CYSTIC FIBROSIS

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CYSTIC FIBROSIS
What are some of the most affective treatments?
65 Roses Not an easier way to deal with the
awful disease, just an easier way to say it...
Done by Nina Furciniti
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WHAT IS CYSTIC FIBROSIS?
Cystic Fibrosis is a genetic disease that causes
the body to produce an abnormally thick sticky
mucus, due to bad transport of sodium and
chloride within the cells lining organs such as
the lungs and pancreas. This prevents enzymes
from reaching the intestines to help break down
and digest food.
WHAT ARE SOME SYMPTOMS?

• Very salty-tasting skin
• Persistent coughing, wheezing, or pneumonia
• Excessive appetite but no weight gain
• Bulky stools

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IN THE BODy
A patient with Cystic Fibrosis develops very
thick mucus that clogs the airways to make it
difficult for a person to breath. Since this
mucus is so thick, bacteria can be brought into
the body and will stick to the walls of lungs to
do some harm. White blood cells will try to fight
it off but then only die to leave behind a trace
of DNA in the stickiness. The DNA will aggravate
the already sticky tissue to make it more
inflamed and infected to add to the blockage in
the airway. This makes the patient cough
constantly to break-up the blockage.
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How to be tested

• Sweat Tests have been done for more than 40
  years to test for CF. Any person of any age is
  acceptable for this testing although infants may
  not produce enough sweat for laboratory analyze.
  The Sweat Test will determine the amount of
  chlorine in the sweat of the person being tested.
  

During the first part of the test, a colorless
and odorless chemical that is known to cause
sweating is applied to a small area of the arm or
the leg. An electrode is the placed on the area
for 5 minutes, and an electrical current will be
sent to the skin to stimulate the sweat glands.
The second part of the test is the process of
cleaning the stimulated area and collecting the
sweat of a piece of filter paper. The specimen
will be in sent to the lab for analysis within 30
minutes. TOTAL TEST TIME 1
HOUR
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• Chest X-Rays in all forms have been used since
  1895 and it changed the practice of medicine
  because it allowed to see inside the body without
  surgery. Now more often used are CAT scans
  because its better in seeing details of organs
  and the inside of the body to diagnosis more
  injuries and illnesses. In using chest x-rays,
  Cystic Fibrosis can be seen in the lungs. The
  foggy,

light part inside the lungs (seen in picture) are
a build-up of flem and mucous which makes
breathing difficult. Most people are
apprehensive about x-rays because of the
radiation but in chest x-rays the radiation dose
is only .25 mRad, which is very small.
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• Genetic Testing is a helpful way to determine
  whether or not
• you are a carrier for the disease. Cystic
  Fibrosis is caused by mutations in the gene on
  chromosome 7 which codes for the protein that
  controls ion transfer across cell membranes.
  This makes Cystic Fibrosis the most common
  hereditary disease that leads to death in white
  people in the United States. Carrier risks vary
  by Ethnic groups
• Caucasian- 1 in 25 risk
• Ashkenazi Jemish- 1 in 25 risk
• Hispanic- 1 in 46 risk
• African American- 1 in 65 risk
• Asian- 1 in 90 risk

A cheek brush sample is the most non-invasive way
to test an adult, a sample of dried blood is used
in newborn screen testing, but prenatal testing
on a pregnant woman (picture shown) can tell if
the fetus has inherited the disease or is a
carrier. Most test are only 90 effective.
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• When the Results are in A NEGATIVE result means
  that there was nothing found that indicated a
  defect in the CF gene. But a negative result
  will still not guarantee a perfect screening.

A POSITIVE result means that there was a change
found in the CF gene. You can either have Cystic
Fibrosis which would have been detected at an
early age, or you are a carrier and you have a
50 chance that you will pass the defected gene
down to your offspring. If your partner also
carries the altered gene, the risk for you child
having Cystic fibrosis is much greater.
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OUTCOMES FROM SCREENING
These to girls have Cystic Fibrosis, but with
early detection and the proper treatments they
feel 75 better than what they would if it wasnt
caught at such an early age.
April
Kelsea
Prenatal testing for a fetus may help its life
span. By being tested at an early age, you can
detect the disease so treatments will come early
and be more helpful. This may be one answer to
premature death in patients with Cystic Fibrosis.
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Treatments for cystic fibrosis

• Pulmonary Therapy helps breathing in many ways
  like all therapies do. A team of doctors and
  respiratory therapists work with a patient of CF
  to monitor the strength of respiratory muscles so
  breathing wont become difficult. Doctors also
  use breathing devices such as

• SUCTION MACHINE TO CLEAR MOUTH AND THROAT
• NON-INVASIVE VENTILATION
• PNEUMONIA VACCINE

Pulmonary therapy also involves manual lung
clearing techniques such as

• CUPPING (percussion)
• PULMONARY DRAINING

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GOOD NUTRITION IS ESSENTIAL
Good nutrition is another way of helping symptoms
of Cystic Fibrosis. Children are most effected by
malnutrition because of the bodys difficulty of
digesting food properly.
Children with CF need the same amount of
nutritional food as any growing child, but a
child with CF needs a diet with more calories and
protein because their bodies dont absorb it as
well.

• Some ways to increase calorie and protein intake
• ADD INSTANT BREAKFAST POWDERS TO LIQUIDS
• ADD EXTRA BUTTER AND OIL TO FOODS
• EATING ICE CREAM, MILK SHAKES, AND PUDDING
• EXTRA CHEESE AND PEANUT BUTTER

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• Oral and Inhaled antibiotics are one of the most
  effective ways of opening constricted airways.
  An inhaler when inhaled relaxes the trachea so
  that air can be better brought in and out of the
  lungs. The same with an oral pill that is taken,
  but which takes longer in reaction time. This may
  not always help with CF.

In studies of the New England Journal of
Medicine shows that a chronic aerosolized therapy
of TOBI (tobramycin solution for inhalation) has
benefited patients suffering with CF.
300 milligrams of TOBI was given to patients to
inhale at home twice a day on a 28 day cycle.
After the 28 days was up, another 28 day cycle
would begin with no TOBI at all. And a process of
meds, no meds would continue 3 times. This showed
drastic improvement to breathing abilities.
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• Ibuprofen Prevents Loss of Lung Function

The anti-inflammatory over-the-counter
painkiller, Ibuprofen, has an ingredient in it
that can help loss of lung function in Cystic
Fibrosis patients. It helped the airways to the
lungs from swelling and helped breathing to be
easier. In a study, CF patients received high
doses of ibuprofen, twice a day for four years.
Patients could maintain their strength of
breathing muscles and their body weight much
better than patients that were not put on the
drug.
Advil can only reduce the severity of the
symptoms associated with CF, nothing has actually
been proven effective in actually curing the
devastating disease.
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• Postural drainage therapy is a procedure that
  are tasks such as turning, postural drainage,
  vibration, percussion and cough.
• Turning and postural drainage is almost the same
  type of therapy. They both involve moving the
  body in different positions to loosen the lung
  segments. While turning is just to break up the
  build-up of flem, postural drainage is more of a
  final step to rid the body of the unwanted flem.