"Suffering the Slings and Arrows of Outrageous Fortune"

Posts tagged ‘RSD/CRPS’

The Big Appointment

I haven’t posted in regards to the important neurology appointment we had this month. Em had been referred to a neurologist at OSU at my request, so we could try to get a diagnosis – either of CRPS or small fiber neuropathy or something. Then the labs done during her LP in January showed some concerning markers for MS and sarcadosis and her local neuro wanted her to see someone more knowledgeable than him. Since we already had this appointment set up, it could serve both purposes. Then, the seizure-like episodes started and added an additional layer of wackiness.

So, basically we threw a whole lot of crazy at him. EDS, CCI,  MCAD, POTS, possible CRPS or “something”, evaluate for MS, seizures…  Did I leave anything out? Actually, I did: Post Concussion Syndrome. Sigh.

We have learned the hard way not to put too much hope or weight on any single appointment, but I would be lying if we didn’t at least sort of hope deep down that he would be able to give us a diagnosis and a quick solution on the first visit. When he didn’t, it was a little disappointing and overwhelming.

What he did was listen and order a bunch of testing:

  • He ordered an inpatient stay on the Epilepsy Monitoring Unit to try and figure out what is going on with these episodes. (Side note: after a month and a half of them and a helpful hint from a reader, I think we have a direction to go in. Video EEG monitoring is definitely needed, but will only be a start. More about that soon!) He definitely agreed that we need to rule out all the medical possibilities before jumping to the conclusion they are psychogenic. Don’t have this scheduled yet, but hopefully we can get it done soon, figure out what the heck is going on and stop it.


  • He ordered a bunch of labs, including mast cell testing. No one, including the allergists Em has seen has been interested in pushing for an actual diagnosis or any treatment other than Zyrtec and Zantac. It would be nice to have someone actually helping with this.


  • He ordered 3 brain MRIs – one with and without contrast, a MRI Angio and MRI Venogram. I am assuming these will help rule in/ out several things, MS for one. He also was not thrilled with the fact that she has been on Diamox for several years with no evidence and is wanting to make sure there is no structural issue causing her high pressure headaches. He did say he wouldn’t mess with her Diamox but he was clearly skeptical. However, he was shocked that cervical fusions are so common for treating neuro symptoms in EDS. We definitely agreed that it was wise to avoid a fusion if possible so that was a start. Perhaps, with time, I can help him understand the rational of the Driscoll Theory and why we absolutely believe Diamox saved Em’s life. There was just too much to cover in this first visit.


  • He ordered an nerve conduction study. This is definitely looking at CRPS but her exam also showed some very concerning numbness in her legs. I knew she had numbness but it was far worse than we knew. He pricked her all over with a pin. She mostly could not feel it in large areas on her legs. Like, at all. Her feet and hands are numb but her legs in particular have very little feeling. He seemed concerned. I know I was. So, perhaps we are looking at a neuropathy instead of CRPS? She is having increasing difficulty walking and I have to wonder if this is part of the problem. I don’t know but it is more than a little scary.


  • He ordered more autonomic testing – another Tilt Table Test and a sweat test and 24 hour Holter Monitoring. He is concerned that much of her problem is autonomic related and seemed to get caught up in the fact that she hasn’t been diagnosed specifically with POTS by the cardiologist. I am honestly not sure what a name does in this case – the cardiologist is treating her for/ as if she has POTS but has said he didn’t want to label it POTS because it is more complicated. So we have just used the term “Autonomic Dysfunction” this whole time. I am conflicted on this – on one hand, I totally agreed with the Autonomic Dysfunction diagnosis as opposed to POTS. On the other hand, I have felt for a long time that they are not doing much for her at the Syncope Clinic. We go once a year and they say, “Increase your Midodrine; see you next year”. So, maybe this reset is what we need. But… I absolutely dread the idea of another Tilt Table Test for her. He says the one she had several years ago wasn’t good enough, that he needs more information. I am not sure I totally see the point, especially considering she will have to be off ALL her pain meds for 3 days. I can’t even say how awful that will be. We are holding off on scheduling this until we talk to him again. We will need some help, some plan to make this happen – a 2 hour car trip is bad enough on a good day, I cannot even fathom how she would manage that trip without pain meds. If it is not absolutely necessary, I can’t put her through that. Yet, I wonder if maybe, with as dire as the situation is, it is necessary to figure out what is going on.

We liked him well enough, but the whole thing was a little overwhelming – he was very clinical, very data oriented and it was both comforting and annoying. Em was frustrated – exhausted, in more pain than she was in to begin with – because while she couldn’t feel most of the pin pricks, it still caused her pain to spike terribly afterwards – and terrified of having to go off her meds. So, she was not exactly rational about the whole thing, not that I blame her.

I eventually came to the realization that he addressed every single issue we brought up with him. He ignored nothing we said and is looking at everything. He was skeptical about CRPS, because it certainly shouldn’t present quite like she does. So it might be a that she has CRPS but the full body pain might be down to Central Sensitization or neuropathy or something else entirely. He seemed to know a little bit about EDS – certainly he knew the connection between EDS and POTS – so we will have to see what level of knowledge he has as time goes by.

The thing that really got me, was that he wanted to see her back in 4 months. When we actually scheduled that appointment, I realized it will be 2 weeks shy of a year since this all started. A year of this torture and no relief in sight. I know the wheels of medicine turn slowly and that we need time to get all of this testing done before we see him again, but knowing we are at least 4 months from any help was absolutely crushing.

So, we are getting all of this testing scheduled  and completed and just hunkering down and trying to survive.


Rare Disease Day 2016



Today is Rare Disease Day, with a focus on Making the Voice of Rare Diseases Heard.

Usually, I write a post on the theme because I feel strongly about this. Ironically, I am feeling overwhelmed by actually living with our allotment of rare diseases and just don’t have the brain power to write something brilliant.

I can share some links that will help you share some awareness on social media. Use your platform to share information because giving a voice to this cause is important. Make your voice heard and support the Rare Disease Community!


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What are you doing to promote awareness today? Leave a comment and share!


Solving the Mystery (or starting to, anyway)

I think the mystery episodes Emily is having are slowly becoming a little more clear, although the “why” is less clear at the moment. Em’s EEG is scheduled for Monday and that should (hopefully, fingers crossed) give us some clarity. In the meantime, we are taking notes, gathering information, getting them on video, and doing our own research. It is very possible that we will know nothing after the EEG and she will need further tests, but, I am hopeful the information we are gathering will be useful for the doctor and get us closer to answers.

She has had more than twenty of these episodes since the 9th of February. Now they have evolved into two distinct types – in one, she goes limp and can’t talk or move for a couple minutes. As soon as she can move, she starts having trouble breathing. Sometimes that is minor, sometimes it is scary and lasts a (relatively) long time. For the most part, she is conscious during these but there are times that she is less “there” and even forgets to breathe. Even though this doesn’t perfectly fit with any specific type of seizure,  I am more and more convinced this is some type of seizure, particularly since she has added myoclonic jerks as part of the episodes. And there have been several times where her head drops or falls to the side, depending on how she is sitting/laying, during the episode.

The new type, which she has started having, seems to clearly fit under the umbrella of simple partial seizures – at least from what I am reading. In the middle of a conversation, she suddenly complains of being exhausted, then her vision changes – she says there is a ball of light between her eyes and the room around her is distorted and rather scary. She says she has a sense of anxiety (maybe more like doom) and paranoia during this.  She has several myoclonic jerks and either her leg, hand, or thumb twitches repeatedly. After a couple minutes (or less) her vision returns to normal and she is exhausted and groggy. Once she lays down for a few minutes, she is totally back to normal and is laughing, talking and generally herself again – anxiety is gone and she carries on. She is totally aware and conscious during these episodes and is able to respond, although she is distressed while they are happening so far. From what I have read, this sounds exactly like a sensory seizure to me, aka focal seizures.

We, of course, are now wondering if this really is all new or if she has been doing some of this all along. If I had to bet money, I would say it is definitely related to the concussion but it is possible that some of this has been around for awhile and it was so subtle that we didn’t see it. I have never actually seen her have an absence (petite mal) seizure but she thinks she might be. And she could be right.

I have learned a whole bunch about seizures in the last few days  – it is all very interesting, although I would really rather not have one more thing to learn about, to be honest. The most interesting thing I learned is that there is a category of autonomic seizures which fall under the umbrella of simple partial seizures. This could explain why her heart rate is bouncing around like crazy during these episodes – it goes from 125 to 85 in the space of a few seconds. (I don’t even know when the last time her HR was that low. It is typically 100 at rest, so 85 seems crazy low for her.) And her oxygen level bounces between 99 and 89 during the go-limp-and-can’t-move-episodes .

In particular, this is interesting in light of one episode (which may be a third distinct type or may just be an anomaly) she had the other night. She overheated (doing absolutely nothing) and felt awful. She had to have an ice pack to cool off (which led to her CRPS pain worsening but whatever) and her right hand was trembling like crazy. Then her thumb twitched for about 30 straight seconds. She had a couple myoclonic jerks then was fine. Back to being normal and said she was hungry. So, is that Dysautonomia or an autonomic seizure? It isn’t anything we have seen before in 5 years of dealing with dysautonomia, that I am sure of. Hopefully we can figure it out soon.

The why is the key here, I think. Is it from the head injury? Is it epilepsy? Is it from CCI? Is it from Dysautonomia? Is it something else entirely? Who knows at this point. But, I am feeling more and more sure that we are dealing with some (probably several) type of seizures and I am hopeful that, once we pin labels down, we can actually treat these.

We are not going to be surprised if she ends up with a big seizure. I hate to even think about that possibility but I am trying to being realistic as I watch the situation evolve and the myoclonic jerks increase. Some of what she is doing can be an aura for a complex seizure so we are just prepared (as prepared as we possibly can be) if it happens and really, really, REALLY hoping it doesn’t. Our goal is to keep her as safe as possible and protect her brain from further injury. She is getting sick and tired of being hovered over but there are some precautions that are just sensible until we get a handle on this. She is very grumpy over the fact that she has to be sleep deprived for her EEG and I am not looking forward to that whole process but we have to find out what is going on so we can get her back on track.

The good news is, her memory has slowly improved and speech therapy is going well. Both therapists she has seen come highly recommended by her dad and she loves them both. And they love her. She is enjoying the therapy and it seems to be helping, even though she is definitely going through some ups and downs that are challenging. It would be nice if her progress could be straight forward, but, that doesn’t seem to be in the cards right now.

With luck, we will know more Monday. Either way, I will post an update when I know more. 🙂

Catching Up and Hanging On: Part 3 Shoulder Woes

To recap: a lot going on… concussion and post concussion symptoms, possible CSF leak, recurrent ear infections. Lots of doctors. Blah. Blah. Blah. Here are Part 1 and Part 2 if you want the longer versions. 

As I have mentioned the brain/ neurological stuff has us concerned but there are always other issues to make things even more interesting. In November, Em screwed up her shoulder. It had been an ongoing issue, one injury after the other and one day it just quit. Got an MRI and it showed tendinopathy, which is a fancy way of saying tendinitis. Basically she has no movement in that shoulder that does not cause pain and has been in a sling day and night since. External rotation is excruciating. There was no evident/major tear on the MRI but we got her in to the Ortho to find out how to proceed.

If you have read for a while, you might recall that Em is rather needle averse. She will do pretty much whatever she needs to do to avoid any and all sharp objects. But, she wasn’t surprised and didn’t object when they offered her a cortisone shot. Well, she did tell the PA who did the shot that he was rude as he inserted the needle. She had been told it wouldn’t really hurt, just a little pressure, and she found that to be not precisely true. He laughed and told her that he was almost done and she could say whatever she needed to say to get through. Which was not a wise offer in my opinion, considering I never know what might come out of her mouth at any given time – and she was suffering from a concussion so he got off lucky, I think! Later she asked how far the needle went in and I told her she really didn’t want to know. She said it felt like he shoved it into her joint and I told her that is sort of the point of the shot. She was not impressed!

Anyway, he didn’t hold out much hope that the shot would do much on its own but he sent her to PT and was hopeful that the shot would help PT be a little less painful and more effective. In all honesty, I don’t think the shot did anything – she has had no noticeable pain relief, but then again who knows what it would be like if she didn’t have the shot. It was definitely worth a try and I was so proud of her for braving it. She pushes through an awful lot on a daily basis but this was kind of a big deal.

On to therapy… She had her eval on the 31st. She is seeing “her” therapist, who has pretty much been with her from the beginning. So, while he is admittedly not an EDS expert, he knows Emily. And we trust him. Her eval revealed nothing we didn’t already know. Her shoulders are extremely unstable. They slip in and out with pretty much every movement. That makes rehabbing her shoulder challenging. She will not tolerate normal rehab; for now, she has very simple isometric exercises that are done with me stabilizing her shoulder.

Her exercises:

  1. arm stabilized and in ‘neutral’ position by her side so she can do a few reps of pushing up, down, in and out. These are isometric exercises so we are talking tiny movements. Doing 5 leaves her winded. Doing 10 exhausts her as if she had just lifted her own body weight. We have pushed up to doing 10 but it is more important for her to do 5 good ones than 10 sloppy ones.
  2. With her arm/ shoulder stabilized, she does a few reps of external rotation. This is very painful. The goal is tiny movements to stay below that pain threshold as much as possible.
  3. with arm/ shoulder stabilized, she squeezes her shoulder blades together. Sounds much easier than it is.
  4. We added to the first exercise at her last appointment – we are going to bring her arm out of neutral, to maybe 20 degrees and try the isometric movements. That one is going to be rough.
  5. When her arm is out of the sling, which he does want her to do as much as possible when feasible, he wants her to stretch her arm out straight, gently move her wrist and fingers, and bend it a few times. That last part is incredibly taxing. She shakes as if she was lifting 100 pounds. Clearly, her muscles are weak.

Unfortunately, her other shoulder is nearly as bad. There is no injury but it is weak and it is suffering from compensating for her left shoulder. We are doing exercises on both sides and hoping for the best.

Everybody we have seen while she has been in the sling has made a big deal of her getting out of it because her shoulder will freeze up. We kept telling the doctors that the problem is her arm falls out of the joint if it is not supported. It is unstable enough that freezing up starts to sound good at this point. (I exaggerate there a bit, freezing up is all too possible and we don’t want that either, but you probably get what I mean.) We felt pretty vindicated when the PT was so concerned about her instability and told her to keep it in the sling. She should, and does, take it out but it has to be supported when not in the sling. So, she either holds it in place with her other hand or props it up with pillows.

The Ortho PA did say if PT doesn’t help, the doctor might need to go in and tighten things up. I didn’t go into it then, knowing we will cross that bridge when we come to it, but surgery is not a good option. Seriously. I mean, it could absolutely become necessary and we may be facing that choice down the road but we are going to do everything humanly possible to avoid it, because once we go down that road, I fear we will never stop. And that doesn’t even bring the issue of CRPS into the mix and how bad an idea surgery would be in that regard. I am only talking about the reality that tightening an EDS joint invariably will fail in a year or two or five and she would be considered lucky to get that much time out of a repair. And yet, we could find ourselves in a position where possibly gaining a year or two of function might start looking mighty good.

Suffice it to say, her shoulders are not good. We feel we are in good hands, both with the Ortho and the PT. We follow up with the Ortho (last time she just saw his PA) on the 18th and hopefully she will be making some real progress in therapy by then. I think it is safe to assume this will be a long, hard, grueling process. There is no easy fix and she is willing to do what she needs to do to get better.

We are concerned about her neck. Of course, wearing a sling is hard on her neck. Her muscles are already tight and it is just getting worse. As I mentioned in Part 2, the Concussion Expert wants her to do stretches to loosen up her neck and  hopefully ease her headache. She can’t really do any of the stretches he suggested, so I have been massaging her trigger points hoping to ease the tightness. Unfortunately, that has led to her neck being rather scarily unstable. When those trigger points are released, she ends up a bobble head. And that is not good. It is a matter of constantly juggling one issue with another and trying to figure out how to manage them both; often, they are competing problems with solutions that cause direct complications for another issue. It feels like we just go round and round.

But, we have a plan and know where we need to be headed. Everything else is just part of the journey. We have been down a similar road before and made it through; there is every reason to believe this time will be no different!

A Win: Finally

Picking up the tale where I left off in yesterday’s post, we were in desperate need of someone to help. Em’s pain was out of control, the Rheumatologist was clearly not interested in helping, and we had wasted 3 months seeking help from doctors who had no interest in helping.

I was done, with the whole situation. Done waiting. Done playing nice. And, I knew that our best chance of getting some help in the near future was from the Pain Management doctor. Yes, he had rather foolishly and unhelpfully stated “if it isn’t broke, don’t fix it” at our last appointment but we have always liked him and he has had Em’s pain under control for 3 years now. So, if it wasn’t him, we were going to have a problem.

I have been researching, talking to others living with CRPS, and formulating a plan of sorts. I will be the first to admit that it may not be a perfect plan, but it is far and away better than anything anyone else has come up with to date. So, I prepared for our appointment and was ready to fight for my plan, while being open to any constructive ideas.

My plan involves two parallel tracks. The first track involves getting to a neurologist who actually knows CRPS. If he has an opinion about Small Fiber Neuropathy, so much the better. We need someone who can advise and guide us, from a position of actual, current knowledge not a position of vaguely remembering learning about CRPS 20 years ago. In a CRPS facebook group,  I heard a name of a doc at OSU whose interest is in CRPS and autonomic dysfunction. I have no idea how good he actually is but it is a name and that is the best I have right now. So, I wanted a referral.

The second track is Ketamine Infusions. This is a very big gun but we are facing a very big enemy. A big gun is absolutely necessary, in my opinion. We know how serious a treatment this is but everything I am seeing says this is our best hope of getting Em into remission. There is no guarantee that it will but I believe that it is our best shot. Because her CRPS (assuming that is what she has) is full body, some of the other treatments are just not options. Furthermore, she is already on many of the meds that would be the first choices to treat it, so our pharmaceutical options are a little limited. Narcotics are not the answer – we need to stop the pain cycle, not drug her. So, Ketamine. (And, may I say, this is the hardest thing about parenting a medically complex kid. The doctors can’t or won’t make these decisions so the parent is left to push for it. All I can do is pray that my instincts are correct.)

At our last visit, I mentioned Ketamine and he was not receptive to it. But, this time I was determined to have a serious conversation. This time, I also had a name of a doctor in Indianapolis who does Ketamine Infusions for CRPS and I was asking for a referral to him. However, I decided that it would be better all around for us to have Dr. V do this treatment at our own hospital – it would be easier on Em to travel less and it would benefit others with CRPS in the area if he can offer this treatment. It seemed wise to push for Dr. V to investigate/ learn how to do the infusions but have the referral request as a back up plan.

So, I had printed off both referral forms for both the neurologist and the other Pain Doctor – I had them filled out as much as I could and had all the paperwork requested attached. All they had to do was fill out their part and fax them. I must admit, this was a stroke of brilliance on my part. 🙂

I also had numerous articles about Ketamine and how this is the treatment of choice, how to actually do the infusions, billing codes (inpatient, not outpatient as we would be hoping for but it proved my point that this is a legit treatment), and a page of contact info of several docs who do these infusion who Dr. V could consult with.

I was ready to fight. To throw a fit. To get hysterical. Whatever it took, this was the day.

We got there and I was already a little discouraged – they were clearly swamped and running late and I worried that we would be brushed off. The NP, who we have normally been seeing for the past year or so, came into the room, her normal cheery self. She took one look at us and knew something was wrong. She sat down and listened intently. As soon as I mentioned Ketamine infusions, she said, “you are going to have to talk to the doctor. I can’t help you.” I expected this, but I had anticipated that we would have to make another appointment. Instead, she fetched him immediately and he sat down with us ready to talk about Ketamine. He expressed his concerns but was open to discussing it. I handed him articles about this treatment and he skimmed through them right in front of me, and made comments that indicated he was actually absorbing the info. He thanked me for doing so much leg work for him.

He stepped out to make a phone call, saying he was getting his billing people to look up billing codes. We discussed it further and we agreed that there were some obstacles to over come – insurance being one. He suggested oral Ketamine as a more immediate option. He stepped out again. I think he talked to someone about finding a compounding pharmacy to prepare it. I am hoping he figures out that Ketamine lozenges are a real option – I know a couple folks who are getting real relief from them.

So, at this point, he is willing to look into infusions. He knows the doc in Indianapolis who I was going to ask to be referred to and can talk to him about how to do the infusions and billing stuff and hopefully make it happen. Not tomorrow, but hopefully soon. And that was as much as I could hope for.

In the meantime, he wanted to try a couple new meds. Cymbalta and Nucynta. And he upped one of her current meds to try and get her some immediate relief. I am not thrilled with adding two more meds but I am very thankful he understood that she needed something NOW. We will just cross our fingers that she tolerates these meds and they don’t cause more problems. Serotonin syndrome is a big concern but we are watching her closely and it is a calculated risk.

He agreed to refer to the neurologist at OSU – the sooner the better because there is always a waiting list. We will fight to get her in asap but we have to get the referral first. He seemed surprised that I had a name, because he has had no one to refer patients to. This could be positive for other patients as well.

He spent an hour with us, on a day when he was not scheduled to see us and when he had patients already backed up in his waiting room. We didn’t get around to talking about what to do about her shoulder and we didn’t get around to talking about a plan on how to handle future ER visits if her pain is out of control. And, we left the whole Ketamine issue a little more open ended than I wanted. He is going to look into it – hopefully he will call with answers, but I can call the office and ask if I don’t hear from him. All things considered, I was so grateful for the time he spent with us and for him taking the issue seriously, that I would not complain at all.

I feel bad that the other patients were waiting so long, especially the gentleman who was already mad about his billing situation and entertainingly vocal about it, but, honestly, Em has been juggled and passed along and ignored for 3 months while living with the most painful condition known. It was our turn to be focused on, to be cared for, and it was, quite frankly, overdue. After the awful ER visit, after being dumped by the Rheumatologist, after months of everyone just saying, “Good Luck”, we needed a win. And this was a win.

In reality, we are no further ahead than we were before Friday – although two new meds to try is progress. We still have no answers, no diagnosis, no obvious path to treatment. But, we have a sketchy plan, a name of someone who might help, a treatment option that wasn’t an option before, and we have a doc who cares. And we have hope. Which we were pretty short on before this visit. So, while we technically aren’t further ahead, it feels like we traveled miles on this journey in one afternoon. Because one doctor went out of his way to help us.

That shouldn’t be amazing. It shouldn’t be a novelty. But it is. And we are very, very thankful.

The Latest in Our Ongoing Adventures

Things have improved, slightly, since our awful visit to the ER. Not her pain, that is still uncontrolled and terrible, but we are a few steps closer to getting answers or at least some real treatment.

MRI Results

Em’s MRI was last Wednesday and it actually showed a problem. Turns out, she has “Tendinopathy of the Supraspinatus Tendon” which means that tendon is either irritated or torn. Putting it in simpler terms, she has a rotator cuff injury. Personally, I think it is a tear, since her description is that she had it resting behind her head in bed and all of the sudden something popped, it went numb, fell down and she couldn’t lift it. Unfortunately, it keeps getting re-injured in her sleep and the slightest movement away from her body causes awful pain and makes it go numb again. We are figuring out what to do about it. The doctor who ordered the MRI probably will not be the doctor who manages the problem, so we are going to have to get her to the ortho ourselves.

And, we have resisted the urge to pop into the ER and see if our “favorite” NP is there and do a we-told-you-so dance at her. Bottom line here, is that Em needed an MRI. We were right, the NP was wrong. X-rays would not have shown a thing and would have only hurt her more and possibly made it worse. (Em is emphatic that she knows that the would have made her move her shoulder in ways that would have hurt and maybe damaged it more. At this point, I am inclined to believe her) And, if she had just gone ahead and ordered an MRI while we were there, we would be a week ahead of figuring out how to treat it.

So, we are feeling pretty validated after finding out the results. That validation comes at a pretty high cost though. However, it does confirm that, generally, we know what we are talking about and that will make facing down rude doctors (or NPs) a little easier. Confidence is  good thing.


The Rheumatologist ordered some bloodwork – mostly to check for Vit B deficiencies – when we were there in November. We had to do it at our local hospital and not Children’s for insurance reasons, and I think the Rheumatologist still doesn’t have the results. I am not quite sure if it is their fault or the hospital’s fault but it is ridiculous. I am not happy with any of them, to be honest. My husband finally just called and got the results. All but one is fine – it looks like her B6 is low – which could contribute to neuropathy. But, again, the doctor who ordered these doesn’t seem to want to manage this, so we are pretty much on our own with dealing with this.


We really liked him – he was a great guy and all – but he seems to be washing his hands of Em. He prescribed a med called Trileptol, in the hopes it would help her pain. As she was titrating up to a full dose, just a couple days into the process, it was helping her pain but it messed with her head in a pretty significant way. She has always been “feisty” but this med made her uncharacteristically angry and unreasonable. And I won’t say she was suicidal, but let’s just say she was talking in a way I had never heard from her before. It was pretty scary. And dealing with it was  complicated because this was all taking place the day before Thanksgiving, the day Em asked to go to the ER. She stopped taking it and the nurse I talked to tried without success to talk to the doc about it. She finally called back, having not been able to talk to him, and said don’t take it anymore and that she would make sure the on call team knew about the issue if we needed more help.

The office eventually called back Monday – after the holiday and the weekend – with his thoughts. He said that med shouldn’t cause mood changes (if that is true, why did the info from the pharamacy say it does and to inform your doc?) and that she should go back on it and titrate up more slowly. Riiiggghhhttt.

So, yeah, Em will not be taking that med anymore. Haven’t heard back from him and the nurse – I don’t know if she was just reading from our appointment summary or if he gave her new notes – informed me during that call that we should just go to the Pain Management doc to get more pain relief and to “find someone to refer us to someone who can investigate a diagnosis of Small Fiber Neuropathy”. That makes me insane: “Oh, I think you don’t have that unusual disease that you think you have, I think it is this one, which is far more rare and nearly impossible to diagnose. And I have no plans whatsoever to help you get that diagnosis.” Hence my belief that he is washing his hands of us. Lovely.

By this point, I was starting to get fairly annoyed. Or possibly livid. Possibly ready to go to war and/ or all Mama Bear on the next medical person we encountered.  The doctor we waited two months to see had nothing to offer and dumped us. That was the final straw. Every single doctor we had seen about the CRPS had played hot potato with this kid. She was tossed to the next guy with a polite smile and a hope that he would do something. And, if that didn’t happen, what did it matter once we were out of their office?

We had a pain management appointment on the 4th and I decided it was going to be our line in the sand. If I had to throw a gigantic fit, he was going to do something constructive to help my child. That appointment deserves its own post, so that will be up tomorrow. It was good and we so needed good right now.

Another Ear Infection

After a very long PM visit on Friday, Em said we needed to get her ear looked at and, since we were right there, we popped into Urgent Care. I don’t think I mentioned here that, while we were at the Rheumatology visit on November 19th, they looked at her ear and said she had a perforated ear drum. At least the doctor doing the exam was pretty sure it was – she looked at it a long time and said she was pretty sure it was perforated and to make sure to keep the ENT visit that is scheduled for the 15th.  So that ear is just an ongoing issue.

Weirdly, which is her norm, during the MRI on Wednesday, the noise made her ear suddenly pop and hurt. Since then, she wasn’t able to hear out of that ear. She knew she wasn’t going to make it another week and a half until the appointment, so we popped into Urgent Care.

Good thing we did. The poor doctor looked in her ear. And looked. And looked some more. Then said – and you just LOVE to hear this as a parent – “I’ve never quite seen anything like this before.” She couldn’t even see the ear drum because there was “white powdery stuff” blocking the view. She grabbed the other doctor to come in and look. Fortunately, this was the same doctor we saw last time and, not surprisingly, she remembered us.

Long story short, it is either a fungal infection (Em asked, “if I google spores, will I be freaked out?” They told her not to google it.) or fluid that has crystallized from the ear canal after the ear drum perforated. It was very fortunate that we knew her ear drum was perforated as of the 19th, because that means ear drops are a no no. Initially, they apologized that they had nothing to offer and said they would tell the ENT to see her asap. They came back and said they had ear drops as a suspension fluid that she could use, so, we have been doing ear drops 4 times a day again. And, I am currently waiting on a call from the ENT. I doubt they will see her today yet, but hopefully they can soon.

We are hanging in there. A lot is happening. We still have few answers but are slowly getting there. I will post about the pain management visit tomorrow.

To the Nurse Practitioner who saw my daughter in the ER Thanksgiving Eve:

To the Nurse Practitioner who saw my daughter in the ER Thanksgiving Eve:

It was the day before Thanksgiving. You probably didn’t want to be there any more than we did. But, I am willing to bet that the time we spent in the ER  left more of an impression on us than it did you. And, I have a few things to say to you.

My daughter – my beautiful, smart, precious daughter who has lived for the last 6 years in such chronic pain that would put you and most other “normals” on the floor, who has had her childhood ripped away by a genetic condition, who has experienced loss and suffering and affliction, the likes of which you can probably only imagine, yet does not view herself as a victim and refuses to be defined by her medical condition – came to you for help. For the first time in 6 years, she asked to go to the ER because she was in such pain.

It was complicated – with her it always is. She is medically complex. A bemusing mash up of chronic and acute issues is what we threw at you that day. That is not what you normally see and it was not clear cut. I get it. I even sympathize, a little. But, this is our daily reality and frankly, I don’t think it is too much to ask for you to do right by a kid who is desperate for help.

I tried to peel back the layers for you – she has Ehlers Danlos Syndrome, she has autonomic dysfunction, and she has something else – probably Complex Regional Pain Syndrome, the most painful condition know to medicine.

It was her shoulder that brought us in to the ER that day. It had dislocated August 1st. That injury started the CRPS, which quickly spread to her whole body and the shoulder has been a worsening problem ever since. We had seen the Rheumatologist a week earlier and he was very concerned about that shoulder, because she couldn’t move it after it simply gave out a couple days earlier. The earliest an MRI could be scheduled was December 2nd. She reinjured it in her sleep and the pain was just too much.

And she asked to go to the ER. Because she needed help. We thought the least you could do was go ahead with an MRI, so we could figure out the source of the pain or if there was any damage. And, I was hoping for something to help get her pain under control so she could participate in our family Thanksgiving.

We weren’t drug seeking. I flat out told you we didn’t want another prescription. She already has pain meds that aren’t working. I don’t want to throw narcotics at her. But, when your child is shaking and pale and nauseous because of her pain and has been for days, and when she has had a bad reaction to the pain medication the Rheumatologist prescribed and for the first time you hear her say ‘death would be easier” and you honestly don’t know if it is the side effects or the pain, you ask for help.

What we got instead was attitude. You were dismissive from the moment you walked into the room. We were probably defensive – living with a rare chronic illness that is poorly understood by the medical community will do that to you. This may come as a surprise to you, but the ER is the last place we wanted to be on Thanksgiving Eve.

You asked, rather snidely, what we expected you to do.  Let me tell you now: I expected you to listen. To show some compassion. To offer ideas. To order an MRI. To show some humanity.

Did my daughter communicate effectively? No, she didn’t. Because she was in profound pain. Agony. She is trying her very best to manage a handful of medical challenges that make adults weep. Of course she didn’t communicate well.

She is still learning to advocate for herself appropriately and effectively. All of the past negative experiences with doctors have built a wall that has to be dismantled if you want to really reach her. Furthermore, her medical conditions cause brain fog and anxiety: she has trouble finding the right word, particularly when stressed and in pain. Complicating all of this is that the new med that was supposed to help, instead turned her brain to an angry mush of aggression and depression and she was not in a good place.

And she is a child,  a scared little girl, a prisoner of her own body,  tortured by her own central nervous system and her genes, who decided to take a risk and ask for help.

You, on the other hand, are the adult. And the professional. The onus of communication is on you. You could have shown some sympathy. You could have listened to her. You could have walked in the room not assuming we were drug seeking. You could have acknowledged her case was complex and confusing and made an effort to understand the challenges that we live with on a daily basis. Instead, you copped an attitude and acted surprised when things went downhill.

You begrudgingly offered an X-ray instead of the MRI that we all knew was necessary. Perhaps if you had been a little more compassionate and actually allowed her to express herself, if you had asked questions and cared about the answers, you would have been included in the conversation that followed. Instead, my daughter, and you would have understood why if you had listened to her, acted like the frightened and angry child she was and refused your x-ray. She may only be 16 but she knows perfectly well that not one of the multitude of x-rays she has had has EVER shown any conclusive results. She knows how much it hurts to have X-rays when you are in pain. She also knew you thought she was drug seeking. And she wanted to leave and never lay eyes on you again. She has zero tolerance for crap on a good day and this was so not a good day.

In the meantime, I was desperately texting an SOS to my husband who was finishing up his work day, three floors up in the hospital. When you walked back in the room, prepared to lay into my child for refusing the x-ray, you were obviously taken aback by a hospital employee that you know, standing in the room and standing up for his daughter. I can only imagine how things would have gone if he hadn’t shown up. I am pretty sure it would have involved my daughter telling you what you could do with both your condescension and your x-ray as she stormed out, with me juggling purse and coats and trying to salvage the situation.

Instead, you perhaps understood for the first time, that we weren’t random people who were crazy and stupid, and you changed your tune a bit. I cannot tell you how sad I am for your other patients who are also not random, crazy, stupid people but don’t know someone who works at the hospital and cannot salvage their visit at all. I mean, you were still pretty snide, but you finally offered a shot of morphine or a pain patch. Emily chose the pain patch just so she could go home and get away from you, vowing to never return, no matter how dire the situation. (That should scare you as much as it scares me.)

Forgive me if I am not overly grateful for the bone you threw us: if you had actually known about EDS like you pretended to, you would have been aware that she has fragile skin and the adhesive on the pain patch could be a serious issue, which it  has. And, I’d probably be more grateful if they had helped, which they haven’t.

This particular day was just another stop on a very long and winding road. We have had some really, really good doctors over the past 6 years. We have had some really bad doctors throughout this journey. The bad ones leave such deep scars that the good ones can barely get near us for our reflexive flinching at a white coat, like a battered and abused child who never knows if the outstretched hand will be gentle or inflict pain.

Unfortunately, you inflicted a wound that will leave a scar. One that will make it harder for her to ever ask for help again. The next time she needs help, she will be more likely to just give up and suffer. And that is potentially deadly. That scar makes my already difficult job parenting a medically complex child that much more difficult. I am angry. I am frustrated. Mostly, I am sad and tired from fighting this fight alone. But, I have sworn a solemn oath to this child that I will never stop fighting for her. I cannot and will not give up.

At the end of the day, we have only our own reaction to this experience – it is the only thing we can control. So, we have decided to turn this into a positive: We will use this experience to empower Emily by helping her learn to communicate better in the future.   And, instead of feeling victimized by this experience, we will have a discussion with the head of the ER. Not to get you in trouble or exact a pound of flesh, but to wrangle some good from a bad situation by educating. We have a unique opportunity to educate and advocate for awareness of EDS and CRPS – if we step outside of our comfort zone and reach out to the ER staff in good faith, it will benefit Emily and possibly countless other chronic illness patients.

We are determined to learn and grow and find some measure of good in this bleak situation and I can only hope you might be willing to do the same. Please, next time you encounter someone like my daughter – hurting, scared, scarred and a little angry – do better. Your actions matter and my daughter, and all of the other chronically ill patients who pass through your hands, deserve better.





Update on CRPS, Shoulder Injury and Ear Infections

A lot has been going on, so I will will try to catch everyone up…

On November 19th, we saw the Rheumatologist (Dr. Henrickson) at Cincinnati Children’s. On the upside, we liked him. We liked the Rheumatology Fellow who talked to Em and took her history. Dr. H said we were educated on Em’s issues and thus in a good position to beat this. When I mentioned being in an CRPS group on facebook, he didn’t chide – he just said our knowledge was useful. That earned him points, let me tell you!

We felt that they took her pain seriously and listened to what she had to say. He added a med to try and help with her pain, Trileptal. She has to titrate up to a full dose over the next week or so, but she thinks the tiny dose she is currently on is helping. That gives us hope that the full dose will help. Which is good because she is in incredible amounts of pain every moment of every day. I have never seen her in pain like this, not even back in the days before she had any pain management. The situation is getting dire and there are no easy answers. I doubt they have a clue how much pain she is in, because she has gotten very good at putting up a front to hide her real desperation. But, they took her as seriously as they could and I appreciate that.

As far as an actual diagnosis, we still don’t have one. He essentially (my best interpretation of what he said) believes that once Complex Regional Pain Syndrome spreads from one region to the whole body, that it is no longer CRPS, that it becomes “something” else. He suggested that, depending on what some of the testing we are doing, the more likely diagnosis is Small Fiber Sensory Neuropathy. The problem is, Cincinnati Children’s can’t do the testing for that. Only a few hospitals in the country can, so this is another rare diagnosis that would be challenging to pursue.

My problem is, as far as I can see, Small Fiber Neuropathy really doesn’t fit her symptoms, not as a whole, anyway. She would be a very atypical case if that is what we are looking at. Emily said that she feels like for the first time a doctor has ignored the more likely explanation and opted for the LESS likely, MORE rare explanation. And, when it comes to treatment for SFN, it seems that it is a matter of addressing the underlying cause (diabetes, for example) and controlling it with pain meds. If, what she has is actually CRPS instead, treatment for SNF would mean the CRPS would go untreated and that would be a very bad thing. So, I am just not buying this idea of SNF – in my heart, I believe she has CRPS, which has rapidly gone full body, and needs aggressive treatment to break the pain cycle that her central nervous system has developed.

Complicating our visit and his understanding of her pain, was a new shoulder injury. The left shoulder, the original dislocation that started the CRPS spread in the first place, has been a problem off and on since. A couple days before our appointment, her shoulder just sort of gave out. Went numb and floppy. She put it in a sling because that has helped in the past but instead of getting better, it got worse over the next couple days. Usually, this type of injury gets better after a few days. Not this time. By the time we saw the Rheumatologist three days after the injury, she couldn’t move it and it was causing her severe pain.

So, he wants her to have an MRI. We have one scheduled for the 2nd. In the meantime, she is profoundly miserable and I think she may have really messed her shoulder up this time.. The pain, a combination of CRPS pain and a damaged shoulder that hurts with every slight movement, is so severe that she shakes and gets nauseated from it. She doesn’t want to, but we may end up at the ER to try to get her pain under control. With luck, they could do an MRI right there so we don’t have to wait until Dec. 2nd. The situation is getting desperate, to be honest. She should not have to be in this amount of pain.

The MRI will help us know what is going on with the shoulder, and he ordered some bloodwork to check on vitamin deficiencies that could cause neuropathic pain. Baring no useful results there, he wants her to do PT and desensitization. If that doesn’t help, pursue SNF testing.

As much as we liked this doctor, I am not loving his plan. I think she needs to be seen by someone – a neurologist who knows both CRPS and SNF. If this is CRPS it is crucial we start treating it – the longer it takes, the worse her prognosis. I am weighing our options and figuring out a path that will take us where we need to go. The treatments for CRPS will not hamper treatment for SNF if it turns out to be the true diagnosis, but, from what I can see, treatment for SNF would not adequately treat CRPS if that is what she has. It seems prudent to pursue the diagnosis/treatment we can now and wait to investigate SNF down the road.

Basically, everything is still up in the air and we have no more answers than we had to begin with. Except the exam at that appointment revealed that she has a perforated ear drum. They either missed it at Urgent Care or it happened since she was last there. We have an ENT appointment set up for the 15th, but her ears are so painful and she has developed vertigo, so we may end up back at Urgent Care in the meantime.

We are tired. And stretched thin.  And scared. And frustrated that we are not being offered any real help in what we know to be a desperate fight for her future.

But, we are hanging in there and at least have an idea of where we need to go. For now, we are just taking this journey one step at a time.



Nervember: CRPS Awareness Month

CRPS help us
November, er, Nervember, is nerve pain awareness month and CRPS is certainly a pain, so we are doing our part in raising awareness. Today, the first Monday of November, is Color the World Orange Day – a day to specifically raise awareness about CRPS/RSD. Orange is the awareness color of CRPS so I am wearing orange today, even though it is the one color I would be least likely to ever willingly wear. Funny side note: Emily has detested the wearing of zebra anything as EDS awareness, which is weird, because who doesn’t like zebra print? Emily, apparently. But, she likes orange much better than zebra stripes so I guess that is a silver lining with all of this. She finally has an awareness color she is ok with.

Anyway, as usual, I will be sharing information about CRPS on my facebook page and here. Sometimes I think about all I do with Facebook is raise awareness but I am grateful to have that opportunity. Because heaven knows people need to know about CRPS. EDS is genetic but anyone can develop CRPS from the most minor, trivial injury.

CRPS ribbon daughter


So, while I am here, I will give you a little update on Em.

We recently saw Dr. Neilson (I plan on writing a post about some of the things we talked about – there were some things that might be of interest to anyone with EDS) and brought up the inordinately long wait to see the rheumatologist (when I made the appointment I was told the earliest she could be seen was January 29th which was insane considering this is an extremely time sensitive diagnosis). The good news was that a mistake was made in scheduling and we don’t have to wait until the end of January to be seen. The bad (frustrating, annoying, pull out my hair in disbelief) news was that we could have already been seen if they had scheduled it right in the first place. 😡

Moving on. We now have an appointment for November 19 and it can’t get here soon enough. If what she is experiencing is truly a full body CRPS spread (and I don’t think there is any other explanation), it is breathtakingly fast. If CRPS is like having a limb set on fire, what she is experiencing is a wild fire, eating up acreage at a frightening rate. I am trying to keep a timeline and a list of her symptoms as they pop up and we are trying to document the physical signs that we see. She has had some swelling but not much at this point and it is fairly subtle when it happens. Her hands and feet will turn bright red and shiny, then, when we try to take a picture, she moves and the color fades. But, I think it is important to document everything we can so we keep trying.

She is absolutely exhausted but is trying so hard to push through this and not give in. The symptoms she is experiencing are so frustrating: worsening brain fog, memory loss, insomnia, incredible pain and, unsurprisingly, irritability. November 19th can’t come soon enough, I tell you. Although, let’s be honest, there is no guarantee that appointment will yield any useful treatment. Which is daunting. But, I would rather face that possibility as soon as possible rather than wait until the end of January to find out. But, something has to be done. I have promised her that we will not stop until she has real treatment that brings her real relief.




Why I Fight For Invisible Illness Awareness


You would think it would be obvious to me, of all people and after all we have dealt with over the past 5 years. It should be obvious that you can’t ‘see’ an invisible illness, right?

EmilyAnd it is, of course. As a parent of a medically complex teenager who lives with Ehlers Danlos Syndrome and POTS and a host of other things that haven’t quite been given a label, I am too familiar with the concept that my kid is ill in ways that even the doctors can’t see. I have felt the questions behind the polite smiles that come after I have explained Emily’s medical issues, when that person catches sight of her smiling and laughing and looking oh-so-normal. I see the doubt that dawns when well meaning friends and family wonder if she is really quite as ill as I claim she is. Because, how can she smile, laugh, walk, play if she is really in so much pain?

I understand those doubts, even when I am annoyed by them. It is all part of the process of spreading awareness and educating people who are not trying to be mean but are facing the equivalent of a calculus equation in a social interaction. If you don’t live with chronic illness, you just don’t get it.  I understand that. I have no problem explaining it over and over and I have been a willing advocate from the moment we put a name to her symptoms.

And I have never once, not for a single moment on this journey of 5 years, doubted what I could not see. I have been bemused, but I have never doubted her experience. I think that is because it really isn’t invisible to me: I see the pasted-on smile, the pallor, the way the pain shadows her face as she tries to ignore it, the blank look and glassy eyes as she starts blacking out and I see her energy draining like a low battery as she fights to appear normal. It isn’t invisible to me, even though it is to everyone else. In fairness, I know what all of those signs will result in: utter exhaustion and perhaps days of recuperating from what any other teenager does all day long, every day. I do not doubt because I see it when others do not.

I get this no-man’s-land of invisible illness, really I do.

But, even a veteran like myself can be reminded how insidious these hidden conditions are in a simple, matter-of-fact conversation…

My daughter’s CRPS (Chronic Regional Pain Syndrome, also known as Reflex Sympathetic Dystrophy or RSD) has returned and with a vengeance that is frightening: it has spread to her whole body in a couple short months. If you are unfamiliar with CRPS/RSD, it typically appears after a minor injury or surgery and is extremely painful. It is the most painful condition known, rating a 46.5 on the McGill Pain Index, considerably above first time, unprepared childbirth at and cutting off a finger. Think filling your limb with lighter fluid and setting it on fire and it never burning itself out. There are some visible symptoms that go along with it but, of course, you cannot see the horrific pain.

She had this once before in her foot 5 years ago. It was excruciating. She couldn’t bear to wear a sock or shoe and couldn’t walk on it. It actually put her in a wheelchair. The whole concept of chronic pain was relatively new to her then. She had been in a lot of pain from EDS for months but the CRPS pain was so extreme and so horrible that she couldn’t ignore it.

Fast forward to this new round of torture. I believe her. 100%. I am her biggest advocate and she has my solemn promise that I will never stop seeking treatment for her. But, it occurred to me that, perhaps, compared to the first round of CRPS, this round was just very unpleasant because it was all over, instead of just in her foot. Because she informs me of how things feel but she doesn’t complain. She doesn’t cry or scream or shout about it. She pushes through, knowing that she can’t give in to the pain and that staying in bed is the worst thing she can do. In short, she doesn’t show her pain, even to me. When she talks about it, it is typically very factual, informative and brief.

So, I asked her, the other day, as we walked together, “Is this pain less than it was the first time?” I thought I knew the answer because, logically it had to be, right? And, honestly, because I wanted it to be so.

She thought a minute, shrugged her shoulder and quietly said, “It’s worse.” She walked on, leaving me stuttering and a bit flabbergasted. I caught up with her and said, ‘But, last time, you couldn’t walk. You couldn’t stand a shoe or even a sock on your foot.” I remember staring dumbly at her shoe encased foot, as I said this, trying to make my brain understand. Again, she just shrugged and nodded and walked on.

And my brain caught up. She has endured so much pain over the past few years. It has been managed but it never goes away. When you live with constant, severe chronic pain, you learn to bear the unbearable. That first round of CRPS was unbearable to her, but, now, years later, she is prepared to face much worse, stoically and with a poise that belies her age and situation. So, the answer is, of course, yes, it is worse. Yes, she can wear a shoe and walk, because she has learned that she has too. She understands that this pain is lying to her: it is not a signal of damage so she must push through if she wants to defeat it.

It doesn’t matter if I can see it or if anyone else can see it. It is real.

But, if I, her biggest believer and advocate, can be confused and deceived by the false appearance of ‘”normal”, of course, other people – friends, family, doctors, therapists – can be deceived as well. Some disabilities are invisible and they are no less real because someone else can’t see the pain. People like Emily hide their pain because if they wore it on their sleeves the weight of it would make them collapse. People like Emily smile to shield their loved ones from the knowledge that they suffer profoundly. People like Emily push through because they have no choice and they push through even though it is agonizing. People like Emily bear the unbearable with little help from medicine or society.

This is why I fight for awareness. Because even when you live with it, it isn’t easy to understand.

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