Managing Fabry Disease Requires Me to Put Mind Over Matter
Throughout my life, I’ve often put mind over matter to cope with the manifestations of my multisystem disease. I used this mindset during my 27-year military career to meet the rigorous physical demands of military life while managing Fabry symptoms, including:
- heart disease
- kidney disease
- lung disease
- chronic pain (small fiber peripheral neuropathy) and chronic achiness
- chronic gastrointestinal (GI) issues
- impaired ability to perspire (hypohidrosis) and overheating easily
- chronic lower leg edema
Looking back, I don’t know how I did everything I was required to do. I simply had a strong willpower to succeed.
Even though I no longer have to meet the physical demands of the military, managing my many Fabry symptoms requires some mind over matter, especially when I can’t rely on medications to correct a deficiency. For years, along with trying to maintain a healthy lifestyle, I used several medications to manage my symptoms, including:
- beta blockers to manage an irregular heartbeat (arrhythmias)
- ACEIs and ARBs to manage kidney-damaging proteinuria
- statins to manage cholesterol and reduce stroke risk
- various medications to manage chronic pain and chronic GI issues
Including heart transplant medications (another story) and some of the medications listed above, I currently take a minimum of 20 pills a day. Taking all these medications requires considerable thought, but it is fairly easy to do and doesn’t require too much willpower. I just need the discipline to develop a schedule and to pay attention to the alarms I set on my phone.
One critical medication I need is an exception. Intravenous enzyme replacement therapy (ERT), which provides the enzyme I am missing (the cause of Fabry disease), is more difficult to manage. The time and effort required are much greater because I have to drive to the hospital every two weeks and sit for hours while I receive the infusion. It’s very doable, though, and I am very thankful to have this lifesaving treatment.
Other symptoms of my disease don’t have medication remedies and take more willpower to manage. White matter disease in the brain comes to mind (play on words intended). Perhaps for this column, “Mind Over White Matter” may have been a more appropriate title.
In recent years, white matter disease has become more widely recognized as another common manifestation of Fabry disease, though it is still not well-studied or well-understood. I was unable to undergo the recommended periodic MRI scans to monitor the effects of lipid accumulation in my brain for 12 years because I had an implantable cardioverter defibrillator (ICD) implanted in my chest.
After receiving the incredible gift of a life-changing heart transplant in September 2020, my situation changed. When my new heart was put in, my ICD — the device that kept my heart rhythm stable and shocked me back into a normal rhythm when needed — was removed. I wrote about this in my column, “Regaining My Strength and Stamina Following Heart Transplant.”
Unfortunately, after being able to resume periodic brain scans, the results of my recent MRI scan indicated continued progression of chronic white matter disease, compared with my scans from years ago before my ICD. I had hoped for a different result after more than 19 years of receiving ERT, but the white matter lesions (microintensities) have continued to grow.
Medical literature about white matter disease mentions an increased risk of stroke, cognitive impairment, memory loss, imbalance, dementia, and other possible effects.
Medications to reduce or stop development of white matter disease are currently unavailable. The interventions are similar to those recommended for maintaining a healthy heart and a healthy lifestyle, with some added brain exercises. For me, it takes much more willpower to eat healthily, exercise frequently, maintain good sleep habits, and take deliberate actions to maintain a healthy brain.
Without direct medical intervention to remediate or cure white matter lesions, keeping my brain healthy through other means is the only tool I have in my toolbox for potentially lessening the effects. An internet search for how to keep your brain healthy will turn up the phrase “what’s good for your heart is good for your brain.” This concept aligns with the advice from my doctors.
A Harvard Health Publishing article titled “12 ways to keep your brain young” contains many essentials for maintaining a healthy heart and a healthy brain. As I age, I feel like my brain isn’t processing as quickly as it used to, and I’m experiencing more memory issues. But I don’t know whether it’s the result of normal aging or the progression of white matter disease. In any case, I’m determined to put my mind-over-matter skills to work!
Note: Fabry Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Fabry Disease News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to Fabry disease.
I am always fascinated by these articles by Jerry. I cannot imagine how he functions at such a high level with all of this tagging alone each day. Great information and great writing.
Thank you, Diane, for your kind words. We play the hands we are dealt as well as we are able. :-)