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The Spina Bifida Association of New Zealand spinabifidanz@zoho.com

15 July 2012 Ministry for Primary Industries PO Box 2526 Wellington 6140 New Zealand

Dear Sir / Madam, The Spina Bifida Association wishes to support the introduction of mandatory folate fortification into New Zealand. INCIDENCE AND PREVALENCE Data sourced from the National Health Committee as provided by National Collections show that to June 2011 there have been an estimated 2,141 cases of Neural Tube Defects (1 per 2,050 people) at a rate of 0.5 per 1,000 live-births.

Recorded cases of Spina Bifida for the same period (as a subset of the above data) show there have been 1,552 cases (1 per 2,840 people). Therefore, it can be surmised that cases of Spina Bifida account for 72.5% of all recorded Neural Tube Defects, with the balance (27.5%) of cases spread between Anencephaly, Hydranencephaly, Encephaloceles and Iniencephaly.


GENETIC LINK BETWEEN FOLATE ABSORPTION AND NEURAL TUBE DEFECTS The MTHFR gene1 provides instructions for making an enzyme called methylenetetrahydrofolate reductase. This enzyme plays a role in processing amino acids, the building blocks of proteins. Methylenetetrahydrofolate reductase is important for a chemical reaction involving forms of the Bvitamin folate (also called folic acid or vitamin B9). Specifically, this enzyme converts 5,10methylenetetrahydrofolate to 5-methyltetrahydrofolate. This reaction is required for the multistep process that converts the amino acid homocysteine to another amino acid, methionine. The body uses methionine to make proteins and other important compounds. In 1962, it was reported that people with a rare genetic condition called homocystinuria were prone to develop severe cardiovascular disease in their teens and 20s2. In this condition, a defective enzyme causes an accumulation of homocysteine in the blood, resulting in very high levels. Studies of children with homocystinuria led to the discovery that elevated homocysteine levels are a risk factor for developing atherosclerosis and blood clots in the arteries and veins. Although homocystinuria is a rare disease (affecting about 1 in 200 000 people), many more people have mildly or moderately elevated homocysteine levels. At least 40 mutations in the MTHFR gene have been identified in people with homocystinuria. Most of these mutations change single amino acids in methylenetetrahydrofolate reductase. These changes impair the function of the enzyme, and some cause the enzyme to be turned off (inactivated). Other mutations lead to the production of an abnormally small, nonfunctional version of the enzyme. Without functional methylenetetrahydrofolate reductase, homocysteine cannot be converted to methionine. As a result, homocysteine builds up in the bloodstream, and the amount of methionine is reduced. Some of the excess homocysteine is excreted in urine. Some people have elevated homocysteine levels caused by a deficiency of B vitamins and folate in their diets. High homocysteine levels are also seen in people with kidney disease, low levels of thyroid hormones, psoriasis, and with certain medications (such as antiepileptic drugs and methotrexate). It has been recognized that some people have a common genetic variant (called methylenetetrahydrofolate reductase, abbreviated MTHFR) that impairs their ability to process folate. This defective gene leads to elevated levels of homocysteine in some people who inherit MTHFR variants from both parents because it follows an autosomal recessive pattern of inheritance.

1 2

http://ghr.nlm.nih.gov/gene/MTHFR http://circ.ahajournals.org/content/111/19/e289.full


There are implications of having elevated homocysteine that are specifically relevant for women. Elevated homocysteine levels have been observed more frequently among women with certain pregnancy complications, including preeclampsia placental abruption recurrent pregnancy loss, and giving birth to a small, low-birth-weight baby. Hyperhomocysteinemia is observed more commonly among women who have a child with a neural tube defect. Approximately 20% of women who have a child with a neural tube defect have abnormal homocysteine metabolism. Several variations in the MTHFR gene have been associated with an increased risk of neural tube defects. The most well-studied polymorphism related to the risk of neural tube defects changes a single DNA nucleotide in the MTHFR gene. This variant, which is relatively common in many populations worldwide, produces a form of methylenetetrahydrofolate reductase that has reduced activity at higher temperatures (thermolabile). People with the thermolabile form of the enzyme have increased levels of homocysteine in their blood. It is unclear how variations in the MTHFR gene increase the likelihood of neural tube defects. However, the increased risk may be related to differences in the ability of methylenetetrahydrofolate reductase to process folate. A shortage of this vitamin is an established risk factor for neural tube defects. Polymorphisms in the MTHFR gene have also been studied as possible risk factors for a variety of common conditions. These include heart disease, stroke, high blood pressure, high blood pressure during pregnancy, an eye disorder called glaucoma, psychiatric disorders, and certain types of cancer. The polymorphism in the MTHFR gene has also been suggested as a risk factor for cleft lip and palate. In the USA parents who have had An NTD affected pregnancy or parents planning a pregnancy are able to be screened for the presence of the MTHFR gene. If present they are put on high doses of folic acid prior as part of pre-conceptual maternity care. ORAL CONTRACEPTIVES AND EFFECT ON FOLATE ABSORPTION Evidence shows that levels of folate and vitamin B12 absorption is reduced in women taking oral contraceptives3. These levels do not return to normal until about three months after usage has stopped, but many women become pregnant during this time. Should a pregnancy ensue within six months of discontinuing OCs, there is a greater incidence of folate deficiency during the pregnancy than in those who had not taken OCs before becoming pregnant. Women becoming pregnant within six months of discontinuing OCs had lower levels of serum and red blood cell folate than those who had not been taking birth control pills shortly before becoming pregnant. In September 2010 the US Food and Drug Administration approved approved Beyaz tablets, an estrogen/progestin combined oral contraceptive that also contains a folate (levomefolate calcium 0.451 mg)4. Beyaz was approved for the secondary indication in women who choose to use an oral contraceptive as their method of contraception, to raise folate levels for the purpose of reducing the risk of a neural tube defect in a pregnancy conceived while taking the product or shortly after discontinuing the product. Beyaz was found to increase folate levels in women. Spina Bifida New Zealand believes that women in New Zealand are poorly educated about the effect that long term oral contraceptives have on folate levels and that this issue must be raised by those

3 4

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2327375/pdf/canfamphys00210-0083.pdf http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm227237.htm


responsible for prescribing (General Practioners, Family Planning etc) and distributing these drugs (Pharmacists). LIVEBIRTH DATA In a recent interview for TVNZ Q+A programme, Professor David Smith, a folic acid researcher was asked why it was necessary to add folic acid in bread. His response was that the idea was to reduce the number of neural tube defect pregnancies in New Zealand. But he also stated that there were two problems: the first being that the number of Neural Tube Defects is not known precisely, because the authorities haven't been measuring neural tube defects properly since 2003. This statement is completely false. In a referral to the National Health Committee Prenatal repair of Myelomeningocele (Spina Bifida) 5 by Spina Bifida New Zealand data sourced from the New Zealand Birth Defects Registry6 showed that in the period 1996-2009 the cases of birth defects diagnosed among live births in New Zealand has showed a steady decline.

Spina Bifida 40

Livebirths

30 20

Spina Bifida Linear (Spina Bifida)

10 0

PREGNANCY TERMINATION RATES AFTER A NEURAL TUBE DIAGNOSIS A poignant quote from the Perinatal and Maternal Mortality Review Committee 5th Annual report states that “the grief of a sudden untimely death will never be forgotten�. The sad reality of receiving a Neural Tube Defect diagnosis is that many babies do not even get to see their birthday. The Abortion Supervisory Committee 2011 Report show that in the year ended December 2011 there were 65 induced abortions carried out at over 20 weeks gestation. Because of the way statistics are recorded in New Zealand, the reason for these abortions are not clear (i.e. the grounds for abortion are not recorded) but it is clear from statistics recorded under Section 8 Grounds for Abortion (Table 8.1) that for the same period there were 206 abortions performed because the unborn baby had some form of disability7. 5

http://www.nhc.health.govt.nz/sites/www.nhc.health.govt.nz/files/documents/pages/010%20%20Spina%20Bifida%20Surgery.pdf 6 http://www.nzbdmp.ac.nz/assets/FILES/Final%20published%20table%202000-2009.pdf 7

http://www.parliament.nz/NR/rdonlyres/F6BCBD9B-BBF7-4856-A94648A89C7E3B6D/209364/DBHOH_PAP_22583_AbortionSupervisoryCommitteeSupple.pdf


Handicapped Child and Mental Danger Handicapped Child, Physical and Mental Danger Handicapped Child and Other Seriously Handicapped Child

132 7 2 65

The World Health Organisation defines foetal death or stillbirth as death prior to the complete expulsion or extraction from its mother of a product of conception, irrespective of duration of pregnancy; the death is indicated by the fact that after such separation the foetus does not breathe or show any other evidence of life such as beating of the heart, pulsation of the umbilical cord or definite movement of voluntary muscles (WHO 1977). Statistics published by the Ministry of Health include only foetal deaths (known also as stillbirths) of 20 weeks’ or more gestation, or 400 g or more birthweight. This is in line with the Births, Deaths, Marriages and Relationships Registration Act 1995, which defines a stillborn child as ‘a dead foetus that: (a) weighed 400 g or more when it issued from its mother; or (b) issued from its mother after the 20th week of pregnancy. Under the 1995 Act, a medical certificate of causes of death and a birth registration form are required to be completed in respect of each stillborn child. This includes stillbirths resulting from terminations of pregnancy. There were 410 stillbirths recorded in the March 2011 year by Statistics New Zealand. The Ministry of Health is responsible for compiling and publishing cause of death statistics for New Zealand and using information provided by the Births, Deaths and Marriages registry, assigns underlying cause of death codes in accordance with the guidelines contained in the World Health Organization (WHO)’s International Statistical Classification of Diseases and Related Health Problems, 10th revision. The 10th Revision, Australian Modification, 2nd Edition (ICD-10-AM-II) was used for coding purposes (National Centre for Classification in Health 2000) for all statistics reported here. The clinical code relating to Spina Bifida Myelomeningocele is Q00 – Q07. ICD code

Clinical code description

Q00–Q07

Congenital malformations of the nervous system

Q10–Q18

Congenital malformations of eye, ear, face and neck

Q20–Q28

Congenital malformations of the circulatory system

Q30–Q34

Congenital malformations of the respiratory system

Q35–Q37

Cleft lip and cleft palate

Q38–Q45

Other congenital malformations of the digestive system

Q60–Q64

Congenital malformations of the urinary system

Q65–Q79

Congenital malformations and deformations of the musculoskeletal system

Q80–Q89

Other congenital malformations

Q90–Q99

Chromosomal abnormalities, not elsewhere classified

A Ministry of Health Report in 2007 on Foetal and Infant Deaths gives a high-level overview of causes of foetal death (20 weeks or more gestation or 400g birthweight)8. The foetal death category includes stillbirths resulting from terminations of pregnancy. Congenital malformations, 8

http://www.health.govt.nz/publication/fetal-and-infant-deaths-2007


deformations and chromosomal abnormalities, including Spina Bifida Myelomeningocele, accounted for 33.3 percent of foetal deaths. Other conditions 1% Congenital malformations, deformations and chromosomal abnormalities 33%

Figure 1: Fetal deaths, causes by ICD-10-AM-II chapter, 2007

Certain conditions originating in the perinatal period 66%


Number 160 140 120 100 80 60 40 20 0 Other disorders Disorders related Respiratory and originating in the to length of cardiovascular perinatal period gestation and fetal disorders specific growth to the perinatal period

Other perinatal conditions

Chromosomal abnormalities, not elsewhere classified

Certain conditions originating in the perinatal period

Congenital malformations of the nervous system

Congenital Congenital Other congenital malformations of malformations and malformations the circulatory deformations of system the musculoskeletal system

Congenital malformations, deformations and chromosomal abnormalities

All other conditions

Other

Cause of death

Figure 2: Foetal deaths, causes by ICD-10-AM-II sub-group, 2007

Congenital malformations, deformations and chromosomal abnormalities, including Spina Bifida Myelomeningocele also accounted for 25.9 percent of neonatal deaths in 2007.

Other conditions 9%

Congenital malformations, deformations and chromosomal abnormalities 26%

Certain conditions originating in the perinatal period 65%

Figure 3: Neonatal deaths, causes by ICD-10-AM-II chapter, 2007


Number 50 45 40 35 30 25 20 15 10 5 0 Disorders related Respiratory and Other disorders Other perinatal Congenital Congenital to length of cardiovascular originating in the conditions malformations of malformations of gestation and disorders perinatal period the circulatory the nervous fetal growth specific to the system system perinatal period Certain conditions originating in the perinatal period

Chromosomal abnormalities, not elsewhere classified

Other congenital Other accidental anomalies threats to breathing

Congenital malformations, deformations and chromosomal abnormalities

Other external causes

External causes of morbidity and mortality

Cause of death

Figure 4: Neonatal deaths, causes by ICD-10-AM-II sub-group, 2007

All other conditions

Other conditions


The table below shows the number of foetal and neonatal deaths by cause and ethnic group in 2007. Maori Total

Fetal

Pacific peoples

Early

Late

neonatal

neonatal

Fetal

Other

Early

Late

neonatal

neonatal

Fetal

Early

Late

neonatal

neonatal

Congenital malformations, deformations and chromosomal abnormalities Q00 Anencephaly and similar malformations

8

1

0

1

0

0

0

5

1

0

Q01 Encephalocele

3

0

0

0

0

1

0

2

0

0

Q02 Microcephaly

3

0

1

0

0

0

0

2

0

0

Q03 Congenital hydrocephalus

6

2

0

0

0

0

0

4

0

0

Q04 Other congenital malformations of brain

8

2

0

0

2

0

0

3

1

0

Q05 Spina bifida

11

0

1

0

2

0

0

6

1

1

Q07 Other congenital malformations of nervous system

8

1

0

0

0

0

0

7

0

0

In yet another set of data the Perinatal and Maternal Mortality Review Committee classifies Spina Bifida under PSANZ Classification PDC 1.1 Congenital Abnormality - Central Nervous System, but this classification includes all central nervous system abnormalities. Table 57 in the PMMRC 5th Annual Report 2009 gives 35 babies dying due to Congenital Abnormality - Central Nervous System. This includes all babies who die born from 20 weeks gestation to 28 completed days after birth, (or weighing at least 400 g if gestation is unknown.) This figure includes termination of pregnancies, stillbirths and neonatal deaths.


For post-neonates the causes of death were more diverse than those for foetal and neonatal mortality. Congenital malformations, deformations and chromosomal abnormalities, including Spina Bifida Myelomeningocele accounted for 19.2 percent of post-neonatal death in 2007. Other conditions 13% Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified 26%

Certain infectious and parasitic diseases 5%

Certain conditions originating in the perinatal period 10%

Congenital malformations, deformations and chromosomal abnormalities 19%

Diseases of the respiratory system 11%

External causes of morbidity and mortality 16%

Figure 5: Post-neonatal deaths, causes by ICD-10-AM-II chapter, 2007 Number 18 16 14 12 10 8 6 4

Diseases of the nervous system

Congenital malformations, deformations and chromosomal abnormalities

External causes of morbidity and mortality

Cause of death

Figure 6: Post-neonatal deaths, causes by ICD-10-AM-II sub-group, 2007

I30–I52

M30–M36

E70–E90

D70–D77

X85–Y09

Y85–Y89

W75–W84

V40–V49

W65–W74

Q90–Q99

Q80–Q89

Q65–Q79

Q38–Q45

Q30–Q34

Q20–Q28

P80–P83

Certain conditions originating in the perinatal period

Q00–Q07

P75–P78

P70–P74

P50–P61

P20–P29

J85–J86

Diseases of the respiratory system

P05–P08

J40–J47

J20–J22

J10–J18

G80–G83

G70–G73

G10–G13

C81–C96

Certain Malignant infectious and neoplasms parasitic diseases

G00–G09

B25–B34

C69–C72

A30–A49

0

A00–A09

2

Other conditions


Professor Smith noted that folate fortification was a public health initiative that would affect the health of the whole population; it is a very serious decision to be taken and one that should not be done lightly, balancing benefit against risk. He agreed that there would be a relatively small benefit in neural tube defect case reduction but the potential for harm is much greater. It is that potential for harm that concerns the Spina Bifida Association the most but for completely divergent reasons than those espoused by Professor Smith. The life prospects for a newborn with Spina Bifida have never been as good as they are today, but the prenatal advice given to parents on diagnosis is too often still based on outdated medical information and negative stereotypes regarding disability. Statistics indicate that up to 90% of pregnancies affected by NTDs are terminated, either in early pregnancy prior to diagnosis but certainly after diagnosis at 18-20 weeks gestation. Data gathered from sources here in NZ from the Ministry of Health and the Perinatal Maternal Mortality Review Committee vary due to slight differences in data collection but data recorded in 2010 indicates that in the case of a pregnancy where Spina Bifida is diagnosed up to 40 pregnancies are terminated after 18-20 weeks gestation. Costweight data on the cost of pregnancy terminations shows potential estimated savings of between $59,470 (50 pregnancies terminated between 6-18 weeks at $1189 each) and $90,302 (40 pregnancies terminated after 18-20 weeks at $2257 each). The costs of continued high risk antenatal management have not been factored into potential estimated savings. The current abortion service cost to the NZ taxpayer was $4.5M to the year ended 30 June 2011 generated by the 175 certifying consultants on the Abortion Supervisory Committee’s list of practitioners who act as consultants for women considering a termination of pregnancy. The Contraception Sterilisation & Abortion Act requires that a woman see two certifying consultants who must both agree that she has legal grounds for an abortion. Each consultation costs $135. Again based on 90 pregnancies terminated because of a prenatal diagnosis of a NTD such as Spina Bifida, potential savings of $23400 could be realised increasing total savings to between $82870 and $113702. The right to life and development was identified as a particular topic of discussion by the United Nations Committee reviewing the Convention on the Rights of the Child (CRC/C/69/329) and the need to oppose ‘with great force’ the abortion of unborn babies known to be handicapped was noted. It is clear from this document that all children have the right to life and that failure to take all necessary measures to promote the survival and development of disabled children on an equal basis with other children represented a profound violation of the Convention on the Rights of the Child. All children were recognised as equal members of the human race and that discriminatory laws which denied their right to life should be repealed. The goal of striving towards perfection in the human beings required challenging in a public debate around medical and scientific research and it was noted that it was one thing to work to eliminate impairment but quite another to eliminate the person with the impairment. The denial of life should not be included as part of any preventive strategy involving disability, rather society must celebrate diversity and learn to celebrate the birth of every child, with or without disability.


THE COST OF NEURAL TUBE DEFECTS NTDs are the second major category of birth defects (after congenital heart defects) and are a major component of foetal and infant death and morbidity. An alternative method of understanding the population impact of Spina Bifida is through the contribution to the overall burden of disease. This takes account of both the years of life lost to premature mortality and the years of life lost (severityadjusted) to disability. Among 0-14 year olds, 15% of the total burden from birth defects is due to Spina Bifida.

In an unpublished 1997 study quoted in reports by the Food Standards Australia New Zealand (FSANZ), Singh & Elliot indicated that the cost of patient care for Spina Bifida in New Zealand up to the age of 20 was NZ$355,060. At that time there were on average about 20 cases of Spina Bifida born per year resulting in a total cost over 20 years of $7,101,200 and did not include other potential costs such as the loss of parental income, special schooling needs, family stress, wheelchair, crutches, occupational therapy, GP visits etc. A 1994 study by Waitzman et al estimated that the direct and indirect costs of treating patients with Spina Bifida over their lifetime was NZ$565,000. A cost-of-illness methodology was employed to estimate the direct (includes medical, special education, and development services) and indirect (includes mortality and morbidity) costs of Spina Bifida and 17 other birth defects in the USA.


Health system costs in the USA are high by international standards, but this data adjusted to NZD equivalents show that Spina Bifida has the fourth highest lifetime cost from birth defects.

The most recent study on the economic burden of neural tube defects9 showed that although there is limited research in this field, the available evidence indicates that the economic burden of NTDs is substantial. The majority of studies carried out to determine the economic burden of NTDs have 9

Yunni Yi : Economic burden of neural tube defects and impact of prevention with folic acid Eur J Pediatr 2011 DOI 10.1007/s00431-011-1492-8


included the medical costs of NTDs. These are costs that are borne by healthcare payers such as health insurance programmes, and include factors such as drugs and hospitalizations both for managing NTDs directly and for managing comorbidities. Other studies have been more inclusive and take into account costs associated with development services and special education for individuals living with NTDs, often described as direct nonmedical costs. Broader costs such as lost work time, caregiver costs, and costs due to premature loss of life were considered in these studies and are typically referred to as indirect costs. Annual direct medical costs per patient was estimated to be $51,574 in 2003 USD$ for NTD and between $11,061 in 1993 USD$ to $65,177 in 2003 USD$ for Spina Bifida in the USA. In Spain, the Social Security system spent direct medical costs of $2,953,138 in 1988 USD$ per year for the care of patients with Spina Bifida representing approximately $2,734 in 1988 USD$ per person per year. A significant proportion of the cost burden occurs during childhood. For example, in the USA, total hospital charges for new born infants with NTDs amounted to $74 million in 2003 USD$ for Spina Bifida. Medicaid in Washington State spent $2.1 million in 1993 USD$ on children with Spina Bifida in 1993, and average payments for children with Spina Bifida were 11.6 times higher than the average payment for all children in the state’s Medicaid programme. While children and adolescents with Spina Bifida incur medical expenditures several times higher than other children and adolescents, young adults with the condition also continue to be high users of medical care. Adults account for 67% of persons with Spina Bifida and 66% of medical expenditures associated with Spina Bifida in USA. At any age, individuals with Spina Bifida incur higher medical expenditures than those without, and costs continue to be high throughout adulthood. Almost half of the hospital admissions for adults with Spina Bifida are due to secondary conditions (such as serious urologic infections, renal calculi, pressure ulcers, and osteomyelitis), and the financial costs of these admissions are substantial. The annual direct medical cost per patient for the treatment of Spina Bifida comorbidities in the USA ranged between ₏2,466 and ₏4,873. Hospital admission rates in Canada for adults with chronic and complex physical disabilities of childhood including Spina Bifida is nine times that of the general population, and adult patients with Spina Bifida are regular users of outpatient and inpatient service. Five studies, which looked beyond the direct costs incurred by the healthcare system when evaluating the burden of NTDs, suggest that indirect costs of Spina Bifida are substantial. There is a substantial impact of caring for a child with Spina Bifida on labour force participation, with participation rates 21% to 27% lower than others in the general population. Caregivers of children with Spina Bifida worked an annual average of 7.5 to 11.3 hours less per week depending on disability severity. Differences in work hours by caregivers of children with Spina Bifida translated into lifetime costs of $111,755 in 2002 USD. There is an estimated average reduction of paid work time of 14 hours per week for mothers and 5 hours per week for fathers of children with Spina Bifida. The estimated caregiver time costs to care for a children with Spina Bifida until age 25 ranges from $164,675 to $197,991 in 2001 USD$.


Total lifetime costs for patients with Spina Bifida amounted to $620,484 in 2002USD$. Just 37.1% of the total was attributable to direct medical costs $220,560, with the costs associated with special education and development services accounting for 6.5% ($41,337) and 0.3% ($2,034), respectively. The remaining 56.1% ($356,553) of the total were indirect costs, of which $138,086 were due to increased morbidity and $218,477 due to premature mortality. A study by Dr Brendon Bowkett10 has showed that the cost of surgery alone is nearly $1M for each child affected by spina bifida and other neural tube defects. Direct surgery and hospital costs for each child, from their birth at Wellington Hospital, has been worked out as $944,000.The study into inpatient Paediatric Neural Tube costs demonstrates the enormous morbidity faced by children with Spina Bifida and their families. Six of the seven patients in the study are paraplegic and children can expect to spend almost six months in hospital by the time of their late adolescent years. In addition to initial spinal cord closure, almost all children require repeated neurosurgical procedures for shunts as well as major bladder, bowel and orthopaedic spine surgery. These procedures prevent deterioration of disability levels rather than restoring normal function. The vast majority of admissions were for surgical reasons and intensive, skilled nursing is required. A qualitative study of faecal incontinence in children with disability including children with Spina Bifida also demonstrated a reduction in the quality of life when access to a tertiary service is limited. Children with Spina Bifida have impaired bladder function and are at increased risk of developing a neurogenic bladder. Recent research has indicated that all neurogenic bladders have an increased risk of malignancy even if not surgically augumented. In the Wellington study, six of seven patients had undergone bladder augmentation surgery requiring life-long cystoscopic urological screening to monitor cancer risk. Two other aspects of care for children with Spina Bifida were raised in this study. The first was the high radiation exposure and the second the number of Ventriculoperitoneal (VP) shunt revisions undergone by patients. It is known that CT scans are associated with an increased cancer risk and a 2007 US review estimated 1 fatal cancer per 1000 paediatric CT scans, leading to 500 deaths a year in the US. The study showed that adolescent patients had had on average 56 X-rays, 8 CT scans and four nuclear medicine investigations. In the study, four out of seven Spina Bifida patients required insertion of a VP shunt at birth. Due to infection, these four patients required on average 3.75 VP shunt surgical revisions. There are recognised negative cognitive outcomes associated with multiple shunt revisions and the mean IQ of patients has been shown to decrease with increasing shunt revisions. For patients with Spina Bifida the need for shunts is life-long and further revisions are likely in most cases. Inpatient costs incurred outside the Wellington paediatric inpatient service were not included in this study. Four of the children came from provincial towns but admissions to their local hospitals were not included. The costs in this study are likely to significantly underestimate the true costs of treating inpatient paediatric NTDs for this and other reasons and was conservative in its estimates in order to avoid ‘double dipping’. Although during the data collection all laboratory investigations were accounted for, the costs for these were not calculated rather they were included in the costs for a day of stay. Secondly the inpatient ward costs for these children would be at the higher end of a range but average inpatient case mix costs were used. Many costs were outside the scope of the study. These include costs incurred to peripheral hospitals, costs associated with prenatal and obstetric care, district nursing, social work, special education, transport, wheelchairs and mobility 10

http://www.stuff.co.nz/dominion-post/news/wellington/4322022/1m-cost-per-child-sparks-folate-call


aids, orthotics, physiotherapy, occupational therapy and outpatient consultations. Data for these costs were not gathered. Social costs to the family such as loss of parental employment were also not calculated. Lastly, there are ‘knockout costs’ to the service due to factors such as latex allergy management, which requires longer theatre times so that facilities can be vented and cleaned prior to surgery. Also, other paediatric surgeries need to be delayed when paediatric patients require emergency ventriculoperitoneal (VP) shunt revision. Even with this highly conservative estimate, the cost analysis still showed that treating paediatric NTDs is significantly more expensive than the only previous New Zealand estimate. The Ministry of Health funds District Health Boards through the Health Funding Authority with funding provided on the basis of cost weights, used both to measure volume and to calculate prices for specified inpatient purchase units defined in the Data Dictionary of the National Service Framework. Such weights are intended to reflect the relative resource consumption between diagnosis related groups. The neonatal case reviewed demonstrates the high cost and morbidity of management that may occur during the neonatal period. (NZ$678,340). Of this care, the earned cost weight to the Wellington Paediatric unit was NZ$245,000 (a shortfall of $433,340 for one patient). The study provided a conservative analysis of the high costs of inpatient paediatric NTD management in just one New Zealand DHB and looked at the cost of care in the first 21 years of life. It is estimated that the overall cost of managing neural tube abnormalities in New Zealand is $39M per year. The connection between disability and economics is nothing new but rational-economic thinking degrades society’s willingness to accept and care for ‘abnormal’ children and adults as well as making people hostile towards parents who choose not to terminate affected babies. Patients with Spina Bifida should not be viewed simply as being an economic cost, but the study provides an indication of the disruption to their lives through the high number of admissions, and the long cumulative lengths of stay in hospital. It is simply not possible to quantify the distress to the child and family, nor the loss of opportunity as a result of having an NTD. The inability to cost this dimension did not indicate that the authors of the study discounted its importance. The high cost of Spina Bifida to New Zealand DHB Paediatric services emphasises the importance of pre-conceptual prevention if at all possible. AIDS AND MODIFICATIONS In a research study by Cochrane of 101 Spina Bifida patients, it was found that 90% of patients with thoracic level lesion used wheelchairs and 45% of patients with a lumbar and 17% sacral level lesion used wheelchairs (average 51%). In the USA in any given year, on average, 33% of individuals with Spina Bifida made claims from Medicaid for some type of mobility-related assistive technology, including wheelchair-related costs, orthotics and prosthetics, ambulatory aids, and communication and hearing aids. Annually, these claims accounted for €297,704 (price year not reported), which represents approximately 3.3% of all reimbursement by Medicaid for all medical care for these individuals. In a report prepared for FSANZ in June 2006 it was assumed that around 40% of those with Spina Bifida require mobility aids (crutches and or a wheelchair) and most require continence aids and that around 50% of those with Spina Bifida require orthotics.


An estimate of the costs associated with personal aids including mobility aids (crutches, wheelchairs), continence aids (pads, lubricants, bowel washers and catheters) and orthotics (leg callipers, footwear and compression stockings) were compiled based on advice from the Spina Bifida community and other primary data sources. Modifications to houses included provision of ramps and handrails and modifications to bathrooms and toilets. Modifications to vehicles included provision of hoists and changes to gears and pedals. These modifications require large outlays but were amortised over a number of years, lowering the average cost per year. Expenditure for children is relatively high reflecting the faster changeover in orthotics aids because of individual growth. In relation to schools and workplaces additional costs for Spina Bifida over and above current disability access to buildings might only include equipment (eg. modified school desk and computer and similar at work). While most children with a NTD attend mainstream schools, the Spina Bifida community advised that many have teachers’ assistants. These costs have not been estimated here but the Ministry of Education provides funding of $5000 per child/year for specialist support teachers for children assessed as having high needs. For a person with Spina Bifida, expenditure on personal aids, and modifications to home and car add significantly to the costs associated with the condition. Personal aids comprise the largest component of expenditure on aids and modifications and are similar in nature to health expenditures. The table below provides estimates of costs per person per year in each age group. Some of these outlays are subsidised by governments. The costs in Table 4:11 represent the full costs.

The lifetime cost of aids and modifications for an Australian with Spina Bifida - assuming life expectancy of 68.1 years for males and 73.8 years for females (Mathers et al 1999) - is around A$183,000. The equivalent life long cost in NZ under the same assumptions is around NZ$195,000. There are potentially significant cost savings to the Ministry of Health for provision of mobility equipment alone through the introduction of mandatory folate fortification. Although the costs for a person with Spina Bifida are funded by the Ministry of Health it is worthwhile comparing the lifetime liability costs from ACC for each of their clients with a Spinal Cord Injury, which although not identical in nature has the same patient outcomes as a person with Spina Bifida. ACC operate under a completely different funding model where costs of injuries are tracked, unlike the MOH. The costs quoted are for social rehabilitation costs only (equipment, attendant care, home help, housing modifications, transport), excluding loss of wages, vocational rehabilitation or medical treatment and don't delineate between paraplegia or tetraplegia.


The lifetime cost of a spinal injury is around $9.6 million per person and is dependant on the age at which a person is injured: This represents the total that ACC expects to pay on an average spinal injury claim over the person’s lifetime – from the time of their accident until their death. If, however, the person is under 20 when they are injured (comparable to a baby born with Spina Bifida), the total rises to $12.1 million because ACC funded support is provided for a longer period of time. As at 31 December 2010 ACC were supporting a total 1,772 people with a spinal injury and actuarial estimates of how much ACC expects to pay supporting these people for the rest of their lives amounts to $3.703 billion. Information received from Accessable notes that the level of impairment/disability experienced by individuals with Spina Bifida vary dramatically and as such, they do not have the ability to provide a clear indication of how much money is spent on an individual over their lifetime with regards to Equipment and Modification Services. They were however able to provide figures based on applications for funding received over the last 12 months or so for housing modifications for those with low to moderate support needs and those with moderate to high support needs. People who are mobile with low-moderate support needs Bathroom/toilet modifications (3 rails and hand held shower)

$2870.00

Access Modifications handrails on 4 steps)

$850.00

(bilateral

People who are non-mobile with moderate -high support needs Bathroom/toilet modifications (combining toilet and bathroom; installing level access shower) Access Modifications (ramp or installation of low rise lift on 4 steps)

$10-14,000

$3,500.00

The Ministry of Health currently provides up to $10578.26 +GST towards the purchase of a vehicle (subject to income and asset testing) and up to $10578.26 +GST towards modifications for eligible persons (not subject to income and asset testing). The cost of a modified vehicle for a wheelchair bound passenger (van with wheelchair hoist) is usually around $31,000.00 The cost of a modified vehicle for a self driver (able to transfer on/off car seat) is usually around $22,000.00 for modifications including hand controls and wheelchair roof hoist The cost of a modified vehicle for a self driver (driving from wheelchair) can be $60-$100k depending on whether it is a second hand already modified vehicle or if the modifications are new. Information received from companies which are funded by the Ministry of Health to supply equipment to people with disabilities show that the following equipment is commonly supplied to those with Spina Bifida.


Equipment Type

Average Cost

Basic 4 section electric hospital bed

$

4,665.00

Pressure care mattress

$

1,718.33

Mobile Hoist

$

2,495.00

Hoist Sling

$

395.00

Wheelchairs (Basic Manual)

$

998.33

Wheelchairs (Power)

$

4,500.00

Wheelchairs (ULW) 3-4 Chairs between 0-16yrs

$

4,294.50

Wheelchair Cushions

$

744.00

Wheelchair Backs

$

1,075.00

Standing Frames

$

4,500.00

Walking Frames

$

5,166.67

Sleep Systems

$

4,000.00

Shower Stool

$

110.00

Over toilet frames

$

130.00

Shower commode chair

$

3,450.00

Kitchen aids

$

60.00

Transfer/handling aids

$

112.50

Between the ages of 0-16 it is common for children to have between 3 and 4 wheelchairs supplied as they grow at an average price of $6113 (including cushion and custom made backrests). Between 1996 and 2009 there have been 275 children born with Spina Bifida. Wheelchair provision is projected to have cost $5,883,900. The Ministry of Health operates a refurbishment programme for all equipment supplied to the disability sector. The above costing does not take this into account. EMPLOYMENT AND WELFARE Many people with Spina Bifida face serious barriers to participation in full time work and a proportion are less likely to be employed (or less likely to be employed full time) than those without Spina Bifida. Most are likely to work in part-time, possibly voluntary positions which they will hold for relatively short periods of time. This may reflect both the impairment of those with Spina Bifida as well as discrimination.


In trying to quantify the extent to which employment rates are reduced relative to the total population, studies have identified that the probability of employment for a person with Spina Bifida is around 60 per cent below average in both Australia and New Zealand. The relatively low levels of employment of those with Spina Bifida mean that they not only forego income, but also pay less personal income tax. People with lower incomes also consume a smaller set of goods and services and indirect taxes levied on consumption also fall. Additional taxes must be raised elsewhere to cover these taxation receipts foregone. People with Spina Bifida who are unable to work due to their disability may be eligible for the Invalids’ Benefit. In a report prepared for FSANZ it was noted that 76 per cent of those with an NTD who are of working age (18 to 64 years) are likely to be in receipt of the Invalids’ Benefit. This compares with 3.1 per cent New Zealanders aged 18 to 64 receiving the Invalids’ Benefit. The difference in the rate at which the general population is in receipt of welfare payments and those with NTD who are in receipt of welfare is 72.9 per cent. Efficiency losses are calculated on these ‘excess’ dependency rates. The average payment rate for the Invalids’ Benefit is $16783 per annum. These payments are calculated as the total expenditure on that benefit divided by the number of people receiving the benefit. The Domestic Purposes Benefit (DPB) is only available if the care recipient has a severe level of disability. It is assumed that children with high level Spina Bifida would meet this requirement. Thirty per cent of carers would be caring for a child with high level Spina Bifida. By comparison, the proportion of working adults receiving the DPB in the general population is approximately 4.1 per cent in New Zealand. For those caring for children with Spina Bifida it is assumed that one parent remains out of work to care for the child for 20 years and receives a carer pension for the full period. The average payment for the Domestic Purposes Benefit is $18238 per annum. This is calculated as the 2009-10 expenditure on carers’ benefits divided by the number of people receiving a carers’ benefit. It is noted that this estimate is likely to be an over-estimate of the average payment which carers of children with an NTD receive because carers’ benefits primarily consist of benefits to sole parents which are payed at a higher rate than payments to people in couples. Welfare payments to people with Spina Bifida and their carers create efficiency losses similar to those for foregone taxation as taxes are required to fund these payments. In New Zealand applicable welfare payments for people with Spina Bifida include the Invalids’ Benefit, the Disability Allowance and the Community Services Card. Parent carers in may be eligible to receive the Domestic Purposes Benefit, are entitled to the Child Disability Allowance irrespective of degree of disability caused by Spina Bifida and the Disability Allowance (income and asset tested eligibility rules apply). In addition to the loss in productivity due to those with Spina Bifida being less likely to work, the labour force participation of their carers is also affected, leading to additional productivity losses. A significant number of people with Spina Bifida receive informal care from family and friends. As informal care is often unpaid, it is sometimes also thought of as free. However, the time devoted by a carer is time he or she cannot use for other activities such as paid employment or leisure activities. The time foregone by caregivers is valued as an opportunity cost ie the value of wages foregone. Estimating the hours per week spent by carers of those with Spina Bifida is difficult. The number of


hours of care needed per week will vary according to the level of impairment, and by the person’s age. Children with Spina Bifida generally require a lot of informal care and it is common that one parent will need to stay out of the workforce (or both will work part time) until the child leaves school or some time thereafter. Once a person with Spina Bifida reaches adulthood, he or she will typically continue to require assistance with nursing and personal hygiene, cooking, house work and maintenance, shopping, and recreational activities which is provided by the MOH through the Home and Community Support Service. People with Spina Bifida and their carers may also obtain paid care through respite services that are one of a range of support services paid for by the Ministry of Health. Respite services are available to disabled people and to carers, family and whanau whose primary role involves the care and support of a disabled family member. Carer support is a subsidy funded by the Ministry of Health to assist the unpaid, full-time carer of a disabled person to take a break from caring for that person. This type of care is in addition to unpaid care above. A proportion of expenditure on these types of services could be potentially avoided through the implementation of mandatory folate fortification. FOLATE FORTIFICATION INITIATIVE From 1990 onwards national and international research has linked increased folic acid intake with reduction in neural tube defects (NTDs) of between 50 to 70 percent. In 1993 and amendment was made to the Food Regulations Act 1984 determining flour fortification level allowed at 100mcg per 35g of flour (ie 285mcg/100g). The Public Health Commission recommended dosage of 0.8mg of folic acid for women of childbearing age four weeks before pregnancy and for 12 weeks into pregnancy and launched a communications programme. New Zealand CCS (now CCS Disability Action) picked up the campaign to raise folate awareness in 1995. The Prevention of Spina Bifida in New Zealand report was produced by Emeritus Professor Bob Elliott and Dr Seema Singh at Child Health Research, Department of Paediatrics, University of Auckland and they recommend the Folate Replenishment-Plus Programme, a proposal to fortify all flour with folate to reduce the incidence of NTDs and bolster the folate status of all New Zealanders. The Public Health Commission distributed consumer pamphlets .The flour milling industry was approached to lend support to the proposal through its research trust. The Folate ReplenishmentPlus Committee funded by the Rotary Club of Newmarket was formed in 1997 to co-ordinate and promote flour fortification initiative and a national nutrition survey established that dietary folate levels in New Zealand are low (200mcg). A consultation and communications campaign began in addition to negotiations with the Ministry of Health. The United States commenced mandatory fortification from 1 January 1998. A study on Consumer Attitudes on the Fortification of New Zealand Bakery Products by the Department of Consumer Sciences, University of Otago was commissioned by the Baking Industry Research Trust. Discussions began with the New Zealand Association of Bakers after the Folate Replenishment-Plus Committee were persuaded to revise the flour fortification proposal to fortification of bread. The Australian and New Zealand Food Authority (ANZFA) launched the folate health claim and continued negotiations with the corporate and business community.


The Ministry of Health issued terms of reference to the Folate Replenishment-Plus Committee in February 1999. In a project funded by the Ministry of Health and ANZFA University of Otago researchers commenced a study to collect data on folate intake and blood folate levels to provide a baseline so that effectiveness of public health strategies to improve folate status could be monitored. A study providing insight into folate intake and status, Nutrition During Pregnancy, was published by Massey University. In March 2000 the Ministry of Health endorsed the folate proposal. The disability sector represented by New Zealand CCS stressed in October 2001 that they were unable to sustain a prolonged campaign either fiscally or philosophically as folate fortification was a public health issue and not a disability issue. The Hon. Annette King gave unqualified support in November 2002 to progressing mandatory fortification with urgency – with introduction no later than November 2004 and instructed that a New Zealand Food Standard requiring mandatory fortification in 2 years be developed. In September 2003 the Ministry of Health released its Public Health Intelligence Occasional Bulletin Number 18: Improving Folate Intake in New Zealand: Policy implications which reviewed the (then) recommendations, and noted that NZ women did not have a sufficient daily intake of folic acid to reduce the risk of NTDs. In 2004 the Australia and New Zealand Food Regulation Ministerial Council asked Food Standards Australia and New Zealand (FSANZ) to look at the risks and benefits of mandatory folate fortification. A consultation document, Proposal P295-Consideration of Mandatory Fortification with Folic Acid was released and sought to review the background science, literature, and research as well as the future options for reducing NTDs in New Zealand. In April 2005, FSANZ commissioned four expert reports from researchers on the risks and benefits of mandatory folate fortification. FSANZ’s provisional assessment was that mandatory fortification could achieve a reduction in neural tube defects conceptions from 8% to 44% at an intake of up to 1mg/day with no significant identified risk. Professor Godfrey Oakley, Professor of Epidemiology at Emory University, said that since 1998 following mandatory fortification in the United States, serum folate concentration increased substantially and there were approximately 1000 fewer Spina Bifida and anencephaly pregnancies a year. There was strong support for the notion of mandatory folate fortification of flour. It was seen to be desirable, financially and technically feasible, of low risk, and of considerable potential benefit for the prevention of neural tube defects. In July 2005 a seminar was hosted by the Australian Health Policy Institute, Effective Healthcare Australia, Global Health Institute and Sydney West Area Health Service to canvass a range of perspectives on folate policy and to critique existing arrangements. It was attended by 70 people including senior health service managers, clinicians and researchers, government officials, academics, industry, consumer and disability representatives. It was noted that although there had been an overall reduction in the number of children born with neural tube defects, Professor Stanley demonstrated that there was an ongoing disparity in the frequency of these defects between Indigenous and non-Indigenous people. She stressed that mandatory fortification of flour with folate is an important preventive strategy that can reach all members of the population. FSANZ have estimated that folate fortification would prevent up to 14 neural tube defects a year.


In a 2006 submission to FSANZ the Paediatric Society of New Zealand strongly supported the mandatory fortification of the food supply with folic acid commenting that the opportunity to reduce the number of affected individuals by primary prevention (that is, ensuring those individuals are born in good health without disability) rather than secondary prevention (that is, terminating the lives of those individuals found to be affected) must not be missed. The emotional and financial consequences to an affected individual, to his/her family, and to society, of Spina Bifida and other neural tube defects are enormous. A reduction in the number of terminations has implications for reducing hospital care costs, and more importantly reducing morbidity associated with termination in these women and improving their reproductive health. The Paediatric Society noted that there is good evidence that interventions such as voluntary fortification, periconceptual supplementation and dietary modification with folate-rich foods do not, either alone or in combination, produce the desired public health outcome. Voluntary supplementation is not effective in achieving adequate folic acid levels in the target population. It is also unlikely that voluntary fortification plus pre-conception folic acid supplementation will significantly reduce the incidence of neural tube defect even with publicity because of the high rate of unplanned pregnancy (50%) and the likelihood that women may not be reached by such a campaign if conducted, supplements are unlikely to be taken consistently and women in a disadvantaged socio-economic situation and at the highest risk of having a diet low in folic acid. may be disadvantaged by the cost of purchasing supplements. The most recent (1997) Adult Diet Survey showed that virtually all women in New Zealand have dietary folate intakes below the 400 micrograms (mcg)/day recommended in pregnancy. The usual daily median intake of folate from food for the New Zealand population was 242 mcg (males 278 mcg, females 212 mcg) with intakes varying little across age groups. 52 countries have recognised the value of folic acid fortification for their population and have proceeded with its introduction and there is an impressive body of research documenting the efficacy of mandatory fortification of flour with folic acid in achieving a reduction in the true incidence of Neural Tube Defects which is unknown in New Zealand. The Paediatric Society believes that the true population incidence is likely to be between 50 and 60 per annum, and that the live birth prevalence now represents only a minority of affected cases and as such claims that the overall incidence is low are fallacious. Professor Fiona Stanley, Australian of the Year, a leading Child Health Researcher and Director of the Telethon Institute for Child Health Research commenting on the Australian State and Federal Food Regulation Ministers commitment to proceed with mandatory fortification of food with folate in October 2006 said that the decision would ensure that many more women received enough folate to protect their babies from devastating neural tube defects (NTDs) such as Spina Bifida in addition to saving families and children from the heartache that comes with the daily challenge of living with a severe disability. She also noted that every month that mandatory fortification of flour is delayed condemns more unborn babies to a life with a severe, but often preventable, birth defect. The cost saving of this initiative to the Australian health system in 2006 was estimated at $120-150 million each year. In 2006 the Australian Medical Association expressed extreme disappointment after the Australia and New Zealand Food Regulation Ministerial Council confirmed that mandatory fortification would be delayed by up to six months while Food Standards Australia New Zealand (FSANZ) reviewed the


proposed standard. The delay meant that potentially 25 more Australian families would experience a pregnancy affected by a neural tube defect. The Australia and New Zealand Food Regulation Ministerial Council met in June 2007 to consider mandatory fortification of food with folic acid and affirmed the draft standard. As per the policy principles agreed by the Ministerial Council the mandatory fortification of the food supply was implemented in response to a demonstrated significant population health need because it was the most effective public health strategy to address the problem, was consistent with the nutrition policies of both Australia and New Zealand, would not result in detrimental excesses or imbalances and would deliver effective vitamins to the target population to meet the health objectives. A transition period of two years was agreed with mandatory fortification to be in place by 27 September 2009. Briefing papers prepared for Hon Kate Wilkinson in June 2009 addressing the case for mandatory folic acid fortification of flour (Australia) and bread (New Zealand) noted a study commissioned by the milling and baking industry in which 87 percent of the population claimed not to favour folic acid fortification, however a more academic survey found that only 13 percent of a random sample of West Australians were concerned about folic acid fortification. Carol Bower, clinical professor and Senior Principal Research Fellow with the Telethon Institute for Child Health Research in West Perth, Western Australia, was the chief author of a work that studied the question of how many cases of NTDs would be prevented by fortification. An estimated 11 NTDs could be prevented each year in New Zealand with an increased folic acid intake of 200 mg per day with the range of NTDs which would be prevented each year in New Zealand between 6 to 18. Using the upper end of that range, as many as 25 percent of NTD cases in New Zealand could be prevented with bread fortification and based on the experience of other countries such as the United States and Canada show that this can be realistically expected. Fortification of wheat flour with iron began in the 1940s, and currently 57 countries require that at least one type of flour be fortified with at least iron or folic acid (returning significant levels of vitamins and minerals to flour that would have been there originally prior to milling). Of those, 51 include requirements for folic acid. The scientific basis for flour fortification was evaluated by nearly 100 leading nutrition, pharmaceutical and cereal scientists and milling experts from the public and private sectors from around the world in 2008. They concluded that when flour is fortified with appropriate levels of folic acid, based on appropriate estimates of per capita consumption of fortifiable flour, the intervention does not appear to pose a public health risk. In April 2009 The World Health Organization released a statement endorsing flour fortification guidelines based on findings from the 2008 workshop with consideration given to recent studies. It includes guideline for fortifying flour with iron, folic acid, zinc, vitamin B12 and vitamin A but decisions about what nutrients and appropriate amounts of such nutrients added being determined by each country. In July 2009 Lyall Thurston noted that the Folic Acid Fortification Programme initiated 20 years ago had reached fruition with bakers legally obliged to add folic acid to their bread, apart from organic, in September 2009. This requirement was met by the Food and Grocery Council and Bakers Association who began an inflammatory campaign to scare the public and force Government to overturn the legislation. An unpublished research paper with pooled analysis of all randomised control trials of folic acid conducted by the Clinical Trials Service Unit at Oxford in the UK covering


35,000 individuals consuming high dosages of folic acid supplementation in countries around the world over the past 14 years was released. A submission to NZFSA by Lyall Thurston in August, 2009 acknowledged that the fortification issue was complex in nature and that it had taken 20 years and six successive government terms, industry and public consultation to get this initiative from the laboratory to legislation to avoid babies being born with avoidable, significant and often fatal neural tube defects or disabilities. In 2001 signed cross party political support was obtained and rigorous due process was completed with the Ministry of Health, NZFSA and FSANZ. Firm alliances with New Zealand’s leading health, woman and child related organisations and professional bodies had been maintained, backed by international experience and the ongoing and in-depth global research and monitoring of 57 countries who now fortified their flour and other food products and supported by the World Health Organisation. He quoted the most recent collated statistics of 12-15 babies per year born or stillborn with NTDs every year with a conservative estimated of a further 25 to 35 babies identified with a NTD through ultrasound terminated at 18 weeks every year. Based on 2009 births of 64,160 in New Zealand more than 130 babies over the next three years were likely to be affected in New Zealand by neural tube defects and most of these will be terminated. Those with any experience or knowledge of NTDs and the emotional pain they cause parents and the physical pain they cause the child support the fortification initiative. If the folic acid fortification initiative had been scuttled by valid evidence of health concerns it would have been accepted but this consistently scrutinised public health initiative was postponed due to a campaign based on lies, mistruths and public manipulation. As a society, we are unable to demean disabled people by parading physically deformed babies before the public or film the pain the parents feel as they watch their baby being terminated to demonstrate the trauma and conflict that a NTD diagnosis causes. A targeted public education campaign on the complexities of folic acid and NTDs would require millions of dollars in ongoing publicity to have any significant effect but the effects of folic acid fortification are monitored and measured internationally every day. What results continue to find is that micrograms of folic acid – a B group Vitamin – benefits all in the population but is the difference between hell and heaven for parents of children born with, and without, a vicious and preventable disability and there is no basis on which to amend the current legislation. As reiterated by the New Zealand Food Standards Authority, the New Zealand population is known to be folate deficient by at least 150 mcg per day. It is also known that folic acid is required as a population as it is converted to folate in the human body which helps enzymes to build DNA and RNA. It also appears to have a role in controlling the activity of genes. Women of childbearing age particularly need it because at least 400mcg/day has been found conclusively to reduce the incidence of NTDs by up to 70 percent. Natural folate is found in foods such as breakfast cereals, wholegrain breads, orange juice, green vegetables, eggs, fruit and yoghurt. To reach the 400mcg women would continue to eat normally with folic acid fortified bread in their diet. The bread supplementation is as a top up, and to replace, and boost the natural folate milled and baked out of the bread during processing. Meeting the requirements of the Australia and New Zealand Food Regulation Ministerial Council has involved extensive research into risks, costs and benefits, full public consultation and hundreds of


thousands of taxpayer dollars. Industry was accommodated by the change in requirement to fortify bread and flour. Checks and balances were implemented with the review requirement after two years and testing the efficiency of the monitoring framework. Direct consultation with the bakers concluded with them asking Government to make it mandatory. The target population for folate fortification are women of child-bearing age which represents at least 840,000 women aged between 15 to 44 (1996 Census) however population benefits have been proven with no evidence of risk. While it is acknowledged that mandatory fortification will not provide maximum protection it does make a significant difference, particularly when combined with a diet high in natural folate. The perceived high level of public resistance is based on misinformation and lack of understanding. An eminent Canadian doctor, Dr AGW Hunter has been quoted as saying that “we need to feel a sense of responsibility and failure every time a baby is aborted because of an NTD, or a child is born with this preventable disability�. Mandatory fortification of folic acid in New Zealand was to have been jointly implemented in Australia and New Zealand in September 2009. Australia implemented the food standard but the incoming New Zealand National Government led by Prime Minister John Key under political pressure from the New Zealand milling and baking industry put implementation on hold. This development is disastrous in terms of public health policy here in New Zealand as there are benefits for all New Zealanders from the introduction of the Standard. The challenge for folate prevention of NTDs is that it needs to be consumed even before women know that they are pregnant because the neural tube closes so early, hence all women of child bearing age need to increase their folate intake to prevent the small number who are at risk of having an affected baby. It is common public health practice to vaccinate the whole population to avoid serious illness, death and disability in an even smaller number of people than are affected by neural tube defects. Professor Smith said in the interview for TVNZ Q+A that a reduction of number of 5 neural tube defect cases could be prevented if folic acid to bread was made mandatory. In contrast, Dr Andrew Marshall, Specialist Paediatrician for Capital Coast Health DHB and also interviewed for the same programme, noted that the Ministry of Primary Industry had also done a very robust calculation and had estimated that 24 neural tube pregnancies a year were likely to be prevented with mandatory folate fortification. Opponents to this public health initiative refer to the drop in numbers of babies born with Spina Bifida. This is due to most women having an ultrasound scan which diagnoses Spina Bifida, with most choose to terminate the pregnancy. The New Zealand Government needs to acknowledge that the termination of an NTD affected pregnancy following prenatal diagnosis is not prevention and instead ensure the implementation of primary prevention measures such as the recommendations on folate levels of women of childbearing age.


The United Nations Convention on the Rights of the Child The United Nations Convention on the Rights of the Child (UNCRC) was signed by the NZ Government on 1 October 1990 and ratified on 6 May 1993 and as such precedes articles included in the United Nations Convention on the Rights of Persons with Disabilities. On 6 October 1997 a general discussion was focussing on the rights of children with disabilities. The committee noted that ‘throughout history children with disabilities had been denied access to education, family life, adequate health care, opportunities for play or for training and to participate in “normal” childhood activities and that their plight rarely figured high on the national or international agenda’. It also noted that there is a responsibility by society to protect children from disability before birth and that we should be concerned about any and all circumstances which might result in disability at the time of birth. The current Folate Fortification initiative designed as a public health initiative to further reduce the incidence of Neural Tube Defects has been calculated to cost $0.25USD per annum per person. The New Zealand Government is bound by their agreement to the principles of the UNCRC to protect children from disability before birth by making the addition of folic acid to bread mandatory. THE LINK BETWEEN FOLIC ACID AND PREVENTION OF CLEFT LIP & PALATE A cleft lip is a separation in the upper lip, while a cleft palate is an opening in the roof of the mouth. Clefts result from incomplete development of the lip and/or palate in the early weeks of pregnancy. A cleft lip and palate occurs in approximately one in 700 live births. Cleft lip, with or without cleft palate, is most frequent in boys and isolated cleft palate is most common in girls. The condition causes significant morbidity: the effects on an individual’s speech, hearing and appearance, along with psychological effects, can lead to long-lasting adverse outcomes for health and wellbeing. Even when repaired, complications such as persistent ear infections, speech impairments, facial deformities and dental problems often persist. It had been thought likely that folic acid had a role beyond that of the developing neural tube and the risk of spina bifida, but previous research had failed to establish any significant linkage. But now the Growing Up in Ireland study, a national project led by Trinity College Dublin and the Economic and Social Research Institute (ESRI) to be published in the British Journal of General Practice, has put the issue beyond doubt. Dervla Kelly and her research colleagues have connected expectant mothers’ folic acid intake and the prevalence of Cleft Lip and Palate. Data from the New Zealand Birth Defects registry shows that between 2000 and 2009 that a total of 212 babies were born with Cleft Lip and Palate, 189 were born with a Cleft Lip only and 644 were born with a Cleft Palate (1,045 babies in total) 11. LINK BETWEEN FOLIC ACID AND IMPROVED COGNITIVE FUNCTION A study published in the American Journal of Clinical Nutrition by Janine Walker, a researcher at Australian National University has found that taking vitamin B12 and folic acid for two years 11

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improves both short and long-term memory. Vitamins have an important role in promoting healthy ageing and mental wellbeing, as well as sustaining good cognitive functioning for longer on a community-wide scale. The researchers asked more than 700 people aged 60 to 74 years to take a daily dose of folic acid and vitamin B12, or placebo pills that resembled the vitamins. The vitamin dose included 400 micrograms of folic acid and 100 micrograms of vitamin B12. After a two year period those who took the vitamins showed more, if modest, improvement in their scores on the memory tasks. Joshua Miller, a professor at the University of California, Davis, said that on a population level, a small increase in cognitive function can have very real ramifications on the functioning of the population as a whole, and on the costs of healthcare. LINK BETWEEN FOLIC ACID AND SPEECH LANGUAGE DEVELOPMENT Folic acid may have other childhood benefits besides reducing neural tube birth defects12. Researchers analyzed language development in 38,954 Norwegian boys and girls whose mothers did or did not take 400 mcg of folic acid per day, from four weeks before becoming pregnant to eight weeks afterward. Doctors measured severe language delay, which they defined as speaking only one word, or making only unintelligible sounds. In children whose moms did not take folic acid, 9 out of 1,000 had severe language delay. For children whose moms did take folic acid, the rate of severe language delay was less than half, or 4 in 1,000. IN SUMMARY To date 75 countries around the globe have joined together and introduced mandatory folate fortification. There is no argument that folate plays a key role in preventing Neural Tube Defects for those women planning a pregnancy, but we have a situation mirrored around the world where 50% of all pregnancies are unplanned. But folate plays a key role in other key areas of health, improving cognitive function, development of speech language and prevention of cleft lip and palate. Folate fortification has health benefits for all of society. These benefits not only have an impact on the health budget but on the lives of everyday New Zealanders. It is time the New Zealand Government introduced mandatory folate fortification to improve the health and welfare of future generations.

Diane Belcher Executive Director Spina Bifida New Zealand 027 878 7584

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SBNZ Subission on Folate Fortification to New Zealand Ministry of Primary Industries