A Research in General Pathology

Submitted by:

F#5

Calibo, Jansen S.

DMD2AA

Submitted to:

Dr. Sales

CYSTIC FIBROSIS

Cystic fibrosis, also known as CF or mucoviscidosis, is a recessive genetic disease

affecting most critically the lungs, and also the pancreas, liver, sinuses, sex organs and intestine.

It is characterized by abnormal transport of chloride and sodium across epithelium, leading to

thick, viscous secretions.

It is an inherited disease of your secretory glands, including the glands that make mucus

and sweat. "Inherited" means that the disease is passed through the genes from parents to

children. People who have cystic fibrosis inherit two faulty cystic fibrosis genes—one from each

parent. The parents likely don't have the disease themselves.

Mucus is a slippery substance that lubricates and protects the linings of the airways,

digestive system, reproductive system, and other organs and tissues. In people with cystic

fibrosis, the body produces mucus that is abnormally thick and sticky. This abnormal mucus can

obstruct the airways, leading to severe problems with breathing and bacterial infections in the

lungs. These infections cause chronic coughing, wheezing, and inflammation. Over time, mucus

buildup and infections result in permanent lung damage, including the formation of scar tissue

(fibrosis) and cysts in the lungs.

CF is caused by a mutation in the gene for the protein cystic fibrosis transmembrane

conductance regulator (CFTR). This gene is required to regulate the components of sweat,

digestive juices, and mucus. Although most people without CF have two working copies of the

CFTR gene, only one is needed to prevent cystic fibrosis. CF develops when neither gene works

normally and therefore has autosomal recessive inheritance.

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PATHOGENESIS

I. Mutation in CFTR and their consequences

The CFTR gene encompasses approximately 180,000 base pairs on the long arm of

chromosome 7. The protein contains 1480 amino acids (Figure 1). More than 1000 disease-

associated mutations have been described in the coding sequence, messenger RNA splice signals,

and other regions. These mutations can be classified on the basis of the mechanism by which

they are believed to cause disease (Figure 2). The most common mutation, which is termed

ΔF508 and is present in approximately 70 percent of defective CFTR alleles and in 90 percent of

patients with cystic fibrosis in the United States, is categorized as a class II defect. CFTR with

the ΔF508 mutation lacks a phenylalanine (F) residue at position 508. The defective protein

retains substantial chloride-channel function in cell-free lipid membranes. When synthesized by

the normal cellular machinery, however, the protein is rapidly recognized as misfolded and is

degraded shortly after synthesis, before it can reach its crucial site of action at the cell surface.

Like ΔF508, several other clinically important mutations — such as N1303K, G85E, and G91R

— lead to misfolded CFTR protein that is prematurely degraded.

About 5 to 10 percent of CFTR mutations are due to premature truncation or nonsense

alleles (designated by “X,” such as G542X, a class I mutation). As a result of a genetic founder

effect, prematurely truncated CFTR is particularly prevalent among persons of Ashkenazi Jewish

descent. Other CFTR mutations encode properly processed, full-length CFTR protein that lacks

normal ion-channel activity. For example, the G551D mutation (class III) is believed to possess

little or no chloride-channel function in vivo because of abnormal function of a nucleotide-

binding domain, resulting in disordered regulation. The A455E mutation (class IV) exhibits only

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partial CFTR ion-channel activity, a feature that probably explains a less severe pulmonary

phenotype.47 Other mutation classes include reduced numbers of CFTR transcripts (class V) and

defective CFTR stability at the cell surface (class VI).

II. Mechanisms Underlying the Sweat Gland Abnormality

There is widespread agreement that defects in ion transport, salt homeostasis, or both are

intimately linked to organ damage in cystic fibrosis. The precise molecular basis for this

connection, however, is unknown. Conversely, sweat glands in patients with cystic fibrosis,

which usually do not become obstructed or show major pathologic abnormalities, have

pronounced abnormalities in sodium chloride homeostasis that are well understood. In human

sweat glands, primary secretion elaborated in the glandular coil is modified as it traverses the

sweat duct, before emerging on the surface of the skin. Under normal conditions, sodium

(followed by chloride counter-ion) is avidly reabsorbed from the ductular lumen, primarily

through apical sodium channels and CFTR (Figure 3).

In patients with cystic fibrosis, the absence of functioning CFTR restricts reabsorption of

chloride, thereby limiting the amount of salt that can be reclaimed. Because there is no other

pathway for effective chloride reabsorption in the duct, sodium is also poorly absorbed, and

sweat emerging on the skin surface contains a high level of salt. By the same token, in cystic

fibrosis, the transepithelial potential difference across the sweat duct (the lumen-negative

transepithelial voltage) is two to three times the normal value. The increased lumen-negative

surface charge is caused by an inability to reabsorb chloride despite the continued existence of

pathways for sodium uptake.

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SIGNS AND SYMPTOMS

The signs and symptoms of cystic fibrosis (CF) vary from person to person and over

time. Sometimes you'll have few symptoms. Other times, your symptoms may become more

severe. One of the first signs of CF that parents may notice is that their baby's skin tastes salty

when kissed, or the baby doesn't pass stool when first born. Most of the other signs and

symptoms of CF happen later. They're related to how CF affects the respiratory, digestive, or

reproductive systems of the body (Figure 4).

I. Respiratory System Signs and Symptoms

People who have CF have thick, sticky mucus that builds up in their airways. This

buildup of mucus makes it easier for bacteria to grow and cause infections. Infections can

block the airways and cause frequent coughing that brings up thick sputum (spit) or

mucus that's sometimes bloody.

People who have CF tend to have lung infections caused by unusual germs that don't

respond to standard antibiotics. For example, lung infections caused by bacteria called

mucoid Pseudomonas are much more common in people who have CF than in those who

don't. An infection caused by these bacteria may be a sign of CF.

People who have CF have frequent bouts of sinusitis, an infection of the sinuses. The

sinuses are hollow air spaces around the eyes, nose, and forehead. Frequent bouts of

bronchitis and pneumonia also can occur. These infections can cause long-term lung

damage.

As CF gets worse, you may have more serious problems, such as pneumothorax or

bronchiectasis.

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Some people who have CF also develop nasal polyps (growths in the nose) that may

require surgery.

II. Digestive System Signs and Symptoms

In CF, mucus can block tubes, or ducts, in your pancreas (an organ in your abdomen).

These blockages prevent enzymes from reaching your intestines.

As a result, your intestines can't fully absorb fats and proteins. This can cause ongoing

diarrhea or bulky, foul-smelling, greasy stools. Intestinal blockages also may occur, especially in

newborns. Too much gas or severe constipation in the intestines may cause stomach pain and

discomfort.

A hallmark of CF in children is poor weight gain and growth. These children are unable

to get enough nutrients from their food because of the lack of enzymes to help absorb fats and

proteins.

As CF gets worse, other problems may occur, such as:

Pancreatitis. This is a condition in which the pancreas become inflamed, which causes

pain.

Rectal prolapse. Frequent coughing or problems passing stools may cause rectal tissue

from inside you to move out of your rectum.

Liver disease due to inflamed or blocked bile ducts.

Diabetes.

Gallstones.

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III. Reproductive System Signs and Symptoms

Men who have CF are infertile because they're born without a vas deferens. The vas

deferens is a tube that delivers sperm from the testes to the penis.

Women who have CF may have a hard time getting pregnant because of mucus blocking

the cervix or other CF complications.

IV. Other Signs, Symptoms, and Complications

Other signs and symptoms of CF are related to an upset of the balance of minerals in your

blood.

CF causes your sweat to become very salty. As a result, your body loses large amounts of

salt when you sweat. This can cause dehydration (a lack of fluid in your body), increased

heart rate, fatigue (tiredness), weakness, decreased blood pressure, heat stroke, and,

rarely, death.

CF also can cause clubbing and low bone density. Clubbing is the widening and rounding

of the tips of your fingers and toes. This sign develops late in CF because your lungs

aren't moving enough oxygen into your bloodstream.

Low bone density also tends to occur late in CF. It can lead to a bone-thinning disorder

called osteoporosis.

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CLINICAL LABORATORY PROCEDURES

Cystic fibrosis carrier detection

This test detects mutations in the cystic fibrosis transmembrane regulator (CFTR) gene.

This genetic test is used to help screen for cystic fibrosis. A sample of blood, buccal cells, or

chorionic villus may be collected for this test.

I. What are other names for this test?

Cystic fibrosis DNA detection

Cystic fibrosis mutation analysis

II. What are related tests?

Serum immunoreactive trypsin measurement

III. Why does patient need this test?

Laboratory tests may be done for many reasons. Tests are performed for routine health

screenings or if a disease or toxicity is suspected. Lab tests may be used to determine if a

medical condition is improving or worsening. Lab tests may also be used to measure the success

or failure of a medication or treatment plan. Lab tests may be ordered for professional or legal

reasons. The following is a possible reason why this test may be done:

A. Cystic fibrosis screening

I. When and how often should I have this test?

When and how often laboratory tests are done may depend on many factors:

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The timing of laboratory tests may rely on the results or completion of other tests,

procedures, or treatments. Lab tests may be performed immediately in an emergency, or

tests may be delayed as a condition is treated or monitored. A test may be suggested or

become necessary when certain signs or symptoms appear.

Due to changes in the way your body naturally functions through the course of a day, lab

tests may need to be performed at a certain time of day. If you have prepared for a test by

changing your food or fluid intake, lab tests may be timed in accordance with those

changes. Timing of tests may be based on increased and decreased levels of medications,

drugs or other substances in the body.

The age or gender of the person being tested may affect when and how often a lab test is

required. Chronic or progressive conditions may need ongoing monitoring through the

use of lab tests. Conditions that worsen and improve may also need frequent monitoring.

Certain tests may be repeated to obtain a series of results, or tests may need to be

repeated to confirm or disprove results. Timing and frequency of lab tests may vary if

they are performed for professional or legal reasons.

II. How should I get ready for the test?

A. Venous blood:

Before having blood collected, tell the person drawing your blood if you are allergic to

latex. Tell the healthcare worker if you have a medical condition or are using a medication or

supplement that causes excessive bleeding. Also tell the healthcare worker if you have felt

nauseated, lightheaded, or have fainted while having blood drawn in the past.

B. Buccal cells:

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There is no preparation needed for this test.

C. Chorionic villus:

CVS is a procedure that requires your written consent. Review the consent form with the

healthcare worker and ask any questions that you have before signing the consent form. Tell the

person doing the CVS if you have a history of pregnancy difficulties, such as premature (early)

labor, incompetent cervix (a weak or failing cervix), placenta previa (a placenta that is

abnormally low, near or over the cervix), abruption placentae (the placenta is separate from the

uterine wall too early), and if you are Rh negative (Rh incompatibilities happen when a baby’s

blood has a protein that the mother does not, thus causing an immune reaction). Tell the

healthcare worker if you have a medical condition or are using a medication or supplement that

causes excessive bleeding. You should also report if you have a history of allergic or other

reactions to local anesthetics. Depending on method used to do the CVS, you may be asked to

drink extra fluids and have a full bladder for the procedure.

III. How is the test done?

A sample of venous blood, buccal cells, or chorionic villus may be collected for this test.

A. Venous blood:

When a blood sample from a vein is needed, a vein in your arm is usually selected. A

tourniquet (large rubber strap) may be secured above the vein. The skin over the vein will be

cleaned, and a needle will be inserted. You will be asked to hold very still while your blood is

collected. Blood will be collected into one or more tubes, and the tourniquet will be removed.

When enough blood has been collected, the healthcare worker will take the needle out.

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B. Buccal cells:

Buccal cells are cells from the inner lining of the cheek or mouth. To collect a sample of

buccal cells, you will need to open your mouth wide. A special brush or swab will be rotated

rapidly up and down on your inner cheeks for 30 seconds. Do not close your mouth when the

sample is being collected. After the sample has been collected, the brush or swab will be taken

out and tested.

C. Chorionic villus:

The chorionic villus is a part of the placenta (the organ that nourishes the baby during

pregnancy). A sample of chorionic villus is collected by a procedure called chorionic villus

sampling (CVS). Depending on the location of your placenta, CVS may be done either through

your cervix (transcervically) or abdomen (transabdominally). Both methods will require you to

lie down and will use ultrasound to assist the sample collection. For a transcervical CVS, you

will be in a position similar to a Pap smear. A speculum will be used to gently spread apart your

vagina. Your cervix or vagina will be cleaned with an antiseptic solution. A flexible catheter will

be placed through your cervix and a small sample removed. For the transabdominal method, a

needle will be used to go through the abdominal wall into the placenta. This will allow a syringe

to draw out a small sample of placenta.

If a transabdominal method is used, you will be asked to lie on your back. An area of skin

on your abdomen will be cleaned with an antiseptic solution, and a sterile area prepared. You

will be given anesthetic to numb your skin. When the area is numb, a needle will be placed

through your skin and into the placenta. A small sample of the placenta will be collected and the

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needle will be withdrawn. All CVS procedures may need to be repeated to collect a sufficient

sample size.

IV. How will the test feel?

The amount of discomfort you feel will depend on many factors, including your

sensitivity to pain. Communicate how you are feeling with the person doing the test. Inform the

person doing the test if you feel that you cannot continue with the test.

A. Buccal cells:

This test usually causes no discomfort.

B. Venous blood:

During a blood draw, you may feel mild discomfort at the location where the blood

sample is being collected.

C. Chorionic villus:

During a transcervical CVS procedure, you may feel mild cramping in your abdomen or

pelvic area. Before a transabdominal or transcervical CVS procedure, a local anesthetic is given

to the procedure site to numb the area. You may feel mild discomfort or stinging when the

numbing medicine is injected. As the procedure needle or catheter is inserted through the

abdomen or cervix, you may feel some discomfort and pressure. You may feel mild cramping in

your abdomen and pelvic area during the procedure. The procedure site may be sore for several

days.

V. What should I do after the test?

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A. Venous blood:

After a blood sample is collected from your vein, a bandage, cotton ball, or gauze may be

placed on the area where the needle was inserted. You may be asked to apply pressure to the

area. Avoid strenuous exercise immediately after your blood draw. Contact your healthcare

worker if you feel pain or see redness, swelling, or discharge from the puncture site.

B. Buccal cells:

There are no special instructions to follow after this test.

C. Chorionic villus:

After all CVS procedures, ultrasound and fetal monitoring may be done immediately

after the procedure. If a needle was used, pressure may be held to the site until the bleeding or

drainage has stopped. A bandage will be placed over the site if a transabdominal method was

used. After all CVS procedures, rest is necessary. Do not have sexual intercourse, douche, and

avoid heavy lifting for at least 24 hours after all the procedures.

Contact your healthcare worker if there is redness, swelling, pus, drainage, or pain at the

procedure site if the transabdominal method was used. For all procedure methods, alert your

healthcare worker immediately should you develop a fever; bleeding (heavier than light

spotting), fluid leakage or discharge from your vagina; or severe abdominal cramping or pain.

An ultrasound is usually done 2 to 4 days after the CVS to make sure that the fetus is doing well.

VI. What are the risks?

Blood: During a blood draw, a hematoma (blood-filled bump under the skin) or slight

bleeding from the puncture site may occur. After a blood draw, a bruise or infection may occur at

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the puncture site. The person doing this test may need to perform it more than once. Talk to your

healthcare worker if you have any concerns about the risks of this test.

Buccal cells: A buccal cell collection is generally considered safe. Talk to your healthcare

worker if you have questions or concerns about the risks of this test.

Placental tissue (chorionic villus): A placental tissue sample is collected by a procedure

called chorionic villus sampling (CVS). Depending on where your placenta is located, different

methods may be used. CVS risks, depending on the method, include bleeding and infection at the

site. If you have a medical condition, or are using a medication or supplement that causes

excessive bleeding, you are at a higher risk of bleeding from the puncture site. It is possible that

the needle or catheter that is used to collect the tissue will injure the baby. You may develop a

fever, an abnormal vaginal discharge, abdominal pain or cramping, or go into labor. If there is a

possibility that you and your baby are not Rh compatible, you may need additional treatment to

avoid further complications. There is a risk that your baby will not survive the procedure, or may

be adversely affected by this procedure. It is possible that the baby’s limbs, fingers, and toes may

be affected by this procedure. The chances of these risks vary depending on your health status,

how long you have been pregnant before the CVS procedure, and other factors. The person doing

this test may need to perform it more than once. Talk with your healthcare worker if you have

any concerns about the risks of having CVS.

VII. What are normal results for this test?

Laboratory test results may vary depending on your age, gender, health history, the

method used for the test, and many other factors. If your results are different from the results

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suggested below, this may not mean that you have a disease. Contact your healthcare worker if

you have any questions. The following is considered to be a normal result for this test:

Negative

VIII. What follow up should I do after this test?

Ask your healthcare worker how you will be informed of the test results. You may be

asked to call for results, schedule an appointment to discuss results, or notified of results by mail.

Follow up care varies depending on many factors related to your test. Sometimes there is no

follow up after you have been notified of test results. At other times follow up may be suggested

or necessary. Some examples of follow up care include changes to medication or treatment plans,

referral to a specialist, more or less frequent monitoring, and additional tests or procedures. Talk

with your healthcare worker about any concerns or questions you have regarding follow up care

or instructions.

A. Chorionic villus:

After a CVS procedure, it may take from 1 to 4 weeks to receive results. There is a

possibility that you may need an amniocentesis if the CVS was not successful.

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DIFFERENTIAL DIAGNOSIS AND TREATMENT

Doctors diagnose cystic fibrosis (CF) based on the results from various tests.

I. DIAGNOSIS

A. Newborn Screening

All States screen newborns for CF using a genetic test or a blood test. The genetic test

shows whether a newborn has faulty CFTR genes. The blood test shows whether a newborn's

pancreas is working properly.

B. Sweat Test

If a genetic test or blood test suggests CF, a doctor will confirm the diagnosis using a

sweat test. This test is the most useful test for diagnosing CF. A sweat test measures the amount

of salt in sweat.

For this test, the doctor triggers sweating on a small patch of skin on an arm or leg. He or

she rubs the skin with a sweat-producing chemical and then uses an electrode to provide a mild

electrical current. This may cause a tingling or warm feeling.

Sweat is collected on a pad or paper and then analyzed. The sweat test usually is done

twice. High salt levels confirm a diagnosis of CF.

C. Other Tests

If you or your child has CF, your doctor may recommend other tests, such as:

Genetic tests to find out what type of CFTR defect is causing your CF.

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A chest x ray. This test creates pictures of the structures in your chest, such as your

heart, lungs, and blood vessels. A chest x ray can show whether your lungs are inflamed

or scarred, or whether they trap air.

A sinus x ray. This test may show signs of sinusitis, a complication of CF.

Lung function tests. These tests measure how much air you can breathe in and out, how

fast you can breathe air out, and how well your lungs deliver oxygen to your blood.

sputum culture. For this test, your doctor will take a sample of your sputum (spit) to see

whether bacteria are growing in it. If you have bacteria called mucoid Pseudomonas, you

may have more advanced CF that needs aggressive treatment.

D. Prenatal Screening

If you're pregnant, prenatal genetic tests can show whether your fetus has CF. These tests

include amniocentesis and chorionic villus sampling (CVS).

In amniocentesis, your doctor inserts a hollow needle through your abdominal wall into

your uterus. He or she removes a small amount of fluid from the sac around the baby. The fluid

is tested to see whether both of the baby's CFTR genes are normal.

In CVS, your doctor threads a thin tube through the vagina and cervix to the placenta.

The doctor removes a tissue sample from the placenta using gentle suction. The sample is tested

to see whether the baby has CF.

E. Cystic Fibrosis Carrier Testing

People who have one normal CFTR gene and one faulty CFTR gene are CF carriers. CF

carriers usually have no symptoms of CF and live normal lives. However, carriers can pass faulty

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CFTR genes on to their children. If you have a family history of CF or a partner who has CF (or

a family history of it) and you're planning a pregnancy, you may want to find out whether you're

a CF carrier. A genetics counselor can test a blood or saliva sample to find out whether you have

a faulty CF gene. This type of testing can detect faulty CF genes in 9 out of 10 cases.

II. TREATMENT

Cystic fibrosis (CF) has no cure. However, treatments have greatly improved in recent

years. The goals of CF treatment include:

Preventing and controlling lung infections

Loosening and removing thick, sticky mucus from the lungs

Preventing or treating blockages in the intestines

Providing enough nutrition

Preventing dehydration (a lack of fluid in the body)

Depending on the severity of CF, you or your child may be treated in a hospital.

A. Specialists Involved

If you or your child has CF, you may be treated by a CF specialist. This is a doctor who is

familiar with the complex nature of CF. Often, a CF specialist works with a medical team of

nurses, physical therapists, dietitians, and social workers. CF specialists often are located at

major medical centers. The United States also has more than 100 CF Care Centers. These centers

have teams of doctors, nurses, dietitians, respiratory therapists, physical therapists, and social

workers who have special training related to CF care. Most CF Care Centers have pediatric and

adult programs or clinics.

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B. Treatment for Lung Problems

The main treatments for lung problems in people who have CF are chest physical therapy

(CPT), exercise, and medicines. Your doctor also may recommend a pulmonary rehabilitation

(PR) program.

C. Chest Physical Therapy

CPT also is called chest clapping or percussion. It involves pounding your chest and back

over and over with your hands or a device to loosen the mucus from your lungs so that you can

cough it up. You might sit down or lie on your stomach with your head down while you do CPT.

Gravity and force help drain the mucus from your lungs.

Some people find CPT hard or uncomfortable to do. Several devices have been developed

that may help with CPT, such as:

An electric chest clapper, known as a mechanical percussor.

An inflatable therapy vest that uses high-frequency airwaves to force the mucus that's

deep in your lungs toward your upper airways so you can cough it up.

small, handheld device that you exhale through. The device causes vibrations that

dislodge the mucus.

A mask that creates vibrations that help break the mucus loose from your airway walls.

Breathing techniques also may help dislodge mucus so you can cough it up. These

techniques include forcing out a couple of short breaths or deeper breaths and then doing relaxed

breathing. This may help loosen the mucus in your lungs and open your airways.

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D. Exercise

Aerobic exercise that makes you breathe harder can help loosen the mucus in your

airways so you can cough it up. Exercise also helps improve your overall physical condition.

However, CF causes your sweat to become very salty. As a result, your body loses large

amounts of salt when you sweat. Thus, your doctor may recommend a high-salt diet or salt

supplements to maintain the balance of minerals in your blood.

If you exercise regularly, you may be able to cut back on your CPT. However, you

should check with your doctor first.

E. Medicines

If you have CF, you doctor may prescribe antibiotics, anti-inflammatory medicines,

bronchodilators, or mucus-thinning medicines. These medicines help treat or prevent lung

infections, reduce swelling, open up the airways, and thin mucus.

Antibiotics are the main treatment to prevent or treat lung infections. Your doctor may

prescribe oral, inhaled, or intravenous (IV) antibiotics.

Oral antibiotics often are used to treat mild lung infections. Inhaled antibiotics may be

used to prevent or control infections caused by the bacteria mucoid Pseudomonas. For severe or

hard-to-treat infections, you may be given antibiotics through an IV tube (a tube inserted into a

vein). This type of treatment may require you to stay in a hospital.

Anti-inflammatory medicines can help reduce swelling in your airways due to ongoing

infections. These medicines may be inhaled or oral.

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Bronchodilators help open the airways by relaxing the muscles around them. These

medicines are inhaled. They're often taken just before CPT to help clear mucus out of your

airways. You also may take bronchodilators before inhaling other medicines into your lungs.

Your doctor may prescribe mucus-thinning medicines to reduce the stickiness of your

mucus and loosen it up. These medicines can help clear out mucus, improve lung function, and

prevent worsening lung symptoms.

F. Treatments for Advanced Lung Disease

If you have advanced lung disease, you may need oxygen therapy. Oxygen usually is

given through nasal prongs or a mask.

If other treatments haven't worked, a lung transplant may be an option if you have severe

lung disease. A lung transplant is surgery to remove a person's diseased lung and replace it with a

healthy lung from a deceased donor.

G. Pulmonary Rehabilitation

Your doctor may recommend PR as part of your treatment plan. PR is a broad program

that helps improve the well-being of people who have chronic (ongoing) breathing problems.

PR doesn't replace medical therapy. Instead, it's used with medical therapy and may

include:

Exercise training

Nutritional counseling

Education on your lung disease or condition and how to manage it

Energy-conserving techniques

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Breathing strategies

Psychological counseling and/or group support

PR has many benefits. It can improve your ability to function and your quality of life.

The program also may help relieve your breathing problems. Even if you have advanced lung

disease, you can still benefit from PR.

H. Treatment for Digestive Problems

CF can cause many digestive problems, such as bulky stools, intestinal gas, a swollen

belly, severe constipation, and pain or discomfort. Digestive problems also can lead to poor

growth and development in children.

Nutritional therapy can improve your strength and ability to stay active. It also can

improve growth and development in children. Nutritional therapy also may make you strong

enough to resist some lung infections. A nutritionist can help you create a nutritional plan that

meets your needs.

In addition to having a well-balanced diet that's rich in calories, fat, and protein, your

nutritional therapy may include:

Oral pancreatic enzymes to help you digest fats and proteins and absorb more vitamins.

Supplements of vitamins A, D, E, and K to replace the fat-soluble vitamins that your

intestines can't absorb.

High-calorie shakes to provide you with extra nutrients.

A high-salt diet or salt supplements that you take before exercising.

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A feeding tube to give you more calories at night while you're sleeping. The tube may be

threaded through your nose and throat and into your stomach. Or, the tube may be placed

directly into your stomach through a surgically made hole. Before you go to bed each

night, you'll attach a bag with a nutritional solution to the entrance of the tube. It will feed

you while you sleep.

Other treatments for digestive problems may include enemas and mucus-thinning

medicines to treat intestinal blockages. Sometimes surgery is needed to remove an intestinal

blockage.

Your doctor also may prescribe medicines to reduce your stomach acid and help oral

pancreatic enzymes work better.

I. Treatments for Cystic Fibrosis Complications

A common complication of CF is diabetes. The type of diabetes associated with CF often

requires different treatment than other types of diabetes.

Another common CF complication is the bone-thinning disorder osteoporosis. Your

doctor may prescribe medicines that prevent your bones from losing their density.

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