SLEEP, LUNG FUNCTION AND EXERCISE

Adrian H Kendrick, PhD

Sleep Breathing Disorders Service

Department of Respiratory Medicine

University Hospitals, Bristol, UK

NORMAL SLEEP

& what disturbs it!

WHAT IS SLEEP?

Sleep is -

➢ A reversible state of perceptual disengagement from and unresponsiveness to the environment

➢ A complex mix of physiological and behavioural processes

➢ Usually, but not always accompanied by postural recumbency, closed eyes etc

NORMAL SLEEP

Non-Dreaming sleep (NREM)

➢ Normal sleep length varies from individual

to individual

➢ Accounts for 75% of the night

➢ About 25% of sleep is “deep” (N3) sleep

➢ Required for normal daily functioning

➢ Observed in the early part of the nights

sleep

-

Dreaming Sleep (REM)

➢ Accounts for about 25% of a normal sleep

pattern

➢ Cycles every 90 to 120 mins

➢ Increases in duration during the night

➢ Results in relaxed muscles during the

night - prevents you acting out your

dreams!

➢ Not essential for normal daily functioning

SLEEP DISTURBANCES - CAUSES

EFFECTS OF SLEEP DISTURBANCE

▪ Daytime drowsiness

▪ Micro sleeps

▪ Sleep seizures

▪ Mood shifts

▪ Stress / Anxiety

▪ Lethargy

▪ Reduced productivity

▪ Reduced concentration

▪ Reduced short-term memory

▪ Reduced creativity

RETT SYNDROME

BREATHING WHEN ASLEEP

➢ May be normal during sleep, but stopping breathing (apnoea)

is observed in many patients

➢ Shallow breathing, breath-holding and central apnoeas may

lead to severe hypoxia, which may lead to seizures

Rett syndrome – National Best Practice 2013

IMPAIRED SLEEP

➢ Night Terrors

➢ Inappropriate laughing and jerking

➢ Initiating sleep may be difficult due to -

❖ Seizures

❖ Irregular breathing

❖ General restlessness

Rett syndrome – National Best Practice 2013

ASSESSMENT

EVENTS AND SYMPTOMS

Sleep-wake patterns

Day-time

activities

and symptoms

Pre-sleep

activities and

symptoms

Events during

sleep

Events on

awakening

SLEEP

12:00 00:00 12:00

ASSESSMENT PROCEDURES

• Good clinical history – parents and carers are key here

• Bring video recordings of patient awake and sleep to show their breathing

• Overnight oximetry as baseline study

• Limited or Full Polysomnography• Limited - Assesses breathing patterns, leg movements

• Full - Sleep and Breathing etc

• Actigraph Studies • Continuous 24/7 recordings of daytime activity and night-time sleep

over a period of time under everyday conditions

SUBJECTIVE ASSESSMENT

Sleep history➢ Sleepiness and/or snoring

➢ Situations patient feels sleepy in and falls asleep in

➢ Sleep initiation

➢ Refreshing sleep? – Daytime functioning

➢ Medications

EPWORTH SLEEPINESS SCORE

Situation Chance of Dozing

Sitting and Reading

Watching TV

Sitting Inactive in a public place (theatre or meeting)

As a passenger in a car for an hour without a break

Lying down to rest in the afternoon when circumstances

permit

Sitting and talking to someone

Sitting quietly after lunch without alcohol

In a car, while stopped for a few minutes in the traffic

Score –

0 for no chance

1 for slight chance

2 for moderate chance

3 for high chance

EPWORTH SLEEPINESS SCORE

PULSE OXIMETRY

➢ Non-invasive method to assess sleep-breathing issues

➢ Measures pulse (heart) rate and oxygen saturation

➢ Provides a simple screening tool for oxygen levels

OVERNIGHT SLEEP STUDY

Oxygen

Levels

Heart

Rate

OVERNIGHT SLEEP STUDIES

RECORDING

REM RELATED ISSUES

ACTIGRAPHY

➢ Simple, easy to use wrist mounted device

➢ Monitors activity and may take into account light patterns etc.

➢ Allows monitoring of activity over 22 – 30 days continuously

➢ Software allows assessment of daytime activity, nap periods and sleep

➢ Reviews sleep-wake patterns and activity during periods of sleep

ACTIGRAPHY

LUNG IMAGING

• CT scans provide good images of lung structure

• Changes in lung structure may be reflected in the ability to exchange gas from the lungs to the blood

• Damage to lungs in younger patients may affect breathing in later life

FLYING

• Patients with known lung problems may have problems flying as blood oxygen may fall during flight

• In the plane at cruising altitude, less oxygen than when on the tarmac

• Simple test breathing 15% oxygen will suggest whether oxygen in-flight is needed to complete the journey

TREATMENT

TREATMENT

➢ Depends on assessment outcome

➢ Discussion of options between clinical team and patient/carer

➢ Viability of treatment

➢ Ability of patient and carer to manage treatment at home

➢ May include –➢ Drug therapy

➢ CPAP

➢ Non-Invasive Ventilation (NIV)

TREATMENT – CPAP OR NIV

• Controls breathing problems when asleep

• Improves quality of sleep overall, leading to

improvements in daytime functioning

• Sophisticated devices now allow more precise

control of breathing patterns

• Modem technology allows assessment of usage

and can be linked to oximetry data

Pre + Post CPAP

CPAP OR NIV

➢ Treatment helps to stabilize the breathing at night

➢ Reduces the numbers of apnoeas (obstructive and central) occurring

➢ Better controls carbon dioxide and oxygen levels

➢ May reduce observed seizures in some patients

➢ Increases quality of sleep

➢ Should improve daytime functioning

➢ Less moody, less grumpiness – happier overall!

REBREATHING MASK

➢Better controls hyperventilation by

increasing dead-space breathing,

raising PCO2

➢May reduce “seizures” by

maintaining raised PCO2 and

reducing decreases in PO2

➢Appears to be tolerated

LUNG FUNCTION & EXERCISE

LUNG IMAGING

Lung high-resolution CT imaging features of respiratory bronchiolitis-associated

interstitial lung disease in patients with Rett syndrome. Peribronchial wall thickening

(A), centrilobular nodules (B), GGOs (C, D, and E), and bronchiolectasis (F) are shown.

GGOs = ground-glass opacities.

Claudio De Felice et al (2010)

• Assessed lung pathology using HRCT (n = 27)

• 15/27 patients had abnormal images

• The implications of these results need further investigation

LUNG GAS EXCHANGE?

Claudio De Felice et al (2014)

• Assessed gas exchange using a complex model.

• 81% (n = 228) had gas exchange issues

• Observed 4 variations of breathing (V) to blood flow (Q) -

• A – Low V/Q (35%)

• B – High V/Q (40%)

• C – Mixed V/Q (20%)

• D – Simple Mismatch (5%)

A B

C D

EXERCISE

• Exercise is a key component of everyday fitness

• Daily physical activity aimed at people with disabilities has gained increasing popularity, except for people with Rett syndrome who are still not fully included in this exercise

RETT & TREADMILL USE

RETT & TREADMILL USE

• Participants with Rett syndrome reacted in the same manner as the healthy controls when walking on a treadmill continuously at individual’s maximum sustainable speed for up to six minutes

• There is no reason to exclude persons with Rett syndrome from reasonably self-tailored physical activities

FUNCTIONAL CAPACITY

• This is key in determining preoperative risk

• Metabolic equivalents (METs) where –

1 MET = basal metabolic rate = 3.5 mL/min/kg

BENEFITS OF EXERCISE

• Improved conditioning allows individuals to be healthier overall

• Improvement in quality of life status

• Helps maintain weight at an appropriate level for individual

• Pre-operative fitness for surgery is important for post-operative management and outcomes

SUMMARY

• A high proportion of RTT patients have significantly disturbed

breathing patterns in the daytime and during the night

• Night-time disturbance may have a significant impact on sleep

quality resulting in poor quality daytime functioning

• Patients should at least undergo full video-polysomnography and

daytime monitoring of breathing patterns

SUMMARY

• Understanding of the respiratory status of an individual patient, in

terms of breathing and gas exchange measurements is essential

in directing the optimal treatment using non-pharmacological

methodology

• Increasing use of regular exercise should improve overall fitness

and hence quality of life and may reduce risks of surgery

• A multidisciplinary approach will help in assessing and managing

the complexities Rett patients

THANK YOU