Tales from the Hydrant

by Kathy Wechsler

When one member of the family is found to have ALS, everybody in the family — including the family dog — is affected by the disease in interesting and unpredictable ways. Some family members adapt better than others.

The stories of Mike, Sahara and Coolidge illustrate a few of the different ways in which your pets may respond to your physical changes brought on by ALS.

Mike (aka “the German Shedder”)

Stephen Hallgren and Mike.

Stephen and Glory Hallgren live in Somerset, Wis., with their three cats, 14 chickens and a 110-pound German shepherd named Mike.

Stephen, 57, who received a diagnosis of ALS last spring, says that the 5-year-old shepherd seemed to sense the disease’s onset. Back in the summer of 2003, Hallgren fell in his front yard.

“The next summer, after my leg had healed [from the fall], Mike wouldn’t leave my leg alone,” Hallgren says. “He kept licking my lower left leg, and that’s where my ALS started the next fall when I first noticed a problem.”

Mike could also sense when Hallgren’s ability to walk began to deteriorate.

“He has become much more aware of my movements,” says Hallgren, who walks with the aid of a cane. “If he is lying in a path that I need to use, all I need to do is walk up to him and he knows that I can no longer jump over him, so he gets up and moves.

“Another thing I have been able to convey to him since my energy has diminished is to just go ‘psst,’ and he knows that it’s nap time,” and goes to his bed.

Hallgren misses his regular three-mile runs with Mike.

“He was more my dog, but I’m not doing much with him, and I think he’s becoming more Glory’s dog because she takes him on walks,” he says. “I still spend the whole day with him, so he’s pretty close to me.”

Sahara

Sterling and Jeannette Kelley of Hallsville, Mo., have a 9-year-old border collie who’s always been a part of the family. Sahara’s loving, playful personality changed in 2003, when Jeannette, now 73, was found to have ALS.

As Jeannette’s bulbar-onset ALS progressed, a feeding tube and then a tracheostomy became necessary, and Sterling has become her full-time caregiver. Jeannette’s speech is affected, and she uses a power wheelchair for mobility.

“I don’t have time to take the dog out and play with her,” Sterling says. “This disease has pretty much ruined our relationship with Sahara because my wife has to stay so close to the suction machine and I have to stay close to her.”

To Jeannette’s dismay, Sahara has become very reclusive. Fearful of the noises made by Jeannette’s trach and suction machine, the black-and-white border collie spends much of the time hiding in her kennel in the back bedroom.

“Now [Sahara] stays away from my wife because she doesn’t understand the noises — my wife can’t talk to her,” says Sterling. “It’s changed their relationship entirely. Sahara no longer feels comfortable in her own home.”

To give Sahara time to be an ordinary dog, the Kelleys often send her home with friends or their two grown daughters’ families, where Sahara can be her active self again.

Coolidge

After receiving a diagnosis of ALS in May 2004, Judson Harmon moved to St. Paul, Minn., to live with his daughter, Kathryn Harmon Ledo, her husband, Rob Ledo, and their 11-month-old son, John Judson Ledo.

Last year when Harmon, 71, who now uses a power wheelchair, was able to walk with the aid of a walker, he stumbled and fell in the carpeted living room, “probably yelling ‘Ooooff’ and muttering several rude words as I hit the floor.”

The house was empty except for two cats, a pigeon, and Kathryn’s 3-year-old black-and-tan American coonhound, Coolidge. Harmon, unhurt but unable to get up, had no choice but to wait for Kathryn to return home from a short errand.

“I felt Coolidge put his head firmly under my left arm and my chest, as if to help me rise to my feet,” says Harmon. “I immediately thought ‘How wonderful!’ and later, cynically, ‘Maybe he’ll help me find and balance my checkbook after this.’”

By the way, Coolidge wasn’t successful at raising Harmon to his feet, but he was good company while Harmon waited to be rescued.

“So, what was Coolidge’s purpose when he came to my side and put his head under me as I knelt, helpless, on the living room floor? Was it to cuddle, to stop my yelling, to see if I had any treats, mere curiosity, or to raise me up? He didn’t say.”

Coolidge is protective of both Judson Harmon and his grandson John.

Coolidge has always been a protective and caring dog. For example, Harmon gets his legs stretched once a week by a home health aide. When the stretching gets painful and Harmon cries out, Coolidge often comes over, puts himself between Harmon and the health aide, and licks Harmon’s hand, as if to ease his pain.

“Coolidge also watches out for baby John and warns Dad when his wheelchair gets too close,” says Harmon’s daughter, Kathryn, who’s the alpha female of the pack. “He’s given Dad a gentle nibble or even a growl when Dad has approached the play pen too quickly.”
“When we play tug-of-war, [Coolidge] adjusts to me and doesn’t pull very hard,” Harmon says. “He also knows he will get a high-quality scratching from me.”

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ALS RESEARCH ROUNDUP

by Margaret Wahl

Angiogenin Mutations Likely Boost ALS Susceptibility

Mutations in the gene for angiogenin (ANG), a protein that aids in the formation of new blood vessels, are new suspects in ALS susceptibility, says a multinational research group whose findings were published online Feb. 26 in Nature Genetics.

The team, led by Matthew Greenway and Orla Hardiman at the Royal College of Surgeons in Dublin, Ireland, included Robert Brown, professor of neurology and director of the MDA/ALS Center at Massachusetts General Hospital in Boston.

In a study of 1,629 people with ALS and 1,265 without ALS, the investigators identified seven genetic mutations (alterations) in the ANG gene in four people with familial ALS and 11 with no family history of the disease (sporadic ALS). Twelve of the 15 were of Irish or Scottish descent. Only one person, a 65-year-old man, had an ANG mutation without having ALS.

Angiogenin resembles another protein, vascular endothelial growth factor (VEGF), in that both cause the formation of new blood vessels in response to a low-oxygen environment. Unusually low levels of VEGF have been implicated as a possible factor in ALS.

The ANG gene variations may be risk factors only in some populations, Brown says, although they still may be important. “The caveat from the VEGF story is that those variants were found to be highly related to sporadic ALS only in the Belgian population,” he says. “This has not been reproduced in other populations.”

ANG mutations are “certainly overrepresented in ALS and certainly significantly overrepresented in the population of Ireland and Scotland,” Brown says.

These types of variations are being sought on a large scale at the Translational Genomics Research Institute in Phoenix (see “Gene ‘Flavor’ Differences”).

Spinal Fluid Protein Levels Useful in ALS Diagnosis

ALS doesn’t have clear biological markers (biomarkers) of disease, which are useful not only to diagnose a condition but as indicators of whether or not a treatment is working.

Merit Cudkowicz

Neurologist Merit Cudkowicz, an MDA research grantee at Massachusetts General Hospital, and Robert Brown (see “Angiogenin Mutations”), were part of a multi-institutional group that recently identified three spinal fluid proteins for which levels are lower than normal in ALS. The researchers say these protein levels may prove useful as ALS biomarkers.

The team published its results online Feb. 15 in Neurology.

These three proteins — cystatin C, VGF (not VEGF), and a third identified so far only by its weight — were lower in concentration in the ALS patients’ spinal fluid samples than in samples from unaffected study participants.

“Finding biomarkers that can assist physicians with diagnosis would be beneficial,” Cudkowicz said. “Biomarkers are also important to help understand disease mechanisms and potential treatment pathway targets, and could also potentially help expedite clinical trials by providing ... outcome measures,” she said, adding that the group’s results need to be replicated by others and in larger sample sizes.

CDC Gets $900,000 to Start ALS Registry; ‘Now What?’ Is the Next Question

by Christina Medvescek

The national Centers for Disease Control and Prevention (CDC) have been instructed by Congress to allocate $900,000 for starting a nationwide ALS registry.

The allocation is a scant fraction of what’s needed for a registry, and there’s no guarantee of more; in fact the project was specifically eliminated in the 2007 presidential budget proposal.

But some sort of ALS information-gathering project continues to inch forward, though its final form is unclear. The goal is to paint a bigger picture about the occurrence and progression of ALS in the United States, in order to further research and treatments.

Get the Ball Rolling

Senators Harry Reid (D–Nev.) and Tom Harkin (D-Iowa) managed to insert the ALS project into the 2006 Health and Human Services (HHS) budget signed by President Bush in February.

Reid also co-sponsored the ALS Registry Act, which seeks to create a national database at a cost of $25 million the first year. (See “National ALS Database Proposal Before Congress,” September 2005.) Both registry bills (H.R. 4033 and S. 1353) currently are stuck in committee.

The HHS allocation was an attempt to get the ball rolling. However, the project was eliminated in President Bush’s 2007 proposed HHS budget, which slashed $1.5 billion from programs like centers for traumatic brain injuries, an Alzheimer’s disease education program and inner-city Indian health clinics.

Coordinated Effort

Now the question is: What can be done for $900,000?

Not much but something, says Dee Williamson, an epidemiologist with the CDC Agency for Toxic Substances and Disease Registry in Atlanta.

The agency already had been counting the number of cases of ALS and multiple sclerosis (MS) in specific areas, in hopes of developing a protocol for gathering data nationally. However, counting cases (a surveillance project) is far simpler and less expensive than a registry, which gathers detailed, long-term data on disease risk factors and progression.

”We were looking for a way to tell how many people have these conditions, and to be able to say if there’s more in one place than another,” explained Williamson. Some data has been gathered but no report issued. (For a 2003 CDC fact sheet on the project, see www.atsdr.cdc.gov/DHS/MS_Fact_Sheet.html.)

The CDC will meet this spring with representatives of ALS databases and ALS groups, to discuss the situation, goals and ways to maximize resources.

Among the few existing databases is the new ALS Connections registry (www.alsconnection.com) funded by MDA and run by Robert Miller through the Forbes Norris MDA/ALS Research Center in San Francisco. It’s the online version of the paper-based ALS C.A.R.E. registry (www.outcomes-umassmed.org/als/).

ALS Registry Web Site Seeks Volunteer Designer ALSconnection.org is looking for a volunteer with experience in Web design to make the site more appealing. ALSconnection is a Web-based registry for people with ALS, administered through the Forbes Norris MDA/ALS Center in San Francisco, which gathers information to aid research efforts and provides information about ALS. The ideal volunteer would devote approximately four hours a week for six months to the project. For more information or to apply, contact Dina Scholtz at scholtd@sutterhealth.org.

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Insurance Claims:
Don’t Take ‘No’ for an Answer

by Christina Medvescek

Last fall, Catherine Wolf, 58 and a nine-year ALS survivor, won a war with her insurance plan over long-term nursing coverage. Wolf, who is vent dependent, now has insurance coverage for round-the-clock skilled nursing. The experience taught her valuable strategies for fighting insurance company denials.

The keys, say Wolf and her private attorney Mark Scherzer, who works with New Yorkers for Accessible Health Coverage, are: dogged persistence, documentation, and “don’t believe everything you hear.”

Winning the War

Insurance companies most commonly deny requested items because they’re “not medically necessary” or “not a covered charge.”

Experts note that while some denials are legitimate, others are due to coding or paperwork errors, or to differing interpretations of what your condition requires. Occasionally, they’re just a brazen attempt by the insurer to hang on to its money.

No matter the reason, insurance companies count on the fact that most people don’t appeal a denial.

Appealing isn’t easy, but then again, neither is ALS. Here are a few hard-won tips:

Know Your Policy: Wolf was told by her insurer and her corporation’s human resources department that her preferred provider plan (PPO) didn’t cover long-term skilled nursing. “This turned out to be false,” she says via e-mail from her home outside New York. “Don’t believe everything you hear. Demand documentation. I sound militant but it’s best to be polite and establish good relations. Put all requests in writing and mail them ‘return receipt requested’ so you have a paper trail.”

Catherine Wolf

Even if it turns out the requested benefit isn’t covered, it doesn’t hurt to ask the insurer to cover it in your case. This strategy works best if you document how it will save money in the long run, and if your employer is large enough to have some clout with the insurer. A lawyer can help make this case most effectively, says Scherzer.

Know the Law: Aspects of ALS care, such as deep suctioning, giving medications when the patient isn’t able to indicate consent, and chest physical therapy, require a skilled professional under Medicare guidelines and some state laws, says Wolf. “If you’re requesting skilled health services, find out from your state health department who is allowed to administer the requested services.”

Also determine whether the insurance company employee who issued the denial has a medical license. Some state laws require a doctor’s denial on items like power wheelchairs or some medical procedures.

Get an Effective Letter of Medical Necessity: Wolf’s insurer claimed her nursing care was “custodial” rather than medical and denied it as “not medically necessary.” It’s critical in this case that a doctor writes an effective letter of medical necessity.

“Some doctors write angry letters to insurance companies saying basically, ‘How dare you treat my patient this way?’ But that’s not medical facts,” says Scherzer. Letters should include: the specifics of the patient’s condition; why the requested coverage must be delivered as prescribed; why it can’t be done another way; and the costs of a worst-case scenario.

If the patient has communication difficulties, doctors should indicate that as another reason skilled nursing is medically necessary. “Patients can’t always self-report symptoms, side effects, skin breakdown, etc.,” says Scherzer. “Even if family members can learn (to give care), many patients are more safely cared for if someone with more training is observing them.”

You can offer to help your doctor write the letter, and to mail it once it’s signed. Letters never should state the service would be beneficial for the caregiver as well. Send a copy to the state insurance commission and indicate that this has been done.

Document Everything: In arguing for nursing care, Wolf and Scherzer noted that a ventilator failure or mucous plug could happen at any time and lead to death without prompt attention. They documented instances of ventilator failures in the presence of both a skilled nurse and unskilled aides and family members.

They also documented Wolf’s declining communication abilities, requiring a nurse to assess her condition. “Dated notes about your condition or equipment failures, written by a doctor, nurse, aide and family members, are important in establishing your case,” Wolf advises.

Get a Lawyer: Every insurance company has a defined appeals process and a lawyer isn’t necessary to use it. But if the appeal is denied and the case goes to court, evidence will be limited to what was presented during the appeals process. “That’s why it’s a good idea to have legal help (during appeal) because you’re creating the record that will be considered by the court,” says Scherzer.

A lawyer also knows your rights. For example, Scherzer requested all medical information about Wolf held by her insurer and employer. This information contained evidence that nursing care was improperly denied. “I didn’t know I had the right to access this information for my appeal,” says Wolf. “A consultation with a lawyer who specializes in health insurance is valuable. It’s not necessary to be near your lawyer. We did everything by e-mail and fax.”

If you can’t afford an attorney, Scherzer suggests consulting with a social worker or health advocate about your options. Check with your state insurance commission to see if an external review agency is available to review your dispute.

Wolf can be reached at cgwolf@optonline.net. New Yorkers for Accessible Health Coverage is found at www.cidny.org

Resources for Fighting Insurance Denials A Consumer Guide to Handling Disputes with Your Employer or Private Health Plan, by the Kaiser Family Foundation and the Center for Consumers Health Choices at Consumers Union. This free online guide helps consumers navigate their health plan's internal grievance procedure and external review programs, with information for specific states. Visti www.kff.org (publications) or call (650) 854-9400 and ask for the Publications Department.

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Keys to Safety

by Alyssa Quintero

For five decades, medical alert bracelets and pendants have provided emergency responders with vital medical information. These simple devices speak to emergency personnel so they can treat people for pre-existing conditions and avoid medical errors.

Rick McClure, Fire Captain/Paramedic for the Los Angeles Fire Department’s Emergency Medical Services section, explained, “Those are items that we’d look for right away at the scene. Medical alert bracelets give us a reference point when doing the evaluation, and those are key signals ...”

Medical ID bracelets are the standard in “speaking” for a person in an emergency. But more technologically sophisticated medical alert devices and emergency response systems also offer added safety, peace of mind and independence for people with disabilities, including those with ALS.

Here’s a glimpse at some that might fit your needs…

Alert Sentry Emergency System

Alert Sentry Emergency System
(877) 253-7899
www.alertsentry.org
One-time activation fee - $19.95
Monthly fee - $29.95
The push of a button on either the portable, waterproof pendant or the main unit calls for emergency assistance. The system, which features a hands-free function, works with your existing telephone line. The response includes a call to local fire or police, or to a family member or friend. If the call center operator can’t get a verbal response from you, the operator calls for help according to your prearranged plan. Operators also convey general information about your medical condition to emergency personnel.

Fred 911
(888) 373-3911
www.FRED911.com
Retail price - $229.95 (MDA clients only)
Fred 911 is a fast-response emergency dialer that features a remote control with a large red button and automatically dials 911. The dialer plugs into any standard phone jack, and the remote control pendant activates the dialer from up to 150 feet away. You can talk “hands-free” to the dispatcher from up to 60 feet away from the dialer.

If you can’t speak or are more than 60 feet away from the base unit, you can still press the button to call for emergency assistance. NeoGenesis Marketing representatives suggest that you check with your 911 call center for details about local response procedures.

Lifeline
(800) 380-3111
www.lifelinesys.com
Installation fee - $100; Monthly fee - $45
Lifeline’s Personal Help Button pendant works with either the Lifeline CarePartner Telephone or the CarePartner Basic Unit, and can be used anywhere in or around your home. Once you push the Help Button, the Basic Unit or Telephone is activated and dials the Lifeline Response Center. Response personnel who have instant access to your profile will contact you. If you can’t answer, Lifeline will send help.

The Basic Unit works with your existing telephone and offers 24-hour personal response, with two-way voice communication. For added safety, the unit features a monthly test call reminder and a 15-hour battery backup. The telephone unit has programmable reminders. And, remote call answering allows you to answer the phone by pressing the Personal Help Button.

For people with ALS who can’t speak or can’t press the help button, Lifeline offers assistive devices, for example, a sip-and-puff device that will activate the system or answer incoming calls with the remote answer feature and speakerphone. The system can be mounted to a wheelchair, and you can purchase the assistive device and transmitter box for $195.

MedicAlert E-HealthKEY
(888) 633-4298
www.medicalert.org
Retail price - $49.95
In addition to gaining access to the 24-hour Emergency Response Center,
MedicAlert members may purchase the E-HealthKEY, a USB key that contains your complete medical history to help track and manage your health. Membership costs $35 for the first year and $20 each year after. You can retrieve and update information, which is then transmitted to MedicAlert’s information repository via the Internet. The software is built into the key and requires Windows XP.

A password can prevent others from going beyond the emergency screen, which contains your member identification number, name, call center phone number, and lists of medical conditions, allergies, medications and medical devices. If the key is lost, MedicAlert will disengage the device.

MedicTag
(866) 246-2247
www.medictag.com
Retail price - $39.95
MedicTag stores all of your emergency information — including existing conditions, medications and emergency contacts — and the USB key fits on your key ring. You simply plug the tag into a computer USB port, and access, print, or update and save your personal medical information. The device requires Windows and a compatible word processing program.

Ideally, an emergency responder with a laptop could use the device. MedicTag recommends against installing a password for the file because it would prevent emergency responders or medical personnel from unlocking the document. When the medical file is opened, a yellow block area appears on-screen and flashes the patient’s most critical medical information.

MyVoice ID

MyVoice ID
(866) 667-5768
www.myvoiceid.org
Retail price - $29.95
You can store emergency contact information and record audio medical information on a pager-sized device. A pushbutton gives emergency responders and medical personnel access to a voice recording with 60 to 80 words of information, including your blood type, allergies, medical conditions, medications, doctor’s name and phone number, and emergency contacts. It also comes with a medical card for printed health information and emergency contacts. You can update and re-record your information at any time.

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