"Suffering the Slings and Arrows of Outrageous Fortune"

Posts tagged ‘chronic illness’

An Update: Neurology, Ophthalmology, EMG

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

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

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

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

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

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

 

So, maybe some baby steps in the right direction.

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.

 

 

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.

 

 

Another Much Needed Win

Every once in a while, amid utter frustration and continual wearing down by the whole medical system, we get a win. Perhaps not a huge victory in the big scheme of things and there are certainly bigger battles looming. There are lots of ups and downs on this journey, even with doctors we like and respect, and that makes for a bumpy ride. But, when we actually have a great experience, especially when we weren’t expecting it, it gives us strength to keep going.

Em’s follow up with the Cardiology Syncope Clinic this past week is a good example of such a time. She has been seen in the Syncope Clinic for 3 years or so. We like the doctor, although for a while now, we have felt a growing  ambivalence about him and her treatment. So often, what we have experienced, even at Cincinnati Children’s is a subtle communication that it is all EDS and there just isn’t much to do outside their carefully constructed box of best practices. When we threw CRPS at the docs down there, everyone we saw just kind of said, “Oh, that is too bad. Not sure what to tell you. Good luck!” So, as much as I like some of the doctors, we don’t always feel  like we are getting anywhere. That is not a big deal when things are going well, but when things are not going well and you need help, it is a very big deal.

We went into this appointment, with rather low expectations and fully expecting it would be like the most recent follow ups: the nurse would ask a bunch of questions, we would see the doc for maybe 10 minutes, he would say increase your meds if you need to and we would be on our way.

Em was exhausted and feeling awful. It is a twoish hour trip and she doesn’t travel well on a good day and getting up early just makes it a not at all good day. She was in a lot of pain and her head was hurting. She had one seizure in the car on the way. She typically has little patience for being asked a ton of pointless questions and being treated like a statistic rather than a living, breathing person who is suffering. (This isn’t necessarily how she IS being treated but definitely how she perceives it.) On Wednesday, she had even less patience than usual and was ready to let somebody have it, even if it was undeserved or unwise.

As the mom, I always feel like I am walking a mine field between Emily, who is rightfully sick and tired of being sick and tired, and doctors who have an awful lot of power over us and who just don’t quite “get it”. I was just thankful they didn’t make her do the questionnaire like in the past: if they had tried, there would have been a visible mushroom cloud over Cincinnati and the news would have reported that the epicenter of the explosion was on the 4th floor of location C at Children’s.

So, the appointment proceeded as usual – the very sweet nurse took all of the info of what had been happening since we had seen them a year ago. There was an awful lot to share so it took awhile. Then we were informed that we would be seeing the Nurse Practitioner, instead of the doctor. We weren’t sure if that would be a good thing or a bad thing because we had never seen her before, so we were both sort of “meh” about it. Honestly, with our recent luck, we had little reason to expect much good.

Martha popped into the room with a smile and introduced herself. She talked about how to tweak Emily’s medicine to make it work a little better. We talked about Em seeing the doctor at OSU in a couple weeks and she put any med changes in our hands in light of seeing him. We could stay the course with Midodrine, increasing it for now. Or we could add something, although she was a little reluctant in light of everything going on and not wanting to muddy the waters for the new doc. And, she said, if we get to him and he wants to add a med but wants them to follow up with it, to just call and it will be no problem. We opted to increase her Midodrine for now but hold off on beta blockers, etc until we talk to him. It was refreshing to be given options and allowed to choose the best course.

She actually gave some practical advice – if support stocking are painful and hard to wear, abdominal binders might be better/ easier and even wearing spanx (bicycle length) can help. Further, she suggested wearing two pairs of leggings (she and I are both of an age that once upon a time called them stretch pants and we both chuckled about that) – that doubling them up may give enough compression that it could help. She was quick to point out this was not supported by any medical data, but that people have found it useful. She and Dr. Grubb’s  NP, Barbara, share ideas so I know she is getting information from a very reliable source. These ideas may or may not help Em, but the point is she was trying to offer practical solutions rooted in the real world, not some medical fantasy land.

Speaking of medical fantasy land, the  current recommendations from Genetics and Cardiology for improving EDS and POTS, is 30 minutes of exercise a day. This exercise should have the heart rate elevated but cannot be done from an upright position. So, something like a rowing machine or recumbent bike. They apparently have had good results and if Em were well enough, we would probably be eager to try this. But, after the CRPS started and she had ear infections and a concussion and lumbar puncture and blood patch  and now seizures, this just is not feasible. Martha recognized that and didn’t even give us the speech. She just suggested Em try to stand for a couple minutes, leaning against the wall and do a few modified squats each day, careful not to dislocate her knees.

When I asked if it would be wise to do some blood work to check Em’s electrolytes in light of her seizures and needing to figure out what is causing them and maybe rule some things out, she said the blood work wouldn’t be very useful to HER but if I wanted it for the OSU doc and for when we see the epileptologists, she would order it. So she went down the list of options and ordered everything that might be helpful. When we were ready to leave, the nurse brought the printed orders to me (we can’t have blood work done there, have to do it at home thanks to insurance) I suddenly recalled that the electronic signature they use isn’t sufficient for our hospital – they always have to call and get a signature faxed and it is generally a pain in the butt. So, I apologetically asked if she could sign it by hand. But, since there were about a billion (give or take) separate orders, the nurse said she would have to sign each one. I hated to bother her with that since she had spent so much time with us already, but the nurse said it wasn’t a problem and Martha signed each order for us.

She was just so kind and supportive – inquiring about Em’s mental health and morale in a way that was genuinely  caring and not clinical or intrusive. Far more than the electronic questionnaire that asks Em to rate how often she has felt depressed via a rubric of Never, Occasionally, Often, or Always.

And, when we were wrapping up the appointment, she wanted to know if there was anything else she could do for us.  I had a request that was a little odd for a cardiology visit and probably would not have asked if we had seen the doc instead of the NP. Em’s ear has been hurting a lot again. We weren’t sure if it is just being aggravated by her jaw or if it was actually infected again. Getting an emergency appointment with the ENT is challenging so if it is not visibly infected, well, maybe we just wait and see.

When I explained all that and asked if she would just peek in that ear and give us some guidance, she had no problem doing so. She looked in both ears, then in that misbehaving right one for a long time. (Which is what every single person who has ever looked at her ears has done, trying to figure out what the heck they are seeing.) Finally, she grabbed a marker and drew a diagram of what she saw on the whiteboard. She saw fluid in that ear, probably not infected yet but definitely fluid, and scar tissue from what looks like a previous perforation. Her suggestion was to give the ENT a call if it keeps hurting or gets worse, which was precisely the advice I needed.

She spent about 45 minutes with us and it has been a long time since we felt so cared for, certainly down at Cincinnati. When she walked out of the room, I looked at Emily and told her we needed to kidnap Martha and keep her with us all the time. Emily, who had been ready to go in to this appointment with guns blazing, agreed completely and was laughing with me about my goofy suggestion. It is truly amazing the impact a single person can have on the people around them and how far a little kindness and support can go towards making someone who is suffering feel better about the world and giving them the strength to carry on.

I wish all appointments could be like that for EVERYONE who is in our shoes, but, since that is wishful thinking, I will just be very thankful for Martha and her kindness at a time when we really needed it.

 

Rare Disease Day 2016

 

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

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

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

 

Rare Disease Day

 

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

 

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.

 

 

 

 

Catching Up and Hanging On: Part 3 Shoulder Woes

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

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

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

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

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

Her exercises:

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

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

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

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

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

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

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

Never a dull moment…

So… another installment of Em and Beth’s excellent adventures. (Doesn’t quite have the same ring as “Bill and Ted’s” but whatever…)

New Meds

Since I last posted, Em has had to go off the Cymbalta. She has been on several meds that, combined, can cause Serotonin Syndrome, and Cymbalta was one more to add to that list. When she displayed confusion, lethargy, and worsening muscle twitching, we got concerned. The doctor wasn’t – but I was and so I called the pharmacy. The guy I talked to spent a fair amount of time on the phone with me and finally said if it was his kid, she would not take the new med anymore. Which sealed the deal for me. Nucynta is another one that can be a problem, but in a much lesser way than the Cymbalta, so we are hopeful that she can perhaps try it once the Cymbalta is out of her system and maybe it will help. Again, it is a thing that it was helping her pain (although she didn’t actually notice much relief when she had taken it, but rather when it wore off) but she just can’t tolerate it. That gets heartbreaking, to be honest.

I actually haven’t talked to the pain doc about stopping the med – we made an “executive decision” to stop it. He wasn’t a bit concerned when I talked to him about the worrisome symptoms, which is rather concerning to me, if I am honest. I know he is just trying his best to help and sometimes we have to take calculated risks. This just is not one that worked out.

Because he sort of blew off my concerns, I am hesitant to call back and tell him that we decided it best to take her off the med. I don’t think it will be a problem but I just haven’t had the time or inclination to jump back into that fight, for reasons that will be apparent in a moment.

Another Trip to the ER

We are thinking that Em has developed a CSF leak and made a trip to the ER yesterday at the advice of the neurologist. Or, more accurately, the nurses at the neurology office because they didn’t get around to returning my second call until the end of the day when they couldn’t get a hold of the doctors.

It was Dr. Diana that suggested the possibility of a leak – that perhaps the weirdness with her ear was due to a leak. (Thank God for Dr. D, I don’t know where we would be without her.) Now, all of these new and worrisome symptoms were sort of mashed up together, but after she stopped the Cymbalta on Tuesday, she started complaining of a headache that went away when she laid down and quickly got horrible when she is upright. So, she had already been in bed for a few days because the Cymbalta knocked her out. But, starting Monday or Tuesday, every time she sat up, the headache was awful and when she lays down it goes away. So, that seems like a low pressure headache and/ or a leak. Who knows how long it would have taken me to figure it out without a helpful word from Dr. D?

Diamox made it worse, so we pulled back on it and let her miss a dose to see what happened. Her head got a little better, but still, she couldn’t be upright. And, there was the concern of meningitis. So, I called the office late on Wednesday, called back on Thursday when I didn’t hear back and they finally got back to me and they said to go on to the ER.

You can imagine that this was not well received after our last trip to the ER. Understandably, we were both anxious about how it would go.

Fortunately, this trip was much better. They took her pain and symptoms seriously and treated her well. They started an IV, ran some fluids and gave her some pain medication. It took the edge off of her headache and let her rest a little. They did a CT scan, which found no big leaks, but could not rule out a small leak. They could not do the test to check for a leak at that time of night. I am not sure what he was referring to there, but we will follow up with the neurologist and pursue it further.

He said there was no indication that she has meningitis but the only way to tell for sure would be to do a spinal tap. He did not recommend it and we all agreed. Wholeheartedly. And, he clearly didn’t feel a lumbar puncture was necessary which I was also in wholehearted agreement with.

He felt comfortable sending her home to sleep in her own bed and just follow up with the doctor, saying we can come back if it gets worse. I had packed to stay a few days if necessary (the last time we had a real emergency I didn’t and we ended up staying several days at Riley Children’s after a swift ambulance ride in the middle of the night and an appendectomy) and I was pretty darn glad it was unnecessary! If packing to stay is all it takes to get sent on home, I will do it every time from now on out!

This morning, her head is no better when she is upright. So, I think we are dealing with a low pressure headache. She took a small dose of Diamox yesterday afternoon before we went to the ER, but that is all she has had since Wednesday. She can make the call as to whether she needs it or not. And, we will play it by ear until we can get in to see neurology. I am also hoping that when we see the ENT, he might be able to help us figure out if her ear has anything at all to do with her headache. If it is a leak, with any luck it will heal itself and need no further treatment.

It is more than a little frustrating to have to work so hard to get answers – we spent hours in the ER and came away with no answers. And, yet, I am thankful that it was a positive experience, that they took her pain seriously and treated it, that they were caring and compassionate, and that we were able to rule the big stuff out. Of course, that leaves all the little stuff up for grabs, but that is our life.

When the doctor first examined her, he said he was going to go read her records and see if they could give him more insight into what was going on. When he came back in, a couple hours later, after the pain meds and CT scan, he sat down and said that he had been reading about Em and that it was enough to keep him busy for awhile.

How right he is! Having been kept busy for the last 6 years, I can tell you that truer words were never spoken.

 

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