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
Arrhythmia and tachycardia
Bad breath
Clubbing
CO2 and Oxygen Flow
Delayed growth
Diabetes and cystic fibrosis
Enlarged heart and cystic fibrosis
Fever
Gastroesophageal reflux
Hemoptysis
Laryngitis
Pancreatic cystic fibrosis
Pneumothorax (respiratory system)
Thirst

TREATMENT

Antibiotics

Antibiotics, intestinal flora and probiotics
Antibiotics and Length of Treatment
Antibiotics: Milk and Alcohol
Antibiotics: Vitamins
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
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

Blood types
Grapefruit
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
Hair removal
Indoor Plants
MRSA
Multiresistant Pseudomonas
Pneumococcal Vaccination
Research Phases
Sports to Avoid
Terminology
Vaccines and travel

Indoor Plants

Is it true that people with cystic fibrosis are advised not to keep house plants? I have heard that damp earth is an excellent breeding ground for bacteria. What’s your opinion?

I do not discourage persons with cystic fibrosis from owning house plants. Keep in mind that our environment is full of bacteria. Our skin and digestive tract are colonized by many types of bacteria too. Although most bacteria are harmless, some can be potentially dangerous. This is where our body’s defence mechanisms come into play to protect us. The bacteria found in plants and potting soil are no more threatening than those in our environment.

On the other hand, there is a fairly high incidence of allergies among persons with CF, so there is a greater risk of allergic reactions to the plants. Although very few of them are allergenic, we know that certain types of shrubs, such as small maples, can produce allergenic pollen. Furthermore, potting soil sometimes harbours mites —tiny, highly allergenic parasites that are present in dust (carpets, bed linens), so we recommend that people who are allergic to mites not keep plants in the bedroom.

HEALTH COLUMN
SVB/ 2003, No 27, page 38

Ecstasy

I love going to raves and taking ecstasy. Do you think that ecstasy is more harmful to persons with cystic fibrosis than to the general population? Have the dangers of this drug been overestimated?

Before answering your question, I would like to explain what this drug is made of. Ecstasy is the popular name for MDMA (3, 4-methylenedioxymetamphetamine). It is derived from amphetamine, which was synthesized in 1914 and used as an appetite suppressant. It was recycled in the 1970s and 1980s for use in psychotherapy: some therapists used it because it reduced the patients’ inhibitions and helped them talk more openly about their problems. Ecstasy became very popular in the 1980s, when it was adopted in English-speaking countries as a recreational drug at raves. It was then taken off the market. Today, you can buy ecstasy in pill form or capsules on the black market.

The effect of ecstasy on the brain lies somewhere between that of amphetamines (stimulant) and mescaline (hallucinogenic). Ecstasy users also behave in a sensuous manner because the drug makes them feel close to others. In small doses, the side effects are fairly harmless: reduced appetite, dry mouth, palpitations, tightness in the jaw, insomnia, hot flashes and sweating. Once the effect wears off, withdrawal symptoms may include fatigue and varying degrees of depression. With higher doses, however, the consequences can be more serious, including high fever, a rise or drop in blood pressure, irregular heartbeat, cerebral or gastric hemorrhage, convulsions and acute liver or kidney failure, all of which are potentially fatal complications. Psychological effects range from anxiety combined with feelings of panic, to psychosis or serious depression. All these complications can occur in a normal individual. A person with cystic fibrosis who has major lung and digestive problems would be more at risk of developing serious complications, in my view.

Mild form of CF and male fertility  

I’m 22 years old and have cystic fibrosis. Luckily, I have no symptoms. Is it possible for men with a mild form of CF to be fertile?

Unfortunately, the infertility rate in men with cystic fibrosis is very high, whether they have a mild or severe form of the disease (95 to 99%, according to studies). Furthermore, even male carriers of the abnormal CF gene whose lungs or digestive tracts are not affected by the disease have the same type of sterility as men with CF i.e., the absence or degeneration of the vas deferens (ducts that carry seminal fluid from the testes to the penis).

Men with CF can, however, have biological children. The spermatozoa are removed from the testes to fertilize the spouse’s eggs in vitro. A fertilized egg can then be transferred to the uterus and result in a normal birth. However, the procedure is costly ($5,000 to $10,000). It is not covered by health insurance, and success is not guaranteed.

HEALTH COLUMN
SVB/ 2003, No 27, page 39

Pancreatitis

I have repeated bouts of pancreatitis. I would like to understand why I’m one of the few persons with cystic fibrosis who experiences this type of complication. How does pancreatitis develop and how can I reduce the number of episodes? Finally, I would like to know whether it is possible to solve this problem once and for all.

It is true that most cystic fibrosis patients do not suffer from acute pancreatitis. Right from birth, the secretions in most of these patients are so viscous that they obstruct the pancreatic ducts. This leads to deterioration of the pancreas and the development of scar tissue and cysts (thus the name: cystic fibrosis of the pancreas). The production of pancreatic enzymes is seriously compromised, so these patients, who are described as pancreatic insufficient, have to take enzymes in capsule form to absorb nutrients.

A minority of patients, however, can be carriers of a so-called minor mutation on one of the chromosomes. In these cases, the digestive system is affected to a much lesser degree. The pancreatic secretions are less viscous and the pancreatic tissue is relatively intact because there is sufficient pancreatic enzyme production. These CF patients, who are described as pancreatic sufficient, do not suffer from malabsorption and thus do not have to take enzymes with every meal. Their pancreatic secretions are nevertheless more viscous than those of normal people and can cause an obstruction leading to an accumulation of enzymes in the healthy pancreatic tissue. This, in turn, leads to pancreatic self-digestion causing severe inflammation, which is the source of the pain associated with acute pancreatitis. Obstruction can also result from biliary calculi (gallstones), which usually develop in the gallbladder (gallstones occur more frequently in persons with cystic fibrosis than in those who do not have the disease). The gallstones can block the main pancreatic duct and cause acute pancreatitis. Pancreatitis can also be triggered by toxicity resulting from alcohol or drug consumption, or an excessive amount of triglycerides or calcium in the blood. Many forms of pancreatitis can be prevented, except those related to excessively viscous pancreatic secretions.

HEALTH COLUMN
SVB/ 2003, No 27, pages 39-40

Ibuprofen and scarring

Is it true that recent studies have shown that ibuprofen hinders scarring and that for this reason, it is not recommended for persons with cystic fibrosis?

There are two broad classes of anti-inflammatory agents: steroidal (cortisone) and non-steroidal (ibuprofen and many other drugs). Although it is clear that steroidal anti-inflammatory drugs can hinder scarring, there has not yet been a convincing study proving that ibuprofen and other non-steroidal anti-inflammatory drugs have the same effect on the scarring process.

An initial U.S. study found that the anti-inflammatory action of ibuprofen could delay the deterioration of lung function in persons with cystic fibrosis. The effect would be more significant in young persons with mild lung involvement. A pan-Canadian study involving many cystic fibrosis clinics (some of which are in Quebec) will soon be completed and will either confirm or refute the findings of the U.S. study.

HEALTH COLUMN
SVB/ 2003, No 27, page 40

Enlarged heart and cystic fibrosis

Does the right side of the heart become enlarged over time as the disease progresses? If so, is this dangerous? Does the heart return to normal after a lung transplant?

The right side of the heart can, in fact, be affected at a very advanced stage of lung disease such as cystic fibrosis. The drop in blood oxygen creates increased pressure in the pulmonary arteries due to blood vessel contraction. The high pressure impedes the right heart’s ability to pump the blood in the peripheral venous system to the lungs. The effort exerted by the right heart weakens and dilates it. The enlarged and weakened right heart then allows blood to accumulate in the peripheral venous system, resulting in oedema (swelling due to the retention of fluids), which is especially visible in the lower limbs.

The best way to avoid right heart insufficiency is to oxygenate the blood as much as possible with oxygen supplements administered through a nasal tube or a mask. If the right heart still becomes enlarged, the situation can be reversed following a successful lung transplant, and the heart will regain its original shape and strength.

HEALTH COLUMN
SVB/ 2003, No 27, page 40

Laryngitis

After antibiotic therapy, a bad cold or the flu, I often get laryngitis. I would like to understand this physiological phenomenon. Why does this type of reaction occur? Why do I get laryngitis more often than other people do? Although laryngitis is not painful, is it possible to get it less frequently?

Laryngitis associated with a cold or the flu is the result of an inflammation of the larynx and vocal cords caused by the virus itself, and not by the antibiotics. The resulting inflammatory oedema can be perpetuated by frequent coughing, causing an additional mechanical irritation of the larynx. Colds may be responsible for the cough, but so can bronchitis, which often follows a cold. In addition, speaking loudly for long periods can cause persistent inflammation of the vocal cords, which is why resting the vocal cords is often required for the voice to return to normal. Some people, such as singers and teachers, are more prone to vocal cord problems because they use their vocal cords extensively almost every day. The same goes for smokers (smoke is a significant irritant for the entire respiratory system) and chronic coughers, such as people with cystic fibrosis. For the latter group, adherence to all the conditions of their respiratory treatments should reduce chronic coughing to a minimum, which will make the vocal cords less vulnerable.

HEALTH COLUMN
SVB/ 2005, No 29, page 38

Acute Sinusitis

I have a sinus problem that is poisoning my life. I am a veritable gold mine for my surgeon. I would like to solve this problem once and for all. Is it possible to block or remove the frontal sinuses? If so, what are the risks of this type of surgery?

Sinusitis is almost a universal problem in persons with cystic fibrosis, and is often accompanied by nasal polyps. Acute sinusitis can be treated with antibiotics, but some degree of chronic sinusitis always remains, which produces fewer symptoms. Nasal polyps are more symptomatic: they obstruct airflow in the nose and interfere with the sense of smell. Ear, nose and throat surgery, also known as otorhinolaryngology surgery, is mostly performed to remove polyps. However, despite preventive treatments with cortisone sprays (Nasonex® or Nasacort®), polyps frequently recur and the surgery often has to be repeated. Unfortunately, the sinuses cannot be blocked or removed by surgery.

HEALTH COLUMN
SVB/ 2005, No 29, page 37

Cortisol

I was recently diagnosed with a cortisol problem. According to my physician, the level of cortisol in my blood is too low, which explains why I have so little energy. What exactly is cortisol? Where does it come from and what are its properties? Why are some people with cystic fibrosis more likely than others to have a problem with the production or distribution of cortisol? Is it easy to solve this type of problem?

Cortisol, or cortisone, is a hormone produced by the adrenal glands, which are also called suprarenal glands and are located on top of both kidneys. This hormone, which is secreted in large quantities during periods of stress, has multiple functions too numerous to list completely in this text. Among other things, it helps maintain proper energy levels and prevents a drop in blood pressure. When it is secreted in high doses, it has an anti-inflammatory effect, but it can also weaken the body’s defences and raise blood sugar levels. People with cystic fibrosis have no more cortisol production or distribution problems than the general population, because the cystic fibrosis gene does not affect the adrenal glands. However, cystic fibrosis patients are likely to take cortisone or other products with similar effects, such as prednisone, in pill form or by injection, to treat asthma or allergic broncho-pulmonary aspergillosis associated with cystic fibrosis. When they take cortisone for more than two weeks in quantities greater than the daily quantity produced by the body, the adrenal glands secrete less of their own cortisone. When oral cortisone treatments are stopped, the adrenal glands may be temporarily too lazy to produce sufficient quantities of cortisone, especially in periods of physical stress (infection or surgery). This results in adrenalin insufficiency, the symptoms of which are fatigue, weakness, nausea, vomiting, diarrhea and a drop in blood pressure that could lead to shock. Treatment then consists in putting the patient back on cortisone, in pill form or by injection, according to the severity of the case.

HEALTH COLUMN
SVB/ 2005, No 29, page 36

Cipro® and fitness training

I’m a fitness nut, and have been following a weightlifting program for many years to increase my muscle mass. Lately, I had to start taking Cipro® (ciprofloxacin) to control a lung infection in its early stages. I continued with the weight training during the treatment despite the fact that my joints started to ache. Do you think that this could be caused by the Cipro®? Since the pain is tolerable, do you think there is any danger in continuing the weight-training program while I’m taking Cipro®?

To answer your first question: yes, it is possible that the Cipro® is causing your joint pain. However, this undesirable side effect is rare. I have personally prescribed Cipro^® to many patients—with and without cystic fibrosis — and none of them has ever had that side effect. The cause of the pain could be something else altogether, and it may be just a coincidence that it occurred while you were taking the Cipro®. It could have been triggered by the intensive training or a viral infection, which often results in aching muscles and occasionally in joint pain. Then again, the pain might be related to an inflammation associated with hypertrophic osteoarthropathy, which affects the ends of the long bones and the adjacent joints (mainly ankles and knees). This problem is not rare in persons with cystic fibrosis, and episodes often occur during an exacerbation of lung infection.

I advise my patients not to stop taking Cipro® when it is indicated for lung infection. I also recommend that they continue with their usual activities while undergoing treatment. However, when they are having a serious episode of respiratory infection, they should temporarily reduce or stop the fitness training.

HEALTH COLUMN
SVB/ 2005, no 28, page 44

Methadone

Can you tell me what methadone is and how it differs from other narcotics? Why is it that only specialists can prescribe it?

Methadone is a drug in the opiate class, which includes heroin, morphine and codeine, among others. It is a powerful, long -acting narcotic analgesic. Because its duration of action is 36 to 48 hours, and because it is ingested, this substance has proven to be very useful in treating opiate addicts, both for short-term and long-term detoxification. Until recently, this product was used almost exclusively to treat drug addicts, and only certain doctors specialized in drug addiction could prescribe it. However, in late June 2004, the Régie de l’assurance-maladie du Québec announced that it was adding Metadol^TM (trade name for methadone) to the list of exception drugs for the treatment of cancer pain and chronic pain. Like the other exception drugs, Metadol^TM is authorized once the usual treatments fail.

HEALTH COLUMN
SVB/ 2005, no 29, page 38