HIPE Virtual 2023, three community members' questions were addressed.
Question: Would putting your loved one on female hormone therapy, like low- estrin, bring down sexual aggression in a male living with Huntington's disease? Have there ever been any studies on this?
Dr. Karen Anderson Responds
That is a terrific question, and as you said, I'm so glad that people are not afraid to ask about sexual experiences in HD in general, particularly about aggression that seems to be sexually driven.
There have not been terrific studies on this in HD, unfortunately. If you look in the world of treatment of sex offenders, which is where we get some of our information on this, there are a couple of classes of drugs that are used with repeat sexual offenders. Not to say that people with HD or sex offenders, this is just where we're getting our information, where there's been the most studied.
So, to back up for a second, putting a man on estrogen might not be the best thing to treat sexual aggression because you could cause breast development and also testicle shrinkage. There are a couple of alternatives. The simplest one, and one that I do use sometimes, is to use SSRI antidepressants.
There are a number of SSRI antidepressants like Paxil, Zoloft, and Prozac. They're pretty common and pretty easy to prescribe. For some people, an antidepressant helps because of serotonin.
For some people, an increase in the level of serotonin in the brain calms them and helps with irritability. So that in and of itself can be really good. It also decreases sex driving. So we're using a side effect.
Some people on SSRIs don't like them because they can decrease sex drive, but if that's a problem and it's causing aggression, then it can actually be a good treatment option. So that's a case where we're using a side effect of an antidepressant to treat something. There is literature from the world of treating sex offenders using drugs that suppress testosterone, like progestins and anti-androgens.
So that would be something where it might take a bit more effort to find a doctor who has experience prescribing this. But that is another option that you could pursue rather than thinking about giving estrogen to a man for that particular indication.
Question: What is the most important thing to do before testing for HD besides getting life insurance?
Emilee Piechota Responds:
That is a good question. This a very common question that I receive. I would say here at Georgetown; we do things a little differently than a typical Center of Excellence. So when you call to schedule that initial genetic testing appointment, you're calling me directly, the social worker.
And when you call, I want to know more about where you are in your HD journey. I want to know when and why. So, when did you decide to be tested, and why did you decide to be tested or to begin this process? And more often than not, individuals who are calling me to be tested grew up in an HD family.
So, they grew up taking care of dad with HD or taking care of mom with HD. They grew up with grandparents, aunts, uncles, and siblings. So they know what HD is. They aren't calling me to tell them what HD is. They already know what HD is.
And many people grew up their entire life knowing in the back of their minds that they were probably going to get tested at some point. And more often than not, it's more, when I receive that call, it's because they're going through a major life event. So maybe they just got married, perhaps they're thinking about having kids or thinking about buying a home.
They just finished college. They finally feel like they are established and should have time to go through that process. So again, bringing in that, where are you in your HD journey? When did you decide you wanted to get tested? And why you want to get tested is so important. And by doing that, I'm able to bring up, "what are you going to do if you test positive"?
"What are you going to do if you test negative"? And being able to manage those thoughts and feelings. I'm, of course, am going to have the conversation about life insurance, long-term life insurance, and the importance of advanced directives, but I just want to continue to revisit where they are in their journey.
Here at Georgetown, we are a bit unique as well because, as a social worker, you can call me as many times as you need to. I am more than happy to sit down with you, talk with you on the phone, have a telehealth conversation with you before you come in, while you come in, and after you receive those results.
Our genetic counselor is also free, so you can also connect with her as often as you want as well. So again, having everything in place, long-term care, life insurance, making sure you have a good support system, but also making sure you're preparing yourself to have either a positive or a negative result.
Question: Would you recommend someone with HD in later stages to get a feeding tube?
Dr. Erin Furr-Stimming Responds:
That is another great question. And first, thank you so much for having me today, and thank you to all the brave folks that submitted these tough questions.
This is a question that we frequently get in our clinic. And unfortunately, I don't have a perfect answer or solution. This is a question that that in which the answer differs for everyone. So as most of you know, as HD advances and progresses, swallowing becomes more difficult.
And so, you may have heard the term dysphagia, which just means difficulty swallowing. Dysphagia can put individuals at risk of aspirating so that food or liquid they're consuming may end up heading down the wrong pipe and end up in the lungs instead of the intended target, the stomach.
And so, aspiration, unfortunately, is not uncommon. And if folks’ aspirate, they may develop aspiration pneumonia. We know that it can be very difficult for individuals with HD to keep their weight up to maintain a healthy weight. And there are various reasons for that.
And malnutrition is not uncommon as the disease progresses. And suppose our patients are experiencing dysphagia, and they're having a tough time keeping their weight up. In that case, we may have this discussion about whether or not to place a feeding tube, otherwise known as a peg, which stands for Percutaneous Endoscopic Gastrostomy.
This is a tube that's placed directly into or a catheter that's placed directly into the stomach that allows individuals to receive liquid nutrition. There are many factors that individuals really need to think about and talk about with their loved ones before pursuing potentially a PEG, I do like to initially bring up just some points and clarify some misperceptions that many of my patients may have about a PEG tube.
So if an individual has a PEG tube placed, they can, importantly, still consume food and liquids orally. We will often call that pleasure feeding. So because we all enjoy eating and drinking, that can still occur if you have a feeding tube or a PEG tube in place.
Most individuals that have a PEG tube will receive their food intermittently. So they don't have to be connected to feeds 24/7. They may be, but they don't have to be. So they can receive what we call bolus feeds.
The procedure itself to place a PEG tube is fairly straightforward.
It’s usually done in an outpatient setting by one of our gastro GI colleagues and gastrointestinal colleagues. And it's a straightforward procedure. However, there can be complications. And so even though the procedure itself is straightforward, there can be complications. The most common complication is an infection.
PEG tubes even if individuals are not consuming foods orally and have a PEG tube placed, PEG tubes do not necessarily decrease or eliminate the risk of aspirating. And that's something important to be aware of. We don't have a ton of literature surrounding PEG tubes in individuals specifically with HD, although there was a relatively recent study published in 2021.
And Dr. Sam Frank, who will speak with you later today, was the senior author of this paper. And this was a paper that was a retrospective chart review. So, the authors reviewed charts from individuals that were in a long-term care facility on the East Coast. And they reviewed the charts from 148 individuals.
Thirty-nine of these individuals had a PEG tube placed. And ultimately, they found that individuals that had PEG tubes placed had an extended duration of life on average of three years.
They found that they did not have a decrease in the incidence of pressure ulcers or a reduction in the frequency of pneumonia. And the PEG tubes did not improve their nutritional status. Now, this was, as I said, a chart review. We know there are inherent limitations to these types of studies, but it gives us some data in HD.
And so there are still many questions when you're reviewing the literature as to whether we should move forward with the PEG tube. The bottom line is there's no absolute wrong or right answer when it comes to placing a PEG tube.
It's important that you have this discussion, if you can, with your loved ones early in the disease course when you're thinking about your advanced directives. It's an important discussion that we should all have with or without HD.
Know that it's appropriate to ask your neurologist or primary care doctor this question and know that it's okay to be conflicted about whether or not to move forward. But there are absolutely folks who can help you and guide you in trying to make the best decision to pursue or not pursue a PEG.
To see more of the HIPE Panel please go to https://www.youtube.com/watch?v=_5964VFlgVk