1. Define cystic fibrosis
- Cystic fibrosis (CF) is an autosomal recessive disorder which causes
mucus to build up in the lungs, pancreas, and other organs.
2. Describe the most common populations affected by this disease, including age,
gender, and ethnicity
- CF is most common in the US Caucasian population, it is diagnosed in
newborns and infants, about 1 in 2,500 newborns are affected. The
disease affects both girls and boys equally. CF is not as prevalent in other
races including African Americans, and American Indians.
3. This disease is an autosomal recessive disorder affecting the CFTR gene on
chromosome 7. What does this mean? Describe what is currently understood about
the genetic characteristics of this disease.
- the CFTR gene is involved with managing chloride channels that
transport negatively charged chloride ions into and out of the cells. The
mutation on chromosome 7 causes the CFTR gene to create a disruption
of chloride ions preventing them from being able to cross cell
membranes. This causes cells that line the pathways of the lungs,
pancreas, and digestive track causing mucus to become thick and sticky
as opposed to the usual slippery and wet consistency. This disease is
autosomal recessive which means that both parents must have a copy of
the mutated gene
4. How is this disease diagnosed? List at least three methods that are used
- Newborn screening test (IRT): needs to be within 90 days
- Sweat chloride test
- The presence of two disease causing mutations in the CFTR gene- absent
or defective function of CFTR
5. For each of the following organs or organ systems, describe the most common
physical changes that occur as a result of the abnormality of the CFTR gene. Explain
how these changes may affect Lily’s nutritional status.
a. Respiratory: the thick mucus can cause blockage to the airways of the lung,
causing chronic coughing, wheezing, exertion dyspnea. Often CF patients
experience pneumonia which can lead to chronic endobronchial infection
and lung infection. Chronic lung problems can cause structural damage to
the airways which then lead to cyst and abbess which then lead to lung
disease. Obstruction in the airway causes decreased appetite and decreased
oral intake. Chronic coughing prevents adequate energy intake, but the
energy requirements are actually increased.
b. Reproductive: for males, the vas deferens are altered and epididimis and
seminal vesicle may be altered or absent causing the majority of males
suffering from CF to be infertile. For females the cervical mucus may be
altered which would result in infertility. For Lily, she needs maintain proper
medication to keep the correct electrolyte balance to prevent infertility.
c. Pancreatic: the thick mucus can cause blockage in the pancreatic ducts which
would then prevent insulin from being produced, and not allow digestive
enzymes to aid in digestion and break down fat, protein, and carbohydrates.
For Lily, it might affect nutrient malabsorption related to inadequate weight
maintenance and may lead to weight loss. It may also affect her growth, and
she may be at a greater risk for diabetes. It may affect steatorrhea so she
must make sure she is taking a pancreatic enzyme. Failure to take this would
result in steatorrhea, and chronic steatorrhea can lead to hyperproteinemia,
edema, and severe muscle wasting. However, Lily has been preventing this
by taking her Pancrease.
d. Gastrointestinal: linings to the gut may be obstructed which causes
malabsorption and loss of nutrients. The gastrointestinal and pancreatic
changes are closely related. For Lily, if the GI tract is blocked then she will
not be able to properly absorb the nutrients. Malabsorption will lead to FTT,
poor weight maintenance leading to weight loss, and steatorrhea.
6. Lily was admitted and diagnosed with bacterial pneumonia. Why is this the most
common hospitalization for patients with CF? Explain.
- The problems with the lungs in patients with CF are difficult to treat with
medication. Since patients with CF have lower airway inflammation and
the lung defenses are lowered the thickened mucus makes it difficult for
the patient to cough out any pathogens which leads to lung infection, the
most common being bacterial pneumonia. Patients with CF are immunocompromised which makes raises their risk for any infection.
7. What are the most common nutritional consequences of cystic fibrosis?
- Since the pancreas can be affected by CF it makes it difficult to get enough
protein, fat, fat-soluble vitamins, and calories in the body. The thickened
mucus prevents any of the nutrients from being absorbed.
8. Describe the major modifications for carbohydrate, protein, and fat intake that
would be needed as components of nutrition therapy for CF
- Lily must be on a high-calorie, high-protein diet. Her energy intake is 120150% higher than the recommended daily intake. Her protein intake is 1.5-2
g/kg, and fat should be between 35-40% of her daily calorie intake. There
are no major modifications for carbohydrates.
9. Is Lily at risk for electrolyte imbalances? Specifically, address her sodium and
chloride requirements? Is there additional information from Lily’s history that puts
her at risk for changes in her sodium and chloride levels?
- Patients with CF are at risk for electrolyte imbalance to the mutated CFTR
gene causing chloride ion abnormality. Lily is at the normal range for her
electrolytes. Her temperature was slightly elevated due to an infection,
and her activity level may put her at a higher risk due to loss of
electrolytes in sweating. One of the side effects of Pancrease is electrolyte
imbalance, however Lily’s lab show she is at a normal level.
10. What is pancrease? Lily mentioned that she did not know how much to take. What
are the recommendations?
- Pancrease is a pancreatic enzyme to help the body absorb fat ad protein
and should be taken with all meals and snacks. Because Lily has been
seeing a drop in weight, she should increase the amount of pancrease she
is taking with meals.
- Enzyme dosage: 500 unites lipase/kg/meal=23,180 unites lipase/meal.
Pancrease comes in 4000, 10000, 16,000, and 20,000 unites lipase
capsules so Lily will want to be taking 2-3 (10,000 unit) capsules at every
meal.
- If Lily has been using the maximum dosage and still seems symptomatic,
she should try switching her brand to try Creon, and also look at nonadherence and understand if she is taking it with every snack/drink/etc.
11. Assess Lily’s weight and height. Plot her height and weight on the appropriate
growth chart. Calculate her BMI. Calculate her %UBW. Explain what each of the
assessments provides any why one or more provides the most relevant information
for Lily.
- BMI: 17 (underweight) between 10th and 25th percentile for her age
- UBW: 110-115 (3 months ago)
- %UBW: 102/110 x 100= 92.7%
- 11.3% loss in 3 months – severe weight loss
- Lily’s BMI is a good indicator for her general age and gender, but it does
not take into account her CF. It is recommended to have an increased BMI
percentile for patients with CF. %UBW is a better indicator to tell us if
the BMI is normal, it gives more specific information relevant to her
current condition.
12. Determine Lily’s energy and protein requirements. You see that she typically runs
5-7 miles 3-4 times per week as well as taking a dance class 3 times per week for 1
hour. Make sure this is taken into consideration when calculating her energy
requirements. Your recommendations for Lily should include the appropriate
macro- and micronutrients based on the requirements for adolescent with cystic
fibrosis.
- 3062-3533 kcal/day (based on 120-150% increase)
- Protein: 1.5-2 x DRI= 59-78 g/day
- Fat: 35-40% calories= 1071-1413 kcal from fat/day
- Carbohydrate: 265-283 g/d (regular DRI)
13. Analyze Lily’s nutritional intake according to the usual dietary intake. Attach your
computerized analysis for this assessment.
14. Compare your analysis to her estimated nutritional needs
-Protein: According to the nutrient analysis of the 24 hour food recall,
Lily is consuming 55g, which is 4 grams short of the 59-78 g/day which we
had calculated. Therefore she is not quite meeting her needs
- Fat: According to our nutrient intake Lily is consuming 56% of the
calories from fat. This is a lot higher than the 35-40% which we have
prescribed earlier.
- Calories: According to our nutrient intake Lily is consuming 1669
calories, which is much lower than she should be consuming.
- Vitamin A: she is consuming 308 ug RAE which is less than half of the 700
ug which is needed for her diet
- Vitamin D: according to the nutrient intake Lily is not consuming any
Vitamin D
- Iron: According to the nutrient intake Lily is consuming 7mg of Iron
which is less than half of the 15 mg recommended.
- Zinc: According to the nutrient intake Lily is consuming 5 mg of Zinc,
when her recommended intake should be 9 mg
- Calcium: According to the nutrient intake Lily is consuming only 219 mg
when she should be consuming 1300 mg
15. Identify 3 specific vitamins and minerals that are needed in increased amounts
during adolescence. Explain why they are of special important for an adolescent.
Will Lily’s CF affect the metabolism of these nutrients? Do Lily’s diet history and 24hour recall indicate that she consumes adequate amounts of these nutrients?
- Vitamin A: is important because of its role in the maintenance of mucus
secreting epithethial cells, low Vitamin A levels are associated with a
worsened clinical status, impaired lung function, lower weight status, and
decreased bone mineral density. No she is consuming less than half of the
recommended intake
- Vitamin D: it is especially important because deficiency is common among
patients with CF. Vitamin D deficiency may cause rickets and
osteoporosis. According to the nutrient intake Lily is not getting any
vitamin D
- Iron: Iron is important in adolescences especially females due to the
onset of menstruation. According to the nutrient intake, Lily is
consuming a little less than half of her recommended intake of 15 mg.
16. From the information gathered within the intake domain, list possible nutrition
problems using the diagnostic term
- inadequate energy intake ( NI 1.4)
- Inadequate oral intake (NI 2.1)
- Increased nutrient needs due to adolescence and CF (NI 5.1)
o Specifically vitamin A, D, iron and calcium
- Excessive fat intake (NI 5.6.2)
- Inadequate protein intake (NI 5.7.1)
- Excessive carbohydrate intake (NI 5.8.2)
-
-
Inadequate vitamin intake (NI 5.9.1)
o A, D, thiamin, riboflavin, B12, folate
Inadequate mineral intake (NI 5.10.1)
o Calcium, potassium, copper, iron, magnesium, phosphorous, zinc
Altered GI function due to CF (NC 1.4)
Impaired nutrient utilization (NC 2.1)
Food-medication interaction vs. predicted food-medication interaction (
NC 2.3)
o Pancrease (fat and protein digestion)
o Prevacid (decreases acid, problems with digestion vitamin
suppressing interaction)
Excessive physical activity (NB 2.2)
Underweight (NC 3.1)
Unintentional weight loss (NC 3.2)
Food and nutrition related deficit (ND 1.1)
Altered nutrition related laboratory values (N.C. 2.2)
17. After reading the physician’s history and physical, identify the signs and symptoms
that are consistent with Lily’s admitting medical diagnosis
- recent unintentional weight loss- approximately 10# in the last 3 months
- cold in the past two weeks
- elevated temperature of 99.1 F
- pharynx reddened with postnasal drainage
- decreased breath sounds, percussion hyperresonant, rhonchi and rales
present
- Skin pale
- Increased WBC – sign of infection, labs indicate 13, elevated from normal
values of 4.8-11.8
18. Evaluate each of the medications that Lily takes as an outpatient. Determine the
function of each medication and identify any nutritional implications
Medication
Pancrease
Function
helps to improve the digestion of foods
and prevent frequent, fatty, foulsmelling bowel movements. Pancrease
down fats, proteins, starches into
smaller parts that are more easily
digested.
Prilosec/Prevacid treats and prevents stomach ulcers
cause by bacteria, decreases the amount
of acid made in the stomach to prevent
damage to the stomach or intestine
lining.
Humabid
Relieves coughing and mucus in the
Nutritional Implication
Disruptive absorption. May
impair absorption of iron
and folic acid
Affects absorption of betacarotene, iron, vitamin B12
No known drug interactions
Multivitamin
Proventil
chest, helps to thin the mucus making it
less sticky and easier to cough up to
reduce congestion
Help reach vitamin/mineral needs
Prevents and treats wheezing, difficulty
breathing, and chest tightness caused
by lung disease such as asthma and
COPD. Relaxes and opens the air
passages to the lungs to make breathing
easier
Exceeding vitamin or
mineral values can cause
toxicity, which can lead to a
variety of different health
issues
No known interactions
19. Evaluate Lily’s laboratory values. In the following table, list any laboratory values
that are abnormal. What is the most probably cause of the abnormality?
Abnormal Normal
Reason for
Nutritional Implication
Lab
Value
Abnormality
WBC 13
4.8-11.8
Infection, decreased
Need for increased caloric intake,
(high)
immunity
Vitamin C, and fluids
HGB 11.5
12-15 g/dl
pneumonia infection,
increased needs for iron, protein
(low)
chance of anemia,
and Vitamin B
inflamed pancreatic
tissue, may be due to
medication interaction
HCT 33%
37-47%
Low Hgb, pneumonia
Increased needs for iron, protein,
(low)
infection, chance of
and vitamin B. Poor oxygen
anemia, inflamed
transport in the body.
pancreatic tissue,
predicted medication
interaction
Ferritin 19 20-120 mg/l
Low iron, predicted
Increase iron intake, could cause
mg/l
medicaton interaction
iron deficiency anemia,
(low)
pneumonia can cause decreased
ferritin levels- competing for
increased needs due to infection
Transferrin 250-380
Inflammation due to
Iron cannot be transported into
219
pneumonia and
cells which may lead to anemia
(low)
predicted medication
interaction
HbA1c
6.3%
(high)
3.9-5.2%
Magnesium 1.8-3.0 mg/dl
1.6 (low)
Too much sugar in the
blood due to deficient
insulin response from
impaired pancreas
functioning
Pancreatic insufficiency,
may be due to prevacid
medication
Chronic high blood sugar levels
could cause damage to body and
cells leading to diabetes. Extra
circulating glucose expels the
extra sugar into urine causing
frequent urination. If body can’t
absorb glucose into cells, the
body loses significant source of
calories.
Blocks body’s use of calcium.
Increase calcium needs, Mg
deficiency can lead to
hypokalemia, hypocalcemia,
hypoparthyroidusm and
decreased calcitriol
20. List possible nutrition problems within the clinical domain using the diagnostic
term
- Impaired nutrient utilization (NC 2.1)
- Altered GI function due to CF (NC 1.4)
- Food-medication interaction vs. predicted food-medication interaction (
NC 2.3)
- Underweight (NC 3.1)
- Altered nutrition related laboratory values (N.C. 2.2)
- Unintentional weight loss (NC 3.2)
21. After reading the history and physical as well as the nutrition history, identify
factors that may impact the success of Lily’s current medical and nutritional care for
her cystic fibrosis
- Lack of nutritional knowledge and adherence – implement nutrition
education
- Lack of knowledge of foods that affect and promote medication
- Lack of proper use of medication
- Continue to monitor how much pancrease she is taking and what works
best
- Ensure she is taking multivitamin every day
- Meal changes: Increase nutrient dense foods, implement breakfast and
snacks throughout the day, pre and post workout foods, increase small
meals throughout the day
- Monitor weight and ensure she is maintaining proper weight
22. Select two high-priority nutrition problems and complete the PES statements
- Involuntary weight loss related to CF and pneumonia as evidenced by
10# weight loss in the last three months
- Increased iron needs related to medication interaction as evidenced by
abnormal transferrin, ferritin, HGB, HCT levels.
23. For each of the PES statements that you have written, establish an ideal goal (based
on the signs and symptoms) and an appropriate intervention (based on the etiology)
- Gain 1-2# each week until reach UBW of 110-115# will do this by
modifying distribution, type, or amount of foods and nutrients within
meals or at specified time (N.D-1.2). Serve small, frequent meals.
- Serve commercial beverage (N.D -3.1.1) such as Ensure or Boost to reach
adequate caloric needs
- Increase iron levels to average levels of 20-120 mg/ml by increasing
specific foods/beverages or groups (N.D.-1.3) foods that are iron-rich,
also take mineral, iron (N.D. 3.2.4) and vitamin B12 (N.D. 3.2.3)