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

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