

Is it safe to use the bronchodilator, Atrovent and a hypertonic saline solution (5%) in a mixture in my nebulizer?
It is safe to use Atrovent and Hypertonic Saline but ideally we suggest bronchodilators first so that the hypertonic saline can penetrate further into the smaller airways and do its job to loosen thick sputum.

However, there may be time constraints for you which makes it impractical to do 2 separate nebulisations.

CF reduces the effects of Cholera?

Does CF reduce the effects of Cholera?
An interesting and topical question in view of the recent outbreak of Cholera in northern South Africa.

The basic defect in Cystic Fibrosis is an absent or abnormal CFTR channel which allows the movement of chloride and water through cell walls. The vibrio Cholera organism secretes a toxin that binds the CFTR channel causing it to turn on and secrete large amounts of chloride followed by water giving rise to the classical watery diarrhoea.

The Cholera toxin cannot bind to an absent or fault CFTR channel and this is how both the carrier and the person with CF has an advantage. The heterozygote (carrier) will therefore have a milder form of Cholera as will the patient with Cystic Fibrosis. This has apparently been proven in CF mice models.

A word of caution is one should still take precautions, however, if in a Cholera area!

Cigarette smoke and CF

What are the effects of secondary (cigarette) smoking on a child with cystic fibrosis and is it any different to the effects on a normal (non-CF) child?
Secondary cigarette smoking is deleterious to all lungs and in particular to patients with underlying chronic lung disease such as asthma or cystic fibrosis. The cigarette smoke irritates the airways of the lungs and exacerbates the underlying inflammation in the walls of the airways giving rise to tight airways and more mucous production.

We instruct our families with a CF sufferer in their homes to avoid exposing the patient to cigarette smoke wherever possible.

Daily food intake for adult with CF

What kind of food should an adult CF patient be eating?
All CF patients require balanced meals, which include all macronutrients such as carbohydrates, proteins and fat. They should be having 3 meals and healthy snacks in between. The quantities of food portions should be equivalent to that of any other individual, except if they show signs of malnutrition.

The type of foods is no different to other individuals. All types of foods to be encouraged. Supplementation with a high energy nutritional supplement drink is recommended for those with malnutrition and poor food intake. Options are: Nutren Activ, Ensure, Fresubin energy drink, etc. These drinks should be taken with the guidance of a qualified dietician.

An example of a balanced healthy eating daily plan:

Breakfast:

  • Cereal, high fibre with cup full cream milk, fruit OR
  • Cup full cream yoghurt and muesli, fruit OR
  • Toast with avocado and bacon, fruit
  • And cup of coffee/juice

Snack:

  • Snack bar/ fruit
  • savoury biscuit with cottage cheese

Lunch:

  • Sandwich [brown or whole wheat bread] with protein based topping [e.g. tuna/chicken/peanut butter/beans] with lettuce/cucumber/tomato and margarine
  • Fruit

Snack:

  • Popcorn/ cup full cream yoghurt +fruit/ bran muffin /nuts

Supper:
Cooked meal for example:

  • Protein item: chicken/ meat/ fish
  • Carbohydrate: rice/potato/pasta
  • Vegetable: 2 green and yellow/orange/red

Late night snack:

  • Glass of Milo/ Hot chocolate mixed with full cream milk
E-Flow nebulizer

Do you have any idea where I can get an e-flow nebulizer in South Africa? And the cost?
The physio recommended we look at one. I know they are very expensive.
You can get them from Clicks Direct Medicines and they are about R 15000.00. (Some medical aids do fund e-flow nebulisers with justification from a specialist doctor).

Clicks Direct Medicines can be contacted on www.directmedicines.co.za or as per info below.

Call Centre: 0861 444 405
Switchboard: 011 997 3000
Operating Hours: Monday to Friday – 08h00 – 16h30
Physical Address: 36 Ayrshire Road, Longmeadow, Business Park, Ext 1, Modderfontein, 1645
Postal Address: P.O. Box 751902, Gardenview, 2047

Is CF Curable?

Is CF curable?
So far, medical science has not found a cure for CF but doctors all over the world are working very hard to find a cure. Check out the About Cystic Fibrosis page to see what treatment is currently available for Cystic Fibrosis.

Lung transplantation

Lung transplantation – what to expect?
Please look under our “About CF” page under “Transplantation”. It is recommended that you also need to discuss transplantation with your CF specialist and look at the options for you individually.

Maintenance IV Treatment

I wanted to know what the thinking was on maintenance IV and how many times per year would be beneficial? My daughter is 8 years old and has maintenance IV 3 times a year. She seems to benefit from it, because for the last 3 years we have had no hospital admissions. I am interested to hear what the other regions do.

Thank you for your question about maintenance IV antibiotic treatment. The use of frequent regular IV antibiotic courses was first introduced by the Danish CF clinics many years ago and resulted in their overall survival rates improving dramatically. There is no doubt that there is benefit from regular IV antibiotic therapy in patients who are permanently infected with Pseudomonas and in particular those with bronchiectasis as seen on CT scan of the lungs.

The debate is really centered around how frequently you give these courses. Some people will suggest every 3 – 4 months, others less frequently and I would say it depends really on each individual patient as no patient is alike. Some clinics, especially with the introduction of long term weekly use of Zithromax and the more regular use of inhaled antibiotics, have reduced the frequency of IV courses.

You may well find that various clinics differ slightly on how they enforce regular frequent IV antibiotic therapy but certainly this depends on the individual patient and how they respond and whether the inhaled antibiotics given during alternate months or on an ongoing basis hold their pulmonary infection in check.

Maternal Screening

My wife and I are from the US and have just discovered that she is pregnant. We are interested in pre-natal maternal screening for CF carrier status. Is that test offered in South Africa?

Screening for Cystic Fibrosis carrier status is available in South Africa. The 30 most common CF mutations are screened for but random prenatal maternal screening is not offered as this would flood the services.

However, should there be a family history of Cystic Fibrosis this would be done. Newborn screening is available via a simple heelprick blood spot test. This is available as part of the screening for other inherited disorders, and you should notify the Paediatrician who is caring for your newborn baby.

N-Acetyl Cysteine (NAC)

NAC (with trade name ACC200 locally) has been used for years as a mucolytic in both aerosol and tablet form, and is used widely in USA as part of the CF treatment regime. Apart from the mucolytic role, a paper has also been produced indicating modulation of inflammation in CF (Stanford University School of Medicine). Locally a number of CF patients are using NAC apparently with noticeable benefits.
Should NAC be promoted more for the treatment of CF, even if it is only by way of informing CF patients about it?

Thank you for this interesting question. I had to consult Sandoz for further information. As far as we are aware there has only been one publication to date with the results of a small study involving 18 CF adult patients.

In the short term it appears that high dose oral NAC blocks the abnormal migration of neutrophils (inflammation cells) into the airways. However, there was no change in lung function and encouragingly there were no concerns about safety or adverse side effects.

However, the long terms safety and therapeutic effects as well as the mode of action of high dose oral NAC treatment remains to be tested. In the meantime it is important that patients do not use the drug in uncontrolled situations until long terms studies are complete.

Only with carefully designed and controlled clinical studies may oral NAC eventually prove a useful preventative therapy for airway inflammation in CF.

Nasal Polyps

My daughter (24) has quite a few nasal polyps & the ENT has suggested surgery. She is otherwise well, and mainly suffers from allergies & asthma.

Is the surgery usually effective? Also she has no active infections at the moment but her clinic insist that she should have intense Antibiotic therapy before the surgery is this necessary?

Nasal polyposis in Cystic Fibrosis patients is very common and occurs in the majority of adults with Cystic Fibrosis. We only suggest surgery if the patient is very symptomatic e.g. severe nasal blockage and headaches or recurrent acute sinusitis.

The surgery is often effective but for a short while as unfortunately the sinus lining and polyps return. The concern about regular ENT surgery is that this often requires exposure to a general anaesthetic which may be detrimental to the chest condition.

If your daughter is allergic then the nasal allergy should be treated with nasal cortisone sprays and anti-allergy treatments such as Singulair. Intensive antibiotic therapy with physiotherapy is usually indicated to optimally assist a person with Cystic Fibrosis going for surgery as there may be a flare-up of the lung infection postoperatively.

Pulmozyme

My 10 year old daughter has just had Pulmozyme added to her list of meds. In the morning, she does her inhaler, followed by physio and then is nebulised with Colomycin. This is done in the late afternoon as well. She has been told to do the Pulmozyme at midday, but due to school commitments and extramurals, this is not always possible. It would be great if we could fit it in to either our morning or evening regime.

Any suggestions on how to fit it in and at what point. Does it follow physio, inhalers etc., or can it be done on its own?

I realize that your daughter has a very time consuming treatment schedule in the morning and evening so the addition of Pulmozyme does make the whole process longer. The role of Pulmozyme is to break down thick secretions so it is best to do it prior to physiotherapy and the inhalation of antibiotics should be performed last when the airways are as clear as possible.

I would not suggest Pulmozyme in the evening as she may then have to deal with increased fluid secretions after going to sleep at night and this may keep her up coughing. I think the best would be for her to use the Pulmozyme first thing in the morning followed by physio and then the antibiotic nebulization.

Salt therapy

I have recently heard of a salt cave opening in Randburg area. I have a 13 year old cystic child. Would it be beneficial for her to sit in the cave? The visits are 45 minutes to an hour and the testimonials sound good. She is battling to get rid of a persistent cough. What is the feeling regarding this therapy?

A session of this therapy may be beneficial at the time of therapy but for a better long term result daily nebulising with Hypertonic Saline is recommended. It assists with loosening of sticky secretions in the lungs which causes the persistent cough.

Sweat conductivity test

My 10 year old daughter is being treated for reflux, she had a lung function test which showed a low PEF level and she also has had problems with constipation in the past. I had a sweat conductivity test done and her result was 57. The peadiatrician is adamant that she does not have enough mucous and coughing to have CF but I am still concerned.

Should I insist on a sweat chloride test and would I need a referral from the pediatrician for the test?

There is unfortunately a crossover of symptoms between milder forms of Cystic Fibrosis and Asthma, as well as Reflux Related Respiratory Disease.

Your daughter’s sweat conductivity test result fell into the uncertain (or grey) range with normal values being < 50 and definite positive values >70. Under these circumstances I would definitely suggest doing a formal sweat test and you would need a referral from either a paediatrician, General Practitioner or member of a CF clinic (details on the SACFA website for your nearest clinic).

Vitamins (A, B, C, E and K)

What is the recommended daily allowance for a child with Cystic Fibrosis?

Fat soluble vitamins

A large percentage of individuals diagnosed with Cystic Fibrosis will have pancreatic insufficiency, i.e the inability to enzymatically breakdown their food particles, particularly fat. These patients are likely to have fat malabsorption and therefore require pancreatic enzyme replacement therapy, like Creon to digest their food particles.

All Cystic Fibrosis patients with pancreatic insufficiency requires fat soluble vitamin supplementation. These fat soluble vitamins are Vitamin A, D, E and K. It is recommended that annual blood levels of these fat soluble vitamins be measured to ensure that any deficiencies be corrected.

The following levels of Fat soluble vitamin supplementation recommended by the South African CF guidelines are:

  • vitamin A 4000 – 10000 units
  • vitamin D 400 – 800 units, and
  • vitamin E 100 – 200 IU.

These are considerably greater than the usual daily recommended intake. These amounts need to be prescribed by your medical doctor as they are depended on the age of your child. Levels will be adjusted as well with annual bloods if any deficiencies are detected.

Each fat soluble vitamin has vital functions in the metabolic processes of your body. They are:

  1. Vitamin A
    Vitamin A plays an essential role in the process of vision, growth and immune system. Deficiency can result in night blindness in older individuals. They are found in food like, liver, egg yolk, chicken and milk. The right dose needs to be administered as too much can be problematic.
  2. Vitamin D
    Vitamin D main function is in bone development therefore deficiency may result in rickets and osteomalacia or osteoporosis in older individuals. We receive good amounts of Vitamin D just from sunlight alone.
  3. Vitamin E
    Vitamin E serves as an important antioxidant, i.e it helps the body clean up any damaging effects of an infection. It is also involved in neurological processes. Vitamin E can be found in foods such as sunflower oil and wheatgerm.
  4. Vitamin K
    Vitamin K is involved in blood clotting processes and bone development. Supplementation is not given routinely only if signs of deficiency is present. It is partly made by our microflora in our large intestines.