Myth #4: People with CF should not drink milk because it makes too much mucus

Fact: While it may feel like it, milk does not increase mucus production.

A 2005 review of studies concluded that there was no link between milk consumption and mucus production.

In one study, participants infected with the common cold virus reported symptoms of increased in mucus production after drinking milk, but when their mucus production was actually measured there was no statistical difference.

People with CF need to be able to perform airway clearance in order to get as much mucus as possible out of their airways. There are many devices available to assist with this such as vibrating vests, percussors, and even a technique called “clapping” which is performed by another individual.

Additionally, there are medications that have been developed to help thin the mucus and make it easier to cough out. These include hypertonic saline, DNase (Pulmozyme) and guafeneisin, which is an expectorant.

Milk, particularly whole milk, is actually a good choice of beverage for people with CF. It contains plenty of calories, fat, protein and vitamin D to help keep the body healthy. Milkshakes made with ice cream and whole milk are one way for people who are pancreatic insufficient to put on some much needed weight.

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Source: http://www.abc.net.au/health/talkinghealth/factbuster/stories/2007/11/28/2102703.htm

What is BiPAP?

BiPAP stands for Bi-level Positive Airway Pressure. It is a breathing apparatus that helps people get more air into their lungs. The airways are kept open using a BiPAP machine. BiPAP machines are adjusted to dual settings so that the pressure of the air coming in on inspiration can be different from the pressure of the air being exhaled. This is beneficial because it allows the patient to get more air in and out of the lungs without having to exert a lot of muscle energy.

A person’s forced vital capacity (FVC) as determined by a Pulmonary Function Test is largely what determines if a BiPAP machine is necessary. BiPAP is often used for people who have experienced pneumothorax (or collapsed lung) and as a result have loss of ability of the air sacs at the outermost portions of the lungs.

BiPAPs are usually used while a person is sleeping. It is used for conditions other than just cystic fibrosis. Sleep apnea sufferers for example, are often fitted with a BiPAP machine and face mask. Unlike supplemental oxygen, BiPAP masks are worn so that they completely enclose the mouth and nose. Most manufacturers of BiPAP face masks have different models available and all are designed to be as comfortable as possible.

Failure to Thrive (FTT)

The first few years in a child's life are critical period during which the most dramatic changes in growth and mental development take place. When a baby or toddler doesn't gain weight and grow at the predicted rate, a doctor may say that the child is failing to thrive. In many cases, failure to thrive and its accompanying symptoms can be the key to effectively diagnosing cystic fibrosis as early as possible.

Failure to Thrive (FTT) is detected when a child falls below the 80th percentile for height and weight at a given age. Failure to thrive is a somewhat ambiguous term, is not a formal diagnosis. However, it serves as a starting point for exploring the underlying cause of the condition.

Symptoms or behaviors that may indicate failure to thrive include:

-Frequent diarrhea or vomiting

-Irritability and colic

-Unfocused eyes, inability to concentrate

-Developmental benchmarks such as sitting up, talking, etc. are not reached when expected

-Refusing food

-Repeated infections

In many cases, failure to thrive is connected to a baby's inability to eat or utilize calories and nutrients properly. The causes can be either physical, as in the case of a child with a cleft palate who cannot suckle for breast or bottle feeding. Failure to thrive may also be the result of a chronic condition like cystic fibrosis, in which the pancreas cannot secrete the enzymes required to help the body absorb food. Food allergies, celiac disease, and inability to tolerate milk protein are just a few of the underlying causes of failure to thrive in infants.

A child who is failing to thrive may also be the product of a poor socio-economic environment. Living in poverty without access to health care, or an inability to buy the appropriate foods for an infant, can cause failure to thrive. Sadly, failure to thrive is sometimes caused by child abuse.

Doctors rely on growth charts to determine whether an infant is growing normally. Specifically, a baby's birth weight should double by the age of 4 months, and should grow almost 10 inches in the first year. Although birth weight may drop initially, when an infant does not recover that lost weight in a suitable amount of time (2 weeks), the doctor will look into the reasons as to why. Even babies who are born prematurely are expected to progress according to an adjusted growth chart.

Correctly diagnosing the cause of FTT as early as possible is essential to prescribing the most appropriate and effective treatment plan.

How We Breathe: Understanding Oxygenation of the Blood

One of the main functions of the lungs is to oxygenate (bring oxygen into) a person's blood. When a person takes a breath of air, either through the mouth or the nose, it travels down the trachea (windpipe) where it then flows to tubes called bronchioles. At the end of the bronchioles are little sacs. These sacs are called alveoli and are the part of the lungs where oxygenation takes place.

Alveoli have very thin walls, but they have amazingly large surface area. This allows the oxygen in the alveoli to diffuse across the surface as much and as quickly as possible. Oxygen diffuses into the blood, and carbon dioxide is removed from the blood. This process is called gas exchange. The red blood cells, specifically hemoglobin, now full of life-giving oxygen, go out from the lungs via the pulmonary vein, and dispersed throughout the rest of the body.

In patients with cystic fibrosis, the thick, sticky mucus that blocks the airways, bronchioles and alveoli, greatly impedes the body's ability to perform the function of gas exchange. As a result, the blood stream retains too much carbon dioxide. As the carbon dioxide builds up in the bloodstream, a person may begin to experience grogginess, confusion or dizziness. Carbon dioxide in the blood stream also has the potential to damage muscle tissue, including the heart, when it builds up to the point of forming carbonic acid.

Carbonic acid is the same thing that gives a soda its "fizz." Have you ever accidentally burped soda through your nose? Remember how much it burned and irritated your throat and nasal passages? That's because the carbonic acid was damaging the sensitive tissue. The same thing happens in the body when gas exchange cannot proceed normally.

Read the rest of the article here...

Nasogastric (NG) Tube Feeding

Cystic fibrosis patients are encouraged to follow a diet that is high in calories. Doctors recommend that the calories come from high energy foods such as animal proteins and fats. Patients who are pancreatic insufficient have a difficult time gaining and maintaining a healthy weight. As a result, these patients are malnourished. Research has demonstrated that up to 30% of young adult patients with cystic fibrosis (CF) are malnourished.

Failure to maintain a healthy body mass index (BMI) can lead to lower pulmonary function, since the body is not absorbing the nutrients needed to build up strong respiratory muscles. This is just one of the reasons why weight loss is often a precursor or indicator of a respiratory infection. When patients are severely malnourished they may become candidates for what is called "nutritional rehabilitation." This means that the patient is given additional calories and energy via tube feeding.

When Is It Used?

The most popular way of tube feeding is called nasogastric (NG) feeding. Nasogastric feeding involved inserting a thick, flexible tube through the nose, back down the esophagus and into the stomach. From there liquids can either be added to or removed from the stomach. There are different scenarios in which NG feeding is used. These may include instances when a patient is experiencing a bowel blockage and must not eat solid food by mouth; post-operational use to prevent the acidic contents of the stomach from being aspirated (inhaled.) Perhaps the most familiar use of NG tubes is when a person has to have their "stomach pumped" in order to remove poison, or an overdose of oral medication.

NG-tubes are intended for infrequent, short-term use. For general purposes such as post-operative emptying of the stomach, they are only in place for two or three days. However, in the case of cystic fibrosis patients who require tube-feeding, this may be longer. Some cystic fibrosis patients have a new NG-tube inserted on a nightly basis, so they can receive nutrition while they sleep.

Read the rest of the article here...

Nasal Irrigation

Cystic fibrosis patients are prone to nasal congestion, which can inflame the upper respiratory tract and lead to sinus infections. One of the ways to help keep the sticky mucus from staying trapped in the sinuses is to gently flush them with a saline solution. Such rinses are effective in reducing the inflammation and clearing the symptoms of allergies or rhinitis.

Commercially available sinus rinses and saline sprays are available in different concentrations of salinity. Some kits come with the salt water already mixed, while others require you to add a salt packet to warm water that you put into specifically designed container such as a squeeze bottle.

To make your own saline rinse, you can follow this easy recipe:

Isotonic saline

½ teaspoon of sea salt or kosher salt

1 cup of warm water (or room temperature will do)

½ teaspoon of baking soda

Combine all ingredients in a cup or bowl. Using a bulb syringe, draw the salt water into the bulb.

Proper administration of a saline flush is necessary in order to reap its benefits. A thorough flushing can be a messy process, so it’s best to lean over the bathroom sink or shower when rinsing with it. Start by tilting your head down toward the bowl of the sink. Leave your mouth open. With the filled bulb syringe (or squirt bottle that came in the kit) squeeze firmly into one nostril. The water should flow out the other nostril. If your sinuses are quite congested, some water may flow down the back of your throat and out your mouth. This is normal. Continue rinsing with the other nostril.

Follow up the procedure by gargling with water. This will keep the salt from irritating your throat and causing a cough reflex.

A more aggressive way to flush congested sinuses is to run the saline mixture through a Water-Pik. Don’t use the attachment as is; cut it back about an inch so that the pressure of the water coming out is reduced. This method is quite effective for dislodging stubborn mucus that lingers following a sinus infection. Water-Pik now makes a nasal flush attachment tip that makes flushing your sinuses even easier.

Hypertonic saline is useful in stimulating the linings of the nasal passages to produce some moisture of their own. This helps give the mucus a more slippery surface to slide across, making it easier to clear out any congestion. Hypertonic saline is a saltier concoction and therefore may sting sensitive sinuses. It’s best to slowly increase the salinity (saltiness) of the rinse solution by adding gradually more salt.

CF Patient Preferred Sinus Rinse Products

• Breathe Ease
• Ocean
• Oasis
• Salt-Air

Nutrition, Exercise and Well Being

Lungs aren’t the only organs affected by the thick, sticky mucus that cystic fibrosis produces. The pancreas, an exocrine gland that normally secretes digestive enzymes necessary to absorb nutrients from food, can be coated with mucus as well. In fact, nearly 85% of CF patients demonstrate pancreatic insufficiency before age 10. This can lead to issues of malabsorption and malnutrition due to the body’s inability to break down fats, carbohydrates and protein before they pass into the intestinal system.

Nutrition and Calories

In order to remedy this problem, many CF patients are prescribed digestive enzyme supplements to help the body better absorb the vitamins and minerals that are present in the diet. However, digestive enzymes are only part of the solution. A person with CF needs to incorporate more calories into their diet than other healthy people the same age. Important ways that you can help your child with CF grow healthy and strong include providing good overall nutrition along with extra fat and calories as well as prescribed enzymes.

Table 1. Caloric Intake Ranges Based on Age Group and Activity

It is important for everyone with CF to combine good nutrition with appropriate exercise. One of the challenges that await school-aged children upon returning to the classroom is that eating habits may need to change. For example, a child who has been able to “graze” frequently during the day may have trouble adjusting to the pre-determined eating schedule set by the school or classroom.

Another problem that may indirectly affect CF students is the obesity epidemic. Many schools are adopting low-calorie, low-fat meal programs in an attempt to address the alarming rise in the number of overweight and obese children. If your child is participating in a school lunch program, be sure that the meal offers the appropriate amount of calories for your child’s age and activity level.

On school days, offer your child or young adult a large, balanced breakfast. Meals that include complex carbohydrates (e.g. whole grain toast) take longer for the body to break down and provide energy for a longer period of time. You the school nurse, who in turn may speak with your child’s teacher or the school administration, to permit additional snack breaks as necessary.

Correlating Nutrition and Lung Health

The need for adequate nutrition cannot be stressed enough. The Cystic Fibrosis Foundation (CFF) has identified a correlation between nutritional health and lung health. Studies have shown that patients with better Body Mass Index (BMI) values for their age, height, and weight, typically perform better on pulmonary function tests. Many CF physicians stress the importance of patients reaching healthy weight and BMI goals before age 6 in order to reduce the likelihood of hospitalizations due to pulmonary exacerbations.

Certain sports related activities, such as running and jumping, generate vibrations in the chest cavity which may stimulate coughing. Coughing should never be suppressed, because it is how the body rids itself of infection-prone mucus in the lungs. Getting the body moving acts as a natural form of chest physiotherapy.

Exercise is also an effective way to prevent infection and inflammation of the lungs. Scientific studies have demonstrated that physical exercise is just as important as chest physiotherapy. Any sort of activity that forces the respiration rate to increase will naturally strengthen the muscles used to cough. Strong back, chest, and abdominal muscles that are well toned from exercise can make coughing easier and less exhausting. Aerobic (oxygen using) and cardio-pulmonary exercise is particularly important for people with CF. As the heart rate increases, the rate of blood flow increases, thereby delivering vital oxygen throughout the body.

Although it may be tempting for parents of students with CF to want their child to opt-out of physical education class, this is not a good idea. The school nurse should speak with coaches and PE teachers about the importance of exercise and its benefit to the student with CF. Students with CF should be encouraged to participate as much as they are able in sports or other fitness activities. If outdoor activity aggravates allergy symptoms, look for other ways to encourage rigorous cardio-pulmonary activity.

Community classes in martial arts, dance, or gymnastics may be an alternative. Another option is to have your student enroll in the school’s band or orchestra. Wind instruments such as the clarinet, trombone, trumpet or French horn are excellent for improving and maintaining lung function. This can also open the doors to scholarship opportunities when your student is ready to apply for college.

The combination of nutrition and exercise can lead to lasting benefits for all ages of those with cystic fibrosis. Many CF Team Clinics have nutritionists and/or dietitians on staff to help determine the best way to incorporate adequate nutrition and exercise into the busy lives of CF patients and their care givers.

Understanding Meconium Ileus

Meconium ileus is one of the prevalent first signs of cystic fibrosis in infants. 1 in 5 people with cystic fibrosis have undergone treatment for meconium ileus. Meconium refers to a baby’s first bowel movement. Often it is greenish in color, and has built up in the infant’s bowels prior to birth. Normally this material is expelled early on. In babies with CF however, the thick, sticky mucus present in the intestines prevents the thick, tar-like meconium from moving. It remains in the bowel, causing an obstruction and a bloated abdomen. In many cases surgery is required to remove the blockage.

The blockage, which accumulates at the lower end of the small intestine (i.e., the ileum) must be removed to prevent damage to the bowel. Treatment for meconium ileus depends on the severity of the blockage. Early detection using an x-ray or CT scan helps determine whether treatment will require surgery or simply a strong course of mucolytics.

-What is an Ileostomy?-

Treating meconium ileus with surgery requires that the blocked portion of the intestine be removed. In order for them to heal properly from the procedure, an ileostomy must be done. An ileostomy is the technical term for the surgery in which an opening in the abdomen, called a “stoma” is created. The ileus, which is the end portion of the small intestine, is brought through the stoma. A small bag is placed at the site and taped to the skin. Fecal material is excreted into the bag instead of at the anus.

Once the intestine heals and blockage is no longer a risk, another surgery called an “ostomy reversal” is done to put everything back together inside the body. The first bowel movements upon reversal are quite frequent. It’s normal to have several per day, possibly in excess of 10.

-Life After Ostomy-

CF patients who have undergone an ileostomy and reversal must consider carefully the foods they eat. Failure to absorb nutrients and vitamins from food is a common problem for all CF patients who are pancreatic insufficient. Those who have had bowel surgeries should take extra care to make sure they eat enough calories per day to off-set the nutritional loss that occurs as the result of frequent stools.

Maintaining adequate hydration is also key, since people who have had intestinal blockages in the past are most likely to experience them again.

Understanding Bowel Movements

Much to the chagrin and embarrassment of CF patients everywhere, bowel movements are an important part of our lives. In many ways our bowel movements serve as a benchmark that lets us know how things are going, so to speak, with our digestive systems. Cystic fibrosis patients with pancreatic insufficiency should be especially aware of all things related to their digestive process, and be able to identify what’s normal for them and what isn’t.

When the pancreas is too blocked by mucus, it can’t deliver the enzymes necessary to break down the foods we eat, particularly proteins and fats. As a result, people with CF may have more frequent bowel movements because they are not absorbing the protein and fat that they are eating. As if that weren’t embarrassing enough, CF patients are also prone to flatulence (gas). Their gas and BMs can be particularly foul-smelling as the result of proteins and fats not being absorbed by the body.

So how can a person with CF determine what’s a normal BM and what isn’t? Consider these three factors:

1) Color

2) Condition

3) Quantity

COLORS

Brown is generally the “preferred” color of healthy stool, though there may be different variations on that theme.

The liver excretes bile salts into the stool, giving it a normal brown color. Obstruction to the flow of bile out of the liver (you may see the word "cholestasis"), or liver infections like viral hepatitis, may produce clay-colored stools.

Black, tarry stools with a foul odor can be the result of eating certain foods, taking iron supplements, or possibly from internal bleeding. Foods that are dark blue or black in color may also cause black stools. Examples of foods and minerals causing dark stools include: black licorice, blueberries, iron supplements, lead, Pepto Bismol (contains bismuth.)

Green, leafy vegetables contain chlorophyll which could be coloring the stool green. Another possibility is that the stool has passed too quickly through the large intestine and has not yet had all of the bile absorbed. Sometimes newborns have green colored stools, and this is perfectly normal.

Patients with cystic fibrosis often have gall bladder issues, which can cause the stools to appear orange. This is because bile, which normally helps process fats in foods may not be excreted adequately. Stools are usually orange if they contain a lot of oil and fat that the bile salts have not had enough time to break down. A lack of bile (because of bile blockage such as gall stones) can also cause stools to appear orange. Orange, greasy stools are particularly foul-smelling, and may be difficult to control.

CONDITION

Healthy stools should be uniform in shape and consistency. They should sink and not float. Floating stool is evidence that you are not absorbing enough fat from your food.

Loose, watery stools may indicate a stomach virus, especially if they are concurrent with vomiting. In young children especially, it’s important not to let diarrhea lead to dehydration. Dehydration as a result of lost water through the stools may also contribute to intestinal blockage. If the small intestine, which usually adds water to digested food, is unable to do so, the fecalization process cannot take place and the stool can back up into the upper gastrointestinal system.

Stools that are difficult and painful to pass are often the result of not enough fiber in the diet. Patients with cystic fibrosis have to strike a delicate balance between having just enough BMs to prevent bowel obstruction, but not so many that they lose water and vital nutrients.

QUANTITY

Cystic fibrosis patients are encouraged to eat several small meals throughout the day instead of 3 large ones, as a way to avoid painful bloating and stomach pains. This means that for some people, going to the bathroom 6 times a day is just part of life. Some patients with cystic fibrosis are very regular in their bathroom habits, and can accurately predict which foods will go through more quickly than others. Usually it’s the foods that are high in fat content that cause a person to use toilet shortly after eating.

One of the earliest signs of cystic fibrosis is a condition called meconium ileus. Approximately 20% of cystic fibrosis will be diagnosed at birth because of mecomium ileus. Meconium is the material present in the intestines of a newborn. In meconium ileus, intestinal obstruction results from the impaction of thick, tenacious meconium in the small bowel.

Gall Bladder Issues in Cystic Fibrosis

The gall bladder is a small, pear-shaped organ that connects to the liver by a series of ducts. As part of the digestive system, the gallbladder’s role is to store bile (a bitter liquid produced by the liver) that will be used to digest food, particularly fat, on its way to the small intestine.

Nearly every cystic fibrosis (CF) patient has or will develop gallbladder problems at some point during his or her lifetime. In people with CF, the gallbladder is abnormally small and fails to function properly due to blockage by thick bile. In many cases, the gallbladder becomes atrophied because of non-use. Gall stones may form when the gall bladder fails to empty properly for long periods of time. In cases where the gall bladder becomes blocked because of gall stones, the risk of pancreatitis (swollen pancreas) increases.

Symptoms that may indicate gallbladder irregularities include:

-abdominal pain in the pit of the stomach

- pain and discomfort when eating

-difficulty taking deep breaths

-vomiting

-pale stools

-dark urine

-abnormal or unusual bloating with indigestion

In order to determine if the gall bladder is functioning properly, or if gall stones are creating a problem, your doctor may order imaging tests, such as an ultrasound, abdominal CT scan, or x-rays. One of the most effective ways to see how well the gall bladder is working is by performing a CT scan with contrast. In this type of scan, a special type of dye is injected into the patient’s arm. The CT scanner will then take a series of images as the dye moves from the liver to the bile duct, gall bladder, and ultimately, small intestine.

Occasionally the gall bladder will need to be surgically removed. This is often done via laparoscopy, a procedure in which only a few small incisions are made in the abdomen. This type of surgery is relatively simple, typically performed on and outpatient basis. Removal of the gall bladder will not impair digestion. The only difference is that your body will no longer be able to store bile between meals. Once the gall bladder is removed, bile will move directly from the liver to the bile duct and into the small intestine.

According to Dr. Brian K. Weinstein, "Up to 27% of patients with CF have gallstones, but only about 4% are symptomatic. Biliary colic is the pain associated with stones as they pass. This pain usually passes, but can recur. When the gallbladder is swollen, with pain, this can be cholecystitis, an inflammation and infection of the gallbladder. This can initially be treated with antibiotics, but usually is an indication for surgery."

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Supporting links

Gall Bladder Disease in Cystic Fibrosis

The Gall Bladder and Biliary Tract in Cystic Fibrosis

Bile Acid Secretion in CF: Evidence for a Defect Unrelated to Fat Malabsorption

Understanding Bowel Obstruction

The biggest challenge to the digestive system in persons with CF involves the failure of the pancreas to secrete the enzymes necessary to break down food before it reaches the intestines. A number of supplemental synthetic enzymes have been developed by pharmaceutical companies to aid in this process. However, complications can still occur.

Thick mucus present in the intestines prevents the body from absorbing vitamins, nutrients, fats and proteins thus rendering a person with CF malnourished. Blocked intestinal and pancreatic ducts contribute to episodes of bowel obstruction, which occur when stool content blocks the bowel and prevents the digestion process from proceeding normally.

Read the complete article on the Progression, Treatment and Prevention of Bowel Obstruction.

Understanding Pancreatic Enzymes

Definition of an enzyme:

(EN-zime) protein made by the body that brings about a chemical reaction, for example, the enzymes produced by the gut to aid digestion.[1]

One of the functions of the pancreas is to secrete enzymes into the small intestine. Enzymes are critical to the digestion process, because they break down food. In persons with cystic fibrosis, the pancreas is unable to release enzymes because the ducts (i.e. passageways) are blocked by thick sticky mucus.

In order for the body to make use of food, it has to have a way to absorb the nutrients from that food. Protein, carbohydrates and fat are the three sources of calories in food. There are specific types of enzymes that break down each of these. Amylase helps the body absorb carbohydrates. Lipase is effective in breaking down lipids and fats. Protease breaks down protein.

People with CF are prescribed one of the following brands of supplemental enzymes:

Creon ®, Ultrase ®, Ultrase ® Viokase ®, Pacreacarb ® MS, Pancrease ®, Pancrease ® MT

No matter which type of supplemental enzymes a person takes, they all contain a mixture of lipase, protease and amylase. The only difference is in how many units of each is contained in each capsule. The capsule is filled with tiny beads—these are the enzymes.

Enzyme supplements are sold as “enteric coated capsules.” Enteric coating means that the capsule is designed to break down in the small intestine, and not the stomach. This is so that the beads of protease, amylase and lipase are released in a way that mimics the body’s natural digestion process.

The number of enzyme capsules a person must take varies depending on the type of food being eaten. The Cystic Fibrosis Foundation states that foods containing only simple carbohydrates and no fat, do not need to be accompanied by enzymes.[2] These foods include jelly beans, popsicles, tea and coffee.

The strength of a supplemental enzyme (and sometimes its name) is derived from the number of lipase units. Below is a list comparing amounts of protease, amylase and lipase in some name-brand enzyme supplements. For additional information, check the product manufacturers websites listed at the end of this article.

CREON 10

Lipase 10,000 USP units
Protease 37,500 USP units
Amylase 33,200 USP units

PANCREACARB MS 8

Lipase 8,000 USP units
Protease 45,000 USP units
Amylase 40,000 USP units

ULTRASE MT 20

Lipase 20,000 USP units
Protease 65, 000 USP units
Amylase 65,000 USP units

Determining which type of enzyme supplements to take and how many capsules requires the assistance of a nutritionist or dietitian. This may require a 3 day fecal fat study. During the study, you will be asked to follow a strict diet that has a minimal amount of fat calories. A stool sample will then be collected which will be analyzed to see how much fat is not being absorbed by the body. This information will enable the dietitian to calculate how many units of lipase, protease and amylase should be taken with meals.

There is no “one-size-fits-all” solution when it comes to supplemental enzymes. However, supplements are required in people with CF who demonstrate pancreatic insufficiency. There are many ways to adjust the dosages of enzymes. There are differences in opinion as to whether they should be taken with a meal, before a meal, throughout the meal, or after the meal. Based on the way supplemental enzymes are designed to mimic pancreatic enzymes and their release into the small intestine, it seems to make the most sense to take supplements before the meal. That way, they have been broken down, and will sit in the gut awaiting food that will travel from the stomach to the intestines.

No matter which type of enzymes have been prescribed, it is important to keep your CF doctor informed about how well you’re eating and moving your bowels. A mismatch between food habits and enzyme dosages could result in just a stomach ache, or a more serious bowel obstruction.

Most importantly, without enzymes, the body would not be able to absorb the nutrients and calories it needs to function and grow. Good nutrition is essential to overall health for a person with CF. In fact, studies have demonstrated a link between higher lung function and higher nutritional status.

Like other medications, there are some side effects that could occur by taking enzyme supplements. Mostly these include stomach ache, possible bowel blockages, or bloating. However, these are easily avoided by taking the correct dosage and checking in frequently with the nutritionist at the CF clinic.

Although there are generic enzyme supplements available, the Cystic Fibrosis Foundation urges patients to use brand names only. Generic supplements are not as strictly formulated and are not guaranteed to have a consistent ratio of lipase, amylase and protease in each capsule.[3] A number of CF patients have reported extreme discomfort and major gastrointestinal problems as the result of taking generic enzymes.

The effectiveness of enzyme supplements has been called into question in the last few years. Because enzymes only work properly under very precise conditions, the FDA[4] is working hard to ensure that patients who receive enzyme supplements are indeed receiving the freshest, safest product. Clinical trials are underway and are schedule to conclude sometime in 2008.

Some important things to remember about enzymes:

-do not refrigerate them
-do not let them stay in the sun
-do not let the pharmacy substitute generic for name-brand
-do not skip doses

For more information, be sure to visit the links below:

Axcan Pharma (Maker of Ultrase)

CFF.org Enzyme Supplements

Solvay Pharmaceuticals (Creon)

Enzyme Stuff.com

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[1] Diabetes.org

[2] Enzyme Replacement Fact Sheet (pdf)

[3] New York Times article

[4] CFF info on enzymes

How Vitamins Help

People with CF tend to be underweight and malnourished despite ravenous appetites. Furthermore, the mucus causes many gastro-intestinal problems, including malabsorption. Vitamin supplements are often prescribed in order to make up for everything the body is not getting from digested foods. Fat is exceptionally difficult to digest. Vitamins A, D, E and K, all of which are fat soluble, are the most common supplements taken by CF patients. In general, these help the body fight off inevitable infections.

-Retinol (vitamin A) promotes good vision, including night vision. It is an antioxidant, which means it wards off anything that would attempt to consume oxygen in the blood. It also helps prevent osteoporosis.

-Vitamin D is especially important for a person with CF when it comes to maintaining bone density. In the absence of vitamin D, osteoporosis may occur. Without a strong skeletal structure, good posture is compromised making airway clearance more difficult. This in turn leads to increased frequency of lung infections.

-Vitamin E, an antioxidant, is integral in the formation of red blood cells. Blood that is well oxygenated helps maintain good pulmonary function.

-Vitamin K, also known as mephyton, helps maintain bone density as well as promote blood coagulation. Although this vitamin is synthesized naturally in the intestines, the amount is not enough. Like vitamin D, mephyton is useful in preventing osteoporosis.

Each of these vitamins work together, which is why the body requires specific amounts of each. People with cystic fibrosis should have the doctor frequently monitor their vitamin levels to ensure proper nutritional status.

Related Resources:

Personal thoughts on Nutrition

Glyconutrients? None For Me, Thanks

Special Programs from SourceCF: Why Pay for Vitamins if You Don't Have To?

Products and Services from Axcan (the makers of ADEKs)