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Huntington's Disease: Reproductive Decision-Making
Bredon Jones and Cynthia Lee
What is Huntington's Disease?
- Also called:
- Huntington's Chorea
- Lund- Huntington's Chorea
- Setesdal jerks
- An incurable, hereditary type
of brain atrophy
- A type of "presenile dementia"
Who does HD affect?
- Affects 1 in 10,000
- 150,000 are at risk in the U.S.
- Children of those who have HD have
a 50/50 chance of inheriting the disease
- Anyone who has not developed the gene
has no chance of passing it on to their children
Mental Symptoms of HD
- Change in personality
- Loss of memory
- Reduced concentration
- Loss of initiative
- Irritability/ aggression
Neurological Symptoms of HD
- Involuntary flicking movements
- These occur suddenly and irregularly
with chance distribution all over the body
- Gait disturbances
- Slow and long- lasting muscle spasms
- Speech and language problems
- Disjoint and indistinct speech
- May not be able to answer questions
Other Signs of HD
- Increased muscle activity often makes
eating and swallowing difficult
- Altered 24-hour schedule
- Patient may become nocturnal
- General lack of personal hygiene
Normal Brain vs. Brain Affected by HD
- Some common prenatal tests
- Huntington's Disease
- Tay- Sachs Disease
- Muscular Dystrophy
- Down Syndrome
- Trisomy 18
- Cystic Fibrosis
- An estimated 200 of these tests are
for inherited disorders
Reasons for Having an Abortion
- Jobs and income
- Financial and lifestyle burdens for the family
- Insurance discrimination
- Legally inadmissible but a practical concern
- How long can this be kept from the child?
- Having child = child abuse
- What kind of life will this child have?
- How motivated will this person be to have a productive
- The diseases will become less common and less of
Is it the presence of life...
Šor the quality of living it?
Why you shoulf NOT
abort a fetus that has a strong chance of having Huntington's Disease in
its adult life!
A. J. Plummer and Brian Plummer
I. Overview of Huntington's Disease
II. Clinical Genetic Testing of Huntington's Disease
III. Current Medical Treatment of Huntington's
IV. Case Study
V. Concluding Comments
Overview of Huntington's Disease
- Degenerative brain disorder for which at present
there is no cure.
- 30,000 Americans have Huntington's Disease.
- Males and females are affected equally and HD crosses
all racial and ethnic boundaries.
- A further 150,000 have a 50-50 chance of inheriting
the disease from an affected parent and are said
to be "at risk".
- Those who do not inherit HD CANNOT pass
it on to their children and the chain of inheritance
- Early symptoms can be mild enough to go unnoticed
at first and may include depression, mood swings,
forgetfulness, clumsiness, twitching, and lack
- Since the gene discovery, scientific interest in
HD has greatly increased, and so has the understanding of what causes the
- Hopefully, more research breakthroughs...and a
cure....will soon be forthcoming.
Although, current treatments do not alter the course of Huntington's
disease, there have been some major advancements in finding a possible
The 2 biggest current treatments on the market today are:
- Axokine-Which is produced by a company called Regeneron. The procedure
is that the surgeon directly administers Axokine into the patient's brain
and protects the nerve cells from degenerating. Unfortunalty there is no
significant that this procedure does work in humans. Since it is still
relatively new the only testing is done in rats. But there is significant
evidence to support that the chemical axokine protects nerve cells form
- Neurotransplantation-Is where the surgeon transplants bits of one human's
brain to another. Currently there are 4 medical centers in the U.S. that
are using the procedure. The procedure, which is controversial because
it uses tissue from, aborted fetuses for the transplant. You may feel that
this is a contradiction. But the tissue comes from elective rather than
spontaneous abortions. The women are asked to consent to the use to the
tissue only after the abortion-and she is never paid. Identities of both
donors and recipients are confidential.
Procedure- the nerve tissue form the patient undergoing the transplant
is minced and mixed with prepared tissue from at least 3 fetuses. Then,
2 small holes are bored in the patient's head; degenerative points in the
brain are mapped with the use of a MRI. Then the tissue mixture is grafted
onto damaged sections of the brain with a needle. Although still relatively
new it appears the operation can reverse some to he debilitating Huntington's
Case Study- Jim and Sam Fitz
- Jim, 47, and his brother, Sam, 57
- Both men underwent neurotransplantation surgery
- Prior to the surgery Jim found it difficult to process questions or
from coherent answers.
- While Sam exhibited slurred speech and involuntary muscle spasms that
made him unsteady and created jerky movements.
- After the procedure Jim is now able to converse with family and friends.
- Sam's speech still bears slight traces of slurring, but is remarkably
clear. And as of yet he does not suffer from any tremors.
- This procedure is not a cure both men must undergo therapy in the form
of exercises-both for the body and the brain.
- Quote from Sam, "My body feels like it belongs to me again, before
I felt like a big shaky bowl of jelly, I'm starting to feel like myself
again, I can finally see a future without a wheelchair."
I'm not going to disagree that Huntington's disease is not a terrible
disorder to have and for most people who are in the late symptoms of the
disease a possible cure is probably too late.
But we are talking about an unborn fetus and as statistics show the
symptoms of Huntington's disease doesn't even appear until midlife (30-45)
And on top of that the symptoms may run anywhere from 10 to 25 years.
Say for example, that the woman is pregnant now. That means it may be
2028 at the earliest that this disease starts to show up. And given the
evidence that these treatments I have just discussed who's to say that
there won't be a cure before this person start too show symptoms of Huntingtons's
Also I read that these genetic tests are about 95% accurate. And although
that pretty accurate there is still a 5% chance the test could be wrong.
Case study- 2 women in England
- 2 women entered the hospital the same day to have some tests
- The hospital confirmed that one of the women baby had down's syndrome
- So the women who thought she had a baby with downs syndrome elected
to have an abortion.
- It was only after the abortion that the hospital realized that they
had gotten the two women's lab results mixed up and had actually aborted
a healthy baby.
- When the other women who thought she had a healthy baby found out the
truth she aborted her baby also.
- 2 babies died when only 1 would have.
So with the information presented here by A.J. and I we see no reason
why anyone should abort a fetus even if it shows strong evidence for developing
Many core ethical issues were addressed
in this topic: abortion, genetic testing, and various neurological diseases.
Brian and I posed the argument that you should not abort a fetus based
on the fact that it has the potential to have Huntington's Disease later
in life. In order for us to make this argument, we gave a background on
the clinical genetic testing and treatment that could be done in the case
of Huntington's Disease. For example, in March of 1993 scientists announced
that they had found the gene which causes HD. Due to this somewhat recent
discovery, many avenues of research are presently being investigated in
the search for a treatment or cure for HD. The gene discovery has also
made it possible for a new predictive test for HD which allows those at
risk to find out whether or not they will develop the disease. Because
of these recent advances in the field of genetics, techniques in which
geneticists can determine if a fetus will be deformed or afflicted genetically
are also being developed and modified. The advances in genetic testing,
while remarkable, are also allowing devastating consequences: more and
more parents are choosing to abort their unborn children. The dilemmas
posed by new genetic technology are nowhere more agonizing than for prospective
parents who are increasingly faced with a bewildering range of opportunities
and choices as they contemplate doing what used to be the most natural
thing in the world, and who soon may find themselves having to decide whether
or not to terminate a pregnancy because their child is at risk for Huntington's
Disease. Carried one step further, the ability to find out whether or not
a child will be deformed or afflicted might cause the death of a normal
individual in a effort to be rid of a possibly genetically diseased fetus.
We need to closely examine the ways in which genetic technology affects
our attitudes. Close to 100% of women who have tested positive from amniocenteses
for Down's syndrome have abortions. Aborting defective or diseased fetuses
is widely sanctioned because if born, they are a burden....their families
would suffer, they may suffer, and society suffers. We have this need for
perfection in our society..we need to detect and eliminate defective babies.
We have this warped mentality that we are in control of our very existence
and that we can develop some sort of "super-race". We need to
recognize the limits of humanity and quit trying to play God. I found a
quote from a woman whose fetus was at risk for HD and she refused genetic
testing. She states, "In our success-oriented, beauty-obsessed culture,
the low intelligence or physical handicaps of defective, deformed, or diseased
babies leads us to assume that such an individual's life is not worth living.
And yet we are appalled that anyone would abort a fetus just because it
is a girl. But the Chinese and Indians routinely do so. To Indians, a female
child can be a lifelong burden; to be a female in China is to risk abandonment
or even murder; it's not a life worth living. The point is that the value
we put on human life is socially and culturally conditioned. We may believe
we are doing the right thing, but are we?" Should we abort every deformed,
defected, or diseased baby? If not all of them, then we just abort one.
We can select whose life is going to be more valuable than others, because
every single life has meaning and a purpose.
Brian and I made several arguments opposed to abortion
in this case. For example, if one does carry the gene for HD they have
a 50% of not getting the disease. In retrospect, they also have a 50% chance
of developing symptoms. Even then one does not usually develop symptoms
until the fourth or fifth decade. So one could live a completely normal
life until age 40 or 50. Many middle-aged men and women lead fulfilling
lives and can contribute to society. HD usually progresses over a 10 to
25 year period, so one would live for 60 or 75 years. In our opinion, 40
years is better than no years! In 40 years there could be a cure for all
we know! We all die sometime. If you knew your child was going to be in
a car accident and be a quadriplegic from age 16 on, would you abort him/her
from birth...or would you just wait until he/she was handicapped and then
kill them? Of course you wouldn't! You would love that person and that
individual would have probably contributed some meaning to your life. Suppose
your "healthy" child developed spinal meningitis, are your going
to kill that child too because he is a burden? Every life has the potential
to affect another's whether the individual is healthy, deformed, or diseased.
Take a chance on life, your life and theirs.