by Robert Lanz, LCSW
Marfan’s syndrome is rare and many hospital social workers will go years without coming into contact with one of the unfortunate patients who have this illness. It is more likely that the ER social worker will be acquainted with a Marfan‘s patient than the social workers ‘upstairs.’ At least, that’s how it seems to me.
Unlike the more enigmatic neurological disorders like multiple sclerosis, Parkinson‘s disease, amylatropic lateral sclerosis, myasthenia gravis and all the rest of those diagnoses that are difficult to pin down at first, Marfan’s, if it serious enough, can be diagnosed fairly early in life. But because it is a spectrum disorder with different levels of symptomatology, it may also be discovered late in life. Either way, it’s a difficult diagnosis for the patient if they are at the high end of that spectrum where it causes weakening in the connective tissues- joints and heart valves and even eyes and intestines may be affected. With luck, in a mild case, patients may not even know they have it. Without luck, as one grows through adolescence, the most obvious visual symptom is noticeable gangliness. These patients are taller than other family members and have spindly fingers, flat feet and their extended arm span is greater than their height.
Other obvious signs are spine curvature and a protruding breastbone, but the most dangerous complication is leaking heart valves. Leaking valves can cause blood to pool in the heart chambers which can lead to clots, a potentially fatal condition. Some of these patients need to have those valves replaced surgically and they also have to take blood thinning medication-the dreaded Coumadin Curse. Coumadin is like warfarin, what you get at the drug store when you buy rat poison. When ingested by rats, it thins their blood to the point where it all leaks out of the rat’s blood vessels and they die quietly in the garage or down in your basement where you later find them mummified. Warfarin can do the same to humans too if the human isn’t careful. If that isn’t enough to terrify you so far, there is some older literature that refers to a few Marfan’s patients with developmental delays and thought disorders, also.
Of course, if there were a frequent flyer patient with Marfan’s and schizophrenia, she would be on my list, especially if she had lower than normal intelligence and some symptoms of a histrionic personality disorder, what some ER nurses refer to as a “train wreck”. A lot to work with but not impossible for a skilled clinician. Over time, our Little Miss Marfan’s became one of the regulars and had thrown a few tantrums in the ER, so she was often referred to the social worker for intervention. Despite the constraints of time and the difficulties of her diagnosis, over an extended period she and I were able to grow close enough to engage in a behavioral truce she wasn’t willing to share with other staff. The triage nurse would call me as soon as she showed up at the check in desk.
“Bonnie’s here, Bob.”
I’d go out front and start schmoozing, because if I was successful, her ER visit would probably go OK. For the most part, everyone respected our relationship and I did too. It was a great deal easier to have her as a friend than as the kind of enemy she could turn into, with her history of extensive treacherous ranting and raining hell down on the staff during busy shifts. It took time, finding a commonality we could use as a bond, finding a neutral zone we agreed would be anger-free and require tolerable behavior toward all of the staff. I think her flirting with me and me letting her do it was what finally worked, although I could be wrong about that. Whatever the reason, the ER time we spent was generally serene and I considered it to be good social work intervention. Not exactly a dream date, but workable.
The Coumadin Curse can strike anywhere, any time, to anybody. When it was finally my turn, it was the treatment, or torture, of choice for a deep vein thrombosis that caught me off guard when I was battling a painful case of diverticulitis. The diverticulitis was over after a couple of weeks “upstairs” but the thrombosis lingered for almost eight months. So did the Coumadin regimen, a curse especially brutal for an active guy. Thin blood precludes fighting, surfing, skiing, mountain biking and a few other of my normal activities. I was stuck in adrenaline limbo, left only with walking my dog for excitement, as I had done when I had a significant back injury when she was just a pup. Boring except for the dog bonding part.
The Coumadin diet you get forced into is broad enough to satisfy most folks, especially vegetarians, but once you settle in on your diet choices and have them synched up with your meds you can’t change it much. A dietary screw up could change this thing called your INA levels and make your blood thinning medicine less effective or even dangerous if you are a glutton like I sometimes am. That’s why Coumadin patients have to go to the clinic every week to have their blood tested. Too bad there isn’t a clinic for the rats and mice.
One of the secretaries in the Coumadin clinic, right across the street from the emergency entrance, also worked part time with us in the ER. On my second week check up, she dropped a bomb on me as I was signing in.
“Look Bob. There’s Bonnie just ahead of you. She’s in the waiting area.”
And just when I thought life couldn’t get any more cruel.
She was in the waiting area and I couldn’t avoid her. She started flirting immediately, just as if we were in the emergency room and I was the social worker. Now we had two things in common. Coumadin and flirting. Hard to tell which was the more difficult for me and I wondered when things would stop piling up, but then realized what a wimp I was, because some day I would be back to normal and she never would. Some day we’d be over the clinic flirting and get back to ER flirting. Some day there would, for me at least, just be the ER face time together again and I hoped to get back to our previously agreed upon truce.
That went on with Little Miss Marfan’s until I got over the DVT and the Coumadin Curse became just a bad memory. We did our truce dance in the ER until I retired some ten years later. What changed with Bonnie and me in those years was my appreciation for how difficult it was to live with that Coumadin inconvenience even when I knew it would eventually be gone, a relief she was never going to know. To have the Coumadin Curse for life along with all her other mental and physical challenges was a burden I didn’t even want to contemplate. Now we were bonded with the curse and if that made our relationship better, and it always seemed to, so be it, and I was willing to let her flirt with me any time she wanted…