Huntington's Disease: Reproductive Decision-Making

A Debate

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
Touchiness
Depression
Delusions

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

Dysphagia

Increased muscle activity often makes eating and swallowing difficult

Incontinence
Altered 24-hour schedule

Patient may become nocturnal

General lack of personal hygiene

Normal Brain vs. Brain Affected by HD

NORMAL AFFECTED

Prenatal Testing

Some common prenatal tests

Huntington's Disease
Tay- Sachs Disease
Muscular Dystrophy
Down Syndrome
Hemophilia
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

Stigmatization

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 life?

The diseases will become less common and less of an issue

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 Disease
IV. Case Study

V. Concluding Comments

Overview of Huntington's Disease (HD)

- 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 is broken.

- Early symptoms can be mild enough to go unnoticed at first and may include depression, mood swings, forgetfulness, clumsiness, twitching, and lack of coordination.

- Since the gene discovery, scientific interest in HD has greatly increased, and so has the understanding of what causes the disease.

- Hopefully, more research breakthroughs...and a cure....will soon be forthcoming.

TREATMENT

Although, current treatments do not alter the course of Huntington's disease, there have been some major advancements in finding a possible cure.

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 degeneration.
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 symptoms.

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."

CONCLUSION

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 disease.

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 run
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 a disorder.

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.