Persistent ductus arteriosus
The ductus arteriosus is a blood vessel which links the pulmonary artery with the aorta
during the foetal period. During the foetal period, the vessel is kept open by
prostaglandins because circulation through the lungs is then not necessary. After the
birth, the blood vessel closes quickly, usually during the first day, but the closure is often
delayed in premature children. In Sweden, approx. 60 per cent of the extremely
premature infants are treated for persistent ductus arteriosus (PDA) and the shorter the
length of the pregnancy, the more common it is [4, 26].
When the flow resistance in the pulmonary circulation is reduced, with PDA there is a
leakage of blood from the aorta to the pulmonary artery (known as a left-right shunt). If
the shunt blood flow becomes significant, symptoms appear in the form of greater
breathing and heart rates, a greater need for oxygen and sometimes respirator-dependent.
The child can also suffer heart failure, apnoea (interrupted breathing), a drop in blood
pressure, kidney failure and digestion problems. PDA has been associated with intraventricular brain haemorrhage (IVH), necrotising enterocolitis (NEC, inflammation of
the bowel) and chronic lung disease (bronchopulmonary dysplasia, BPD) [64].
Although PDA is common and can have significant consequences for the child, the
scientific support is unclear when it comes to the most suitable diagnostics and treatment
[65]. As a rule, the diagnostics are based on ultrasound examinations of the heart
(echocardiography) but there are no uniform criteria for when PDA becomes significant
and must be treated [66, 67]. PDA can be closed pharmacologically or surgically, but the
treatment has been questioned because spontaneous, delayed closure of PDA is common
[64, 68]. Pharmacological treatment is also less effective among the extremely premature
infants than in the more mature children [69].
Preventative measures
The Swedish National Board of Health and Welfare’s assessment
o
o
The preventative measures for persistent ductus arteriosus (PDA)
ought to include the treatment of pregnant women threatened with
premature birth with corticosteroids because, as well as effects on lung
immaturity, it also reduces the occurrence of significant PDA.
Prophylactic closure of ductus arteriosus (with medicines or surgery) is
not recommended.
The assessment is based on systematic charting.
In order to prevent significant PDA, intervention ought to begin before the birth or
during the first day of life. Treatment with antenatal (administered
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before the birth) corticosteroids is recommended for pregnant women threatened with
premature birth because it has been shown to reduce the occurrence of significant PDA in
children who are born prematurely, even if not all scientific studies have been able to
show this effect [11, 70, 71]. Corticosteroids reduce the sensitivity to prostaglandins
which otherwise keep the ductus arteriosus open [72-74].
Where extremely premature infants are concerned, prophylactic treatment with
medicines has been shown to reduce the occurrence of significant PDA as well as reduce
the need for surgical duct closure. However, there is a risk of side effects and unclear
safety aspects. Nor has it been shown that prophylactic treatment lowers the mortality rate
or improves the psychomotor development in the long term [75-78]. The treatment cannot
therefore be recommended at the moment.
Prophylactic duct ligation (within 24 hours of the birth) in children with an extremely
low birth weight (less than 1 000 g) has not been well studied. Studies have shown that
with the operation there was no difference in mortality, severe pre-maturity retinopathy
(ROP) or serious IVH compared with the standard treatment. The occurrence of NEC did
fall on the other hand. The scientific support as to whether prophylactic duct surgery is
linked to the development of BPD is not unequivocal [79-82].
Not cutting the umbilical cord until 30-120 seconds after the birth and early CPAP
treatment (continuous positive airway pressure) facilitate the circulation adaptation at the
birth and both of these interventions have been shown to reduce such ill-health which is
also linked with PDA [14].
PDA diagnostics
The Swedish National Board of Health and Welfare’s assessment
o
PDA diagnostics ought to take place using echocardiography where the
first examination is done within one to three days.
The assessment is based on consensus between the chairpersons of the
expert groups.
Echocardiography is the crucial diagnostic tool and all extremely premature infants ought
to undergo an ultrasound of the heart to assess PDA, the first time within one to three days
of the birth. The clinical picture and x-ray finds can also be important in terms of
diagnosing and assessing PDA. Low diastolic blood pressure can be a sign of PDA, as can
heart murmurs at the time of auscultation and brisk peripheral pulses, particularly early on
in the course of events.
The echocardiographic assessment is a qualified task requiring knowledge and
experience. The size of the ductal shunt depends on the duct width as well as the pressure
and resistance conditions in both the pulmonary and systemic circulation, which
complicates the assessment. The examination conditions are also often difficult with very
limited echocardiographic windows. An important
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aspect of the diagnostics is also that a ductus-dependent heart defect needs to have been
precluded before with any duct-closing treatment can be relevant.
Despite a large number of studies within the field, both strong scientific support for
or consensus surrounding the diagnostic criteria are absent [2, 4, 8]. However, the
following echocardiographic criteria can be used as signs of haemodynamically
significant PDA:
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Duct width exceeds 1.5 mm.
Left atrium is enlarged, which can be measured using the diameter of the left atrium
(LA = left atrial) and the aortic root (Ao = aortic). An LA/Ao quota greater than 1.5
is a sign of a significant shunt and values exceeding 2 indicate a severe shunt.
The child has low or reversed diastolic blood flows in the descending aorta,
mesenteric artery or cerebral artery (a strong indication).
There is a diastolic forward flow in the pulmonary artery branches. An end-diastolic
velocity exceeding 0.2 m/sec is a sign of a significant PDA, and a velocity
exceeding 0.5 m/sec indicates a severe shunt.
A duct examination usually also includes an assessment of the size of the left chamber.
However, for the extremely premature infants, there is currently no information on the
reliable normal values.
In recent times, bio markers such as pro-BNP, a peptide that is excreted from the
myocardium when loaded, have started to be used in the assessment [83]. However,
experiences and the scientific support are currently insufficient to give any
recommendation.
Treatment of PDA
The Swedish National Board of Health and Welfare’s assessment
•
Early treatment of PDA ought to be considered for the occurrence of one or
more echocardiographic criteria and clinical symptoms of significant PDA. If
there is intervention,
o pharmacological treatment with Ibuprofen should be used as a first
choice for haemodynamically significant PDA;
o surgical treatment (ligation of PDA) should be used restrictively, but can
be considered if therapy fails, there is a late recurrence or if there is a
contraindication for pharmacological treatment.
The assessment is based on systematic charting and consensus between the
chairpersons of the expert groups.
PDA treatment means an intervention that usually begins 1-14 days after the birth and
which aims to put an end to symptomatic PDA. Pharmacological or surgical closure of
the ductus constitutes the basis of the treatment arsenal. The proportion of those who do
not respond to pharmacological treatment is higher among children with a lower
gestational age (level of maturity). The spontaneous PDA closure rate is also at its
lowest in this group.
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As well as pharmacological and surgical treatment, other interventions can also have an
effect. CPAP treatment has low scientific support when it comes to closure of the PDA,
but is supported by physiological knowledge and tried and tested experience. Fluid
restriction has some support in observational studies and can be considered if it is possible
to ensure adequate nutrition. Loop diuretics can impair ductus closure and these medicines
ought therefore to be used only for children with clear clinical signs of over-circulation in
the lungs and other signs of acute heart failure [84-87]. Blood transfusion also does not
facilitate the closure of the PDA and therefore cannot be recommended [88].
Pharmacological treatment with
cyclo-oxygenase inhibitors
Pharmacological treatment of PDA currently takes place using non-selective cyclooxygenase inhibitors. Ibuprofen is recommended as the first choice preparation for PDA
before Indomethacin, even if the preparation is comparable as regards the effect on the
PDA (three out of four children respond with closure of the PDA), treatment failure,
recurrence and PDA requiring surgery (approx. 10 per cent) [89]. The recommendation is
based on Ibuprofen’s more favourable side effects profile (lower risk of NEC, oliguria and
kidney impairment) [89-91].
Ibuprofen is given intravenously, usually for three days (one dose per day) [92, 93] and
after this treatment, another round of treatment may be appropriate. There is some
scientific support for a second round of treatment leading to the closure of the ductus
arteriosus [94-96]. The clearance of Ibuprofen increases relatively rapidly with the child’s
age and a higher dose may then be more effective [97-99]. Enteral (through the
gastrointestinal tract Ibuprofen treatment appear to be just as effective as intravenous [89],
but enteral administration can increase the risk of gastrointestinal haemorrhage [76].
Early pharmacological treatment of PDA (during the first day of life) has not been
shown to be able to reduce serious neonatal morbidity such as BPD, NEC or ROP
compared with later treatment (at five to seven days of age) [100].
Surgical treatment
One in four children who were part of the EXPRESS study (extremely preterm infants in
Sweden study) was operated on for PDA. The operation took place at a median age of 18
days after the birth, and the reason in 65 per cent of the cases was that pharmacological
duct closure had failed. The remaining proportion of the children was operated on as the
primary option. An examination of all children in EXPRESS who were treated surgically
for PDA showed that the nutrition was inadequate at the time of the operation [101].
Surgical treatment of PDA can be considered if therapy fails or there is a recurrence
following pharmacological treatment. Surgical treatment may also be appropriate if the
child shows symptoms which mean that pharmacological treatment may be considered to
be relatively or fully contraindicated (greater tendency to haemorrhage and recent or
ongoing internal haemorrhage in the brain or the gastrointestinal tract) [64]. Where there
is IVH grade I (milder haemorrhage), repeating the ultrasound examination of brain after
one day is recommended and, if the haemorrhage remains unchanged, it is not considered
to constitute a contraindication for pharmacological treatment. If blood is shown in the
ventricular system, the assessment ought to be assessed more cautiously as regards
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starting pharmacological treatment. A grade IV haemorrhage (larger haemorrhage) is
judged to be a contraindication for pharmacological treatment.
An impaired neurosensory capacity has been reported among children who have had
an operation for PDA [102, 103]. However, available data is not sufficient to determine
whether the link is due to the fact that the group who had operations were particularly ill
and vulnerable, or due to the fact that there are risk factors that are specifically
associated with the surgical intervention. Paralysis of the vocal cords (caused by damage
to recurrent nerve) [104] and postoperative blood pressure drop [105] have also been
shown to be common complications, and ductus surgery therefore ought to be carried
out on a restrictive basis on extremely premature infants.
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The immature brain
The immature brains of extremely premature infants have a greater risk of being affected
by damage and deviating development. During the neonatal care period, several factors
can affect and disrupt the brain’s growth because it is in a dynamic development phase.
This means that the children have a greater risk of being affected by neurological and
cognitive function impairments as well as by neuropsychiatric condition such as ADHD
and autism. These conditions are associated with complications that arise during the
pregnancy and the neonatal period [106-108].
It has been shown that improved neonatal care reduces the brain injuries [3] and it is
therefore extremely important for the initial care of these children to take place at
hospitals that have substantial experience of and competence in neonatal specialist care.
The Swedish National Board of Health and Welfare’s assessment
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The care is of substantial significance to the risk of developing brain
haemorrhages and damage in the brain’s white matter. It is therefore extremely
important for the care to take place at units that have substantial experience of
extremely premature infants.
The brain ought to be continuously assessed to diagnose injuries and deviations as
well as to inform the parents of the child’s condition and prognosis. The
assessment ought to take place with the help of
o clinical examinations;
valid examination methods to detect brain injury, such as ultrasound,
electroencephalography and magnetic resonance tomography.
The businesses ought to draw up procedures to reduce risk factors that are linked
to the development of brain injuries.
Neurological and cognitive follow-ups ought to take place until school age in
accordance with the national follow-up programme.
The assessment is based on systematic charting and consensus between the
chairpersons of the expert groups.
Brain injuries
The immature brains of extremely premature infants develop milder injuries such as
haemorrhages and matter loss. The brain injuries which are diagnosed in the neonatal
period in the first instance are known as intraventricular haemorrhages (IVH), which arise
in an area from which nerve cells migrate [109-111]. In 90 per cent of the cases, IVH
occur during the first week and usually already during the three first day of life. The size
of the haemorrhage affects the prognosis where milder haemorrhages (IVH grades I-II)
often do not lead to a great risk of a later impact, while larger haemorrhages
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(IVH grades III-IV) involve a high risk of development of subsequent function
impairments.
Approx. 10-15 per cent of the extremely premature infants are also affected by
haemorrhages in the cerebellum. The haemorrhages increase the risk of subsequent
cognitive and motor function impairment.
The risk of injuries in the brain’s white matter is also higher for these children. Cystic
periventricular leukomalacia (cPVL) leads to a loss of matter in the brain which is linked
to the development of motor and perceptual (the ability to perceive) function
impairments. More diffuse white matter injury is also relatively common.
Improved neonatal care, which is adapted to the very smallest children, has led to a
fall in the occurrence of IVH grades III-IV and cPVL (and thereby also the risk of
cerebral palsy), but 15-20 per cent of the extremely premature children [3] are still
affected.
Methods of detecting brain injuries
Neurological assessment can be difficult to do on extremely premature infants. The
child’s neurological function (such as muscle tone, activity, spasms, sleep and alertness)
ought to be clinically assessed.
Ultrasound examinations of the brain can give direct information on morphological
injury. Both IVH and PVL can be detected, while substantial experience of
haemorrhages in the cerebellum is required for them to be visible [109, 111, 112].
Ultrasound examinations ought to be performed on all of these children as a matter of
routine within the first 3 days, after 3-7 days and after 14-21 days when the child has
reached the estimated full term as well as in addition to that where necessary Where
there is an increasing accumulation of cerebrospinal fluid (post-haemorrhagic ventricular
dilation, PHVD) and the development of hydrocephalus (water on the brain), more
frequent ultrasounds ought to take place and early contact be established with a
neurosurgeon. The circumference of the child’s head ought to be routinely measured
each week or more often if necessary.
More diffuse white matter injury can be diagnosed using with magnetic resonance
tomography (MR). After just one day, the brain activity registered with EEG
(electroencephalography) or amplitude-integrated EEG (aEEG, a modified EEG), has
been shown to predict a later prognosis. It shows the importance of the early brain
injury’s role in the child’s future prognosis. EEG and aEEG examinations have also
shown that subclinical (not fully developed) epileptic attacks are relatively common,
primarily in children with IVH [109, 111, 112].
An ultrasound or MR of the brain ought to take place at full term or in the weeks
before this. The injuries that can be seen at this time are often predictors of later
problems but unfortunately, a normal examination does not always predict normal
development. The examinations show that extremely premature infants, irrespective of
whether or not there is a brain injury, have a lower brain volume when they have
reached the full estimated term compared with full-term children. However, the
scientific support for whether ultrasound [113, 114] or MR [115] gives the most suitable
predictive information is not unequivocal.
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Prevent brain injuries - risk factors
There are no specific interventions to treat brain injuries that have arisen without focus
being primarily on running the care in such a way that the children’s exposure to risk
factors that are linked to brain injuries is minimised as far as possible. These risk factors
include:
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inadequate and immature control of cerebral blood flow
instable circulation
unsuitable blood gas levels
lung immaturity
chronic lung disease
interrupted breathing (apnoea)
inadequate nutrition
pain and stress
surgical intervention
persistent ductus arteriosus (PDA)
transportation
high bilirubin levels
infection
inflammation.
Some of the injuries that arise can be related to the children’s relatively low and immature
cerebral blood flow regulation. The child’s blood gas levels ought to be monitored
continuously because high carbon dioxide levels lead to a greater risk of IVH while low
levels increase the risk of cPVL and cerebral palsy [35]. Simple care measures such as
changing nappies and adjusting the position of the tube can also affect the blood
circulation and oxygenation of the brain. Lung immaturity and chronic lung disease are
well-known risk factors for brain injury [116]. One of the most important results of giving
mothers antenatal steroids so that the foetus’ lung function matures is that this also leads
to a reduction in the risk of IVH.
Both acute and more chronic nutrition problems are common in very premature
children, and this increases the risk of injuries in and inadequate development of the brain.
Hypoglycaemia is associated with impaired development, but moderate hyperglycaemia
during the first day of life also involves a risk factor for white matter injury and death
among these children [117]. A high sodium intake as well as the administration of
hyperosmolar solutions have been shown to be linked with the development of IVH and
ought therefore to be avoided [118].
Frequent pain and stress are linked to reduced brain growth and a change in cerebral
organisation and can lead to both acute, physiological effects and permanent changes in
the response to pain and the central nervous function [119, 120]. Painful measures should
always be minimised. Surgical intervention and transportation also increase the risk of
brain haemorrhages.
High bilirubin levels can lead to brain injuries caused by kernicterus, although this is
uncommon in Sweden. On the other hand, milder bilirubin encephalopathy (reversible
brain damage) is more common and can, for example, express itself in terms of
pronounced tiredness. The majority of extremely premature infants need light treatment to
lower their bilirubin level, but it can be difficult to detect symptoms and see the
characteristic yellow coloration of the skin in these children.
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Infections
Preventing infections is a key factor in all forms of care. It is particularly clear in the
care of extremely premature infants because their defence against infections is underdeveloped. At the same time, the care of these children, like all intensive care, leads to
considerable risks of infection, among other things through many invasive measures and
the use of advanced technical equipment.
The Healthcare and Medical Treatment Act (1982:763) requires care to maintain a
good standard of hygiene and the hygiene requirement is also regulated in The Swedish
National Board of Health and Welfare’s provisions and general advice (SOSFS
2007:19) on basic hygiene within healthcare and medical treatment, etc. There is also a
requirement regarding measures to prevent infection in The Swedish National Board of
Health and Welfare’s provisions and general advice (SOSFS 2011:9) on management
systems for systematic quality work.
Prevent the spreading of infections
The Swedish National Board of Health and Welfare’s assessment
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Units that care for extremely premature infants ought to have safety procedures
which aim to prevent the occurrence of infections and spreading of infection.
Extremely premature infants are often cared for at several hospitals and the care
providers therefore ought to coordinate their hygienic procedures and the
information concerning the risks of infection.
In the systematic quality work, it is necessary to register care-related infections
with follow-up and feedback.
The assessment is based on hygiene requirements in the Healthcare and
Medical Treatment Act (1982:763) and The Swedish National Board of Health
and Welfare’s provisions (SOSFS 2007:19 and SOSFS 2011:9) as well as on
knowledge bases and reports from The Swedish National Board of Health and
Welfare and recommendations from the Medical Products Agency.
Care-related infections are a documented problem at several neonatal units.
Shortcomings in care hygiene have been noticed particularly in connection with the
spreading of multiresistant bacteria, including ESBL-forming gram-negative bowel
bacteria [121].
Crowded care spaces can increase the risk of care-related infections, which is
described in SOSFS (2011:9). The extremely premature infants ought to be cared for in
appropriate premises, which is not always the case today. The technical medical
equipment requires more room and the fact that parents assist with the care means that
each child needs considerably more space than before.
The parents’ assistance is an important and key part of Swedish neonatal care and the
businesses must therefore have procedures to inform the parents of the hygiene rules
that apply. Basic hygiene rules must always
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be applied to point-of-care work to prevent the spreading of patient and hospital flora.
Since an extremely premature child is cared for at several different hospitals, coordination
is needed between different care providers’ hygiene procedures.
The use of antibiotics within neonatal intensive care is extensive. Antibiotic treatment
often starts before the suspicion of an infection is confirmed because serious infections
are relatively common and the symptoms are usually difficult to interpret when the course
of events starts. The Medical Products Agency has produced recommendations for the
treatment of serious neonatal infections [122] where you can read things like: “antibiotic
treatment must be designed optimally with regard to the choice of antibiotic and the
period of treatment to minimise the risks of treatment failure and resistance
development”.
Read more
Information from the Medical Products Agency:
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Recommendations for the treatment of neonatal sepsis (Neonatal sepsis - ny
behandlingsrekommendation (English: Neonatal sepsis - new treatment
recommendation). Information from the Medical Products Agency 2013; 24(3):1525).
The importance of hygiene procedures to prevent infections and the reasonable use of
antibiotics (Riesenfeld-Örn, I, Aspevall, O. Vårdrelaterade infektioner, antibiotika
och antibiotikaresistens (English: Care-related infections, antibiotics and antibiotic
resistance). Information from the Medical Products Agency. 2013; 24(3):77).
Information from The Swedish National Board of Health and Welfare:
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Inventory of problems and proposed measures for the spreading of infection within
Swedish neonatal healthcare (Smittspridning inom svensk neonatalsjukvård
probleminventering och åtgärdsförslag. The Swedish National Board of Health and
Welfare; 2011).
A knowledge basis for the prevention of care-related infections
(Att förebygga vårdrelaterade infektioner - Ett kunskapsunderlag. The Swedish
National Board of Health and Welfare; 2006).
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Nutrition - a precondition for
growth and development
Extremely premature infants often have an inadequate nutritional intake and
demonstrate inhibited postnatal growth, which can be explained partly by undernutrition
[123]. The children are unique when it comes to the need for nutrition and the nutrition
during the first months of life plays a major role. In order to achieve normal growth and
development, premature children ought to grow in accordance with the curve for normal
foetal growth [124]. Foetal growth is much faster than that of the newborn, which leads
to extremely premature infants needing more nutrition than full-term children. A child
who is born after 24 full weeks of pregnancy have passed and weighs around 700 g, for
example, is expected to increase its body weight by five times over the next 3 ½ months
of care in neonatal unit. The nutrition must not only result in normal growth, i.e. weight,
height, head circumference and body composition, but also in normal growth and
maturation of all organ systems.
Nutritional intake and monitoring of the
growth
The Swedish National Board of Health and Welfare’s assessment
•
•
The child’s nutritional intake needs to be safeguarded through individual
nutritional calculation and continuously assessed in order to detect a nutritional
shortcomings, excess nutrition or disturbances to the metabolism.
o Recommended nutritional intake is shown in Appendix 1.
o the child’s weight ought not to drop by more than 5-10 per cent in the
first three days of life.
The child’s nutrition and growth ought to be followed up after discharge.
The assessment is based on consensus between the chairpersons of the expert
groups.
Extremely premature infants are at great risk of being undernourished during the care
period at a neonatal unit [123, 125]. In the short term, this is linked with poor growth
and in the long term with severe complications such as vision impairment, development
disorder and cardiovascular disease [6]. It is not possible to give any general
recommendations for nutritional intake because the need for nutrition varies and
depends on the week of pregnancy in which the child is born, postnatal age, state of
health, weight, growth pattern, nutritional status and the share of enteral (through the
gastrointestinal tract) or parenteral (intravenous) nutrition. The nutritional intake ought
therefore to be calculated on an individual basis at regular intervals. Recommended
nutritional intake is presented in Appendix 1 [126-128].
The extremely premature infant will rapidly form substantial accumulated nutritional
shortcomings if the prescribed nutritional intake is lower than that which is
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recommended, and it is therefore necessary to think about the nutritional intake on a daily
basis [129]. The nutritional intake is at its lowest during the first four weeks of life [123]
and the weight loss at its fastest during the first week, but the children often demonstrate a
progressive growth inhibition during the first four weeks where weight, height and head
circumference are concerned. During this period, the children are usually given a
combination of parenteral and enteral nutrition. Other undernutrition risk periods are at
the time of surgery [101], when the child has been discharged from the neonatal unit and
when the child changes from tube nutrition to nursing because that is when it becomes
more difficult to estimate the nutritional intake. Nursing is important, however, and breast
milk is the best food for the children. After going home, the follow-up and advice to the
parents vary, and the rate of growth can fall rapidly if the child is not mature enough to
regulate his own intake or has difficulties eating. In order to optimise growth and
development, it is important for the children to be followed up at outpatient appointments
within neonatal outpatient care or in cooperation with primary care or specialist clinics
[130].
There are no evaluations of the effect of the upper limits for a safe intake of many
nutrients. An unnecessarily high intake of iron (at the time of blood transfusions for
example) which cannot be excreted from the body has been linked to impaired growth and
an increase in the risk of infections and retinopathy of prematurity, among other things
[131, 132].
Growth measurements
Weight is measured on a daily basis if possible, while height and head circumference can
be measured once a week. Any growth deviations are clarified by plotting the growth
against the normal growth curve for healthy foetuses. During the first three days of life
there will be an initial weight loss which ought to be no more than 5-10 per cent, but after
that, the child is expected to grow in parallel with the intrauterine growth curve. Children
who have full enteral nutrition and enjoy good and stable growth can be weighed every
other day [133].
Blood tests
During the first two weeks of life, the level of glucose and electrolytes as well as the acid
base status in the blood need to be followed up. This applies in particular if the child is
clinically unstable. Triglycerides, urea and creatinine also need to be followed up. The
indication for a blood test must be weighed against the negative effects such as pain and
anaemia.
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Parenteral nutrition
The Swedish National Board of Health and Welfare’s assessment
o
Parenteral nutrition solution ought to be given as soon as possible
(within a few hours) after the birth, along with an early start to an
enteral supply of breast milk.
The assessment is based on systematic charting.
Parenteral nutrition is an invasive treatment which involves a high risk of complications
(particularly care-related infections) and there should therefore be substantial
experience, well-functioning procedures and careful monitoring of the treatment.
The child’s rapid growth requires a very high supply of energy and nutrients already
during the first hours of life. This can take place using a concentrated parenteral
nutrition solution through a central vein along with a rapid increase in the enteral supply
of breast milk [134, 135].
Fluid and salt balance
The Swedish National Board of Health and Welfare’s assessment
o
The fluid and salt balance ought to be carefully controlled, particularly
during the first week of life.
The assessment is based on consensus between the chairpersons of the
expert groups.
An adequate fluid and salt balance is necessary to avoid disorders such as dehydration,
overhydration, electrolyte disorders and metabolic acidosis, which in turn can lead to a
rise in mortality and morbidity [45].
In extremely premature infants, the body’s total fluid content corresponds to 90 per
cent of the birth weight The children’s kidney function is immature, limited and borders
on kidney failure levels. These special circumstances mean that there is a fine balance
between fluid and salt; disorders are common and small imbalances can have major
consequences, primarily during the first few weeks of life.
An early supply of phosphate, calcium and magnesium ought to be given if possible.
Low quantities of sodium and potassium can be tolerated in connection with satisfactory
nutrition. The fluid and sodium chloride intake ought to be limited during the first three
days of life (Appendix 1). Fluid sometimes also needs to be restricted later on for
clinical reasons, and then it is particularly important to provide adequate nutrition by
using sufficiently concentrated nutritional products.
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Enteral nutrition
The Swedish National Board of Health and Welfare’s assessment
•
The businesses ought to have written procedures for the handling of breast milk
and bank milk, as well as access to a breast pump. The giving of breast milk
should
o be the first choice for enteral nutrition
o be enriched on an individual basis to optimise the nutrition
o be encouraged in the form of nursing following discharge.
The assessment is based on systematic charting.
The gastrointestinal tract becomes functionally mature more quickly if the enteral supply
is started early on, which also reduces the need for parenteral nutrition and thereby the
risk of invasive infections [136, 137]. The enteral nutrition is escalated with the aim of
achieving full enteral nutrition within 14 days of the birth.
If possible, the child ought to be given drops of its mother’s colostrum or breast milk
by mouth because it gives protection against infection and positively stimulates the sense
of taste [138]. Breast milk is superior as nutrition for extremely premature infants, partly
because it reduces the risk of developing necrotising enterocolitis (inflammation of the
bowel) [139]. If the mother does not have her own milk, bank milk (donor milk) is the
best alternative (following the parents’ consent) [139]. Only in the third instance can it be
relevant to use infant formulas for children who are born prematurely. To promote
nursing, it is good if the first milk is obtained within six hours of the birth if possible, as
well as continuing with milkings at least six times a day [140]. Mother and child skin-toskin contact supports the production of milk and the child’s nursing behaviour [141]. The
parents ought to be given information, preferably before the birth, on the importance of
colostrum and breast milk as well as the importance of early milking.
Rearing with non-fortified breast milk, primarily bank milk, involves a major risk of
protein shortfall as well as lack of energy and various minerals. In order for the nutritional
intake via breast milk to be sufficient, the milk needs to be analysed and almost always
fortified [142, 143]. The fortification ought to be individualised because it reduces the risk
of under and overnutrition. The easiest way of creating a suitable fortification is to
regularly analyse the breast milk content for protein, fat and carbohydrates [144].
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Retinopathy of prematurity
Extremely premature infants are at risk of being affected by the eye disease called
retinopathy of prematurity (ROP) which is due to a change in the blood vessel
development in the eye. The vascular disease can cure itself and the visual impairment
can be prevented through timely laser treatment or cryotherapy. Approx. 30 per cent of
extremely premature infants develop serious ROP which requires acute treatment to
prevent the retina from becoming loose and vision being lost (less than 1 per cent of the
children are blind) [5].
The earlier the child is born, the greater the risk of being affected by ROP, which is
due to the less-developed blood vessels in their retina. The supply of oxygen to these
children ought to remain at a suitable and stable level because the children are sensitive
to both high (hyperoxia) and low oxygen pressure in the blood. Hyperoxia and major
fluctuations in the oxygenation early on in life are associated with a greater risk of ROP.
The risk of ROP is reduced with lower exposure to oxygen, but too severe a restriction
is associated with an increase in mortality [37]. A more detailed description of oxygen
treatment is given in the guideline’s chapter on lung diseases and breathing support.
Undernutrition and protein shortfall have been linked to an increase in the risk of
ROP, and there is some scientific support for the fact that a high intake of iron also
increases the risk [131, 132].
Diagnostics and treatment
The Swedish National Board of Health and Welfare’s assessment
•
•
There ought to be procedures for regular ophthalmological examinations to
diagnose retinopathy of prematurity (ROP) early on.
Advanced ROP (stages 3-4) requires acute eye surgery (usually laser) and this is
done under general anaesthesia.
The assessment is based on the Swedish Ophthalmological Society’s guidelines
and consensus between the chairpersons of the expert groups.
Screening for ROP ought to include all extremely premature infants and be carried out
in accordance with Sweden’s Ophthalmological Society’s guidelines [145]. The
examination result ought to be registered in the national quality register (SNQ with its
SWEDROP register) [146]. An eye examination for the assessment of ROP is a painful
procedure and the pain is prevented through eye drops along with non-pharmacological
support [147, 148].
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15
Read more
•
16
“En kunskapsöversikt om prematuritetsretinopati” (English: A knowledge overview
of retinopathy of prematurity) (Holmström, G. Retinopathy of prematurity, state of
the art - document 2012. [quoted 201407-01]; Available from:
http://swedeye.org/wp-content/uploads/ROP-2012.pdf).
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Treatment of pain
Children who are born prematurely and who are cared for at neonatal intensive care
units are often exposed to pain due to their immaturity, different states of health,
complications and care procedures. The pain can have negative consequences in both
the short and the long term because the immature child is in a sensitive period of strong
growth and differentiation of the central nervous system. Therefore, the number of
painful interventions ought to be minimised and painful conditions be treated.
Pain ought in the first instance to be dealt with using non-pharmacological treatment,
but there are circumstances when the pharmacological treatment of pain is necessary.
The majority of the medicines used are not fully tested and documented (effect, safety
and dose) for extremely premature infants, so the treatment is based largely on tried and
tested experience rather than scientific support. The fact that medicines lack an
approved indication need not mean that the knowledge is inadequate since there is often
good clinical experience of them.
Extremely premature infants ought to be ensured a pain-free existence as far as
possible, despite the fact that they have other physiological conditions to react to and
express their pain compared with full-term children. The risks of treatment with
medicines (toxic effect and side effects) must be weighed against the pain the child
perceives without pharmaceutical painkillers and also against the injuries that can arise
due to the pain. This is a difficult but very important fine line to walk and modern
neonatal treatment of pain is therefore based on a balanced, multimodal strategy [149].
This strategy involves regular pain assessment, individualist behaviour support (nonpharmacological) treatment and, if necessary, pharmacological treatment as well.
Pain assessment
The Swedish National Board of Health and Welfare’s assessment
•
For pain diagnostics, pain ought to be assessed with the help of valid
instruments (adapted according to age, level of maturity and type of pain) as far
as possible.
The assessment is based on Swedish guidelines from the Swedish Child Pain
Society and guidelines drawn up by an international consensus group.
Both international and national guidelines and procedures recommend that all units
which care for newborn children must have procedures that include a structured pain
assessment model [150, 151]. It is fundamental for an objective pain assessment to be
able to provide adequate and safe treatment of pain for these children. There is no
golden rule for objective pain assessment, but different observational scales are usually
used. However, it is urgent to
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choose valid instruments that have been designed for the child’s level of maturity and type
of pain, such as acute procedural pain or continuous pain and stress (for example through
respirator treatment or postoperative care). Appendix 2 shows the pain assessment
instruments that are often used and which are recommended in today’s neonatal care.
Non-pharmacological treatment of pain
The Swedish National Board of Health and Welfare’s assessment
•
•
The number of painful interventions ought to be minimised.
Extremely premature infants ought always to be given non-pharmacological,
individualised care to reduce the perception of pain and stress. This may include
o thinking through and optimising the care environment, calm for example;
o the participation of the parents;
o the use of skin-to-skin care and supportive cohesion;
o ensuring that the child is replete, dry and warm before procedures;
o ensuring that the child is lying comfortably;
o ensuring that the child is given the option of something non-nutritive on
which to suck.
The assessment is based on Swedish guidelines from the Swedish Child Pain
Society and guidelines drawn up by an international consensus group.
Children who are cared for at neonatal units undergo a large number of measures that are
painful to a greater or lesser extent on a daily basis. The very smallest and youngest
patients are the most sensitive and can also experience nappy changing or turning over as
painful. The basic principle is that the number of painful interventions should always be
minimised.
There are several non-pharmacological strategies that can reduce the child’s pain
reaction and have a calming effect. The care environment ought to be optimised,
including by minimising disruptive visual and audible impressions. An example of this is
subdued direct lighting, particularly to start with when the child is especially sensitive.
The child ought to be replete, dry and warm before painful procedures. The child ought
also to be assisted with something non-nutritive on which to suck, which means that the
child sucks on something such as a dummy, a hand or a finger (its own or that of the
parent) [152]. If possible, the parents ought always to be engaged in the treatment of pain,
partly so that they can report the child’s pain and partly because they will be able to offer
supportive measures such as skin-to-skin care (HMH) or supportive cohesion [153].
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Pharmacological treatment of pain
The Swedish National Board of Health and Welfare’s assessment
•
•
Each unit ought to have well designed pharmacological pain treatment
procedures which are also suitable for acute situations. The procedures ought to
cover pain in situations such as:
o procedural pain, including intubation
o postnatal and postoperative pain
o treatment of continuous pain and stress during respirator treatment.
Pharmacological treatment ought to be administered in good time before painful
procedures and ought always to be supplemented with non-pharmacological
support.
The assessment is based on Swedish guidelines from Swedish Child Pain Society
and guidelines drawn up by an international consensus group.
More painful intervention, such as pinpricks, the insertion of a central venous catheter or
of drainage, intubation, operation and respirator treatment, usually requires
pharmacological treatment of pain in addition to the non-pharmacological treatment
which always constitutes the basis of the pain treatment strategy.
The pharmacological treatment can include both analgesics (painkillers) and sedatives
(calming or soporific medicines) which have been selected on the basis of how painful
the condition or measure actually is. There is clinical experience of which preparations
ought to be used for extremely premature infants, but the scientific support is limited.
Medicines ought to be prescribed using a conscious strategy. Each unit ought to have
procedures with proposed treatments that are well-established, that are safe to use even
in an emergency situation, and that are carefully documented and followed up.
For mild to moderate pain, a painkilling effect can often be achieved by administering
sweet solutions (concentrated glucose or sucrose) by mouth to newborn children [154].
The positive effects have also been seen in extremely premature infants, even though the
scientific support is limited [155]. Premedication ought always in principle to be given
prior to intubation as well as directly after the birth or in other acute situations when
there is no intravenous access. Postoperative pain and painful conditions such as
necrotising enterocolitis should always be treated pharmacologically. With respirator
treatment, non-pharmacological support may be sufficient if there is no other reason for
the pain such as a painful condition or during postoperative care. However, treatment
with medicines may be relevant as age and treatment time increase because the
perceived stress of respirator treatment can then increase [156].
Medicines ought to be administered in good time before a painful procedure. Where
there are combinations of medicines, they should be given sequentially based on time of
onset, properties and effect. Preparations with a rapid time of onset and short duration of
effect are considered to be ideal for a short-term and acute procedure such as intubation
[157]. For
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sedation, the child ought to have stable blood pressure because this always leads to some
risk of a drop in blood pressure. Sedation with benzodiazepines is not advised for
extremely premature infants [158].
Medicinal substances are usually absorbed and eliminated more slowly in newborn
children, which is much more the case in extremely premature infants. If several
medicines are used simultaneously, an effective combination of as few medicines as
possible needs to be found because combinations can be risky. However, in some cases,
primarily when using opioids, it can be advantageous to use combinations of several
different medicines where the effects of the different preparations fortify one another,
which means that the strength of the doses can be reduced and thereby also the side
effects of each individual medicine. This applies to postoperative care, for example, when
treatment with Paracetamol, opioids and Clonidine may be appropriate.
The time to discontinue certain analgesics such as opioids ought to be individualised.
The time depends on the dose that the child has received as well as the length of time for
which the treatment has lasted. First of all there ought to be a gradual reduction in the size
of the dose followed by a reduction in the number of doses. Abstinence symptoms may be
difficult to interpret in extremely premature infants but if symptoms do arise, the original
dose should be used and the dose not continue to be reduced until the child is abstinencefree.
Read more
•
•
20
Businesses that must produce procedures can obtain support from guidelines from a
workgroup within the Swedish Child Pain Society. Nationella riktlinjer för
prevention och behandling av smärta i nyföddhetsperioden (English: National
guidelines for the prevention and treatment of pain in the neonatal period), revised
2013 [quoted 04/07/2014]; Available from:
http://www.svenskbarnsmartforening.se/svenskbarnsmartforening/docum
ent/Nationalriktlinjer-2013.pdf).
There is information from the Medical Products Agency on pharmacological preparations for full-term children which may also give some guidance for the care of
extremely premature infants (Behandling av barn i samband med smärtsamma
procedurer i hälso- och sjukvård - kunskapsdokument (English: Treatment of children
in connection with painful procedures in healthcare and medical treatment –
knowledge document). Information from the Medical Products Agency 2014; 25(3):922).
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children SOCIALSTYRELSEN
Nursing
The care of extremely premature infants concentrates on saving lives but also on
promoting the child’s long-term health and development. Right from the birth and
onwards throughout the care period, the nursing ought to be adapted to the child’s
relevant level of development so that stimuli are as beneficial as possible and negative
effects of stress and pain as small as possible. High quality nursing ought to be
individualised, support development and be centered around the family.
Care centered around the patient and the
family
The Swedish National Board of Health and Welfare’s assessment
•
The care of extremely premature infants ought to be organised so that it is
centered around the patient and the family. This means that the care should be:
o
o
o
o
o
individualised
support development
offer family care
offer integrated care
actively involve and inform the parents.
The assessment is based on systematic charting and consensus between the
chairpersons of the expert groups.
Care centered around the patient and the family is an approach whereby the care is not
limited to just being disease-orientated but is extended to cover other needs of the child,
parents and any siblings. The UN’s Children’s Convention forms the basis for the child’s
rights as an individual and as part of the family [159, 160]. There are also specific policy
documents for family-centered [161, 162] and neonatal nursing [163, 164]. Care
centered around the patient and the family involves the following:
•
•
•
•
•
Family care is offered, which means that parents and children are not separated The
care ought thereby to offer accommodation for the parents to stay at the newborn’s
unit.
Mothers with their own medical needs ought as far as possible to be integrated into
the care with the child at the newborn unit.
The family’s individual needs being respected as far as possible.
The parents’ sensitive needs being noted. The parents ought to be offered
psychosocial support and support in the bonding and the anaclytic process, which
also includes nursing support (see also the chapter on nutrition).
The parents being encouraged to take responsibility themselves for the child’s
nursing. The development benefits from parents being present for a lot of the time
and from early interventions focusing on the interaction between the child and the
parents.
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•
•
All information is shared with the parents if there is no obstacle in doing so in the
Public Access to Information and Secrecy Act or Chap. 6, Sections 3 and 4 of the
Parental Code.
Facilitating the cooperation between parents and personnel.
Care centered around the patient and the family is key to successful bonding and the
anaclytic process between children and parents. The anaclytic process is crucial to the
development of the brain and the child’s ability to handle stress, which in turn affects the
child’s general development and its future health. A good anaclytic process is also
important so that the parents can feel secure in their parental role [165]. However, the
short pregnancy period can make this difficult because the parents may have a natural
crisis reaction and because the anaclytic process has to be developed during the neonatal
care period. Extremely premature infants also give weak signals and often have behaviour
that is different and more difficult to interpret compared with full-term children [166,
167]. It is therefore essential that the units have competence to read the premature child’s
signals.
Development-supporting nursing
Development-supporting care is based partly on the medical treatment but also on
sociology and behavioural science The basis is the competence to understand the child’s
behaviour, to support the child’s autoregulation (of the nervous system, alertness and
interaction with the surroundings, for example) as well as benefitting the parents’ and the
care personnel’s interaction with the child. Individually-adapted, development-supporting
care ought to be offered since the care gives positive short-term effects and increases the
child’s well-being during the neonatal care period, even though the long-term effects have
weaker scientific support. Adapting the care to the individual and striving for calm
surroundings increases the possibilities of undisturbed sleep and a more beneficial
development for the child. The lower the lower level of maturity, the clearer the positive
effects on the child’s development.
There are different intervention programmes that can be used within the care of
extremely premature infants. NIDCAP (newborn individualised developmental care and
assessment programme) is a programme that can be carried out throughout the care period,
starting directly after the birth, which is significant from a neurobiological development
perspective [168]. A key moment in NIDCAP is the individual assessment of the child’s
responsiveness to and capacity to handle stimuli. Other elements include the positioning
of the child, adaptation of the surrounding environment as well as conduct at the time of
specific care measures. There is some scientific support to show that NIDCAP has
positive short-term effects on the more serious forms of bronchopulmonary dysplasia as
well as reducing the incidence of necrotising enterocolitis and improving the situation for
the families. The studies also showed positive long-term effects on the children’s
behaviour and motor skills [163, 169]. Other studies have shown that NIDCAP has a
positive impact on the maturity of the brain and on the cognitive development [170, 171]
as well as leading to shorter care periods [172].
Many neonatal units use modified NIDCAP care which works towards the same target
with the same means but do not fully include all observational elements. The methods
MITP (mother infant transaction programme) and IBAIP (infant behavioural assessment
and intervention programme) are based on the same theoretical basis as NIDCAP. The
methods are primarily intended to
22
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be used following discharge and aim to strengthen the communication between children
and parents. MITP has been shown to reduce the level of stress in the parents during the
child’s first year and a beneficial effect was seen on the child’s cognitive development
at five years of age [173, 174]. IBAIP has also been shown to improve the motor
development for children with a birth weight of less than 1 500 g and, at the five-year
follow-up, showed better cognition (performance IQ) as well as the ability to coordinate
visual impression and movement patterns (visual-motor integration) [175, 176].
One method that is often used and which ought to be offered 24 hours a day is skinto-skin care (HMH, also called kangaroo mother care, KMC). The method is based on
the fact that the child has direct skin contact with a parent or a close family member.
The scientific support for the positive effects of the method is found mainly in the low
income countries [177-179]. Studies have shown that HMH contributed to lower
mortality, fewer serious infections and other medical conditions, better temperature
regulation as well as shortened care periods. The method has also been shown to act as a
pain alleviator [152, 180, 181] and to have a positive effect on the child’s growth, the
mothers’ satisfaction and bonding with the child following discharge [182], the mother’s
milk production and the child’s nursing behaviour [141]. In turn, a longer nursing period
has a positive effect on the child’s cognitive development [182].
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The dilemma surrounding life support
treatment
•
Healthcare and medical treatment must offer all children good healthcare and medical
treatment on the same terms as the general population. All living premature children
must be given a chance of survival, irrespective of age, as long as this is compatible
with science and tried and tested experience.
The care of extremely premature infants is complicated. It includes forming opinions
concerning suitability for life beyond the foetal stage and decisions to refrain from or turn
off life support treatment. The national study EXPRESS (extremely preterm infants in
Sweden study) showed that 40 per cent of the children who had survived at least 24 hours
but who later died during ongoing neonatal intensive care died following a decision to
stop life support measures.
EXPRESS showed that there were major differences in the care between children who
are born in the 22nd and 23rd week of pregnancy: neonatal care (48 compared with 83 per
cent), intubation at the time of the birth (59 compared with 81 per cent) as well as the
survival rate upon birth and admission to the neonatal intensive care department (38
compared with 81 per cent) [4]. The handling must not be based solely on the basis of the
duration of the pregnancy because the care must not discriminate against a patient on the
basis of age, which is shown by the Healthcare and Medical Treatment Act (1982:763)
(HSL) and the Discrimination Act (2008:567). Such handling is also limited by the
accuracy of the pregnancy dating and by individual variations in level of maturity and risk
factors.
Forming opinions
It is impossible to give detailed guidance that can be applied in all different situations.
The care personnel must use their professional knowledge to make the medical
assessments (Chap. 6 of the Patient Safety Act [2010:659] (PSL) as well as Chapters 2
and 3 of The Swedish National Board of Health and Welfare’s provisions and general
advice [SOSFS 2011:7] on life support treatment). One condition for the care personnel’s
work is that the care provider must ensure that there is a management system that is used
to systematically and continuously develop and secure the quality of the activity (Chap. 3,
Section 1 of The Swedish National Board of Health and Welfare’s provisions and general
advice [SOSFS 2011:9] on management systems for systematic quality work). Situations
that are not clear and are difficult to assess will always arise, and it is sometimes
necessary to consider whether it is compatible with science and tried and tested
experience to provide life support treatment if there are no further cures that can be
offered (the principles are based on 6 Chap. of HSL, PSL, SOSFS [2011:7] and The
Swedish National Board of Health and Welfare’s handbook, Om att ge eller inte ge
livsuppehållande behandling - Handbok för vårdgivare, verksamhetschefer och personal
(English: To give or not give life support treatment - Handbook for care providers,
operations managers and personnel) [183]). It is an obvious thing to avoid or stop those
measures that are doing more harm than good. In some situations and following an
individual assessment,
24
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the doctor responsible may assess that life support treatment must not be started even if
the child shows signs of life when born.
•
•
•
“Before forming an opinion not to introduce or continue life support treatment, the
permanent care contact must consult at least one other registered professional. The
permanent care contact should also consult other professionals who are taking part or
have taken part in the patient’s care” (Chap. 3, Section 2 of SOSFS [2011:7]). “If no
doctor has yet been appointed to be responsible for the planning for the patient, a
responsible consultant or second on call must apply the regulations...” (Chap. 3,
Section 4 of SOSFS [2011:7]).
The patient must be offered palliative care with as good a quality of life and
symptom-alleviating powers as possible [183].
Feedback consultations and informative talks with the parents or close relatives must
be given (HSL and SOSFS [2011:7]).
The participation of the parents
It is vital that the parents are informed of the child’s condition and chances of survival as
early as possible. According to Swedish Law, the guardian is the legal representative of
patients under the age of 18 and must decide on the child’s personal affairs. The parents’1
wishes regarding the child’s care must be respected as long as this can be seen to benefit
the child’s interests and right to a life of value as well as well as being compatible with
expert and scrupulous care that satisfies the requirements regarding science and tried and
tested experience (Chap. 6 of PSL, Chap. 6, Section 11 of the Parental Code [ 1949:381]
and The Swedish National Board of Health and Welfare’s handbook on life support
treatment [ 183]).
A doctor cannot be forced to carry out a measure that would not benefit the patient but
it can sometimes be important to give parents or close relative time, provided that this
does not lead to suffering for the child, to digest the bleak prognosis of the condition
before treatment is stopped. If a parent does not want life support treatment to begin, it is
important for the care contact to ensure that he or she comprehends the information, is
able to realise and survey the consequences of not starting or continuing the treatment, has
had sufficient time to consider matters and is firm in his or her opinion (Chap. 4, Section
1 of SOSFS 2011:7).
Documentation
One way of ensuring the child’s and the parents’ moral and legal rights, as well quality
assuring the care, is to individualise the forming of opinions and to improve the
documentation on decisions to refrain from or stop life support treatment for extremely
premature infants. Good documentation, procedures and a clear allocation of
responsibility makes it easier to examine different courses of events in the care, such as if
a decision regarding life support treatment is subsequently questioned. Requirements
regarding documentation are set in Chap. 3 of The Swedish National Board of Health and
Welfare’s provisions and general advice (SOSFS 2008:14) on handling information and
keeping records in healthcare and medical treatment as well as in Chap. 3, Section 3 of
SOSFS (2011:7) where it says: “The permanent care contact must document in the patient
record
1
25
Translator’s note: it is assumed that this is what was intended rather than the typo in the Swedish.
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1.
2.
3.
4.
5.
6.
7.
26
his or her opinion on life support treatment,
when and on which grounds he or she has made said opinion,
when and which professional he or she has consulted,
at which times consultation with the patient has taken place,
if consultation with the patient has not been possible and, if so, the reason for this,
when and in which manner the patient and close relative have received individuallyadapted information in accordance with Section 2 b of the Healthcare and Medical
Treatment Act (1982:763), and
which opinion on the life support treatment the patient or close relative expresses”
CARE OF CHILDREN WHO ARE BORN EXTREMELY PREMATURELY
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Follow-up of the children
The Swedish National Board of Health and Welfare’s assessment
•
Businesses caring for extremely premature infants ought to follow up the
children in the short and long term.
The assessment is based on consensus between the chairpersons of the expert
groups.
The increase in survival rate among premature children and changes to treatment
methods lead to greater knowledge of the long-term and often complex consequences of
premature birth. To increase the knowledge and further improve the work, a structured
follow-up system is required which includes several different professions and which
follows the children up to school age. The Swedish Neonatal Society will facilitate an
equivalent follow-up of neonatal infants at risk in Sweden and the Society is therefore in
the process of producing national recommendations for such a structured follow-up
[184].
The objective of such a follow-up is to identify and diagnose deviations in the
individual child at an early stage and thereby provide conditions for early treatment and
appropriate support for the child and its family. By collecting and compiling follow-up
data, it is also possible to follow up the quality and scientifically assess the neonatal care
and the treatment. Swedish follow-up data is also needed to be able to inform parents
who have recently had very sick children of the future prognosis their child. The
Swedish neonatal quality register (SNQ) is an important source of knowledge for this.
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Project organisation
Project management
Lars Björklund
lung diseases expert group chairperson and
responsible for the taking into care section
Dr. of Med. and consultant, Skåne University Healthcare
Mats Eriksson
nursing, treatment of pain and brain expert group
chairperson, as well as scientific advice at The Swedish
National Board of Health and Welfare
senior lecturer at Örebro University and specialist nurse at
University Hospital Örebro
Lena Hellström Westas
scientific advice at The Swedish National Board of Health
and Welfare and expert group convenor
Professor at Uppsala University and consultant at
Akademiska sjukhuset
Stellan Håkansson
transportation expert group chairperson, senior lecturer
and consultant, Norrland University Hospital
Mikael Norman
PDA expert group chairperson Professor at Karolinska
Institutet, and operations manager and consultant at the
Astrid Lindgren Children’s Hospital
Staffan Polberger
nutrition expert group chairperson
senior lecturer and consultant, Skåne University Healthcare
project manager (2014), The Swedish National Board of
Health and Welfare
Eleonora Björkman
Charlotte Fagerstedt
Anette Richardson
project manager (2013-2014), The Swedish National Board
of Health and Welfare
unit manager, The Swedish National Board of Health and
Welfare
Michael Soop
project manager (2010-2011), The Swedish National Board
of Health and Welfare
Andor Wagner
project manager (2012), The Swedish National Board of
Health and Welfare
Participants in the lung diseases expert group
28
Thomas Abrahamsson
Veronica Berggren
Dr. of Med. and consultant at the University Hospital in Linköping
registered children’s nurse at the Astrid Lindgren Children’s Hospital
Kajsa Bohlin
Kristina Bry
Dr. of Med., Karolinska Institutet, and consultant at the Astrid
Lindgren Children’s Hospital
Professor and consultant at Drottning Silvia’s Children’s Hospital
Johanna Dalström
Aijaz Farooqi
paediatrician at Falu General Hospital
Dr. of Med. and consultant, Norrland University Hospital
CARE OF CHILDREN WHO ARE BORN EXTREMELY PREMATURELY THE
NATIONAL BOARD OF HEALTH AND WELFARE
Ola Hafström
Dr. of Med., consultant and head of section at Skåne University
Healthcare
Baldvin Jönsson
senior lecturer at Karolinska Institutet and consultant and
head of section at the Astrid Lindgren Children’s Hospital
Kenneth Sandberg
senior lecturer and reg. doctor at Drottning Silvia’s
Children’s hospital
Richard Sindelar
Dr. of med. and consultant at Akademiska barnsjukhuset
Participants in the transportation expert group
Uwe Ewald Anders
Adj. Professor and consultant at Akademiska sjukhuset
Fernlöf Barbara
hospital engineer at Akademiska sjukhuset
Graffman
paediatrician and neonatologist at the University Hospital in
Linköping
Boubou Hallberg
Dr. of Med. at Karolinska Institutet and consultant and head
of section at the Astrid Lindgren Children’s Hospital
Tova HannegårdHamrin
specialist doctor in anaesthesia and intensive care, senior
registrar at the Astrid Lindgren Children’s Hospital
Elisabeth Hentz
Dr. of Med. and consultant at Drottning Silvia’s Children’s
hospital
Sven Johansson
Johan Robinson
Bo Selander
Johannes van de Berg
consultant at Ryhov County Hospital
consultant at Norra Älvsborg County Hospital
consultant at the Central Hospital in Kristianstad
Ulf Westgren
senior lecturer at Lund University, and consultant at
Helsingborg General Hospital
nurse, deputy lecturer and transport manager at Norrland
University Hospital
Participants in the PDA expert group
Anna-Karin Edstedt
Bonamy
Dr. of Med. and assistant consultant at Sachsska Children’s
Hospital
Ola Hafström
see above
Stellan Håkansson
see above
David Ley
Professor and consultant at Skåne University Healthcare
Per Winberg
Dr. of Med. and consultant at the Astrid Lindgren
Children’s Hospital
CARE OF CHILDREN WHO ARE BORN EXTREMELY PREMATURELY
THE NATIONAL BOARD OF HEALTH AND WELFARE
29
Participants in the immature brain, treatment
of pain and nursing expert group
Lena Hellström Westas
see above
Ann-Sofi Ingman
specialist nurse and NIDCAP trainer at the Astrid Lindgren
Children’s Hospital
Elisabeth
Dr. of Med. and consultant at Skåne University Healthcare
Norman
Mats Blennow
Professor at Karolinska Institutet and consultant at the
Astrid Lindgren Children’s Hospital
Björn Westrup
Dr. of Med. at Karolinska Institutet and consultant at the
Astrid Lindgren Children’s Hospital
Participants in the nutrition expert group
30
Magnus Domellöf
lecturer and unit manager, Umeå University
Ann Dsilna Lindh
Dr. of Med., Karolinska Institutet, and specialist nurse at
the Astrid Lindgren Children’s Hospital
Uwe Ewald Renée
see above
Flacking
Dr. of Med. and lecturer at Högskolan Dalarna and senior
research fellow at the University of Central Lancashire in
the UK
Elisabeth Olhager
Dr. of Med., consultant and operations manager at Skåne
University Healthcare
Mireille Vanpée
Dr. of Med. at Karolinska Institutet and consultant and
Specialist Director of Studies at the Astrid Lindgren
Children’s Hospital
Inger Öhlund
Dr. of Philosophy and paediatric dietician at Norrland
University Hospital
CARE OF CHILDREN WHO ARE BORN EXTREMELY PREMATURELY THE
NATIONAL BOARD OF HEALTH AND WELFARE
Appendix 1.
Recommended nutritional
intake
Nutrient (kg/d)a
Liquid (ml)
Energy (kcal)
Protein/aa (g)
Carbohydrates (g)
Glucose (mg/kg/min)
Fat (g)
DHA (mg)
Arachidonic acid (mg)
Na (mmol)
P (mmol)
Cl (mmol)
Ca (mmol)
P (mmol)
Mg (mg)
Fe (mg)
Zn (mg)
Cu (Mg)
Se (Mg)
Mn (Mg)
I (Mg)
Vit A (RE) (IE)
Vit D (IE)
Vit E (TE) (mg)
Vit K (Mg)
Vit C (mg)
Thiamine B1 (Mg)
Riboflavin B2 (Mg)
Pyridoxine B6 (Mg)
Niacin (NE) (mg)
Pantethine (mg)
Biotin (Mg)
Folate (Mg)
Vit B12 (Mg)
Day 0b Day 4c
EN full dosed
TPN full dosee
80-100
130-160
135-200
135-180
50-60
105-125
115-135
90-115
2-2.4
3.5-4.5
4.0-4.5
3.5-4
7-10
11-16
9-15
13-17
5-7
9-12
1.0-1.5
4-6
5-8
3(-4)
12-60
11-60
18-45
14-45
0-1
2-4
3-7
3-7
0-1
1.0-2.5
2-3
2-3
0-1
2-4
3-7
3-7
0.5-1.5
2.2-2.7
3.0-3.5
1.5-2
0.5-1.5
1.7-2.5
2-3
1.5-1,9
0-4
6-11
8-15
4,3-7,2
0
2-3
0,1-0.2
1-1.5
1.5-2.5
0.4-0.45
70-110
120-200
20-25
2-5
2-7
2-5
0-4
1.0-7.5
0-1
10-30
10-50
10
1 000-2 300
1 300-3 300
700-1 500
220-600
400-1 000
40-160
2.2-7
2.2-11
2.8-3.5
4.4-20
4.4-28
4.4-16
13-35
11-46
15-25
140-300
140-300
200-350
150-300
200-400
150-200
45-250
45-300
150-200
0.4-7,0
0.4-5,5
4-7
0.3-2,0
0.3-2,1
1-2
1.7-12,0
1.7-16,5
5-8
35-90
35-100
35-80
0.1-0.6
0.1-0/77
0.1-0.5
Reference: [126-128]
a
Per kilo body weight and day for all units. The relevant weight is used for body weight
except for the first few days when the birth weight is used until it has been achieved and
passed.
b
Here, day 0 is defined as the date of the birth, i.e. from the birth until the morning of the
next day. The recommendation applies to a full day and needs to be individually adjusted
down depending on the time when the child is born
c
The child ought to be given a full dose of nutrition at least as of the fourth day of life
(but still with some fluid restriction). The recommendation in this column is approximate
and is based on 50 per cent enteral and 50 per cent parenteral nutrition. The exacta targets
(which must be individually calculated) depend on the proportions of the parenteral
supply of the nutrient in question, so the target will be slightly lower than stated if the
3
1
child receives a greater share of parenteral nutrition for example.
d
Recommended intake for full enteral nutrition (EN).
e
Recommended intake for total parenteral nutrition (TPN).
3
2
Appendix 2. Examples of pain assessment instruments
Pain assessment instrument
Reference
Astrid Lindgren and Lund children's hospitals pain [185]
ALPS-Neo
and stress assessment scale for preterm and sick
newborn infants
Astrid Lindgren children's hospital pain
ALPS 1
assessment scale for term neonates
Behavioural Indicators of Infant Pain
[186]
[187]
[188]
NFCS
E'chelle Douleur Inconfort
Nouveaune
Neonatal Facial Coding System
NIPS
Newborn Infant Pain Scale
[191]
N-PASS
Neonatal Pain, Agitation, and Sedation
Scale
Premature Infant Pain Profile Premature
Infant Pain ProfileRevised
[192]
BIIP
COMFORTneo
EDIN
PIPP
PIPP-R
[189, 190]
[193] [194]
Dimensions
Behaviour: facial expression, level of consciousness, activity and tone in
extremities.
Physiological: breathing
Behaviour: facial expression, level of consciousness, activity and tone in
extremities
Physiological: breathing
Behaviour: facial expression, hand activity, sleep
Behaviour: facial expression, level of consciousness, movement and tone
in extremities, crying
Emphasis
Continuous
Procedure
Continuous
23-32 weeks
24-43 weeks
Behaviour: facial expression, body movements, quality of sleep, contact,
consolability
Behaviour: facial expression
Continuous
34-37 weeks
Continuous
Procedure and
Continuous
Procedure
Behaviour: facial expression, breathing patterns, extremity movements,
level of consciousness, crying
Behaviour: crying, level of consciousness, facial expression, extremity tone Procedure and
Physiological: heart rate, breathing rate, blood pressure, oxygen saturation Continuous
Behaviour: facial expression
Procedure
Physiological: heart rate, oxygen saturation
Context: gestational age, level of consciousness
CARE OF EXTREMELY PREMATURE INFANTS
THE SWEDISH NATIONAL BOARD OF HEALTH AND WELFARE
33
Validated for gestational age
< 42 weeks, cared for at a
neonatal unit directly after
the birth
Full-term until one month
old
23-40 weeks
24-48 weeks
3
4