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Nov 5, 2009
Lost a loved one
Do you really have ALS?

A diagnosis of ALS or any other motor neuron disease (PLS, PMA, PBP, MMN) is made “by exclusion” (no higher probability presents itself) and by published criteria, most critically symptoms and muscle/nerve tests.

Doctors may legitimately differ on whether you have “definite,” “probable” or “possible” ALS, or even something else, yet certain benefits like Medicare, Social Security, private disability and State benefits can all rest on what that diagnosis is. And, for your own peace of mind, you want a second set of experienced eyes on all the tests and symptoms you have had.

Ideally, seek a second opinion at the ALS center where you would plan to receive initial care if the diagnosis is confirmed. That way, if you decide you like the center, you can continue there, and, if not, you can look further.

There is no financial benefit to the center for confirming or disputing your diagnosis; their reimbursement is the same for all the tests and examinations they do, and they can be sued if they incorrectly diagnose you, same as any other clinic.

Check the academic medical centers in your area (universities with medical schools), and with the MDA and/or ALSA, both of which have online lists. To find the closest center, or a few options, see this map (zoom in to see your region). It is OK to call more than one and go where you can get a second opinion first. You may also pick up cues about where you’d rather be from speaking with staff on the phone. Of course, you can also ask to be notified if a cancellation opens up an earlier appointment.

Centers may be certified by either the Muscular Dystrophy Association (MDA) or ALS Association (ALSA). This relates to their supplemental funding only and does not imply better or worse staff, equipment or reputation. For example, Johns Hopkins is an MDA center but the Mayo Clinic is an ALSA center.

Preparing for the second opinion appointment

First, obtain and transfer complete records from the office(s) where you have been recently, especially the one who diagnosed you. The center may volunteer to obtain these, but double-check a couple of days before the appointment that they have been received or are available on line. Normally, the other office is accommodating in forwarding or providing records for a second opinion for this purpose. If your records are electronic, you are able by law to get them in that same form.

Most centers use the Cerner or Epic portals and you can go into your account with one institution to link your records there to another. If available, that is the fastest way to share records.

Never give up your only copies of any records, no matter how safe they’re assured to be. Get or burn duplicate film of any imaging studies (X-ray, CT, MRI), make your own copies before you bring a set to anyone, even if you are assured “I’ll bring these right back.” Academic medical centers are legendary for losing materials with which they are provided.

Since most ALS centers are in a health system with an EMR (electronic medical record), you should sign up for an account on the system’s portal (ask how when you visit). Though most provider portals as yet do not display the complete medical record for patients to view on line, in the United States, you are legally entitled to a complete or partial electronic record at any time, requiring that you pay only “labor costs” of copying the information onto disk or into a soft copy.

Bring and store

So records and information to bring or make sure the center receives includes:
• Possible diagnoses that you want to make sure are excluded [in your own notebook, separate from your records]. Wait until you are examined and tested before going through this list with the doctor. S/he can easily skim your list.

• Brief chronology about how/when your symptoms began/progressed, and a list of current medications and major conditions (not the place for chronic toenail fungus or childhood fractures). This should all be on one page. Try to provide concrete examples, e.g. not “felt weak,” but “has been unable to use the bath tub since June, 2015 due to weakness on standing.”

• Reports and tracings (squiggly lines and tables of numbers) on paper or in a soft copy document) for EMGs and nerve conduction testing

• Labs from the last two years, separately in date order. Imaging reports and CDs or DVDs with copies of the scans themselves, also separately, but going back further than 2y if you have them. Chart notes from the appointment(s) where the symptoms that were diagnosed as ALS have been evaluated.

**Bring items in a file folder, rubber banded if needed, never hole-punched or otherwise defaced.**

To expedite using and transferring your medical history, start your own PHR, a personal health record. You can scan documents that you have on paper and add them as well. The PHR with best marks for keeping your personal data private, only sending your record to those you request, is NoMoreClipboard, and it’s free. If you’d prefer, you can also download a paper form at MyPHR.

Either way, give a regularly-updated copy to all your docs, and a one-page summary of conditions, medical contacts and medications to the person most likely to rush to the ER if something happens to you. Keep forms and a printout of your records summary on your refrigerator, in your glove compartment, wallet, purse and in your desk if you work.

Arriving at the final diagnosis

Often an ALS center will want to do its own EMG and nerve conduction tests, even though you have had them already. That is to make sure that the latest equipment and interpretation methods are available to you. So the “second opinion” process often takes two visits — one to be examined and have tests ordered, one to do the tests and hear results. If multiple tests are ordered, try to time them so you don’t need a third visit to find out results. EMG and nerve testing are done by the center’s own staff, so no delay for results is needed.

Unlike tests like CT scans, where you could be harmed by too much testing (CTs produce radiation), re-doing the EMGs and nerve tests won’t increase any known risks, though as you know already they are uncomfortable. As always, if you’re not sure, ask what a test is ruling out or meant to confirm. Some, which you may feel free to refuse, are for research purposes only (a spinal tap frequently falls into this category, though not always) and will not affect the diagnosis.

Ask questions like, “What differentials [alternative diagnoses] are still on the table?” “How will we rule these out?” “Is there anything significant that doesn’t fit the picture of ALS?”

If the physician acknowledges that s/he is sure it’s ALS, but your case simply doesn’t yet meet the published criteria, ask if, given the absence of a competing differential, s/he feels comfortable coding the encounter as ALS, so as to document the diagnosis for the federal, State and private company benefits to which you may be entitled.

After the appointment, ask for copies of all reports and results (labs, imaging, chart notes) and add them to your folder and/or PHR. Make sure there is a plan of action — a referral to someone else, getting results to you within a specified time, more tests — and that you know what it is — before you leave the office. If the expected follow-up does not occur, reach out yourself.

If you were diagnosed with ALS by a previous clinician, something is wrong with you. You want to know, after the process completes, what the final diagnosis is (if it is “probable” or “provisional,” what will make it definite (sometimes, simply the passage of time with continued progression of symptoms) and what’s to be done. Some diseases are so related to ALS that treatment is pretty similar. Others, like multifocal motor neuropathy, have a different treatment, which should be started as soon as possible.

If you have had two different and incompatible diagnoses, a third opinion is prudent. You might visit a second ALS center, or a neuromuscular disease specialist.

If it’s ALS…the first clinic

Once you are diagnosed, the ALS center will try to set up a “first clinic appointment” and then generally try to see you once every three months or so. Try to make the first appointment a few weeks out from the diagnosis, so it has time to sink in and for you to obtain information, but if you have pressing equipment needs, for example, you will want the first available appointment (which often will be a month or two out anyway).

Most ALS clinics only see patients one or two days per month, so it can take a while to get an appointment. If you feel that you need to be seen sooner than the time slot you are offered, get on the cancellation list and plead your case with the clinic nurse. And remember, if you don’t click with anyone at the first visit, you may have another center to check out. Just like any other clinics, ALS centers differ.

Before each clinic:

• Make sure the clinic has or will have your records if you were treated for ALS manifestations elsewhere

• Bring a list of current changes, questions and concerns that need to be addressed. Often these will be written on a white board when you arrive.

• Plan for and expect a long, hurry-up-and-wait day, meeting a lot of different people. Store contact information for each, but identify your primary contact for the clinic and how you will communicate with that person (phone, e-mail).

• Get some sleep to be at your best to take in and obtain everything that you can. Leave early if you haven’t been to the building before. If you use valet parking, make sure you know their hours and how to retrieve your vehicle if they are closed.

What to bring:

• A notebook or tablet to record notes; also, grab a business card from anyone who has one

• A wrap, blanket or sweater if you get cold at times

• Urinal, special straw/cup, bib, beverages, lunch, snacks; anything you’d need at home during the day to be comfortable. You may not have time or access to a cafeteria or vending.

• Any mobility aids you use when you’re tired, like a cane or walker

• Any catalog item you’re considering if you’d like staff opinion, or an item you’ve bought that you’re not sure about

What happens at clinic?

Most ALS clinics will:

• Offer a social worker (often from the MDA or ALSA) to help you determine eligibility for financial aid and other resources such as CALS respite care

• Refer you to clinical trials for which you may be eligible, though this is less likely to happen without your prompting if your clinic is not one of the trial centers

• Refer you to specialists outside the clinic if/as appropriate. If you have an issue that seems to stump your staff, or you’ve tried everything they have to offer, you have the right to ask if they are aware of anyone with more specific expertise. And, of course, always post a question here at the Forums!

• Measure and track muscle strength, lung capacity, atrophy and visible fasciculations [every visit]

• Work with a DME firm (that will come to your home as well) to get you a power wheelchair that meets your needs and can suggest new parts or adjustments as ALS progresses. Power wheelchair orders take a long time, so when a PALS begins to lose the ability to stand or walk, it is time to start the process (as well as to look into lifts).

• Advise on dietary adjustments to address decreased ability to chew, drink and swallow

• Recommend on timing and referring you to radiology for a feeding tube

• Track your cognitive and emotional status, with advice to you and your caregiver if/as signs of dementia or other significant impairment are detected. If a PALS finds this distressing, like any other aspect of clinic, you may ask that this not be a part of future appointments

• Write a prescription for BiPAP when you need it (that will be filled by a DME, or you can purchase your own). Same for a suction machine, CoughAssist, oscillation vest [not all of these are needed by all patients, but BiPAP generally is at some point].

• If/as desired, address questions about the option for a tracheostomy (invasive ventilation) to extend life when BiPAP is no longer sufficient

• Advise on timing and options for speech generation if/when you become unable to speak

• Write an order for home health care when it becomes difficult for you to go out (usually physical and occupational therapy to evaluate equipment needs, start an exercise regimen that caregivers continue). Some agencies also offer a social worker for occasional visits. Later, the same agency may offer palliative care or hospice nursing, or you may need a different agency for that. Note: in most areas, there are multiple home health agencies. If you don’t like the first one your center recommends, gather referrals and try another. The same may be true for DME providers, even if your clinic recommends the one(s) they like.

• Write an order for other equipment that you may seek Medicare and/or other insurance reimbursement for, such as a Hoyer-type lift and a hospital bed

• Write rx and recommend OTC and lifestyle changes that help with other symptoms, such as constipation, mucus, etc., and later, to help with air hunger

• Advise on and write an order for hospice care, if you choose it

Though this is a long list, it is still up to you to bring up issues as they arise. Don’t wait for someone to ask. ALS is a complex disease and your clinical team members don’t know when you will experience what.

However, clinic staff are often familiar with resources in the community with more ALS experience than others, so if they suggest some kind of assistance, e.g. “You should have an AFO for your foot drop,” ask, “Where would you recommend I go to get a personalized orthosis?” and try to follow the advice, even if it is further from home. Sometimes providers can come to the clinic so you do not need to make a separate trip. It doesn't hurt to ask!

What about more routine medical issues?

Don’t think of your ALS clinic as your medical home. It’s likely to be too far and too difficult to get into for that to work, even if it were appropriate to get all your care there, which it’s not. Neurologists, physiatrists and P/OTs aren’t the best resources for non-ALS-related health issues. But you still need to address something like a possible infection promptly, because your body is less able to deal with infection when it is relatively immobile.

Given the dangers of respiratory illness, especially if you are immobile and/or if your breathing is already compromised, it is wise to avoid from ERs and urgent care centers if possible. Such facilities typically have little experience with ALS, or time to consider all your risks, so their handling and protocols could expose you to other harms, such as dislocated joints.

If you like your PCP or primary clinic, ask about options you may not have used, like e-mail, video visits (telehealth), phone conversations, for advice and rx when an in-person visit is not needed.

Later in the disease, if your PCP is not as accessible as you would like (check if they do video; many now do), many communities have geriatric nurse practitioners, who have Master's degrees, or even physicians who make house calls. There are also “on call” services in some areas for house calls and certainly for telemedicine, but it is best to see the same people as consistently as possible since your medical situation is more complicated than others’.

In a pinch, though, there are many 24/7 telemedicine networks, so if you need to "see someone" at 3 am, there is nothing wrong with using 3rd party telehealth to do so.

After the first clinic

Visit the clinic as often you, literally, need to. There is no magic about once a quarter. Sometimes you may feel that you need to get in earlier than scheduled, and sometimes less often. Also ping your clinic when you need something, which often does not require a visit.

Physicians at ALS centers have different philosophies and training around discussing prognosis and progression — like how long you will live and with what symptoms. Some routinely share their opinions. Others will do so only after you’ve asked a couple of times. Some will try to remain noncommittal throughout your disease.

Share early and often how much you want to know, and you are more likely to understand what you need when you need it. Some physicians need to hear, “I understand that this is not a perfect science…I am looking for your best estimate…Based on your experience, do you think it would be a good idea to move up my daughter’s wedding that is planned for next June?”

Summing up

You may have a fatal disease, but with ALS, you are still a health care “customer;" your providers work for you. So do your due diligence, monitor the Forums, and let your resources know if/how you can best make use of them, within the limits of your disease.
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