"Suffering the Slings and Arrows of Outrageous Fortune"

As I mentioned in my previous post, my daughter and I are writing a faith based book that we are hoping to have published. This book focuses on mostly the emotional aspects of living with chronic illness, although some practical advise is shared. It is aimed at chronic illness in general and not only EDS.

We are in the final stages of getting the book ready to submit and I am doing a bit of research that you can help me with. (I have a few future projects in mind as well, including a companion journal that could accompany our current project. This journal would include recommended reading (by me), inspiring/encouraging songs (recommended by Em), and activities/exercises that expand on the topics covered in the book.)

All you need to do is take a moment and answer the questions in this poll if you are affected by chronic illness. The more responses I get, the better!

Thanks in advance!

Check all that apply:

 

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.

So, Em is struggling with a low pressure headache again. Can’t sit up without excruciating pain, nauseated, her ears feel full, her neck is stiff, the Lumbar Puncture site is sore again. Hopefully, it is just low pressure and not menengitis also. The symptoms has been going on for a couple weeks now and they are not improving on their own. We have been pushing fluids and she is spending a lot of time laying flat. She sneezed a couple times a few days ago and it has gotten worse since then. I don’t know if the leak is in her ear(s) or at the LP site or both, but she is having text book symptoms so it is obvious something is going on. She has no quality of life right now – every few weeks, she ends up flat on her back for days until it heals up. Sadly, no one seems to know what to do to break this cycle or even cares enough to try.

We have tried to wait this out, but I started suspecting another blood patch is needed and, indeed, the doctor wants her to go to the ER to get one. She is, um, less than thrilled about this. The blood patch in January was a horrifically traumatic experience. They used latex gloves, even though she has a severe latex allergy, and she did not ever get numbed sufficiently so the procedure was excruciating. She is not eager to go through that again. And when I say not eager, I mean “you shall not pass”, “over my dead body”, “you’ll have to tie me up and drag me there, ’cause I ain’t going willingly” not eager.

The doctor can’t even see her in the office until July 8th and that is squeezing her in. If they see her at the ER, they will tell us to follow up with the neurologist. When I pointed that out, I was told that she can be seen sooner by one of the other neurologists, so that is maybe a silver lining? And I am hoping that, if I can convince her to go to the ER, since we have already been informed that a blood patch is not an emergency procedure, maybe we can arrange for her Pain Management doc who is an anesthesiologist, to do the procedure. He knows her, knows what she has been through, and hopefully can manage her pain both during and after.

I know we need to document this as an ongoing problem. At some point, one of the scans is going to show SOMETHING a knowledgeable doc is going to recognize as a leak and be able to do SOMETHING about it. We have to do SOMETHING to address this round of low pressure because she has no quality of life right now. We have to keep pushing to get this addressed. Now I just have to convince her to go to the ER again and to trust that it won’t be like it was last time. I have no idea why she would believe me if I promise her that, but that is what I have to do. As a side note, it is times like this that I would love to have every doctor who has treated her badly or hurt her or trivialized her suffering to see what their ignorance has wrought. Anyway…

A while back, I found a video that I meant to share here and just didn’t get around to it. It is a really useful video and it has helped me to gain a little more knowledge and fortitude to keep fighting this particular fight. It is about an hour and a half long but, if you are struggling with these symptoms, know someone who is, or has EDS which makes you more prone to developing these symptoms,  it is well worth the time to watch this video. And, he mentions EDS as one of the frequent causes of CSF leaks so it is great in the respect that it is spreading the word about EDS at least. If I was in California, I would be pounding on this doctor’s door and begging him to help my daughter…

The Mystery Headache: Migraine, Postural Headache, Spinal Fluid Leak

Em sees the eye doctor once a year, as is recommended for EDSers. This time it got stretched out to more like 18 months because we ended up having to see a new doctor (this is her 4th OD, we have liked most of them and we really liked the new one) and had a wait until she was able to take our insurance. The wait was not a big deal, but with all of the issues Em has been having (particularly the vision issues and possible migraine symptoms that have developed) the timing of this appointment yesterday was pretty good.

For better or worse, Em said her eyes were as bad as they had ever been yesterday so the timing was great to have them really see what she was experiencing. Her vision has definitely worsened since the concussion and then there was this weird thing of floating black spots that she has complained about.

Anyway, the docs in this practice have always been good about doing a thorough exam and the new doctor was no exception. We were actually there for a good 2 hours – even for an exam including dilation, that is a long time. There were some issues found.

  • There is some cloudiness on both lenses. This has developed since her last exam and the cloudiness seen is more than expected. The doctor said it is not affecting her vision yet, but, and I quote, “she may need cataract surgery sooner rather than later”. What?? She isn’t even 18. I don’t even know what to think about this, honestly. (I’ve seen various positions on whether this is an EDS thing or not but haven’t found anything conclusive yet.) The doctor said it is not a big deal and we will just keep an eye on it but it feels a little freaky to me.
  •  Her complaints of double vision are real. We are being referred to another Dr in the group who will evaluate her further and hopefully address the convergence issues that have worsened. It was already an issue (no one has ever followed through with dealing with it) but the concussion probably has brought it to a serious head. They are trying to get her in asap but there will likely be a bit of a wait. Should know soon.
  • Her vision complaints (the black spots, the black line that appears during her migraine like headaches)  are likely not in the eye but rather in the visual pathway in the brain. This is very possibly from the concussion, although, these were issues before the concussion. They did a scan of her retina, mainly for a baseline for future use, and it was fine.  Excellent even, so that was good news.
  • The doctor said it was a very good idea to have her worked up for migraines, considering the visual symptoms Em described. (To which I say, “na na na na boo boo” to the neurologist who ignored these symptoms. Not very mature, I suppose but so be it!)

Hopefully we can get her some relief on this – she is having an increasingly difficult time reading and functioning. I don’t even know what to think about the cataract issue but I am very glad she is in competent hands who are taking her seriously.

 

 

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.

 

 

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!

One reason I started this blog was to have a place that I can record symptoms, so I have a record that I can refer to as time goes by. If it helps others, that is icing on the cake. So I am doing just that today – if others have similar experiences and want to comment, great. If somebody has a clue what is up with this newest weirdness and wants to comment, also great.

As I have posted previously, Em is having episodes that look seizure-like but are possibly a type of migraine. Sometimes she has visual disturbances with them and I have asked her to describe those disturbances. She has specifically described that she has a dark band across her visual field or black spots during many of the episodes. Which I just accepted and entered into the log I am keeping.

But a little more questioning has revealed that this vision thing is not new. After  a recent episode she said that these black spots are not new, just worse than ‘normal’.

So, she has black spots (like a darkly tinted window, transparent but dark)  that drift across her vision during the episodes. Multiple black spots that converge and, when they do, they become less transparent and more solid black as they layer on top of each other. After a while, they drift back out of her visual field. Her eyes aren’t always open during the episodes but when they are, these black spots are typical. The solid black line happens occasionally rather than the spots.

None of that is unexpected with the neurological natures of these episodes.

What is unexpected is that she says, for her whole life – as long as she can remember, she has had these black spots. They aren’t floaters – she is describing large spots in both eyes where her surroundings disappear or are blocked. They drift in to her vision and drift out again. They occur daily, multiple times, as far as I understand, and are big enough to block out someone’s face or whatever is in her field of vision. Then they drift off (I think, as opposed to disappear) and her vision is normal again.  She doesn’t remember a time when she didn’t have them.

Mind you, she has had yearly exams by an ophthalmologist since she was diagnosed with EDS and had had her eyes examined several times before her diagnosis. Has she ever once mentioned these spots? No. In fact, her defense is that the eye doctor always says her eyes are normal so she just figured it wasn’t anything to worry about. Annoying and inconvenient, but not a big deal in her mind. I know the danger of having so many weird symptoms is that you don’t know what is normal and what isn’t, but it is frustrating to realize she is 17 and never saw fit to mention this. Then I start wondering what other symptoms is she experiencing that I don’t know about…

She has an appointment with the eye doc in late May, so we will definitely be bringing this up then. We also will see the local neurologist before then, so can talk to him about it. She did just have 3 brain MRIs so at least the generalities should already be covered. Hopefully we can get the inpatient EEG monitoring scheduled soon so we can at least start figuring out what is going on.

 

 

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.🙂

 

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