introduction, etiology, clinical features, investigations, management of cystic fibrosis. physiotherapy management for cystic fibrosis.

1. CYSTIC FIBROSIS
DR.T.SUNIL KUMAR

2. • CYSTIC FIBROSIS (CF) IS THE MOST COMMON,
AUTOSOMAL RECESSIVE DISORDER, WITH THE BASIC
DEFECT IN THE GENE LOCATED ON THE LONG ARM OF
CHROMOSOME 7, WHICH RESULTS IN THE DEFICIENCY OF
CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE
REGULATORY PROTEIN (CFTCR).
• CFTCR IS A CHLORIDE CHANNEL ACTIVATOR, ACTIVATED
BY A COMBINATION OF PHOSPHORYLATION OF PROTEIN
KINASE ‘A’ AND BINDING OF ATP.
• THIS GENETIC DEFECT RESULTS IN A REDUCTION IN THE
MOVEMENT OF IONS IN AND OUT OF CELL AND A
REDUCTION IN THE AMOUNT OF WATER IN THE
SECRETIONS.

3. • IN LUNGS, CF AIRWAY EPITHELIA EXHIBIT BOTH
INCREASED TRANSPORT RATES FOR NA+ AND
DECREASED ION PERMEABILITY FOR CL–; THERE
IS RAISED TRANSEPITHELIAL ELECTRIC
POTENTIAL DIFFERENCE.
• CF EPITHELIA DO NOT RESPOND TO Β AGONISTS
OR AGONISTS OF PROTEIN KINASE C, WITH
CHLORIDE SECRETION AS NORMAL AIRWAY
EPITHELIA DO. THERE IS HYPER ABSORPTION OF
NA+.
• CF IS A MULTI-SYSTEM DISEASE INVOLVING LUNG,
PANCREAS, SWEAT GLANDS, AND UROGENITAL
TRACT.

4. ETIOLOGY AND PATHOGENESIS
• IN CYSTIC FIBROSIS, THE AIRWAY EPITHELIUM
SHOWS A COMBINATION OF DEFECTIVE
CHLORIDE SECRETION AND INCREASED SODIUM
ABSORPTION THAT LEADS TO CHANGES IN THE
COMPOSITION OF THE AIRWAY SURFACE LIQUID,
AND PREDISPOSES THE LUNG TO CHRONIC
PULMONARY INFECTIONS AND BRONCHIECTASIS.

5. • THE GENE FOR CYSTIC FIBROSIS IS LOCATED ON
THE LONG ARM OF CHROMOSOME 7 AND
ENCODES THE CYSTIC FIBROSIS
TRANSMEMBRANE CONDUCTANCE REGULATOR
(CFTR), AN AMINO ACID PROTEIN FOUND IN
VARIOUS CELL TYPES, INCLUDING LUNG
EPITHELIUM, SUBMUCOSAL GLANDS, PANCREAS,
LIVER, SWEAT DUCTS AND REPRODUCTIVE
TRACT.
• THE HIGH SWEAT SODIUM AND CHLORIDE
RESULTS FROM DEFECTIVE ION ABSORPTION
ALONG THE SWEAT DUCTS WHICH ARE
IMPERMEABLE TO WATER.

6. CLINICAL FEATURES
RESPIRATORY/CARDIOVASCULAR
A. BRONCHITIS, BRONCHOPNEUMONIA,
BRONCHIECTASIS, LUNG ABSCESS.
B. ATELECTASIS
C. SINUSITIS, NASAL POLYPOSIS
D. PULMONARY HYPERTENSION
E. COR PULMONALE AND CONGESTIVE HEART
FAILURE
F. HAEMOPTYSIS
G. PNEUMOTHORAX
H. RESPIRATORY FAILURE.

7. REPRODUCTIVE SYSTEM
A. MALES: STERILITY: ABSENT OR DEFECTIVE VAS
DEFERENS, EPIDIDYMIS AND SEMINAL VESICLES (IN ABOUT
99% OF MALES).
B. FEMALES: DECREASED FERTILITY
SKELETAL
A. RETARDATION OF BONE AGE
B. DEMINERALISATION
C. HYPERTROPHIC OSTEOARTHROPATHY.
OTHERS
A. SALT DEPLETION
B. HEAT STROKE
C. SALIVARY GLAND HYPERTROPHY
D. RETINAL HAEMORRHAGE
E. HYPERTROPHY OF APOCRINE GLANDS.

8. COMPLICATIONS
1. RECURRENT RESPIRATORY INFECTIONS
(PSEUDOMONAS, STAPHYLOCOCCI,
BURKHOLDERIA AND H. INFLUENZAE).
2. PNEUMOTHORAX
3. MASSIVE HAEMOPTYSIS
4. RESPIRATORY FAILURE
5. COR PULMONALE

9. DIFFERENTIAL DIAGNOSIS
1. PRIMARY CILIARY DYSKINESIA MAY LEAD TO
BRONCHIECTASIS, SINUSITIS, AND INFERTILITY.
SWEAT CHLORIDE VALUE IS NORMAL.
2. SHWACHMAN SYNDROME – PANCREATIC
INSUFFICIENCY, CYCLICNEUTROPENIA AND LUNG
DISEASE MAY SIMULATE CF, BUT SWEAT
CHLORIDE VALUE IS NORMAL.
3. YOUNG SYNDROME (BRONCHIECTASIS,
SINUSITIS AND AZOOSPERMIA) IN MEN LACKS GI
SYMPTOMS AND HAS NORMAL SWEAT CHLORIDE
LEVELS.

10. INVESTIGATIONS
• GENE MUTATIONS – ALL PATIENTS MUST BE SCREENED
FOR KNOWN COMMON CYSTIC FIBROSIS GENE
MUTATIONS.
• SWEAT TEST – BOTH CHLORIDE AND SODIUM SWEAT
CONCENTRATIONS ARE > 60 MMOL/L, CHLORIDE MORE
THAN SODIUM (SWEAT IS COLLECTED USING
PILOCARPINE DIRECTED TO THE SWEAT GLANDS BY
IONTOPHORESIS). ELEVATED SWEAT CHLORIDE LEVEL IS
PATHOGNOMONIC OF CYSTIC FIBROSIS AND LEVELS > 70
MEQ/L DIFFERENTIATES BETWEEN CYSTIC FIBROSIS AND
OTHER LUNG DISEASES.
• THE NASAL PD MEASUREMENT CAN DOCUMENT CFTR
DYSFUNCTION IF THE SWEAT CHLORIDE TEST IS NORMAL
OR BORDERLINE AND TWO CF MUTATIONS ARE NOT
IDENTIFIED.

11. • ASSESSMENT OF EXOCRINE PANCREAS – FAECAL
ELASTASE IS A USEFUL SCREENING TEST FOR
PANCREATIC DAMAGE. LEVELS ARE LOW.
• LUNG FUNCTION – DETERIORATES WITH DISEASE
PROGRESSION BY ABOUT 3% PER YEAR. THE PICTURE IS
TYPICALLY OBSTRUCTIVE.
• CHEST RADIOGRAPH – ACCENTUATED BRONCHIAL
MARKINGS, SMALL RING SHADOWS, NODULAR SHADOWS
AND MORE EXTENSIVE CONFLUENT CONSOLIDATION ARE
CHARACTERISTIC FINDINGS.
• GUTHRIE TEST – FOR NEONATAL DIAGNOSIS. A HEEL
PRICK BLOOD TEST FOR TRYPSIN LEAKING FROM THE
PANCREAS INTO THE BLOOD. IMMUNOREACTIVE TRYPSIN
LEVELS ARE INCREASED BY 2–3 TIMES.

12. MANAGEMENT
ANTIBIOTICS – ORAL OR IV DEPENDING ON THE ORGANISM.
IT IS NECESSARY TO CLEAR LUNG OF SECRETIONS FOR
WHICH CHEST PERCUSSION THERAPY AND INHALATION OF
HYPERTONIC SALINE IS ADVOCATED.
RECOMBINANT HUMAN DNASE (RHDNASE) –NEBULISED
ONCE DAILY TO BREAK UP LONG STRAINS OF DNA
RELEASED BY DEGRADING LEUCOCYTES MAKING
SECRETIONS VISCOUS. TREATMENT ALSO DECREASES
FREQUENCY OF RESPIRATORY EXACERBATIONS.
ANTI-INFLAMMATORY AGENTS – TO CONTROL CHRONIC
INFLAMMATION IN THE LUNGS – CORTICOSTEROIDS AND
NSAIDS.

13. IMMUNIZATION – PATIENTS SHOULD BE GIVEN USUAL
CHILDHOOD IMMUNIZATIONS.
NUTRITION – HIGH-CALORIE ORAL SUPPLEMENTATION WITH
FAT-SOLUBLE VITAMINS A, D AND E.
PANCREATIC ENZYMES – DISTAL INTESTINAL OBSTRUCTION
SYNDROME OFTEN RESULTS FROM INADEQUATE
PANCREATIC SUPPLEMENTATION.
TREATMENT OF LIVER DISEASE – ORAL
URSODEOXYCHOLIC ACID WITH OR WITHOUT TAURINE.
TRANSPLANTATION – SEQUENTIAL SINGLE-LUNG OR
HEART-LUNG TRANSPLANTATION IS AN OPTION IN END-
STAGE CYSTIC FIBROSIS.

15. PRINCIPLES OF PHYSICAL THERAPY
MANAGEMENT
THE GOALS OF LONG-TERM MANAGEMENT OF THE PATIENT
WITH CYSTIC FIBROSIS INCLUDE THE FOLLOWING:
• • MAXIMIZE THE PATIENT'S QUALITY OF LIFE, GENERAL
HEALTH AND WELL-BEING, GROWTH AND DEVELOPMENT,
AND PHYSIOLOGICAL RESERVE CAPACITY
• • EDUCATE THE PATIENT AND FAMILY ABOUT CYSTIC
FIBROSIS, SELF-MANAGEMENT, NUTRITION, PREVENTION
OF ACUTE EXACERBATIONS OF THE DISEASE, INFECTION
CONTROL, AND MEDICATION'S USES, MODES OF
ADMINISTRATION, MUCOKINETICS AND TIMES TO PEAK
EFFICACIES.
• • FACILITATE MUCOCILIARY TRANSPORT
• • OPTIMIZE SECRETION CLEARANCE

16. • OPTIMIZE ALVEOLAR VENTILATION
• OPTIMIZE LUNG VOLUMES AND CAPACITIES AND FLOW RATES
• OPTIMIZE VENTILATION AND PERFUSION MATCHING
• REDUCE THE WORK OF BREATHING
• REDUCE THE WORK OF AND STRAIN ON THE HEART
• MAXIMIZE AEROBIC CAPACITY AND EFFICIENCY OF OXYGEN
TRANSP0RT
• OPTIMIZE PHYSICAL ENDURANCE AND EXERCISE CAPACITY
• OPTIMIZE GENERAL MUSCLE STRENGTH AND THEREBY
PERIPHERAL OXYGEN EXTRACTION

17. PHYSIOTHERAPY MANAGEMENT
• THE PRESENTING PROBLEMS OF EACH PATIENT WILL VARY,
AND WILL FLUCTUATE FROM A CHRONIC STABLE STATE TO AN
ACUTE CHANGING STATE.
• AN EXACERBATION OF A BRONCHOPULMONARY INFECTION
WILL PRODUCE CHANGES WHICH CAN BE DETECTED BY
ACCURATE ASSESSMENT OF THE SIGNS AND SYMPTOMS.
• PHYSIOTHERAPY MAY HELP IN THE TREATMENT OF PATIENTS'
PROBLEMS OF EXCESS BRONCHIAL SECRETIONS, REDUCED
EXERCISE TOLERANCE, BREATHLESSNESS AND CHEST WALL
STIFFNESS AND PAIN OF MUSCULOSKELETAL ORIGIN.
• ARTHROPATHY, UNSTABLE DIABETES AND THE ABDOMINAL
PAIN OF MECONIUM ILEUS EQUIVALENTARE EXAMPLES OF
MEDICAL PROBLEMS WHICH WILL AFFECT THE
PHYSIOTHERAPIST'S TREATMENT PLAN.

18. • PATIENT MONITORING INCLUDES DYSPNEA,
RESPIRATORY DISTRESS, BREATHING PATTERN
(DEPTH AND FREQUENCY), ARTERIAL
SATURATION, CYANOSIS (A DELAYED SIGN OF
DESATURATION), HEART RATE, BLOOD
PRESSURE, AND RATE PRESSURE PRODUCT.
• PATIENTS WITH CARDIAC DYSFUNCTION OR LOW
ARTERIAL OXYGEN TENSIONS REQUIRE ECG
MONITORING, PARTICULARLY DURING EXERCISE.
• SUBJECTIVELY, BREATHLESSNESS IS ASSESSED
USING A MODIFIED VERSION OF THE BORG
SCALE OF PERCEIVED EXERTION.

19. EXCESS BRONCHIAL SECRETIONS
• WITH CYSTIC FIBROSIS, BRONCHIAL SECRETIONS MAY BE
MINIMAL OR COPIOUS. THE INFANT AT THE TIME OF DIAGNOSIS
MAY BE ASYMPTOMATIC, BUT THERE IS EVIDENCE OF
INFLAMMATION AND INFECTION IN THE LUNGS.
• MOST PAEDIATRICIANS RECOMMEND INTRODUCING
PHYSIOTHERAPY AT THIS TIME IN AN ATTEMPT TO DELAY THE
DESTRUCTIVE PROCESS OF INFECTION AND FIBROSIS.
• IF PHYSIOTHERAPY BECOMES AN ACCEPTED PART OF LIFE,
COMPLIANCE WILL PROBABLY BE BETTER THAN IF
PHYSIOTHERAPY IS INTRODUCED AT A LATER STAGE.
• A CLOSE BONDING USUALLY DEVELOPS BETWEEN THE
PARENTS AND THE CHILD. IT IS IMPORTANT THAT BOTH
PARENTS ARE INVOLVED AND THAT THE SIBLINGS ARE
INCLUDED IN THE CARE OF THE AFFECTED CHILD SO THAT THEY
DO NOT FEEL LEFT OUT

20. • THE TECHNIQUES OF POSITIONING, CHEST CLAPPING AND
CHEST VIBRATIONS WILL ASSIST THE MOBILIZATION OF
SECRETIONS AND STIMULATE COUGHING.
• FOR THE INFANT, CHEST CLAPPING IS PERFORMED USING
THE FIRST THREE FINGERS OF ONE HAND WITH THE
MIDDLE FINGER SLIGHTLY ELEVATED AND SHOULD ALWAYS
BE DONE OVER A LAYER OF CLOTHING.
• TREATMENT SHOULD BE UNDERTAKEN BEFORE FEEDS AND
PROBABLY FOR 5-10 MINUTES TWICE A DAY, FOR EXAMPLE
SITTING UP AND THEN POSITIONING THE INFANT FOR THE
LOWER LOBE AND MIDDLE ZONE OF EITHER THE RIGHT OR
LEFT LUNG, DIE OTHER SIDE BEING TREATED DURING THE
SECOND SESSION OF THE DAY.

21. • INFANTS WITH CYSTIC FIBROSIS HAVE A HIGHER
INCIDENCE OF GASTRO-OESOPHAGEAL REFLUX (GOR), BUT
THERE IS CONFLICTING EVIDENCE AS TO WHETHER THIS IS
EXACERBATED BY POSITIONING WITH PHYSIOTHERAPY,
ESPECIALLY THE HEAD-DOWN POSITION.
• OWING TO THE EFFECT OF GRAVITY IN THE TIPPED
POSITION, INTRA-ABDOMINAL PRESSURE WILL BE AT ITS
LOWEST AND MERE WILL BE AN INCREASE IN INTRA-
THORACIC PRESSURE.
• THIS TOGETHER WITH AN INCREASE IN DIAPHRAGMATIC
ACTIVITY MAY ENHANCE THE COMPETENCE OF THE
OESOPHAGEAL SPHINCTER.
• WHEN AN INCREASE IN GOR IS SUSPECTED THE EFFECT
OF POSITIONING MUST BE ASSESSED. ANTI-REFLUX
MEDICATION MAY BE PRESCRIBED.

22. • IF AN INFANT OR CHILD HAS SPECIFIC RADIOLOGICAL
SIGNS, CHEST CLAPPING IN THE APPROPRIATE GRAVITY
ASSISTED POSITIONS SHOULD BE USED.
• TREATMENTS MAY NEED TO BE MORE FREQUENT AND, IF
TOLERATED, OF SLIGHTLY LONGER DURATION.
• TREATMENT EVEN AT A YOUNG AGE SHOULD BE FUN.
• THE YOUNG CHILD CAN BE BOUNCED UP AND DOWN ON
HIS PARENT'S KNEES AND ANOTHER EXERCISE WHICH IS
FUN FOR THE FAMILY IS 'WHEELBARROWS'.
• LAUGHING WILL ALSO STIMULATE COUGHING AND THE
MINI TRAMPOLINE CAN BE INTRODUCED

25. • FROM THE AGE OF 2 YEARS, 'HUFFING' GAMES CAN BE
STARTED, FOR EXAMPLE BLOWING PIECES OF COTTON WOOL
OR TISSUE USING A TUBE IN THE MOUTH.
• THE WHOLE FAMILY CAN BE INVOLVED IN THESE GAMES.
• FROM 2-3 YEARS OF AGE THE CHILD CAN BE ENCOURAGED
TO TAKE DEEP BREATHS DURING THE PERIODS OF CHEST
CLAPPING, BUT THIS SHOULD BE FOR NO MORE THAN THREE
OR FOUR BREATHS BEFORE PAUSING FOR A PERIOD OF
BREATHING CONTROL.
• THIS IS THE INTRODUCTION OF THE ACTIVE CYCLE OF
BREATHING TECHNIQUES.
• AT THIS STAGE IF THE HEADDOWN POSITION IS INDICATED
THE CHILD CAN LIE OVER A WEDGE OF FOAM OR PILLOWS.

28. • INFANTS AND SMALL CHILDREN SWALLOW THEIR
BRONCHIAL SECRETIONS, BUT AS SOON AS
POSSIBLE EXPECTORATION SHOULD BE
ENCOURAGED.
• NASOPHARYNGEAL SUCTION SHOULD ONLY BE
USED IF IT IS ESSENTIAL TO OBTAIN A SPUTUM
SPECIMEN OR IF THE INFANT IS DISTRESSED BY THE
SECRETIONS.
• LEARNING TO BLOW THE NOSE IS IMPORTANT TO
KEEP THE UPPER AIRWAYS CLEAR.
• BY THE AGE OF 8 OR 9 YEARS THE CHILD CAN BEGIN
TO DO SOME OF THE TREATMENT HIMSELF AND
GRADUALLY LEARN TO BE INDEPENDENT OF HIS
PARENTS FOR PERIODS OF TIME.

29. • MOST ADOLESCENTS PREFER TO TAKE
RESPONSIBILITY FOR THEIR OWN PHYSIOTHERAPY,
BUT ASSISTANCE WITH TREATMENT IS OFTEN
APPROPRIATE DURING AN EXACERBATION OF
INFECTION AND FOR PATIENTS WHO ARE TOO FRAIL
TO MANAGE ON THEIR OWN. SOMETIMES PATIENTS
HAVE A PREFERENCE FOR ASSISTANCE.
• MANY PATIENTS WITH CYSTIC FIBROSIS HAVE A
MARKED DEGREE OF AIRFLOW OBSTRUCTION AND
TO CLEAR SECRETIONS EFFECTIVELY, SUFFICIENTLY
LONG PERIODS OF BREAMING CONTROL NEED TO BE
EMPHASIZED.

30. • PAROXYSMS OF COUGHING ARE EXHAUSTING
AND INEFFECTIVE. THEY CAN BE MINIMIZED BY
ADAPTING THE LENGTH OF THE HUFF AND
USING BREATHING CONTROL. WHEN CONTROL
IS GAINED, ONE OR TWO HUFFS COMBINED
WITH BREATHING CONTROL WILL BE MORE
EFFECTIVE THAN COUGHING IN THE
CLEARANCE OF SECRETIONS.
• THE PHYSIOTHERAPIST SHOULD BE INVOLVED
WITH THE CHANGES IN TECHNIQUES FROM
INFANCY TO ADULTHOOD.

31. • ASSESSMENT AND REASSESSMENT OF THE
PATIENT'S CONDITION ARE ESSENTIAL FOR THE
NECESSARY CHANGES IN TREATMENT TO BE
RECOGNIZED AND RECOMMENDED.
• THE FREQUENCY AND DURATION OF TREATMENT
WILL VARY. WHEN SECRETIONS ARE MINIMAL,
TREATMENT ONCE A DAY MAY BE SUFFICIENT BUT
ADDITIONALLY SOME FORM OF EXERCISE SHOULD
BE ENCOURAGED.
• MANY PATIENTS WILL REQUIRE TREATMENT TWO OR
THREE TIMES A DAY, BUT THE PROGRAMME SHOULD
BE REALISTIC AND ALLOW FOR OTHER NORMAL
ACTIVITIES.

32. • TREATMENT IS USUALLY MORE EFFECTIVE IF NO
MORE THAN THREE POSITIONS ARE USED, AS A
MINIMUM OF 10 MINUTES IN ANY ONE
PRODUCTIVE POSITION IS RECOMMENDED.
• ALTHOUGH THE CAUSE IS UNKNOWN, THE UPPER
LOBES ARE FREQUENTLY THE MOST SEVERELY
AFFECTED AND IT IS IMPORTANT TO CONSIDER
THE ANTERIOR AND POSTERIOR SEGMENTS OF
THE UPPER LOBES WHEN ASSESSING THE
PATIENT AND PLANNING TREATMENT.

33. • ON OCCASIONS DEVICES SUCH AS POSITIVE
EXPIRATORY PRESSURE (PEP), THE FLUTTER OR
MECHANICAL OSCILLATORS AND PERCUSSORS MAY
INCREASE ADHERENCE TO TREATMENT. AUTOGENIC
DRAINAGE IS WIDELY USED IN SOME COUNTRIES.
• THESE REGIMENS HAVE BEEN DEVELOPED IN
DIFFERENT PARTS OF THE WORLD THAT DOES NOT
REQUIRE ASSISTANCE AND THEREBY IMPROVES
PATIENT COMPLIANCE.
• MANY OF THESE REGIMENS NOW INCLUDE THE
FORCED EXPIRATION TECHNIQUE FROM THE ACTIVE
CYCLE OF BREATHING TECHNIQUES AND THIS HAS
INCREASED THE EFFECTIVENESS OF THESE
REGIMENS.

34. • INHALATION OF DRUGS. BRONCHODILATOR DRUGS
MAY BE PRESCRIBED AND THESE SHOULD BE
INHALED BEFORE TREATMENT TO CLEAR
SECRETIONS.
• IN SOME PATIENTS THE AIRFLOW OBSTRUCTION IS
PARTIALLY REVERSIBLE WITH BRONCHODILATORS
ANOTHER POSSIBLE EFFECT OF ADRENERGIC
DRUGS IS AN INCREASE IN DUAL ACTION AND THIS
MAY IMPROVE MUCOCILIARY CLEARANCE.
• NORMAL SALINE (0.9%) OR HYPERTONIC SALINE (3-
7%) MAY BE INHALED BEFORE PHYSIOTHERAPY TO
ASSIST IN CLEARANCE OF SECRETIONS.

35. • IF HYPERTONIC SALINE IS USED A TEST DOSE
SHOULD BE GIVEN WITH RECORDINGS OF PEF OR
FEV, BEFORE AND 5 MINUTES AFTER INHALATION TO
IDENTIFY ANY INCREASE IN AIRFLOW OBSTRUCTION.
• MUCOLYTIC AGENTS, FOR EXAMPLE
ACETYLCYSTEINE, REDUCE MUCUS VISCOSITY. THEY
SHOULD BE USED WITH CAUTION AS
BRONCHOSPASM MAY BE INDUCED.
• AEROSOL ANTIBIOTICS SHOULD BE INHALED AFTER
SECRETIONS HAVE BEEN DEARED. SPIROMETRY IS
NECESSARY BEFORE AND AFTER THE INITIAL DOSE T
DETECT ANY INCREASE IN AIRFLOW OBSTRUCTION.

36. • IF THIS SHOULD OCCUR THE EFFECT IS
USUALLY MINIMIZED BY THE INHALATION OF A
BRONCHODILATOR BEFORE TREATMENT.
• ACUTE EXACERBATION OF A
BRONCHOPULMONARY INFECTION. SIGNS OF
AN ACUTE EXACERBATION INCLUDE AN
INCREASE IN THE VOLUME AND PURULENCE OF
SPUTUM, BREATHLESSNESS, FEVER, A
DETERIORATION IN LUNG FUNCTION, POSSIBLE
PLEURITIC CHEST PAIN AND A REDUCTION IN
EXERCISE TOLERANCE.

37. • AN INCREASE IN THE DURATION AND
FREQUENCY OF PHYSIOTHERAPY TREATMENTS
WILL BE INDICATED AND THE PATIENT WILL
REQUIRE ASSISTANCE WITH CHEST CLAPPING,
SHAKING AND COMPRESSION.
• THE PAUSES FOR BREATHING CONTROL MAY
NEED TO BE LENGTHENED AND TREATMENT
SHOULD BE DISCONTINUED BEFORE THE
PATIENT BECOMES TOO TIRED. IT MAY NOT BE
POSSIBLE TO REACH THE 'END-POINT’ OF
TREATMENT AT THIS STAGE.

38. • IF OXYGEN THERAPY HAS BEEN PRESCRIBED THIS
SHOULD BE CONTINUED THROUGHOUT TREATMENT
WHEN SECRETIONS ARE VERY TENACIOUS
HUMIDIFICATION SHOULD BE CONSIDERED, EITHER
CONTINUOUSLY WITH OXYGEN THERAPY OR FOR 10-
15 MINUTES BEFORE PHYSIOTHERAPY.
• INTERMITTENT POSITIVE PRESSURE BREATHING
(IPPB) MAY BE INDICATED TO REDUCE THE WORK OF
BREATHING AND ASSIST IN THE CLEARANCE OF
SECRETIONS.
• IF THE PATIENT HAS HAD A RECENT PNEUMOTHORAX
OR A HISTORY OF RECURRENT PNEUMOTHORACES
IPPB IS PROBABLY CONTRA INDICATED.

39. MAINTENANCE/INCREASE IN EXERCISE
TOLERANCE
• EXERCISE SHOULD PLAY AN IMPORTANT PART IN THE
MANAGEMENT OF CYSTIC FIBROSIS THROUGH ALL
STAGES OF THE DISEASE TO IMPROVE GENERAL
PHYSICAL FITNESS AND MUSCLE STRENGTH.
• IT HAS BEEN SHOWN TO IMPROVE
CARDIOPULMONARY FITNESS AND MUSCLE
ENDURANCE, TO REDUCE BREATHLESSNESS AND TO
IMPROVE SELF-ESTEEM AND PROMOTE A FEELING
OF WELL-BEING.
• EXERCISE INCREASES MUCOCILIARY CLEARANCE,
BUT IT IS LESS EFFECTIVE IN THE CLEARANCE OF
BRONCHIAL SECRETIONS THAN THE ACTIVE CYCLE
OF BREATHING TECHNIQUES.

40. • FROM THE TIME OF DIAGNOSIS, EXERCISE SHOULD
BE AN INTEGRAL PART OF THE MANAGEMENT. THE
FAMILY SHOULD BE ENCOURAGED TO TAKE UP SOME
FORM OF EXERCISE THAT THEY WILL ALL ENJOY.
• CHILDREN SHOULD TAKE PART IN NORMAL SCHOOL
GAMES WHEN POSSIBLE AND ADULTS SHOULD BE
ENCOURAGED TO TAKE SOME FORM OF ENJOYABLE
EXERCISE REGULARLY.
• IN THE WINTER MONTHS WHEN OUTDOOR SPORTS
MAY NOT BE APPROPRIATE A STATIONARY BICYCLE
IS OFTEN USEFUL TO PROVIDE A PROGRESSIVE
EXERCISE PROGRAMME.

41. • EXERCISE CAPACITY CAN BE ASSESSED AND
MONITORED BY MEASURING MAXIMUM OXYGEN
UPTAKE (V02MAX) USING A BICYCLE ERGOMETER
TEST.
• A PROGRESSIVE EXERCISE PROGRAMME SHOULD
BE BASED ON A WORKLOAD TO ACHIEVE 50-60% OF
VTXMAX.
• IF FORMAL EXERCISE TESTING IS NOT AVAILABLE,
50% OF PEAK WORK CAPACITY (PWC) CAN BE USED
AS THE STARTING POINT FOR EXERCISE.

42. • THE PWC (IN WATTS (W)) CAN BE CALCULATED
USING A BICYCLE ERGOMETER. THE PATIENT STARTS
CYCLING AT A LOW WATTAGE, FOR EXAMPLE 10-25 W
AND THIS IS INCREASED BY 10-25 W EACH MINUTE
UNTIL THE PATIENT CAN CYCLE NO FURTHER.
• IN PATIENTS WITH CYSTIC FIBROSIS THE LIMITING
FACTOR TO EXERCISE MAY BE EITHER
BREATHLESSNESS OR MUSCLE FATIGUE.
• THE WORKLOAD REACHED IN THIS TEST
APPROXIMATES THE PWC. OXYGEN SATURATION
AND HEART RATE SHOULD BE MONITORED.
• MANY OF THESE PATIENTS WILL TOLERATE A LOW
OXYGEN SATURATION (SAQ>).

43. • IN PATIENTS WITH ADVANCED PULMONARY
DISEASE WHO DESATURATE DURING EXERCISE,
SUPPLEMENTAL OXYGEN HAS BEEN SHOWN TO
INCREASE EXERCISE TOLERANCE AND AEROBIC
CAPACITY, AND IT CAN REDUCE EXERCISE-
RELATED ARTERIAL OXYGEN DESATURATION.
• THE LONG-TERM EFFECTS OF OXYGEN
DESATURATION AND THEREFORE THE PLACE OF
OXYGEN THERAPY IN CHRONIC PULMONARY
DISEASE REMAINS CONTROVERSIAL.

44. • POSTURE AND TRUNK MOBILITY EXERCISES SHOULD
BE ENCOURAGED TO TRY TO MAINTAIN FLEXIBILITY
OF THE THORACIC CAGE.
• EXERCISE PROGRAMMES SHOULD COMBINE
ENDURANCE EXERCISES FOR AEROBIC FITNESS AND
MUSCLE STRENGTHENING EXERCISES.
• EXERCISE SHOULD BE DISCONTINUED IF A PATIENT
DEVELOPS A FEVER AS HIS METABOLIC
REQUIREMENTS WILL BE INCREASED DURING THIS
PERIOD. HOWEVER, IF CONFINED TO BED MUSCLE
STRENGTHENING EXERCISES ARE IMPORTANT.

45. • A PATIENT WHO HAS EXERCISE-INDUCED ASTHMA
SHOULD REMEMBER TO INHALE HIS
BRONCHODILATOR BEFORE STARTING EXERCISE.
WHEN EXERCISING A PATIENT WHO HAS A SMALL
PNEUMOTHORAX, OR FOLLOWING A RECENT
PNEUMOTHORAX OR HAEMOPTYSIS, THE
PHYSIOTHERAPIST SHOULD MONITOR THE SIGNS
AND SYMPTOMS DURING AN EXERCISE SESSION.
• IN THE OCCASIONAL PATIENT WITH
OSTEOARTHROPATHY EXERCISE MAY BE
CONTRAINDICATED DURING A PERIOD OF ACUTE
JOINT INVOLVEMENT.

46. • THE PATIENT WITH DIABETES SHOULD MAINTAIN AN
ADEQUATE SUGAR LEVEL DURING INCREASED
PHYSICAL ACTIVITY AND A SWEET DRINK OR BISCUIT
BEFORE EXERCISE MAY BE ALL THAT IS REQUIRED.
• SALT DEPLETION MAY OCCUR IF EXERCISING IN HOT
WEATHER OR WHEN IN A HOT CLIMATE, AND SALT
SUPPLEMENTS MAY BE NEEDED.
• SOME PATIENTS EXERCISE AFTER POSTURAL
DRAINAGE, EITHER BECAUSE THEY ARE TOO
BREATHLESS TO EXERCISE UNTIL THEY HAVE
CLEARED THEIR SECRETIONS OR BECAUSE THEY
FIND IT MORE SOCIALLY ACCEPTABLE TO BE
COUGHING LESS WHILE PARTICIPATING IN SOCIAL
SPORTS.

47. BREATHLESSNESS
• THE USE OF BREATHING CONTROL WHILE WALKING
UP STAIRS AND HILLS SHOULD INTRODUCED WHEN
BREATHLESSNESS ON EXERTION BECOMES
NOTICEABLE.
• AN IRRITABLE COUGH AT NIGHT OR
BREATHLESSNESS MAY BE MINIMIZED BY THE BE
USE OF THE HIGH SIDE LYING POSITION AND OTHER
REST POSITIONS ARE OFTEN OF VALUE TO REDUCE
BREATHLESSNESS.
• FOR EXAMPLE: RELAXED SITTING, FORWARD LEAN
SITTING, RELAXED STANDING AND FORWARD LEAN
STANDING.

48. CHEST WALL STIFFNESS AND PAIN OF
MUSCULOSKELETAL ORIGIN
• ALTERATIONS IN CHEST WALL MECHANICS
PROBABLY DEVELOP AS A CONSEQUENCE OF AN
INCREASE IN THE WORK OF BREATHING AND
HYPERINFLATION OF THE CHEST LEADING TO A
SHORTENING OF THE ACCESSORY MUSCLES OF
RESPIRATION.
• MANUAL THERAPY TECHNIQUES MAY INCREASE
THORACIC MOBILITY IN PATIENTS WITH CYSTIC
FIBROSIS AND MAY IMPROVE LUNG FUNCTION.

49. • EDUCATION FOCUSES ON TEACHING
PREVENTATIVE HEALTH PRACTICES AND
INFECTION CONTROL (E.G., COLD AND FLU,
AEROBIC EXERCISE, NUTRITION, HYDRATION,
RELAXATION, STRESS MANAGEMENT, ACTIVITY
PACING, AND ENERGY CONSERVATION).

50. THANK YOU