"Suffering the Slings and Arrows of Outrageous Fortune"

Posts tagged ‘pain’

An Update: Neurology, Ophthalmology, EMG

Been awhile since I posted. Honestly, it has been pretty blah around here but maybe we are finally, slowly taking a few steps in the right direction.

~ Em’s CSF leak healed up without intervention, so we were able to avoid the ER. (She actually just refused to go and it healed up on its own.) She has had a couple weeks of feeling better now, so maybe the leak is done? I kind of doubt it but we can hope.

~ We will be seeing a new local neurologist tomorrow, since her NP, who has been seeing her for 4 years and originally prescribed Diamox, has left. That is a little nerve wracking but hopefully the new doc is able to continue to help.

~ Last week, we saw the ophthalmologist for Em’s double vision. She has a prescription for glasses that he says will help – reduce eye strain, reduce double vision and make reading/ seeing easier. She should have the glasses in a week or so. Praying they help her.

~ She had her EMG to investigate the numbness in her legs. It was, of course, normal. Everything is always normal. It was such a letdown – the tech assured us we would have answers before we left that day. The neurologist (the one at OSU we are seeing to figure out what the heck is going on with her pain, etc) did the deep part of the test and pronounced everything normal and that was that. Well, that was AN answer but certainly not THE answer. The test itself was no big deal – it wasn’t painful and she had no problems with it. She did have one bruise on her hip but other than that it was not a big deal.

~ We followed up with him yesterday. It was a better appointment, thank God. He is convinced her issues are all autonomic based and has a few options to try. An abdominal binder, compression stockings, a new med. I have my doubts but I guess if we need to jump through these hoops to rule stuff out, so be it.  He is backing off ordering the tilt table test – he finally heard us when we said going off all her meds was too big of a challenge right now. We are going ahead with the 24 hour holter monitor and will get that set up. We will follow up with him in 4 months. The one answer we did get yesterday is that he does not believe Em has MS. So, that is something. A relief for her and me too.

 

So, maybe some baby steps in the right direction.

Inpatient EEG Monitoring

Finally…

We will be heading to OSU bright and early on Wednesday the 18th to begin the inpatient EEG monitoring that will hopefully rule in or, more likely in my opinion, rule out seizures. Clearly, something is going on that looks like seizures but I now have doubts that they are epileptic in origin. As I previously shared, I really believe Em is experiencing Complex Migraines – which can look a lot like seizures or even a stroke.

This stay has been a LONG time coming. These episodes started in February, after her concussion. She had a few months of Speech Therapy, which did seem to help a lot. Mid April, she said it felt like her brain was waking up and she was functioning better. The episodes stopped and she was able to do some school work and generally was doing better.

Then, she bumped her head again – again, a minor bump, but it was enough to cause a problem – and a week later the episodes started back up. I don’t think that is a coincidence. I feel certain they will be able to trigger the episodes and get some good information and a direction to go in.

We aren’t exactly looking forward to this – we are prepared for some serious unpleasantness. She will have an IV, can only be up in her room with supervision, and they will most likely deprive her of sleep to try and trigger a seizure. Basically, it is going to be a lot like the regular EEG that she had – only over days instead of an hour. That was not a ton of fun but she survived.

Insurance approved us for 3 days, rather than the max of 5. That shouldn’t be a problem since her episodes are pretty easy to trigger. I hope, anyway! One positive, we will have wifi, so maybe between strobe tests and hyperventilation and keeping her awake, I will be able to post during our stay.

I just pray that we will get some answers for going through  this unpleasant experience. This kid really deserves a break!

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.

 

Who Knew Ears Could Be So Fun?

Last Tuesday, we had our long awaited visit with the neurologist at OSU. It is complicated and we are still absorbing it. I will post later this week about it – I think my head is finally to the point that I can write about it.

But the fun, and I say that with the greatest sarcasm, never stops around here. A couple weeks ago, Em dislocated her thumb badly  (as only an EDSer can)  and we are probably going to have to get it looked at. Not getting better on its own and isn’t likely to, I think.

Then, on Friday, we were coming home from Speech Therapy and had just pulled into our driveway, when Em suddenly felt a loud pop in her ear and got very dizzy. We knew there was fluid behind her ear drum but had been told to just keep an eye on it. Honestly, it never stopped hurting – she just didn’t complain much about it in the last couple weeks because she had so many things going on:  something like an ear ache just doesn’t register very high on the priority list when everything is falling apart.

I called the ENT – it was late on Friday afternoon so I really didn’t think there was any chance of him seeing her and I found out he wasn’t in anyway so it was a wasted call. The nurse said to just go to Urgent Care. We were both too tired to deal with it on Friday but when her ear still hurt and she was still so dizzy on Saturday, off we went.

Sure enough, her ear drum is perforated. Again. She is on antibiotics and I guess we just wait it out. She has an appointment with the ENT in April, but I guess if it continues to be a problem I can call and hope he can see her earlier. Clearly, something is going on with that ear – not sure what it is and no one seems terribly interested in figuring it out. At least the perforation is documented and we know to be very careful about getting fluid in her ear for awhile. (I still think she had a CSF leak in her ear and that was the fluid buildup, but what do I know?)

The funny part about our Urgent Care visit, is that the Neurology Nurse Practicioner who has seen her for 4 1/2 years (and is the one who prescribed her Diamox) works over there on weekends and he is the one who saw her. So, we had a little impromptu neurology visit and were able to update him on what all has happened since we last saw him. I was still pretty ticked about the fact that he tried to send Em to psychiatry for her seizure episodes but I let that pass and opted not to strangle him or anything. We are getting the episodes addressed and he is fine with the course we are on (not that it would matter if he wasn’t, tbh) so it wasn’t a bridge that needed burning on that day.

I will update more about the episodes in the near future as well, since the new neuro is addressing them.  Brian did earn back a few points when he asked what antibiotics she could safely take. He knows she is complex and I appreciated that. And he totally understood when I said anything but the fleuroquinilones. I might be able to eventually forgive him assuming her seizures are psychogenic before attempting to rule them out. Maybe.

So, anyway, another perforated ear drum – the second since November. Like she needs another problem.

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!

Catching Up and Hanging on: Part 2 Concussion

Part 1 can be read here.

As we waited to get to the concussion experts – and fortunately we did not have to wait long – Emily’s concussion symptoms remained concerning. She is having trouble reading and comprehending, she struggled to write – to spell and remember the mechanics of language. As we passed the 3 week mark of her head injury, she was not getting better and, while she was perhaps not getting worse, more deficits were being revealed. I am not sure we even know the extent of her deficits at this point. When she is searching for a specific word, we go through a very entertaining version of verbal charades where she tries to make me understand what she is trying to say, with sometimes hard to follow logic and obscure literary references. Yesterday, she tried to use a story from the Little House on the Prairie books, to get to the word “fraternize”. We got there but, man, it was a circuitous journey! As always, we are still able to laugh at ourselves and the situation.

At the Concussion Clinic, we saw a rehab doctor, a neurologist and a doctor who I believe was a fellow, which is very common in our experience at Children’s. The exam revealed that her short term memory is crap, to put it plainly. She was given 3 words to remember. Maybe 5 minutes later she was able, with some effort, to pull up the first one, “green”. She was eventually able to guess the second word, “liberty” when given a prompt that it was another word for freedom. She could not recall the third, “automobile” even when given a prompt that it was a word for getting around. She guessed “car”, was told it was something like that and guessed “bus”. She remembered the word when he said it. She also took a fair amount of time to be able to say when her birthday is, which is not normal and is something she answers every single time she is at the doctor.

Her balance is off – she struggled to walk a straight line. Her eyes are not right – she either showed nystagmus or saccadic oscillations/ interruptions. (Her eyes were jerky when tracking side to side; I was able to clearly see them from where I was sitting. There was some discussion about this – I was familiar with nystagmus but had to look up “saccadic”. I was able to do so, even though it was a totally unfamiliar word, because we had a funny moment when Emily thought the doctor called her “psychotic”, “saccadic” does sound similar. He laughed and told her he would never call her psychotic to her face. We liked him quite a bit. 🙂 So, I was able to find what they said, I just don’t know the significance of it.)

So, they all agree that she did have a concussion and she has post concussive symptoms. Likely the fact that she (probably) had a CSF leak made her brain extra vulnerable to injury at the time because there was less cushion to protect it. They were concerned with the Diamox contributing to her CSF volume, but she was not taking it at the time of her injury. It was a day or two later that she started taking it again. They have referred us to speech therapy where they will assess her and do cognitive therapy to help get her brain back in shape. We are going to do that at our local hospital and they were fine with that. We will go back in a month and they will do neuropsych testing to further evaluate her, particularly if therapy is not helping as much or as quickly as we would like.

They were understandably concerned with the number of meds she is on. Frankly, they can join the club. Of course we are concerned and, yet, this is what has made her functional and removing any of them seems insane. They didn’t push that and said they would not mess with her meds since she has other docs overseeing them. I was thankful for that. Very thankful. Particularly that they left the Diamox alone.

The neurologist, through no fault of her own, should have come with a trigger warning. She was nice enough but she had the same basic advice that we got at the headache clinic at Cincy 4 years ago: eat your vegetables and drink your milk. Once again, her point is absolutely correct, as it was 4 years ago. Eating well and nourishing your body is vital to wellness. Unfortunately, that gets a little complex when one has a handful of medical conditions that make eating/digesting hard. Furthermore, when we mention that Emily is lactose intolerant and that detail is ignored because it goes against their standard protocol, it makes her a little tiny bit furious. That day, she was exhausted, hurting, scared and now this “chick” is telling her to drink milk. That said chick was a little heavy on barking orders and light on (visible) compassion did not help. Em has a, ahem,  less than favorable view of her but, I know she was just doing her job. And we have the same goal – to help Em. Once they left Em let loose about her and didn’t even laugh at my favorite medical joke: How are God and a neurologist different? God doesn’t think he is a neurologist. She did say she wanted to go give her neurologist at home a hug for not being a jerk and taking good care of her. And, presumably not telling her to drink milk.

We were suitably impressed with them over all; I feel like she is in good hands. However, I am not sure they truly understand her complexity. My guess is they don’t see a ton of (diagnosed) EDS patients in their clinic and so would have little reason to be familiar with it. I mean, you can understand the general mechanics of connective tissue and not fully grasp the entirety of what EDS will do to a person or how the simplest thing is a huge challenge.

They knew her shoulder was bad, she was wearing a sling – the MRI report was in her records, but it was like they didn’t quite grasp how unstable it is, how much it hurts and how much the CRPS pain affects her. The Rehab Doc was concerned over the tightness/knots in her neck and shoulders, because that typically is seen with concussions and contributes to the headache. What we were not able to really communicate is that that is not new – that is her baseline. And when we relieve those knots or muscle spasms, the adjoining joints often are unstabilized. Could that tension be contributing to her “concussion headache”? Sure. Is it going to be easily managed? Probably not. He wanted her to do a few stretches, some of which she cannot do because of her shoulder. In the end, he told us to talk to her PT and have him work on addressing it. The PT was incredulous that the doc would have her doing anything with her hypermobile neck and said, “Look, I don’t want to tell you to he is wrong, but don’t do any of that. We have to protect your neck. And you can tell him I said so.”

Once again, we are trying to navigate this ocean of conflicting advice. We just do our own research, try to know what we need to know and make an educated decision. We tend to go with the advice that makes the most sense at any given time and aligns the most with our understanding of all of the pieces of the puzzle. We also tend to go with the advice from the professionals who know her best.

So, the situation is at least as serious as I feared and perhaps more. It was really hard to see her struggle and see the fear and frustration on her face when asked to do a task she couldn’t do. But, concussions can take months to recover from and it is entirely possible that we will see her brain heal spontaneously over the next few weeks. Therapy will help and my husband, as always, has handpicked the best therapist for Em and her situation. We are optimistic and hopeful that she will get better in the near future.

 

 

Continued in Part Three: Shoulder…. Because that is a thing. Just not quite as big a thing as her brain.

 

 

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.

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.

 

 

 

 

One more thing…

Thursday morning, Em  told me her ear was feeling funny when she woke. She had tried to gently clean it out, she used a Q-tip (just the tip, didn’t even insert the cushioned part all the way) but heard a big pop and it hurt a lot. She couldn’t hear out of that ear, so, although it seemed unlikely, I was concerned that she somehow managed to perforate her ear drum or that it was infected.

So, we talked about it and waited a little while to see if it got better and discussed it some more. And, decided to head to Urgent Care. Her ears were filled with wax so they had to clean them out.

(We had an entertaining moment when the nurse casually mentioned she was going to “shoot” water into Em’s ears, while holding, what looked to Em, to be a gigantic hypodermic needle. It, of course, was not a needle, but Em thought it was. I don’t know which she was more concerned about – getting “shot” in the ear with water or getting “a shot” in her ears. The nurse hastened to assure her there was no shot of any kind forthcoming – it was not a needle but soft plastic and the water would be gently squirted into her ears. We all got a good laugh out of that little misunderstanding. It is possible that I thought it was funnier than Em did but she did laugh.)

At that point, I was hopeful that her ear drum was fine, there was no infection and just cleaning out the wax was going to be the answer.

But, once the doc was able to look in her ears, the painful right one was infected. At 16 years old, she can now say she has had an ear infection. For all of her issues over the years, one thing that was never an issue was ear infections –  I am pretty sure this is her first. And my son only had one – when I was in the hospital recovering from my hysterectomy, my husband brought him to visit and he confided that his ears hurt. They trooped down stairs to Urgent Care and, sure enough, he had a double ear infection at the most inconvenient possible time. So, this ear infection thing is a little foreign to me – I didn’t even deal with Luke’s because I was in the hospital.

Seems odd, having an ear infection out of the blue like this, at her age. Is it just a one off , a fluke accompanying some virus? Is it an indication that her immune system is down? Is it a sign of problems to come? Who knows.

All I know is that she feels awful. Her ear hurts. Her head hurts and noise is painful. She isn’t sleeping well. She can’t eat. The antibiotics are making her more nauseated that usual. It has been 4 days and she still feels absolutely terrible. She is being referred to an ENT and I think that is probably a good idea. She is just so complicated and this is just one more thing to deal with on top of everything else.

 

Why I Fight For Invisible Illness Awareness

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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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