Getting Going

Download PDF "Getting Going"

Own Your PXE webinar

  PXE Vision: By and For PXEers   by Pat Manson

Getting Going

November 2005


Each PXEer´s journey starts with the shock of discovering that you—or a loved one—has PXE or that the symptoms described long ago as a vague possibility have indeed arrived. Where that journey takes you and how far you progress is largely up to you. It won´t be your doctor, your spouse, your employer or your insurer that will plot your path to recovery. It´ll be you alone. After all, it´s your PXE. Ideally, your journey will end in “recovery,” a place or state of mind where you are capable of leading a happy, fulfilled life, satisfied that you have done your best to regain as much as possible of your former life or to create a new one. But not everyone will get that far. The purpose of this column is to outline what it takes to get all the way there.

Once a person who has sustained a loss gets past the shock/denial phase, he has a big choice to make. This choice will have a profound effect on how that individual lives the rest of his life. For us, this choice can be stated as simply as this: do you want to be PXE or do you want to beat PXE? I have chosen the latter, and I hope you have (or will), too.


I Am Not PXE

There are some patients—certainly not limited to PXEers—who become their disorder. It´s as though their disease isn´t satisfied to just inhabit their bodies; it must possess their personalities as well. We have all known people like this. They have chosen to make their disorder the center of their lives, allowing it to define them. They become obsessed with their disorder, often turning conversations around to discuss their problem. They try to tie every other ailment they have to their disease. Some even feel strangely empowered by their victim status.

There are some who take this unhealthy fixation to an even greater extreme, adopting a passive approach to their disorder, “accepting” whatever their disorder dishes out, rather than striving for the best possible outcome for themselves. I am not particularly religious, but I do believe the popular expression: God helps those who help themselves. To not take advantage of the opportunities available - treatments, physicians, counselors or support groups - is a waste, or worse. Furthermore, I don´t believe we´re empowered by our condition, nor are we improved by it somehow. It´s an obstacle, a hindrance, dammit. If you want to get past that, to recover, you need to get going.


A Multi-Front War

To start with, you will need to attack the enemy - PXE - on several fronts at once. You don´t have the luxury of taking a wait-and-see approach. PXE itself has already taken the offensive, and it´s time you countered. You also cannot afford to wage war on only one front at a time because a loss on any front can mean defeat for you.

Be realistic though: you aren´t going to win each battle. You´re going to suffer some setbacks. Your progress on each front won´t be constant or continuing. It´ll be stop-and-go at times and often one step backwards, two (we hope) steps forward. But you´ve got to keep pushing ahead. Get going! PXE has a head start on you.

I know all this military jargon is corny, but it works. Plus it underscores how strongly I feel about our need - obligation even - to fight back, to give it our best.

Here are the areas on which PXEers must concentrate, the fronts on which we must be prepared to do battle. I plan to explore each of them more fully in future columns.



Each front is nothing less than critical, but this one drives all the others. So nail this one down first. It starts with your medical team.

If you have begun experiencing vision loss, you should, at a minimum, be seeing a retinologist, not just an ophthalmologist. Be sure your retinologist is treating plenty of AMDers (whose CNV is superficially similar to that of PXEers) and has some familiarity with PXE. If your doctor is considering hot laser or PDT, he should also have done a substantial number of them previously. Don´t be afraid to ask about this directly. It´s your body, and you do not want to be a guinea pig.

You will also need other medical care, either a very involved primary care physician (PCP) or a cardiologist and a gastroenterologist. He—or they—will check you for hypertension (high blood pressure), high cholesterol and other cardio problems as well as for GI bleeding—all issues for PXEers. While these manifestations of PXE are much rarer, they are also much more serious. Don´t skimp on these. Monitoring (and, if necessary, treating) these will need to be life-long, continuing endeavors.

Depending on whether you have skin lesions and, if so, their severity, you may also want to add a dermatologist to your medical team.

Mental Health

Virtually all PXEers are depressed from time to time. We've lost or are losing (or at best fear the loss of) the lives we've built. Therefore, it's OK, natural even, to be depressed, particularly early in the journey. Now do something about it. Go see a psychiatrist (most insurance will cover) and ask about a prescription for an anti-depressant to get you through this period. This doesn´t mean you´re weak. Everyone´s body naturally produces certain chemicals that need to be maintained in correct balance. If depression sets in and its underlying cause doesn´t abate, your chemical balance can get so out of whack that you can becomeclinically depressed. When that occurs, the healthiest person cannot will himself out of it. That may then call for a temporary prescription of an anti-depressant to get you back in balance. Remember: this isn´t necessarily a lifelong prescription.

But there´s more. Don´t allow your vision loss or your depression to shut you down. Get out of the house. Walk in the neighborhood or get a friend to take you some place where you can operate independently (a place you already know or which is easy to navigate). Do yard work. Work out. Dance. Play the clarinet.

A note on self-esteem: it will suffer, but don´t give in. OK, you're disabled or think you´re on the way. Many of us are. It stinks, but it's not your fault. You may not be a [teacher, accountant, bus driver, social worker, jeweler] anymore, but you're still you. You may not be what you used to be at work, but you are still a spouse and a parent. A son or daughter and a son- or daughter-in-law, too, probably. These are important roles that come with responsibilities. People need you…still. Perhaps even more than before.



If you´re still working, keep at it as long as you can, but not to the point that it harms you. If you leave work because of your disability, money will quickly be an issue. You will have three potential sources of future income: 1) disability insurance benefits, 2) social security disability benefits (SSD), and 3) your personal savings.

First, check out any disability insurance coverage you may have. If you´re employed by a company of any size or by a public entity, you probably have disability insurance. Read that insurance policy and the disability plan summary thoroughly.

Most employer-provided disability plans have an initial phase called short-term disability (STD). This often covers the first six months of disability. If the employee is still disabled after that period, the employer´s long-term disability (LTD) policy comes into play. Most LTD plans include a two-step definition of disability. Typically, for the first two years of LTD, you are considered disabled if you are unable to do your current job. This is “own occupation” coverage. After that, the definition will probably change to “any occupation,” meaning you´d be covered only if you cannot do any job, a more difficult standard to meet.

Benefits provided by employer plans are usually taxable. The best place to start is your employer´s human resources department.

The second possible source will be social security disability (SSD) benefits. This also follows an “any occupation” standard with benefits beginning after five months of continuous disability. It provides a monthly taxable benefit.

You can apply for SSD yourself, starting with your local Social Security Administration (SSA) office. You can get the ball rolling by a call to one of SSA´s toll-free numbers. See or check the government section of your telephone directory.

The third and final source of income is your own savings. This will, of course, vary with each individual. Nevertheless, you can take steps now to shore it up. If you suspect that you will be leaving the workforce soon or want to reduce your risk profile, plan ahead by trying to clean up your balance sheet. Pay off, as best you can, your debts, beginning with higher-interest debt. Keep your spending down. Defer major expenditures to a time when the future becomes more certain.

I applied for and was approved for both employer-provided LTD and SSD benefits. Legal blindness was not a difficult sell to the claims reviewers I encountered. There were a few administrative hoops to jump through, but it wasn´t too hard.

Family and Friends

Communicate openly and often with your spouse and kids about what a hard time you're having, what helps you, what hurts you. Encourage them to do the same with you. I promise you: they're hurting, too. Find a counselor (a psychologist—your psychiatrist can recommend one—or your minister, priest or rabbi) for both your spouse and you (insurance may cover, too). You go first. Then your spouse, then your kids. Then combinations of your family members. The counselor can facilitate the better communication you all may need.

You need to kindly but firmly let your family know that this #$%! has happened to all of you, that it is no one's fault, that together you can all handle it, that you need to work together to regain as much as possible of your former lives.

My wife Gerlinde has had a hard time since my eyes went bad. Her grief is not only for me but for the loss of the life we had together. Sometimes that grief translates into impatience and irritability and a sense of being overwhelmed by the new responsibilities she's had to assume. So we talk and reassess and move in a different direction. It's not a linear progression, however. Some days are better than others. We're still finding our way, reinventing our relationship. Nevertheless, we just celebrated our 25th wedding anniversary, and we (I did ask her) think we´re going to be fine.


Low Vision

If you hope to succeed in your new fuzzy, darker world, you will need to adapt. Searching for greeting cards, finding items on grocery shelves, dialing a telephone number, ordering from a menu, plugging in a lamp—all of these are more difficult, and you'll need help sometimes.

A quick and partial solution: a hand-held magnifier. You can buy one from Maxiaids or another distributor. Here´s a helpful hint: I have several—one for the car, one for the kitchen, one for each desk or table in my study. I take one with me everywhere. Each is illuminated by a LED light and has a 4X or 6X magnified lens. They cost about $US 40 each--ask for one for Christmas, for your birthday, or for Mother's or Father´s Day.

A hand-held magnifier, though, is just a start. If you haven't already, visit your nearest state department of the blind's low-vision center. The government centers can provide some adaptive devices and other aids for free or at reduced cost. Their products include magnifiers, glasses, software, closed-circuit television systems (CCTV), lamps, etc. There are also private low vision centers associated with some eye hospitals and other treatment facilities.

I have used three different low vision centers. In addition to providing the products described above, the Virginia Department of the Blind and Visually Impaired, located in my hometown of Richmond, Virginia, also offers vocational and rehabilitation services – training for walking in crowds, cooking, shopping, shaving, writing checks and other life skills. The facility where I see my retinologist, the Wilmer Eye Institute in Baltimore, Maryland, also has a low vision center. Finally there is a small center in Richmond attached to a facility housing numerous doctors who treat the eye. See what options are available to you.

Yes, everyday tasks will unquestionably be harder and will take longer, but you can do it. In fact, you can do almost everything you could before, just differently . . . and more slowly.



That´s all for now. I will explore more fully each of the fronts I´ve described above. In the meantime, please keep trying to be, as the U.S. Army´s commercials say, the best that you can be.

Next time: No preaching, but “What I Miss Most (about not seeing)".

Back to PXE Vision: By and For PXEers