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.”
These are not all the risks from use of NUEDEXTA.