The Last Right

by Marianne Thamm

  Itching sometimes happens in NF1. This seems to be worse when exposed to heat (for example, when taking a hot bath). Avoiding situations that you know will make matters worse is helpful. Medication such as antihistamines or a simple emollient can sometimes help – ask your GP for guidance.

  Learning and behaviour problems

  Most people with NF1 have normal intelligence, but around two thirds of people who have the condition will experience some problems with learning. It is the most common “complication” of NF1. The majority of children who have NF1 are educated in mainstream schools and do not require special education, although they may benefit from extra help.

  Children and adults who have NF1 and who have learning problems may have difficulty reading and writing, they may find it hard to concentrate, have memory difficulties or have poor coordination. Where someone with NF1 has learning problems, this is often evident from their earliest years at school and, as children, they may underachieve at school and have difficulties establishing and maintaining peer friendships.

  Research has demonstrated that there is an increased incidence of Attention Deficit Hyperactivity Disorder (ADHD) and Attention Deficit Disorder (ADD) in NF1. There is also an increased incidence of mild autism (sometimes called Asperger’s). These conditions should be assessed, diagnosed and supported in the same way as any other individual where this is suspected.

  There can sometimes be behaviour difficulties at home and at school.

  These difficulties can be similar to the difficulties found in children who do not have NF1, where there are conditions such as dyslexia, dyspraxia (clumsiness) and attention difficulties.

  It is important that these problems are recognised as early as possible so that teachers are aware of how NF1 can affect school performance. It is useful to have a frank discussion with teachers so that common misconceptions about NF1 can be dispelled and the child can get help in school if he or she needs it.

  Learning and behaviour problems in NF1 are not progressive, that is they do not get worse over time. In fact, they can usually be improved with appropriate help.

  Deciding to have children if you have NF1

  NF1 is caused by a change in the structure of a gene. Each person has about 30 000 genes in their body. Genes are the set of instructions within cells that tell the body how to grow, develop and function.

  Genes come in pairs, so we all have two copies of the NF1 gene. If a person has NF1, one of these copies will have an alteration or miscopy.

  When someone with NF1 has a child, he or she passes on one of their two copies of the gene: either the normal one or the copy with the NF1 alteration. If the normal copy of the gene is passed on, the child is very unlikely to have NF1. If the altered NF1 gene is passed on, the child will have NF1.

  Therefore, every person with NF1 has a 50:50 or one in two chance of passing the condition on to each of their children, boys or girls.

  For adults with NF1 who are planning their family, there is no way to predict how mildly or severely a child who inherits NF1 will be affected.

  The decision whether or not to have children is a very personal one for a couple and may depend on personal experience of NF1.

  Prenatal diagnosis (testing an unborn baby in the womb) and preimplantation genetic diagnosis (PGD) techniques are now available to couples planning a family.

  NF1 and pregnancy

  If you are generally in good health, there are no specific concerns about pregnancy and NF1. If you are pregnant and you have NF1, you should let the maternity unit know that you have NF1 at the first antenatal visit.

  Some women report an increase in the number of neurofibromas and an increase in size of existing neurofibromas. When your baby arrives, they should be checked for features of NF1 from the first months of life by a community or hospital paediatrician, geneticist or clinic specialising in NF1.

  There is no reason why a mother with NF1 should not be able to breastfeed. Occasionally the presence of neurofibroma(s) around the nipple can create difficulties for the baby to “latch on”.

  What do I need to do to look after myself or my family if we have NF1?

  If you have NF1, you should expect to live a long life in good health. Most people who have NF1 go through life with relatively few medical problems. NF1 can cause life-threatening problems but these are rare. It is important to have regular medical follow-up so that any complications can be identified early.

  If you notice unusual health changes or symptoms it is important to seek medical advice sooner rather than later.

  Remind your doctor that you have NF1 and ask the question: “Could this be linked to my NF1?”

  If the problem does not go away or is getting worse, return to your doctor for further advice or ask to be referred to a specialist.

  It is important to note that your GP may not see many people with NF1 and for this reason they may not know how variable NF1 can be from person to person.

  If you are an adult, it is a good idea to see your GP once a year for a general health check-up and to have your blood pressure checked. It is also a good idea to have regular eye checks with your optician. See your doctor if you have any new or unexplained symptoms.

  If you have a child with NF1, it is advisable that their growth, development and general health is monitored from diagnosis until adulthood. The paediatrician will advise you how frequently your child needs to be reviewed.

  Brain scans are not routinely performed in NF1 unless there are specific reasons to do this, for example unusual symptoms. If you notice any changes in your health (as described earlier) outside of routine review appointments, it is sensible to seek medical advice. If symptoms do not go away, then it is important to return to your doctor for further advice because if you do not, the doctor will assume that you have recovered from the problem.

  Authors:

  Rosemary Ashton (Abbott) – Neurofibromatosis Specialist Advisor, The Neuro Foundation

  Hilda Crawford – Neurofibromatosis Specialist Advisor, Belfast City Hospital Trust

  Co-authors and medical advisors:

  Professor Patrick Morrison – Consultant in Clinical Genetics, Belfast City Hospital Trust

  Dr Shane McKee – Consultant in Clinical Genetics, Belfast City Hospital Trust

  Dr Susan Huson – Neurofibromatosis Centre Genetic Medicine, St Mary’s Hospital, Manchester

  For more information visit www.nfauk.org.

  2

  The Law

  “The greatest insult to the sanctity of life is indifference to laziness in the face of its complexity.”

  – Ronald Dworkin

  The Global Perspective

  In November 1994, the US state of Oregon approved the Death With Dignity Act (DWDA) and became the first state in that country to allow physician-assisted suicide for terminally ill patients.

  The DWDA requires individuals who seek assisted suicide to meet strict conditions, including the voluntary self-administration of lethal medication, that they be resident in the state, and that the prognosis is for a life span of six months or less.

  Since the DWDA was enacted in late 1997, a total of 935 people have had prescriptions written and 596 patients have died from ingesting medications prescribed under the law.* The anomaly in the totals proves that some people, once they have obtained permission for an assisted suicide and receive the prescription, opt not to use it. The knowledge that they can resort to the prescription if required has been shown to lessen anxiety about end-of-life issues and some patients move on to receive standard palliative care.

  In 2008, physician-assisted suicide with certain restrictions became legal in the state of Washington with the passing of the Death With Dignity Act. The law in this state also requires that the patient ingest medication without assistance.

  In January 2009, Montana became the third state in the US to legalise self-administered lethal doses of medication prescribed by doctors to terminally ill, mentally competent individuals. At the time of writi
ng, New Jersey state had introduced a bill to decriminalise assisted suicide, which polls suggested was likely to be passed.

  At the turn of the century, two countries in Europe legalised assisted suicide and euthanasia – the Netherlands in 2002, followed by Belgium later the same year.

  While active euthanasia is illegal in Switzerland, the country provides a unique legal framework for assisted suicide, including for foreign nationals. Assisted suicide is permitted only if the recipient is able to make an autonomous choice and physically take the lethal cocktail without assistance. The specific clause, Article 115 of the Swiss Penal Code, was approved in 1937 but only came into effect in 1942.

  In 2012, for the first time, the Swiss Federal Statistical Office (FSO) released statistics on assisted suicide in that country.* In 2009, there were 300 assisted suicides, which corresponds to 4.8 per 1 000 deaths. Statistics prior to 1998, when just fewer than 50 assisted suicides were recorded, are not available. Statistics for the same year in Belgium are 7.9 per 1 000 deaths, while in the Netherlands only 2.3 per 1 000 deaths were reported in 2010.

  In 2009, Luxembourg became the third European country to permit assisted suicide with the passing of the Palliative Care/Euthanasia Bill, which requires terminally ill patients to draw up an Advance Directive or Living Will.

  International discussion about the question of assisted suicide and euthanasia has grown increasingly urgent, as several countries in the world, including the United Kingdom, Scotland, New Zealand and Australia, have reopened debate.

  Euthanasia was briefly legal in the Northern Territories of Australia with the passing of the Rights of the Terminally Ill Act of 1995. However, an amendment to the Northern Territories Self-Government Act by the Commonwealth government resulted in the controversial repeal of the Act in 1997. At present euthanasia and assisted suicide are illegal in Australia, however, the issue is being debated by the New South Wales Parliament.

  Debate in New Zealand has been particularly robust, with two attempts at changing the current legal status. The Death with Dignity Bill was championed in 1995 and 2003, when it was narrowly defeated in Parliament by three votes (60 to 57).

  In 2008, a controversial publication, The Peaceful Pill Handbook, was unbanned in New Zealand and is permitted to be sold to readers over the age of 18 and only if it is sealed and the age restriction displayed. However, author Philip Nitschke, a vocal advocate for assisted suicide and euthanasia in New Zealand, agreed to cut a chapter that set out specific methods of suicide.

  In March 2012, Member of Parliament for the New Zealand Labour Party, Maryan Street, forwarded a private members bill, the End of Life Choice Bill, to the parliamentary ballot box to forward the debate.

  South Africa

  South Africa’s laws currently prohibit assisted dying and euthanasia. However, in 1998, the South African Law Reform Commission (SALC) published a report, Euthanasia and the artificial preservation of life project 86, which included a draft bill, the End of Life Decisions Bill 1998.

  President Nelson Mandela commissioned the report after an approach by SAVES, a Living Will society. It was tabled in Parliament in 2000 but has received no attention since then.

  The End of Life Decisions Bill proposes the legalisation of passive euthanasia, which is defined as the withholding or withdrawal of artificial life support without which a person would die naturally.

  Section 4(1) of the draft reads: “Should it be clear to a medical practitioner or a nurse responsible for the treatment of a patient who has been diagnosed by a medical practitioner as suffering from a terminal illness that the dosage of medication that the patient is currently receiving is not adequately alleviating the patient’s pain or distress, he or she shall – with the object to provide relief of severe pain or distress, and with no intention to kill – increase the dosage of medication (whether analgesics or sedatives) to be given to the patient until relief is obtained, even if the secondary effect of this action may be to shorten the life of the patient.”

  The Bill also provides three options on voluntary active euthanasia, to be discussed in Parliament and opened for public comment: the first is that the law remains as it is; the second is the legalisation of active euthanasia with a doctor making the decision to euthanise; and the third that it be legalised with the final decision resting with an independent panel.

  In March 2012, Professor Willem A Landman, Executive Director of the Ethics Institute of South Africa, Professor Extraordinaire in the Department of Philosophy at the University of Stellenbosch and co-founder with Professor Melodie Slabbert and Professor Sean Davison of Dignity SA, presented a position paper at the First Annual Congress of the Faculty of Consulting Physicians of South Africa.

  Professor Davison founded Dignity SA in September 2011, after returning to South Africa from New Zealand on bail. He was later sentenced by a New Zealand court to five months’ home detention for helping his terminally ill mother, Patricia, to die in 2006.

  The organisation, says Davison, aims to inform the public about euthanasia and to seek legal reform in South Africa. Since its founding scores of people have turned to Dignity SA with heart-wrenching stories of tremendous end-of-life suffering.

  Professor Landman’s document, rather than an exhaustive academic research paper, was intended to put forward an ethical case for end-of-life decision-making in South Africa.

  Such legislation, he suggests, would clarify areas of legal uncertainty, most notably in respect of terminal-pain management, withholding and withdrawal of life-sustaining treatment (also referred to as “passive euthanasia”), and advance directives (a Living Will and a durable power of attorney for healthcare).

  Professor Landman’s paper also argued for the inclusion of assisted dying (assisted suicide and voluntary active euthanasia) in a comprehensive end-of-life decision-making bill, which would mean decriminalising what is currently unlawful.

  Professor Landman has argued that the End of Life Decisions Bill 1998 should serve as a basis for public debate.

  “Globally, many countries are addressing assisted dying, albeit in different ways. Some have legalised assisted dying and others have set criteria for not prosecuting it. In South Africa, the debate should be based on the spirit, values and rights in the Constitution, particularly the interpretation of the right to life and its intimate connection with the right to dignity.”

  The South African Constitution should shape “a moral community that binds us together as citizens, despite the different personal, ethical and religious beliefs we might have in our local communities. A constitutional democracy means, among others, that we resolve our differences about the social arrangement of society by appealing to the Constitution, thus granting others the right to live by their convictions even if we disagree with them.”

  The most urgent issue is the current suffering of people in the end-of-life stage, and that the law, as it currently exists, fails to recognise appropriately that human life is finite and that the dying process is a natural part of life.

  The following chapter features an extensive extract from Professor Landman’s paper.

  3

  Legal Reform and Clarity

  In May 2012, Professor Willem Landman of the Ethics Institute of South Africa presented a research paper, End-of-life decisions, ethics and the law: A case for statutory legal clarity and reform in South Africa, in Cape Town at the First Annual Congress of the Faculty of Consulting Physicians of South Africa.

  Professor Landman’s paper is an exhaustive review of the law in South Africa, but for purposes of this work, we have extracted his research specifically in relation to assisted suicide. For the full report, go to www.ethicssa.org.

  ASSISTED DYING (assisted suicide and voluntary euthanasia) is an end-of-life decision-making practice that should recognise the moral right of individuals in the terminal phase of dying to assisted dying, as well as the corresponding moral obligation of caregivers, family and the state to respect that right.

  Wh
ereas the other three practices in end-of-life decisions – terminal pain management; withholding and withdrawal of potentially life-sustaining treatment; and advance directives – simply require greater legal clarity than is currently the case, assisted dying would require substantial legal reform (decriminalisation) since it is unlawful in both its forms.

  The key question is whether legalising assisted dying would be consistent with – or perhaps even required by – the Bill of Rights in the Constitution, particularly the right to life.

  Key terms for a discussion of the ethics and law of assisted dying are the following:

  Assisted dying, in its wider sense, refers to all end-of-life interventions in the dying process – terminal-pain management and comfort care; withholding and withdrawal of potentially life-sustaining treatment; respecting advance directives; and assisted suicide and voluntary euthanasia. In its narrower sense, “assisted dying” refers only to the last two, namely, assisted suicide and voluntary euthanasia. The significance of using an umbrella term – such as “assisted dying” or “euthanasia” – for assisted suicide and voluntary euthanasia, is that they represent two distinguishable acts that are sufficiently similar from ethical and legal points of view to justify such a joint classification. Whereas the SALC (South African Law Commission) Report and draft bill use the umbrella terms “assistance with dying” and “euthanasia” for these two acts, this Position Paper prefers “assisted dying” or “assistance with dying” for these two acts, that is, in their narrower sense. (Analogously, we decriminalised “termination of pregnancy”, not “abortion”.)

  Assisted suicide (or “doctor-assisted suicide” or “physician-assisted suicide”) occurs when a medical practitioner or other person intentionally provides the means for a competent person to take their own life, in which case the patient’s act is the proximate cause of death in the causal chain of acts, events and conditions that results in the patient’s death. For example, a medical practitioner, in response to a request by a competent patient, writes a prescription for a lethal drug whereupon the patient ingests it. Or a family member gathers a sufficient dosage of lethal substances, enabling the patient to ingest them.