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

Arteriosclerosis and Heart Disease

Does cystic fibrosis make us immune to that which ails our society? Everyone talks about how arteriosclerosis, heart disease and diabetes result from poor eating habits, yet our diets don’t seem to take this into account. Why?

This is a very complicated subject. First of all, arteriosclerosis and the ensuing arteriosclerotic heart disease result mostly from four main factors: heredity, smoking, high blood pressure, and high levels of blood fats (triglycerides and cholesterol). The last factor is not only caused by “poor eating habits”; hereditary diseases and the malfunction of certain organs are often involved. Cystic fibrosis causes insufficient absorption of fats, so blood fat levels are usually quite low. Pancreatic enzymes and a “rich” diet aim to rectify this situation, by bringing blood fats up to normal levels. This is essential because fats are necessary for the proper functioning of the body.

Diabetes is more prevalent within the CF population than the population in general. This is not because of excessive sugar, but rather because the affected pancreas does not secrete enough insulin. The main part of the treatment consists of compensating for this lack of insulin with injections.

As for sugars, they must not be completely eliminated because they too are necessary for proper body function. Diabetics should avoid sugar in concentrated form (chocolate, sweet deserts), however. After this long preamble, I will now answer your initial questions.

A – No, cystic fibrosis does not make us immune to the above-mentioned diseases, despite the fact that it naturally tends to lower blood fat levels.

B – Dieticians design specific diets to achieve two goals: to provide enough calories, fats, sugars, proteins, and vitamins to meet the body’s requirements and to maintain normal (neither too high, nor too low) blood sugar and fat levels.

HEALTH COLUMN
SVB/ December 1989, No 10, pages 39-40

Diabetes and Cystic Fibrosis

I have just been told that infections can cause hyperglycemia. When I have a lung infection I feel weaker, so I increase my sugar intake. Should I stop this?

CF carriers of diabetes or “pre-diabetes” (glucose intolerance), but not other CF patients, may experience an elevation in blood sugar due to acute physical stress such as a lung infection. The elevation is generally not very serious, usually produces no symptoms, and everything returns to normal once the infection has been treated. Nevertheless, a temporary increase in insulin dosage may occasionally be needed for a diabetic.

Weakness and fatigue during lung infections are due to the infection itself and to the deterioration of respiratory capacity it causes, not by changes in blood sugar levels. There is no need to deprive yourself of sugar or to consume it excessively: just continue the regular diet recommended by your dietician.

HEALTH COLUMN
SVB/ December 1989, No 10, pages 39-40

Pneumothorax (Respiratory System)

What is a pneumothorax?

To understand what a pneumothorax is, you must know that the lungs are surrounded by a double-layered membrane called the pleura, and are located in the thoracic cavity. A pneumothorax is an air pocket that forms within the two layers of the pleura. It can happen “spontaneously” when an emphysematous bulla ruptures, or it can be brought on by trauma such as an automobile accident or the insertion of a central venous tract by subclavian catheter.

In cystic fibrosis, the forming of these emphysematous bullae is part of the natural evolution of the disease. Optimal treatment of this lung disease will at least delay the forming of these bullae.

Pneumothorax symptoms consist of sudden chest pain that increases with breath intake, combined with a variable increase in breathlessness.

A mild pneumothorax (less than 20%) can be treated by rest and observation and may actually diminish spontaneously. If this should fail, or in more severe cases, a tube will have to be inserted into the pleura through the rib cage under local anesthetic to suck out the air that is there. If a relapse occurs, there are two possible routine treatments. The first is a chemical pleurodesis, which consists in administering an “irritating substance” into the pleura, thus creating inflammation. This reaction will cause the parietal and visceral pleura to join together, preventing air from getting between them. The other solution is surgery.

For recurrent episodes in CF patients facing eventual lung transplantation, it is best to repeat simple drainage rather than resort to chemical pleurodesis or surgery, because both of these solutions cause adhesions that might complicate lung transplantation.

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

Digital Hippocratism or “Clubbing”

My fingers have significant clubbing. This disorder bothers me so much that it has become an obsession. Why is my clubbing more severe than that of my CF friends who are sicker than I am? Are there any tried-and-true methods to make it disappear?

Clubbing is linked to the dilation of vessels and proliferation of tissues under the fingernails and toenails. The end result is a lifting at the base of the nail and an increase in size at the tips of the toe and fingers. The severity of clubbing is measured by the increase of the angle at at the junction of the base of the nail and the adjacent skin.

Clubbing doesn’t occur only in cystic fibrosis; other lung diseases, heart conditions, liver and bowel disorders can also cause this phenomenon.

In cystic fibrosis, the extent of clubbing varies in individuals with similar lung problems, and there are no known reasons why. In general, however, clubbing progresses with the deterioration of the lungs.

Clubbing may disappear when the underlying illness is cured, e.g. by removing a lung tumour or by healing a lung abscess. In the past, this could not be done for CF patients. It was recently noted, however, that clubbing had diminished or even disappeared in people who received lung transplants.

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

Bad Breath

I have bad breath. No matter how much I brush my teeth or gargle, the problem persists. Why do I have this problem and what can I do to get rid of it? Please help me before I lose confidence in myself and my relationships.

Bad breath most often originates in the mouth for individuals with cystic fibrosis, as for people in general; oral hygiene, cavities and gingivitis are often the causes. Nasal and sinus infections (rhinitis, polyps, sinusitis) are much more frequent in people suffering from cystic fibrosis and can sometimes cause bad breath. It must be noted that in cystic fibrosis, the dilated and deformed bronchi (bronchiectasis) are targets for chronic infections. Apart from the common bacteria Hemophilus aeruginosa, other “anaerobic” bacteria (that grow without oxygen) can cause severe bad breath. In such cases, our experiments show that good results have been obtained using an antibiotic such as clindamycin. All things considered, a dentist, doctor or ENT specialist can usually solve the problem.

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

Gastric Reflux

In the last two to three years I’ve been having severe gastric reflux — even antacids can’t seem to ease the trouble. How does one develop such a problem, and why are some people with cystic fibrosis more susceptible to this condition than others? Is stomach surgery necessary, and are the results of this operation conclusive?

What you are talking about is gastroesophageal reflux, which is the reflux of stomach acid into the esophagus (the passage that connects the throat to the stomach). It is not unusual to have some reflux, but it is abnormal to have too much. In this case, the acidity of the gastric fluid backflow irritates the more sensitive esophageal mucous membrane, causing "burns” around the sternum and sometimes even in the throat. This irritation can also cause spasms in the esophagus: the pain feels like a tightening similar to that of angina. People suffering from cystic fibrosis have this problem more often than the general population. Chronic coughing, which increases pressure in the stomach, can induce reflux. Malfunctioning of the gastroesophageal sphincter (located at the stomach opening, it expands to let food through, then contracts to prevent reflux) can also cause reflux. Several factors influence the contraction of the sphincter: smoking and certain foods, such as alcohol, coffee and chocolate, all weaken the contraction of the sphincter. Bronchodilator drugs, such as theophylline and salbutamol (Ventolin®), have the same effect.

How do you treat this problem? First, some basic advice: avoid big meals and nutrients that weaken the contraction of the sphincter. Also, avoid eating before going to sleep, and raise the head of the bed. No need to discuss smoking, since we all know about its toxic effects on the respiratory system. You should not stop using bronchodilators, however, when they are needed in respiratory treatment. As far as medication goes, your first choice should be antacids, which neutralize acidity and can be effective for a minor problem. Antacids don’t work for long, however, and when the problem is more severe, products that reduce stomach acid production can be taken. Antihistamines such as ranitidine (Zantac®) are effective; omeprazole (Losec®) and other medications in this category are even better. If this treatment is insufficient, it can be combined with medications that accelerate the emptying of the stomach (e.g. Motilium® or Prepulsid®). These measures will control most cases of gastroesophageal reflux. Surgery is a final option and isn’t often resorted to; it is effective, but risky, especially for patients suffering from serious lung diseases.

HEALTH COLUMN
SVB/ Fall 1997, No 22, pages 30-31

Anemia and Cystic Fibrosis

My doctor says I have anemia. What exactly does this mean? Can this condition be cured easily? Would certain changes in lifestyle, such as diet, help solve the problem?

Anemia is defined as a reduction of red corpuscles in the blood. These corpuscles contain hemoglobin, which transports oxygen from the lungs to the tissues, and carbon dioxide, one of the body’s waste products, from the tissues to the lungs. A lack of red corpuscles is characterized by pallor, fatigue, and shortness of breath accompanying physical exertion.

It is important to realize that there are several causes for anemia. It could stem from a deficient production of red corpuscles in the bone marrow, which is often related to a lack of nutrients such as iron, folic acid, or vitamin B¹². This lack of nutrients may be due to poor nutrition, but also to malabsorption in the digestive tract. Furthermore, anemia can be caused by the premature loss of red corpuscles: either they escape from the blood vessels (bleeding), or they are destroyed within the blood vessels.

To cure anemia, the cause must be corrected. Iron deficiency can be rectified with iron supplements, and bleeding, by stemming the flow. Proper nutrition, according to the dietary advice given by the clinic, and the regular intake of prescribed pancreatic enzymes are the most important measures for the prevention of anemia. Given the multiple causes of anemia, however, regular blood tests at the clinic can lead to early detection and allow for diagnosis and treatment before it becomes serious.

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

Delayed Growth

I just turned 16. My problem is that I am much too short. No use telling me that I am not through growing; I am afraid I will stay short my whole life. Are there tests that could let me know if I will grow, and by how many centimetres? Do you think that my doctor would agree to prescribe growth hormones for me? Reassure me, Doctor. Time may be working against me.

As a physician for adults, I must admit that I have little experience with growth problems, so I thought it wise to consult colleagues in pediatric endocrinology. Through our discussions, I learned that the maximum definitive size reached by a given individual depends on several factors. The first factor is heredity: if your parents and grandparents are not tall people, your chances of being tall are reduced. The second factor is the endocrinal (hormonal) status. Young CF patients, like other young people, may be deficient in certain hormones, particularly thyroid and growth hormones. These deficiencies are not very common and can certainly be eliminated, because there are specific, effective treatments to remedy them (hormone supplements) . Moreover, what we find most frequently among CF patients is retarded growth associated with malnutrition, and especially poorly controlled chronic infection. These two factors can retard sexual maturation and bone growth, which are closely related. These factors are also tied to the optimal treatment of cystic fibrosis itself; I cannot emphasize emphasize strongly enough the importance of strict adherence to daily treatment. In certain cases, it may be necessary to accelerate sexual maturation and bone growth through the use of sex hormones.

As far as your own situation is concerned, it must be precisely determined which of these factors has retarded your growth. The pediatrician at your clinic is in the best position to conduct this evaluation. If necessary, he or she can consult an endocrinologist. After blood tests and X-rays, the most appropriate treatment (not necessarily hormonal) can be applied.

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

Fever

When I compare myself to other CF persons, I realize I often run a fever. Can your explain why some CF persons have this problem? Are there effective ways to alleviate the discomfort associated with bouts of fever?

Fever is a sign indicating inflammation in the body. Although fever can be linked to a tumour or an inflammatory illness, it is most often caused by a microbial infection. The main germs that cause infections and fever are viruses (unaffected by common antibiotics) and bacteria (sensitive to antibiotics). CF persons are not more prone than others to viruses such as colds, the flu or gastroenteritis. These viral infections usually bring on a light to moderate fever that lasts no more than two or three days. Because of the abnormalities associated with their disease, however, CF persons are very prone to the bacterial infections of the respiratory system that often follow initial viral infections. These infections (sinusitis, bronchitis, pneumonia) may cause higher, prolonged fever requiring antibiotic treatment. These bacterial infections sometimes occur without serious fever, however, probably because CF persons have a more developed immune system. These enhanced defence mechanisms prevent the bacterial invasion of the circulatory systems, where they are most likely to bring on a fever. On the one hand, the frequency of a fever can be partially explained by the condition of the immune system. On the other hand, persons whose lungs are more seriously affected will suffer infections that can trigger a fever more often. Although some bacterial infections don’t cause fever, it is still important to treat them thoroughly with antibiotics.

Fever can be treated with antipyretic medications (anti-fever) until the infection causing the fever has been cured. The two main medications used are acetylsalicylic acid (Aspirin®) and acetaminophen (Tylenol®, Atasol^TM). For cystic fibrosis, acetaminophen is preferred because Aspirin has more side effects. It irritates the stomach, thins the blood (Aspirin® is not recommended to people who spit up blood), and in the long run, causes ulcers. Aspirin is linked to rare, but serious reactions in certain viral infections in children. Rest and plenty of fluids are also recommended for fever.

HEALTH COLUMN
SVB/ Winter 1995, No 19, pages 28-29

Thirst

Is it true that CF persons don’t experience thirst as often as so-called "normal” people? If so, is there an explanation why the thirst message isn’t communicated as well or isn’t properly decoded by the brain? Could improper hydration have dramatic consequences on the body?

As far as we know, the thirst perception mechanism of CF persons is not affected. Actually, this perception centre is located in an area of the brain where the disease has no known effect. Nonetheless, it is important to drink lots of fluids on a regular basis. Among other things, it contributes to proper bowel function and increases the fluidity of respiratory secretions. You should pay close attention to fluid intake during the dog days of summer, after strenuous physical exertion, and during episodes of respiratory infection, when salt must be added to liquids.

HEALTH COLUMN
SVB/ Fall 1997, No 22, pages 29-30

CO₂ and Oxygen Flow

Why does my body retain more CO₂ when I increase the oxygen flow from my oxygen concentrator?

Carbon dioxide (CO₂) is one of the organism’s waste products and blood levels reflect ventilation (gaseous exchanges) produced by the lungs. However, the brain (most of the time in an involuntary, automatic manner) orders the respiratory muscles to perform ventilation. There are three kinds of signals that alert the brain when ventilation is needed: a decrease in blood oxygen (O₂) levels and increase in CO₂ and acidity in the blood. For some who suffer from chronic respiratory insufficiency, there may be loss of sensitivity in the brain to the stimulus provided by increased CO₂ levels; ventilation is therefore not increased. These people generally have to leave it up to the decrease of O₂ in the blood to stimulate respiration. But if there is too great an increase of O₂ resulting from increased oxygen flow from the concentrator, the brain loses its main stimulus for triggering ventilation, so CO₂ levels rise, causing headaches, drowsiness and even confusion. In these cases, O₂ deficiency should not be corrected too aggressively: blood oxygen levels should be slightly lower than the norm. You should be aware that there may be other reasons for increased CO₂ levels, especially when respiratory muscles are exhausted.

HEALTH COLUMN
SVB/ October 1999, No 24, page 26