Guidance

Newborn and infant physical examination (NIPE) screening programme handbook

Updated 16 October 2024

NHS newborn and infant physical examination (NIPE) screening programme handbook: markdown text

This handbook informs and supports best clinical practice and should be used in conjunction with the NIPE programme standards and the NIPE screening pathways.

1. Newborn and Infant Physical Screening Examination (NIPE)

The NIPE programme recommends the offer of screening to all babies born in England for conditions relating to the:

• eyes
• heart
• hips
• testes (if applicable)

The NIPE newborn screening examination should be completed before, or at, 72 hours of age (KPI NP1). The NIPE newborn screening examination in England forms part of the wider systematic examination of the newborn and is usually undertaken at the same time.

NIPE infant screening examination is then offered at 6 to 8 weeks of age, usually in primary care/GP practice.

All references to the NIPE screening examination in this document relate to the 4 screening elements described above.

The NHS newborn and infant physical examination (NIPE) programme’s main aims are to:

• identify and refer all children born with congenital abnormalities of the eyes, heart, hips and (in males) testes, where these are detectable, within 72 hours of birth
• further identify those abnormalities that may become detectable by 6 to 8 weeks of age, at the NIPE infant screening examination
• reduce morbidity and mortality

These ages are recommended based on best practice and current evidence and should facilitate a prompt referral for early clinical assessment.

References to parent(s) also relate to carer(s), if appropriate.

1.1 NIPE infant screening examination

The NIPE infant screening examination is not a formally managed part of the NIPE programme and regional commissioners will provide scrutiny (as required) to oversee this part of the examination.

The infant screening examination should be performed at 6 to 8 weeks of age for all 4 conditions (as some conditions can develop or become apparent after the newborn screen). This is usually undertaken in a primary care setting. It is recommended that all screening results are recorded on the GP IT system and in the personal child health record (PCHR), the ‘red book’.

Please note there are no NIPE standards for the infant screening examination, as there is currently no national method of recording and measuring uptake and outcomes.  However, the NIPE screening programme does produce best practice guidance and recommended referral timescales in relation to the infant screening examination.

2. Information for parents

It is important that all parents receive/access a copy of Screening Tests for You and Your Baby. This provides information about the screening tests offered during pregnancy and in the neonatal period. This is now digital and QR codes are provided. The link provided below includes access to easy read versions, alternative languages, and instructions on how to print digital leaflets. (https://www.gov.uk/government/publications/screening-tests-for-you-and-your-baby-description-in-brief)

Additional information is also available for parents of babies who are in a special care baby unit, neonatal intensive care or paediatric intensive care unit. (https://www.gov.uk/government/publications/screening-tests-for-you-and-your-baby-babies-in-special-care-units),

For NIPE, this information should be given:

• during the antenatal period

• before offering the NIPE newborn and infant screening examination

Parents should be made aware of the limitations of newborn screening tests, as described in Screening Tests for You and Your Baby.

2.1 NIPE screening examination declined

Consent should be obtained and recorded prior to screening. When consent for the NIPE screening examination (newborn or infant) is declined parent(s) should be fully informed of the different elements of the NIPE newborn and infant screening examinations and why screening is being offered.

Parent(s) have the right to decline all or part of the NIPE newborn or infant screening examination, and If parent(s) do decline all or part of the NIPE newborn screening examination any decision should be recorded as below:

If the parent(s) decline all or part of the NIPE newborn screening examination, the reason this should be documented in:

• the NIPE national IT system (SMaRT4NIPE (S4N)
• the PCHR

This should also be communicated to the GP and health visitor.

Contact details should be provided for the parent(s) from the discharging service, informing the parent(s) that their baby will remain eligible for NIPE newborn screening until 6 weeks of age and will be offered NIPE infant screening at 6 to 8 weeks of age. Parent(s) should be informed they can change their decision regarding screening (if this is within the specific screening timeline) by contacting their current care provider.

If the parent(s) decline all or part of the NIPE infant screening examination, the reason should be documented in

• the GP IT system
• the PCHR

This should also be communicated to Child Health Information Services (CHIS) and the health visitor.

The examination can be undertaken later. See [section 7: Babies who have missed screening] (#section7) below for clinical information regarding screening older children.

3. Screening tests

3.1 NIPE newborn screening examination

The NIPE newborn screening examination should be offered and completed before, or at, 72 hours of age (KPI NP1). For babies born in hospital, it is recommended that the screening examination is completed before discharge home. This will maximise the opportunity for completing the examination within 72 hours of age. There is no lower age limit on undertaking the newborn examination, but if a baby is to be discharged home at 6 hours of age the newborn examination should be undertaken as near to discharge time as possible, or arrangements made to undertake in the primary care setting (see below) 

Local arrangements should be in place to meet NIPE standard NP1 when babies are:

• born at home

• discharged home before NIPE newborn screening is completed

This could include attending a designated NIPE clinic or arrangements being made for the examination to take place at home or in the primary care setting. It is the responsibility of the care provider to ensure the NIPE newborn screening is completed.

Local arrangements should also be in place to meet national timescales when babies are transferred from one acute provider to another before NIPE newborn screening is completed or there are screen positive results requiring follow-up.

Healthcare professionals should inform parents of NIPE screening findings and advise them to report any concerns about their baby’s wellbeing at any time.

4. Babies in neonatal units

If a baby is receiving care in a neonatal unit, this is not a pre-defined reason to delay screening. NIPE newborn screening should be undertaken as soon as the baby is well enough but can be delayed for the following reasons:

4.1 ‘Too young’ for NIPE newborn screening

If a baby is born before 34⁺⁰ weeks gestation, screening can be delayed to 34⁺⁰ weeks corrected age. The baby continues to remain eligible for NIPE newborn screening and screening should be performed as soon as the baby is deemed old enough and is well enough. These babies should be accounted for in key performance indicator (KPI) data, stating the reason for delay (too young) as a mitigation against the performance threshold for KPI NP1 (screening complete by 72 hours of age). Any baby born below 34+0 weeks are automatically identified on the S4N system by a ‘GA’ flag, to assist with management of the records. Please see the S4N user guides within the ‘Resources’ section of the S4N system for further information.

The screening of babies before 34⁺⁰ weeks corrected age (for example, those considered well enough) is a local clinical decision. However, it is not recommended to routinely perform the NIPE newborn screening examination before 34+0 weeks due to the potential for findings to subsequently change, and inappropriate referrals being made. It is envisaged that NIPE newborn screening prior to 34+0 weeks would happen in circumstances where there is a risk that otherwise the NIPE may not be performed later or missed.

If a baby has reached 34 weeks + 0 days corrected age but is still considered ‘too ill’ for NIPE, then screening can be delayed further (please see ‘too ill’ for NIPE newborn screening).

4.2 ‘Too ill’ for NIPE newborn screening

Screening may be delayed if a baby is too ill for screening. These babies should be accounted for in key performance indicator (KPI) data, stating the reason for delay (too ill) as a mitigation against the performance threshold for KPI NP1 (screening complete at or before 72 hours of age).

Possible clinical reasons for this delay include:

• respiratory support (other than low flow oxygen), including the presence of chest drains for the first 72 hours

• any cardiovascular support, for example, inotropes, prostin

• ventilated infant until extubated

• baby on continuous positive airway pressure (CPAP)

• therapeutic hypothermia

• intense phototherapy (double or more, need for immunoglobulin or exchange transfusion)

• chest drain in place (without additional respiratory support)

• umbilical lines and, or arterial lines in place

• post-operative, until off analgesia

• unstable hypoglycaemia until off intravenous dextrose

• where active reorientation of care to comfort or palliative care is taking place

This is not an exhaustive list and clinical judgement should also be used when assessing suitability for newborn NIPE screening.

5. Screening results – NIPE newborn screening examination

All screening and outcome results should be recorded on S4N and in the baby’s PCHR.

5.1 Screen negative

Following the NIPE newborn screening examination, the parent(s) should be informed of the results. For babies who have transferred into the area or between care providers with a documented screen negative NIPE screening result, no further action is required.

The parent(s) should be informed that the NIPE infant screening examination will be undertaken in the primary care setting at 6 to 8 weeks of age (as some conditions can develop or become apparent later).

5.2 Screen positive

The parent(s) should be informed of the results and of any referral process that may be required, including expected appointment timescales. Appointments may be agreed and made at this point or arranged in line with local processes.

If a baby was not brought for any follow-up appointment, further appointments should be made in line with the trust ‘did not attend’ (DNA) or ‘was not brought’ (WNB) policy.

If the baby is already in a treatment pathway for one of the screening elements, the NIPE infant 6 to 8-week screening will still need to be completed for the remaining screening elements.

Where there is a screen positive result and NIPE newborn screening examination has been undertaken after the target timescale, a specialist appointment should be arranged without delay.

Ideally there should be opportunities for shared learning for all clinicians performing NIPE newborn screening to review and learn from postnatal diagnoses.

6. NIPE infant 6 to 8-week screening examination

Information about the NIPE infant screening examination should be given to the parent(s) during the postnatal period and again before the examination is offered and undertaken. This examination typically takes place in a primary care setting.

There should be timely checks to ensure that:

• all eligible babies are offered screening (including those who move into the area)

• the examination takes place between 6 and 8 weeks of age

• any required referrals are made within the nationally recommended timescales

• referrals are followed up so that appropriate interventions take place in line with national guidance

If the baby has had screen positive results for any element of the NIPE newborn screening examination, the practitioner undertaking the NIPE infant screening examination should check the progress along the care pathway to ensure required actions have taken place.

7. Screening results – NIPE infant screening examination

All screening and outcome results should be recorded on the GP IT system, communicated to CHIS and the health visitor, and recorded in the baby’s PCHR.

7.1 Screen negative

Following the NIPE infant screening examination the parent(s) should be informed of the results. For babies who have transferred into the area or between care providers with a screen negative result, no further action is required.

The parent(s) should be advised that the baby will follow the Healthy Child Programme.

7.2 Screen positive

The parent(s) should be informed of the results and of any referral process that may be required, including expected appointment timescales.

Where there is a screen positive result and NIPE infant screening examination has been undertaken after the target timescale, a specialist appointment should be arranged without delay.

8. Record keeping

Providers should obtain verbal consent for screening from parent(s), which should be documented, and a record should be made of the screening result and any referrals following screen positive result(s). The above information should be recorded in:

• S4N (NIPE newborn screening examination) or GP IT system (NIPE infant screening examination)

• baby’s clinical notes

• baby’s PCHR

• local clinical data collection system (where appropriate)

There should be:

• local arrangements to ensure all babies with screen positive results are referred and seen in line with national standards

• regular feedback of attendance at appointment or specialist review after screen positive referrals to enable recording of outcomes in S4N

• a local process to follow up all non-attendance of appointments after screen positive referral

• a process to record all screening results and outcomes (including post referral outcomes on S4N)

Access to S4N can be given to ultrasound practitioners, orthopaedic services or ophthalmology services to support timely input of post referral outcomes for the NIPE hip and eye screening pathways. This can be discussed and agreed locally with the NIPE lead.

9. Babies who move in and out of area (including transfers)

9.1 Babies who move into the area

Babies who move into the area (move into the country or county) are the responsibility of the current care provider.

If a baby is unscreened, NIPE screening should be offered by the maternity, neonatal, paediatric or primary care service depending on the age and condition of the baby. For these babies, screening results will, in practice, only be recorded on S4N if the NIPE newborn screening examination is undertaken by the maternity or neonatal service.

9.2 Transfer of unscreened baby to another care provider

If a baby is transferred to another care provider before the NIPE newborn screening has been undertaken or completed, the transferring care provider should formally hand over responsibility and communicate this to the receiving care provider both verbally and using S4N. Assessment of screening status should be fully embedded in admission processes. 

The responsibility for screening eligible babies remains with the birth unit until responsibility is formally passed and accepted by another provider. Transfer of the baby record between acute care providers should be managed using S4N.

After transfer, the receiving care provider should undertake the NIPE screening examination. Newborn screening results should be recorded on S4N if the NIPE newborn screening examination is undertaken by the maternity, neonatal or paediatric service.

9.3 Transfer to specialist units

This could include a variety of settings, including children’s hospitals, surgical units or cardiac units.

For management of records within S4N where the baby has been transferred to a specialist unit not using S4N, please see further information and advice in the SMaRT4NIPE national IT system section of the handbook.

9.4 Babies with screen positive results who transfer between care providers

The transferring provider is responsible for communication with the receiving provider to ensure completion of any outstanding elements of the screening pathway.

If there is an agreement, the record is formally transferred on S4N. The receiving provider is then responsible for follow-up and recording of post referral outcomes. If the birth unit chooses to keep the responsibility for follow-up appointment (due to local decision) they should ensure outcomes are known, recorded and where appropriate, communicated to the responsible care provider.

If there is not a prior agreement and formal transfer of care, the assurance of the completion of the screening pathway and recording of the outcome remains the responsibility of the referring birth unit.

10. Communication pathways following bereavement

There should be local systems to identify deceased babies and inform other relevant screening and clinical services without delay. If a baby dies before NIPE newborn screening has been completed, this should be recorded as soon as possible within S4N so that the baby’s record is removed from the worklist, and any inappropriate communications are avoided.

11. Babies who have missed screening

The following clinical guidance outlines the appropriate screening tests (depending on age) for babies found to have missed NIPE screening. If the missed screen is due to a screening pathway failure, then actions should be taken in line with national screening incident guidance.

11.1 Classification of delayed or missed NIPE newborn screening examination

All babies should have all elements of the NIPE newborn screening examination undertaken before being fully discharged from the care of any maternity, neonatal or paediatric inpatient services.

11.2 Delayed NIPE newborn screening

A NIPE newborn screening examination is classified as ‘delayed’ if it is not completed by 72 hours of age. Screening should be completed as soon as possible after this time by the current care provider (see the [NIPE newborn screening examination] (#newborn-screening-examination) section above for reasons screening may be delayed). The reasons for the delay beyond 72 hours of age should be recorded on S4N and used locally to collate mitigations against KPI NP1 coverage performance target. Reasons for delayed screening should be locally audited and investigated if appropriate.

Babies remain eligible for NIPE newborn screening examination until 6 weeks of age. This includes unscreened babies who ‘move in’ to an area or between care providers (please see ‘Babies who move into the area’)

11.3 Missed NIPE newborn screening

A NIPE newborn screening examination is classified as ‘missed’ if it is not completed:

• before full discharge from maternity, neonatal or paediatric inpatient services (without provision for completion within the primary care setting)

• by 6 weeks of age for unscreened babies who remain in maternity or neonatal inpatient care

• by 6 weeks of age for unscreened babies who have moved into an area

11.4 Long stay NICU babies

At 6 weeks of age, babies become eligible for the NIPE infant screening examination. If NIPE newborn screening is performed at or after 6 weeks of age (for example long-stay NICU babies), due to the age of the baby it is not necessary to also do the NIPE infant screening examination.

11.5 Missed NIPE infant screening up to 3 months of age

If the NIPE infant screening examination has not been completed by 8 weeks of age, all elements should be completed as soon as possible after this time.

Please note - the screening examination for unstable hips using the Barlow and Ortolani manoeuvres can still be completed but only up to 3 months of age.

11.6 Missed NIPE infant screening older than 3 months of age

The NIPE infant screening examination can still be completed and should be done as soon as possible.

However, the screening examination for unstable hips using the Barlow and Ortolani manoeuvres is no longer accurate if the infant is older than 3 months of age. Any asymmetry of leg length or restricted hip abduction should be assessed, and the child’s gait observed, if walking.

All the other elements of the NIPE infant screening examination (eyes/heart/testes) should be completed in the usual manner.

In line with advice in the PCHR, the parent(s) should be advised to contact their GP or health visitor if they have any concerns regarding their child’s wellbeing.

11.7 Completing a missed NIPE examination

The relevant examination or observation must be undertaken by a NIPE qualified practitioner.

12. Screening examination of the eyes

Eye development is complex, and a wide range of structural abnormalities may occur if the process is disturbed either due to genetic, infective or sporadic causes.

The primary purpose of the NIPE newborn and infant eye screening examination is to identify congenital cataract(s). Some abnormalities, particularly congenital cataract, are treatable but require early detection, rapid referral and management to prevent lifelong visual impairment(https://pubmed.ncbi.nlm.nih.gov/27180288/).

12.1 Incidence

Around 2 to 3 in every 10,000 babies (200 per year in England) are born with cataracts. In over 50% of these, both eyes will be affected. One fifth of affected babies will have a family history of cataracts. Cataract is the most common treatable cause of blindness in childhood in the UK and worldwide.

A congenital cataract is an opacity within the lens of the eye, which is located behind the pupil. It can occur in one or both eyes and range in severity. A severe cataract blocks light and images from reaching the retina, preventing visual pathway development at a critical stage. Although rare, it is the most common, treatable, visually significant condition identified during NIPE eye screening.

The screening practitioner should be alert to risk factors, such as genetic syndrome, pre-natal infection, sensorineural hearing loss, neurodevelopmental issues or a family history of an eye condition with onset in infancy or early childhood.

Babies with other eye abnormalities, incidental findings or risk factors identified at the NIPE newborn or infant screening examination should be referred for specialist review according to locally agreed pathways.

Please note the guidance below relates to both the NIPE newborn and infant eye screening examinations unless otherwise stated.

12.2 Before the NIPE eye screening examinations

Before the examinations, practitioners should:

• discuss the screening test and gain consent from the parents to undertake the examination

• establish the mother’s recent obstetric history

• establish if there are any clinical risks for eye disorders (see below)

12.3 Risk factors for congenital cataracts

Babies with a family history of bilateral congenital or hereditary cataracts in a first-degree relative are at risk of developing early cataracts. Even if the NIPE screening examination is normal, these babies may be considered for referral for early specialist opinion, via locally agreed pathways.

12.4 Risk factors for other eye or visual problems

Risk factors include:

• a first-degree relative with an ocular condition which was congenital or developed in early childhood, for example aniridia (absent iris), coloboma (malformation of the eye) or retinoblastoma (malignant retinal tumour) childhood

• prematurity

• genetic syndromes, such as trisomy 21, associated with eye and vision disorders

• extensive port wine stain involving the eyelids, which can cause glaucoma

• maternal exposure to viruses during pregnancy, including rubella and cytomegalovirus

• neurodevelopmental conditions or sensorineural hearing loss (deafness caused by abnormal nerve function in the inner ear)

Although practitioners should be aware of these risk factors, they do not alter the NIPE national eye screening pathway. Babies identified with such risk factors should be managed via locally agreed pathways.

12.5 The eye examination

NIPE newborn eye screening examination

The primary aim of the NIPE eye screening examination is the detection of congenital cataracts. In addition, although not part of the screening programme, it is considered good practice to include assessment of the:

• eyelids, to exclude malformation and skin abnormality, such as extensive port wine stain involving the eyelids, which can cause glaucoma and affect the baby’s ability to fully open the eyelids

• presence of both eyes

• symmetry of eye size

• symmetry and clarity of the cornea (the cornea is the circular transparent window of the eye through which the iris and pupil can be seen – its diameter in a term baby should be similar to the width of the practitioner’s little finger-tip, the roundness and symmetry of the pupils)

NIPE infant eye screening examination

In addition to the assessment described for the newborn eye screening examination, the 6 to 8-week infant eye screening examination should also include checking:

• if the parents have any concerns about the baby’s visual behaviour, for example asking if the baby looks at them steadily and has started smiling back at them

• the ability of the baby to fixate the practitioner’s face steadily, without nystagmus (wobble of the eyes)

• the ability of the baby to fix and follow a large, bright target by moving their eyes (not just by moving their head)

• the alignment of the eyes, which can be variable at this age but a consistently and significantly deviated eye is not normal

The red reflex examination

The red reflex should be assessed. This is the normal reflection of white light from the back of the eye which is seen as a red glow in the pupil on ophthalmoscopy and is like the red-eye effect seen on flash photography.

To undertake the examination, the practitioner should:

• dim the overhead lights and make sure that the baby is settled

• hold the eyepiece of the ophthalmoscope up to his or her eye, at arm’s length from the baby’s face

• direct the circle of light from the ophthalmoscope towards the baby’s eye while gently parting the baby’s eyelids, if necessary

• view the red reflex through the ophthalmoscope eyepiece – the colour, brightness and presence of any shadows on the red reflex is noted in each eye

The examination can be repeated by an experienced practitioner if the examination is equivocal. This should ideally be within the 72-hour guideline period of the NIPE newborn examination.

The normal red reflex varies in hue depending on the baby’s ethnicity. White babies have an orange-red reflex. The reflex can be less bright and appear magnolia in colour in black, Asian or minority ethnic babies. If the assessment is difficult, it can help to assess the baby’s parents’ red reflexes to determine the expected reflex colour.

Examples of normal red reflex by ethnicity of baby (from left to right: black, Asian, white)

Congenital cataracts

Congenital cataracts cause a central shadow, completely obscure the red reflex or may make the reflex in one eye appear duller than the other. A severe cataract can make the pupil appear white when viewed with the naked eye.

The red reflex is abnormal if it is completely or partially obscured, is abnormal in shape (iris coloboma or aniridia), white or asymmetrical in colour or brightness to the other eye.

Examples of partially (left) and completely (right) obscured red reflexes

An example of a white ‘red reflex’

12.6 Screen negative (normal red reflex assessment)

Babies with screen negative eye results for the NIPE newborn screening examination should have the NIPE infant screening examination at 6 to 8 weeks of age. Infants with screen negative eye results for the NIPE infant screening examination should follow the Healthy Child Programme.

Babies with screen negative eye results but with risk factors described above may be referred via locally agreed pathways.

12.7 Screen positive (abnormal red-reflex assessment)

Babies with screen positive eye results following the NIPE newborn screening examination should be urgently referred via the NIPE pathway and seen by an ophthalmologist within 2 weeks of the NIPE newborn screening examination. However, if there are significant concerns at the time of the NIPE newborn screening examination, discussion with the ophthalmology service before the baby’s discharge home should be considered.

Surgery for severe cataract is usually undertaken between 6 to 10 weeks of age for optimal visual outcome.

Babies with screen positive eye results following infant screening examination should be referred promptly and seen by a consultant ophthalmologist or paediatric ophthalmology service by 11 weeks of age.

12.8 Parental concerns

Parents should be advised to contact their midwife, GP or health visitor if they have any concerns about their baby’s eyes or visual behaviour, including:

• inability of their baby to fully open their eyes or if eyelid opening appears asymmetrical

• apparent deterioration of visual interest

• a wobbling of the eyes

• a consistent eye misalignment

• an abnormal appearance of the eyes

• a white reflex, consistently seen on flash photography

• asymmetry of the red reflex, consistently seen on flash photography

Note that a family history of an eye or vision condition developing later in life does not require a neonatal referral to ophthalmic services.

Other eye abnormalities should be managed according to local referral pathways.

13. Screening examination of the heart

Congenital heart disease (CHD) is a term used to describe a problem with the structure and function of the heart that is present at birth.

The purpose of screening is the early recognition of signs and symptoms which may be indicators of major or critical CHD.

For the purposes of NIPE newborn screening, CHD can be categorised as:

• critical congenital heart disease (CCHD), which includes all potentially life-threatening duct-dependent conditions and those conditions that require procedures within the first 28 days of life

• major CHD, which includes defects not classified as critical but requiring invasive intervention in the first year of life

• CHD requiring intervention but not in the first year of life (for example a primum ASD)

*CHD which is unlikely to require intervention (for example small PDAs or small VSD)

13.1 Incidence

The incidence provided below is from a variety of sources with references provided to give background and context for heart screening.

Congenital heart disease is the most common birth defect in the UK (Children’s Heart Federation)

At least 8 in every 1,000 babies are born with a heart or circulatory condition

In England the birth prevalence for all CHD is 62.6 per 10,000 total births (6.3 per 1000 births)

For a more detailed breakdown, please see the NCARDRS congenital anomaly statistics NCARDRS congenital anomaly statistics: annual data - GOV.UK (www.gov.uk)

Some critical and major cardiac lesions may be detected during pregnancy at the 20-week ultrasound scan as part of the NHS Fetal Anomaly Screening Programme (FASP). The acceptable FASP standard target detection rate for specific cardiac abnormalities (atrioventricular septal defect [AVSD], Tetralogy of Fallot [TOF], hypoplastic left heart syndrome [HLHS] and transposition of the great arteries [TGA]) is displayed in this threshold table

13.2 Risk factors for CHD

Identification of risk factors for CHD may help to inform the discussion with parents prior to screening, and the wider physical examination.

Although practitioners should be aware of these risk factors, they do not alter the NIPE heart screening pathway. For further information on the NIPE heart screen positive pathway please see section 13.10

The practitioner should have increased vigilance during the examination if one (or more) of these risk factors is present:

• chromosomal or genetic abnormalities which may be associated with congenital heart disease
• Trisomy 21 is classed as ‘screen positive’ and should be managed separately as part of the national screening pathway
• family history of cardiac problem in first degree relative – diagnosed at birth or in childhood requiring surgery or regular follow up
  • where family history of heart muscle or electrical problems in first degree relatives is present, this should be discussed locally with a senior paediatrician or neonatologist (ideally with expertise in cardiology), who may then choose to consult further with a specialist tertiary paediatric cardiologist. Parents may already have a management plan if the mother was seen by fetal cardiologist during pregnancy
• parental consanguinity
• maternal exposure to viruses
• maternal conditions, such as diabetes (type 1), epilepsy, systemic lupus erythematosus (SLE)
• teratogenic drugs taken during pregnancy

Babies identified with any of the above risk factors should be managed via locally agreed pathways.

Information about risk factors for heart is not routinely monitored by the NIPE screening programme. However, risk factor data can be recorded on S4N, and could be used for local audit.

13.3 Babies with suspected or diagnosed cardiac problems identified in the antenatal period

Before the examination, practitioners should establish relevant information regarding:

• mother’s medical and recent obstetric history, including any medication
• any relevant antenatal or paediatric alerts
• any antenatal screening ultrasound results, and any specific advice pertaining to cardiological assessment in the neonatal period

If a baby has a suspected or diagnosed cardiac problem on antenatal ultrasound scan (+/- fetal echo), they will have an individualised care plan which should be followed alongside the routine NIPE newborn screening examination.

It is imperative that all elements of the NIPE screening are completed within the usual timeframes. For babies who are ‘too young’ or ‘too ill’, screening may be delayed – please see ‘Babies in neonatal units’ for further information.

13.4 Undertaking the newborn heart examination

Before the examination, parents should be informed about the limitations of NIPE newborn heart screening.

Practitioners should establish relevant information regarding:

• mother’s medical and recent obstetric history, including any medication
• any antenatal or paediatric alerts
• any antenatal screening ultrasound results, and any specific advice relating to cardiac assessment, management and follow up in the newborn
• baby’s family history
• risk factors for CHD (see section 9.2)
• baby’s health since birth

Parents should be asked:

• if their baby is generally well
• if they have any concerns about their baby’s breathing or colour when their baby is at rest or feeding.
• if their baby is not feeding well
• is ever too tired to feed, quiet, lethargic, or has poor muscle tone (floppy). This may be particularly relevant to the infant examination.

If a NIPE practitioner is unsure of their findings, or unsure if referral is required following the NIPE newborn screening examination, the baby should be reviewed by a senior neonatal clinician from Tier 2 rota or above within 24 hours of the NIPE newborn examination, but the urgency will depend on suspected condition.

13.5 Observation

Observation includes reviewing the baby’s:

1. general tone
2. central and peripheral colour
3. size and shape of chest
4. respiratory rate
5. symmetry of chest movement, use of diaphragm and abdominal muscles

and also observing for:

• ·         signs of respiratory distress (recession or grunting)
• dysmorphic features (as they can be associated with various syndromes with a higher incidence of CHD)

13.6 Palpation

Palpation involves examination / assessment of the following:

• femoral and brachial pulses for strength, rhythm, and volume. Absent or weak femoral pulses may suggest certain types of CHD (including coarctation of the aorta). Palpation of the femoral pulses is an essential part of the NIPE screening examination. An attempt to palpate the brachial pulses simultaneously for comparison should be made, as this adds to the sensitivity of the investigation and may help to detect weak femoral pulses.
• perfusion through capillary refill time (the midpoint of the sternum/forehead should be pressed for 5 seconds; normal reperfusion is within 3 seconds)
• position of cardiac apex (to exclude dextrocardia)
• palpation of liver (to exclude hepatomegaly - may be present in congestive heart failure)
• presence of a thrill (palpable murmur/vibratory sensation felt on the chest wall)

13.7 Auscultation

Auscultation is the assessment of the quality of heart sounds at designated key areas, and includes the identification of a murmur, either systolic or diastolic and loudness.

The key areas for auscultation are:

• the upper right sternal edge
• the upper left sternal edge
• the lower left sternal edge

• the apex

• mid scapulae

Auscultation of key areas do not need to be undertaken in any specific order, but all should be included.

It is important to note that critical congenital heart disease does not always present with a murmur, therefore auscultation alone cannot be used to determine if CHD is present.

13.8 Pulse oximetry

The UKNSC does not currently advocate universal pulse oximetry as part of newborn heart screening.

However, if there are any concerns following observation, palpation or auscultation, the baby should have a full diagnostic assessment which would include pulse oximetry testing.

13.9 Screen negative results

Babies with screen negative heart results following the NIPE newborn screening examination should continue with routine postnatal midwifery care and follow the Healthy Child Programme: Pregnancy and the First 5 Years of Life - GOV.UK (www.gov.uk) with the GP and specialist community public health nurse (health visitor). The baby should be offered NIPE infant screening at 6-8 weeks.

Infants with screen negative heart results following the NIPE infant screening examination at 6-8 weeks, should follow the Healthy Child Programme. Parents should be advised to contact their healthcare professional or emergency services if they have any concerns about their baby.

13.10 Screen positive results

Screen positive criteria

1. Babies with an antenatal diagnosis of trisomy 21 (Down’s syndrome)

CHD is present in approximately 40-60% of babies born with Down’s syndrome, and of these 30-40% are complete atrioventricular septal defects (AVSD)[i].

All babies with trisomy 21 meet the screen positive criteria and require review by a paediatric or neonatal consultant within 24 hours.

In addition, providers should refer to the Down’s Syndrome Medical Interest Group (DSMIG) who have produced guidelines in order that cardiac conditions can be diagnosed and treated promptly.

1. Babies with postnatally suspected T21

These babies also require review by a paediatric or neonatal consultant within 24 hours of the NIPE newborn examination; ongoing referrals will be decided based on examination findings and genetic result

1. Babies with signs and symptoms that may suggest major or critical CHD. These include: - tachypnoea at rest - episodes of apnoea lasting longer than 20 seconds or associated with colour change - intercostal, sub-costal, sternal or supra-sternal recession, nasal flaring - central cyanosis - visible pulsations over the precordium, heaves, thrills - absent or weak femoral pulses - presence of heart murmurs or extra heart sounds (please see section 9.12 for additional supporting information about murmurs)

If a suspected major or critical heart condition is found on the NIPE newborn screening examination, this should be urgently escalated to a paediatric or neonatal consultant. The level of urgency will be determined by the severity of the suspected condition, and the clinical presentation of the baby. All screen positive referrals require review within 24 hours of the NIPE newborn examination, but in the case of suspected major or critical heart conditions or concerning symptoms, review would take place much earlier.

If NIPE practitioners are unsure of findings, or unsure if referral is required following the NIPE newborn screening examination, the baby should be reviewed by a senior neonatal clinician from Tier 2 rota or above within 24 hours of the assessment, but the urgency will depend on suspected condition.

For all screen positive referrals, confirmation of review and assessment within the 24 hour timescale needs to be recorded as a screen positive post referral outcome in SMaRT4NIPE (S4N).

Babies with an antenatally suspected or diagnosed cardiac problem should follow their individualised clinical care plan which should already be in place (see section 9.3 for further information).

Babies with screen positive heart results following the NIPE infant screening examination should follow the locally agreed referral process. Urgency will depend on the clinical condition of the infant.

13.11 Additional information

Many babies will have cardiac murmurs in the first 24 hours of life in the absence of a cardiac defect (linked to physiological changes at birth). However, cardiac murmurs may be absent in babies with a significant cardiac defect.

NIPE practitioners may find the additional information below useful in clinical practice:

Significant murmurs

These are:

• usually loud (+/- a thrill)
• usually heard over a wide area
• usually with a harsh rather than soft quality
• possibly associated with other abnormal findings

Benign murmurs

Benign murmurs are typically short, soft, systolic, and localised to the left sternal border, although they can be heard elsewhere. They have no added sounds or other clinical abnormalities associated with them.

13.12 Heart screening clinical standards

Although there is no national NIPE heart screening performance standard, it is recommended that the national clinical pathway above is followed. Practitioners should be familiar with, understand and follow any existing local policies, guidelines and referral pathways.

13.13 Acknowledgements

We gratefully acknowledge the members of the NIPE expert heart working group for their contribution to the update of this section of the handbook.

14. Screening examination of the hips

The guidance below relates to both NIPE newborn and infant screening examinations unless otherwise stated. National hip risk factors and suspected abnormality on clinical hip examination all follow the same pathway.

The primary purposes of screening are:

• early identification of a dislocated or a dislocatable hip(s)

• the identification of sonographic pathological hip dysplasia through selective ultrasound scan (USS)

• minimising the risk of long-term complications through:

• timely hip ultrasound scan

• further expert assessment

• early intervention

14.1 Incidence

The incidence of babies requiring treatment is:

• 3 to 5 per 1,000 live births that may require a Pavlik Harness

• 1 to 2 per 1,000 live births that may require surgery

Undetected unstable hip(s) with delayed treatment may result in the need for complex surgery and, or long-term complications such as impaired mobility and pain osteoarthritis of the hip and back.

Early diagnosis and intervention should improve health outcomes and reduce the need for surgical intervention.

14.2 NIPE hip risk factors

NIPE national hip risk factors include:

• first-degree family history of hip problems in early life – this includes baby’s parents or siblings who have had a hip problem that started as a baby or young child that needed treatment with a splint, harness or operation

• breech presentation at or after 36 completed weeks of pregnancy, irrespective of presentation at birth or mode of delivery – this includes babies who have had a successful external cephalic version (ECV)

• breech presentation at the time of birth between 28 weeks gestation and term

14.3 Multiple pregnancy

Risk factors

All babies from a multiple pregnancy where any of the NIPE hip risk factors listed above are present should have a hip ultrasound. This is because if one of the babies meets the criteria of breech presentation described above during pregnancy, it may be difficult to accurately identify which baby was affected.

All babies from a multiple pregnancy should be referred for hip ultrasound if at least one of the babies has a screen positive result from clinical examination of the hips. This is because the other baby(s) now has a sibling with a history of a hip problem in early life, which is one of the NIPE national hip risk factors. This applies to both the NIPE newborn and infant screening examination.

14.4 Undertaking the hip screening examination

Before the examination, practitioners should establish:

• the mother’s recent obstetric history

• the baby’s family history

• the presence of any NIPE hip risk factors

The examination should take place in a warm environment and on a firm, flat surface with the baby undressed and settled.

14.5 Observation and manipulation

Observation covers:

• symmetry of leg length

• level of knees when hips and knees are bilaterally flexed

• restricted abduction of the hip in flexion

Please note that observation of skin creases for symmetry is not part of the NIPE screen. This is no longer regarded as a screen positive finding.

NIPE Practitioners should undertake both the Barlow and Ortolani test manoeuvres on each hip separately to assess hip stability. The Barlow manoeuvre is used to screen for a dislocatable hip (a dislocatable hip will be displayed out of the joint by this manoeuvre). The Ortolani manoeuvre is used to screen for a dislocated hip (a dislocated hip will be felt clunking into place).

14.6 Screen negative

Babies with screen negative hip results on NIPE newborn clinical examination and no national hip risk factors present should have the NIPE infant screening examination at 6 to 8 weeks.

Infants with screen negative hip results on NIPE infant examination (and no national hip risk factors –), should follow the Healthy Child Programme.

Any outstanding hip pathway referrals from the NIPE newborn screening examination should be followed up

14.7 Parental concern

Parents should be advised to contact their healthcare professional at any time if they have any concerns about their baby’s hip(s). This includes if:

• one leg cannot be moved out sideways as far as the other when changing the baby’s nappy

• one leg seems to be longer than the other

• one leg drags when their baby starts crawling

• their child walks with a limp or has a ‘waddling’ gait when they start walking

14.8 Screen positive

A screen positive hip result is an abnormal clinical hip examination (with or without risk factors) and, or presence of NIPE hip risk factors.

A suspected abnormality on clinical examination is defined by:

• difference in leg length

• knees at different levels when hips and knees are bilaterally flexed

• restricted unilateral limitation of hip abduction (with a difference of 20 degrees or more between hips)

• gross bilateral limitation of hip abduction (loss of 30 degrees abduction or more)

• palpable ‘clunk’ when undertaking the Ortolani or Barlow manoeuvre

Babies with screen positive hip results following NIPE newborn screening examination should be managed as outlined below.

14.9 Referral and outcome decision

The [standards S03 and S04] can be found at (https://www.gov.uk/government/publications/nhs-population-screening-reporting-data-definitions)

With effect from April 2024 standard S03 will be stood down as a key performance indicator (KPI). Standard S04 will be a KPI (NP4). A performance threshold has now been set for standard 4 (NP4 - outcome decision). Please see the NIPE standards for further information Newborn and infant physical examination screening: standards - GOV.UK (www.gov.uk)

Trusts are still advised to meet the current recommended 4-6 week timeframe for hip ultrasound. Clinically this is the most efficient pathway, reducing the need for repeat ultrasound scanning.

This approach focuses performance on outcomes, ensuring that babies complete the screen positive pathway by the required 6 weeks.

14.10 Standard 3 (NIPE S03 )

For babies with screen positive hip results, NIPE standard 3 requires the hip USS to be undertaken within the target timescale.

For babies who are born at <34⁺⁰ weeks gestation, hip USS should be undertaken between 38⁺⁰ and 40⁺⁰ weeks corrected age.

Referral to orthopaedic services after hip USS will then depend on the scan result and local management policy in place.

Please see the Good practice guidance section below for further guidance.

14.11 Standard 4 (NIPE S04- KPI NP4)

For babies who attend for ultrasound scan of the hips after screen positive newborn hip referral, standard 4 requires an outcome decision to be made within the target timescale. This is to ensure that babies who require treatment enter the treatment pathway by 6 weeks of age.

For babies who are born ≥34⁺⁰ gestation, an outcome decision should be made by 6 weeks of age.

For babies who are born <34⁺⁰ weeks gestation, an outcome decision should be made by 40⁺⁰ weeks corrected age.

An outcome decision is either:

• discharge from the hip screening pathway after review of normal hip USS results

• attendance for clinical assessment by orthopaedic specialist (it is required that hip USS has been undertaken in advance of the decision)

Infants with screen positive results following NIPE infant 6 to 8-week screening examination should be referred directly to paediatric orthopaedic surgeon for urgent expert opinion and be seen by 10 weeks of age.

14.12 Good practice guidance and additional information

A ‘one stop shop’ model can be provided, in which ultrasound and review by orthopaedic specialist or specialist practitioner is undertaken on the same day, during the same care episode. In order to meet the national standards associated with the hip screening pathway, this joint care episode should be between 4 and 6 weeks of age (or between 38⁺⁰ and 40⁺⁰ weeks corrected age for babies born <34⁺⁰ weeks gestation).

If hip USS and orthopaedic specialist assessment are undertaken separately, hip ultrasound will need to be routinely done soon after 4 weeks of age (or equivalent corrected age) to give time for orthopaedic appointment to be arranged, outcome decision to be made and, if required, treatment to be started by 6 weeks of age (or equivalent corrected age).

In line with national standard S04 and after local agreement, babies can be discharged by ultrasound services after normal hip scan. However, please note that some areas may wish review of all hip scan results by an orthopaedic specialist.

The term ‘orthopaedic specialist’ (standard S04) encompasses anyone with delegated responsibilities to initiate treatment decisions based on local policy or practice arrangements. These may include roles such as specialist paediatric physiotherapists, specialist orthopaedic nurse specialists and experienced radiographers.

If a hip rescan is required for technical reasons, it should, wherever possible, take place before 6 weeks of age (or equivalent corrected age). This will ensure that the final outcome decision is known and, if necessary, treatment can start within the optimum timescales (within 6 weeks of age or equivalent corrected age).

If a hip rescan is required for findings of immature hips (Graf 2a), it is acknowledged that this will be required at a timescale exceeding 6 weeks of age (or 40⁺⁰ weeks corrected age).

In both instances, the need for rescan should be recorded on S4N with the specific reason added in the comments box. Comments should include the keywords ‘technical’ or ‘immature’.

It is vital that S4N data is accurate and complete to enable full analysis when the hip screening pathway is reviewed.

A hip rescan undertaken after 6 weeks of age (or equivalent corrected age) will not count as an outcome decision as the screening pathway is not complete and an outcome decision is still required.

A local agreement should be in place to ensure that the results of the hip ultrasound scan and orthopaedic specialist or specialist practitioner appointment are recorded on S4N. This can be supported by enabling ultrasound and orthopaedic services access to S4N so that outcome data can be added.

From April 2024 the focus of the screening pathway is the final screening outcome and services should endeavour to ensure that all babies with screen positive hip results who require treatment, should be seen by orthopaedic services and in treatment by 6 weeks of age.  I order to achieve this, it may mean that the hip ultrasound scan may be undertaken outwith the 4-6 week timescale although this remains the recommended timescale for optimum care.   

Hip risk factors

To ensure accurate identification of national hip risk factors (in particular after successful external cephalic version), screen positive NIPE newborn results should be cross checked against birth registers and ultrasound records for example. Local retrospective audit of babies with risk factors from a separate source other than S4N will also assist in ensuring ascertainment. These should be regularly undertaken to ensure that any remedial action to arrange missed hip ultrasound can be taken.

It is important that NIPE practitioners recognise national NIPE risk factors and take appropriate actions to refer babies in line with the NIPE hip screening pathway. NIPE practitioners should be aware of all hip risk factor criteria and if unsure use the S4N hip risk factor information icon to check against clinical and family history.    

Note: If NIPE newborn screening is completed in primary care, -this needs to be reported and communicated to the maternity service and CHIS and ideally results recorded on the S4N system.

14.13 Confirmation of breech presentation

In response to queries regarding what constitutes breech presentation (abdominal palpation and, or ultrasound scan) and to make sure national NIPE hip risk factors are accurately defined, please see nationally agreed recommendations below.

Note that it may not always be feasible to arrange for a same day USS.

If the practitioner is sure of the findings on abdominal palpation (at or after 36 weeks gestation) and has recorded breech presentation in the woman’s records, this would count as a national NIPE hip risk factor.

If the practitioner is unsure of the findings on abdominal palpation (at or after 36 weeks gestation) and has recorded them as ‘? breech’ in the woman’s records, this initial finding of ‘? breech’ would not count as a national NIPE hip risk factor. Ultrasound to confirm presentation at the earliest opportunity or senior practitioner assessment would be required.

In both these scenarios, if the woman gives birth before confirmation ultrasound and the presentation at birth is cephalic, the NIPE examiner should seek local senior practitioner advice to assess whether the risk factor would apply (this would be a local decision).

14.14 Management of ‘clicky hips’

Hip screening aims to identify hip instability. The significance of isolated ‘clicks’ is debated. On balance and after review of evidence, isolated clicks are not considered clinically significant by the NHS NIPE screening programme.

Isolated clicks without any other relevant clinical findings should therefore not be classified as a screen positive finding and do not require referral for hip ultrasound.

If the examiner is unsure of their findings, confirmation of the screening outcome should be sought by an experienced clinician. After a second opinion, and if the screening outcome is still unclear, an ultrasound scan may be considered. This would be a local clinical referral and not part of the national NIPE hip screening pathway and should be recorded as an ‘other’ hip finding on S4N.

Local audits may be carried out to assess clinical competency and feedback to practitioners about ‘clicky hip’ findings.

Relevant studies related to ‘clicky hips’ include Kamath and others, 2005, Nie and others, 2017 and Marson and others.

15. Screening examination of the testes

The primary purpose of screening is to identify bilateral undescended testes or unilateral undescended testis.

15.1 Incidence

Undescended testis/testes, whether palpable or impalpable, affect approximately 2 to 6% of male babies born at term.

It is a condition in which one or both testes fail to descend from the abdomen into the scrotum.

A palpable undescended testis is one which can be felt but is not located in the scrotum (often referred to as being located in the ‘inguinal region’).

An impalpable undescended testis is one which cannot be felt at all.     

15.2 Undescended testes are associated with:

• Increased risk of testicular cancer (primarily seminoma, a germ cell tumour of the testicle)

• reduced fertility when compared with normally descended testes (particularly for bilateral undescended testes)

• other urogenital problems such as hypospadias

(https://pubmed.ncbi.nlm.nih.gov/33551366/ )

15.3 Additional Information

Bilateral impalpable testes in the newborn may be associated with disorders of sex development (previously known as ambiguous genitalia). The differential diagnosis includes congenital adrenal hyperplasia (CAH) in a female infant, which needs immediate investigation and treatment.

Early diagnosis and intervention maximise the potential for fertility and reduce the risk of testicular cancer. It may also allow for earlier identification of testicular torsion and therefore a better prognosis.

15.4 Risk factors

Factors that increase the risk of having undescended testes (unilateral and bilateral) include the following:

• a first-degree family history of undescended testes (baby’s father or sibling)
• earlier gestational age (<37 weeks gestation)
• low birth weight for gestational age
• some syndromes (such as Prader Willi syndrome, Prune belly syndrome)

Please note that presence of any of these risk factors does not change the screening pathway.

15.5 Undertaking the examination

Before the examination, practitioners should establish relevant information regarding:

• mother’s medical and recent obstetric history, including any antenatal/paediatric alerts
• baby’s family history
• risk factors as above
• baby’s health since birth

An explanation of the screening examination should be given to the parents.

15.6 Observation

Observe the scrotum for symmetry, size and colour.

15.7 Palpation

After warming the hands, carry out palpation of each scrotal sac to determine location of testes. Undertake palpation of the groins and the perineum if testes are not located in the scrotal sac (please refer to the video in the NIPE e-learning unit-elfh)

15.8 Screen negative

If no abnormality is detected, the baby should continue with routine postnatal care and follow the Healthy Child Programme: Pregnancy and the First 5 Years of Life - GOV.UK (www.gov.uk) with the GP and specialist community public health nurse (health visitor). All babies should be offered NIPE infant screening at 6 to 8 weeks of age.

NIPE practitioners should advise parents to contact their midwife, GP or specialist community public health nurse (health visitor) if they have any concerns about their baby’s testes.

15.9 Screen positive criteria

a)       bilateral impalpable testes 

b)       unilateral impalpable testis with or without hypospadias

c)       unilateral palpable testis not located in the scrotum, with hypospadias

d)       disorders of sexual development (previously known as ambiguous genitalia and sometimes referred to as disorders of sexual differentiation

e)         one or both testis /testes are palpable but not located in the scrotum without hypospadias

Many babies born <37 weeks gestation will have undescended and possibly impalpable testis after birth. These babies are likely to be seen by a senior paediatrician at regular intervals for their general care. The formal NIPE newborn screening examination can take place at the corrected gestational age of 34 weeks and 0 days.

There are the two management pathways depending on the clinical findings at the NIPE newborn screening examination:

Screen positive requiring urgent review

Review by a consultant paediatrician/ associate specialist within 24 hours of the newborn screening examination if following are present:

• bilateral impalpable testes
• unilateral impalpable testis with or without hypospadias
• unilateral palpable testis not located in the scrotum, with hypospadias
• disorders of sexual development

These babies should be managed in line with the local clinical pathways after review by a consultant paediatrician/ associate specialist to rule out any underlying condition such as Congenital adrenal hyperplasia (CAH) and confirm that gender assignment is correct (Society for Endocrinology UK guidance- https://onlinelibrary.wiley.com/doi/epdf/10.1111/cen.14528 ).

Screen positive requiring non- urgent review

Review required at the NIPE Infant screening examination if the following is present.

• one or both testis/testes are palpable, but not located in the scrotum (without hypospadias)

15.10 NIPE infant screening examination

Screen positive criteria

• bilateral impalpable testes
• unilateral impalpable testis with or without hypospadias
• unilateral palpable testis not located in the scrotum, with hypospadias
• disorders of sexual development

These infants should be referred and seen by a senior paediatrician as soon as possible but within 48 hours of the examination.

All babies with one or both testis/testes that are palpable, but not located in the scrotum should have a primary care (GP) review between 4 and 5 months of age and referral to surgeon if testis/ testes are still undescended or impalpable (to be seen no later than 6 months of age).

Spontaneous descent beyond this is unlikely and undescended or impalpable testes are best managed by surgical intervention

15.11 Acknowledgements

We gratefully acknowledge the members of the NIPE expert testes working group for their contribution to the update of this section of the handbook.

16. Training and maintenance of competency

All healthcare professionals have a personal and professional responsibility to maintain their clinical competency in relation to NIPE screening. There is also an organisational responsibility to ensure a safe and competent workforce.

Those who undertake NIPE examinations must work in a framework of professional accountability and code of conduct. Each practitioner is responsible for maintaining their own competence to carry out the examination to the highest standard and to identify gaps in their knowledge and any training needs.

To provide duty of care, an appropriately trained health professional must carry out NIPE examinations.

16.1 NIPE newborn screening examination

It is important to note the difference between the terminology NIPE newborn screening examination and the wider ‘systematic examination of the newborn’ (SEN) as described in the NMC standards of proficiency for midwives standards-of-proficiency-for-midwives.pdf (nmc.org.uk). In England the NIPE newborn screening examination is undertaken as part of the systematic examination of the newborn.

The NIPE newborn screening examination must be completed by a trained practitioner who is competent to undertake all elements of the examination and who has undergone relevant training and a locally agreed competency assessment by a practising NIPE examiner. This can be a midwife, neonatal nurse or health visitor who has successfully completed a university accredited ‘examination of the newborn’ programme of study which includes theoretical and clinical components, or it can be a qualified doctor.

16.2 Further information on required training for midwives

NIPE screening providers should ensure vigilance to confirm that all practitioners undertaking NIPE newborn screening examinations have undertaken the required training to perform these examinations.

Please see below for specific guidance regarding NIPE training and competency for newly qualified and internationally educated midwives. The text below and additional guidance for midwives returning to practice can be found here :

Midwives joining the NMC register completing programmes approved using the 2019 Standards of proficiency for midwives:

• All students successfully completing and exiting a midwifery training programme approved against the 2019 NMC Education Standards should have completed both the theory and practice requirements of the systematic physical examination of the newborn training

• In England, this would meet NIPE screening programme requirements and assuming both theory and practice has been included in the training these midwives are, therefore, able to carry out NIPE newborn screening examinations as newly qualified midwives (NIPE trained)

Pre-registration midwifery programmes (Approved against the NMC Standards of proficiency for midwives (2009):

• There will still be some student midwives on education programmes approved against the 2009 Standards. Some of these students may have:

• not completed any examination of the newborn training (including NIPE screening)
• completed theory only

• completed full (theory and practice) elements (NIPE trained)

• This will depend on the curriculum at the approved education institution and can be ascertained by a discussion with the individual midwife and liaising with the relevant lead midwife for education. These midwives therefore may not be considered trained to undertake NIPE newborn screening.

Internationally educated midwives:

• Internationally educated midwives (IEMs) undertake a systematic physical examination of the newborn (SEN) NMC objective structured clinical examination (OSCE) as part of the required test of competence (ToC). While the OSCE has some principles and elements of a newborn NIPE examination via the simulated assessment, the OSCE standards and marking criteria are for SEN only (not NIPE newborn screening)

• Therefore, successful completion of the SEN OSCE would not constitute examination of the newborn and NIPE university accredited training or assessment. These midwives would not meet the NIPE screening programme training requirements and should not be considered trained to undertake NIPE newborn screening.

When employing midwives in any of the groups above, providers should be diligent and ensure all required training has been successfully completed before a midwife undertakes NIPE newborn screening examinations. 

The above guidance is a result of joint work between NHS England (NIPE screening programme, nursing directorate and workforce, training & education) and the Nursing and Midwifery Council) 

Nursing associates

The national programme considers that nursing associates would not be eligible to undertake examination of the newborn training or the newborn examination (as agreed by the NIPE Education and Training working group).

16.3 Non-university accredited training courses

If practitioners undertook a ‘short unaccredited examination of the newborn course’ (prior to 2016) then in line with national guidance at the time, practitioners need not undertake another course. In such cases, trust(s) would need to ensure they have a process to assess the practitioner competence and CPD and manage any risk associated with this approach.

Trusts should seek university accreditation or a formal university quality assurance process for any in-house NIPE practitioner training courses. In order to ensure appropriate expertise of assessors, any assessment should be undertaken by a university which provides an accredited examination of the newborn practitioner course. A quality assurance process should assess course content in line with required competencies, documentation (including teaching materials and lesson plans) and assessment processes. An annual review should include evaluation strategies, including feedback from students to inform future course planning.

Trusts are responsible for managing any risk associated with the approach where they enable practitioners to undertake the NIPE screening elements of the newborn examination by:

• offering, commissioning, or accessing a locally delivered examination of the newborn practitioner training course

• an unaccredited course

Note that short courses or in-house training may not be recognised by all providers, so this may pose a problem if practitioners were to transfer from one trust to another.

16.4 NIPE infant screening examination (usually undertaken at 6 to 8-weeks)

Doctors

Doctors can undertake all elements of the NIPE infant screening examination. However, not all trainee GPs undertake formal paediatric training, so it is important that NIPE is covered in their GP attachment (ST3 year). Practitioners need to take responsibility for competence and continuing professional development (CPD).

It is suggested that any GPs who have not undertaken the NIPE infant screening examination for some time or for whom it is a new activity should firstly refresh by working through the NIPE e-learning module and the NIPE handbook clinical guidance (this document) .

Post graduation doctors in training (PGDTs ) who have not completed a rotation in paediatrics or have not completed equivalent paediatric experience, and will be undertaking NIPE infant examination as part of their clinical practice, should complete the NIPE eLearning resource, work through the NIPE handbook clinical guidance (this document) and have a local programme of observation and supervision with an experience NIPE practitioner/ GP trainer to assure safe level of knowledge and practical skills. Use of the NIPE annual learning framework and peer review tools are strongly recommended. These can be adapted locally for use in primary care

In addition, a number of universities undertake one-off updates and clinical study days. These could be accessed by contacting the NIPE course leader at local university for details.

If the GP or practice feels it necessary, they may wish to consider the GP working with a colleague who is confident and competent to undertake NIPE practitioner in the practice to offer some mentorship or observed practice.

Physician’s associates

The ability to undertake the NIPE newborn or infant screening examination will depend on the training and qualification achieved. Physicians associates who require training should contact the relevant university provider to discuss enrolment for the examination of the newborn course.

Health visitors or practice nurses

The examination can also be carried out by a health visitor or nurse with suitable competency. To be considered competent, a health visitor or practice nurse is expected to:

• successfully complete a university-accredited examination of the newborn course, as elements of this would also be relevant for the infant examination

• undergo a locally agreed competency assessment by a practising NIPE examiner

Currently there are no courses that specifically focus on training for the 6 to 8-week NIPE infant examination. Current national guidance is that health visitors or practice nurses should undertake an examination of the newborn training, as some elements of the course are transferrable and relevant to undertake the infant screening examination.

16.5 Maintenance of competency

A local mandatory assessment of competency should be in place for all healthcare professionals who conduct NIPE screening examinations. The focus should not be on a minimum number of examinations, but on the quality of the examination performed. Local providers may choose to determine and set minimum numbers of examinations, but this is not a programme requirement.

The NIPE screening programme provides two supportive documents for qualified NIPE screening practitioners to record and evidence their theoretical knowledge and clinical skills in relation to the NIPE newborn screening examination. These documents are intended to be used to demonstrate evidence of continuing professional development (CPD). Providers can also use them to form part of a local competency assessment, These documents were devised for the NIPE newborn screening examination, but could be adapted for use locally for the NIPE infant screening examination.

It is recommended that all NIPE practitioners should use both the NIPE annual learning framework and peer review framework (links below)

The NIPE annual learning framework

The NIPE annual learning framework supports NIPE screening practitioners undertaking the NIPE newborn screening examination, to record and maintain their knowledge and understanding of NIPE screening. It is recommended that the following elements are completed annually:

• The NIPE e-learning module. Registration and a password are required to access the resource on the e-Learning for Healthcare platform.
• Attendance at a local annual NIPE update - this may include practical and theoretical assessment.
• Discussion of national standards, clinical pathways, training and education resources. This should include NIPE screening pathways and local referral processes for all those who undertake the NIPE examination (including midwives, nurses, doctors and other NIPE practitioners). This may form part of a local NIPE annual update.

There are further elements of the annual learning framework which are recommended (for example, the NIPE reflection) but the requirement to complete these, or the frequency of completion can be determined by local agreement. Please refer to the annual learning framework guidance document for further information.

NIPE annual learning framework and guidance on its use: Newborn and infant physical examination: annual learning framework - GOV.UK (www.gov.uk)

The NIPE peer review framework

The NIPE peer review framework supports NIPE screening practitioners to record and maintain the practical skills required to perform the NIPE newborn screening examination.

NIPE screening practitioners can use the peer review framework to demonstrate evidence of good clinical screening practice in line with current guidance.

Peer review is recommended by the national NIPE programme team (part of NHS England) to provide assurance that NIPE examinations are being completed in line with the NIPE screening programme handbook clinical guidance and the NHS NIPE e-learning module.

However, the regularity of peer review for NIPE newborn screening practitioners is a local decision. Please refer to the peer review framework guidance document for further information.

Peer review framework and guidance on its use: NIPE peer review framework - GOV.UK (www.gov.uk)

17. Quality assurance

The NIPE screening programme has a [defined set of standards] (https://www.gov.uk/government/publications/newborn-and-infant-physical-examination-screening-standards/newborn-and-infant-physical-examination-screening-standards-valid-for-data-collected-from-1-april-2021) that providers must meet to ensure local services are safe and effective. Quality assurance (QA) is the process of checking these standards are met and encouraging continuous improvement.

It includes:

• advising on the development of national quality standards

• monitoring how services meet (or fail to meet) standards

• providing expert screening advice for incident management

• facilitating quality review of services, including peer advice in supporting those involved in commissioning or providing screening services

Formal QA visits provide the forum for a peer review of the whole multidisciplinary screening pathway.

Screening quality assurance service (SQAS) teams make recommendations to providers and commissioners about what is needed to make sure the service meets the national service specifications and standards. They monitor and provide expert advice on the management of screening incidents and promote shared learning and examples of good practice at national, regional and local levels.

Participation in a formal process of QA is the responsibility of each local screening provider. The performance of local services is monitored in a variety of ways including the national data reports, at local trust meetings and within screening programme boards.

17.1 Key performance indicators (KPIs)

KPIs provide a way of measuring screening programme performance in specific areas. They contribute to the overall quality assurance of screening programmes but are not sufficient to fully quality assure, or performance manage, screening services. KPIs help local screening services identify potential or actual problems so remedial actions can be taken.

The 2 KPIs for the NIPE screening programme are NP1 and NP4 (S04).

17.2 Coverage data and failsafe

Coverage performance data helps ensure safe and inclusive local delivery of the NIPE newborn screening programme. This data is reported using S4N. Accurate and timely use of S4N supports complete cohort identification, failsafe processes and data reporting.

For babies screened by non-NHS services (independent or private), there is no obligation to complete S4N. However, it is good practice to record that screening has taken place by a private provider if this information is known. Please refer to the S4N user guides and training videos for further details on how to record this correctly.

17.3 NIPE KPI process guidance

NHS Trust KPI data for NIPE newborn screening is taken directly from S4N. Before the report is run, providers will have the opportunity to review and update S4N records (including outcome data) and provide mitigation information before data is submitted.

The NIPE programme produces quarterly reports that are available on S4N

Trusts should regularly review S4N failsafe reports and follow up babies identified as ‘not screened’ or who have not attended for post screen positive referral appointments. These  reports should be reviewed and discussed at local screening programme boards.

17.4 Screening safety incidents

Health professionals should use the managing incidents guidance if there are any problems during screening.

17.5 NIPE standards

NIPE screening programme standards provide a defined set of measures that providers must meet to ensure local programmes are safe and effective.

The NIPE national standards are available.

Trusts should make sure there are local systems for entering all relevant coverage and outcome data on to S4N including date of post screen positive referral appointment or review.

Trusts should make sure all NIPE screening and outcome data is inputted onto S4N to:

• assure functional failsafe processes

• assure  completion of the whole NIPE newborn screening pathway and identify gaps

• record programme performance

Where NIPE newborn screening examinations have taken place in primary care, local feedback mechanisms should be developed and agreed so screening results can be entered on S4N in a timely fashion.

A quarterly screening journey report is provided to commissioners and quality assurance teams which stratifies all NIPE screening data held on S4N by trust and region.

These provide a useful overview and quarter by quarter comparison of the whole NIPE newborn screening pathway. NHSE publishes KPI data reports quarterly. 

18. SMaRT4NIPE(S4N) National IT system

The national IT system for NIPE is SMaRT4NIPE (S4N) and is a clinical management system designed for use at the point of care. 

S4N enables management of babies’ records throughout the screening and referral pathway and provides data to support failsafe, audit, and service development.

This is enabled by:

• interfacing with the English birth registration system to create a unique clinical NIPE screening record for each baby
• identifying the population eligible for screening and supporting local failsafe processes, therefore minimising the risk of babies missing screening or any part of the screening or referral pathway
• providers managing babies through the entire screening journey recording assessment timescales for babies with screen positive results
• storing an ongoing record of screening results, which can be downloaded directly from S4N
• creating consistency of screening data across NIPE screening in England
• facilitating information sharing with other services such as CHIS (via the national events management system – NEMS)
• enabling providers to generate referral letters (if used)
• enabling providers to record outcomes of screening referral appointments which inform performance data reports and support local failsafe processes
• enabling local regional and national data and reporting and audit

18.1 Eligibility for BCG (Newborn Blood Spot risk factor) 

Eligibility for BCG is a national risk factor, and mandatory field on the S4N national IT system. This is part of the Newborn Bloodspot Screening Programme SCID pilot.

It is the responsibility of the maternity services to ensure that staff are trained to assess eligibility for BCG correctly. However, in order to further assist NIPE screening practitioners, links are provided via the information icon on the S4N system to relevant sources of information on BCG eligibility (The Green Book, and World Health Organisation (WHO) estimates of prevalence by country).

18.2 Access

All NIPE practitioners should have locally enabled access to S4N. Information governance processes relating to legitimate access to the ‘Live’ version of the system is managed by the NIPE Lead and Trust S4N administration users. Some Child Health users have limited access permitted to S4N which is also managed via their local NIPE lead (read only access with reporting capabilities).

Link for  S4N ‘LIVE’

There is a training site provided to support education and training for new and existing users so that they can become familiar with the functionality and content within the system. Access to each provider’s training site is via the NIPE system supplier helpdesk (see below):

Link for  S4N training

18.3 Support

There are a number of detailed user guides available on the ‘Resources’ page of S4N and a link to access short training videos on the Vimeo platform S4N training videos.

Any query, change request or issue should be directed to the NIPE screening IT helpdesk (hosted by the system suppliers)

Email: NIPE[email protected]

Phone: 08450 705 902

The helpdesk is open from 7am to 7pm, 365 days a year. If any issue remains unresolved, please contact the NHSE newborn screening team directly.

System unavailability

Occasionally, the S4N system is not available for technical reasons. A local standard operating procedure (SOP) should be in place to ensure that NIPE newborn screening continues, and any outstanding results are inputted into S4N as soon as the system becomes available.

To make this process easier, a template document is available to download in the ‘Resources’ section of S4N, which mirrors the individual set-up of each provider within S4N (local risk factors and local data items). Copies of this can be printed off, and used to manually record screening results when the system is unavailable.

Unconfirmed gestational age - (gestational age (GA) field)

There are some scenarios where it is difficult for S4N to reflect the clinical picture exactly for recording purposes.

For example, a baby(s) with an unconfirmed gestational age should be managed on a case-by-case basis. If it is documented in the maternal or baby record by a clinician that the baby’s gestation has been estimated, or there is a ‘working’ EDD in the absence of a scan, this ‘best guess’ timescale could be used in S4N. However, if there is no gestational age documented a local discussion needs to take place and a case-by-case approach applied, appreciating that a gestational age assessment may be required for other screening programmes or newborn care.

The overall priority is to complete the NIPE newborn screening, and that any screen positive results including follow up are actioned.

Management of records when babies are transferred to specialist units not using S4N

Babies transferred to specialist units not using S4N should have their records retained on the current provider worklist with a ‘SU’ (specialist unit) flag to indicate that screening is still pending/outstanding.

A local process should be in place to communicate with the specialist children’s unit that NIPE newborn screening is outstanding, and if possible, once screening is completed the record should be updated.

If, after all efforts have been made, it has not been possible to determine if newborn screening was completed with the specialist children’s unit within the usual timescales, and/or the baby has been discharged home, then the original site can inactivate the record using the ‘Not Done – Missed/incomplete screen’ status, with a note in the comments box to document this information. This will remove the record from the current provider’s worklist.

A follow up letter should be sent from the provider to the baby’s GP, explaining that there is no assurance that newborn screening has been completed, and requesting the GP to undertake infant screening (if appropriate).

As with any issues regarding missed screening, these should be discussed with your quality assurance advisors (QA) and/or Screening and Immunisation Team (SIT).

We appreciate that these records will be included in your KPI data, however, they can be recorded as mitigations against performance targets.

19. Acknowledgements

We gratefully acknowledge the input of professionals involved (including members of the NIPE screening programme expert working groups) and their contribution to the content of this handbook. If you would like further information, please contact the NHSE newborn screening team

[IEMs] Internationally educated midwives

*[NHSE] NHS England

[OSCE] objective structured clinical examination

[RTP] Return to practice

[ToC] Test of Competence

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Glossary

Hypospadias- is a birth defect (congenital condition) in which the opening of the urethra is on the underside of the penis instead of at the tip

Disorders of sex development (or differences of sex development)- encompass a group of congenital conditions associated with atypical development of internal and external genital structures. These conditions can be associated with variations in genes, developmental programming, and hormones. They can be associated with endocrine conditions which may need investigation and treatment.

Preterm birth- also known as premature birth, is the birth of a baby at less than 37 weeks gestational age

Impalpable testis – a testis that cannot be palpated

Undescended testis – a testis that is either impalpable or, although palpable, has not fully descended into the scrotum

Prader Willi syndrome - a genetic disorder that affects many parts of the body and their growth. It causes mental and behavioural problems

Prune belly syndrome - is a rare congenital disorder of the urinary system, characterized by a triad of symptoms. The syndrome is named for the mass of wrinkled skin that is often (but not always) present on the abdomen of those with the disorder.

Congenital adrenal hyperplasia (CAH) – is a group of inherited conditions that are present at birth (congenital) where the adrenal gland is larger than usual (hyperplasia) and there is a reduction of certain hormone production.