Nuvigil Update

When you last heard from me—if anyone is even paying attention, and this isn’t just my public journal—I was about to start Nuvigil in an attempt to vanquish the Rip Van Winkle-esque state I’ve been existing in for I-Don’t-Know-How-Long. True to form, it’s been about a month. When it comes to blogging, I’m nothing if not consistent.

Anyway, Day 1 on Nuvigil was AMAZING. I took it in the morning and was on fire. I stayed up all day, cleaned the house, was patient with the kids (remembered why I wanted 5 children to begin with), caught up on laundry, scheduled a dinner date with a friend and just felt like my old self again. I felt 22 instead of 82. I’d forgotten what it was like to be human until that day. Day 2 was equally productive.

Wow. Was this really all I’d needed for so long? A little “surfboard-shaped pill”, as Monster would say. Maybe going back to school was within reach. Maybe having another baby was doable. I guess I should note that the first 2 days weren’t without side effects. Both days, I had a dull but consistent frontal headache the entire day, very little appetite, mild to moderate nausea and significantly increased acid reflux. However, I was willing to swap the dead-tired feeling for these.

The headache was the worst on Day 3 and I ended up taking 2 doses of Relpax and some Reglan for nausea. I also didn’t feel very energized, which invoked immediate fear that the medication had failed. I found that I felt better when not upright, so it may have been that I was more POTSy/dehydrated after running around for 2 days and not eating. I tried to ride it out. I rested and waited impatiently. I sipped Gatorade and did shots of Maalox to settle my stomach.

The next few weeks were tough, physically and emotionally. I never had any more days like Days 1 and 2 on Nuvigil. I tried being neurotically consistent with my sleep schedule, my asthma medication, my long-acting opioid—hoping, hoping that one of those was the key to getting back to good. Nope. In addition, my daughter went through a tough medical procedure.

Feeling completely helpless, I stopped taking the Nuvigil entirely, started senseless fights with Cam and resigned myself to a life of exhaustion. Then, in a moment of clarity, I called my psychiatrist. My psychiatrist doesn’t do much for me these days—we check in twice a year, he writes refills for my Cymbalta, lets me vent for 30 minutes. I started seeing him because I couldn’t close my eyes after Atiyyah died without picturing the moment of his death. That was horrible. In contrast, the Nuvigil situation isn’t so bad.

So my psychiatrist saw me the next day, suggested talking to the doc who prescribes the Nuvigil about a dose adjustment and also suggested changing the timing of my Cymbalta dose from PM to AM. Maybe, he theorized, I’m getting walloped with it first thing in the morning and that’s either causing or exacerbating the hypersomnia. And since I was a bit on edge that day and having a hard time with recurring thoughts of my children dying, he added Wellbutrin XL, an antidepressant offering good coverage for anxiety. It has the adding bonus of being “activating” so maybe it would keep me awake, too.

Things have settled down. I’m awake-ish. I’m sure the Nuvigil has some benefit, as I haven’t had those scary falling asleep driving moments. I’m hoping to get more info from the MSLT on October 1^st and fine tune the meds. So all in all, Nuvigil is no panacea, but it ain’t half bad.

I hope you’re all well.

-Xan

Oh yeah! Before I forget…I stumbled upon (without the use of stumbleupon), a fantastic site called Band Back Together. It’s a compilation of writings and resources about everything. The underlying message is “We are none of us alone.” So not only is it a veritable information clearinghouse, it’s an incredibly positive, supportive community. There was recently a submission about Ehlers-Danlos, which is how it popped into my inbox via Google Alerts, but it’s fabulous. I encourage all of you to read, comment and WRITE. I submitted a piece recently about my daughter, so perhaps I’ll let y’all know when it’s going live. Until then, I’m With the Band. I hope you will be there too. <3

Wake Up Call

Last week I had a neurology follow-up, where I again mentioned my constant exhaustion. I hadn’t been seen in clinic for a few months, but the last time I was, the nurse practitioner who serves as my primary contact there ordered a bunch of labs, all of which were fairly normal. My iron was low, as was my potassium, but not low enough to be an explanation. My overnight sleep study wasn’t particularly helpful, either, although the NP also didn’t think absence of REM was in any was normal. She talked about prescribing a stimulant medication, but it seems the doctor she works with is quite stingy when it comes to scheduled medications, which both Provigil and Nuvigil are, and she confessed to having very little experience treating Narcolepsy and related sleep disorders. I believe her exact words were, “Believe me, you don’t want me prescribing your Nuvigil.” Hey, at least she’s honest. I wasn’t all that concerned, since I also see a pulmonologist who specializes in sleep disorders and seemed willing to try medication once I did the next sleep study.

Yeah, that.

I’m limited in where I can go for the study by my health insurance plan’s large deductible and by the privileges of my pulmonologist, whom I’d like to read the study, not just the report. Setting up the study took some work, as I was first booked (again) for a regular overnight study, which wouldn’t have provided the additional information needed to confirm or rule out narcolepsy. When they eventually called me back, they told me to come in on October 1^st.

Two months?

I have come to expect to wait two months to see my daughter’s motility specialist, one of two in the entire country. But I’ve rarely had to wait two weeks, much less two months for any type of study at this hospital, which touts itself as being ranked #1 in the state in Patient Satisfaction for {Some Number of Years} Running!

And yeah, maybe you’ll blame it on my American-overprivileged-entitled mindset that I didn’t just write the date on the calendar and wait and deal--but I didn’t. One thing I have learned during my adventures in medicine is that not everything is as it seems, including wait times for appointments. (Check out this article if you don’t believe me.) So the first avenue I pursued was that of trying to procure an earlier appointment. No dice, at least not yet. I’ve gone around in circles with the scheduling center and played phone tag with the sleep lab, but unless I want to pay a hefty fee by going to another sleep lab, as I have yet to meet my portion of our insurance plan’s 2 person deductible, the October 1^st appointment stands.

For a few days, I was almost ok with that. After all, I’ve been dealing with being tired for quite some time now. I enjoy coffee…and naps…and it’s getting close to Snuggie season.

This morning I reached my breaking point.

Monster had his outpatient Occupational Therapy appointment in the city, a 30 mile drive from our home. Cam had to work, so after getting Miss Puggle on the bus, I packed the three boys in the car. It was just before 9 am; I’d been up for maybe 2 hours.

By the time I reached the light at the top of the main road, a 3-minute drive, I was excessively tired. I knew that the drive was going to be painful.

I sucked down an iced coffee and a shot of espresso, praying the caffeine would counteract the overwhelming fatigue without wreaking havoc on my stomach and forced myself to stay awake and alert by rocking, shaking, adjusting the air & music and talking to the kids. When we arrived safely at Monster’s appointment, I called my pulmonologist’s office crying and asked if there was anything at all they could do to help me. Waiting until October for the MSLT, then for the results, then for treatment to start would mean giving up driving—at least for the most part. There’s no way I could continue to rely on caffeine and sheer willpower.

At first, the medical assistant told me I would have to wait to have the MSLT, but I kindly (but firmly) asked her to at least relay the message to the doctor that my sleepiness was negatively impacting my quality of life and the safety of myself and that of my children. She begrudgingly took my name and number. (Really? It’s just a message!)

Amazingly, I got a call this afternoon that my pulmonologist was calling in Nuvigil for me to try starting tomorrow morning. Finally. Something.

I’m trying not to get my hopes up too much, as I’ve had a lot of awful medication reactions, but I feel like a weight has been lifted. I have hope that life can feel normal again. Even if my life is stressful, painful and complicated, I’d like to be awake to experience it. I’m so tired of being tired.

We’ll see how it goes.

A Birthday

August 10^th is Atiyyah’s birthday. He would have been 9. Old Boy and I brought flowers to the cemetery for him. We shouted “Happy Birthday Atiyyah” as we drove out. While we were at the market buying the flowers, Old Boy happened to tell the florist that it was someone’s birthday today. She must have thought it odd that I shushed him. I felt bad doing it—I like to let Old Boy speak freely about his brother. It was more for the florist’s sake, to save her from that awful moment when the bouncy little 6 year old says the bright flowers he and his mom picked out with such care are actually going to be laid atop the grave of his dead brother. I’d rather she think I’m a jerk than ruin her day with thoughts of us spending ours in mourning.

But as time has passed, the days, even the birthdays, aren’t as sad. Sometimes the grief still swallows me, overpowers me, but today I just enjoyed the sunshine.

Still…I wish you were here, buddy. I miss your sweet face.  

What I've Been Thinking About

Maybe it's something about turning 30, but I have this urge like never before to have a baby. No, not just to have a baby, to have a baby. There, I said it. It doesn't mean I'll actually do anything about that—since that would require a lot of planning, given the sort of relationship I'm in, but I feel it deserves some attention.

Let me back up. When I was first diagnosed, I remember sitting in the office of the esteemed shoulder doctor, my head swirling with questions, hands shaking as they clutched the handwritten lists I'd written but forgotten. My eyes quickly shifted from the brilliant x-rays illuminating my useless bone held together by titanium plates and screws to the surgeon's white coat and kind face to the door. I wanted to run, to escape the future, to forget the words I'd just heard.

“What do I do now?” I stammered.

“There are support groups,” he said. “See a genetics counselor if you want to have a baby. We can talk about what to do if you need more surgery...”

No, no, NO!!

I wanted to scream, to shake him, to tell him that I didn't care about surgery or a baby, that I was 19 and that all I wanted was for him to be wrong. I've been told that my face doesn't mask emotion well, so I'm sure he got it. I know Cam did. We drove the 2 hours home in unusual quiet, occasionally making small talk.

Over the years, Cam and I talked about having a baby, but as my body got worse it seemed obvious that a pregnancy would be hard for me. Not that it would have mattered—since Cam was a little girl, she's wanted to have a baby. She carried the Poodle and her pregnancy was wonderful. I never felt I missed out on anything. Until now.

Cam thinks she might want another baby “in a few years.” Until recently, I was perfectly content, but now I am struggling. It's new. Foreign. We talk about it, but it's painful.

I don't want to give the wrong impression here. Adopting my older children was an amazing journey. I was blessed with four precious beings that I treasure each day. When I think of them, especially of Atiyyah and Miss Puggles, whose birth parents I do not know, I often think there was some sort of mix up that led to their being born into the wrong family and just taking a little longer to get to us. Since I know Old Boy and Monster's biological mother, I feel a little differently—mostly thankful. She gave us the most selfless gift of all, the gift of our boys.

It's not that I don't want to adopt another child, it's just that the internal voice won't relent. That damn maternal instinct (which should be satisfied by parenting four children despite the constant gnaw of pain and pummel of exhaustion) screams, “Time to have a baby!”

And what about that EDS anyway? I may catch some flack for this, but even if the stars were to align and my hips and spine were in good shape to carry a baby and I were off the cocktail of pharmaceuticals, I feel uneasy when I think about knowingly passing this on. Yet I feel just as badly about knowingly saying, “Uh-uh” to the little EDSers-to-be during a potential genetic counseling appointment. What if they had my eyes? My mind? My...? (And this from a staunch defender of reproductive rights. Do you see the mayhem that the big 3-0 has brought to my mind?!)

It's all hypothetical...if that. I will never be able to have a baby. This body barely holds itself together enough to support me, let alone me +1. I've known this for years. I'm fortunate that I have 4 living children. I'm fortunate that I have a partner who could potentially carry another child. I will never be able to have a baby. I've known this—somewhere within myself—for years.

Why then, does typing it bring tears to my eyes?

Chief Complaint: Exhaustion...Any Ideas???

When I was younger and yet to be diagnosed, I had my fair share awful encounters with the medical world. Doctors thought I was faking injuries and ailments, self-inflicting them, that my parents or someone around me was hurting me, that I needed to be institutionalized...the list goes on and on.

Thankfully, that's not usually the case anymore. I occasionally encounter doctors and other professionals that don't understand EDS and make insensitive comments or propose treatments that are inappropriate, but as an adult who is able to choose her own providers, I'm quite happy with my team. There is one area of exception, though.

For the past year or so, I've been exhausted. I'm not sure when my energy was zapped, but I am perpetually operating in a state of "tired"--from the moment I wake up until I go to sleep. I remember a time when I wasn't so tired; I just don't remember when it was. This was of concern to my various doctors until all the tests came back "normal." Then quite a few of them settled in to that oh-so-comfortable default position of "blame the patient." Sort of.

My neurologist said it was because I was in pain and depressed and not eating well. My rheumatologist said I didn't seem depressed but also blamed the pain. My psychiatrist agreed that I wasn't depressed and didn't know what to say about the pain. Maybe acupuncture.

Ok. I understand that chronic pain can cause depression, which can fatigue. I'm really not depressed, though. And on top of the constant fatigue, I also have crushing "attacks" where I suddenly feel completely exhausted and out-of-it, as though I've been awake for days. When these come on, [which is more likely mid-morning, late-afternoon or after being in the sun] I can barely keep my eyes open. (I think this is due to a weird ocular sensation, but it's hard to explain.) When Cam witnesses these, she can see a physical change, although she's not certain the change would be obvious to people who don't know me, besides my seeming tired.

My internist was concerned about narcolepsy, so I had a sleep study. Of course, I couldn't sleep that night--go figure. What the study did show was a complete absence of REM sleep and mildly reduced sleep efficiency. The physician reviewer attributed the lack of REM to my Fentanyl & Dilaudid and still considered the study fairly normal. I do need to have another one, including the MSLT now that I'm out of my cast.

Has anyone experienced this? What do you think? I'd love some opinions, answers and treatment suggestions...this exhaustion is what cripples me more than the pain, fusions, GERD and all other symptoms combined, if that says anything.

Thanks in advance.

*Xan

When A Doc Gets It

Of course it's been nearly 2 months since my last entry...what else is new?!

In my defense, my computer was virus-stricken during that 2 month period. It just returned to me, thanks to my Ninja-Warrior Brother-in-Law who also happens to be a PC repairman. Here's some unsolicited advice (this is my blog, after all): Back up your files, especially pictures and videos of your loved ones. I managed to recover most of what I lost, but it was no easy feat.

When I last wrote, I was not-so-patiently waiting for that darn cast to come off. I'm happy to report that it was removed as scheduled and that I've been hobbling on two feet, assisted by my trusty walking boot and forearm crutch/es for some time now. Even better, at my 2 month follow up, my ankle appeared to be solidly fused. That was great news, as solid union wasn't really expected until about 4 months. I'm all for speedy healing.

I had a milestone birthday this year (complete with a fabulous surprise party), but I'm not too broken up about it. I don't feel any older than I did last year, and despite being a mama and hobbling around like an 85 year old great-grandma, I still have difficulty seeing myself as a real adult. (College students just seem younger each year—what gives? And high schoolers—don't even get me started!) So getting older didn't bother me too much. What did bother me that day was the news Cam brought home from the hospital about my orthopedic surgeon—that he was leaving the practice and moving out of state.

I spent the rest of the day with a lump in the back of my throat. Ortho-Doc has been my only specialist up until this year. I started seeing him the year after he joined the practice here, right after he finished his fellowship. In the beginning, he was very eager to fix me, to do for my right shoulder what no one had been able to do for the left. And while he always respected my wishes when it came to treatment, to surgery, it wasn't until my first (or maybe second) right shoulder surgery failed that he began to truly understand how I ended up with a left shoulder fusion at the ripe old age of 19. Since then, he's let me make all the decisions. When I want to go the surgical route, we come up with a procedure. When I want to try injections or pain management or physical therapy, he facilitates that. In a most un-surgeonly way, he actually listens and involves me in my own treatment. The thought of having to find someone else to deal with me (especially when many surgeons don't want to touch an EDSer with a 10 foot pole) was most unsettling, especially since I'm still in need of more work on my shoulder.

Turns out all is not yet lost. When I actually went in to see Ortho-Doc, he confirmed that he is leaving the practice, but he'll only be about 2 hours away. While I've been quite spoiled over the past few years by being able to roll out of bed ½ hour before my ortho appointments, I'll take the drive if it means not having to deal with someone new! Thankfully my new insurance covers out-of-state specialists. I'm angry and eternally grateful all at once...grateful that I have doctors who are actually willing to [try to] figure me out, treat my pain, and treat me with dignity. STILL, I am angry that this disorder and the thought of losing one of the members of my “team” reduces me to an anxious mess (on my birthday, no less) and that the anxiety isn't unwarranted!

Every doctor should be like Ortho-Doc. Every doctor should “get” it, should treat patients well, involve them in their own care. I'm finding that more and more doctors are at least minimally informed about Ehlers-Danlos Syndrome and are eager to learn. I've had fewer “Oh, so you're a circus freak,” remarks recently. I think we're getting there. Still, I'm going to hold on to the good ones I have!

Be well!

*Xan

Rainy Day Rant

Someday I'd like to believe I won't have to apologize for the length of time between posts. (Absence makes the heart grow fonder?) I am sorry, though. My dear Puggle (AKA my sweet daughter) had a few rough months, but is feeling better. Nope, I haven't updated her blog either. She does have a CarePage, so if you want to stay slightly more up-to-date on her medical stuff, send me an e-mail. Now let's get to it.

I wrote an entire post about my ankle fusion, which went off without a hitch on April 28^th. I'd like to think it was informative, so I'll probably post it sometime in the near future. (I'm guessing some of you have bad ankles, too and might like to know a little more about the whole ordeal. Short synopsis: it wasn't so bad. I think I'm in more pain from having been on crutches for 19 days than from the surgery. I may have a different opinion once I start walking on the screwed-together ankle, I suppose.)

But for now, I have an entirely different agenda. If you'd like a totally feel-good post, you might want to read elsewhere. I may have mentioned earlier that I'm not always a most cheerful person. Although I've brightened up considerably since my emo-ish adolescent days, I don't quite fit in with the other PTO moms. And if I were to pick an alternate title for this post, I'd borrow the words of a bendy friend and go for: “Dear EDS, Fuck You.”

I'm not sure if it's the 19 days of hobbling or the 3 days of rain or the 2 sickly children, but I've had enough. I've had enough of the damn cast, the hospital bed, the feeling of impotence as I watch my family live their lives from whatever seat I'm consigned to at the time. I miss my bedroom, located on the unattainable 2^nd floor of our house. I miss the time when traveling from the living room to the kitchen didn't require prior planning. I miss feeling like a contributing member of anything.

I went in yesterday and had my stitches removed and a new (hopefully final) cast put on my ankle. If all goes as planned, in two weeks, I'll be out of this cast and back in a walking boot (although there will likely come instructions not to do much walking.) This will give me the freedom to climb stairs, carry the Puggle, and do a whole lot more without difficulty (drive, shower, etc.)

Why, then, can't I just look forward to the nicely-fused, cast-free future (i.e. May 31^st @ 9 am, not that anyone's keeping track)?

Because EDS just hasn't given me a break—EVER. Every time I thought, “This is it; I'm done,” out popped some new symptom to be dealt with. Dislocations or pain or dysautonomia or migraines or seizures or reflux or medication intolerance. It's been a long time since I've felt like ME...sometimes I don't even know how that's supposed to feel anymore.

Then again...just when I've resigned myself to a certain level of disability, hope does seem to shine through. Last year, I thought I was wheelchair-headed for sure, but now I don't know. I managed to walk pretty well for a while this year. My new knee brace is promising. My new ankle is promising. I finally found a medication that stopped the headaches. And while I may not be able to eat bagels, my stomach and I are on slightly better terms than we were last year.

So here's the truth—I'm scared. I'm scared of what the future holds. I'm afraid of failure. I'm afraid I will never be able to stop crying.

That may not be very encouraging for those of you who are newly diagnosed or wanted something uplifting, but that's the truth. I want a life that is more than a collection of diagnoses and a laundry list of (failed) surgeries. I want a life in spite of it. I have 4 children who need me. When I was first told I had EDS, at age 19, it meant little to me. It explained my crazy joints and rationalized a shoulder fusion. I had no idea how great the impact would be. I don't think my doctors knew. Ugh. (And if they could see me now!!)

A rheumatologist I saw had this to say about EDS:

“We used to call EDS 'Benign' because it doesn't destroy the joints in the same way as say, Rheumatoid Arthritis, does without treatment. We no longer call it benign because while it may not destroy your joints, it destroys your life.”

True.

But while I'm glad ecstatic for the understanding, and while I am afraid of what the future holds, I refuse to let EDS destroy my life.

And while I don't knock any of the following, I just wish there was treatment besides writing, crying and camaraderie. And joint fusion.

-Xan

P.S. I will have better things to write about in the near future. So thanks for reading and I'd by honored if you continued to do so!