EEG Results

Last week, Em finally had her inpatient EEG monitoring. It wasn’t awful but it certainly wasn’t a whole lot of fun. I think I will do a post in the next few days recapping all the gory details of what that stay was like – since I have found that this issue is pretty common among EDSers. Today I will just focus on the results of that stay…

So, the “events” Em has been having are not epilepsy. That is good news, truly,  although in some ways, epilepsy treatment is more straightforward. The bad news is that they diagnosed her with Psychogenic Non-Epileptic Spells. We didn’t exactly love the neurologist and we certainly don’t love that diagnosis. I have been trying to avoid that diagnosis because I know how hard it is going to make Em’s life in the future. My fear is that, going forward, every new symptom will be chalked up to being in her head. EDSers already are viewed as fakers and crazies. We really don’t need this.

I still believe that a very possible cause of these events are complex migraines. That diagnosis fits at least as well as PNES (in my oinion, at least) but this neurologist disregarded it completely, because, well that is what neurologists do. In fact, she flat out said it was impossible because if Em was having migraines, it would have showed up on the EEG. When she said that, my BS meter started beeping like crazy.

Having looked it up after the fact, I was right to be skeptical – migraines are never diagnosed with an EEG and it is by no means certain that one would even show up on the EEG. They disregarded our descriptions of a headache afterwards and the visual symptoms Em experiences during the events. The doctor threw me a bone and promised to include the fact that these events are precipitated by a headache in her report. Which isn’t even true (the events themselves cause the headaches) but she ignored my objections.

So here is what we are left with: Either we accept this diagnosis and risk ignoring a real medical condition or we reject this diagnosis and risk ignoring a real psychological condition.  Both are risky and dangerous. And for sure, accepting the psychological diagnosis means rejecting the possibility that these episodes have any other explanation. We are on shaky ground with this. Remember Justina Pelletier? This diagnosis is under the same Conversion Disorder umbrella that was slapped on Justina when she was medically kidnapped. I would be lying if I said that doesn’t evoke some serious anxiety.

Our plan right now is to pursue both options. We will seek a therapist who might be able to address the emotional issues involved – for sure Em is dealing with more than enough to cause detrimental stress. Whether this will be specifically to address a conversion disorder or simply to help her learn how to cope with the crap hand she has been dealt, remains to be seen.

At the same time, we will pursue a headache specialist who can help us figure out if this is a type of migraine and hopefully provide some treatment.

The neurologist grudgingly acknowledged that we should find someone to treat Em’s headaches so I am going to take her at her word and do what I think is wise. I suspect they saw what they expected to see so I am reluctant to accept their findings without some skepticism.

Bottom line, I have a responsibility to advocate for Em and make sure she is getting the treatment she needs, whatever that may be and in spite of obstacles put in our way.

I will no doubt post more about this diagnosis in the near future and I will post about our general experience of the inpatient stay in case it might help others who are going down the same path. In the meantime, we are just trying to recover from our hospital stay and trying to absorb the results.

 

 

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!

Seizures or Complex Migraine?

Since February 9th, Em has had 75 episodes that are seizure-like. Sometimes she has several a day. Sometimes she goes a few days between episodes.

They look like a seizure but certainly not exactly. Now, it is possible that some of these episodes could be a type of seizure and the upcoming EEG monitoring should help us figure that out. But, I got a suggestion that tipped me off to what I think is a more likely possibility and I am very VERY grateful to Paulette for suggesting it because I suspect it would have been a very long time before the doctors would have figured it out.

Complex Migraine (or Migraine with Aura) is what I am now convinced this is. Apparently, the aura of a migraine can look very much like a seizure or even a stroke. I think we didn’t notice the headache that typically follows the episodes because, let’s be honest, she pretty much always has a headache. Even when she complained that her headache was horrible afterwards – well, not only does she always have a headache, but headaches often follow a seizure, so it seemed to make sense. But, after doing some research, it makes more sense that this is a Migraine with Aura. Furthermore, I am wondering if she is specifically having basilar-type migraines, which would fall under the umbrella of Complex Migraine.

Initially, I was convinced the episodes were triggered by cervical instability – the first episode was in the car while she was wearing her hard collar because her neck was so unstable. She complained of her vision being “weird”, then was unable to move or talk and was in and out of consciousness – I thought she had just fallen asleep. She was barely able to walk once we got home 30 minutes later, then the symptoms resolved completely and she was fine. (Well, not fine, but the point is the symptoms wholly resolved until the next episode.) By the 4th episode, my records show her describing feeling like her head was “sitting on a burning spike”. Another time, she described the pain like her head was “expanding like a balloon when she breathes”. Looking back, she says she almost always had a worse headache after these episodes.

The length of a migraine aura is 5 to 60 minutes, while seizures typically last 2 or 3 minutes. Her episodes tend to last between 5 and 30 minutes – the longest being far too long for a seizure but right in the range of a complex migraine. In the early episodes, she tended to go limp for several minutes. Eventually, the episodes started looking more like a tonic clonic seizure – but not quite as violent. Just a lot of twitching and jerking and twisting stiffly and then going limp, often repeatedly. She often has trouble speaking, swallowing, and breathing during the episodes. Sometimes her eyes are closed, sometimes her eyes are open and  dart back and forth the whole time. She sometimes has a black horizontal line through her visual field that eventually fades.  She often can’t move her legs for several minutes after. Often, she is aware during, but goes in and out of consciousness. She is dazed, confused, and dizzy for 5 to 15 minutes after.

The headache part is hard because she has such layers of headaches. She has been dealing with high pressure headaches, then low pressure after the LP, then bouncing back and forth. So it has been hard to sift through all of that. But, she has been having what we now believe are legit migraines and they mostly follow these episodes, although she has had a few without the aura. They don’t respond to Diamox, she is very sensitive to light and sound, she is nauseated and the headache eventually passes a few hours later. She sometimes has multiple episodes in a day that result in the headache never going away and just getting worse with each episode. The headache is bilateral and originates at the base of her skull and wraps around to her temples. (These points are what makes me suspect a basilar-type migraine. If I am right, then my initial suspicion of brain stem involvement wasn’t entirely wrong.)

I have been keeping detailed records of the episodes but it finally occurred to me to keep track of her headache pain before and after the episodes. Typically, her headache is a 2 or 3 before and a 6 or 7 immediately after, increasing to an 8 or 9 within a few minutes.

These episodes are now mostly triggered by flashing lights: police lights, strobe lights, sunlight flickering through trees while driving, etc. There is one stretch of road on our way to Speech Therapy that is all woods – if it is a sunny day, she is almost guaranteed an episode. Watching TV shows with flashing lights on NetFlix often ends up triggering an episode. One episode was started by police lights that went by our house. (I never, ever realized how many flashing lights are in this world until now. It is ridiculous!) Fluorescent lighting is problematic, although I don’t think she has ever had an episode specifically triggered by it. She can sometimes avoid an episode by avoiding flashing lights: when driving or watching TV, she can avert or close her eyes and not go there. Not fool proof, but she does have some control. Sleep deprivation also seems to trigger them and sometimes they just happen with no obvious trigger.

The concussion from December probably has a lot to do with these episodes, in my opinion. I think the EEG monitoring will help us figure out any actual seizure involvement, hopefully. I do know that it is possible that the episodes that are more seizure-like and not quite fitting into the Complex Migraine symptoms might not be triggered during the monitoring or that epileptic activity might be harder to catch, so we may not have a conclusion on all of this. The important thing is that we get expert eyes on her. Epilepsy experts and hopefully migraine experts who really know the complexity that can exist  and don’t try to slap a psychogenic label on her.

And, yes, there is a bit of irony here. Early in this journey, we were sent to a Headache expert who idiotically insisted Em was having migraines. We *knew* she wasn’t. We suspected high ICP and just wanted to try Diamox. Eventually we found a doctor who did prescribe Diamox and it made all the difference. So, for all these years, I have battled constantly to keep the right focus on her headaches.

But the situation has changed. I believe that damn concussion has radically changed the landscape and now we are dealing with a whole slate of new issues. So, if this is migraines, so be it and let’s address it. For that matter, if it is seizures, so be it and let’s address it.

I am hopeful that if we get the right people looking at this, we can figure it out and get her some relief. THAT is the important issue.

Finally, I just want to say how much I appreciate everyone who reads, comments and shares. Not only is it crucial for all of us to know that we are not alone on our journeys, but we all have knowledge that can help someone else who is struggling. My hope has always been that sharing our experiences here would help someone else but we have been so blessed by hearing about other’s experiences as well. We would be lost without you, truly. 🙂

 

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.

 

A Letter To Emily’s Mom

This is a piece I wrote last year for the Our Stories of Strength anthology, Living with Ehlers-Danlos Syndrome. I am posting it here as a reminder to myself that we will get through our current struggles and as encouragement for all the moms out there who are facing a diagnosis and are scared to death. We will get through this together and I am thankful for everyone who has walked this journey with us.

 

Dear Emily’s Mom,

Everything is going to be okay. Really, it is.

Yes, I know Emily – your baby girl – has Ehlers-Danlos Syndrome and it seems like the end of the world. I know she is only 11 years old and she is losing everything that matters in her life and your heart is breaking for her and for yourself because your world has been unceremoniously turned upside down. Life as you knew it is a memory and a new life, an uncertain and daunting one, is looming.

Yes, I know she got EDS from her daddy and her brother has it too and you are thinking if only you had known when she was younger, maybe things wouldn’t have to be this bad for her. You are also feeling relief to finally have answers about all the strange symptoms she has had since she was a baby, while simultaneously knowing that relief is a bizarre emotion to be feeling with such a diagnosis.

Yes, I know you are terrified. I know you feel utterly unqualified to deal with this, guilty for not knowing sooner, and heartbroken to see your daughter suffering so. I know you can’t see where this path will lead and I know you hate not being in control.

Five years down the trail from where you sit now, broken and grieving at the beginning of the journey, I can see where you are going and where I have been. Which is why I can say, with certainty,  that everything is going to turn out fine. Truly it is.

Take a moment to breathe and absorb that truth. Everything is going to be okay.

That belief will be sorely tested in the days to come. You are going to have moments where you doubt, where you will not be able to see down the path you are on; moments where it won’t seem possible that the world will ever be right again. But, everything is going to be okay.

I promise.

Of course, “everything is going to be okay” doesn’t mean things will be easy or that events will fall out according to your liking. Spoiler Alert: Events will very rarely, if ever, fall out according to your liking. Honestly, what you think should happen is not always what needs to happen and that will be a hard lesson to learn… One of many hard lessons you will learn, I hate to say.

Somehow, though, even when things don’t go your way, things still turn out okay. When I say “everything will be okay”, what I mean is that, even in the most difficult circumstances, you will be able to honestly say ‘it is well with my soul’. You won’t get there over night, but you will get there.

Faith is at the heart of this journey and it will be your greatest weapon against all of the challenges and uncertainty you face. You will learn that you aren’t in control, no matter how much you want to be, and that you can either be driven crazy by that or simply believe God has more knowledge than you do so you can trust his plans for you. The circumstances you face are going to make you more vulnerable and more adrift than you ever have been and you will survive by leaning heavily on the promise in Romans that tells of how God is working to make all things – even EDS – good. What’s more, you will see that promise come true, time and time again. Soon, you will even come to see the journey as a gift.

Believe it or not, you are being given the amazing opportunity to become more…

More faithful. More patient. More compassionate. More real. More human. You will have the privilege of making friends and meeting people who you otherwise never would have met – a hidden world of people suffering from invisible, chronic illness will be revealed to you and you will be blessed by that revelation and by them.

You will learn the beauty of service: both serving the ones you love and being served by others who care about you. You will gain knowledge and have experiences that will shape you as a human being; a better human being than you were before. You will weep and laugh, pray and think, and learn and grow. It will be painful and profound, but you will find your purpose on this planet and you will grow in love and compassion.  This whole crazy awful business is a precious opportunity to evaluate your priorities, shore up relationships and get rid of all the unnecessary stuff in your life.

No, I know it doesn’t feel like an opportunity right now, it feels more like a punishment. But you aren’t being punished! Rain falls on the righteous and the unrighteous alike. You will witness God working through those bad things and transforming them into something beautiful. And, trust me, the beauty will come. Just watch for it. It will come in big, spectacular ways, in tiny, gentle whispers and though rainbows peeking through the storm clouds. It will come…

Now, I won’t lie…this journey you are on will not be easy. When I talk of the beauty to come, it might be hard to believe when the darkness is pressing in on all sides. Those moments are real and it is okay to despair, to grieve, to doubt – give yourself permission to feel whatever it is that you feel. You don’t have to always see the beauty of the journey – there are parts of it that are just plain hard. When things are rough, just cling to the hope that beauty will be coming during those dark times. That hope will be your anchor.

You are facing the fight of your life – there is no point in sugar coating it. From my vantage point, way down the road from where you are, I can look back and see the steep, rocky trail ahead of you, the pitfalls, the detours, the sleepless nights and the awful times when you are just going to have to sit tight and be patient. However, I can also see the molding of your character and the resilience that comes from the hard work of walking the path you are on; I can see the beauty being wrought from the ashes and the rainbows that have been scattered along the way.

From your vantage point, at the starting line of this marathon, you see only the unknown and it is terrifying. You have every right to be afraid. In fact, fear will be a tool to harness on your journey, but what you need to know is that fear, in the end, is not what will define your journey. Love is.

I know you will struggle to believe this in the beginning, but  the destination is worth the challenges of the journey. The journey will not destroy you. It will make you stronger than you ever thought you could be. You and yours will not only survive this journey – you will thrive.

Here’s another Spoiler Alert: Emily is alright. More than alright, in fact.

She is an amazing young lady. She is not defined nor defeated by her diagnosis. She has faced some pretty tough times and is a fierce fighter – you call her your hero and she has earned that title. And, tough as she is, she still has love and compassion aplenty. She is strong, smart, talented and funny. Your fears that she would not be able to withstand this challenge are completely unfounded. Just as your character is being shaped by this journey, so, too, is hers and, while you wish she could be trouble free, you know that is just the wishful thinking of a parent.  EDS is simply part of her life and she is learning to rise admirably to the challenges that she will face for the rest of her life.

Sure, there is the day-to-day EDS stuff to deal with – the dislocations, injuries, the pain, the headaches, the autonomic stuff. Unfortunately, those never go away. But, somehow, you both will adapt to all of that and learn to roll with it. Like I said, EDS does not define nor defeat her or you, for that matter.

You will find some good medical care along the way and you will find that willingness to think outside the box will serve you well. Family, friends, and faith will keep you strong. You will make mistakes but when you do you will learn from them and you will hone your knowledge to become a formidable advocate. And your daughter will learn how to advocate for herself from your example.

Long story short: you can do this.

I am still looking towards an unknown future so I can’t tell you how the journey ends. We have a long way to go and I have no crystal ball from here on out. What I can tell you, with confidence, is that, while I fully expect troubles because troubles are part of life,  I also have confidence that there will be rainbows that accompany the storms, weeping will turn to joy, God’s  mercies are new each morning and beauty will come from the ashes of the most difficult situation. So, whatever troubles are lurking around the next bend in the road, I know we will be alright.

And so will you.

Hang in there,

Beth (Emily’s Mom)

 

 

 

 

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.

CRPS/ RSD

I don’t remember how much I talked about it back then, but 5 years ago, as we were waiting to see Dr. Tinkle to get a diagnosis of EDS, Emily rolled her foot and ended up with RSD.

(Reflex Sympathetic Dystrophy (also called CRPS, Chronic Regional Pain Syndrome) is an extremely painful condition where the body interprets every sensation – even harmless ones – as excruciatingly painful. The best description is that your limbs feel like they are filled with lighter fluid and have been set on fire. It is rated as the most painful condition, higher than cutting your finger off and unprepared, non-medicated first time childbirth. It typically starts after a relatively minor injury and can be in one limb or travel to the entire body. It is difficult to treat, if only because it is poorly understood.)

We were very fortunate to get a diagnosis quickly and were able to start desensitization to get it under control. Then, one evening as we were doing a desensitization technique, it went away as quickly as it started. Almost as if a switch had been thrown then turned off. Sometimes when she re-injures that foot, the RSD wants to creep back in but we have been able to fight it off all these years…

Then, a few weeks ago, Emily dislocated her shoulder. Within a couple weeks, the RSD pain (it is very distinct and Emily easily differentiates between it and her other layers of pain) had started in that shoulder. Then, within days it traveled down her arm to her fingers. Then it spread to her other arm, from shoulder to fingers. Then it went to both legs: the pain goes from the tips of her toes to above her knees – some days it is up to her hips. In the last week or so, it started in her face and in her scalp. So, now she has full body CRPS/RSD – basically the nightmare scenario. It is crucial to get it diagnosed and treated as quickly as possible for the best chance of remission.

Unfortunately, she is already on several meds used to treat it and they are obviously not helping. The upside of that means we probably won’t have to waste time trialing meds that probably wouldn’t help anyway. The downside is that our treatments options are now in the next category, which is a lot more complicated.

We got in to her pain doctor and he was less than helpful. I think it was just so far beyond him or maybe we caught him off guard but he didn’t exactly rise to the occasion. He started out offering her oxycodone, which A) probably wouldn’t help anyway and B) is not a step we were thrilled about. Then, somehow, he switched horses mid conversation and convinced himself this was just an EDS flare and suggested just keeping on with her current med regimen. Because, and I quote, “If it isn’t broke, don’t fix it.” Even when I pointed out that “it” is indeed broke and in dire need of fixing, he hunkered down and stuck with this Just-an-EDS-flare-that-will-pass idea. The only constructive idea he had was to increase her Lyrica. And he supported the idea of going to Cincinnati Children’s for treatment, mainly, I think because he just didn’t want to deal with it. Which works out well because, turns out, I really don’t want him to deal with it. Not with that attitude.

I have been back and forth with the geneticist’s office about this this past week. It was a shoulder dislocation that started this up and if it is connected with EDS, BCMH should cover it, so I need them to refer us for treatment or I would have just made an appointment myself. It took them abnormally long to respond so we have been waiting all week with a sense that the clock is ticking precious time away. Since those conversations are a work in progress and more complicated than I would like, I think I will save it for another post. Hopefully by this afternoon or tomorrow we will have a better idea of the direction we are going for treatment and I will post about that when I know more.

So, after a couple relatively quiet years, we are back on the roller coaster. And, I may have mentioned previously a time or two, I really don’t like roller coasters!

 

 

 

Our Stories of Strength

My copy of Our Stories of Strength: Living with Ehlers Danlos Syndrome was delivered yesterday. We are pretty excited about it around here. 🙂

 

My submission, A Letter to Emily’s Mom, is on page 163.

Whether you purchase a paperback copy or a kindle version, I highly recommend this book.

In addition to stories from EDSers of all ages, there is poetry, photos and artwork. I have to say, this book, and the anthologies that are to follow, are important. Books like this are critical for the EDS population: to uplift, encourage and to communicate that you are not alone. As I told my daughter: Read this. It was written for you.

Kudos to Mysti and Kendra for compiling a wonderful book that is a gift to the EDS community. The kindle version is handy to have on hand and the print version is beautiful – perfect for leaving on the coffee table as a conversation starter. I know we will read it, share it, and keep it on hand as a tool for helping raise awareness of what life with EDS is like.

Both the print version ($19.99) and the kindle version ($9.99) are available on Amazon.

I also want to say a word of appreciation to everyone who contributed to this book: the stories are beautiful and sad and touching and uplifting and, most of all, real. Thanks to everyone who put their hearts out there for the cause of reaching out to fellow EDSers. The result is amazing.

(If you are interested in contributing to upcoming anthologies, head over to ourstoriesofstrength.com  and see which books are scheduled for release and get information about submissions. You can also like their Facebook page and keep up-to-date on their news.)

Silver Linings…

Knee Update

Em had her MRI on Tuesday – we saw the doctor on Wednesday and got good news… the MRI showed that nothing is torn, so no surgery. He thinks the pop she heard was her patella being dislocated. So, while that explains the bruising, pain and instability, it is obviously long since back in place and now she just needs to heal up. On Wednesday when we saw the doctor, she was still in the wheelchair and in a lot of pain, although she had been able to bear weight on it intermittently over the weekend. Now she is bearing weight even without crutches and is just wearing a brace for stability.

We  were going to do PT, but at this point she is rapidly improving so her dad will probably just work with her at home and that will be good enough. She goes back in 4 weeks for a follow-up but I expect her to be fine by then. I am very, very relieved that we dodged yet another bullet and that she doesn’t have to go through another surgery.

Co-op

I have mentioned numerous times that we are part of a homeschool co-op that meets on Fridays and I have a couple things I want to mention, things that fall in the category of how things just happen to work out in ways we don’t expect but end up just right…

This won’t surprise anyone but Em is at place where she would just prefer to pretend she is a normal girl most days. At co-op, she has done her level best to pull off that illusion for the first few weeks. With lots of new families who weren’t around when she was really sick, she can sort of pull it off…until, of course, she does her knee and has to have her knee in an immobilizer and use a wheelchair to get around. Inevitably that requires some explanation and I suppose it is good practice for the real world. My observation is that good things come when she is honest and open about her issues – I think she sees that as well, but it is still difficult for her.

The first neat thing that happened the week that she was in her wheelchair is that she made a friend. One of the families at Co-op recently adopted a little guy from India. He speaks enough English to get by but, no doubt, feels set apart as he is adjusting to his new life. Not because he is viewed as anything but a blessing but more so because he has some physical limitations.

I am not sure if his issues are congenital or from an accident, but the bottom line is that he is in a wheelchair, at least for now. So, when he saw Em wheel in, his eyes lit up and he went out of his way to talk to her. He was no longer the only kid in a wheelchair and that had to be special for him. It was special for Emily to know she had made him feel more at ease because she certainly still struggles with the feeling of being different. And he was impressed with the gloves she wears to prevent the wheels from tearing up her hands. 🙂

Sometime blessings come when you least expect them: Em felt so discouraged being in her chair again but she ended up making another child feel less alone. Funny how that works.

The second neat thing that happened is that Em was talking to one of the moms and discovered that this lady, Maria, has a sister with EDS.

That is an It’s a Small World moment on its own, but when we saw her the next week,  Maria said she mentioned to her sister about Emily wanting to connect. The funny thing is, the sister asked if Emily’s mom has a blog because she recognized the details, having been following it. [Waves to Maria’s sister 🙂 ]

It is a small world and EDS isn’t nearly as rare as we have been led to believe, but I am still amazed when we run into this type of thing. We got a good laugh out of the fact that we already had a connection with Maria’s sister and just didn’t know it!

So, while Em being injured is never good, it is always interesting to see what good can come of a bad situation. When it is storming and clouds are all around, you may as well look for the silver lining!

 

 

 

 

Talking about EDS

There are times we struggle a bit with our knowledge of EDS ; we see symptoms in several people we know – in friends, in cast members at the theatre, and so on. (I am not even talking about family here – that is a whole other issue to deal with!)

For Em, it is very much a thing of simply not wanting to draw attention to herself; not wanting to be the sick girl and wanting to be normal. For me, it is a matter of wanting to protect Em’s privacy and of not wanting to have others think I am crazy when I bring it up. When I see it in others, the questions of ‘how much do I share?’ and ‘how do I say it?’ come up. How can you share such profound information in a casual situation without seeming like an obnoxious loon? On the other hand, how can you NOT share such life changing information with someone who may desperately need it?

It is an awkward but important decision to have to make.

I think each situation is a unique case, governed by the personalities involved and how well you are acquainted with that person. For instance, my daughter was convinced that one of her best friends has EDS and, at first, I thought she was blowing it out of all proportion. But, the more we talked and the more we discussed symptoms, the more she and her friend convinced me. Then it was up to me to talk to the other mom, who I have known for years. Not an easy conversation. But, it ended up being a good conversation and a productive one.  Do I know if this gal has EDS? Nope. But, I can point the parents in the right direction so they can find out and I can be a support for them no matter what they find out.

Then we have all of our friends at the theatre. From the start of rehearsals for The Sound of Music, we saw hypermobility everywhere. EVERYWHERE. It is like the theatre is an EDS magnet or something!

Now, just because someone is obviously hypermobile doesn’t mean they have EDS, so all we could do is wait and watch. When Em hurt her ankle Saturday before the show and we had to talk about her medical condition, the flood gates opened. At least 2 people we hadn’t even pegged as possible EDS, started getting very curious about what we were talking about because they have so many of the symptoms we mentioned. One I am almost positive has it, the other, very possibly but our conversation got interrupted and so I cannot be sure. (And that doesn’t include the gal my husband talked to and is convinced has it or the several others that we are suspicious of.)

That is where it gets so  awkward. It is a beautiful and wonderful thing to be able to reach out and share information that can help someone. BUT… It is an Incredibly Delicate Thing to essentially inform someone that they may have an incurable genetic condition. It takes some careful dancing around the topic and it is not pleasant in many ways.

But, again, how do you not at least share information with them? If you don’t, you are possibly preventing them from getting the care they need and wouldn’t you bear some responsibility for that? To have The Answer and not give it to them seems unconscionable. How can I, who wishes for nothing more than to have known about EDS before Em got so sick, not reach out to others who may have it? That is the rub. I don’t want to be the one to tell them, but, if I don’t, no one else will and I can’t live with that.

Like I said, each situation is unique. If you know the person well you can judge how best to bring it up, otherwise, you just have to wing it and pray. Unfortunately, we have had several of these conversations and I can share what I have learned because I am getting better at it…

• I tend to wait for an opening  that sort of naturally allows me to broach the topic, like Em’s injury. Even better if THEY open the door wide for you by saying something like ‘Oh, I can pop my hip out…watch me!’ For me, all bets are off at that point and they are fair game. That leads to questions like ‘can you touch your thumb to your forearm? Are your joints unstable?’
• I always, always follow up mentioning loose/unstable joints with the other symptoms. Something like: “Because collagen is all over the body, if it is defective, it can cause problems throughout the body. For instance, the digestive system is mostly collagen, so someone with EDS can have gut problems – IBS, pain, trouble eating, poor appetite. And, the autonomic nervous system can be affected, so they get dizzy when they stand up and maybe even faint. Headaches, neck pain, eye problems, jaw problems, dental problems, chronic fatigue… the list goes on and on…” Often, at that point, their jaw drops because I have just named most of their problems.
• At that point, I might ask “Is this something you might be interested in learning more about? If you are, I can give you more information and answer any questions you might have.” This gives them an out, if it is too much at that moment and gives them time to absorb this new information. If they say yes, I print off a couple articles to share with them and give them links, so they can educate themselves. If they want to pursue it further, I can give them the number for the connective tissue clinic at Cincinnati Children’s.
• I also try to hit the highlights of intervention, so that even if they don’t choose to pursue a diagnosis, they can be aware of the big issues – protect your joints, don’t do those tricks unless it is for a doctor. If POTS is in play, drink lots of water and consider getting it checked out by a doctor. The one gal talked about how her doctor told her to go to the chiropractor and, after gasping in horror,  I calmly told her I would be very cautious about that. Fortunately, she had never gone, but I cautioned her to be very careful to protect the neck at all costs. I figure, if nothing else, I am planting seeds that may bear fruit in the future.

It is always and inevitably an awkward and uncomfortable conversation to have and one never knows when fear and rejection are going to enter the picture and things might get ugly, but, I have always ended up feeling a great sense of accomplishment – as if I am righting a wrong. I know that I cannot find and save every person with EDS. But, I can reach out at every opportunity, educating as I go, and, if I can help just one person, that is something precious. It is worth putting myself out there, risking rejection and anger, and looking a little nuts, because I know what a difference my little effort could make for another person.

What experiences have you had in seeing and pointing out EDS, in friends or acquaintances? How do you handle it?