So how’s your ALS?

RT: “So how’s your ALS?”

Me: “ALS is always shitty.”

RT: “But how’s your progression? Is it slow? Did they say?”

*(RT stands for Respiratory Therapist)

I don’t know fully how to explain how I feel about this exchange with an RT in my home this morning, or how to fully explain all the things that are wrong with it. I am lucky to meet and have the caregiving of many exceptional and compassionate healthcare professionals. But the above is an example of a type that I encounter frequently and which I have grown to loathe.

In the beginning it was easy to be forgiving, to overlook unintentional insensitivity, well intended but misguided advice, kind ignorance, and plain human curiosity.

The curiosity bothers me more and more over time, as I begin to see healthcare providers as using their professional position to satisfy their personal curiosity. I am frequently asked, in that authoritative doctor or nurse (or even dentist) voice, how my ALS symptoms started. This is in a context in which is it totally irrelevant and has no bearing on what the provider is seeing me for. I have been at the ER for a migraine, or the dentist for a cleaning, or at one of my children’s healthcare appointments, and I am asked this, again and again. It is not part of their job in that moment, it is just curiosity. I feel there is a subtle abuse of power there, though the individual is often sympathetic and well intentioned. But have any of them ever thought far enough to realize maybe they are causing me pain to satisfy their morbid curiosity? That maybe it’s hard for me to tell my story again and again?

This morning an RT I’ve met only one other time in my life, asked me casually “how my ALS is doing,” and prodded about my disease progression. As if asking how quickly my death approaches is on par with discussing the chance of rain. This is a person whose only job is to periodically check that my non-invasive ventilator is working and that I have enough supplies.

This same RT tends to speak to me in that voice specially reserved for four-year-olds and, apparently, the very infirm.

The nurse who flushes my port once a month did not know ALS was degenerative and ultimately terminal. After several awkward conversations where she was describing “when you’re better…” and “hope you’ve got some strength back next month!” whilst I tried to respond vaguely, I finally had to explain to her that I cannot recover the strength I’ve lost and I will, unfortunately, not get better. This inevitably led to me comforting her, so when my husband or I encounter this with a professional we won’t see regularly we don’t bother explaining. This happens a lot, despite that these are people involved in my care. But they aren’t ALS people. They serve individuals with all different diagnoses, so I don’t blame them for not knowing all about mine. That doesn’t mean I have endless patience though.

Some providers insist on trying to have a friendship with me. Sometimes I just want them to come, do what they need to do, and leave. Keep it professional. I don’t want to be everyone’s friend. Some nurses I “click” with and we do develop a natural friendship, but I have others that I don’t feel that with, and yet they push me hard for personal chat and I find it really invasive. To be fair, I’m that person who hates going to the hairdresser because they do the same thing, so maybe I’m just too antisocial. But when I put out those “I don’t want to talk” vibes and they push on anyway, I feel like they’re again taking advantage of that bit of power they have where I’m the patient and I’m supposed to answer their questions and do as I’m told. I get angry and resentful.

Sometimes I really think ALS just brings out my inner bitch in a big way. I find myself angry at everyone. Feeling powerless makes you want to lash out. But I think I have some legitimate gripes about some of the care providers I interact with. I wish they understood what’s it’s like to be on the patient end of the relationship.

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Sleeping Beside my Angel

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Reza in bed with a sleeping Daddy.  Unfortunately, since I’m always the one with the camera, there are no pictures of me and him snuggling in bed!

Reza has slept every single night of his nearly 6 years beside at least one, but most usually both, of his parents.  He doesn’t always do much sleeping–I’ve written plenty about that–but when he does sleep, it’s cuddled in our bed.  (In my head I’m hearing Goldsmith crooning I don’t always sleep, but when I do, I prefer to cosleep…)

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While I think this has had an incredibly positive affect on Reza, it also has been a profound source of comfort and connection for me too over the years.  He always sleeps between my husband and me.  Occasionally he would roll to my husband’s side, so when my husband came to bed he’d sleep next to me with Reza on the far side.  In my sleep I’d reach for my son and he wasn’t there and I would panic!  So now my husband always moves him into the middle if he’s rolled too far.  It’s hard to explain, but I spent more than three years breastfeeding him through the night beside me, and now it’s been almost another three years just cuddling him.  At this point the association of sleeping next to him and a sense of peace and wellbeing is so strong.  I know he will need his own bed eventually, but I want to savor this special time while it lasts.

Gradually though, ALS has been elbowing itself in between us.  It started with little things.  It’s hard for me to grip the blanket and pull it over Reza and myself.  So he began rolling closer to his Dad, since he knew Dad could pull the blanket over him the way he likes.  My sweet husband solved that by tucking us both in under the blanket together.  Then I started using a splint to keep my fingers from curling at night.  This lost me the ability to use my left hand to rub his back or hold him.

More recently, I began needing to use a non-invasive ventilator (called a BiPAP) during the night to help me breathe while I’m lying down.  You wear a mask with it, and mine is a full face mask.  I’m mostly stuck on my back.  With the mask on it’s very hard to side-lay while wearing it.  This makes it hard to snuggle my little guy.  I also used to love laying my nose gently against his head, inhaling his sweet scent as I slept.  But now I have this big piece of silicon covering my nose and mouth.  I miss side lying with my arm across him, my nose buried in his hair.  But I still get to feel his warmth beside me, and now he reaches for me, holds my wrist gently in his little grip.

We did have one cute, funny thing with the ventilator.  One night, I was laying in bed and having trouble falling asleep.  As my husband and son drifted to sleep beside me, I noticed that both of them had synced their breaths with the ventilator breaths!  It creates a very soothing ambient noise, and it seems no one can help but sync their own breathing to it along with me!

Lately we’ve been discussing bed solutions.  Our mattress is on the floor with no frame.  Originally this was necessary because Reza would fall off a regular bed in his sleep and hurt himself.  But now it’s hard for me to get in and out of because it’s so low.  However I am not able to climb up into and down from regular bed frame heights.  I’m tiny myself, and with my weakness I can’t do it.  So one option is to eventually consider a hospital bed that would be able to go up and down as needed, and we could adjust elevation of my upper body or feet etc as needed for best positioning.  But if I go that route I will have to give up sleeping with my sweet angel, and my amazing husband.  And I don’t think I’m ready for that.

How to Die

My entire adult life to-date, when I approached a new life stage that I was unsure of, I would find books to advise and guide me through it.  Often, I sought out multiple perspectives on the matter that allowed me to find my way.  Books on marriage, pregnancy, breastfeeding, parenting, and autism still scatter throughout our bookshelves.  So naturally, as I contemplate my own death in terms of my ALS diagnosis, I have been looking for books on how to die.  How do I make the most of whatever time I have left while simultaneously coping with the grief and fear that accompany this process?  How do I find spiritual peace in coming to terms with my end?  How do I manage my time, now that it has become so definitively finite?  Sometimes so many days and weeks pass in mundane every-day-life activities and I begin to feel terribly anxious that I am just wasting time that I cannot afford to waste.  Yet, it is simply not possible to fill all my days with extraordinary experiences.  The questions go on and on, and I felt certain there must be guide books out there- books on how to die.  Unlike marriage or motherhood, death is a certainty for absolutely every single one of us.  So surely, I thought, there must be many books on this subject.  But I couldn’t find a single one.  Not one.

There are related books– books for those we leave behind- on how to cope with a loved one dying.  There are memoirs by those who are diagnosed with terminal illnesses.  There are books on chronic illness, and books on making medical decisions when faced with a terminal diagnosis.  But I could not find any books specifically on how to die in the non-medical sense.

At first I thought it was because no one has died and come back to write a how-to guide for us.  But then I realized that made no sense.  Most pregnancy books are written by doctors not mothers, most parenting books are written by psychologists or other “experts” not parents, and so on.  The reason professionals tend to write these books is because they have seen hundreds of people go through whatever it is they are writing about, whereas those of us who have been through it ourselves only really have our one particular experience to draw from.  So why hasn’t a doctor or therapist or social worker who has watched hundreds of patients go through their deaths written a book to help guide those of us newly on this final journey?

While it’s in the form of a memoir, I did find a book that I thought would offer me some insight, called The Bright Hour by Nina Riggs.  I think it’s particularly relevant since she was also a mother of young children when she received her terminal diagnosis.  I’m going to start with that and see if it is helpful for me.  But I find it strange and rather culturally telling that there are not really books out there on how to die.

Falls

I use a wheelchair part-time right now.  Which basically means if we are going to be somewhere where I have to stay on my feet for more than 10 minutes I use the wheelchair.  I sometimes feel embarrassed knowing people have seen me walk, but have also seen me in my wheelchair.  I wonder if they think I’m a fraud or something.  Part of the reason I use the wheelchair is because I don’t have the stamina or muscle strength to walk for very long.  But another big part of the reason is falls.

For a lot of people with ALS (hereafter I’ll use the acronym PALS), falls are the first symptom of this disease.  The majority of PALS are limb-onset, and of those, it is more common to start in the legs/feet.  I am bulbar onset, meaning it started in my speech/swallow region.  When I was first diagnosed my legs were totally fine to walk and run.  As the disease has progressed it has slowly been making it’s way down to my legs.  Over the past few months I’ve started having falls.  The problem with falls for PALS is that we have weakness in many parts of our body and that often means that we are unable to maneuver ourselves mid-fall.  It’s more of a rag-doll fall, zero control.  This is so hard to describe to someone who hasn’t experienced it.  Suffice to say, you have no idea how skilled you are at falling until that skill is removed.

I had a fall the other afternoon.  My head smacked against something and opened a small wound that bled like crazy and scared my husband!  Luckily, despite all the blood, it did not need stitches so I am recovering in the comfort of my own home.  Falling is dangerous for PALS, and it’s a big reason to start using the wheelchair, or another mobility aide if one is deemed more appropriate.  Not only are falls dangerous in terms of potential for acute injury, but, for reasons not entirely clear, falls seem to speed up disease progression in PALS.  And that is the absolute worst thing for us.

So the next time you see someone in and out of a wheelchair keep in mind there are many people with all different disabilities who legitimately need a wheelchair but can also sometimes walk.

But in this moment..

A few weeks ago I wrote a post that I titled Everything is Not Okay.  I wrote about my deep fears and anxieties about what is happening to me, and about death.  I wrote about my kids and how this affects them.  I wrote about the day-to-day struggles.  I wrote about choking and laryngospasm episodes where I feel like I’m suffocating to death.  Those moments of thinking “I’m really going to die right here at the dinner table in front of my children,” and the trauma that leaves on my psyche afterward.  I got a lot off my chest, which was good, but I couldn’t bring myself to publish the post.  It sits in my draft pile and it will maybe get deleted, or maybe just sit there unpublished like a private diary entry.

So no, everything is not ok.  Even though when asked I always say “we’re ok,” “we’re fine.”  In general I’m objectively not okay.  I have a degenerative terminal disease.  So no, everything is not okay.  BUT.  But..  But in this moment, I am okay.  Okay enough to be here writing to all of you.  Okay enough to share some things, and keep others to myself.  Okay enough to smile at the birds outside my window.

My experience with the unpublished post made me realize I need to share more.  Not everything- I want to keep my darkest most vulnerable stuff to myself.  But I think if I explained some of the things I experience on a regular basis it would both inform others and help me feel less isolated.  So stay tuned.  I’m going to dedicate the next several posts to these topics.

Colliding Worlds

Sometimes my son’s disability and mine meet in a strange and gravely beautiful way.  I hear him vocalize and I think my sweet boy is gaining his voice while I am losing mine.  There is something poetic about it.  I know rationally that the two events have nothing to do with one another.  But I would gladly give him my voice, go silent forever so that he could speak.  Sometimes it feels like that’s what’s happening.  A precious exchange.  Perhaps that is just a notion my psyche has conjured to protect itself from the horrors that await me with this disease.  If I can fantasize some purpose to my loss, some meaning to it, it won’t frighten me so terribly.

I cannot pick my boy up anymore.  It’s been months now that I cannot lift him.  R does not use any functional speech but communicates in a secret body language with us.  He grabs our arms and places them around his hips, pulls and lifts on his toes in a silent but clear request to be picked up.  When he asks me to hold him I call my husband, and my husband lifts his weight while I hold him in my arms in a pantomime of how I would hold him before I got sick.  I have often wondered if he realizes I can’t lift him, or if he thinks we are just behaving strangely.

A few days ago I sat on the grass watching him play with his dad.  He suddenly ran to me, pulled me to my feet, and positioned me as if to say “don’t move.”  He then ran to his dad, hand led him to me, and then requested I lift him while placing his dad’s hand on himself from behind.  It was clear he did indeed understand that we needed dad’s help for me to hold him.  He effortlessly puppeteered us into position for me to pick him up with my husband lifting his weight.  We did, and he lay his head on my shoulder and melted into me.  The intensity of my emotions hit me like a ton of bricks.  I had forgotten how it felt to truly feel the weight of his form against me, his body soft and warm, the smell of his hair and skin.  We’ve done this before but never so absolutely.  This time he gave himself completely to our strange hug and it felt like before, like when I could truly stand and hold him in my arms.  He lifted his head from my shoulder and stared deeply into my eyes while clutching my shirt in his little fist.  This kind of eye contact is so rare and when I get it from him it feels like the most precious priceless gift.  Like the clouds have parted and we are bathed in warm angelic light for a few unearthly moments.

Sometimes I worry that R might lose interest in me as my disease progresses.  That if I can’t speak to him at all, if I can’t use my hands and arms to soothe and tickle and hug and hold him, that he will find he no longer needs me.  But moments like R’s request to be held soothe my fears.  I will always be his mother, and our bond can survive this, it can.

ALS: my story

I’ve been meaning to write about this for a while.  While this blog has focused on my Autistic child, at its core, it has been a tool for me to process my emotions about my son’s autism and my own parenting journey with him.  But my ALS diagnosis has eclipsed just about everything else in my life, autism journey included.  Lately I have been feeling the urge to write out my story, my feelings, my reflections, my fears.  So I am going to do it here and I guess this blog won’t just be about autism or about my son anymore.  Maybe it is better for it to evolve into something that captures more of me and my family than just the autism facet.  So here goes.

Let me begin by saying I did not have ALS remotely on my radar until January 23rd, 2017.  I was given my first diagnosis of Motor Neuron Disease (which is the family of diseases ALS belongs to) on March 3rd, just five weeks later.  Three weeks after that I received my official ALS diagnosis.  So I went from having no clue about ALS to being diagnosed with it in a matter of weeks.  But the first signs of the disease,  though I didn’t recognize them as such, began over a year prior.  My best estimate as to the start of the first symptoms is 15 months prior to diagnosis, around December 2015.

I should also preface with a brief explanation of what ALS is.  Prior to learning I might have it, the only thing I knew about it was that it was what Stephen Hawking has.  I knew it caused him to be paralyzed and use a high tech eye-gaze AAC to communicate.  Because my own child is non-speaking, I’m very pro-AAC, and so my overall impression of ALS was “they use an awesome type of AAC, how cool is that.”  Stephen Hawking is so old and has had ALS for decades, so it never occurred to me that it was a terminal illness.  Hawking, as it turns out, is the longest lived person with ALS, defying the harrowing statistic that 90% of individuals with ALS die within 3-5 years of diagnosis.

ALS, which stands for Amyotrophic Lateral Sclerosis, is a neuromuscular disease affecting the skeletal muscles.  The brain is no longer able to communicate with the muscles, resulting in progressive weakness leading to total paralysis.  This leaves a person unable to move, speak, or eat by mouth.  Eventually it can affect the breathing muscles, resulting in respiratory failure.

It all started with weird muscle cramps.  I say weird because they occurred in unusual muscles, like my tongue and individual fingers, but were notably absent in muscles you expect to have cramp, like your legs.  When I asked my doctor about it she was completely unconcerned and suggested I was dehydrated.  I remember thinking “Shouldn’t that give me a charlie horse in my leg, not a tongue cramp?  Who knew your tongue even could cramp?!”

Around the same time I began experiencing bouts of uncontrollable, inappropriate laughter.  It was very embarrassing and I worried that there was something psychologically wrong with me.  I wondered if I was having stress-induced hysteria, and wondered if it meant this was some kind of breakdown.  I also remember thinking it really reminded me of my grandfather after his stroke- he would cry or laugh inappropriately in the same kind of way it was happening to me.  I later learned that there was nothing “wrong” with me, not mentally anyway, and this was another neurological symptom associated with ALS and other neurological problems or disorders.  It is called Pseudobulbar Affect (PBA) or Emotional Liability.  So it turns out I  was right to see the resemblance between what was happening to me and what I’d witnessed after my grandfather’s stroke.  About 50% of people who have suffered a stroke will experience PBA, and I now see that my grandfather must have been one of them.

In the spring of 2016 I had a bad case of the flu.  After I got better, a nasal, congested sounding voice remained.  But I was no longer congested.  Weeks passed and it still remained.  I did have some other sinus symptoms, like a constant postnasal drip, and a strange inability to close off my sense of smell without manually pinching my nostrils closed.  I thought I had some kind of deep congestion, back behind my nose since I knew my nose was clear.  It lasted so long that I thought it must be a sinus infection.  I went to my PCP who looked in my nose and blew me off completely declaring there was nothing wrong.  Since my insurance doesn’t require referrals, I then went straight to an ENT.  The ENT scoped me and found no visible infection, but did see the abnormal degree of postnasal drip and some inflammation.  He prescribed a round of antibiotics and steroids.  This failed to change anything and a second round was offered.  After that they did a CT scan.  I eventually had a small, endoscopic surgical procedure to help my sinuses drain more efficiently.  None of this changed the symptoms I was having.  These symptoms would all turn out to be caused by muscle weakness compromising the structures in my ear-nose-and-throat area.  I was embarrassed by my nasal voice and always told people I was getting over a cold or allergies, even though it wasn’t true.  Along with these speech and sinus issues, I also noticed I was choking on liquids and saliva a lot more than normal.  It seemed like such an odd, random thing, that I never even mentioned it to my doctor.

At the same time as my voice/speech changes and sinus issues, I noticed weakness in my left hand, which is my dominant hand.  I was taking sign language classes and noticed I could no longer form many signs, especially fingerspelling.  I also noticed that I was having trouble with my pincer grasp, and found it hard to open packaging, unscrew tops, and compress buttons.  I continued to have frequent muscle cramps in the fingers and thumb of that hand, often brought on by attempts to use those muscles.  Though I connected these cramps to the hand weakness, at the time I didn’t connect the tongue and jaw cramps to my speech and sinus issues.  It didn’t occur to me whatsoever that the speech and sinus stuff could remotely be related to my hand weakness.  The hand weakness was worsening at a very gradual rate, and was not painful, and for those reasons I largely dismissed it.  When I went for my annual well visit with my PCP that summer, the doctor was startled by my brisk reflexes.  I had never heard that before but quickly forgot about it.  By late fall/early winter my hand weakness was still persisting and progressing, though still painless.  I actually told my husband, very worriedly, that I thought it might be something neurological.  He suggested it could be a pinched nerve.  I went to my PCP about it and she examined it for signs of carpel tunnel, which she said there was no evidence of.  She seemed very unconcerned and suggested it might be tendonitis.  She told me if it didn’t resolve in a few more weeks to call the Orthopedic Hand Clinic for an appointment.

In late January of 2017 I finally got around to going in to the Hand Clinic.  They examined my hand thoroughly and did many types of strength tests on the hand and fingers.  They took x-rays.  They asked me repeatedly if I had any pain or numbness (no to both every time).  At the end, the doctor told me he was pretty sure there was a neurological problem, and that he was ordering an EMG to rule out a pinched nerve or other problem originating in the hand or arm as opposed to the brain.

The EMG was scheduled for just a few days later.  I spent a lot of time googling now that I knew I most likely had a neurological problem.  I randomly came across ALS and saw the early symptoms list.  I remember that moment so clearly.  I was standing in line at Staples, bored and searching on my phone.  I scanned the list– painless, progressive weakness, slurred speech, nasal voice, difficulty swallowing, muscle cramps, muscle twitching, inappropriate laughter or crying, hyper reflexes, spasticity…  When I saw that list I was overwhelmed with recognition, with this soul-deep knowing.  It was exactly how I felt the first time I came across a list of early signs of autism.  Autism had not been on my radar at all and I had known very little about it.  But the very first time I came across an early signs list while searching to understand R’s atypical development, there was an instant, deep knowing that this was IT.  As I stood in the Staples line and scrolled down to read more, I read that “ALS is universally fatal…there is no cure for ALS.” I felt my stomach drop, and when it came back it was filled with lead.  I remember turning my screen off and putting my phone away and thinking there was no way.  Reminding myself that Dr. Google is notorious for making it seem like your mundane minor symptoms might secretly be a rare and deadly disease.  But a small voice told me that I wasn’t one of those people.  I’m a damn good googler and I couldn’t think of a time when I had mistakenly thought my or my child’s symptoms sounded like they were something this serious.  And I had been googling about my hand weakness only, I had not thought to connect my speech and swallow symptoms to the hand weakness, the ALS page provided that connection, which made it seem more eerily true.

By the time I went in for my hand EMG I had read enough about ALS to know that it was:
1) rare in people under age 40 (mean age of diagnosis is 55)
2) more common in men
3) notoriously hard to diagnose due to mimic diseases and the fact that there is no single “test” that will show it

This information gave me a measure of relief.  Statistically it was so unlikely for a woman my age (31 years old) to get ALS.  It was more likely that a different cause for my symptoms would be discovered.  So I went in for my EMG feeling cautiously optimistic.

When I arrived, the doctor, a neurophysiologist, began asking me about my hand weakness, which I described and showed him.  He examined my hand and then did a quick neurological exam, commenting on my hyper reflexes.  He then began flicking my fingers.  Whatever he saw concerned him, and he flicked them several times more.  I later learned that he was observing that I had Hoffman reflex, an abnormal reflex not usually present in healthy adults.  This, like the hyperreflexia, can be a sign of upper motor neuron degeneration in ALS.  He then performed the EMG and NCV on my hand and arm.  He said that he saw abnormalities and that whatever was wrong was definitely neurological and not in the arm or hand itself.  He then asked about my voice and speech and I told him about my other symptoms.  Though I pressed him, he refused to speculate as to a diagnosis, but told me he was ordering an MRI of the brain and neck and would have them rush it so we could get answers more quickly.

When I got home I did some more research online and discovered that if my MRI was clear, ALS was still on the table, but that if my MRI showed any lesions I might have Multiple Sclerosis or something else, and ALS would be unlikely at that point.

My MRI was clear, but the neurophysiologist referred me to a neurologist specializing in MS anyway.  The neurologist did a comprehensive neurological exam and medical history.  She showed me that I had atrophy (muscle wasting/shrinkage) of my thumb muscle.  She examined my tongue and immediately called over her resident to show her my tongue fasiculations (muscle twitching that occurs in ALS).  At the end she told me that she was concerned that I may have a Motor Neuron Disease like ALS.  She told me she was ordering a more comprehensive EMG, lots of bloodwork, and referring me to the Neuromuscular Clinic.

The comprehensive EMG appointment took 4 hours.  I had continued to do research online leading up to this appointment, so going in, I already knew a few things.  The appointment, like my hand EMG, would consist of two parts, the Nerve Conduction Study, and the needle EMG (electromyography).  In ALS, the NCV portion is usually normal, and it is the needle EMG where abnormalities would show up.  For the NCV the doctor places electrodes over the muscles being tested and sends brief bursts of electricity into the muscle.  The electric conduction is then measured in terms of velocity.  So basically the doctor shocks you over and over.  Fun times, but the fun doesn’t stop there.  Part two, the needle EMG, involves the doctor inserting a needle into the muscle which takes data both while the muscle is still and then again while you flex it.  This is repeated for each muscle being tested.  For me, they did my thumb, two or three arm spots, two lower leg spots, one upper leg spot, two spots on my back, and my tongue.  Everyone I’ve told about this thinks tongue must have been the worst, but to be honest, thumb was the most painful, closely followed by one of the upper arm muscles they did.  The doctor who did the test was a Neuromuscular Neurologist.  She also did a neurological exam and had me repeat the whole story about my symptoms, my previous doctors and tests, and medical history.  At the end of everything she told me that the most likely diagnosis based on my exams and the EMG was ALS.  She gave me a provisional diagnosis of Motor Neuron Disease, ordered more bloodwork, ordered breathing tests, started me on an ALS medication (Riluzole), and referred me to the ALS Clinic for an official evaluation and diagnosis.  This was March 3rd, 2017, and it was my initial diagnosis day.

Three weeks later, On March 24th, 2017, I had my first visit to the ALS Clinic.  It was a 5 hour visit.  I was seen by a Pulmonologist experienced with ALS, and a Neurologist specializing in ALS, as well as several other related clinicians that collaborate at the Clinic.  Extensive neurological testing and strength testing were performed, and I was interviewed at length about everything.  Finally the neurologist told me that I did indeed have ALS.  She told me I was considered bulbar-onset (meaning onset in the speech and swallow region).  She told me that while most patients live 3-5 years after diagnosis, for bulbar-onset patients the average is usually about 2 years.  She was extremely thorough, she provided me with tons of resources, medication to treat my PBA, referrals for a 2nd opinion, a swallow study, and an OT evaluation.  She even called me at 8pm that same night to make sure I had the correct referral information for my swallow study.  She was doing all the right things.  But when my husband and I talked about it later, we both confessed we didn’t like her.  When I shared this with a friend I had made online- another woman living with ALS who was also in my state (thank God for the internet right?), she gently suggested that perhaps we were struggling with our feelings about her telling us those statistics (2 years for bulbar onset ALS).  I realized she was right.  I was angry about the doctor saying that.  I felt the doctor should have slanted it positively- should have pointed out that 10% of people with ALS do live longer than 5 years post-diagnosis.  That I could be in that 10%.  I realized it didn’t make sense to reject the doctor because she told me the stats straight up.  I’m better off with her than someone who tells me things in nicer terms but is less competent.  As an aside, I’ve seen her several times since then and I really like her now.  She is an excellent doctor and is kind and compassionate.  I guess it’s just hard to like someone in the initial aftermath of being told such devastating information.

On April 11th, 2017 we went for my 2nd opinion at the MGH ALS Clinic in Boston.  This was technically a 3rd opinion since I’d had my first diagnosis with the neuromuscular neurologist before being referred to the ALS Clinic.  With ALS the standard is to always get a second opinion with an ALS specialist.  This is because it is a hard disease to definitively diagnose and it is such a serious diagnosis that you need to be absolutely certain.  The 2nd opinion appointment was anticlimactic.  The neurologist had reviewed my file already (my file had been sent over ahead of time).  She listened to my story and did a neurological exam and strength tests.  That took about 20 minutes and then she informed us that she agreed with the diagnosis and that I did indeed have ALS.  She went on to discuss research with us- upcoming clinical trials, a new drug about to receive FDA approval (it has since been approved- the first new ALS treatment to receive FDA approval in 2 decades).  She projected an attitude of hope which I appreciated.  We went home.  The diagnosis process was officially over.

I think I’ll save my reactions to all of this for my next post.  I will say my reactions continue to change between states of shock, denial, disbelief, sadness, anger, numbness, neutrality, and hope.  Sometimes several of those at once.  It has been four months since my initial diagnosis, and I still don’t think it has 100% sank in.  But this post is long enough, so I will end here for now.