Coffee Perk

Huntington’s Disease And Good Nutrition

Huntington’s disease is a rare, hereditary disorder which results in progressive degeneration of the brain. While it can strike at any age at all, the typical age for onset is between 30 and 50. It is estimated that there are around 30,000 cases in the United States and as many as 150,000 other people may be at a 50% risk of developing the disease. (Source: Sarason and Sarason 2005). There are several types of symptoms that are observed in those with Huntington’s disease: dementia, irritability, apathy, depression, and hallucinations or delusions. In addition to these symptoms, there tends to be particular, spasmodic, jerking and twisting movements in the neck, trunk and extremities and facial grimacing. It is important to note that the psychiatric symptoms may start before any of the physical symptoms are noticed. Of these, depression is one of the most common, but obsessions and compulsions are also seen frequently. Impulsiveness is also likely to become apparent as the disorder progresses further. People with Huntington’s disease tend to be deficient in the neurotransmitter gamma-amino-butyric acid. This disease moves at different rates for each person but tends to move very slowly. How severe the symptoms are can be related to the degree of the nerve cell loss. Death occurs anywhere from ten to thirty years after the initial onset of symptoms and it may progress faster in younger people. Vital functions can be adversely affected in the later stages of the disease and because depression is common in Huntington’s disease, suicide is a major risk. The typical cause of death is a complication of the disease itself, including a fall or an infection. In addition, severe dehydration may also be a problem and can lead to electrical imbalances in the heart. Franklin has been having some problems for the past few years and it is becoming evident that he needs to go to the doctor. He has been quick to anger lately and seems to be very depressed. He also appears confused and cannot seem to make any decisions or learn new information. His family has noted that he has several bruises on his arms and legs after several falls in the past few weeks. He is most upset over the swallowing problems that he has been having, which has led to his weight loss lately. Because he does have a family history of this disease, he knows it is time for an appointment, so he goes for a consultation where the doctor performs a physical examination and asks about his medical and family health history. He will also have a full psychiatric examination and will meet with a genetic counselor for blood work to see if he does have the defective gene. Finally, he will also have a CT scan and MRI to rule out any other organic causes for his behavioral, physical and psychiatric changes. Once the diagnosis of Huntington’s disease is confirmed, he will start treatment as soon as possible. There is nothing that can stop the progression of this disease; however, there are some medications that can treat some of the symptoms. Most people with Huntington’s disease will eventually need to have long term care, for instance, in a nursing home. Tetrabenazine (Xenazine) is listed as the first medication that is meant specifically to treat symptoms of Huntington’s disease. This medication works by increasing the level of dopamine in the brain, which may help to reduce some of the jerky movements. There are side effects to this medication which include drowsiness, insomnia, restlessness and nausea, however, it is not recommended for anyone who has signs of depression because it may increase suicidal thoughts. Other medication options include tranquilizers such as clonazepam (Klonapin) and antipsychotic drugs like clozapine and haloperidol can help control some of the movements, the hallucinations and violent outbursts. The side effects with these drugs can include sedation and may make the person stiffer. Franklin will continue taking his medications; however, he will also start speech therapy as well because the disease is starting to affect his ability to speak clearly. In addition to speech therapy, he will go to other therapists as well, including physical therapy to keep his muscles strong and more flexible. In addition, physical therapy will also help to maintain balance and reduce the risk of falls. An occupational therapist will come to his home so that it can be made as safe as possible and to give him tips on how to deal with his concentration problems. As he gets worse, he will also receive help with learning how to deal with problems with dressing, eating and hygiene skills. There are special tools and adaptive devices that he may need to use, including built-up cutlery so that he can manage them on his own. He will also discuss his declining abilities in counseling, as he will, eventually need more help doing even these tasks. He is currently getting help from home health workers who help him do things around his home, as well as exercising, which the doctors recommend as being beneficial. He will also be trying to eat a more nutritious diet as well. People with Huntington’s disease burn extreme amounts of calories each day (as many as 5000 per day), so it is important to try to eat enough food to maintain body weight. Extra vitamins and supplements are also important. Supplements may also help to maintain some independence as well because eating is one of the areas that may be affected by the disease. Dairy products may cause you to have too much mucus, which can increase the risk of choking because of the swallowing difficulties. As the disease progresses, it may be necessary to have a swallowing evaluation and make dietary changes that will protect against these risks. One of the changes that may be made is the need for thickened liquids. There are different levels of thickened liquids, including nectar thick and thicker. Some thickened liquids come premade, while others may have to be thickened at home with a specially made thickener. There is no limit to the types of liquids that can be thickened; even Franklin’s morning coffee can be thickened to the right consistency. In addition to dropping milk from his diet, Franklin will be using a protein supplement called Profect to help make sure that he gets the right amount of protein in his diet every day. It helps to keep his strength up and because it is a small, easy-to-use supplement, he can get it for himself, so he is able to maintain some independence and sense of dignity. Profect is available in a number of different flavors and in different sizes; he simply has his home health worker set up the next day’s supplements the night before so that he grab them whenever he is ready to use them. In addition to giving him 25 grams of protein per serving, he will also get vitamin C and B complex vitamins.

About Protica Research

Founded in 2001, Protica, Inc. is a nutritional research firm specializing in the development of protein-rich, capsulized foods (dense nutrition in compact liquid and food forms). Protica manufactures Profect protein beverage, IsoMetric, Fruitasia and more than 100 other brands in its GMP-certified, 250,000 square foot facility. You can learn more about Protica at http://www.protica.com Copyright – Protica Research – http://www.protica.com

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