cystic fibrosis - chhatrapati shahu ji maharaj...
TRANSCRIPT
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Know the clinical features of cystic
fibrosis.
Know how CF is inherited.
Be familiar with criteria to diagnose
CF.
Become aware of the myriad of
treatments used in CF.
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A multisystem disease
Autosomal recessive inheritance
Cause: mutations in the cystic fibrosis
transmembrane conductance
regulator (CFTR) gene
➢ chromosome 7
➢ codes for a c-AMP regulated chloride
channel
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Very salty-tasting skin
Appetite, but poor
growth & weight gain Coughing, wheezing ,at times
with phelgum &
shortness of breath
Lung infections, e.g.
pneumonia/bronchitis
➢ greasy, bulky stools or
difficulty in bowel movements
6
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C Chronic respiratory disease
F Failure to thrive
P Polyps
A Alkalosis, metabolic
N Neonatal intestinal obstruction
C Clubbing of fingers
R Rectal Prolapse
E Electrolyte in sweat
A Aspermia / absent vas deferens
S Sputum – S.aureus/P.aeruginosa
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CFTR controls
chloride ion
movement in
and out of the
cell.
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The CFTR gene is located on
the long arm of chromosome 7.
There are 1604 mutations in
CFTR listed on the CFTR
mutation database
The most common mutation is
Δ F508---70% CF alleles in
caucasians.
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➢ Defects in (CFTR), - encodes for a protein that functions as chloride channel & regulated by (cAMP).
➢ Abnormalities of cAMP-regulates chloride transport
➢ Defective CFTR - decreased secretion of chloride and increased re-absorption of sodium and water
➢ Reduced height of epithelial lining fluid
➢ Decreased hydration of mucus - that is stickier to bacteria
➢ Result in viscid secretions
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CF:
Clinical Signs
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Cystic fibrosis affects entire body15
• Lungs and sinuses
• GI, liver and pancreas
• Reproductive system
• Nutritional
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Endobronchial disease
➢ Cough / sputum production
➢ Air obstruction---wheezing; evidence
of obstruction on PFTs
➢ Chest x-ray anomalies
➢ Digital Clubbing
Sinus disease
➢ Nasal Polyps / sinusitis/ hemoptysis
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Hyperinflation
Peribronchial
cuffing
Bronchiectasis
Diffuse fibrosis
Atelectasis
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Mucous in the airways cannot be easily cleared from
the lungs.
Presentation of Disease inlung
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Benign lesions in nasal
airway(5-20 yrs)
If large - associated with
nasal
obstruction, drainage, heada
ches, snoring
associated with chronic
inflammation
need surgical intervention
High recurrence rate
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Intestinal abnormality➢ Meconium ileus (15-20% of newborn with CF)
➢ Distal intestinal obstruction syndrome (DIOS)
➢ Intussusception / Rectal prolapse
➢ Volvulus / atresia / meconium peritonitis
Hepatobiliary disease➢ steatorrhea
➢ Focal biliary cirrhosis
➢ Multilobular cirrhosis
Pancreatic endocrine dysfunction➢ Cystic fibrosis related diabetesCy
stic
Fib
ro
sis
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Focal inspissation ofbile
➢ Obstructs biliary ductules
Second leading cause of death inCF
Prevalence 9-37%
Spectrum of disease➢ increased liver enzymes
➢ biliary cirrhosis
➢ portal hypertension
➢ GB stones
Cy
stic
Fib
ro
sis
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Pancreatic insufficiency➢ ―cystic fibrosis of the pancreas‖---mucus
plugging of glandular ducts
➢ Chloride impermeability affects HCO3-
secretion and fluid secretion in pancreatic
ducts
Pancreatic enzymes stay in ducts and are activated
intraductally
➢ Autolysis of pancreas
➢ Inflammation, calcification, plugging of ducts, fibrosis
➢ Malabsorption
Failure to thrive
Fat soluble vitamin deficiency(ADEK)
Cy
stic
Fib
ro
sis
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Men➢ Abnormal embryologic development of the
epididymal duct and vas deferens-- incomplete
or absent
➢ Congential bilateral absence of vas deferens—
97-98% of men with CF
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Women
➢ Lower fertility rate than non-CF women
➢ Viscid mucoid cervical secretions of low
volume in women with CF
Pregnancy and CF:
➢ Goss et al, 2003---no significant difference
in survival in women who became
pregnant with CF compared to women
who did not become pregnant (after
adjusting for disease severity)➢ Fertility is mildly impaired
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CF:
Diagnostic Methods
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One or more clinical features of CF
OR
A history of CF in a sibling
OR
A positive newborn screening test
Plus
➢ Laboratory evidence for CFTR dysfunction: Two elevated sweat chloride concentrations obtained on separated days
OR
Identification of two CF mutations
OR
An abnormal nasal potential difference measurement
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First described by Gibson and Cooke,1950
➢ Chemical that stimulates sweating placed under electrode pad; saline under other electrode pad on arm
➢ Mild electric current is passed between electrodes
➢ Sweat collected(75-100gm)
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➢ Positive Sweat chloride: 60-165 meq/L
➢ Borderine sweat chloride: 40-60 meq/L
➢ Normal sweat chloride:
0-40
False positives:➢ Hypothyroidism
➢ Addison disease
➢ Ectodermal dysplasia
➢ Glycogen storage disease
➢ Edema
➢ Malnutrition
➢ Lab error (evaporation or contamination of sample)
False negatives:➢ Edema
➢ Malnutrition
➢ Some CF mutations
➢ Sample diluted
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DNA testing- 30–80 of CFTR mutations.This identifies ≥90% who carry 2 CF
mutations
increased potential differences across
nasal epithelium with reference to
forearm
loss of potential
difference with topical amiloride
application is more in CF
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Pancreatic function
➢ Fat estimation in stool
➢ OGTT
Pulmonary radiologic finding
Pulmonary function
Microbiologic studies
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➢ More than 1604 mutation identified
➢ 2 CFTR mutations in association with
symptom is diagnostic
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Goal: early diagnosis may be
associated with better nutritional
outcome
➢ Immunoreactive trypsinogen usually first followed by
either sweat or DNA testing
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CF:
Treatment
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Pulmonary Therapy
Inhalation Therapy
Airway Clearance Therapy
Infection-Antibiotic Therapy—
➢ Oral
➢ Aerosolized
➢ IV
Bronchodilator
Antiinflammatory
Endoscopy & lavage
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Nutrition➢ Diet
➢ Pancreatic enz replacement
➢ Vitamins
Gastrointestinal & Rx of complication
Infertility
Others-
➢ Nasal polyp
➢ Rhinosinusitis
➢ Salt depletion
➢ Growth & maturation
Social Issues
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Atelactasis
Hemomptysis
Pneumothorax
Allergic aspergilosis
Nontuberculous mycobactria infection
Bone & joint complication
Sleep-Disordered Breathing
Acute Respiratory Failure
Chronic Respiratory Failure
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Heart Failure
Nutritional therapy.
➢ Follow nutrition parameters closely
➢ Pancreatic enzymes
➢ Vitamin supplementation
➢ Other nutritional supplementation
➢ Tube feedings
➢ High calorie supplemental shakes,
formulas
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Meconium Ileus.
Distal Intestinal Obstruction Syndrome
Gastroesophageal Reflux
Rectal Prolapse
Heptobiliary Disease
Pancreatitis
Hyperglycemia.
Nasal Polyps
Rhinosinusitis.
Salt Depletion
Growth and Maturation Surgery.
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Follow nutrition parameters closely
Pancreatic enzymes
➢ Porcine extract, 2000U/kg/meal
➢ TheraCLEC-Total, micro derived under
Vitamin supplementation-➢ ADEK
Other nutritional supplementation➢ Tube feedings
➢ High calorie supplemental shakes, form
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Microsurgical epididymal sperm aspiration coupled plus in vitro
technology
Percutaneous epididymal sperm
aspiration
Testicular sperm extraction
Maternal genetic testing
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Quality of life
➢ Frequent hospitalizations
➢ Time spent on therapies
➢ Morbidity from disease
➢ Restrictions secondary to disease
Adherence to therapies
Family planning
End of lifeissues
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Mucolytic-
➢ Pulmozyme -to thin mucus
➢ Dornase alfa- enzyme hydrolyse DNA-
improve airway clearance
Antibiotics- Inhaled TOBI
➢ Cayston- Aztreonam, monobactem AB
inhaled for p. aeruginosa
Antioxidant
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ACTs loosen thick, sticky lung mucus➢ move mucus from small to large airways to be coughed or huffed
out.
Coughing is the most basic ACT.
Huffing is a type of cough. involves taking a
breath in and actively exhaling. It is more like
―huffing‖ onto a mirror or window to steam it up
Chest Physio Therapy
Oscillating Positive Expiratory Pressure (Oscillating
PEP) an ACT where the person blows all the way out
many times through a device named
FlutterTM, AcapellaTM, CornetTM and Intrapulmonary
Percussive Ventilation (IPV). Breathing with devices
vibrate & dislodge.
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High-frequency Chest Wall Oscillation
Positive Expiratory Pressure (PEP)
Active Cycle of Breathing Technique
(ACBT) It gets air behind
mucus, lowers airway spasm and clears
mucus.
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Thoracic expansion exercises – deep breaths in.
➢ done with chest clapping or vibrating,
followed by breathing control.
Forced expiration technique – huffs of varied
lengths with breathing control.
Autogenic Drainage (AD) means ―self-
drainage.‖
➢ uses varied airflows to move mucus.
➢ aims to reach very high airflows in different
lung parts. This moves mucus from small to
large airways.
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Lung Transplant
➢ 900 LT /year in USA
➢ 1600 received LT from 1991
➢ 2003– 17 pts received from living donor & 126 from cadaveric lung transplant
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Gene therapy is the use of normal DNA to "correct" for the damaged genes that cause disease.
In the case of CF, gene therapy involves inhaling a spray that delivers normal DNA to the lungs.
The goal is to replace the defective CF gene in the lungs to cure CF or slow the progression of the disease.
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ThankYou!