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

Pancreatic enzymes

My physician is having a hard time specifying the quantity of pancreatic enzymes I should be taking with meals. How do you determine the right quantity and proper concentration of enzymes for patients? Is it better to take these enzymes before, during or after meals?

The quantity of pancreatic enzymes required for proper digestion and food absorption can be roughly estimated by the quantity of fat ingested at each meal. However, there are significant differences from one person to the next for reasons that are not always properly understood. Some of my patients need only 20,000 units of lipase per meal, while others take 10 times more. The dose is usually adjusted on the basis of clinical criteria: we gradually increase the dose until the symptoms of malabsorption (stomach cramps, diarrhea and foul-smelling, greasy stool) disappear. However, there may be other causes for stomach pain and diarrhea besides insufficient pancreatic enzymes. When an increase in dosage fails to make the symptoms go away, it is necessary to look for other potential causes and, sometimes, measure the amount of fat in the stool.

Lastly, pancreatic enzymes should be taken at the beginning of meals that are not too copious or drawn out. For longer, more abundant meals, it is better to take some enzymes before and some in the middle of the meal.

HEALTH COLUMN
SVB/ 2007, No 31, page 39

Viagra^TM

I have been having significant coughing fits for almost a year. I cough more during intercourse, which makes it difficult to maintain an erection. I find this very annoying. Do you think that Viagra^TM could help me maintain my erections?

Erections are the result of a complex process involving physiological and psychological phenomena. On the physiological level, erections occur as a result of an accumulation of blood in the corpus cavernosum (erectile tissues) of the penis. This involves vascular elements (blood is transported through the contraction and dilatation of blood vessels), hormonal elements (mostly testosterone, the male hormone, but other hormones also) and neurological elements (the spinal cord and peripheral nerves that transmit electrical stimulation to the blood vessels). Psychological factors also play an important part in triggering and maintaining erections, although the mechanisms are not as well known. These factors include libido (sexual desire), fatigue, stress, depression and performance anxiety.

In young men with cystic fibrosis, the vascular, neurological and endocrine mechanisms are usually intact. Regarding the situation you described in your question, deteriorating health caused by severe respiratory infection could be the cause, but psychological factors are certainly also involved. Coughing would be an embarrassing, annoying distraction that could trigger performance anxiety. One of the solutions would be to control your coughing through better treatment of your lung condition. Also, you might benefit from counselling by a psychologist or sexologist. If the problem persists despite these interventions, you might find phosphodiesterase inhibitors (Viagra^TM, Cialis^TM, Levitra®) helpful. These drugs work by producing smooth muscle relaxation in the corpus cavernosum of the penis and allowing inflow of blood to produce an erection. The main contraindication for these drugs is concomitant use of nitroglycerin, a drug used in treating arteriosclerosis.

HEALTH COLUMN
SVB/ 2007, No 31, page 39

Semen

Some of my ex-partners have pointed out that I produce very little semen compared to other men. Is that normal? If the problem is related to cystic fibrosis, as the disease progresses, will I stop producing semen and will this affect my orgasms? Are there any drugs that increase semen production?

Regarding your first question, that is, whether it is normal for a man with cystic fibrosis to produce less semen than other men, the answer is yes. Semen is made up of spermatozoa and fluid secreted by the testes. This fluid is transported in the vas deferens and, just before it is expelled, secretions from the seminal vesicles and prostate are added. We know that in 97 to 99% of men with cystic fibrosis, the vas deferens have either atrophied or disappeared, most likely because of obstruction in utero, so the important contribution of the testicles is missing in these men. However, although they produce less semen, production is constant, and the quantity and quality of orgasms are not affected in any way. To my knowledge, there are no drugs for increasing semen production.

HEALTH COLUMN
SVB/ 2007, No 31, page 40

Hair removal

I would like to have my underarm and leg hair permanently removed using laser technology. Is there any danger in this for a person taking oral or intravenous antibiotics? Is laser technology contraindicated for people who have had a transplant? What is your opinion about electrolysis?

According to a dermatologist colleague of mine, laser technology for permanent hair removal appears to be safe and not to present any dangers for people taking oral and intravenous antibiotics. Also, there is no contraindication for transplant patients, at least not once their medical condition has become stable following surgery. As for electrolysis, this technique appears to be just as safe.

HEALTH COLUMN
SVB/ 2007, No 31, page 40

Vaccines and travel

I plan to travel after my transplant. Some of the countries I want to visit require certain vaccinations. Should I get these vaccines before my transplant, even though I’m quite ill?

It is important to consider that following a lung (or any organ) transplant, immunosuppressive drug therapy is administered to prevent rejection of the transplanted organ. Consequently, vaccination with live viruses or even live attenuated viruses is contraindicated. Before transplantation, however, even if lung condition is poor, there is no immunosuppression, so there are no contraindications to vaccinations.

I therefore advise that, before your transplant, you update all the childhood vaccinations you may have missed: measles, mumps and rubella, polio, diphtheria, whooping cough and tetanus. The chicken pox vaccine could also be included if you have never had that disease. For travel, you should add vaccines for tropical diseases, such as yellow fever and Japanese encephalitis, depending on your destination. However, following your transplant, I would not recommend that you travel to countries where medical care is rare or non-existent. Even if transplant patients are physically fit at first, it should never be forgotten that they are always in an immune suppressed state, so they are more vulnerable than the general population to unusual infections.

HEALTH COLUMN
SVB/ 2007, No 31, page 40

Glucose intolerance

I recently underwent an oral glucose tolerance test (OGTT). The results indicated that I am glucose intolerant. I would like to understand how we become glucose intolerant and how this diagnosis will affect my diet. Can I be optimistic that I will never become diabetic?

The oral glucose tolerance test measures the body’s ability to metabolize sugar. It involves ingesting 75 g of liquid glucose after fasting. First, blood sugar is measured after fasting, then, two hours after ingesting glucose. Normal fasting blood sugar levels are below 6.1 mmol/L, and after ingesting glucose, between 7.8 and 11 mmol/L.

Diabetes is defined as fasting blood sugar levels equal to or higher than 7 mmol/L or afterload glucose levels higher than 11 mmol/L.

In both these metabolic anomalies, blood sugar levels are too high because not enough sugar is entering the cells to act as fuel. In cystic fibrosis, this phenomenon is mainly attributable to insufficient insulin production by the pancreas, whose action is impeded by fibrosis and cysts associated with the disease. Insulin plays a crucial role in allowing glucose to enter the cells. In periods of secondary lung infection, the inflammation increases, thus impeding insulin’s action. This reaction contributes to the marked increase in blood sugar levels during infection outbreaks. Oral or intravenous cortisone also blocks the action of insulin and contributes to higher blood sugar levels.

Glucose intolerance occurs in about 60% of adults with cystic fibrosis, while diabetes occurs in about 15% of these individuals. It should be noted that all people who are glucose intolerant are susceptible to becoming diabetic. Glucose intolerance naturally has an impact on diet, but you should not necessarily avoid dietary sugars. Complex sugars should be chosen over concentrated sugars, and sugar should be consumed at various times of the day. However, at the diabetes stage, diet may not be enough and subcutaneous insulin injections (or maybe soon, an insulin nasal spray) may become necessary to supplement the lack of insulin. Lastly, in some cases, oral hypoglycemiants in pill form may make up for insulin insufficiency, at least temporarily.

HEALTH COLUMN
SVB/ 2008, No 32, page 39

Enlarged spleen

For as long as I can remember, my physicians have always been impressed by the size of my spleen. I would like to understand the connection between cystic fibrosis and my spleen problem. If it’s true that the spleen is not a vital organ in adults, and that there are serious risks in having an abnormally large spleen, then why is my physician so reluctant to remove it?

Enlargement of the spleen, or splenomegaly, can be explained in persons with cystic fibrosis by the presence of cirrhosis of the liver associated with the genetic disease. The cirrhosis is caused by congestion of the bile ducts because of excessively thick bile. The cirrhosis causes venous hypertension in the spleen, which leads to a gradual increase in the volume of the spleen. Unlike the tonsils and appendix, the spleen is an important organ, particularly with respect to protecting the body against certain aggressive bacteria. Also, even though a large spleen that is not protected by the rib cage could more easily be traumatized and might bleed substantially, and even though it may be uncomfortable and sometimes painful, we try to preserve this organ, which provides protection against potentially fatal infections.

Also, when the liver has deteriorated to the point where a transplant is required, we keep the enlarged spleen. It tends to shrink a little after the transplantation because the surgery corrects the venous hypertension that caused the enlargement of the spleen.

HEALTH COLUMN
SVB/ 2008, No 32, page 39

Gene combination and life expectancy

To my great surprise, I learned that there is a connection between how the disease progresses in a patient and that patient’s gene combination. Does that mean that there are “good” and “not-so-good” gene combinations that affect the life expectancy of persons with cystic fibrosis?

There are five or six types of genetic mutations among the 2,000 known mutations of the cystic fibrosis gene. Depending on the type of mutation, the CFTR (chloride channel that also performs other functions) protein may not be produced at all or, at the other extreme, its function may be only slightly reduced. Some genes in the latter category are associated with pancreatic sufficiency; individuals who carry these genes are not affected seriously enough, at least in childhood, to require pancreatic enzyme supplements. These genes, which we could call “minor genes,” are usually associated with less severe lung involvement and a later diagnosis of the disease. However, this is not an absolute, because I remember a patient who had to have a lung transplant before the age of 25, even though she was able to maintain pancreatic sufficiency.

Moreover, the lungs of people with the same mutations (for instance the ?F508 mutation, which accounts for 70% of the cystic fibrosis gene mutations) could be affected in very different ways. This phenomenon suggests that other factors are instrumental in the seriousness of the lung involvement. These factors could be environmental (smoking, air pollution) or related to other genes besides the cystic fibrosis gene. Research is now being conducted, particularly in Canada, to discover the modifier genes that can influence the severity of organ involvement in cystic fibrosis.

HEALTH COLUMN
SVB/ 2008, No 32, page 40

Nasal polyps and sense of smell

I have had many operations for nasal polyps in the past few years. Now, I can hardly smell anything. Can you tell me what was surgically removed from my nose or sinuses that has so severely affected my sense of smell? Will my sense of smell ever come back?

To answer your question, I consulted a colleague who is an ear, nose and throat specialist and has a lot of experience with nasal polyps, especially in people with cystic fibrosis.

Nasal polyps and chronic rhino-sinusitis in cystic fibrosis are problems that are often hard to control. They often recur following surgery and do not respond well to steroids (cortisone) administered either topically (by inhalation) or systemically (in pill form), compared with the same problem in people with asthma.

The sense of smell is affected, at least at first, because of the obstruction of the airways leading to the olfactory receptors (sensory receptors of the nerve cells responsible for our sense of smell). In this case, polypectomy (surgical removal of polyps) may restore the sense of smell. However, chronic inflammation caused by recurrent nasal polyptosis can destroy these receptors. It is this phenomenon, rather than the surgery itself, that may lead to irreversible loss of the sense of smell.

HEALTH COLUMN
SVB/ 2008, No 32, page 40

Antibiotics, intestinal flora and probiotics

I have heard that the numerous antibiotics I take attack not only the bacteria destroying my lungs, but also the bacteria that play a positive role in my intestinal flora. Everyone seems to be saying that probiotics can restore intestinal flora. What exactly are probiotics? How good are they?

It is a well-known fact that systemic antibiotics (oral and intravenous) can affect normal microbial flora in the mouth, vagina and intestines. Antibiotic sprays, however, do not produce this inconvenient effect. Antibiotic therapy can affect the intestinal flora without producing any symptoms, but patients may experience abdominal pain and soft diarrhea-like stools. There may also be a worse complication: C. difficile colitis, which is also called pseudomembranous colitis. This complication occurs mostly in hospital environments, where patients may be colonized by this toxin-producing bacterium. Colitis can sometimes be severe and destroy the colon, requiring surgical resection or even causing death.

Are probiotics, which are made up of similar bacteria to those found in the intestinal flora, effective in preventing digestive complications?

The experience of many patients using probiotics suggests some effectiveness. However, the ultimate medical proof of effectiveness lies in double-blind clinical trials involving a large number of patients, in which either probiotics or a placebo (inert substance) is administered concurrently with antibiotics. The superior performance of the probiotics over that of the placebo would prove their effectiveness.

The problem is that there have not been any studies to date, except for a recent study conducted in England. This study seems to demonstrate the relative effectiveness of a probiotic preparation composed mainly of lactobacilli. However, further studies would be required to confirm the results. Another problem lies in the very nature of probiotics. They are classified as a natural product, not a drug, and there is no control over the quality of this type of product so we cannot be certain that the probiotic contains the right number of bacteria or even the kind of bacteria it claims to contain. That is why gastroenterologists and infectious disease specialists do not systematically recommend probiotics to prevent or treat intestinal complications when administering systemic antibiotics. In addition, according to an infectious disease consultant, probiotics are contra-indicated in immunodepressed people (such as cystic fibrosis patients following a lung or liver transplant).

HEALTH COLUMN
SVB/ 2009, No 33, page 37

Blood types

I am waiting for a lung transplant. I have O negative blood (a rare type, evidently). Can you explain what distinguishes blood types? Why do I have to wait for an organ from someone with the same blood type? How does my blood differ from that of universal receivers?

In the ABO and Rhesus (Rh) blood group systems, the antigens on the surface of the red blood cells determine the main blood types. In the ABO system, group A has the A antigen, group B has the B antigen and group O has no antigens. In the Rh system, the Rh-positive group has the D antigen, which is missing in the Rh-negative group.

Those in whom an antigen is missing have an antibody against that antigen, which can destroy the red blood cell.

Ideally, people should receive blood transfusions from people in the same blood group. Since this is not always possible, transfusions can be done with blood from compatible groups.

Group O is not rare; it accounts for 45% of the population. Members of this blood group, however, can only receive blood from group O, because their antibodies will attack A and B antigens, although they can donate to all groups: these people are called universal donors.

People in the AB group (5% of the population) can only give blood to people in the same group, but they can receive blood from all the other groups: they are universal receivers. People in group A can receive blood from groups A and O, and people in group B can receive blood from groups B and O.

People in the Rh-negative group cannot receive Rh-positive blood so, since you have O negative blood, you can only receive blood or a transplant from someone in the same group, and this is not a rare occurrence.

HEALTH COLUMN
SVB/ 2009, No 33, page 37-38

Hemoptysis

Would you explain what hemoptyses are? Why is it that some adults with cystic fibrosis who are much sicker than I am don't get them, but I do? How do you prevent hemoptysis? Should I worry about repeated bronchial embolizations?

Hemoptysis is the expectoration of blood from some part of the respiratory tract. Before considering hemoptysis, the first thing to do is make sure that the blood is not coming from the ear, nose or throat (usually the nose) or the digestive tract (usually the stomach). In people with cystic fibrosis, hemoptysis occurs with the rupturing of blood vessels supplying bronchiectases (bronchi that are dilated and deformed by chronic infection and obstruction). These blood vessels are eroded by inflammation caused by chronic infection.

Light bleeding is common in people with cystic fibrosis. Heavy bleeding (> 240 cc/day) is much rarer, occurring once a year in one per cent of patients, mostly in those with major lung impairment, therefore older patients. That being said, patients without severe lung involvement may still occasionally suffer from major hemoptyses; conversely, some patients with severe lung impairment may never experience a serious hemoptysis. The best way to prevent this condition is to optimize respiratory treatments on a daily basis through pharmacological and lung physiotherapy treatments. However, hemoptysis could still occur despite the best treatments. Patients who have had a severe hemoptysis are at greater risk of having a subsequent episode.

Bronchial embolization is the preferred procedure for treating major hemoptyses and persistent minor episodes (100 cc/day for three to seven days). Embolization is a relatively safe procedure performed under local anesthesia, which consists in accessing the bronchial arteries through the femoral artery (groin) or sometimes the humeral artery (bend in the elbow). Once the bronchial arteries likely to be responsible for the bleeding have been identified, they are blocked with microparticles, which stops the bleeding. There is no reason to fear repeating the procedure if major hemoptysis recurs. Before embolization, patients had to undergo surgical resection in the part of the lung where the bleeding occurred. That procedure is now used only when embolization has been unsuccessful or when it is not technically possible. The surgical procedure can be done through thoracoscopy, but there are clearly more risks of complications.

HEALTH COLUMN
SVB/ 2009, No 33, page 38

Arrhythmia and tachycardia

To my surprise, my heart recently started racing for a few moments. I thought I was having a heart attack. I went to the emergency ward and was told that I had arrhythmia. What is arrhythmia and how does it differ from tachycardia? Do people with cystic fibrosis often have these two conditions? Is arrhythmia fatal?

Arrhythmia is a very general term for an irregular heartbeat. It may be a slowing down (bradycardia) or speeding up (tachycardia) of the normal heart rate, which is called the sinusal heart rate because the electrical impulse originates in the part of the heart called the sinus. There are other types of arrhythmia in which the heart rate is no longer sinusal and the heartbeat is too slow, too fast or irregular.

Among those types of arrhythmia, tachycardia is the one that cystic fibrosis patients most often have. It is usually sinusal and consists in an acceleration in the normal heart rate due to exercise, fever or lack of oxygen. This is a normal, non-threatening heart response; to slow down the heart rate, the underlying cause has to be treated. The other types of arrhythmia are infrequent in people with cystic fibrosis. Paroxysmal supraventricular tachycardia is an anomaly in the heart's electrical circuit; it occurs from time to time and begins and ends suddenly. Although it produces stronger palpitations than sinusal tachycardia does, it is not usually dangerous. It can be treated with drugs or by correcting the anomaly in the electric circuit during cardiac catheterization. The most dangerous tachycardia is ventricular tachycardia, which can degenerate into ventricular fibrillation and lead to cardiac arrest. These arrhythmias occur mostly in people with heart disease (angina, heart attack). I have never seen this type of arrhythmia in cystic fibrosis patients, not even in those with severe lung involvement. However, with the increasing prevalence of diabetes and an increase in the age of the cystic fibrosis clientele (especially post-transplantation), it is possible that in the future, people with cystic fibrosis will no longer be safe from heart disease and the malignant arrhythmias associated with it.

HEALTH COLUMN
SVB/ 2009, No 33, page 39

Grapefruit

I have had a lung transplant, and I love the taste of juicy grapefruit. Unfortunately, it seems that grapefruit does not go well with the drugs I have to take. Why is grapefruit dangerous and not recommended for people who have had a transplant?

Grapefruit is not dangerous in itself. Like many other fruits, it is an excellent source of nutrients and vitamins that are good for us. The problem is that it inhibits the CYP3A4 liver enzyme. This enzyme is needed to metabolize a number of medications. Luckily, the drugs normally used by clients at our cystic fibrosis clinics, including antibacterial antibiotics, are not affected by such interactions. These interactions mostly occur with drugs used to treat cardiovascular diseases, lipid disorders and HIV. CYP3A4 also metabolizes certain antirejection drugs (cyclosporine, tacrolimus, sirolimus) used by people who have had a lung transplant. Blood levels of these drugs have to be precise to prevent rejection and avoid overdose toxicity. That is why eating grapefruit and drinking grapefruit juice is not recommended for people taking these antirejection drugs.

HEALTH COLUMN
SVB/ 2009, No 33, page 39