Back in June I wrote a blog on pseudobulbar affect (PBA). Many had never heard of this while others
were so thrilled that they were not going crazy and finding out that other
people experienced this also. Like many
other MS patients, I have mentioned this behavior to my neurologist but he just
shrugs and ignores it. I was contacted
by a reader of my blog who asked if I would like to interview a couple who are
dealing with PBA. I was thrilled to be
asked and quickly accepted the offer.
Just for a brief
introduction, David and Arlene Diehl have been married 37 years and live in
East Helena, Montana. They have three
grown children and five grandchildren. David was initially stricken
with Transverse Myelitis in 1991. He
went from normal to paraplegia in less than 5 days. He is a complete T-11 paraplegic from this
attack. Seven years later, in 1998, he
developed MS, and was started on Avonex shortly after that. According to Arlene, in 1998 she noticed
David’s emotional displays and reactions were not normal. She immediately noticed that it was similar
to what she had seen in her father, who had MS for 22 years before passing away
when she
was 17 years old. It was not until 2003 that they learned from David’s
neurologist that what David had was called PBA.
PBA is a neurological, not
psychiatric illness caused by an underlying neurological condition, such as
Multiple Sclerosis, traumatic brain injury, Alzheimer’s disease, Lou Gehrig’s
disease (ALS), Parkinson’s and stroke. Due to a lack of awareness and knowledge
of PBA in the medical community, PBA is often misdiagnosed as depression or
part of the primary neurological disease—when in fact, it’s a separate,
treatable condition. Nearly two million people with an underlying neurological
condition may have PBA, but many go undiagnosed. PBA occurs when certain
neurologic diseases or injuries damage the areas of the brain that control
normal expression of emotion. This damage can disrupt brain signaling, causing
a ’short circuit’ and triggering episodes of involuntary crying or laughing.
These outbursts are not consistent with the patient’s current emotional state,
leaving them to laugh or cry when they don’t find things funny or sad and can
be frequent, severe and disruptive to everyday life. These episodes are often
so disruptive they can interfere with routine activities or cause patients to
avoid social situations altogether. The condition has a tremendous impact on
loved ones and caregivers as well.
Arlene states that in David’s
case, his PBA would manifest in him laughing when trying to have a serious
conversation, or would often laugh far longer than he wanted to. He also would cry over a touching commercial,
all the while knowing that it was not proportionate with the degree of his true
feelings about the event. I asked Arlene when she came to the conclusion that
her husband had a problem and what David’s doctor did. She told me, “I noticed it right away in
1998, when he was diagnosed with MS, most likely because I had seen it in my
father. David’s personality changed and
it was very disruptive to our lives. At one point I told the doctor, “Either he
is going on a treatment, or I am. His
unpredictable laughing and crying episodes were hard on our relationship and I
felt that he/ I needed some help.”
David
tells of being at a funeral where a joke was told, laughing long after, and
feeling embarrassed and trapped because he was seated in the front of the room.
Our children used to say that their dad had days where he was “happy-grumpy” in
other words, he was grumpy, but would be laughing as he was scolding or
complaining about something. This often
led David to feel disrespected. There have been times when David would try to
tell me something sweet or try to apologize for something, and laughter would ensue. At first, I would be upset until I learned to
give grace because I knew that he was having what we later learned was PBA.” I asked Arlene if David was an emotional
person before the PBA and she said no, that he was very controlled emotionally.
I asked Arlene what adjustments
the family has had to make with these emotional changes in David. “We have learned the disconnect in his
emotional reactions is part of the disease”, she told me. “We now know Happy Grumpy describes PBA and
not who David truly is. I have to give
extra grace and consideration, because for David, his ability to handle ‘frustration
has been greatly affected.’” Arlene further stated that they had
previously discussed David’s difficulties with the neurologist (Dr. Dennis
Dietrich) and tried several antidepressants. It was not until his doctor was
involved in a clinical trial that they understood that what David was
experiencing was PBA. David and Arlene noticed similar symptoms in David’s
brother Dan, who died from ALS in 1995. David thought his little brother was
being overly emotional because they were unaware of PBA at that time.
As far as David’s symptoms, Arlene
says that initially David was treated with antidepressants, but they did not
mitigate his laughing and crying episodes. Also, David insisted that he was not
depressed, and she had agreed, but they also knew that they needed
something. Once Nuedexta® was
available, they noticed a definite reduction in his PBA symptoms. Nuedexta has been a very good solution for
David’s PBA. He has not suffered any
side effects from treatment and is pleased to be taking one drug instead of
three to mitigate his laughing and crying episodes. The reduction in symptoms
has been a huge improvement for them. Arlene states that she has seen many
people who appear to display PBA symptoms, people with strokes, ALS, brain
injury and MS. “When I describe the
symptoms to others, nearly all believe they have seen it in someone. I am happy
to tell people there is a very effective option for this condition.” Since
then, they have slowly taken him off of all the antidepressants.
I told the Diehls that most of
us with MS seem to have a hard time coming to the realization that we may need
to have help with other issues that arise. She told me this: “I think that it
is hard to admit that you are struggling with uncontrollable emotional
displays. Often David did not recognize
that he was overreacting. He had to take
our word for it. Also, when you go to
the doctor, your time is taken discussing the physical issues that are affected
and one does not want to say, “Hey—by the way, my emotional displays are not
normal!!” Those who are already struggling with diminished physical well-being
may be reluctant to disclose what may sound like their “head is not screwed on
right.” This can be embarrassing and
discouraging. It also adds another layer of complexity when some PBA symptoms
can appear to disguise themselves with symptoms of depression.”
I asked Arlene what a normal
day is like for them. This was her answer:
“David and I start our day about 7am. I do assist
him with some aspects of his morning routine, but otherwise, he is primarily
independent. I work at our church as an
assistant to our pastoral care pastor and David spends the morning at the ranch
office, driving down to the ranch on his ATV.
He also rides a hand cycle most days and puts around 800 – 1000 miles a
year on that bike. He returns home on his ATV and is able to rest most
afternoons. His time is spent marketing
the grain for the ranch operation and he also helps to find equipment
online. We are both very active
in our church and know that our lives are made better by our personal faith.
Without our faith, the years we have struggled against David’s sickness would
likely have destroyed our marriage. All
the drugs in the world can’t fix all that his health issues have taken--our church life and faith in Christ have helped to make up
the difference in many regards.”
About NUEDEXTA
NUEDEXTA®
is the first and only FDA-approved treatment for pseudobulbar affect (PBA).
NUEDEXTA is an innovative combination of two well-characterized components;
dextromethorphan hydrobromide (20 mg), the ingredient active in the central
nervous system, and quinidine sulfate (10 mg), a metabolic inhibitor enabling
therapeutic dextromethorphan concentrations. NUEDEXTA acts on sigma-1 and NMDA
receptors in the brain, although the mechanism by which NUEDEXTA exerts
therapeutic effects in patients with PBA is unknown.
NUEDEXTA is
indicated for the treatment of pseudobulbar affect (PBA). PBA occurs secondary
to a variety of otherwise unrelated neurological conditions, and is
characterized by involuntary, sudden, and frequent episodes of laughing and/or
crying. PBA episodes typically occur out of proportion or incongruent to the
patient's underlying emotional state. Studies to support the effectiveness of
NUEDEXTA were performed in patients with amyotrophic lateral sclerosis (ALS)
and multiple sclerosis (MS). NUEDEXTA has not been shown to be safe and
effective in other types of emotional lability that can commonly occur, for
example, in Alzheimer's disease and other dementias. The primary outcome
measure, laughing and crying episodes, was significantly lower in the NUEDEXTA
arm compared to placebo. The secondary outcome measure, the Center for
Neurologic Studies Lability Scale (CNS-LS), demonstrated a significantly
greater mean decrease in CNS-LS score from baseline for the NUEDEXTA arm
compared to placebo.
NUEDEXTA
Important Safety Information
NUEDEXTA can
interact with other medications causing significant changes in blood levels of
those medications and/or NUEDEXTA. NUEDEXTA is contraindicated in patients
receiving drugs that both prolong QT interval and are metabolized by CYP2D6
(e.g., thioridazine and pimozide) and should not be used concomitantly with
other drugs containing quinidine, quinine, or mefloquine. NUEDEXTA is
contraindicated in patients taking monoamine oxidase inhibitors (MAOIs) or in
patients who have taken MAOIs within the preceding 14 days. NUEDEXTA is
contraindicated in patients with a known hypersensitivity to its components.
NUEDEXTA may
cause serious side effects, including possible changes in heart rhythm.
NUEDEXTA is contraindicated in patients with a prolonged QT interval,
congenital long QT syndrome or a history suggestive of torsades de pointes, in
patients with heart failure as well as patients with, or at risk of, complete
atrioventricular (AV) block, unless the patient has an implanted pacemaker.
NUEDEXTA causes
dose-dependent QTc prolongation. When initiating NUEDEXTA in patients at risk
of QT prolongation and torsades de pointes, electrocardiographic (ECG)
evaluation of QT interval should be conducted at baseline and 3-4 hours after
the first dose.
The most common
adverse reactions in patients taking NUEDEXTA are diarrhea, dizziness, cough,
vomiting, asthenia, peripheral edema, urinary tract infection, influenza,
increased gamma-glutamyltransferase, and flatulence.
NUEDEXTA may
cause dizziness. Precautions to reduce the risk of falls should be taken,
particularly for patients with motor impairment affecting gait or a history of
falls.
Patients should
take NUEDEXTA exactly as prescribed. Patients should not take more than 2
capsules in a 24-hour period, make sure that there is an approximate 12-hour
interval between doses, and not take a double dose after they miss a dose.
These are not all the risks
from use of NUEDEXTA.
