Ehlers-Danlos mama trying not to twist things up *too* much!

Wednesday, November 3, 2010


There have been about a million things that I've wanted to blog about in the past few weeks, but by the time everyone is taken care of, fed, changed, medicated, and in bed, my fingers are just too tired.

Big news for today: my Social Security Disability was approved! First time. No re-application, no appeal, no court, no fighting. What a relief!!! I should get the official letter with the detailed information within the next few days, which is good, because I sort of spaced out during the phone call after I heard “Approved.”

I have not felt too confident about the SSDI application these past few months. Almost everyone I've talked to told me horror stories about years of appeals, court, invasion of privacy, impartial medical examinations. I was only being treated consistently by one specialist, and while he's an awesome doctor, he wasn't immediately willing to send back the letter of support requested by my attorney. All through the process, I got the feeling that my attorney wished I had a mental health condition rather than a physical disability. Even when I communicated with a disability specialist, I felt as though I'd done everything wrong. Every time I carried my child into my house out of pure necessity, no matter how much pain it caused, I worried about a disability spy watching from the bushes, ready to deny my claim.

Thank goodness all of that is over.

Now life goes on. On one hand, it hurts that the government has deemed me unable to work any job by nature of my disability. I know that this isn't a death sentence—this is what I wanted: the ability to take care of myself. So that's the other side of this, the positive. I did the best I could for a long time. This is what I need now.

Besides that big news, I also went back to PT to try to decrease the frequency of my migraines. My PT and I spoke a bit about cranio-cervical instability, as one of the orders from my neurologist was for cervical traction. (In the words of my PT, “If I did that to you, I'd probably take your head right off.") There's a new spinal surgeon starting at the orthopedic group I go to; my PT thought she might be worth seeing to talk about cervical flexion/extension fluoroscopy and treatment. Never a dull moment.

Wednesday, October 20, 2010


So yesterday I implored the general public to be generally nicer...and today it happened. So you'd think I'd be prepared, right?

In the words of my 3 year old, “No, nope, never.”

I was actually outside the preschool of said-3 year old when it happened. It was 20 minutes before closing and I had been driving for an hour, having left Old Boy's outpatient OT appointment right at rush hour.

Puggles and the Poodle were sleeping in the van.

Monster's preschool is in a large building in the back of an old mill complex. Since there is no view of the parking lot from his classroom and picking him up is often an unnecessarily long process fraught with odd rituals and delays, I am uncomfortable leaving the two little ones alone in the car.

Since I can't walk well and neither of them can walk at all, the only choice the three of us were left with was to use the double stroller. Luckily, our double stroller is light (enough). However, I was completely exhausted, so the thought of lifting anything more than a banana felt overwhelming. And I knew that getting the stroller out was only the first of several undesirable steps including probably waking two children, rushing in to pick up my anxious toddler (who hates being picked up when “the sun is sleeping”), then repeating the whole process to get back into the van and home.

I must have looked as tired and miserable as I felt at the moment that I opened the rear door of my mini van and pulled out the stroller because another mom a few cars over asked, “Do you need some help?”

I was so taken aback by the question that I didn't quite know what to say. I'm fairly sure I stared at her blankly for a moment.

--Am I dreaming? Is someone actually offering help?--

Of course, I graciously accepted stammered something unintelligible before saying, “No, I'm ok.”

She gave me a look of complete non-belief.

“I just have to pick up my son,” I continued, as though the sling, brace, limp, and obvious exhaustion were commonplace... “My two little ones are sleeping and I have to bring them in with me. It just takes a little longer, that's all.” Oh lies, lies, lies.

“Oh, ok,” she said, probably half-relieved and entirely confused. “It's just that you look so--” Disabled? Exhausted? Forlorn? All three? “But as long as you're alright.”

“I'm alright,” I parroted. “Thank you, though.”

She drove off and I slowly got the little ones into the stroller, managing to keep the Poodle sleeping and Puggles hooked up to her feed. I hobbled in to get Monster, who ran happily to meet me, which temporarily eased the exhaustion. I gladly chatted with him and piled everyone back in the car in what I felt was a reasonable amount of time.

Still, I could have accepted this kind stranger's assistance. I could have asked her to stand with the kids for a minute or to run into the preschool for me, or something. For goodness sake, I'm a mom and beyond that, I'm human. If I had seen anyone in the shape I was in tonight, I would have offered to help them--and not just out of obligation, but because I'd genuinely want to do so. Instead, tonight I was just too me—shy and stubborn and independent and leery all rolled into one.

Jeez. No wonder people drop the door on me.

Tuesday, October 19, 2010

Week in Review, My Thoughts on Public Hobbling

This past week has been crazy-busy with appointments of every kind for me and the kids. We also seemed to have wrapped up the week of stomach-yuckiness, until I was stricken yesterday afternoon. I think I made out much better than my poor kids and wife, though. Everything has stayed in, perhaps as a result of the many painkillers, but I feel gross. Thankfully, the big guys had school today and Cam took the Poodle out with her, so Puggles and I just maxed and relaxed. It's funny—Puggles is exclusively tube fed as a result of her genetic disorder and dysmotility. (I will eventually get her blog to be more than a figment of my imagination), but I swear sometimes she wants to eat. Either that or she's teasing me. Today, as I was forcing myself to eat Saltines and sip ginger ale so that I could take my regular meds, she was practically wiggling out of her seat whining at me. Out of common courtesy, I offered her a cracker. She looked at me like I was crazy!

So aside from my current stomach woes, I've actually been getting out of the house recently—to places other than the hospital and pharmacy, no less. It's still a work in progress. Some days I feel fabulous. On Sunday, we had the Poodle's birthday party, and I had so much fun.

We have some pretty awesome friends and family. One of Puggles' little friends, Skye, came over after the party with her parents, and we all enjoyed feeling normal for the evening.

I notice now when I go out in public (right arm in sling, left in brace, limping with a crutch), I'm met with one of two reactions:

  1. The Over-Reaction
    People keep their kids away from me and generally treat me like I'm dying. Also, like I'm deaf. A very strange conversation took place behind me at Stop & Shop. It was between two women trying to figure out how to pass me in an aisle and was **VERY** dramatic. I felt like turning around and informing them that I was able to hear them or like whacking them with my crutch as they attempted to rush by. I didn't.
  2. Complete Ignorance
    People either have no common courtesy, are completely oblivious to what goes on around them or think that a person with a physical disability would be appalled at any offer of help. This past weekend I went to Old Navy to get a Halloween costume with Old Boy. After shopping for a while and standing in line, he was even more wired than usual; I was even more tired. We walked to the doors with a group of people no more than three feet behind us. Instead of taking a step forward to grab the door for me as I struggled with a large bag, a crutch, a heavy door and a hyperactive child, the group pushed into us and huffed.

For cripes sake. It isn't that difficult. To the over-reactors: If I were in some critical condition, it's unlikely you'd find me at the grocery store or Old Navy. You don't need to whisper near me or avert your eyes. To the second group: have some decency. I'm not suggesting that anyone make a big show out of helping a person with a physical disability if they appear to be doing fine on their own, but if someone seems to be struggling, it doesn't hurt to ask if they need a hand. Furthermore, this doesn't just hold true for a person with a cane, but for any person. (After all, I'm still disabled on days when I'm not wearing the sling and using the crutch.) What's the worst that could happen? You might get snapped at. But chances are, you won't. And as a person with a crutch, I've really appreciated door-holding recently!
So overall, a pretty good week. I actually had two very productive appointments with new providers (for myself.) I have a great deal of anxiety when it comes to seeing new providers, especially since I am prescribed a lot of pain medication and have had some unpleasant experiences along those lines. To my pleasant surprise, both the neurologist and the gastroenterologist took the time to research EDS and were thoughtful and kind. Wow.

Wednesday, October 13, 2010

Story of My Right Shoulder

I had my post-op with my surgeon/wound check yesterday. All went well. Last week's cellulitis scare has reduced itself to nothing more than a few scabs where the blasted Tegaderm maimed me. I'm still on antibiotics for the next few days and have lost a few layers of skin near the end of my shoulder incision as well, but I am hopeful that I am heading in the right direction (and away from the Wound Care Center) at this point.

For those of you who are interested, here's the story about the procedure I had: Last year around this time, my right shoulder was very unstable, primarily posteriorly. I'm not sure if an of my fellow EDS-ers have experienced this with their shoulders, but I've always had primary posterior instability, then multi-directional. This has made for some awful interactions with the medical community, especially before my diagnosis. As a teenager, I would end up in the Emergency Room with a posterior shoulder dislocation only to be x-rayed, sent home, then called back once a radiologist viewed the films and realized that my shoulder was out. This led to the total destruction of my left shoulder, but also to my EDS diagnosis once I saw an appropriate specialist.

Back to last year. My right shoulder had been reconstructed 3 times. Each time, my surgeon tightened it to a point where “No normal person would have regained motion.” Each time, I did. Sadly, each time I not only regained my normal motion, but my hypermobility. Shortly after sensing hypermobility, I would subluxate, then dislocate. The surgeries each lasted about 8 months, so after the initial period of immobility, recovery and therapy, I usually had a few months where I felt like a “normal” person. Each time the surgery failed, I felt unlucky, but not necessarily doomed.

That summer day, I was in considerable pain, as I'd just undergone reduction mammoplasty in an effort to alleviate the severe cervical pain I'd suffered since pre-adolescence. Unfortunately, I'd suffered some EDS-related wound closure complications. So, as I sat in the orthopedic surgery office, sweating beneath layers of surgical dressing and my enemy tegaderm, I wasn't having complete clarity of mind. I was expecting to hear that we would have to repeat the shoulder reconstruction, as we had the past few years.

Instead, my usually busy surgeon sat down with us. “Look, “ he said. “There is a spectrum of Ehlers-Danlos. You have proven where you fall on that spectrum. Another soft tissue procedure is not going to work.” If my left shoulder wasn't already fused, he would have recommended fusing my right. Instead, he wanted to try a posterior bone block, a non-anatomical procedure designed to make it anatomically impossible for my shoulder to dislocate posteriorly by adding donor bone to my glenoid. (Building a better socket, where the ball couldn't just slide around at will.) He wanted to use donor bone because the alternative, taking bone from my own hip, is very painful. I remember his words exactly. “I wouldn't want that done to myself. Therefore, I'm not going to do it to you.”

Last November I had the posterior bone block with donor allograft. The recovery wasn't bad at all...little blips here and there, but after a few months, my shoulder worked quite well and didn't cause me any pain. In April, I had my ankle reconstructed and ended up non-weight bearing, on crutches, for 3 weeks. Toward the end of the third week, my right shoulder really started to hurt. I had a cortisone shot, but didn't think much of it. They freed me from the crutches early, so as not to torture my shoulders.

In the months that followed, I had pain in my right shoulder more frequently. I was going to PT for various other joints at the time. I went in one day in so much pain that she said, “You need to tell your ortho about that shoulder.” I made an appointment, thinking that it would be nothing another cortisone shot couldn't fix. I don't know that he was overly worried at the time, either. He injected me and sent me for a CT-scan.

My CT-scan follow-up was grim. Apparently, the allograft bone block had been resorbed by my body, which meant I had extra screw sticking out and presumably catching on my once-again unstable shoulder. I had 3 options: 1. repeat the surgery with the iliac crest autograft 2. reverse shoulder replacement (which has only been done in the US for 8 years—I'm 29 and planning on using my shoulder for longer than that!) 3. shoulder fusion

I obviously went with option 1 and am happy with it today, 13 days out. Should it fail, I probably will have to go with one of the other 2. My biggest concern until recently about shoulder fusion was really pain. I did not have good pain control after my left shoulder fusion in 2000. My doctor is more concerned about how I will manage my life and happiness and personal care, but I think he forgets that 1. I am more flexible than he is and 2. I have adjusted to not being able to do certain things. I'm still adjusting. Losing swimming, for me, was huge, but I moved on.

But today, I don't have to think about bilateral fusion. The shoulder is good. The pain control is good. The weather here in New England is beautiful and I am going to try to get out and enjoy it while my boys are at school.

Have a good day, friends!

Sunday, October 10, 2010

Feeling Foggy

The past 10 days have been a blur. This is probably a good thing.

As far as I know, surgery went well. My post-op appointment with John, the Physician's Assistant at my ortho's office, was largely spent discussing my pain and the cellulitis I'd developed near my surgical site. I have the misfortune of having very sensitive skin, one of my sensitivities being to medical tape, including Tegaderm. As a result, several large blisters formed under the dressing covering the incision on my hip. (I shall save you any more gory details. Long story short—this led to an unpleasant infection.) I left the appointment with a prescription for antibiotics, a stronger dose of the Fentanyl patch I've been using and for muscle relaxants. Since my surgery, I've had awful spasms in both limbs. My leg is worst at night and seems to encourage my left, currently uninjured leg to join the fun. At one point last week I was shaking so badly I was concerned I might be seizing, but my mostly intact mental functions seemed to rule this out.

The cellulitis has improved significantly since starting the antibiotic, so I am less worried about readmission to the hospital. Since the increase in Fentanyl, my pain is quite manageable, as long as I don't overexert myself. This is easier in theory than practice, although Cam has been a rockstar as far as caring for the children is concerned.

(She's so fast! By the time I've managed to hobble down the stairs each morning, she's medicated Puggles, dressed 1 or 2 of the boys, started coffee and breakfast...I just don't know how anyone can move so quickly in the morning.)

But back to the blur...a few times this past week, people have brought up pieces of conversation that they allegedly had with me while I was hospitalized. (This is usually after I've asked some question that I should already know the answer to or repeated a story or that sort of thing.) An OT from the home care agency came to my house and had apparently met with me while I was inpatient—I really didn't remember her at all.

Well, it occurred to me a few days ago that I'd had possession of the laptop while hospitalized and that the hospital had free wi-fi. Yikes. I frantically checked my Facebook account for any incriminating information posted while I was slightly incapacitated—thankfully not much there. I did notice that I wrote a blog entry, which was not too shoddy given the altered state I was in.

I'd like to think that my mind is in a better place this week, but I'm struggling to find words that usually flow freely—hopefully a temporary condition and part of adjusting to a higher dose of pain medication, not a sign that my brain is turning to mush.

Cam discovered my blog, which I hadn't told her about, given its humble beginnings. She was so sweet and has proudly boasted of it to our friends and family. I've forbade her sharing the link until I've written a little more—great incentive to get my brain working again.

And on that note, time to sleep. I hope you're all having as gorgeous of weather as we are this weekend.

Sunday, October 3, 2010


Still in the hospital recovering after Thursday's surgery. The pain in my hip, the site from which the autograft was harvested, was awful for the first 72 hours, but now seems to subsiding. My shoulder feels ok, but I've been through that piece a lot. My knee is still a mystery. My ortho is now thinking ACL injury. More on that later...

There are several hospitals in my area with large orthopedic programs. I'm sure each has its advantages and disadvantages. My surgeon is affiliated with a hospital without a residency program. At this point in my life, I see this as a positive. I spent many, many years being a medical guinea pig as a result of my Ehlers-Danlos Syndrome. Because EDS is so rare, residents love to use me as a learning experience. Sometimes, this is fine. Others, when I'm ill or in severe pain, I don't want to tell my story just so that everyone has the opportunity to practice their interview skills.

This hospital has a dedicated Ortho wing, but it seems that all the beds there were taken on Thursday night. No major complaints, though--I'm still in a private room with a wall-mounted flat screen LCD tv, a pull-out couch, a recliner and ample table and closet space.

The staff here have been so kind--hence the "Hospital/ity" title--and I've certainly needed a lot of help! Between Thursday's surgery involving both extremities on my right side, a shoulder fusion and unstable (post-op) ankle on the left and the need to use a forearm crutch and drag around a huge IV pole, I've been pretty useless. Throw in some POTS and medication side effects and I'm probably completely useless.

Overall, it's nice to get medical care in a hospital where I'm more than just a number or a syndrome.

It's taken me several hours to write this post, which is unlikely to be read by anyone. Since I'm falling asleep (again), that's all for now.

Wednesday, September 29, 2010

Pre-Op ::ANXIETY::

Surgery tomorrow. The overly friendly caller informed me that I should arrive at the hospital at 6 am, having had nothing to eat or drink after midnight. Bring nothing valuable.

I've been through this many, many times. I've been through this exact surgery before, save the iliac crest harvesting.

Still, I am a big ball of anxiety. My heart is racing, my thoughts are jumbled, I'm close to tears.

I've been looking forward to this surgery since the day, months ago, when I found out what was causing the pain this time--and what would end it. (Isn't this supposed to be the dream of chronic pain sufferers--something to end it?)

Tonight, even though my shoulder seems intent on reminding me why I need this surgery, I can't get beyond the anxiety.

I had hopes of getting some cleaning done, packing my stuff, relaxing with some Kung-Pao tofu.

Nope. I just want to sleep and get that much closer to 8 am, when I'll be comfortably numb.

Tuesday, September 28, 2010

Wheels for One of Us

After many straight days of migraine, I finally woke up headache-free this morning. Score!!

Puggles' wheelchair was delivered this morning--it's adorable. Odd description of a wheelchair? Perhaps, but please remember this is the wheelchair of my 2 year old daughter. Even the name is cute: the Convaid "Cuddlebug." Cute as it is, the damn thing is heavy. There's no way I'm getting it in or out of the car any time soon, if ever. We could really use an adaptive van, but there's no way that's happening any time soon. It's a good thing Cam is strong.

I used to be the strong one. (Sigh.) Even a year or so ago, I could throw the Monster over my shoulder and carry him up the stairs. When Atiyyah was alive, I used to carry him, his ventilator, his go-bag. Now, I struggle to carry Puggles and she's a peanut at 16 pounds.

EDS has not been kind to me these past few years. I am afraid of what the next decade holds for me. If (or more realistically, when) I do end up in a wheelchair, let's hope I get one as nice as the Cuddlebug.

Saturday, September 25, 2010


I used to write.

A few years ago, I overheard my brother telling a story. It went something like this:

"As a kid, Xan had an old school mac. By the age of 8, she had completely filled the hard drive with Word documents."

True. I was sort of a lonely kid, I guess.

Over the years, things became more complicated and my writing, which had also been fairly introspective, became too emotionally painful. I gave it up entirely a few years ago.

I'm not looking to make money. Heck, I'm not even looking for fame. On some level, I'm looking for a connection to the outside world, which is hard to maintain as a person with a disability and a parent. I have major blog-envy--there are a few blogs I've been reading on and off for the last year and I wish I had the sort of following they did. Of course, it would help to write!

So here's my start. Nothing glamorous. I'm Xan. I have 4 wonderful children and another in heaven. My little girl, "Puggles" is facing some major medical challenges. As though that wasn't enough, I have Ehlers-Danlos Syndrome, a connective tissue disorder that gifted me with faulty collagen, unnatural flexibility and chronically painful, unstable joints. I'm booked for surgery #5 on my right shoulder this week, so I should have some time to devote to blogging while I recover.

Well, that's all the fingers will allow for now.

Thanks for reading!