"Suffering the Slings and Arrows of Outrageous Fortune"

Posts tagged ‘Headaches’

Rinse and Repeat: Low Pressure Headache

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

Eye issues: Real and a Bit Concerning

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.

 

 

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!

More Eye Weirdness

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.

 

 

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.

 

EEG

Emily had her EEG on Monday afternoon. Amazingly, she had two episodes – perfectly representative of what she normally does – during the test. We could not have planned or timed it better!

She started having a few sporadic twitches as she was being prepped and I knew we were on borrowed time. I got to stay with her; I had been worried about it and was grateful that I didn’t have to leave. The tech started the test and had her essentially hyperventilate for 3 minutes. After about a minute of that, I could see that she was starting to fade, but she completed the 3 minutes then said she felt like she was going to have one. A few seconds later, she went limp and stopped responding. She twitched and jerked every 10 seconds or so and the tech recorded those in her notes. She started having labored breathing and gasping for air like she usually does. At about the 5 minute point, her eyes opened and she was able to respond a little, although she was still dazed.

Then she went straight into the second episode. This one was longer – about 10 minutes and the twitching and jerking was stronger and more frequent. The tech finally gave up recording each jerk because she couldn’t type fast enough. Again, she had labored jerky breathing and I had to remind her to breathe a few times. She finally opened her eyes and could respond. Once she recovered a little the tech was able to complete the EEG and we  were done in less than an hour. She twitched and jerked during the strobe test, but didn’t go into an episode.

So, I feel like they should have gotten pretty good information from the EEG. It very well may not show anything, depending on what is causing the episodes (I really want to just call them seizures and be done with it but I won’t yet). But, she had two episodes and that is all we can ask for – they were witnessed and documented so we have some validation. I do wish they could have caught the second, more visual/ sensory type during the test as well but those happen much later in the day so it never likely that she would have that kind then anyway. I am grateful they saw anything, to be honest.

I am waiting to hear the results from the doctor. They didn’t call first thing this morning so I left a message, because it may not seem urgent to them but it surely is to us.

She has now had 35 episodes since the 9th and that will, if the pattern holds, increase to 38 or 39 this afternoon. They are getting longer and stronger. Last night, she had the visual type combined with the unresponsive type and jerked constantly for 6 minutes – she swayed, then saw the usual light and visual distortions, then went limp.  I laid her back on the pillows where she jerked and twitched worse than she has before. Then it was over and she was fine, after she got over feeling like she had been run over by a truck.

She is having less warning  before it happens, so she is less able to call for help. She is going limp quicker, which means, if it hits when she is upright, she could be seriously hurt. Realistically, it is just a matter of time before that happens, unless she gets some effective treatment, asap. So, if I make a nuisance of myself, so be it. If the doctor’s office is annoyed, they can deal with it and come up with a plan or refer us promptly to someone who can.

But, the upside  is that she had episodes during the EEG so I am hopeful that they gathered information that will help us get to someone who can helps us figure this out. Because, frankly, it sucks. A lot. And we have to do something. Soon.

Another mystery to solve…

Once again, we are in the process of trying to figure strange stuff out. And it is confusing, with everything Emily has been dealing with over the last few months. At this point, I am starting to wonder if there are some threads that tie some of the odder symptoms together. This post is going to contain details of the episodes she is having so I have a record here of them and a fair bit of speculation, trying to tie those threads together. I am waiting for the doctor to call back today. Hopefully we find someone to help us figure this out and soon.

Pertinent Back Story:

  • Blood work has shown some signs of MS 
  • Blood work has shown signs of sarcadosis. Lung x ray was clear but neurosarcadosis could be a possibility
  • Concussion (sandwiched between several  minor(?) head injuries) and Post Concussion Syndrome
  • Her neck has been pretty unstable – to the point of needing to wear her hard collar at times.
  • She has had ongoing issues with her throat [feeling as if it is] collapsing. She gags/coughs, strains, and stretches until she can breathe and swallow. It had settled down but is becoming a problem again.
  • CSF pressure is bouncing between high and low. If there was a leak, it seems to be better now and she is back to taking a small dose of Diamox three times a day. However, last night she felt like she had low pressure so who knows – it is all a guessing game at this point. None of the doctors seem particularly interested in pursuing this and we have bigger problems now, at any rate.

Last week, Emily started having “episodes”, for lack of a better word.

Tuesday 2-9

The first happened in the car on the way home from speech therapy last Tuesday. Her neck had been unstable since they messed with it on Friday at the Concussion Clinic and she was wearing her hard collar. Part way home, she commented that her vision went wonky and fell asleep – or so I thought. Turns out she was just sort of out of it – couldn’t speak and she appeared to be asleep but she was aware. When we got home, I had a hard time waking her up and when I did, she couldn’t move her arms and legs and couldn’t really speak. I got her out of the car, she was able to walk a little – she leaned heavily on me and I had to lift her legs up to step up into the house. Once I got her sat down, she started doing better. After a few minutes, I helped her to the bathroom and then to her room. She was still a bit out of it but then she cracked her neck and was fine.

I assumed this was all her neck because she said her neck was in a weird position in the car. It bears pointing out that she has said from the beginning that it wasn’t her neck that it was her brain, but we will get to that in a minute.

Wednesday 2-10

She had another episode the next day, this time laying down on her side. She was talking to me and said her vision got weird again and she felt groggy. Then she couldn’t move her arms and legs or talk for about a minute. I carefully moved her arms (she tends to lay with one arm or the other over her head) and she got better after a minute or so. Her pulse was 110 and her BP was 105/59 during the episode. Pulse was a bit high and BP was a bit low but neither was concerning.

Thursday 2-11

The doctor ordered an X ray of her neck, which we did on Thursday. (Still waiting on those results, but I would be shocked if it actually shows anything useful.) She said she thought she might have had an episode in her sleep and all day Thursday she had times where she felt groggy and felt an episode coming on but it wasn’t until late afternoon that she had a full episode. Again, she was laying on her side and the groggy feeling started, her vision dimmed and she couldn’t move her arms or legs. She could whisper one word responses to questions. I moved her arm and her leg and she got feeling back – first in her fingers, then her hand, then her arms, then her legs. She said she could breath during the episode but not deeply because her chest muscles were paralyzed like everything else.

Friday 2-12

She was sitting up writing in her journal and she suddenly complained that her vision got weird. She had been tracing over a word that she had written earlier. She though she was doing it carefully but her writing was shaky and all over the place. She felt an episode coming on and tried to hold it off. She laid down to rest and watch a TV show and suddenly she stopped talking and moving. She sort of hummed to get my attention. I moved her arm and rubbed her hand until she could move and talk. It appeared to me that her breathing was normal during the episode – a bit shallow and rapid but fine.

Saturday 2-13

I checked on her and found her groggy, mumbling. She rolled over and then couldn’t move. Again, she could give one word answers in a whisper. I moved her pillow because her neck was at a weird angle and, after a minute, she could move again. She started having trouble breathing and swallowing (looked very much like earlier episodes where her throat ‘collapsed’) so I helped her sit up. She asked for a warm wet washcloth to wrap around her throat and it helped. My husband witnessed this episode – he was puzzled but said it sort of looked like a seizure of some kind. I was still on the idea that it was related to the position of her neck but he got me thinking.

Sunday 2-14

She had three episodes in the space of an hour. For these three she wasn’t laying down – just reclining back on her pillows or sitting up on the edge of the bed. Twice we were talking and all of the sudden she got quiet and stopped moving. The third time I had left her alone for a couple minutes and found her unable to move when I came back.

She just stares forward and tries to answer my questions when this happens but can’t do more than whisper one word or just grunt or make some noise. I swiped my fingernail up both of her bare feet to see if she would react. She didn’t. I did it harder and she winced but her foot didn’t twitch. I pinched the pads of her fingers and pushed my thumb nail into her nail beds – no reaction. After about a minute (one episode last almost 2 minutes), she was able to gradually move again and started gasping for breath. I got a warm washcloth again and her throat relaxed so she could breath and swallow.

 

It was at this point I seriously started reconsidering that it is her neck. I started doing some computer searches and found a description of atonic seizures. It isn’t a perfect fit but it makes some sense. If she is laying down, which is doing a lot of these days, she wouldn’t drop so it wouldn’t be as obvious as it is in many cases.

These episodes last about a minute (other than that first one in the car, which was more like 15 minutes) and she is conscious and remembers everything. She seems to have some warning that it is coming on – a feeling of grogginess and then her vision dims. She cannot move or speak normally for a minute. If she was standing up, I suspect she would just drop – which is a terrifying thought in light of her recent concussion. On that subject, I am wondering if the time she fell forward off her bed and hit her head wasn’t because she was just dizzy but rather one of these episodes. And, having read up on seizures, I am wondering if some of her muscle twitches and tremors might be a type of seizure as well. Anyway, as soon as the episode is over, she is fine – able to talk normally and laugh and such.

The breathing trouble afterwards may just be incidental. From what I can see, during the episodes it looks like she is temporarily paralyzed. Like her brain isn’t communicating with her muscles. When communication is restored, her throat seems to spasm. Once that spasm is calmed, she is totally back to normal. Her head hurts afterwards and she continues to have neck pain but I am thinking that may be a result of her muscles relaxing so much – she has to put her shoulders back in after each episode.

Maybe it is her neck but I am doubtful at this point. I now think my moving her arms or legs had nothing whatsoever with the episodes ending – they likely will end after a moment regardless of what I do. I guess the other possibility is that she is having TIAs, although she doesn’t neatly fit in that box either. The elevated ACE test indicated sarcadosis – seizures and some of her symptoms can be a part of neurosarcadosis, so that needs to be considered. And the possibility of MS has to be on the table as well. I am going to try to get some video of these episodes so we have a record of them – maybe that will help us get this figured out.

So now we are at a point of who can help us figure this out? She has an appointment with the neurologist at OSU but that is a month away. We are beyond our local neurologist but if they can set us on the right track and maybe do some testing it would help guide me in seeking help at Cincinnati Children’s or at OSU. I don’t need to be right – I just need someone to help us figure out what is wrong and fix it.

 

 

 

 

Everything else that we are dealing with…

So much has happened in such a short time, I can hardly remember what I have posted about and what I put off for later. In addition to the lumbar puncture, the blood patch, the ongoing high pressure issues and the likely CSF leaks, Em has been dealing with a concussion and that injured rotator cuff and, of course, what we presume to be CRPS.

Shoulder Injury

PT dismissed her because she was struggling intensely with the most basic exercises she was given. At the time, we were suspecting that her neck had become unstable and that was a big concern – for some reason people get a little jumpy when you mention her neck is unstable! Fortunately, a lot of the issues that we assumed were down to instability eased up when they did the lumbar puncture, easing the high pressure.

So, the plan was, talk to the specialists at Cinci and wait and see. In the meantime, her neck is better and  she has been able to gain a lot more use of her shoulder and is out of the sling. At this point, we will just carry on and hope the shoulder doesn’t get reinjured. In a perfect world, we would be able to focus our energy on her shoulder but, as we are far from a perfect world, there are too many other things to worry about. Sadly, since it seems to be improving, her shoulder is low on the list of priorities at the moment. I am just glad she has use and motion back and has less pain.

Concussion

She is doing better, but still not well. I think I mentioned that she fell and hit her head (again) on the 9th, which worsened the memory loss and balance problems. That was a little scary, to be honest but the doctor felt like the second injury should not have been enough to cause any damage and waiting it out was the thing to do. We have just made it a priority to protect her head as much as possible and keep her safe until she is steady again. Some of our precautions she doesn’t particularly appreciate, but protecting her brain is non-negotiable.

Her balance has improved from what it was after bumping her head that second time, but it is still not great. She is using her wheelchair when we are out in public. That is frustrating for her, but, I can tell you that I am so grateful we have that wheelchair in times like this.

She has started speech therapy to help rehab her brain. She had her second visit yesterday and will go again tomorrow. Her memory loss is better – she is able to recall details much better than before – but she is still incredibly frustrated when she is searching for a word and just can’t pull it up. And she is bemused at what her brain does recall and at how odd it feels to not know she knows something until she suddenly remembers.

Right now, we are still being guided by her symptoms – she can do the speech exercises in varying chunks of time before she starts hurting. Sometimes she can go straight through for a considerable length of time before her head starts hurting and other times she hits the wall sooner. This week she is going to write answers on a worksheet with various questions and we will see how that goes. She had a disconcerting time a couple days after the second bump when she realized that her brain was sort of disconnected to the actual process of writing. She said it felt like a reflex but that her brain wasn’t really doing it.

We go back to the concussion doctor on the 5th and she will have neuropsych testing done. I think she is definitely better  and continuing to improve: we are all happy with the progress she is making, but this is all new ground for us and it will be good to have knowledgeable eyes on her.

She did sit in her room and play her guitar a couple days ago and that was a wonderful sound. It has been a while and she is just now getting to the point of being able to remember the cords and lyrics that once came so easily to her. Music is such a part of her life – being able to play again is a huge morale boost.

CRPS

Way back at the beginning of December, we saw the pain doctor and emphatically expressed our need for help. (Sort of a comin’ to Jesus type event.) Bless him, he spent an hour with us on a day we weren’t even scheduled to see him and his waiting room was packed.

Anyway, I had taken in a referral form for a doctor I wanted him to send us to – a neurologist specializing in neuromuscular diseases at OSU medical center. I had filled the form out as fully as I could (and told them what to put in the lines they needed to fill out) in the hopes that there would be no excuse to not promptly refer us. It did take a polite reminder but they did eventually get us referred. Honestly, I wasn’t even sure if this doctor would see Em since she is 17 so it was a shot in the dark. His office called to ask some questions and clarify the situation and to inform us that he normally wouldn’t see anyone under 18 but that he would consider it.

Thankfully, he is willing to see her and we see him on the 15th of March. I am cautiously optimistic that he will be helpful in giving her a diagnosis and figuring all of this out. Recently, her lab results got a little more complicated and it is doubly good that we are going to him – I will get to that issue in a just a minute. I hear he is very smart and very kind so I believe we are on the right track and I am beyond grateful he is willing to see her.

New Diagnoses

Em has had a ton of labs done – between the CSF testing from the lumbar puncture and the blood work that has been done – we have at least been able to look at a lot of things that can be ruled out or addressed. We saw the neurologist last week and I was not surprised to hear him say Em is beyond him and needs someone with more knowledge than he has. He has absolutely done right by her for 4 years, so that was a little emotional. He will be available if we need him, but we need to get somewhere with more knowledge.

He was concerned – not overly but said it needs to be looked at – with one of her labs. The one test shows some markers for MS. She has three bands and four indicate MS. Now, he doesn’t think she has MS and I don’t think she has MS. I just think her entire nervous system is on fire and there is nothing at all conclusive about that test in regards to an MS diagnosis. However, she needs to be evaluated by someone who can handle all of her complexities. Fortunately, we already have an appointment with a doctor specializing in neuromuscular disease. That is exactly where we need to be and I doubt we would be able to get a sooner appointment anywhere. So, it all seems a bit Providential when I look at it.

Also, the doctor ordered an additional blood test which has come back elevated, indicating Sarcadosis. Am I surprised that at this point she is showing signs of an autoimmune issue? Not at all. Truly, I am just surprised she hasn’t shown any autoimmune signs previously. Again, and almost as always, she has enough symptoms that sort of tick the boxes for one thing but that also overlap with about a dozen other things. So, who knows. She also had a lung x-ray and we are being referred to a pulmonologist. Again,  I am not getting too worked up about this – if it is sarcadosis, it is treatable. If it isn’t, the symptoms (which, if they exist, are being masked by everything else that is going on) are certainly explained by everything else she is dealing with.

ER Complaints

We have taken our complaints about the whole lumbar puncture/ blood patch/ ER/ latex exposure debacle to administration and we are, fortunately, being taken seriously. So far, profuse apologies and some ideas on how to make sure this never happens again – not to us or anyone else – have been offered. I am not going into details right now, because this is, after all, my husband’s employer, and I want to tread lightly but suffice it to say they are doing right by us and I think we will be satisfied by the time it is all done. I am very hopeful that this will be an opportunity to share awareness about Emily’s rare conditions and help foster an atmosphere that better understands how to treat complex, chronic illness. Which, in the end, is what matters to us.

One Last Thing

Finally, I would like to ask for your prayers for my mom. She has been diagnosed with breast cancer and will be having a mastectomy in the near future. But, she has great doctors and her prognosis is good, so, even though this is a blow, we have faith that this is all going to be ok.

 

So those are the highlights from our crazy life right now. If it all sounds exhausting, it is! But, we are hanging in there and trying to keeping our sanity intact.

 

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