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

Monoclonal Antibodies

What can you tell us about ”monoclonal antibodies”, a latest discovery in the fight against infectious diseases?

Antibodies are molecules produced by a type of white blood cell (plasma cell) to neutralize substances that are foreign to the body (germs or other substances).

The distinctive characteristic of monoclonal antibodies is that we have succeeded in producing a large number of a specific type of antibodies—i.e. those aimed against a specific element of a foreign substance—in the laboratory. Currently, monoclonal antibodies are especially used for diagnostic purposes in clinics and in research. As far as I know, the on-going projects using monoclonal antibodies here at the Centre hospitalier de l’Université Laval are for diagnostic ends.

In other laboratories, however, experiments are being conducted on the therapeutic possibilities of monoclonal antibodies. It would thus be possible to link an antibiotic to an antibody aimed at Pseudomonas, so that the antibiotic could reach the bacteria more directly and effectively. But as far as I know, there is currently no such treatment available in clinics.

HEALTH COLUMN
SVB/ June 1991, No 13, pages 32-33

Arterial Blood Gas

Now and then, my doctor asks that I have an arterial blood gas analysis. What exactly is this? Also, I would like to better understand what is meant by PCO₂.

To understand what arterial blood gases are, you must first know what an artery is. The circulatory system includes:

1) arteries that carry blood that has been freshly oxygenated by the lungs, from the heart to peripheral tissues;

2) capillaries, which are tiny blood vessels where gaseous and nutritive exchanges are made with the tissues;

3) veins that carry blood from the peripheral tissues back to the heart.

Most blood samples are taken from veins. Some are taken by pricking the fingertip, where capillary blood is obtained. When accurate information on lung function is needed, it is preferable and more precise to collect blood that has just come from the heart-lung area. A blood sample is usually taken by puncturing the superficial artery, which is the radial artery (located on the inside of the wrist at the base of the thumb). A local anesthetic may be necessary and pressure must be applied to the artery after the blood is collected to prevent bleeding, because blood pressure in this vessel is much higher than in a vein.

Blood acidity (Ph), oxygen or O₂ levels (PO₂) and carbon dioxide or CO₂ levels (PCO₂) are analysed in an arterial blood gas sample. We all know that oxygen (O₂) is the body’s ”fuel” and carbon dioxide (C0₂) is one of the body’s wastes. The lungs’ main function is to supply oxygen to the blood and eliminate carbon dioxide. Blood acidity (Ph) and CO₂ levels (PCO₂) are measured quite accurately with capillary gas (taken from the fingertip), but the oxygen level (PO₂) reading is not as reliable. Moreover, in cases of severe lung disease where gas readings are necessary to determine whether oxygen therapy is needed (and if so, how much), an arterial blood gas sample is required.

There is a device known as the oxymeter or saturometer that is now more readily available in clinics. A small clip attached to an optical system is placed on the fingertip; the oxygen level in the blood can be measured quite accurately without piercing the skin. Although arterial blood gas tests are not completely eliminated, this device can reduce them greatly in number!

N.B.: Normal PO₂ levels are between 80 and 100
Normal C0₂ levels are between 38 and 42.

HEALTH COLUMN
SVB/ June 1992, No 15, pages 29-30

Cystic Fibrosis and Blood Donations

I’m 22 years old and have cystic fibrosis. I consider myself to be in good shape. I plays sports regularly and rarely need to be hospitalized. Blood donor clinics are often held at the university I attend. Is it safe for me to give blood? If so, how many times a year?

Most people with cystic fibrosis shouldn’t give blood. In fact, the first requirement for giving blood is that the donor be in good health.

Blood donations must not cause problems in the receivers. Most people with cystic fibrosis who are old enough to give blood have chronic bronchial infections, and there is a chance that the bacteria could end up in their blood at some point. Blood containing bacteria could cause infections in the receivers. Moreover, cystic fibrosis patients often take antibiotics, which also end up in the blood. It is important to know that receivers could be allergic to one of these antibiotics and develop a reaction following a blood transfusion.

The majority of people with cystic fibrosis have a malabsorption problem due to pancreatic insufficiency, which is treated with enzymes and nutritional vitamin supplements. Although anemia is infrequent thanks to these treatments, people with cystic fibrosis often have low iron reserves. These shortages could be exacerbated through blood donation and cause real anemia.

In light of these explanations, the only people with cystic fibrosis who could qualify to give blood are those with a very minor form of the disease: they don’t need pancreatic enzymes or nutritional supplements; they rarely, if ever, suffer from bronchiectasis; they take antibiotics only when needed, and have not taken them 72 hours before donating.

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

Ibuprofen

I have heard of the many benefits of ibuprofen. Would you explain exactly what ibuprofen is (its properties)? Is it available? Who can benefit from it?

Ibuprofen is a “nonsteroidal anti-inflammatory” drug ( not derived from cortisone). It has anti-inflammatory properties, but it is not as potent as cortisone and its derivatives. It is mostly used to treat musculoskeletal diseases, especially rheumatoid arthritis. Ibuprofen is also an analgesic (painkiller) that can be used for headaches and is in fact currently sold as a mild over-the-counter drug under the name of ”Advil®”.

Knowing that bronchial inflammation is probably a major factor in pulmonary deterioration, we readily understand why there is an interest in anti-inflammatory drugs such as ibuprofen.

The only published clinical study to date comparing this drug to a placebo (a pill containing no active substance) appeared in March 1995. Eighty-five CF patients participated over a period of four years. They were afflicted with only mild lung problems and the drug dosages were adjusted according to blood levels. Results showed a less significant decrease in the respiratory capacity of patients taking ibuprofen. Only those under 13 years of age showed improvement, however, and hospitalization did not occur any less frequently. Ibuprofen had no more side effects than the placebo.

This was only a preliminary study and deserves to be confirmed by further studies, preferably involving a greater number of patients. At first glance, this drug seems less effective for older patients or for those who are more severely afflicted. These same patients might also experience more side effects in their digestive system (irritation, ulcers and gastrointestinal bleeding), which are quite frequently found in arthritic patients who regularly take drugs in this category.

To sum up, I believe it is too early to use this drug on a wider scale.

HEALTH COLUMN
SVB/ Winter 1996, No 20, pages 24-25

Oxygen Therapy

My doctor told me that I should start taking oxygen at home. How do doctors decide whether a patient needs oxygen? Is it really beneficial to take oxygen at night (as my doctor prescribed) if I don’t take it during the day? Isn’t there a risk that I will eventually become dependent on this type of therapy?

The criteria for prescribing home oxygen are based on studies made on patients suffering from severe chronic obstructive lung diseases (chronic bronchitis and emphysema usually related to smoking). The first study, made in 1970, showed that oxygen prolongs the life of people with respiratory problems. Further studies have shown that receiving oxygen is beneficial when taken for a stable pulmonary condition and from a partial pressure lower than 55 mm Hg. Oxygen is also beneficial for people who have a pressure of less than 60 mm Hg and who suffer from heart or kidney problems stemming from lack of oxygen. As for the duration of oxygen therapy, these studies have also shown that prescribing oxygen at night is only insufficient for people who match the criteria just mentioned. A continuous use of oxygen (24 hours a day) is ideal; however, in practice, we aim for a minimum of 18 hours a day. There is no physical or psychological dependency on oxygen as there is with alcohol or some medications. Nevertheless, once a cystic fibrosis patient has begun oxygen therapy, therapy is continued on a permanent basis or until the patient has had a successful lung transplant. Discontinuing oxygen therapy would lead to deterioration of the patient’s clinical condition and potential fatal heart problems.


HEALTH COLUMN
SVB/ Fall 1998, No 23, pages 28-30

Cough Syrup

When I go to bed at night, I often have terrible coughing fits that prevent me from sleeping. This is why I occasionally take cough syrup. As long as I don’t overdo it, do you think this is a good idea?

It is a well-known fact that coughing is a constant symptom in cystic fibrosis. The reflex is usually triggered by the presence of an abnormal quantity of secretions in the bronchial passages. In this context, it is a useful and necessary reflex for eliminating these secretions (and the bacteria they contain). Therefore, most of the time it is better not to suppress the coughing. However, coughing can also be triggered by other stimuli (foreign bodies, blood, inflammation) located in other parts of the body (throat, larynx). Consequently, sometimes it is appropriate to suppress coughing with medication, as in the case of an ”irritating” cough, which is stimulated by a process that causes more irritation than secretions do. It is also best to suppress a cough when there is bleeding in the bronchi (hemoptysis), so as not to aggravate the bleeding. Medication includes cough suppressants, which calm the coughing reflex. It consists mainly of narcotics such as codeine and dextromethorphan (DM), which can be taken in syrup or in tablet form. When coughing is caused by bronchial hyperreactivity (e.g. related to inflammation from asthma), anti-inflammatories, especially those in the corticosteroid class (such as Pulmicort^® and Flovent^®) are very useful for keeping it under control. Finally, if the cough is due to an episode of bronchial infection, it may be necessary to adjust the antibiotic treatment. If the cough is frequent and causes discomfort, it is important to discuss it with your physician in order to determine its exact cause and find the appropriate treatment.

HEALTH COLUMN
SVB/ November 2000, No 25, page 37

Tamiflu®

I heard about an anti-flu medication called Tamiflu®. How does it work? Is it as effective as they claim it is? And finally, is this product contraindicated for persons with cystic fibrosis?

On the subject of the battle against influenza, effective antivirals have recently been developed against these viruses (but not against cold viruses). Previously, there were products such as amantadine, which were somewhat effective against Influenza A viruses but not against Influenza B viruses. These products had fairly strong side effects. Two new medications have appeared on the market very recently: Tamiflu® (oseltamivir) and Relenza^MC (zanamivir). The first comes in tablet form and the second is an inhalation preparation. They both work the same way: they prevent the virus that has already infected a cell in the respiratory tract from spreading to other cells. They are both equally effective as long as treatment begins within 48 hours after the symptoms appear. They cut short the illness by one and a half to two days, reducing fever, coughing and muscular pain. Most importantly, they decrease the number of complications from additional bacterial infections. These medications must be taken twice a day for five days. Tamiflu® tablets may cause nausea and vomiting at the beginning of treatment, but in general, the medication is well tolerated. Relenza^MC, which is inhaled, causes very few side effects, but the patient has to master the inhalation technique (it is not very complicated). Neither of these medications is contraindicated in cystic fibrosis. They can be useful to those who, for whatever reason, were not vaccinated before the influenza season (December to March), and for the few individuals who contract the flu despite the vaccination.

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

Vitamin E and Cystic Fibrosis

I can't understand why my physician insists that I take vitamin E on a daily basis. Why is this vitamin so important for people with cystic fibrosis?

Vitamin E is liposoluble, meaning it can only be absorbed through fat in the intestines. In cystic fibrosis, the deficiency of pancreatic enzymes leads to malabsorption of dietary fat, among other things. Pancreatic enzymes in capsule form only partially compensate for this malabsorption, and the vitamin E naturally found in food is insufficient. To achieve normal blood levels, it is necessary to add vitamin E supplements to your diet.

Vitamin E has many qualities, including antioxidant properties, which are among the most significant. This vitamin can neutralize oxidants likely to cause inflammation. We all know how harmful and destructive inflammation is in cystic fibrosis, especially in the lungs. Furthermore, it is becoming increasingly certain that vitamin E has a positive effect on nerve and muscle cell function, red blood cells, and coagulation.

HEALTH COLUMN
SVB/ 2001, No 26, page 42

Weight and Force Feeding

I have a very hard time gaining weight. Recently, I asked to be force-fed intravenously, but my physician seems very reluctant, and I can't understand why. Is it the cost, the effectiveness, the risks, or all of these reasons together?

The intravenous force-feeding to which you are referring is usually called parenteral hyperalimentation in this case. It is used as a last resort for malnourished persons who cannot absorb nutrients through their digestive system.

However, when oral alimentation is insufficient, but the digestive tract remains functional, which can happen in cystic fibrosis patients, parenteral alimentation is used. This can be done in three ways: by means of a nasogastric tube (from the nasal passages to the stomach), a gastrostomy tube (inserted directly into the stomach) or a jejunostomy tube (inserted directly into the intestine).

Intravenous or parenteral hyperalimentation is generally used in hospitals when the intestines are not functional (because of an inflammatory disease, infection, tumour, or following surgery). It is, indeed, more costly, and, furthermore, it is also can cause increased infectious or thromboembolic complications (blood clots in the veins) following prolonged use of central catheters.

HEALTH COLUMN
SVB/ 2001, No 26, page 43

Transplantation: Pancreatic Transplantation

Would a pancreatic transplantation be helpful to CF adults?

The pancreas has two main functions: supplying enzymes to help digest food, and insulin to metabolize absorbed sugars.

In cystic fibrosis, the first function is deficient. It must be noted, however, that diabetes may also occur in about 15% of the adult CF population.

Up to now, pancreatic transplantation has been performed to treat diabetes. This type of transplantation is usually done at the same time as that of a vital organ such as a kidney. Although technology is advanced enough to re-establish normal enzyme production through transplantation, it is still necessary to regularly take medication that is far from harmless (such as cyclosporine and cortisone) to guard against rejection. Since, in most cases, we are able to control insufficient pancreatic enzyme production with an enriched diet, vitamin supplements and enzyme capsules, it is best to continue with the conventional treatment for now.


HEALTH COLUMN
SVB/ June 1990, No 11, page 31

Transplantation: Kidney Problems

Is it true that transplant recipients eventually develop kidney problems? If so, can you explain why? Is it possible to avoid this kind of complication?

Transplant recipients (lung or other organs) are indeed at risk of developing kidney problems, primarily due to the necessary use of immunosuppressants, which prevent organ rejection. Cyclosporine A is the most important immunosuppressant drug. Although it decreases blood filtration through the kidneys and may cause hypertension (which can in turn affect kidney function over time), Cyclosporine A is nevertheless an essential anti-rejection drug. The dose has to be precisely regulated and any hypertension must be treated with medication. A similar drug, Tacrolimus, is very effective in preventing organ rejection, but has the same side effects as Cyclosporine A (i.e. kidney dysfunction and hypertension).

With the discovery of new forms of anti-rejection treatment (less toxic medications, cyclosporine in aerosol form), it will be easier to maintain kidney function after transplantation.

HEALTH COLUMN
SVB/ Fall 1998, No 23, pages 28-29