2. CYSTIC FIBROSIS (CF)
Cystic fibrosis is a genetic condition. It's caused by a gene mutation in [cystic fibrosis
transmembrane conductance regulatory gene CFTR/ chromosome no.7].
The gene mutation affects the movement of salt and water in and out of cells.
This, along with recurrent infections, can result in a build-up of thick, sticky mucus in
the body's tubes and passageways – particularly the lungs and digestive system.
• Inherited autosomal recessive disorder
• CF incidence of 1:2500 live births
• Survival is improving; median age of patients has exceeded 30 years
• Epithelial cells and exocrine glands secrete abnormal mucus (thick)
• Affects respiratory tract, sweat, salivary, intestine, pancreas, liver, reproductive tract
5. Symptoms
• The dysfunctional CFTR protein channel is found all over GI tract
• It causes elevated Sweat- Electrolyte balance.
• A persistent cough that produces thick mucus (sputum)
• Wheezing
• Exercise intolerance
• Repeated lung infections
• Inflamed nasal passages or a stuffy nose
• Recurrent sinusitis
6. DIAGNOSIS OF CYSTIC FIBROSIS
• Neonatal screening provides opportunity to prevent malnutrition in CF
infants
• Sweat test (Na and Cl >60 mEq /L)
• Chronic lung disease
• Malabsorption
• Family history
7. Complications of CF
• Damaged airways
• Coughing up blood (hemoptysis)
• Chronic infections
• Pneumothorax
• Respiratory failure
8. NUTRITIONAL IMPLICATIONS OF CF
• Infants born with meconium ileus are highly likely to have CF.
• 85% of persons with CF have pancreatic insufficiency Plugs of mucus
reduce the digestive enzymes released from the pancreas causing mal-
digestion of food and malabsorption of nutrients.
• Decreased bicarbonate secretion reduces digestive enzyme activity.
• Decreased bile acid reabsorption contributes to fat malabsorption.
• Excessive mucus lining in the GI tract prevents nutrient absorption by
the microvilli.
9. GASTROINTESTINAL COMPLICATIONS
• Bulky, foul-smelling stools
• Cramping and intestinal obstruction
• Pancreatic damage causes impaired glucose tolerance
• (50% of adults with CF) and development of diabetes
10. COMMON TREATMENTS
Pancreatic enzyme replacement
• Adjust macronutrients for symptoms
• Nutrients for growth
• Meconium ileus causes the intestinal obstruction So, enzymes, fiber, fluids, exercise,
stool softeners are given.
Digestive Enzymes:
• Enteric-coated enzyme microspheres withstand acidic environment of the stomach,
they release enzymes in the duodenum, where they digest protein, fat and
carbohydrate.
11. NUTRITIONAL CARE GOALS
• Control malabsorption.
• Provide adequate nutrients for growth or maintain weight for
height or pulmonary function.
• Prevent nutritional deficiencies.
12. In children energy requirement should be based on the weigh gain and
growth.
Energy need for CF children without ventilation are comparable to
healthy children (100 – 110% of RDA).
In case of significant lung disease, malabsorption, ER increase 120 -
150% of RDA
Medical Nutrition Therapy
13. Protein Requirement:
Protein needs are increased in CF due to malabsorption
If energy needs are met, protein needs are usually met by
following 15-20% protein or use RDA
Or 2.2/kg b/w
14. Fat Requirement:
• Fat intake 35-40% of calories (in fat malabsorption), as tolerated
• Helps provide required energy, essential fatty acids and fat-soluble
vitamins.
• Limits volume of food needed to meet energy demands and improves
palatability of the diet
• Essential Fatty Acids deficiency sometimes occurs in CF patients
despite intake and pancreatic enzymes.
• Fat restriction is not recommended
• Important energy sources, fat used = MCT oil, fish oil.
15. CHO Requirement :
• Eventually intake may need to be modified if glucose intolerance
develops.
• Some patients develop lactose intolerance.
Vitamins requirement:
• With pancreatic enzymes, water soluble vitamins usually adequately
absorbed with daily multivitamin will need high potency
supplementation of fat soluble vitamins (A, D, K, E)
16. FEEDING STRATEGIES: INFANTS
• Breast feeding with supplements of high-calorie formulas and
pancreatic enzymes.
• Calorie dense infant formulas (20-27 kcals/oz) with enzymes.
• Protein hydrolysate formulas with MCT oil if needed.
17. FEEDING STRATEGIES: CHILDREN
ANDADULTS
• Regular mealtimes
• Large portions
• Extra snacks
• Nutrient-dense foods
• Nocturnal enteral feedings
• Intact or hydrolyzed formulas
• Add enzyme powder to feeding or take before and during
18. PNEUMONIA
It is a infection in the lungs caused by the microbe invasion in lungs. It causes
inflammation of air sacs.
Bacterial Pneumonia
• Streptococcus Pneumoniae
• Mycoplasma Pneumoniae
• Staphylococcus Aureus
Viral Pneumonia
• Haemophilus Influenzae
22. Hospital Acquired
Pneumonia
(Nosocomial)
• A person gets it when he
is admitted in the hospital
with any other disease
condition.
• Microbes in hospitals are
more antibiotic resistant.
• Example: MRSA
Community Acquired
Pneumonia
• A person gets it outside a
hospital setting, either from
the environment.
• The infection causes
deterioration of lung
resulting in fluid
accumulation and breathing
difficulties.
Types and Occurrence of Pneumonia
23. Ventilator Acquired
Pneumonia
• It is a subset of
Hospital acquired
pneumonia.
• It happens when a
person is on ventilator.
Aspiration Pneumonia
• Aspiration is when a Food,
Drugs, Gastric Content
passes into the trachea.
• Material like, saliva, nasal
secretion, bacteria, foods.
• The food items have
microorganisms on it that
causes infection which
ultimately leads to
pneumonia.
26. NUTRITION IMPLICATION
Patients admitted to the hospital due to CAP (community acquired
Pneumonia), most important risk factor associated to mortality.
• Low serum albumin (<3.0 g/dl)
• The depress albumin is associated to the inflammation response rather
than malnutrition.
Other indices of poor nutrition status associate to death are low triceps
skinfold (TSF) and low BMI.
27. Medical Nutrition Therapy
• Energy required is based on the underlying diseases (often hypermetabolic/ Elevate
Resting Energy Expenditure), 1.2 – 1.4 x BEE.
REMEMBER do not over feed the patient.
• Overfeeding Increase CO2 production.
• The provision 25 kcal/kg appears to be adequate to most pt.
• Protein requirement: 1.2 – 1.5 g/kg.
• CHO may be given according to pt. needs.
• Fluid balanced should be monitor closely as pulmonary effusion can occur.
• Pulmonary edema, the use of fluid restricted enteral formulation (1.5 – 2 kcal/cc)
may be helpful, for those need for fluid restriction.
• Supplementation of potassium, calcium, and magnesium may be administered as
these are lost in the urine.
28. • Among the pulmonary infections with nutritional implications is pneumonia.
The role of vitamin A in treating pneumonia yields some possible results.
• Because of their role in inflammation and immunity, epidemiologic researches
investigating the role of EFAs. Such research shows a possible protective effect
against pneumonia by the ingestion of a-linolenic and linoleic acids.
• Pneumonia usually occurs as a nosocomial infection or as a consequences of
aspiration of food, fluid, or secretions such as saliva.
• Optimal nutrition status and proper feeding techniques aid in preventing this
pulmonary infection. Aspiration is common in infants, children, and adults who
are frail, have frequent coughing spasms, are unable to effectively chew or
swallow their foods and beverages or have inadequate head and neck control
during eating.
29. RESPIRATORY FAILURE
In respiratory failure, the gas exchange between the air and circulating blood is severely impaired,
resulting in abnormal levels of tissue gases that can be life threatening.
The normal value of arterial partial pressure of oxygen and carbon dioxide is,
PAO2: 10.6 -13.3kPa
PACO2: 4.7 -6.0 kPa
Hypoxemia: PAO2: < 10.6 kPa
Hypercapnia: PACO2: >6 Kpa
Respiratory failure
PAO2: <8kPa
PACO2: >6.7 kPa
30. Causes Of RF:
Any of a large number of conditions that cause lung injury or impair lung function can be the
underlying cause of failure; examples include infection (such as pneumonia or sepsis), physical
trauma, neuromuscular disorders, smoke inhalation, and airway obstruction.
Occur when the respiratory system is no longer able to perform its normal function.
It result from long standing chronic lung disease like COPD, CF or as a result of an acute abuse
to the lung such as acute respiratory distress syndrome (ARDS).
Categories of acute respiratory distress syndrome:
• Directly cause injured to the lung e.g. Pneumonia, aspiration or inhalation injury.
• Indirectly cause injury precipitated by event outside the lung e.g. sepsis, trauma, or
pancreatitis.
31. Medical Nutrition Therapy
• Energy required is based on the underlying diseases (often hypermetabolic) 1.2 – 1.4
x BEE
* REMEMBER do not over feed the patient as it increases CO2 production
• Increase ventilator demand associated with overfeeding;
• Excess glucose administration (>5 mg/kg per min).
• The provision 25 kcal/kg appears to be adequate to most patient
• Fluid balanced should be monitor closely
• Protein requirement: 1.2 – 1.5 g/kg (to promote nitrogen retention without being
excessive)
• Pulmonary edema, the use of fluid restricted enteral formulation (1.5 – 2 kcal/cc)may
be helpful, for those need for fluid restriction.
32. •Acute respiratory distress is associate with production of oxygen free radical and
inflammatory mediators, recent study shows:
• EPA in fish oil and GLA in borage oil can reduce the severity of inflammatory injury.
• High level of antioxidant: α-tocopherol, β-carotene and vit C at higher level than
DRI
• Increase serum α-tocopherol, β-carotene & prevent further oxidative damage.
• Phosphate is essential for optimal pulmonary function and normal diaphragm
contraction.
• Hypophosphatemia increase hospital stay and dependence to ventilation. Need to
monitor phosphate and supplementation should be initiated in hypophosphatemia
37. Nutritional factors that increase risk of TB:
• Many patients are developing drug-resistant TB
• Nutritional factors that increase risk of TB:
• Protein-energy malnutrition: affects the immune system;
debate whether it is a cause or consequence of the disease
• Micronutrient deficiencies that affect immune function
(vitamin D, A, C, iron, zinc)
38. Nutritional consequences:
• Increased energy expenditure
• Loss of appetite and body weight
• Increase in protein catabolism leading to muscle breakdown
• Malabsorption causing diarrhea, loss of fluids, electrolytes
39. Medical Nutrition Therapy
• Energy: 35-40 kcals/kg of ideal body weight
• Protein: 1.2-1.5 grams/kg body weight, or 15% of energy or 75-100
grams/day
• Multivitamin-mineral supplement at 100-150% DRI.
• Provide energy dense foods to overcome overfeeding.
• Provide colorful fruits and vegetables rich in antioxidants.
• Foods rich in Vitamin A, C and E and Zinc.
CFTR gene codes CFTR protein – which is a chloride channel – It help the ions to move in and out.
Epithelial cells are the cells that covers inside and outside surface of body
Inflammation and infection of sinus. As it effects the Epithelium layer in upper respiratory tract.
The mucous blocks the digestive enzymes from reaching the intestines. So Fats and proteins are not digested properly. Pancreas and Intestines cant work properly
Whistling sounds while breathing
The dark green substance forming the first faeces of a newborn infant.
Meconium ileus is a bowel obstruction that occurs when the meconium in your child's intestine is even thicker and stickier than normal meconium, creating a blockage in a part of the small intestine called the ileum. Most infants with meconium ileus have a disease called cystic fibrosis.
Fat soluble vitamins.
Inflammatory response– WBS, protein, fluid and WBC
Inflammation can cause fluid retention in alveoli
Alveoli are tiny air sacks in grape like structure that has capillaries in it. The gas exchange occurs in alveoli.
With the air we inhale certain microorganisms. Mucociliary escalator has tiny villi that structure that prevents the micrograms. Macrophages engulfs the microorganisms.
sthethoscope
Protein needs are increased in patients with lung inflammation or Acute Respiratory Distress Syndrome.
For mild or moderate lung injury, protein recommendations range from 1.0 to
1.5 grams of protein per kilogram body weight per day
a-linolenic and linoleic acids is present in sunflower seeds, nuts, soybean, canola oil.
EFAs [essential fatty acids]
The rate of oxygen diffusion across the alveoli capillary membrane with respect to elimination of co2
Movement of substance from higher concentration to a lower conc.
ARTERIAL BLOOD GAS TEST
Protein needs are increased in patients with lung inflammation or ARDS.
For mild or moderate lung injury, protein recommendations range from 1.0 to
1.5 grams of protein per kilogram body weight per day
Gama Linoleic acid
Goazban
Alpha tocopherol: prevents blood clotting, anti oxidant [free radical damage]
Beta carotene : Anti oxidant