Advanced Stages of Huntington's Disease Caregivers Handbook

Medical Issues
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Advanced Stages of Huntington's Disease
Caregivers Handbook
Medical Issues
Sleeping, Screaming, Sweating & Temperature Variation, Urinary & Bowels,
Seizures, Fevers, Contractures & Chorea

SLEEP AND SLEEPLESSNESS

Some people have difficulty sleeping. A change in one's sleep pattern, much more of it or much less of it, is a classic sign of depression. Little sleep and a very high activity level while awake could be a sign of mania. If you see this, report it to the appropriate health care professional so a complete evaluation can be made.

Due to daytime fatigue in advanced HD, some folks accidentally fall into a cycle of napping during the day and then being unable to fall asleep at night. You can usually find the right balance between conserving energy during the day and being tired enough to sleep through the night. Try to help them maintain their "rhythm of life".

UNEXPLAINED SCREAMING

Certain people with HD persistently scream for reasons that are not readily apparent to their caregivers. Since they're unable to simply say why they're screaming, it's a challenge to their caregivers to figure it out.  It could be a need that's been overlooked and gone unmet.

They could be in pain or panic; hallucinating or heartbroken. They might be frightened, anxious, grieving or hurt and have just one way to express all these different feelings... screaming! In the most advanced stages of HD, it may be related to medication or the cramps that come with the changes in their muscle tone.

Try to figure out exactly what triggers the screaming. This will mean systematically trying one approach after the other, asking other caregivers and the family for ideas of what may be causing it.

Some folks may also make frequent unusual sounds as they encounter problems coordinating their breathing: gasping, sniffing, grunting, slurping sounds, etc.  Since these sounds are not under their control, caregivers should graciously tolerate them.

EXCESSIVE SWEATING, TEMPERATURE, AND THIRST

People with HD may be more comfortable in surroundings that are cooler than typical, perhaps as low as 18° Celsius. This may be related to some aspect of the disease that affects metabolism. They may also have episodes of excessive sweating. In some cases this may be related to certain medications.

Others may have a compelling thirst. Those who drink an excessive amount of liquid per day should consult their physician regarding potential problems with electrolyte imbalance and kidney function.

FREQUENT URINATION AND CONSTIPATION

He will have more and more difficulty thoroughly emptying his bladder, as his muscles become progressively uncoordinated. Increased thirst may lead to increased fluid consumption. This often causes him to sense the need to urinate more often than usual.

Problems controlling impulses, coupled with the increased urges to urinate, often lead to him demanding to go to the bathroom over and over, often after just having urinated. Do not remind him that he just went to the bathroom. Do not ask him to wait. He may have an accident or only be further antagonized.

Constipation is a common problem in the more advanced stages of HD.  Filling up on high-calorie low-fiber foods to keep weight on, the loss of some fiber in altered texture diets, and an increasingly sedentary lifestyle can all add to the problem.

After a thorough assessment, constipation is often treated with increased fluids, more frequent position changes, and a regimen of stool softeners.
SEIZURES

It is not uncommon for people with juvenile-onset HD to have seizures. Occasionally, those in the most advanced stages of adult-onset HD will have seizures, too.

It is more likely, though, that you may see sudden, brief, involuntary jerks involving groups of muscles that are easily mistaken for seizure activity. These large muscle jerks are called "myoclonus" and usually are not treated.

HIGH FEVERS

Late in the progression of the disease, a very small number of people experience recurring high fevers, at times reaching 40° Celsius and higher. As in other times of high fever, the person's level of activity will decline.

These high fevers occur despite physicians' best efforts to identify infectious causes. Consult your physician immediately. As you work together, pay close attention to room temperature and how much fluid he is drinking. It may be medications that interfere with sweating and the regulation of body temperature that are the cause.

CONTRACTURES

A contracture is a permanent shortening of a muscle that causes a deformity with or without pain.  Providing frequent changes in position and range of motion exercises is important to prevent contractures. The participation of a physiotherapist in his care is critically important to prevent serious progressive deformity.

In the advanced stages of HD, his ability to control movement becomes severely compromised. Those who once had involuntary movements may now be rigid and vulnerable to developing contractures. Even though he may still have involuntary movements, he cannot change his position.

The fluctuations in muscle tone and the involuntary movements make it difficult to prevent and manage contractures. Typical approaches such as orthoplastic splints can easily cause skin problems.

More useful, especially for knee and elbow contractures, are newer air-assist splints which use air bladders for support. The "give" in the soft splint prevents skin from breaking down. There are also lightweight, washable foam-core splints that can be helpful in maintaining functional positions of the hands.

Since rigidity is typical in people with juvenile HD,  contractures may be a problem earlier in their disease. People with adult-onset HD may have involuntary movements that progress into rigidity. People who develop HD in their early twenties may present with stiffness, slowness, and occasional involuntary movements.

Over time all involuntary movements are slowed, and dystonia and loss of motor control dominate.

SEVERE CHOREA

Most physicians and physiotherapists familiar with HD tend not to treat chorea. Many people with HD who have taken medication to suppress their chorea feel that it is easier to live with their chorea than with the side effects of the medication used to suppress it.

There are, however, people whose chorea is so severe that it actually causes them bodily harm. In these cases medication is most helpful. In addition, carefully selected padding of the environment is required. It may even become necessary to pad parts of the body if they are being repeatedly injured. Padded mitts as well as knee and elbow pads for athletes can be used.

 

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