Category Archives: Symptoms

Autism Awareness Month

Autism spectrum disorders (ASDs) are a group of developmental disabilities that can cause significant social, communication and behavioral challenges.

ASDs are “spectrum disorders” which means ASDs affect each person in different ways, and can range from very mild to severe. People with ASDs share some similar symptoms, such as problems with social interaction. But there are differences in when the symptoms start, how severe they are, and the exact nature of the symptoms.


Types of ASDs
There are three different types of ASDs:

  • Autistic Disorder (also called “classic” autism)
    This is what most people think of when hearing the word “autism.” People with autistic disorder usually have significant language delays, social and communication challenges, and unusual behaviors and interests. Many people with autistic disorder also have intellectual disability.
  • Asperger Syndrome
    People with Asperger syndrome usually have some milder symptoms of autistic disorder. They might have social challenges and unusual behaviors and interests. However, they typically do not have problems with language or intellectual disability.
  • Pervasive Developmental Disorder – Not Otherwise Specified (PDD-NOS; also called “atypical autism”)
    People who meet some of the criteria for autistic disorder or Asperger syndrome, but not all, may be diagnosed with PDD-NOS. People with PDD-NOS usually have fewer and milder symptoms than those with autistic disorder. The symptoms might cause only social and communication challenges.


Signs and Symptoms
ASDs begin before the age of 3 and last throughout a person’s life, although symptoms may improve over time. Some children with an ASD show hints of future problems within the first few months of life. In others, symptoms might not show up until 24 months or later. Some children with an ASD seem to develop normally until around 18 to 24 months of age and then they stop gaining new skills, or they lose the skills they once had.

A person with an ASD might:

  • Not respond to their name by 12 months
  • Not point at objects to show interest (point at an airplane flying over) by 14 months
  • Not play “pretend” games (pretend to “feed” a doll) by 18 months
  • Avoid eye contact and want to be alone
  • Have trouble understanding other people’s feelings or talking about their own feelings
  • Have delayed speech and language skills
  • Repeat words or phrases over and over (echolalia)
  • Give unrelated answers to questions
  • Get upset by minor changes
  • Have obsessive interests
  • Flap their hands, rock their body, or spin in circles
  • Have unusual reactions to the way things sound, smell, taste, look, or feel


Diagnosis
Diagnosing ASDs can be difficult since there is no medical test, like a blood test, to diagnose the disorders. Doctors look at the child’s behavior and development to make a diagnosis.

ASDs can sometimes be detected at 18 months or younger. By age 2, a diagnosis by an experienced professional can be considered very reliable. However, many children do not receive a final diagnosis until much older. This delay means that children with an ASD might not get the help they need.


Treatment
There is currently no cure for ASDs. However, research shows that early intervention treatment services can greatly improve a child’s development. Early intervention services help children from birth to 3 years old (36 months) learn important skills. Services can include therapy to help the child talk, walk, and interact with others. Therefore, it is important to talk to your child’s doctor as soon as possible if you think your child has an ASD or other developmental problem.

Even if your child has not been diagnosed with an ASD, he or she may be eligible for early intervention treatment services. The Individuals with Disabilities Education Act (IDEA) says that children under the age of 3 years (36 months) who are at risk of having developmental delays may be eligible for services. These services are provided through an early intervention system in your state. Through this system, you can ask for an evaluation.

In addition, treatment for particular symptoms, such as speech therapy for language delays, often does not need to wait for a formal ASD diagnosis.

Learn about types of treatments »


Causes and Risk Factors
We do not know all of the causes of ASDs. However, we have learned that there are likely many causes for multiple types of ASDs. There may be many different factors that make a child more likely to have an ASD, including environmental, biologic and genetic factors.

  • Most scientists agree that genes are one of the risk factors that can make a person more likely to develop an ASD.
  • Children who have a sibling or parent with an ASD are at a higher risk of also having an ASD.
  • ASDs tend to occur more often in people who have certain other medical conditions. About 10% of children with an ASD have an identifiable genetic disorder, such as Fragile X syndrome, tuberous sclerosis, Down syndrome and other chromosomal disorders.
  • Some harmful drugs taken during pregnancy have been linked with a higher risk of ASDs, for example, the prescription drugs thalidomide and valproic acid.
  • We know that the once common belief that poor parenting practices cause ASDs is not true.
  • There is some evidence that the critical period for developing ASDs occurs before birth. However, concerns about vaccines and infections have led researchers to consider risk factors before and after birth.

ASDs are an urgent public health concern. Just like the many families affected in some way by ASDs, CDC wants to find out what causes the disorder. Understanding the risk factors that make a person more likely to develop an ASD will help us learn more about the causes. We are currently working on one of the largest U.S. studies to date, called Study to Explore Early Development (SEED). SEED is looking at many possible risk factors for ASDs, including genetic, environmental, pregnancy, and behavioral factors.


Who is Affected
ASDs occur in all racial, ethnic, and socioeconomic groups, but are almost five times more common among boys than among girls. CDC estimates that about 1 in 88 children has been identified with an autism spectrum disorder (ASD).

More people than ever before are being diagnosed with an ASD. It is unclear exactly how much of this increase is due to a broader definition of ASDs and better efforts in diagnosis. However, a true increase in the number of people with an ASD cannot be ruled out. We believe the increase in ASD diagnosis is likely due to a combination of these factors.

Within the past decade, CDC’s Autism and Developmental Disabilities Monitoring (ADDM) Network has been estimating the number of people with an ASD in the U.S. We have learned a lot about how many children in the U.S. have an ASD. It will be important to use the same methods to track how the number of people with an ASD is changing over time in order to learn more about the disorders.


If You’re Concerned
If you think your child might have an ASD or you think there could be a problem with the way your child plays, learns, speaks, or acts,contact your child’s doctor, and share your concerns.

If you or the doctor is still concerned, ask the doctor for a referral to a specialist who can do a more in-depth evaluation of your child. Specialists who can do a more in-depth evaluation and make a diagnosis include:

  • Developmental Pediatricians (doctors who have special training in child development and children with special needs)
  • Child Neurologists (doctors who work on the brain, spine, and nerves)
  • Child Psychologists or Psychiatrists (doctors who know about the human mind)

At the same time, call your state’s public early childhood system to request a free evaluation to find out if your child qualifies for intervention services. This is sometimes called a Child Find evaluation. You do not need to wait for a doctor’s referral or a medical diagnosis to make this call.

Where to call for a free evaluation from the state depends on your child’s age:

  • If your child is not yet 3 years old, contact your local early intervention system.You can find the right contact information for your state by calling the National Dissemination Center for Children with Disabilities (NICHCY) at 1-800-695-0285.Or visit the NICHCY website. Once you find your state on this webpage, look for the heading “Programs for Infants and Toddlers with Disabilities: Ages Birth through 3″.
  • If your child is 3 years old or older, contact your local public school system.Even if your child is not yet old enough for kindergarten or enrolled in a public school, call your local elementary school or board of education and ask to speak with someone who can help you have your child evaluated.If you’re not sure who to contact, call the National Dissemination Center for Children with Disabilities at 1.800.695.0285 or visit the NICHCY website. Once you find your state on this webpage, look for the heading “Programs for Children with Disabilities: Ages 3 through 5″.

Research shows that early intervention services can greatly improve a child’s development. In order to make sure your child reaches his or her full potential, it is very important to get help for an ASD as soon as possible.

January Is Glaucoma Awareness Month

Glaucoma is a very misunderstood disease. Often, people don’t realize the severity or who is affected.

Key Facts About Glaucoma

  • Glaucoma is a leading cause of blindness
    Glaucoma can cause blindness if it is left untreated. And unfortunately approximately 10% of people with glaucoma who receive proper treatment still experience loss of vision.
  • There is no cure (yet) for glaucoma
    Glaucoma is not curable, and vision lost cannot be regained. With medication and/or surgery, it is possible to halt further loss of vision. Since open-angle glaucoma is a chronic condition, it must be monitored for life. Diagnosis is the first step to preserving your vision.
  • Everyone is at risk for glaucoma
    Everyone is at risk for glaucoma from babies to senior citizens. Older people are at a higher risk for glaucoma but babies can be born with glaucoma (approximately 1 out of every 10,000 babies born in the United States). Young adults can get glaucoma, too. African Americans in particular are susceptible at a younger age.
  • There may be no symptoms to warn you
    With open-angle glaucoma, the most common form, there are virtually no symptoms. Usually, no pain is associated with increased eye pressure. Vision loss begins with peripheral or side vision. You may compensate for this unconsciously by turning your head to the side, and may not notice anything until significant vision is lost. The best way to protect your sight from glaucoma is to get tested. If you have glaucoma, treatment can begin immediately.

Some Statistics About Glaucoma

  • It is estimated that over 2.2 million Americans have glaucoma but only half of those know they have it.
  • In the U.S., more than 120,000 are blind from glaucoma, accounting for 9% to 12% of all cases of blindness.
  • Glaucoma is the second leading cause of blindness in the world, according to the World Health Organization.
  • After cataracts, glaucoma is the leading cause of blindness among African Americans.
  • Blindness from glaucoma is 6 to 8 times more common in African Americans than Caucasians.
  • African Americans are 15 times more likely to be visually impaired from glaucoma than Caucasians.
  • The most common form, open-angle glaucoma, accounts for 19% of all blindness among African Americans compared to 6% in Caucasians.
  • Other high-risk groups include: people over 60, family members of those already diagnosed, diabetics, and people who are severely nearsighted.
  • Estimates put the total number of suspected cases of glaucoma at over 60 million worldwide.

Lewy Body Dementia

What Is LBD?
LBD is not a rare disease. It affects an estimated 1.3 million individuals and their families in the United States. Because LBD symptoms can closely resemble other more commonly known diseases like Alzheimer’s and Parkinson’s, it is currently widely underdiagnosed. Many doctors or other medical professionals still are not familiar with LBD.LBD is an umbrella term for two related diagnoses. LBD refers to both Parkinson’s disease dementia and dementia with Lewy bodies. The earliest symptoms of these two diseases differ, but reflect the same underlying biological changes in the brain. Over time, people with both diagnoses will develop very similar cognitive, physical, sleep, and behavioral symptoms.While it may take more than a year or two for enough symptoms to develop for a doctor to diagnose LBD, it is critical to pursue a formal diagnosis. Early diagnosis allows for important early treatment that may extend quality of life and independence.LBD is a multisystem disease and typically requires a comprehensive treatment approach. This approach involves a team of physicians from different specialties who collaborate to provide optimum treatment of each symptom without worsening other LBD symptoms. Many people with LBD enjoy significant improvement of their symptoms with a comprehensive approach to treatment, and some can have remarkably little change from year to year.Some people with LBD are extremely sensitive or may react negatively to certain medications used to treat Alzheimer’s or Parkinson’s in addition to certain over-the-counter medications.

Who was Lewy?
In the early 1900s, while researching Parkinson’s disease, the scientist Friederich H. Lewy discovered abnormal protein deposits that disrupt the brain’s normal functioning. These Lewy body proteins are found in an area of the brain stem where they deplete the neurotransmitter dopamine, causing Parkinsonian symptoms. In Lewy body dementia, these abnormal proteins are diffuse throughout other areas of the brain, including the cerebral cortex. The brain chemical acetylcholine is depleted, causing disruption of perception, thinking and behavior. Lewy body dementia exists either in pure form, or in conjunction with other brain changes, including those typically seen in Alzheimer’s disease and Parkinson’s disease.

Early and accurate diagnosis of LBD, while not always easy to do, is of critical importance for two reasons.

  • First, people with LBD may respond more favorably to certain dementia medications than people with Alzheimer’s, allowing for early treatment that may improve or extend the quality of life for both the person with LBD and their caregiver.
  • Secondly, many people with LBD respond more poorly to certain medications for behavior and movement than people with Alzheimer’s or Parkinson’s, sometimes with dangerous or permanent side effects.

By learning about common forms of dementia, you can help your physician most quickly identify what type of dementia has developed.

Common Forms of Dementia

Alzheimer’s disease symptoms include a progressive loss of recent memory; problems with language, calculation, abstract thinking, and judgment; depression or anxiety; personality and behavioral changes; and disorientation to time and place.

Lewy body dementia (LBD) is an umbrella term for a form of dementia that has three common presentations.

  • Some individuals will start out with a memory or cognitive disorder that may resemble Alzheimer’s disease, but over time two or more distinctive features become apparent leading to the diagnosis of ‘dementia with Lewy bodies’ (DLB). Symptoms that differentiate it from Alzheimer’s include unpredictable levels of cognitive ability, attention or alertness, changes in walking or movement, visual hallucinations, a sleep disorder called REM sleep behavior disorder, in which people physically act out their dreams, and severe sensitivity to medications for hallucinations. In some cases, the sleep disorder can precede the dementia and other symptoms of LBD by decades.
  • Others will start out with a movement disorder leading to the diagnosis of Parkinson’s disease and later develop dementia and other symptoms common in DLB.
  • Lastly, a small group will first present with neuropsychiatric symptoms, which can include hallucinations, behavioral problems, and difficulty with complex mental activities, leading to an initial diagnosis of DLB.

Regardless of the initial symptom, over time all three presentations of LBD will develop very similar cognitive, physical, sleep and behavioral features, all caused by the presence of Lewy bodies throughout the brain.

Vascular dementia is caused by a series of small strokes that deprive the brain of vital oxygen. Symptoms, such as disorientation in familiar locations; walking with rapid, shuffling steps; incontinence; laughing or crying inappropriately; difficulty following instructions; and problems handling money may appear suddenly and worsen with additional strokes. High blood pressure, cigarette smoking, and high cholesterol are some of the risk factors for stroke that may be controlled to prevent vascular dementia.

Frontotemporal dementia (FTD) includes several disorders with a variety of symptoms. The most common signs of FTD include changes in personality and behavior, such as inappropriate or compulsive behavior, euphoria, apathy, decline in personal hygiene, and a lack of awareness concerning these changes. Some forms of FTD involve language and speech symptoms or movement changes.

An experienced clinician within the medical community should perform a diagnostic evaluation. If one is not available, the neurology department of the nearest medical university should be able to recommend appropriate resources or may even provide an experienced diagnostic team skilled in Lewy body dementia.

A thorough dementia diagnostic evaluation includes physical and neurological examinations, patient and family interviews (including a detailed lifestyle and medical history), and neuro-psychological and mental status tests. The patient’s functional ability, attention, language, visuospatial skills, memory and executive functioning are assessed. In addition, brain imaging (CT or MRI scans), blood tests and other laboratory studies may be performed. The evaluation will provide a clinical diagnosis. Currently, a conclusive diagnosis of LBD can be obtained only from a postmortem autopsy for which arrangements should be made in advance. Some research studies may offer brain autopsies as part of their protocols. Participating in research studies is a good way to benefit others with Lewy body dementia.

Medications
Medications are one of the most controversial subjects in dealing with LBD. A medication that doesn’t work for one person may work for another person.

Prescribing should only be done by a physician who is thoroughly knowledgeable about LBD. With new medications and even ‘over-the-counter,’ the patient should be closely monitored. At the first sign of an adverse reaction, consult with the patient’s physician. Consider joining an online caregiver support group to see what others have observed with prescription and over-the-counter medicines.

Risk Factors
Advanced age is considered to be the greatest risk factor for Lewy body dementia, with onset typically, but not always, between the ages of 50 and 85. Some cases have been reported much earlier. It appears to affect slightly more men than women. Having a family member with Lewy body dementia may increase a person’s risk. Observational studies suggest that adopting a healthy lifestyle (exercise, mental stimulation, nutrition) might delay age-associated dementias.

Clinical Trials
The recruitment of LBD patients for participation in clinical trials for studies on LBD, other dementias and Parkinsonian studies is now steadily increasing.

Prognosis and Stages
No cure or definitive treatment for Lewy body dementia has been discovered as yet. The disease has an average duration of 5 to 7 years. It is possible, though, for the time span to be anywhere from 2 to 20 years, depending on several factors, including the person’s overall health, age and severity of symptoms.

Defining the stages of disease progression for LBD is difficult. The symptoms, medicine management and duration of LBD vary greatly from person to person. To further complicate the stages assessment, LBD has a progressive but vacillating clinical course, and one of its defining symptoms is fluctuating levels of cognitive abilities, alertness and attention. Sudden decline is often caused by medications, infections or other compromises to the immune system and usually the person with LBD returns to their baseline upon resolution of the problem.  But for some individuals, it may also be due to the natural course of the disease.

Symptoms

Lewy body dementia symptoms and diagnostic criteria
Every person with LBD is different and will manifest different degrees of the following symptoms. Some will show no signs of certain features, especially in the early stages of the disease. Symptoms may fluctuate as often as moment-to-moment, hour-to-hour or day-to-day. NOTE: Some patients meet the criteria for LBD yet score in the normal range of some cognitive assessment tools. The Mini-Mental State Examination (MMSE), for example, cannot be relied upon to distinguish LBD from other common syndromes.

LBD is a an umbrella term for two related clinical diagnoses, dementia with Lewy bodies and Parkinson’s disease dementia.

The latest clinical diagnostic criteria for dementia with Lewy bodies (DLB) categorizes symptoms into three types, listed below.  A diagnosis of Parkinsons’ disease dementia (PDD) requires a well established diagnosis of Parkinson’s disease that later progresses into dementia, along with very similar features to DLB.  A rather arbirary time cutoff was established to differentiate between DLB and PDD.  People whose dementia occurs before or within 1 year of Parkinson’s symptoms are diagnosed with DLB.  People who have an existing diagnosis of Parkinson’s for more than a year and later develop dementia are diagnosed with PDD.

Central feature

  • Progressive dementia – deficits in attention and executive function are typical. Prominent memory impairment may not be evident in the early stages.

Core features

  • Fluctuating cognition with pronounced variations in attention and alertness.
  • Recurrent complex visual hallucinations, typically well formed and detailed.
  • Spontaneous features of parkinsonism.

Suggestive features

  • REM sleep behavior disorder (RBD), which can appear years before the onset of dementia and parkinsonism.
  • Severe sensitivity to neuroleptics occurs in up to 50% of LBD patients who take them.
  • Low dopamine transporter uptake in the brain’s basal ganglia as seen on SPECT and PET imaging scans. (These scans are not yet available outside of research settings.)

Supportive features

  • Repeated falls and syncope (fainting).
  • Transient, unexplained loss of consciousness.
  • Autonomic dysfunction.
  • Hallucinations of other senses, like touch or hearing.
  • Visuospatial abnormalities.
  • Other psychiatric disturbances.

A clinical diagnosis of LBD can be probable or possible based on different symptom combinations.

A probable LBD diagnosis requires either:

  • Dementia plus two or more core features, or
  • Dementia plus one core feature and one or more suggestive features.

A possible LBD diagnosis requires:

  • Dementia plus one core feature, or
  • Dementia plus one or more suggestive features.

Symptoms Explained
In this section we’ll discuss each of the symptoms, starting with the key word: dementia. Dementia is a process whereby the person becomes progressively confused. The earliest signs are usually memory problems, changes in their way of speaking, such as forgetting words, and personality problems. Cognitive symptoms of dementia include poor problem solving, difficulty with learning new skills and impaired decision making.

Other causes of dementia should be ruled out first, such as alcoholism, overuse of medication, thyroid or metabolic problems. Strokes can also cause dementia. If these reasons are ruled out then the person is said to have a degenerative dementia. Lewy Body Dementia is second only to Alzheimer’s disease as the most common form of dementia.

Fluctuations in cognition will be noticeable to those who are close to the person with LBD, such as their partner. At times the person will be alert and then suddenly have acute episodes of confusion. These may last hours or days. Because of these fluctuations, it is not uncommon for it to be thought that the person is “faking”. This fluctuation is not related to the well-known “sundowning” of Alzheimer’s. In other words, there is no specific time of day when confusion can be seen to occur.

Hallucinations are usually, but not always, visual and often are more pronounced when the person is most confused. They are not necessarily frightening to the person. Other modalities of hallucinations include sound, taste, smell, and touch.

Parkinsonism or Parkinson’s Disease symptoms, take the form of changes in gait; the person may shuffle or walk stiffly. There may also be frequent falls. Body stiffness in the arms or legs, or tremors may also occur. Parkinson’s mask (blank stare, emotionless look on face), stooped posture, drooling and runny nose may be present.

REM Sleep Behavior Disorder (RBD) is often noted in persons with Lewy Body Dementia. During periods of REM sleep, the person will move, gesture and/or speak. There may be more pronounced confusion between the dream and waking reality when the person awakens. RBD may actually be the earliest symptom of LBD in some patients, and is now considered a significant risk factor for developing LBD. (One recent study found that nearly two-thirds of patients diagnosed with RBD developed degenerative brain diseases, including Lewy body dementia, Parkinson’s disease, and multiple system atrophy, after an average of 11 years of receiving an RBD diagnosis. All three diseases are called synucleinopathies, due to the presence of a mis-folded protein in the brain called alpha-synuclein.)

Sensitivity to neuroleptic (anti-psychotic) drugs is another significant symptom that may occur. These medications can worsen the Parkinsonism and/or decrease the cognition and/or increase the hallucinations. Neuroleptic Malignancy Syndrome, a life-threatening illness, has been reported in persons with Lewy Body Dementia. For this reason, it is very important that the proper diagnosis is made and that healthcare providers are educated about the disease.

Other Symptoms
Visuospatial difficulties, including depth perception, object orientation, directional sense and illusions may occur.

Autonomic dysfunction, including blood pressure fluctuations (e.g. postural/orthostatic hypotension) heart rate variability (HRV), sexual disturbances/impotence, constipation, urinary problems, hyperhidrosis (excessive sweating), decreased sweating/heat intolerance, syncope (fainting), dry eyes/mouth, and difficulty swallowing which may lead to aspiration pneumonia.

Other psychiatric disturbances may include systematized delusions, aggression and depression. The onset of aggression in LBD may have a variety of causes, including infections (e.g., UTI), medications, misinterpretation of the environment or personal interactions, and the natural progression of the disease.

Treatment Options

LBD is a multi-system disease and typically requires a comprehensive treatment approach, meaning a team of physicians from different specialties, who collaborate to provide optimum treatment of each symptom without worsening other LBD symptoms.  It is important to remember that some people with LBD are extremely sensitive or may react negatively to certain medications used to treat Alzheimer’s or Parkinson’s in addition to certain over-the-counter medications.

Cognitive Symptoms
Medications called cholinesterase inhibitors are considered the standard treatment for cognitive symptoms in LBD. These medications were developed to treat Alzheimer’s disease. However, some researchers believe that people with LBD may be even more responsive to these types of medications than those with Alzheimer’s.

Movement Symptoms
Movement symptoms may be treated with a Parkinson’s medication called levodopa, but if the symptoms are mild, it may be best to not treat them in order to avoid potential medication side-effects.

Visual Hallucinations
If hallucinations are disruptive or upsetting, your physician may recommend a cautious trial of a newer antipsychotic medication. (Please see WARNING below.)  Of note, the dementia medications called cholinesterase inhibitors have also been shown to be effective in treating hallucinations and other psychiatric symptoms of LBD.

REM Sleep Behavior Disorder (RBD)
RBD can be quite responsive to treatment, so your physician may recommend a medication like melatonin and/or clonazepam.

Neuroleptic Sensitivity
Severe sensitivity to neuroleptics is common in LBD. Neuroleptics, also known as antipsychotics, are medications used to treat hallucinations or other serious mental disorders. While traditional antipsychotic medications (e.g. haloperidol) are commonly prescribed for individuals with Alzheimer’s with disruptive behavior, these medications can affect the brain of an individual with LBD differently, sometimes causing severe side effects (see below). For this reason, traditional antipsychotic medications like haloperidol should be avoided. Some newer ‘atypical’ antipsychotic medications like risperidone may also be problematic for someone with LBD. Quetiapine is preferred by some LBD experts. If quetiapine is not tolerated or is not helpful, clozapine should be considered, but requires ongoing blood tests to assure a rare but serious blood condition does not develop. Hallucinations must be treated very conservatively, using the lowest doses possible under careful observation for side effects.

WARNING:
Up to 50% of patients with LBD who are treated with any antipsychotic medication may experience severe neuroleptic sensitivity, such as worsening cognition, heavy sedation, increased or possibly irreversible parkinsonism, or symptoms resembling neuroleptic malignant syndrome (NMS), which can be fatal. (NMS causes severe fever, muscle rigidity and breakdown that can lead to kidney failure.)

Medication Side Effects
Speak with your doctor about possible side effects. The following drugs may cause sedation, motor impairment or confusion:

  • Benzodiazepines, tranquilizers like diazepam and lorazepam
  • Anticholinergics (antispasmodics), such as oxybutynin and glycopyrrolate
  • Some surgical anesthetics
  • Older antidepressants
  • Certain over-the-counter medications, including diphenhydramine and dimenhydrinate.
  • Some medications, like anticholinergics, amantadine and dopamine agonists, which help relieve parkinsonian symptoms, might increase confusion, delusions or hallucinations.

NOTE: Be sure to meet with your anesthesiologist in advance of any surgery to discuss medication sensitivities and risks unique to LBD. People with LBD often respond to certain anesthetics and surgery with acute states of confusion or delirium and may have a sudden significant drop in functional abilities, which may or may not be permanent.

Possible alternatives to general anesthesia include a spinal or regional block. These methods are less likely to result in postoperative confusion. If you are told to stop taking all medications prior to surgery, consult with your doctor to develop a plan for careful withdrawal.

Non-Medical Treatments

Physical therapy options include cardiovascular, strengthening, and flexibility exercises, as well as gait training. Physicians may also recommend general physical fitness programs such as aerobic, strengthening, or water exercise.

Speech therapy may be helpful for low voice volume and poor enunciation. Speech therapy may also improve muscular strength and swallowing difficulties.

Occupational therapy may help maintain skills and promote function and independence. In addition to these forms of therapy and treatment, music and aroma therapy can also reduce anxiety and improve mood.

Individual and family psychotherapy can be useful for learning strategies to manage emotional and behavioral symptoms and to help make plans that address individual and family concerns about the future.

Support groups may be helpful for caregivers and persons with LBD to identify practical solutions to day-to-day frustrations, and to obtain emotional support from others.

End-of-Life
Planning for the end of life can be a valuable activity for any family. The links below offer general guidance and some specific suggestions for families who face the burden of a disease such as Lewy body dementia.

Advanced Directives – a Caring Connections site with state-specific advanced directives

Caring Connections – home page of consumer Web site about hospice and palliative care managed by the National Hospice and Palliative Care Organization

Palliative Doctors – a Web site for consumers managed by the American Academy of Hospice and Palliative Care about palliative care

Accessible Vehicles And Adaptive Mobility Equipment Q&A

Rear entry vs. side entry. Buying online. Buying used. What do you need to know to get maximum benefit for minimum expense?

Good information is the key to saving money and getting the most value for the dollar when making a big-ticket purchase like a wheelchair-accessible vehicle.

With that in mind, Seek out and find experts who truly care for answers to some common questions about adaptive mobility equipment.

Q: Can I just go to a car dealer down the street or do I need a certified mobility dealer?

A: Certified mobility dealers help consumers buy the right vehicle and adaptive mobility equipment to meet their mobility needs now and in the future. Future planning is especially important for people with muscle diseases that get progressively worse over time.

“There are so many different products out there, and technology has improved so much. We just want to help people make the right decision,” says Jim Sanders, president of Automotive Innovations based in Bridgewater, MA for over 25 years.

“Many times, consumers will go to a car dealer and buy [a vehicle] that can’t be modified or one that doesn’t fit their needs. And once you buy a vehicle, normally it’s very difficult to return it.”

The National Mobility Equipment Dealers Association (NMEDA), a nonprofit organization that provides consumer guidance and ensures quality and professionalism in the manufacturing and installation of mobility equipment. Members include mobility equipment dealers, manufacturers, driver rehabilitation specialists and other professionals.

NMEDA member-dealers must follow the safety standards established by the National Highway Traffic Safety Administration (NHTSA), in addition to NMEDA’s own stringent guidelines.

Some dealers choose to enroll in NMEDA’s Quality Assurance Program (QAP), which requires them to adhere to national motor vehicle safety standards, and use proven quality control practices to yield the highest level of performance and safety. Automotive Innovations was the First Mobility Dealer in Massachusetts to enroll and exceed the safety standards.

“The QAP dealer is audited by an outside engineering firm to verify that technicians have been trained, make sure the dealer has insurance and make sure the facility is ADA-compliant,”

So it means the QAP dealer is going above and beyond.”

Other reasons to seek out a certified mobility equipment dealer include:

They provide a link to qualified service and repair, that it’s crucial to have done on a adapted vehicle serviced.

Some manufacturers of adapted vehicles sell directly to consumers, cutting costs by cutting out the middle man, says Jim Sanders, of VMi New England, based in Bridgewater, MA.

But expert assessment and “try before you buy” remain essentials for prospective buyers, with or without a dealer in the middle.

For example, We, a NMEDA QAP-certified member, send representatives to customers’ homes for assessment and test drives before they buy, and also offer unmatched service/maintenance to just about any modified vehicle including Rollx vans.

Q: Can I get a better price if I buy online rather than from a dealer?

A: As with any online shopping, the warning “buyer beware” rings true. Buying online without trying out different vehicles with different conversions can be a costly mistake. Furthermore there are many grey market converted vans being offered as quality conversions.

Online, clients are mostly shopping blind. Typically they have no idea how the vehicle they need will even work fro them, even if they have specific recommendations from a driver evaluator or occupational therapist.

“You definitely shouldn’t buy it online,” “There not trying to assess your needs by e-mail or over the phone. There just trying to sell you something.

Some online dealers even have a questionnaire on its Web site to try and give you the idea your getting what you need. But, it will never replace being able to go to a local mobility dealership and try the vans out first hand.

A mobility vehicle is probably the second-largest purchase after a house. You should see it, try it out, and make sure it’s something that will work for you. It’s horrible when people get something that they’re disappointed in.

Every vehicle is a little bit different — such as in the dimensions, electrical and fuel systems, or suspension modifications. “If you go online and buy [based] on price, you’re not really looking at the total package.”

While buying online maybe able to save money up front, it wont over the long term.

In addition to consumers missing out on the important local service contact that a mobility equipment dealer provides, these online deals or grey market vans are worth much less when it comes time to trade it in.

Where do you want to sit? If you plan to drive from your wheelchair, then a side-entry conversion is what you’ll need, unless you can transfer to the driver’s seat (rear entry). With a rear-entry conversion, the wheelchair user typically is positioned in the back or between two mid-row captain’s seats, while a side entry offers a wheelchair user multiple seating options in the driver, front passenger and middle sections.

Q: What are some common mistakes people make when buying a modified vehicle?

A: Manufacturers and mobility dealers agree that one of the most common — and costly — mistakes is buying the vehicle first and then shopping for the conversion or adaptive mobility equipment. Not all vehicles can be converted.

For example, If you purchase a minivan from a traditional car dealership you can hit a roadblock if it doesn’t meet specific requirements to have the floor lowered for a rear- or side-entry conversion.

Q: What are some good questions to ask a dealer or manufacturer?

A: Although buying a modified vehicle can be “a daunting experience,” says VMI’s Monique McGivney, it also can be “exciting and fun when you walk in armed with good questions and information.”

Prior to getting an assessment from a mobility dealer, evaluate your needs and try answering the following questions:

  • What vehicle will fit in my garage?
  • What kind of parking issues will I encounter where I live?
  • What is the size and weight of my wheelchair?
  • What is my seated height in the wheelchair?
  • How many people will ride in the vehicle?
  • In what part of the vehicle do I want to sit?
  • Will I be able to drive with hand controls?
  • Do I want a full-size van, minivan or alternative vehicle?
  • Do I want manual or power equipment?
  • Will an in-floor ramp or fold-out ramp meet my needs?
  • What is my budget, and do I have access to supplemental funding?

The first question mobility dealers usually ask a client is: “What is your seated height in the wheelchair?” From there, the dealer can advise whether a full-size or minivan is appropriate, and what kind of conversion is needed.

Be sure to ask the dealer about the warranty and how the vehicle can be serviced.

Q: Which is better: rear entry or side entry?

A: The most important difference between a rear- and side-entry conversion is that with a rear entry, wheelchair users can’t drive from their wheelchairs nor can they ride in the front passenger seat. From there, the choice comes down to personal preference and budget.

In recent years, because of quality, convenience and cost, there’s been a shift toward side entry vehicles. Rear entry is more of a frugal modification, involves a less of conversion process and is typically a little less expensive than a side-entry conversion.

Many people prefer side entry with a in-floor conversion for many safety reasons additionally because they can park almost anywhere and not worry deploying the ramp out into traffic. Also, side entry allows the consumer to ride in the passengers front position along with maintain the rear seats in a minivan because the conversion doesn’t affect that area.

Rear entry is harder to get out of compared to a side-entry.

Anyway you look at it side-entry vehicles are more versatile. For example, side entry allows someone with a progressively worsening condition to use the vehicle for a longer period of time. A wheelchair user can start out driving from his or her chair, and then move to several other positions in the vehicle when no longer able to drive.

Side-entry conversions typically are a little more expensive than rear-entry because they’re more intrusive and labor intensive. For example, with a minivan, the entire floor and frame must be removed and replaced with a lowered floor and new frame.

Q: What’s the difference between a fold-out ramp and in-floor ramp?

A: This decision comes down to safety, aesthetics, convenience and cost.

A fold-out ramp folds up into the vehicle, takes up valuable space in the passengers front area and must be deployed whenever the door is opened.

The in-floor ramp slides under the floor, so it safer for anyone seated in the passengers front position, mid-ship position, there’s no obstruction to the door, and other passengers can enter and exit without deploying the ramp. In-floor ramps only are currently only available for side-entry minivan conversions, and there is even a manual (unpowered) option.

In-floor ramps in addition to being safer will generally provide more room in the vehicle because there’s nothing blocking the doorway. The ramp is “out of sight, out of mind and may last longer because it doesn’t have to be deployed each time the side passenger door opens.

Fold-out ramps generally cost a little less than in-floor, and consumers can select from manual and power versions; a power fold-out ramp still costs less than an in-floor ramp.

If an in-floor ramp system breaks down or the vehicle loses power, VMI’s in-floor ramp systems have a backup system (sure-deploy) that bypasses the vehicle’s battery.

A lot of people just feel more secure knowing there isn’t a fold-out ramp next to them in the event of a accident.

Q: I use a wheelchair, but a van or minivan just isn’t “me.” Are they my only options?

A: You have some choices.

Lowered-floor conversions with fold-out ramps can be done on the Honda Element, Chrysler PT Cruiser and Toyota Scion. The conversions are small and don’t fit as many people.

Due to them being built on a much smaller scale, the ones we have seen have not been built with the same level of quality of mini van conversion. Parts availability and repairs have been a problem, some of the companies that converted them are out of business and or have no support for “something they used to build”

For those who prefer to keep their standard car rather than purchasing a modified vehicle — and who can make the transfer from a wheelchair to a car seat — the answer may be as simple as a set of hand controls or a left foot gas pedal

Turning seats can be used in a wide range of vehicles, from sedans to SUVs and pickup trucks. A way to transport the wheelchair (like a rear lift) also is needed.

The rate at which your disease symptoms are worsening is one thing to consider when looking at turning seats — is it likely you’ll be able to transfer and ride in a car seat for many more years? Also, be sure to check with a mobility dealer to determine if your vehicle can accommodate a turning seat and a wheelchair lift.

Q: Why are modified vehicles so darned expensive?

A: A vehicle conversion can cost consumers upwards of $27,000 — and that’s just the cost for the conversion, not the vehicle. The total package can run between $45,000 and $80,000 — or more.

Besides the cost of the components, the reason it’s so pricey is that basically there is a lot of work involved to build a quality vehicle.

Modified vehicles from certified manufacturers and dealers must meet NHTSA’s Federal Motor Vehicle Safety Standards (FMVSS). That means all modified vehicles must be properly crash tested. (To learn more, visit www.nhtsa.dot.gov.)

It’s quite a labor-intensive process because of the customization. When you make structural modifications to a vehicle, you have to go through all of the crash testing, and you have to show that the vehicle is compliant again, and those tests are very expensive.

Most of the time lowering the floor in a minivan requires replacing or moving the fuel tank. Once the conversion is finished, the vehicle still has to meet the original requirements for evaporative emissions, in addition to NHTSA requirements.

Q: How can I pay less?

A: Consumers have some options.

Many consumers cut costs by purchasing pre-owned vehicles with new conversions, typically saving around $10,000 to $12,000.

The previous van owner already has absorbed the depreciation hit on a new van, which essentially occurs right after you’ve driven off the dealer’s lot.

Buying used can be beneficial for first-time buyers who want to try out a vehicle for a few years before buying new.

But if you plan to buy used, do some research and make sure the vehicle is structurally sound including the conversion. Ask for a vehicle history (CARFAX) report, and get the vehicle inspected by a mobility dealer to ensure it’s in good shape and was well taken care of.

Q: How do people manage to pay for it?

A: Many consumers used home equity loans to purchase a vehicle and adaptive equipment. But with home values decreasing.

Many dealers and manufacturers work with lending institutions that offer extended-term financing, including 10-year loans, allowing consumers to make lower, more affordable monthly payments. The downside is that consumers are locked into the vehicle for 10 years, and end up paying more in interest.

If you finance for 10 years, and you’re not going to keep the vehicle for that amount of time, you’re going to lose money when you try to sell or trade it because you haven’t paid off much of the balance.

When you buy a new vehicle, many car manufacturers offer mobility reimbursement programs (up to $1,000) to help offset the cost for the purchase and installation of adaptive equipment.

Williams syndrome

Williams syndrome is a genetic condition that is present at birth and can affect anyone.  It is characterized by medical problems, including cardiovascular disease, developmental delays, and learning disabilities.  These occur side by side with striking verbal abilities, highly social personalities and an affinity for music.

WS affects 1 in 10,000 people worldwide – an estimated 20,000 to 30,000 people in the United States. It is known to occur equally in both males and females and in every culture.

Unlike disorders that can make connecting with your child difficult, children with WS tend to be social, friendly and endearing.  Parents often say the joy and perspective a child with WS brings into their lives had been unimaginable.

But there are major struggles as well.  Many babies have life-threatening cardiovascular problems.  Children with WS need costly and ongoing medical care, and early interventions (such as speech or occupational therapy) that may not be covered by insurance or state funding.  As they grow, they struggle with things like spatial relations, numbers and abstract reasoning, which can make daily tasks a challenge. And as adults, most people with WS need supportive housing to live to their fullest potential.  Many adults with WS contribute to their communities as volunteers or paid employees, for example working at senior homes and libraries or as store greeters or veterinary aides.

Just as important are opportunities for social interaction. As people with WS mature – beyond the structure of school and family activities – they often experience intense isolation which can lead to depression.  They are extremely sociable and experience the normal need to connect with others; however people with Williams syndrome often don’t process nuanced social cues and this makes it difficult to form lasting relationships.

Common features of Williams syndrome include:

  • Characteristic facial appearance
    Most young children with Williams syndrome are described as having similar facial features. These features include a small upturned nose, long philtrum (upper lip length), wide mouth, full lips, small chin, and puffiness around the eyes. Blue and green-eyed children with Williams syndrome can have a prominent “starburst” or white lacy pattern on their iris. Facial features become more apparent with age.
  • Heart and blood vessel problems
    The majority of individuals with Williams syndrome have some type of heart or blood vessel problem. Typically, there is narrowing in the aorta (producing supravalvular aortic stenos is SVAS), or narrowing in the pulmonary arteries. There is a broad range in the degree of narrowing, ranging from trivial to severe (requiring surgical correction of the defect). Since there is an increased risk for development of blood vessel narrowing or high blood pressure over time, periodic monitoring of cardiac status is necessary.
  • Hypercalcemia (elevated blood calcium levels)
    Some young children with Williams syndrome have elevations in their blood calcium level. The true frequency and cause of this problem is unknown. When hypercalcemia is present, it can cause extreme irritability or “colic-like” symptoms. Occasionally, dietary or medical treatment is needed. In most cases, the problem resolves on its own during childhood, but lifelong abnormality in calcium or Vitamin D metabolism may exist and should be monitored.
  • Low birth-weight / slow weight gain
    Most children with Williams syndrome have a slightly lower birth-weight than their brothers or sisters. Slow weight gain, especially during the first several years of life, is also a common problem and many children are diagnosed as “failure to thrive”. Adult stature is slightly smaller than average.
  • Feeding problems
    Many infants and young children have feeding problems. These problems have been linked to low muscle tone, severe gag reflex, poor suck/swallow, tactile defensiveness etc. Feeding difficulties tend to resolve as the children get older.
  • Irritability (colic during infancy)
    Many infants with Williams syndrome have an extended period of colic or irritability. This typically lasts from 4 to 10 months of age, then resolves. It is sometimes attributed to hypercalcemia. Abnormal sleep patterns with delayed acquisition of sleeping through the night may be associated with the colic.
  • Dental abnormalities
    Slightly small, widely spaced teeth are common in children with Williams syndrome. They also may have a variety of abnormalities of occlusion (bite), tooth shape or appearance. Most of these dental changes are readily amenable to orthodontic correction.
  • Kidney abnormalities
    There is a slightly increased frequency of problems with kidney structure and/or function.
  • Hernias
    Inguinal (groin) and umbilical hernias are more common in Williams syndrome than in the general population.
  • Hyperacusis (sensitive hearing)
    Children with Williams syndrome often have more sensitive hearing than other children; Certain frequencies or noise levels can be painful an/or startling to the individual. This condition often improves with age.
  • Musculoskeletal problems
    Young children with Williams syndrome often have low muscle tone and joint laxity. As the children get older, joint stiffness (contractures) may develop. Physical therapy is very helpful in improving muscle tone, strength and joint range of motion.
  • Overly friendly (excessively social) personality
    Individuals with Williams syndrome have a very endearing personality. They have a unique strength in their expressive language skills, and are extremely polite. They are typically unafraid of strangers and show a greater interest in contact with adults than with their peers.
  • Developmental delay, learning disabilities and attention deficit disorder
    Most people with Williams syndrome mild to severe learning disabilities and cognitive challenges. Young children with Williams syndrome often experience developmental delays.  Milestones such as walking, talking and toilet training are often achieved somewhat later than is considered normal. Distractibility is a common problem in mid-childhood, which can improve as the children get older.

Older children and adults with Williams syndrome often demonstrate intellectual “strengths and weaknesses.” There are some intellectual areas (such as speech, long term memory, and social skills) in which performance is quite strong, while other intellectual areas (such as fine motor and spatial relations) show significant weakness.