Connecting all the Pieces

I was recently asked by a friend how Robert’s pulmonary issues are connected to his seizures.

I thought this was a terrific question and one I have been trying to understand ever since his pneumonias started showing up a couple of years ago.

But first, I had to sing “The knee bone is connected to the thigh bone, the thigh bone is connected to the hip bone, the hip bone is connected to . . .” You get the idea.

I couldn’t help myself.

That is the million dollar question. How are the seizures related to his proclivity to develop pneumonia at the drop of a hat?

Robert’s pneumonias have always been classified as aspiration pneumonia. Sometimes, the pneumonia doesn’t even show up on the x-ray which confuses the heck out of the ER personnel. However, a doctor once told me that it is not always possible to diagnose pneumonia by x-ray – it is diagnosed through symptoms.

Robert has the symptoms every time and we are well aware of those symptoms (cough, fever, high pulse, low blood pressure, reduced mobility and “brain fog”).  In Robert’s case, sepsis is usually an unwelcome companion with his pneumonia (and no matter how quickly we recognize the pneumonia, the sepsis sneaks in there).

The pieces are starting to come together with each new specialist Robert sees.  A year ago during one of the hospitalizations, Robert saw an ENT and was diagnosed with dysphagia (a swallowing disorder) which was thought to be causing his aspiration pneumonias.

Great! He was put on a modified diet (called either “chopped” or “mechanical soft”) and thickener was added to all of his drinks.

Two months later he was hospitalized again with pneumonia so this wasn’t the only piece of the puzzle.

Earlier this year, Robert’s pulmonary doctor did a couple of scans of Robert’s lungs and concluded that his lung muscles were weak and there really wasn’t much more to do other than try to manage the symptoms before Robert needed hospitalization. He explained that the lung muscles were weakening much like Robert’s leg muscles and other muscles were weakening and it would just be a matter of time before he couldn’t recover from one of his pneumonias.

I appreciated his frankness and the explanation of what was happening. Since then, Robert developed his typical pneumonia symptoms five times (that’s just this year) and the pulmonary doctor put him on antibiotics each time and we were able to keep him home while he recovered.

In June, however, Robert’s mobility was really taking a hit.  His leg muscles were getting exceptionally weak. He could no longer use a walker and we had to use the wheelchair 100% of the time.

I still didn’t know what was causing this muscle weakness and the decline. The decline seemed to be so rapid that it was head-spinning. It was all my husband and I could do to try to keep up with Robert’s increasing needs.  

Finally, the neurologist told us to go to the hospital.  They weren’t sure what was going on either but suspected a neck compression on the nerve in his spinal column (which would really suck). He needed to be in the hospital to investigate further.

We were fortunate that the neurologist on staff during Robert’s hospital stay was a specialist in movement disorders.  She was easy to talk to but exceptionally brilliant. Robert couldn’t have been in better hands.

She determined that Robert did have a neck compression but it wasn’t severe enough to cause him to lose his mobility. Instead, she diagnosed him with Cumulative Traumatic Encephameylopathy and Parkinsonism and started him on a new medication (Sinemet) which helps manage the symptoms. Combined with physical therapy, Robert’s mobility returned to a level I hadn’t seen in months.  It was such a relief.

I had a question about Parkinsonism which the neurologist answered at Robert’s most recent neurology appointment: it is different than Parkinson’s disease. Parkinsonism is just the symptoms of Parkinson’s disease and not the disease itself.  Parkinsonism can cause muscle weakness (including the lungs), movement disorder (Robert’s muscles were weak but they were rigid), tremors and swallow difficulties.

Things were starting to make sense.

From what I understand from the neurologist, the CTEM and Parkinsonism are caused from brain trauma Robert sustained due to a lifetime of uncontrolled seizures, two brain surgeries and dozens of concussions (caused from falling during a seizure).

This does make more and more sense to me. I think of Muhammad Ali who developed Parkinson’s syndrome due to being bashed in the head multiple times during his boxing career.

The brain can only take so much trauma.

Robert sees many specialists (three neurologists, a pulmonary specialist and not just an ENT but an Otolaryngologist for his swallow disorder) - and don’t ask me to pronounce that type of doctor!

It seems recommendations for treatment and piecing together the puzzle of his illnesses is pretty fluid. Prescriptions change, medication dosage changes, even his diet recommendations change (Robert no longer needs thickener as the doctor thinks that makes swallowing more difficult for him). 

Richard and I just manage the best we can and help Robert understand what is going on with him as much as we can.

We know that Robert’s body doesn't handle illness as well as the rest of us because of the compromised brain function which ends up affecting his mobility and creating sort of a "brain fog." He forgets words, has trouble telling us what he wants to say and has a weaker grip on his pen so has trouble with his word search books. He gets drowsy and slow and the ability to walk becomes a luxury until he recovers. For the same reason, it also takes him a lot longer to recover from any sort of illness.

He is sick again now and on antibiotics. He has trouble walking, is having breathing difficulties and has his brain fog. I am monitoring his vitals closely in case there is an indication he needs to be hospitalized.  

That's my understanding of the inter-related workings of his issues. 

Epilepsy with uncontrolled seizures to brain trauma to Parkinsonism and CTEM to pulmonary problems, dysphagia and mobility issues. 

I may not have all the pieces or understand all of what is happening but the idea that everything that is going on is related and is really ultimately caused by his uncontrolled seizures renews my advocacy to spread awareness about epilepsy.

There is not a lot of research out there about the effects of epilepsy on adults with uncontrolled seizures.  However, I don’t need to learn what happens by reading about it.  We’re living it and I would love nothing more than to stop epilepsy in its tracks.

(Obviously, not everyone will have the same experience as Robert so it would be nice to have more research about the effects of uncontrolled seizures.)

Please help me spread awareness about epilepsy and, if you’re so inclined, check out a few epilepsy organizations that do terrific work researching epilepsy and educating people about it:

CURE Epilepsy

The Epilepsy Foundation

Comfort in a Diagnosis

Robert has declined so quickly this year and had so many different things going on, my head has been spinning.  I just want to find out what’s wrong!

 

Looks like Robert's appetite is back!

Questions have swirled this year: Why is he getting pneumonia so frequently? Do we use antibiotics when he has bronchitis and risk resistance to them or risk the bronchitis turning into pneumonia? (Side question: Why aren’t there any good choices?) Why does he now sometimes have both urinary and bowel incontinence?  Do we blame the antibiotics or is there something more going on?  What’s with the increased seizures? The irritability? The trouble walking? The sleeping 18 hours a day?  The eating half of what he used to?

We got a possible diagnosis of Cervical Disc Disease with Myelopathy at the last neurology appointment.  Boy, that didn’t sound good.  The neurologist needed an MRI to confirm what he suspected but Robert couldn’t tolerate the MRI.  They decided to do an MRI with anesthesia and wrote a referral for that.

Just a few short days after that, Robert was literally unable to move his legs.  Richard (yes, my husband with the severe and chronic back pain) had to lift Robert from his wheelchair to the bed. The day after that, Robert slept and slept and slept.

Neurology suggested we take him to the ER and get a CT scan done and then the hospital could do the MRI with anesthesia. It was a Tuesday afternoon.  How bad could the ER be?

Oh how naïve I am!

It was a very long day.

Almost as soon as Robert was settled in his hospital room he started throwing up and running a fever off and on.  What in the world?!  Day Program informed me the following day that other consumers had been throwing up lately. Great. Robert has a stomach virus on top of everything else.

And, it’s contagious. Thank you very much.

After several days of waiting for the MRI with anesthesia (we had to wait for the stomach virus to subside, then there was the 4^th of July and then a weekend and then a promise of “maybe we’ll do it Monday but for sure Tuesday”).

By late afternoon Tuesday, the neurologist was not hopeful and was as frustrated as the rest of us.  For whatever reason, the hospital does not actually schedule MRIs with anesthesia for people actually admitted to the hospital.  They “squeeze” them in between emergencies and the general public appointments (as in, if I had taken Robert home and then made him an appointment, he probably would have gotten it a lot faster).

The neurologist did what she could and called hospital administrators (the hospital neurologist is not his usual neurologist).  They pulled some strings and we were on our way to the basement of the hospital housing the MRI machine.

It was miles away from his room and I did my best to keep up with the transport team pushing the gurney but I wasn’t about to complain.  We were getting this done and we will be able to actually find out what is wrong!! Woohoo!! 

Until I hear the word “cancelled” on the mikes hanging around the necks of the transport team and they stop cold.

Oh hell no.

We were no more than ten feet from the door leading to the MRI.  This test we have endlessly waited for.  A voice from the room said, “He has hardware in him still.” 

NO HE DOESN’T!

I made my way around the transport people and past the gurney into the MRI room.  Mr. Radiologist, MRI technician and the transport people looked as if they just might call security.

He does not have hardware in him.  The VNS and DBS have been removed.  I was assured by the neurology team at his former hospital – he can have an MRI. 

GET HIM IN THERE!

Mr. Radiologist towered over me but respectfully talked to me, even while I ranted and raved and told him he was flat out wrong.

He insisted there was a piece of the VNS still wrapped around the vagus nerve and that Robert’s nerve could be burned if he had the MRI.

But he’s had an MRI since the equipment was removed! We’ve waited for a freaking week for this test!  This is ridiculous!

All Mr. Radiologist could do was say how sorry he was.  He was not backing down. The transport team was already leaving with Robert, walking back to the room and I just threw up my hands and stormed back right behind them.

As soon as we got to the floor, I looked at the nurse – who was as surprised and disappointed to see us as I was to return – and said, “Call neurology!”

One of the neurologists from Robert’s team showed up within minutes. She was mortified and apologetic and by this time, I was in tears. A moment later, Mr. Radiologist peeked into the room and I dropped my head into my hands. 

I’m sorry I lost it but we need to find out what’s wrong.  We’ve been waiting for a week for this test!  I just can’t take this!

He was very sympathetic but again explained how dangerous it would be for Robert to have the MRI.  I told him I understood and appreciated him coming to the room to further explain his position. 

I get it.  I do.  I just want answers.

The lead neurologist on the team came in a short while later and apologized profusely. She missed the hardware on the CT scan. She should have seen it and didn’t.  She was kicking herself and felt absolutely horrible. She assured me the entire team would learn from this which really was of no comfort to me at that point.

What’s next?

She had a plan and for that, I was grateful. 

She decided the next best test would be a CT scan with myelogram.  

Will this get us a diagnosis?  When can we get this?  Can this happen tomorrow?  It’s already Tuesday night – we have been here a week and Robert is losing function by the minute. 

Please!

According to the neurologist, there were two options: Cervical Disc Disease with Myelopathy or Cumulative Traumatic Encephalopathy. The CT scan would be able to confirm or rule out the Cervical Disc Disease.

She made it happen and the next morning preparations were made for the CT scan.  By noon we were done and after waiting an hour for transport (perhaps the story of the lunatic sister was making the rounds and they were drawing straws for the job), we were back in the room waiting for the diagnosis.

The results were that there was some narrowing of the spinal column in the C5/C6 area but there was enough space left that they were comfortable ruling out Cervical Disc Disease.

The neurologist said she thinks all of Robert’s symptoms and decline are a result of Cumulative Traumatic Encephalopathy.  Since all I had was time at the hospital, I had done research on this so asked if it was CTE or CTEM. 

CTEM? 

I didn’t really mean to play “stump the neurologist.”

The resident on the team went to the computer to look it up: Cumulative Traumatic Encephameylopathy.

The neurologist confirmed it was CTEM, the slightly more aggressive and serious cousin of CTE.

This is not something that can be fixed and it is extremely serious and progressive (sort of like ALS, aka Lou Gehrig’s disease).

But it is a diagnosis and I find great comfort in knowing what we are dealing with.  While we can’t predict exactly how this will progress in Robert, it makes sense.  His lung muscles are getting weaker, he has a swallow disorder and his leg muscles are unable to get moving. His brain is forgetting to tell his muscles to work.

For me, not knowing what is going on or what to expect is worse than knowing. 

For me, there is comfort in a diagnosis.

For Robert, he knows he is going to get physical therapy for a few weeks at a Skilled Nursing Facility. He knows he is getting meals served to him and he’s looking forward to the activities at the SNF. 

That’s all the comfort he needs for now.