With summer heat cranking up and promising to a few more precious months of summer, it’s important to remember that while dehydration is a long term problem for those with HD it is also a seasonal issue as well.
HD patients can have problems with dehydration for many reasons.
1. Decrease in ability to notice body temperature changes
2. As we age, body water content decreases.
3. Some medications increase dehydration.
4. Reduction in thirst leading to decreased intake.
5. Difficulty swallowing
The effects of dehydration in the extreme are often recognizable to those in the HD community, we see it in our late stage relatives as their general consumption decreases with disease advancement. But some effects of dehydration can be difficult to spot in an HD individual because they can exacerbate symptomatic behavior.
Studies suggest that even a slight dehydration of 2% in healthy individuals can negatively effect cognitive function, mood, choice reaction time, short and long-term memory, and attention.
One of the best ways to keep your HD loved one hydrated is to offer water frequently and often. Simply having a cold water bottle nearby might be enough to help initiate water intake, but may take some nudging from a caregiver.
Also, if a loved one is perhaps stubborn about drinking water, try to freshen up the options. For instance, berry and coconut water can be an excellent way to hydrate a loved one while also boosting some vital nutrients. To make a palatable coconut water “cocktail”, simply make a sugar/water syrup (if you’ve made hummingbird feed at home you’re probably well acquainted with this step) by boiling sugar and water in a 1:3 ratio in a pot until the mixture dissolves and becomes a thicker syrupy concoction. Refrigerate. Next, simply blend any berry of your choice (blueberries are packed with antioxidants which are great for HD patients) with the syrup and coconut water, strain if desired, and serve over ice. The mixture is super healthy and refreshing!
For more insight into how much water you or your loved one are taking in vs how much you may need, check out this: Hydration Calculator
My mother often, throughout the last 20 years but in particular during certain periods, would exhibit “perseveration”—the repetition of a topic, function, or strategy beyond its context and usefulness. The term may also refer to the obsessive and highly selective interests of individuals suffering a wide range of neurological disorders or brain trauma.
For her it was almost always the recurrence of topic in conversation. The best, well only, way to deal with it was just to let her run the gamut of her opinions on the topic (often gossipy things about people I didn’t know, or my sister not calling, or me getting dental insurance, etc), and afterward trying to lure her away with an engrossing activity that could make her brain shift gears. There were times when she would call me a dozen times a day and leave the same exact message every time. I always explained it to my husband like her thoughts were a car on a muddy trail that would always find the same ruts.
This infographic is a bit small to see on a phone, but it is jam packed with useful information about this term and its full meaning.
In this podcast from the Michael J. Fox Foundation, Kevin Kwok discusses his experience after having deep brain stimulation surgery. Advances in the technique promise a future where stimulation can be fine tuned for individual needs and thus, perhaps, reduce the side effects associated with DBS.
While selective activation of the brain has proven to relieve many symptoms of Parkinson’s Disease, such as tremors, rigidity, and gait issues, DBS has obtained very selective application approvals from the FDA. Many new studies look at deep brain stimulation for use with “mental health issues” and is most commonly associated, especially in the Parkinson’s community, with the work of reducing tremors; but it’s also gained some ground in the area of chronic pain relief. It is this application that I think most exciting for the HD community.
Huntington’s patients commonly have uncommon reactions to prescription medications. Sometimes pain relieving medications have limited effectiveness, while others create exaggerated side effects. Add to this the complexity of chronic pain in general, and the HD patient’s increasingly dysfunctional interpretations of pain and neuron messaging, and effectively treating chronic pain in the HD patient can become a series of guesses; in which doctors, patients and caregivers spends months or years testing approach after approach to relieve pain.
But DBS could be exciting for any number of reasons, once its mechanisms are more fully understood. Fox’s foundation is highlighting some really interesting research that is producing real time results with actual patients, not just early stage mouse studies, and this is yet another area that should be given serious attention.
The article linked below discusses how as we age we can more readily recall information when we are with a partner we’ve spent decades with. I think this is true for other close relationships, not just a husband and wife.
But I think it’s interesting because it highlights the importance that loved ones and caregivers play in keeping our loved one from losing themselves and their history. My mom would habitually form false memories, and I would often find myself delicately trying to suss them out and encourage a more accurate picture.
As an HD individual progresses from middle to late stage, their communication diminishes and it’s hard to know what impact our visiting or speaking as on their psyche. This study holds the implications that, perhaps, without our social circle, without our core family, without our partners, in whatever form they may come (sister, mother, spouse), we stand to lose ourselves and/or our loved ones all that much quicker.
As the aging process gradually transforms our bodies, changes in vision are a natural evolution. Since we enter the era of most dramatic eye degeneration around the same time as HD onset, it is important to understand how vision naturally changes and how that may add to the challenges Huntington’s presents.
In parallel to the average age of HD onset, one of the most common processes affecting vision, called presbyopia, develops around age 40. Presbyopia is the gradual hardening of the eye lens, which causes difficulty in focusing on small objects or close items. Presbyopia is typically treated with reading glasses, bi-focals or transitional lenses. As hardening continues with time, so too prescriptions may need repeated adjustment from year to year.
Other frequently developing conditions include decreased color vision, dry eyes, and tearing (having too many tears). Peripheral vision loss also begins, at a rate of about one to three degrees per decade of life. By 70 to 80 years of age, there can be a decrease of 20 to 30 degrees of peripheral vision. Additionally, the muscles controlling pupil size loses strength, reducing the eyes’ responsiveness to light. Because of this, adults in their 60’s require three times more ambient lighting to read comfortably than those in their 20’s.
After age 50, the risk of eye diseases increase, particularly for glaucoma, macular degeneration, and cataracts.
While technically classified as an age related disease, cataracts are so common some consider them a natural part of aging. Cataracts cause blurred or hazy vision and can exist as small easily ignored areas, or cover the entire eye. Cataracts disrupt contrast sensitivity, which is the ability to discern between bright and dim elements in a static image. Research from Indiana University indicates that symptomatic HD patients also show a decline in this sensitivity. Surgery is the typical treatment for cataracts, and usually restores the vision lost or hampered by the cataracts. However, if the decline in contrast sensitivity is due to HD progression, daily adjustments to create a higher contrast environment can help.
Unlike the hazy look cataracts cause, macular degeneration (AMD) manifests in distorted vision and a dimming or loss of central vision. Macular degeneration is incurable and the leading cause of blindness among American seniors. However, treatments to slow the disease include quitting smoking, eating leafy greens, and taking an AREDs supplement.
Risk for glaucoma increases each decade after age 40, from around 1% up to 12% by age 80. While there is still research ongoing determining the cause, intra-ocular pressure plays an important role. Glaucoma can develop over time, or it can be brought on by eye infections, blocked blood vessels inside the eye, or injury to the eye. In most cases, there are no identifying symptoms until the optic nerve has been damaged. It is for this reason that regular eye exams are crucial for early detection.
HD patients often develop ocular motor apraxia (OMA), a condition where purposeful horizontal and quick eye movement becomes impaired. There are several elements to this apraxia, including delayed initiation of movement, fixation impairment, and inability to follow an object without moving the head. Unlike those that develop OMA due to hemorrage or tumor, OMA in HD patients does not seem to effect the vestibular-ocular reflex. This is the reflex which causes the eye to move in the opposite direction of head movement to maintain image stability.
Like so many elements of Huntington’s, there is no solution to resolve the issue of OMA; however, healthy lifestyle choices such as abstaining from smoking and attention to diet can help reduce the severity of many of these conditions. For some diseases of the eye early detection and condition management is the only path. Understanding how these various impairments present themselves is therefore important not only for ourselves, but for the ones that we look after.
“Sleep is the interest we have to pay on the capital which is called in at death; and the higher the rate of interest and the more regularly it is paid, the further the date of redemption is post-poned.”–Arthur Schopenhauer
The internet is a veritable buffet of lists: 7 Superfoods to Eat, 10 Relationship Red Flags, 12 80’s Movies You Should Watch Again and, more pertinently to today’s blog, lists upon lists about getting sleep, sleeping better, and the detriments of losing sleep. List fatigue or no, sleep research is still churning out some very interesting data. Specifically, research into the complex process of circadian rhythm and hypothalamus dysfunction, which has specific indications for neurodegenerative disorders.
Sleep disturbance can cause a host of negative influences throughout the body. Sleep deprivation leads to increased irritability, agitation, decline of cognitive function, even disrupting the breakdown of sugar. Since behavior, such as eating, television, and exercise habits, can greatly influence the sleep process, we find that correcting detrimental behaviors can increase the amount and quality of our sleep and return our bodies to a healthy schedule and stasis.
For the HD patient recuperating from a bout of sleep dysfunction may not be as easy as catching a few extra hours of sleep. The major effects of sleep deprivation exacerbate mood disorders and cognitive dysfunction that the HD individual is already experiencing. This can create a spiraling situation for the individual, and further aggravate relationships potentially already under strain. Addressing this issue is of great importance; however, these outward behaviors only scratch the surface of how circadian disruption disturbs the body’s entire system. What we’re examining here is not just sleep and sleep dysfunction. Sleep is a circadian behavior, and research suggests that circadian dysfunction is at play in disease dysfunction for neurodegenerative diseases. For instance, sleep dysfunction can appear in Parkinson’s patients years ahead of visible motor dysfunction.
Understanding the Science
Sleep cycles are controlled by the circadian rhythms in the body, all of which are controlled by the suprachiasmatic nucleus (SCN), a small area located in the hypothalamus. Research has shown hypothalamic function is greatly affected by HD, most easily evidenced through weight loss. It is unclear exactly how this occurs, but research shows possible connections with oxidative stress and mitochondrial dysfunction. The SCN is directly above the optic chiasm and utilizes specialized light receptors to dictate the circadian rhythm timing system. The SCN uses hormones, particularly cortisol and melatonin, to regulate the system. The SCN can be considered the body’s master clock, coordinating the cycles of the body’s various other clocks (which regulate specific organs and systems within the body).
This process effects every aspect of the human body, down to transcription of each cell. These active and passive cycles allow our bodies to “time” its efforts. We can think of the circadian rhythm (and the SCN that regulates them) like the shift in objectives at a 24 hour store. Staff that arrives in the morning ensures displays are ready (the body waking up), handles customers and balances product (sorting out the input as we move through our day), while the night shift cleans up (our body processing our meals, eliminating toxins), fills the shelves (we rejuvenate during sleep), etc.
Research by Mortin and colleagues has shown a progressive breakdown of circadian and activity cycles for the HD patient. Preliminary research with mice shows SCN neurons firing more sluggish during the day, and less quietly at night as are found in HD-free specimens. This generates a comparatively balanced activity chart, with activity leveling out between cycles. While we may seek balance in many other areas of the body, this activity level is a dysfunction in the system. The body should be highly active at times and quietly resting at others. This dysfunction leads to the hypothesis that HD patients are further hindered in their ability to process hormones, toxins, and metabolize in the manner it typically would, in addition to becoming less and less able to truly rest the body’s systems.
This dysfunction doesn’t just affect the cognitive and emotional processes. Dr. Chris Colwell of UCLA illustrated that mice experiencing this disruption had elevated blood pressure and pumping dysfunction. Further, Colwell’s research indicated molecular physiology that appeared to be working, but a growing drift in the synchronization of the various systems’ clocks. Colwell’s hypothesis, then, is that circadian dysfunction accelerates disease dysfunction.
Adjusting Behavior in the Face of Disease Acceleration
Following the logic of this hypothesis, what are the steps for retarding the dysfunction? First, we must consider the circadian/sleep dysfunction as an integral part of the disease. Second, we should identify some key elements that present environmental barriers to regulating sleep/circadian cycles.
Our body responds to full spectrum natural light (which is not the same as the “full spectrum” marketing term used for light bulbs) and regulates our cycles accordingly. This means that living under strictly fluorescent lighting, which emits ultraviolet light waves, can confuse the SCN. Hospitals and nursing homes typically have poor lighting in this regard. Many retailers now carry light boxes, which emit up to 10,000 lux. Light boxes are used in what is called phototherapy, and is used for people with circadian disorders as well as sufferers of seasonal affective disorder (SAD). Large units (of the 10,000 lux variety) can run several hundred dollars, with smaller units costing less than $100. Utilizing the light box can delay the onset of sleep and activate the SCN to recognize the active part of its cycle. Blue light glasses can also be obtained, which serve to block the blue light and induce the release of melatonin.
Exercise is a vital element for care, for all individuals, but in particular HD patients. According to a study at the University of Georgia, brief periods of exercise for just 20 minutes facilitates information processing and memory functions. Research out of UCLA illustrates that exercise for merely one week of activity presented increased axonal regeneration. Exercise facilitates brain growth and increases sensory neurons’ ability to create new connections and pathways. This function is vital for those with neurodegenerative disorders.It’s also important to schedule exercise for the morning/afternoon portion of the day. Exercising in the evening or after dinner can delay the release of melatonin and onset of sleep.
The body needs to be conditioned to relax into the sleep cycle. To this end, meals should ideally be scheduled allowing enough time for the body to process it before retiring for bed. Snacking late at night is a common behavior for many adults, but especially HD patients. Curbing late night snacking can help the body shut down properly. As Colwell discussed, refraining from eating late not only aids in bringing on sleep but lead to improved performance on motor tasks.
Prescription medications are another element for consideration. Aside from the possible side effects of a given drug, timing of drug treatment matters. Certain medications, such as betablockers for hypertension, should be taken in the morning, while other medications should be taken in the evening, depending on how the body will metabolize those drugs. Adding a supplement of melatonin can help some whose body isn’t producing enough of the hormone. It’s best to discuss the sleep cycle disturbance or concern with the prescribing physician to ensure that all medication directions optimize the goal of regulating the sleep cycle.
The research into circadian dysfunction in Huntington’s Disease is in preliminary stages, but the combined efforts of many past studies lends the belief that this is a promising area of research. If we can aid in the regulating of the circadian cycle, and if this can help slow the progressive deterioration in the brain, then we’ve got a tangible, actionable course of treatment that can have short term and long term benefits.
Seth Rogan is stirring up some press today for Alzheimer’s and the seeming lack of congressional interest in the disease. Before we become too personally infuriated with congressional leaders, it’s important to note that most speeches made to congressional hearings are made, not to senators, but to their aides and the staff. However, I whole heartedly agree that a speech about Alzheimer’s should rank higher than a lobbyist’s lunch date. Are we all really comfortable with the knowledge that low attendance is the norm on capitol hill?
On the upside, it’s all generating talk about Alzheimer’s and the plight of “elderly disease” in this country. Here is Rogan’s site, Hilarity for Charity, which seeks to use humor and good times to raise money for a serious cause. All work that is beneficial for not only the Alzheimer’s community but the HD community as well.