CMS: New HCBS Rule...but NOT Immediate changes!

An Update from the CMS:

January 9, 2015
New Year Brings New HCBS Settings Rules ... but NOT immediate changes in services!

As states and stakeholders across the country assess their Medicaid-funded home and community-based settings for compliance with the 2014 regulations, many people with disabilities and their families have asked whether their current services will change immediately, due to the regulation.

In other words, they want to know:
If a state determines that a current HCB setting is not compliant with the new regulation, does it have to stop providing services in that setting immediately?

The answer is NO.
 
If a state determines that HCBS are currently being provided in settings that do not provide opportunities for participants to seek employment and work in competitive settings, engage in community life, control personal resources, and access the community to the same degree of access as individuals not receiving Medicaid HCBS, or if individuals receiving HCBS are not residing in settings that meet the HCB settings requirements, the state has until March 2019 to bring its HCBS programs into compliance with the rule, consistent with its State Transition Plan. States can claim for federal matching funds for these services during the transition period.

For more information about the Medicaid HCBS regulation, including a settings requirements toolkit, please visit: http://www.medicaid.gov/Medicaid-CHIP-Program-Information/By-Topics/Long-Term-Services-and-Supports/Home-and-Community-Based-Services/Home-and-Community-Based-Services.html

How far have we come in preventing disabilities in Preemies?

The answer is mixed, according to an article in Disability Scoop, "Despite Advances, Many Preemies Still Face Severe Disabilities" by Michelle Diament, 12/11/12.

Neither one of my sons' disabilities were caused by prematurity. Although they both survived because of care given to them in intensive care neonatal nurseries, that care was not enough to spare them from lives with profound mental and physical disabilities.

After Danny was born in 1976, I used to see photographs and news stories about premature infants and others who had survived and thrived after overcoming extremely difficult circumstances at the time of birth. I noticed one day at the hospital where Danny was born - I think we were making one of our treks to see his orthopedic surgeon - a bulletin board full of photographs from a recent reunion of "graduates" from the neonatal intensive care unit. Hmm?, I thought. We weren't invited. (You see how petty I can be when it comes to a perceived insult to a child of mine.)

Among the children at the reunion, not one was in a wheelchair. One wore glasses but otherwise there were no visible signs that any of them had significant disabilities. In the crowd I hung out with, mostly parents whose children were similar to Danny, many of those children had done time in the same neonatal intensive care nursery. I have no idea if there was any deliberate plot to exclude children like mine from the reunion party - maybe their parents were too tired or just didn't feel like celebrating. But since then, I have always been somewhat skeptical about claims that we are making tremendous progress in preventing and treating severe disabilities.

The Disability Scoop article sites two British studies. One concludes that, "Survival of babies born between 22 and 25 weeks’ gestation has increased since 1995 but the pattern of major neonatal morbidity [the incidence or prevalence of disease in a population] and the proportion of survivors affected are unchanged. These observations reflect an important increase in the number of preterm survivors at risk of later health problems."  Another, in comparing outcomes between 1995 and 2006, concludes that "At follow-up the findings are mixed: there is some evidence of improvement in the proportion of babies who survive without disability, an improvement in developmental scores, and a reduction in associated neuromorbidity (seizures and shunted hydrocephalus), but no change in the rate of severe impairment."

Overall, there are more preemies who survive without any disability, and good for them, but the proportion of those with severe disability has remained the same.

A Different Path newsletter for parents of special needs children

Washtenaw County, Michigan, is the home of A Different Path newsletter, a non-profit quarterly publication that relies solely on individual donations to cover mailing and printing costs. I have posted the Fall 2012 newsletter here.

As always, A Different Path is written and produced by local parents and is both heartfelt and informative. This issue has an especially good article written by two parents on approaches to using assistive technology with two different children with cerebral palsy. Access to assistive technology is best viewed as an "engineering challenge" that allows children with CP to overcome physical barriers to give them more control over their environment. There is advice on who to talk to and costs for setting up assistive technology systems that work for the child.

Throw a few bucks their way by sending a check to: 

Different Path
P.O. Box 8276
Ann Arbor, MI 48107-8276

The House of Gort: a glimpse into the life of a Michigan family caring for two severely disabled daughters

The House of Gort from Steve Tatzmann on Vimeo.

"The House of Gort" is an exceptionally well-done documentary giving a glimpse into the life of the Gort family. Gwen, the first daughter of Tim and Gina Gort, was born with cerebral palsy. The second, the adorable Violet, has no disabilities. The third daughter, Eliza, was born healthy but suffered a cardiac arrest due to a medical error in the hospital. She has severe cerebral palsy as a result of the brain damage she suffered. [Gwen and Eliza are no less adorable than Violet, but they get starring roles in the video. Violet will have to settle for "best supporting actress" with extra accolades for being adorable.]

The video documents the constant care that the Gorts give their daughters and the consequences of medical errors. Their story was covered in the Grand Rapids News on 8/12/12 in an article by Sue Thoms - Parents sue DeVos Children's Hospital over newborn daughter's brain damage: 'They know they are wrong'.  It was also covered in a Grand Rapids TV news story on 8/12/12.

The Gort's have a blog where you can follow their story.

The risks of dental sedation and how to make it safer

My son Danny is one of those impossible dental patients who cannot be worked on safely without sedation. A little Valium before a dental appointment does not do the trick. It may be enough to put him to sleep during the van ride to the dentist, but as soon as anyone gets near his mouth or touches his face he is a wide-awake wild man. 

Danny has had his teeth cleaned successfully using IV sedation, but this is not a risk-free procedure. A Detroit Free Press article from February 19, 2012, "Painless, at what price? Risks of dental office sedation prompt calls for tighter regulation" by Patricia Anstett, exposes the risks of dental sedation and how to improve training for dentists and the overall safety of sedation procedures.

The use of dental sedation is growing most with the use of conscious or moderate sedation involving the use of oral or intravenous medicine to relax a person. Deaths from dental sedation are rare but alarming, especially because some of the deaths have occurred in children with cerebral palsy.

According to the article:

• "Michigan has not updated state laws to comply with 2007 national recommendations from the American Dental Association calling for more training of doctors performing sedation, and it is one of only two states that don't require permits for providers doing the procedures." 
• "Michigan's Board of Dentistry has been reviewing ways to strengthen dental sedation regulations, which have been largely unchanged since 1997. The board expects to adopt new standards this year and is likely to address them at its April 12 meeting, said Rae Ramsdell, director of the Bureau of Health Professions in the Michigan Department of Licensing and Regulation…The rules are likely to require more training for dental practices offering moderate sedation."
• Deaths from dental sedation are difficult to track. Many deaths and complications are never recorded as dental-related because they may occur at home or in a hospital.

 
The Michigan Board of Dentistry will be releasing a final draft of regulations to improve the training of dentists and the safety of dental sedation soon. It must hold a public hearing on the new regulations and publish the standards.
 
How to comment on the proposed regulations: Write: Michigan Board of Dentistry, Department of Licensing and Regulation, Bureau of Health Professions; P.O. Box 30670, Lansing 48909

A drug to prevent premature birth priced out of reach for most women?

[Premature birth is a significant risk factor for cerebral palsy and other disabilities]

The Detroit Free Press featured an article on March 21, 2011, (Drug price for a safe pregnancy: $30,000 by DFP medical writer Patricia Anstett) about a drug that has been effective in reducing premature births and has been available for pregnant women since 2003. The cost has been $200 for a regimen of 20 injections.

The Federal Drug Administration has granted final approval for Maken, the brand name of the drug that is now produced exclusively by Ther-Rx, a subsidiary of K-V Pharmaceutical of St. Louis. The drug has been designated an orphan drug (benefitting fewer than 200,000 people) which gives special incentives so that the drug company can make money on it. A regimen of 20 injections will now cost $30,000.

The pharmaceutical company has created a financial aid program to help poor women get the drug for free and others to obtain discounts, but the high price may deter Medicaid from paying for the drug and generally reduce access to the drug by pregnant women who need it.

The Website "A Healthier Michigan", sponsored by Blue Cross Blue Shield of Michigan, posts an article, Inflated Drug Price Lands Pharma Company in Social Media Crisis, discussing the PR problems the drug company is facing with the launching of a facebook page opposing the exorbitant price increase.

More background on the story is available in an article from the St. Louis Dispatch from March 10, 2011. It turns out the company that has exclusive rights to market the drug did not invent it. The new cost of the drug will make it more expensive to use the drug to prevent premature births than to not treat women and pay the added costs of the premature births that will occur.

Just one more clue as to why health care costs are so high.

Community Resources: Waiting for spring at the Botanical Gardens

Anyone who has spent time with my son Danny knows that his mood can fluctuate wildly, between sublime and exuberant joy to extreme irritation and frustration. Even on a good day, he is often cranky. Danny cannot communicate in any specific way about what he is feeling or why he feels the way he does. My educated guess (after 34 years) is that his hypersensitivity to touch and sometimes sound, combined with discomfort related to his severe cerebral palsy including gastrointestinal problems, have a lot to do with it. Danny seems to know, however, what makes him feel better: if he could, he would spend most of his time sitting outside in his wheelchair, listening to the birds, and breathing fresh air.

Winter is a rough time of year for Danny, but we have found an almost perfect place to take him to make the wait for warm weather a little easier. The University of Michigan Matthaei Botanical Gardens in Ann Arbor are full of all the sensory experiences that Danny can appreciate. Although Danny has very little functional vision, he can hear, feel, and smell all that the greenhouses have to offer.

In the tropical greenhouse, the air is warm and humid. There is a fish pond with water dribbling from it and a relatively large water fall whose sound sends Danny into a fit of ecstasy.  We enjoy the lush displays of orchids, the banana trees producing one of Danny's favorite fruits, and we see and smell the new green growth and flowering tropical plants.  We have contributed a couple of overgrown trees to the Botanical Gardens that we visit on each trip - a Key lime tree that we started from seed and a Plumaria, both from Florida. 

The temperate greenhouse is cooler but also light and spring-like. It has a koi pond with water dripping over ferns into a pool. I always stop by the rosemary bush to give Danny a whiff of rosemary and pull gardenias or other sweet smelling flowers close enough for him to inhale. The third house is a dry desert environment that is cooler and houses exotic cacti, Spanish bayonets, Century plants, and other oddities.

Danny can hear the sounds of people milling around, especially small children who delight him with shrieks and chatter. On this particular Sunday, there is something like a scavenger hunt going on where children mark off items on a list of objects and plants that they discover on their route through the greenhouses. As an added bonus, Danny discovers that in the desert house there is black sheet metal along the walls covering up a heating apparatus. Danny finds that it is at wheelchair height and begins pounding on the sheet metal with his right arm. (He also does this when he gets near metal file cabinets or large cardboard boxes as a way to express his inner rowdy nature.) I let him bang on the metal until it gets so loud that some of the children look alarmed and begin staring. There is plenty of room in the greenhouses to move Danny past the temptation to bang on sheet metal, and so we move on. It's not nice to frighten the children.

On our way out we walk through a fine mist that periodically sprays water to humidify the tropical plants. Danny loves the hissing sound the sprayers make and also appreciates the warm dampness that surrounds him.

The botanical gardens are such a delight, that I would hate to spoil the experience by claiming that it does anything to improve Danny's status in the community or that it in any way dispels the notion that some people with disabilities have problems that can't be overcome by high expectations. Watching Danny's infectious joy is enough for us and we suspect that it has not gone unnoticed by our fellow fans of the Botanical Gardens.

Disability cures and treatments: hype and hope

By the time Danny was five years old, it was pretty clear to my husband and I that we were going to be in the disabilities game for a long time. In the early years, I read and heard about many purported cures and treatments that promised to make our boy "snap out of it" and get on with his life as a normal person. There were always caveats, however. If the treatments and therapies did not have the desired effect, it was probably because the parents weren't doing it right or they weren't sufficiently committed to the idea to make it work. Because Danny's care was so exhausting, it was a relief to me when the latest fad proved to be without merit before I had a chance or inclination to try it out. After a while, the fads that I might have fallen for earlier began to seem absolutely bonkers.

At a United Cerebral Palsy conference, I sat through a presentation about how you can improve your child's emotional health and behavior by painting his room the correct color to match his condition. "The spinal cord is a rainbow!" said the man who was presumably paid to present this drivel. As I recall, he had charts and diagrams showing how the color of the room somehow worked its way through the eyes and brain to the spinal cord with miraculous effect. I later referred to this method as Cure by Interior Decoration.

Then there was patterning that promised that after a mere 8 or 9 hours per day of taking Danny through rigorous movement exercises, new pathways would be forged in his damaged brain, allowing him to progress. Swimming with dolphins, said Parade Magazine, improved the speech of Down Syndrome children by 20% (20% of what, it did not say). Behavior modification could produce improvement of inappropriate or sometimes just annoying behaviors through positive and negative reinforcement to encourage the child to behave. In case that didn't work, aversive behavior interventions were available to threaten, intimidate, and physically force the child to do what you wanted.

In an odd twist, advocates of Neurodiversity oppose spending money on research to find cures for autism and related disabilities. They believe that people with these conditions represent diversity on the broad spectrum of human behavior and thought. Since there is nothing wrong with them, they don't need to be cured. They especially oppose research on genetic causes of autism that might lead to their eugenic elimination. I wonder how many people with these "differences" take medication to reduce symptoms such as anxiety, depression, and obsessive compulsive disorder? Are they being true to their beliefs when they refuse to accept "differences" that can be conveniently treated with medication? Is it cheating for a person with seizures to take anti-seizure medication or a diabetic to take insulin? The Neurodiversity idea raises more questions that it answers.

Once in a while a really kooky idea comes along that actually works. The Ketogenic Diet, for instance, is a high-fat, low-carbohydrate diet that reduces or eliminates seizures in some kids. Recommending the diet doesn't take a lot of high-pressure salesmanship on the part of doctors who are willing to monitor kids on this diet, because the results speak for themselves. That this is a difficult diet to maintain and hard on families is acknowledged upfront and the parents ultimately decide whether it is worth a try to stop intractable seizures in their children.

Recently, the New York Times published an article, Promise Seen in Drug for Retardation Syndrome, 4/29/2010, that describes hope for a medical treatment for some symptoms of Fragile X Syndrome. Fragile X is "the most common cause of inherited mental impairment. This impairment can range from learning disabilities to more severe cognitive or intellectual disabilities. ... FXS is the most common known cause of autism or 'autistic-like' behaviors. Symptoms also can include characteristic physical and behavioral features and delays in speech and language development". (This is according to the National Fragile X Foundation Web site.)

Research on Fragile X is done by real scientists (rather than interior decorators and dolphin trainers) who work to improve understanding of the genetic causes of the syndrome in hopes of finding a way to lessen its symptoms and perhaps point the way to treating other related conditions. Here are the fascinating details from the article on how Fragile X Syndrome works, in case you're interested:

"Fragile X is caused by a genetic stutter in which a portion of the gene gets repeated like a scratched album. With each subsequent generation, the number of repeats tends to rise. So if a mother has 10 repeats, her child might have 11 or 12. For reasons that are not well understood, however, this process of repeat amplification can suddenly go haywire. So mothers who have 55 or more repeats tend to have children with hundreds.

"In anyone with 200 or more repeats, the body shuts off the gene. Since genes are used to make proteins, this genetic silencing means the encoded protein is never made. The absence of this protein in cells causes the wide-ranging effects of fragile X syndrome. Those with 55 to 200 repeats are considered carriers, and recent research shows they can have severe neurological declines late in life that mimic Alzheimer’s and Parkinson’s."

Getting back to the new discovery that the article features, scientists have found that fragile X patients have an "overload of unchecked synaptic noise" (the synapses are the junctions between brain neurons). The new drug produced by the drug company Novartis appears to reduce the noise by replacing at least one missing protein so that "memory formation and high-level thinking can take place allowing children to develop normally."

Wow! The president of the Fraxa Research Foundation that finances fragile X research says "this may be the key to solving the mystery of autism and other developmental disorders." A spokesperson from Autism Speaks says pretty much the same thing. A former researcher at another big drug company Merck says this is "the most promising therapeutic discovery ever for a gene-based behavioral disease." The best news is that if you are a mouse, a fish, or a fruit fly that has been genetically engineered to have fragile X, you will be rendered practically normal if you take a compound including the missing fragile X protein.

Is this too good to be true? Probably. If you read the article carefully you find out that the trial for the drug involved only a few dozen patients with fragile X and only some of those benefited. The study was too brief to measure effects on basic intelligence. Improvement was seen in an "undisclosed biological trait". A total of two parents were euphoric with the results of the trial. The Novartis results were not published or peer reviewed (this means they were not refereed by other experts in the field, including competitors, for publication). For commercial reasons, many details were not divulged. The drug is years from being available if it even survives more rigorous trials and further study.

The article includes many fascinating details of fragile X syndrome and how research has progressed over the years, but the drug companies and the groups that raise money for fragile X and autism research will probably benefit the most from the publicity. Parents are prone to euphoria when they hear of a solution to a difficult and sometimes desperate situation with their child, and who can blame them. Hope is never a bad thing, but exploitation of that hope for publicity and fundraising makes me queasy.

Buyer beware! You might want to investigate Web sites like Quackwatch before investing money, time, and energy in alluring cures and treatments that aren't all they pretend to be.

The AACIL: Activities for People with Disabilities

The Ann Arbor Center for Independent Living (AACIL) offers lots of activities for people with disabilities. Although their focus is generally on people with physical disabilities, they also serve people with other developmental and cognitive disabilities. Even if your child has never participated in AACIL programs, it is worth a look to see if there might be something for a more severely impaired child or adult.

The AACIL also has an email news letter with updates on CIL activities:

eNews for Youth
A Periodic ePublication of the Ann Arbor Center for Independent Living
for Young People with Disabilities

Call to be placed on the email list.

Contact the AACIL at:

Ann Arbor Center for Independent Living
3941 Research Park Drive
Ann Arbor, MI 48108
(734) 971-0277
phone: (734) 971-0826 fax: (734) 971-0277

How to survive to twenty-five: Ian B. has finally arrived.

Happy Birthday, Ian!

Ian was born a few weeks early by an emergency C-section at Stanford Hospital in 1985. He was found to have an alpha strep infection that he contracted in utero, a very unusual occurrence. A medical student made a little project out of this and found only 30 mentions in the medical literature of this happening.

Ian started his life deathly ill with a six-week stay in the neonatal intensive care unit at Stanford. After the strep infection subsided, he was left with severe brain damage. A CT-scan showed a large cyst at the back of his brain that should have left him blind. As it turned out, he does see, although his vision is not normal, but it is useful to him and for this we can be grateful.

Ian has severe cerebral palsy, severe mental retardation, and is unable to communicate in any specific way. He is, however, a charming young man with many fans. One teacher at his school told me once that Ian is the nicest person she has ever met. The effect he has on people is pretty amazing considering that he has no specific way of telling them what he thinks of them or any way of doing things for them except to cheer them up.

From the very beginning, Ian had high expectations that the world is a happy and kind place. When it disappointed him, everyone in his sphere of influence seemed to know that it was his or her job to fix things for him, to restore his faith in the world as it should be. Life for Ian has not always been easy, but he retains an innate cheerfulness when things are going well.

About the only thing that Ian can actively do is move his left hand so that he can bang on a toy piano. The piano in this picture was a piano/xylophone that had an octave and a half worth of keys. For Ian's twenty-first birthday, John took it apart to give it a really good cleaning and discovered that our daughter, at a very young age, had stuffed gummy bears and other debris into the innards of the piano. Sadly, the piano disintegrated about a year later and was replaced with another that is perfectly adequate, but just not the same. But Ian keeps plunking along.

The University of Michigan studies thinking speed in children with cerebral palsy

This is a request for participants from the University of Michigan Adapted Cognitive Assessment Laboratory in a study of thinking speed in children with cerebral palsy:

The Adapted Cognitive Assessment Lab (ACAL) at the University of Michigan is actively recruiting students to participate in a Thinking Speed Study. Students must be:

• Between the ages of 8 and 16 years of age.
• Have a medical diagnosis of Cerebral Palsy.
• Have a parent/guardian present to provide written and informed approval for their child to participate in the study.
• Have no medical or psychiatric condition that affects the tests, including changing doses of medication or a history of brain injury

Participants can expect to spend two to three hours in the study, and will be paid a $50.00 honorarium for their time and effort.

This study is funded by the National Institutes of Health and the U.S. Department of Education and is approved by the University of Michigan’s Institutional Review Board (HUM00014311).

Standardized testing requires a child to speak, write, or point to answers. This study is designed to separate physical capability from measurement of thinking capability. The ACAL investigators conduct these tests through a computerized program that uses Visual Inspection Time and Assistive Technology. Visual Inspection time is measured by the amount of time a person needs to look at something before they can correctly make a simple judgment about what he/she just saw – slow movement does not mean slow thinking.

To learn more about how your child can participate in the Thinking Speed Study e-mail ACAL-Research@umich.edu or call 734-936-6604 or 734-763-6189.

If you are interested in Cerebral Palsy research and Adapted Cognitive Assessment Laboratory studies you may also enroll your child with the University of Michigan’s engage Registry here.

Physical Medicine and Rehabilitation
Adapted Cognitive Assessment Lab
325 E. Eisenhower, Suite 100
Ann Arbor, MI 48108
734-936-6023