Health Column

The questions and answers in this column were taken from past issues of the SVB newsletter. All the information in this section is periodically checked and brought up to date by Dr. Michel Ruel of the Centre hospitalier universitaire de Québec (CHUQ), CHUL Pavilion.

The importance of this column lies in the fact that it provides answers to questions most frequently asked by CF patients to physicians who are specialized in cystic fibrosis. By clicking on a topic, you will access the questions and answers related to the chosen theme.

SYMPTOMS

Acute Sinusitis
Anemia and cystic fibrosis
Arteriosclerosis and heart disease
Bad breath
Clubbing
CO2 and Oxygen Flow
Delayed growth
Diabetes and cystic fibrosis
Enlarged heart and cystic fibrosis
Enlarged spleen
Fever
Gastroesophageal reflux
Glucose intolerance
Laryngitis
Pancreatic cystic fibrosis
Pneumothorax (respiratory system)
Thirst

TREATMENT

Antibiotics

Antibiotics: Vitamins
Antibiotics: Milk and Alcohol
Antibiotics and Length of Treatment
Cipro® and Fitness Training
Photosensitivity and Intravenous Antibiotics
Tobi®

Catheters long catheter
Catheters P.A.S. Port and Port-A-Cath
Corticosteroids (cortisone): Action and Side Effects
Cortisol
Cough Syrup
Cyclosporine: Action and Side Effects
Desensitization
Ibuprofen
Ibuprofen and Scarring
Methadone
Monoclonal Antibodies
Nasal polyps and sense of smell
Omega-3
Oxygen Therapy
Pancreatic enzymes
Super anti-inflammatory drugs (Vioxx^TM, Celebrex^TM and Bextra^TM)
Tamiflu®
Ventolin® Storage
Vitamin E and Cystic Fibrosis
Weight and Force Feeding

TRANSPLANTATION

Pregnancy and Lung Transplantation
Transplantation: Pancreatic Transplantation
Transplantation and Kidney Problems

SEXUALITY

Exercise
Semen
Vaginitis
Viagra^TM

MOTHERHOOD, FATHERHOOD

Male Infertility
Mild Form of CF and Male Fertility

COMMUNITY LIFE

Contamination Risks

GENERAL

Acne and Accutane^TM
Anti-Viral Vaccines
Arterial Blood Gas
Cystic Fibrosis and Blood Donations
Candida albicans
Childhood diseases (smallpox, measles, German measles, mumps, etc.)
Clostridium difficile
Donor Virus
Ecstasy
Flu Vaccine
Gene combination and life expectancy
Hair removal
Indoor Plants
MRSA
Multiresistant Pseudomonas
Pneumococcal Vaccination
Research Phases
Sports to Avoid
Terminology
Vaccines and travel

Anti-Viral vaccines

Last year, I had a lung transplant. Since I am in great shape, I want to fulfil a life long dream: a trip to Central Africa. Would anti-viral vaccines be risky for me? If so, what should I do to guard against infections?

You did not choose the safest destination for a lung transplant recipient, even a healthy one. The risk of infection in Central Africa is quite high, unless you stay in the bigger hotels. Moreover, the quality of health care in theses countries is usually far below North American standards. This being said, I contacted an infectious disease specialist to answer your question. He believes that anti-viral vaccines pose a theoretical risk: they stimulate the immune system, which could, in theory, result in rejection of the transplanted lungs. Given this possibility, it would be better to abstain. However, you may use other preventive measures: for hepatitis A, instead of a vaccine, you can receive gamma globulin injections (antibodies), which provide good temporary protection. Malaria and intestinal infections occur frequently in these countries. To guard against the malaria, you need to apply bug repellent and take oral anti-malarial medication before, during and after your trip. To prevent intestinal infections that cause fever and diarrhea, take the usual precautions (drink only bottled water, peel fruits and vegetables before eating them) and use ciprofloxacin if you do contract an infection. So before travelling to any ”exotic” country, it is important to consult someone who works in a specialized travellers’ clinic in order to get the best preventive measures adapted to your destination and state of health.

HEALTH COLUMN
SVB/ October 1999, No 24, pages 26-27

Flu Vaccine

Every fall, my physician insists on the importance of my receiving the flu vaccine. I am not convinced that this is necessary. Is this vaccine really effective? Isn’t it dangerous to be vaccinated year after year? How do you explain the flu-like side effects that some people experience?

Before answering this question and the following one, I would like to provide a few details about the flu. The “real flu” is caused by influenza viruses. There are only a few dozen of these viruses, but they cause respiratory infections that are usually more serious than the other respiratory viruses at the origin of colds, which are far more numerous. While the flu is accompanied by high fever, headache and muscular pain in addition to respiratory symptoms (sore throat, nasal congestion, cough), colds consist of respiratory symptoms without the other symptoms, except perhaps a slight fever. Complications from viral respiratory infections (earache, sinusitis, bronchitis and pneumonia, all caused by bacteria) are more frequent and serious with the flu than with a cold. These complications are also more prevalent in older people and in those with chronic diseases such as cystic fibrosis. This is why it is important for these two groups to receive protection. Fortunately, the influenza (flu) vaccine has been around for many years now. However, it offers no protection against the numerous cold viruses. The vaccine is 80 percent effective in young adults, but it must be administered annually because it does not provide long-term protection and is directed against the viruses that are prevalent in the world at a given time. When the virus mutates, the vaccine must be modified. Besides slight sensitivity at the site of the injection, some people experience light flu symptoms: low-grade fever with muscular pain, sometimes accompanied by minor respiratory symptoms. These side effects usually last 24 to 48 hours at the most. They are caused by the mobilization of the immune system (white blood cells and antibodies), which is responding to the vaccine.

HEALTH COLUMN
SVB/ November 2000, No 25, page 38

Ventolin® : Storing Ventolin

Is it true that it is best to keep Ventolin® for aerosol therapy in the refrigerator? If so, could you explain why?

One thing must be made clear concerning the storage of Ventolin® for aerosol therapy: once opened, the single-dose nebules must be used and cannot be stored.

The multidose bottles contain a preservative, however. Unopened bottles must be stored between 2ºC and 25ºC away from light, up until the expiration date on the bottle.

Once the multidose container is opened, storage depends on the stability and the sterility of the product. Regarding product stability, we can guarantee use for 30 days after it has been opened (it must be kept away from light between uses). Product sterility (uncontaminated by germs) depends mostly on the handling technique, i.e. the person who prepares the product and the environment in which it is prepared. It is important to remove the solution in the most sterile way possible, close the bottle as soon as the solution has been removed and refrain from opening it needlessly. Despite these precautions, contamination may still occur. Check the appearance of the solution before use: if there are any particles, or if the solution seems altered or discoloured, or if you have any doubt whatsoever about its sterility, throw it out. In general, storing an opened bottle in the refrigerator will reduce the proliferation of germs in a slightly contaminated solution.

All in all, we recommend that unopened bottles be stored at room temperature and away from light. Once opened, they can be kept for a maximum of 30 days, either at room temperature or in the refrigerator.

Health Column
SVB/ Winter 1993, No 16, pages 25-26

Ventolin® : Ventolin® and Exercise

Right before sexual intercourse, I take Ventolin® to clear my bronchi and avoid coughing. But I read that Ventolin® may have harmful effects, including heart complications. As it is, Ventolin® makes me shake. Are there any risks to my using Ventolin® before sexual intercourse?

Ventolin® is prescribed for CF patients because over half of them suffer from bronchial hyperactivity, the severity of which may vary over time. Bronchial hyperactivity, also found in all asthmatics, can be defined as hypersensitivity of the bronchi, which causes them to overreact to various stimuli. One of these reactions is called a bronchospasm (a contraction of the muscular layer of the bronchus), which reduces the diameter of the bronchus. Ventolin® is a bronchodilator that prevents contraction and produces dilation of the bronchial muscle, thus keeping the bronchus ”open.” Other stimuli that can cause excessive reactions of the bronchi include allergens, general irritants (strong odours, cigarette smoke), respiratory infections, as well as exercise. Regarding the latter, we know that Ventolin® can prevent bronchospasms due to physical exertion when taken 15 to 20 minutes before exercise.

In view of these facts, individuals with CF who experience bronchial hyperactivity following physical exertion could benefit from taking Ventolin® before physical activities (amorous or athletic)! The metered-dose inhaler would appear to be more appropriate than the nebulizer. The doses can be repeated, but must be taken about six hours apart. Devices used with powdered Ventolin® (Rotacaps-Ventodisk) are just as effective and require less co-ordination. Similar drugs such as Berotec®, Bricanyl® and ProAir®, can be used as well.

The side effects of Ventolin® (and similar drugs) are generally limited to shaking of the extremities and tachycardia (accelerated heart rate). These side effects are benign and can be eliminated by decreasing the dose and by leaving adequate time between doses.

Have a good ”workout”!

HEALTH COLUMN
SVB/ Summer 1993, No 17, pages 30-31

Research Phases

In cystic fibrosis research, people often mention Phases I, II, III and IV. What do these phases refer to? Should they be taken into consideration by candidates applying to take part in such research?

In clinical research, the studies are classified under Phases I, II, III, and IV. Phase I studies evaluate the safety of a drug. They are conducted on a small number of healthy volunteers, and are used to evaluate what happens to the drug in the human body The side effects are, therefore, closely examined.

Phase II studies involve a larger number of people and provide information on the way a drug functions, as well as its benefits and side effects. These are random, double-blind studies, which means that one group is given the new drug and the other group is given the standard drug or a placebo (sugar pills). Neither the patients nor the researchers know which group has been given the new drug until the study is completed.

Phase III studies are somewhat similar to Phase II studies, except that they include a much greater number of people (thousands) and can last for years. These studies give researchers a good idea of the effectiveness, benefits and side effects of a new drug. These are also usually random, double-blind studies. Following successful completion of a Phase III study, developers can file an application for marketing approval.

Once a new drug receives marketing approval, Phase IV studies are often conducted. The purpose of these studies is to evaluate the drug's long-term benefit and to compare the cost- benefit ratio with that of the traditional treatment.

HEALTH COLUMN
SVB/ 2001, No 26, pages 42-43

Pregnancy and Transplantation

1 - I am waiting for a lung transplantation. After the transplant, I would like to raise a child. My question is threefold.

1.1 Is it true that the medication I will have to take after the operation might be harmful to the foetus?

There is a high risk that the immunosuppressant drugs would have serious side effects on the foetus, ova and spermatozoa, so pregnancy is not advised when taking post-transplantation drugs regularly. Recently, however, a woman with cystic fibrosis who had had a lung transplant gave birth to a child who seems healthy, at least for the time being.

1.2 Does Quebec have a surrogate mother program? What do I have to do?

There are no surrogate mother services available in Quebec at the present time.

1.3 If the medication is dangerous for the foetus, could it also affect the ova that I will produce? In the meantime, should I have some ova withdrawn and frozen? I hesitate, because it seems that high doses of hormones are injected before the ova are withdrawn. I worry that the hormones would have a negative effect on my health before the transplantation.

Unlike embryos, (ova fertilized by spermatozoa) ova cannot be frozen for later use. My colleague would not consider injecting high doses of hormones in order to withdraw ova. As it is, he does this only for women who are perfectly healthy.

Vaginitis

Are women who have cystic fibrosis more prone to vaginitis? If so, could you explain why? Is there a way to avoid this kind of infection? Does the pill have an effect one way or the other?

Women who have cystic fibrosis are in fact more prone to vaginitis, particularly to vaginal infections caused by the Candida albicans fungus. The vagina normally has good bacteria that play a useful role. CF patients must frequently take antibiotics, either orally or intravenously. These antibiotics can kill vaginal bacteria, thus promoting the growth of fungi. Symptoms include itching and thicker, more abundant vaginal discharge. Infection can also spread to the vulva, causing redness and itching in that area.

Vulnerability to this type of infection varies from one person to the next, so we do not usually suggest preventive treatment. There are different kinds of topical treatments, however, such as creams and vaginal ovules that are very effective against these fungal infections. There have been some reports of increased incidences of fungal vaginitis among users of oral contraceptives, but generally, the problem is more serious with antibiotics.

HEALTH COLUMN
SVB/ January 1992, No 14, page 30

Male Infertility

I am 23 years old. My wife and I would like to know whether I am sterile. I have heard that I could have a spermogram done. Can you explain what a spermogram entails? Is the procedure 100% reliable? I would prefer that the medical team treating me not know the results. What do you suggest?

A spermogram is simply the analysis of semen obtained by masturbation after two or three days of abstinence. The liquid collected in a tube must be kept at body temperature and brought to the lab within the hour. The semen is studied under a microscope; the spermatozoa are counted, and their shape and mobility are checked. More than 95% of men with cystic fibrosis are sterile. For these men, the problem lies in the ducts that carry spermatozoa from the testicles (where they are produced) to the urethra: the ducts have either degenerated or are absent. It is a transport problem. In these cases, the spermogram will show a total absence of spermatozoa. When such a report is received, one can be 100% sure of the infertility diagnosis. As for confidentiality, all doctors can prescribe a spermogram, but this test is not available in all hospitals. I believe it would be helpful, however, that the physician treating you at the CF clinic be informed of the results!

HEALTH COLUMN
SVB/ June 1992, No 14, page 30

Sports to Avoid

I am 22 years old and I have cystic fibrosis. I play many sports such as softball, volleyball and badminton. For some time now, I have been dying to try scuba diving. Since I am only slightly affected, do you think it would be dangerous for me to practise this sport? What about skydiving?

To answer our sports buff, let me say that scuba diving is not advisable for a person with cystic fibrosis. The lungs of most CF adolescents and adults are at least slightly affected, and there is therefore the possibility of emphysematous bullae, which may not be visible in a lung X-ray. Pressure changes when diving might cause the bullae to burst, thereby causing a pneumothorax.

As for skydiving, atmospheric and oxygen pressures change only slightly at the altitudes the plane flies. Skydiving is therefore not discouraged for people whose lungs are only slightly affected, but their condition should be stable and they should ensure that there is no increase in infection. It is important to be re-examined by the attending physician before attempting this kind of activity.

HEALTH COLUMN
SVB/ Winter 1993, No 16, pages 25-26

Terminology

I am 33 years old and I have cystic fibrosis. I am an old hand at this disease; I know all of its forms and guises. Yet…The other evening to my great amazement, I realized that I did not understand the use of the term ”cystic fibrosis of pancreas” rather than ”mucoviscidosis” (In my opinion the latter term better describes the disease).

You are absolutely correct! The term ”cystic fibrosis of the pancreas” does not indicate the extent of the disease; it only describes the changes occurring in the pancreas. However, the term used today is simply ” cystic fibrosis,” which also encompasses the changes that occur in the lungs, including both fibrosis and the formation of cyst.

The European term ”mucoviscidosis” (viscous mucus) take into consideration the physiopathological aspects more than the descriptive aspects of the disease; but it does not include disorders of the sudoriparous glands (swat glands), which produce an abnormally saline sweat. The hyperviscosity of the mucus and the highly saline sweat result from the deficient transport of electrolytes (especially chloride), which is characteristic of the disease.

In conclusion, there is no perfect term, and mucoviscidosis could actually be the better term to describe this disease.

HEALTH COLUMN
SVB/ Winter 1996, No 20, page 24

Risk of Contamination

I’m getting ready to apply to CEGEP to study medical laboratory technology. My ambition is to work in a hospital laboratory. Since laboratory technicians have to handle bacteria, viruses and parasites, do you think that my choice is inappropriate considering the risks of contamination?

Far be it from me to discourage you from working as a technician in a hospital laboratory. Keep in mind that not all technicians handle germs, only those who work in microbiology. Technicians in biochemistry and hematology are more rarely exposed to germs. Even in microbiology, however, the risk of infection for a CF patient is hardly any greater than for anyone else. CF individuals’ immune systems (the body’s defence system against germs) are not deficient. I therefore have no objection to your working in a microbiology laboratory. In fact, my patients include a medical student and a graduate student who are both working on different research projects in microbiology. My only reservation (perhaps unfounded) is that you avoid working with strains of Burkholderia cepacia until the mode and risk of transmission of this bacteria are better understood.

HEALTH COLUMN
SVB/ Winter 1994, No 18, pages 27-28