Who Knew Ears Could Be So Fun?

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

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

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

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

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

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

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

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

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.

 

Catching Up and Hanging on: Part 2 Concussion

Part 1 can be read here.

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

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

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

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

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

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

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

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

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

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

 

 

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

 

 

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.

 

The Latest in Our Ongoing Adventures

Things have improved, slightly, since our awful visit to the ER. Not her pain, that is still uncontrolled and terrible, but we are a few steps closer to getting answers or at least some real treatment.

MRI Results

Em’s MRI was last Wednesday and it actually showed a problem. Turns out, she has “Tendinopathy of the Supraspinatus Tendon” which means that tendon is either irritated or torn. Putting it in simpler terms, she has a rotator cuff injury. Personally, I think it is a tear, since her description is that she had it resting behind her head in bed and all of the sudden something popped, it went numb, fell down and she couldn’t lift it. Unfortunately, it keeps getting re-injured in her sleep and the slightest movement away from her body causes awful pain and makes it go numb again. We are figuring out what to do about it. The doctor who ordered the MRI probably will not be the doctor who manages the problem, so we are going to have to get her to the ortho ourselves.

And, we have resisted the urge to pop into the ER and see if our “favorite” NP is there and do a we-told-you-so dance at her. Bottom line here, is that Em needed an MRI. We were right, the NP was wrong. X-rays would not have shown a thing and would have only hurt her more and possibly made it worse. (Em is emphatic that she knows that the would have made her move her shoulder in ways that would have hurt and maybe damaged it more. At this point, I am inclined to believe her) And, if she had just gone ahead and ordered an MRI while we were there, we would be a week ahead of figuring out how to treat it.

So, we are feeling pretty validated after finding out the results. That validation comes at a pretty high cost though. However, it does confirm that, generally, we know what we are talking about and that will make facing down rude doctors (or NPs) a little easier. Confidence is  good thing.

Bloodwork

The Rheumatologist ordered some bloodwork – mostly to check for Vit B deficiencies – when we were there in November. We had to do it at our local hospital and not Children’s for insurance reasons, and I think the Rheumatologist still doesn’t have the results. I am not quite sure if it is their fault or the hospital’s fault but it is ridiculous. I am not happy with any of them, to be honest. My husband finally just called and got the results. All but one is fine – it looks like her B6 is low – which could contribute to neuropathy. But, again, the doctor who ordered these doesn’t seem to want to manage this, so we are pretty much on our own with dealing with this.

Rheumatologist

We really liked him – he was a great guy and all – but he seems to be washing his hands of Em. He prescribed a med called Trileptol, in the hopes it would help her pain. As she was titrating up to a full dose, just a couple days into the process, it was helping her pain but it messed with her head in a pretty significant way. She has always been “feisty” but this med made her uncharacteristically angry and unreasonable. And I won’t say she was suicidal, but let’s just say she was talking in a way I had never heard from her before. It was pretty scary. And dealing with it was  complicated because this was all taking place the day before Thanksgiving, the day Em asked to go to the ER. She stopped taking it and the nurse I talked to tried without success to talk to the doc about it. She finally called back, having not been able to talk to him, and said don’t take it anymore and that she would make sure the on call team knew about the issue if we needed more help.

The office eventually called back Monday – after the holiday and the weekend – with his thoughts. He said that med shouldn’t cause mood changes (if that is true, why did the info from the pharamacy say it does and to inform your doc?) and that she should go back on it and titrate up more slowly. Riiiggghhhttt.

So, yeah, Em will not be taking that med anymore. Haven’t heard back from him and the nurse – I don’t know if she was just reading from our appointment summary or if he gave her new notes – informed me during that call that we should just go to the Pain Management doc to get more pain relief and to “find someone to refer us to someone who can investigate a diagnosis of Small Fiber Neuropathy”. That makes me insane: “Oh, I think you don’t have that unusual disease that you think you have, I think it is this one, which is far more rare and nearly impossible to diagnose. And I have no plans whatsoever to help you get that diagnosis.” Hence my belief that he is washing his hands of us. Lovely.

By this point, I was starting to get fairly annoyed. Or possibly livid. Possibly ready to go to war and/ or all Mama Bear on the next medical person we encountered.  The doctor we waited two months to see had nothing to offer and dumped us. That was the final straw. Every single doctor we had seen about the CRPS had played hot potato with this kid. She was tossed to the next guy with a polite smile and a hope that he would do something. And, if that didn’t happen, what did it matter once we were out of their office?

We had a pain management appointment on the 4th and I decided it was going to be our line in the sand. If I had to throw a gigantic fit, he was going to do something constructive to help my child. That appointment deserves its own post, so that will be up tomorrow. It was good and we so needed good right now.

Another Ear Infection

After a very long PM visit on Friday, Em said we needed to get her ear looked at and, since we were right there, we popped into Urgent Care. I don’t think I mentioned here that, while we were at the Rheumatology visit on November 19th, they looked at her ear and said she had a perforated ear drum. At least the doctor doing the exam was pretty sure it was – she looked at it a long time and said she was pretty sure it was perforated and to make sure to keep the ENT visit that is scheduled for the 15th.  So that ear is just an ongoing issue.

Weirdly, which is her norm, during the MRI on Wednesday, the noise made her ear suddenly pop and hurt. Since then, she wasn’t able to hear out of that ear. She knew she wasn’t going to make it another week and a half until the appointment, so we popped into Urgent Care.

Good thing we did. The poor doctor looked in her ear. And looked. And looked some more. Then said – and you just LOVE to hear this as a parent – “I’ve never quite seen anything like this before.” She couldn’t even see the ear drum because there was “white powdery stuff” blocking the view. She grabbed the other doctor to come in and look. Fortunately, this was the same doctor we saw last time and, not surprisingly, she remembered us.

Long story short, it is either a fungal infection (Em asked, “if I google spores, will I be freaked out?” They told her not to google it.) or fluid that has crystallized from the ear canal after the ear drum perforated. It was very fortunate that we knew her ear drum was perforated as of the 19th, because that means ear drops are a no no. Initially, they apologized that they had nothing to offer and said they would tell the ENT to see her asap. They came back and said they had ear drops as a suspension fluid that she could use, so, we have been doing ear drops 4 times a day again. And, I am currently waiting on a call from the ENT. I doubt they will see her today yet, but hopefully they can soon.

We are hanging in there. A lot is happening. We still have few answers but are slowly getting there. I will post about the pain management visit tomorrow.

CRPS/ RSD

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

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

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

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

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

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

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

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

 

 

 

Our trip to the Syncope Clinic

‘Emily had her year follow up with at the Syncope Clinic at Cincinnati Children’s. It was a relatively brief visit that ended up with him increasing her Midodrine dosage because it is not working as well as it had previously (which was news to me, but that’s ok.). Apparently that is pretty common with Midodrine. He basically said she could increase it to 10 mgs when she needs it and play with an increased dose throughout the day, but hopefully not 10 mgs 3 times a day. That seems like a good plan because it allows her to judge and adjust according to her activity level.

We also had a burning question to ask: what about driving in regards to her autonomic dysfunction? Em is 16 and would like to drive, but I am concerned about her safety while recognizing her need for independence. She was convinced he would say she couldn’t drive (actually, she was convinced that I would convince him that she couldn’t drive) and was predictably grumpy about it. But, to her surprise, but not really to mine, he said she has no restrictions except that she must be mature and responsible and make the best choices for her safety. He has only had one case of a patient actually fainting while driving and there were extenuating circumstances. So, his advice is: If there is any possibility she might faint, she must pull over. She must stay hydrated and take her medicine. She must be aware of triggers that might cause her to be lightheaded or faint. If she is having a bad day, she might need to choose not to drive. Other than that, she can just proceed as a normal teenager. I think that verdict surprised her a lot.

(Now I just have to wrap my head around the fact that my youngest is old enough to drive. I think we will take it rather slowly and let her get lots of time of instruction in so she feels really confident by the time she actually gets her license. There will probably be lots of prayer involved as well…)

The other drama going into the appointment was that they have always had Em fill out a questionnaire – with questions about her function and other questions she found utterly ridiculous and objectionable. Like, are you depressed, are you bullied, how’s life, etc. She was annoyed the first time she had to do it, peeved the second, livid the third. Her position is that she is not there to be treated for her mental health and it is none of their business. While I sort of agree with her, I also see it from their point of view and know that it actually does all overlap.

This time, she flat out said she would not do it. Period. End of story. If it was to be done, I would have to ‘kill her and physically manipulate her fingers to answer on the touch screen’ ( a direct quote, by the way). That didn’t seem like a really viable option, although I was perhaps a little ready to wring her neck when all was said and done.

While I didn’t personally see it as that big of a deal, she did and I decided to respect that. She has been such a trooper about the craptastic hand she has been dealt and so rarely throws a fit, that just couldn’t see making this my hill to die on, not when she was apparently perfectly ready to make it hers.

And, I want her to be an active participant in her own treatment: she needs to learn to advocate for herself and she needs to be able to take an unpopular position with a doctor and stand her ground. (On second thought, apparently she seems to be fine with that last one! No worries there!) So, I told her I would support her in not participating but she had to find a polite way of refusing. One that did not involve threats of physical violence on everyone involved or general obnoxiousness.

By the time we were called up to get signed in (the point at which the dreaded tablet with the dreaded questions is handed over) she was ready for a fight and I was just sick of the whole thing. And, wouldn’t you know it, there was no dreaded tablet with the dreaded questions this time. No pointless, invasive questionnaire that delved into her mental status. No fight to be had. I don’t know if she was relieved or disappointed!  For myself, I was relieved and I hope that she gained something from the experience that will go with her into adulthood. And, I refrained from wringing the neck of my teenager who decided to act like a teenager for once, so all’s well that ends well!

 

The Newly Revised Driscoll Theory

This post is long overdue (and I apologize for that!) but I said I would post when the updated version of the Driscoll Theory was available so, here goes! (Better late than never!)

You can go to Dr. Diana’s website and purchase the ebook. The $19.99 cost will help fund continuing research (and there is more on the horizon, trust me!)

 

From Dr. D:
This book is written by doctors and patients who live with EDS/POTS every day.
Revealing the cause of “EDS/POTS” results in a new treatment paradigm.
All proceeds from this book will be used to fund further research.
Gentle hugs to you all…

 

I was privileged to help out a bit as Diana was working on this revision, as well as to contribute a piece about our experiences with the Driscoll Theory. Our story (Emily’s Story) is in the book (in Chapter 10), as are touching stories from other patients who have been helped by Dr. Diana’s work. In addition, there are chapters from physicians who have both personal and professional knowledge to share. Of course, POTS, intracranial pressure, mast cells, vascular abnormalities and more are explained and discussed.

Pop over to prettyill.com and grab your copy of the new Driscoll Theory. (I have it on my Kindle, for late night references and doctor visits, as well as my computer, so it is always available!)

Feel free to come back here and comment, once you have read it! If you have questions, either comment here or go to the Ask a Question page and comment there. If I can answer your question, I will. If I can’t, I will pass it along to Dr. D and have her answer you.

Having the Driscoll Theory in print once again is the next step to ensuring the paradigm shift that started more than 3 years ago becomes a standard of care for EDSers. So, it is important that we share this with everyone who could benefit!

Finally, I would like to thank Dr. Diana for her work in making her theory available for all of those who desperately need a bit of hope. I think we all sometimes forget that Diana is just like all the other EDSers out there – she has her own medical challenges to face and she has a family to care for – so that makes this project, that she worked so hard on, even more special. Thank You Dr. D!

 

Now, go get your copy and enjoy it! 🙂

Four Years of EDS

 

May of 2010 was a watershed month for us. On the 10th we went on a field trip to the zoo. Our lives have never been the same since that day. I remember being a little concerned about how Em’s legs would handle all the walking that a trip to the zoo would require; I had no idea that that walking would send her into a flair that would take years to recover from. The fallout from that trip resulted in us figuring out that these strange symptoms were, indeed, EDS and embarking on a long and winding journey that continues even today.

That first May was a time of being battered by this storm that suddenly appeared in our lives. We were in shock and just trying to figure out how to survive. I don’t know that I was even aware that May that it was EDS Awareness Month and, if I did, it slipped by with no acknowledgement on my part. Truth is, I was desperately trying to become aware myself, I had no reserves for sharing knowledge with anyone.

Our first proper EDS Awareness Month, in 2011, found me in even deeper despair than the previous one did, Emily receiving an official diagnosis by the country’s leading EDS expert notwithstanding. For, at the inception of the storm in 2010, there was still hope that perhaps Emily would not be a bad case, that she would avoid much of the horror stories I was reading. By May of  2011, we were in a very low spot because she had not avoided the worst and was clearly a bad case. Not only did she have daily dislocations and severe pain, but she had been plagued by debilitating autonomic symptoms and the neurological symptoms had progressed to the point of the idea of a cervical fusion being floated. She had been in a wheelchair for months at that point and there seemed to be little hope. Little did we know that hope was just around the corner. The next month I would find The Answer  and Em’s life was about to be wrestled back.

May of 2012, saw us frustratingly bowed down by her sudden appendectomy, after months of stunning improvement. We had found the Driscoll Theory and eventually had the opportunity to try Diamox. What a difference it made. Truly, Em had her life back. No, it wasn’t all perfect but the reduction and control of that awful headache gave us the opening to make real progress. To have that scary surgery threaten all of our progress was extremely frustrating. However, we greeted May that year with a profound thankfulness, because we understood how close we came to losing her.

 

In 2013, we flipped the calendar page from April to May with some weariness and caution. Not only were we apprehensive about what the coming year might look like,  but Em was still not completely over her appendectomy; that May, she wasn’t totally recuperated but was finally making headway again. We started getting cautiously optimistic about the future. She was generally doing better, seemed to be stabilizing. It was almost too good to believe.

 

This May, our fourth year of [knowingly] living with EDS behind us, we can look back and see how far we have come. Em is still struggling, but that is to be expected. She is doing well on her medication regime, it is giving her the opportunity to have a life. She has, after all these years, finally been able to do theatre again. She was a nun in The Sound of Music and it was an amazing experience. Maybe it didn’t make up for all the lost years, but it did give her something to be proud of and to aim for.

We have learned a lot over these past 4 years. I have grown and this blog has grown with me. I started off blogging in the depths of despair, in our darkest season. I truly didn’t know if anyone would ever read, but I had to get the words and fear and frustration out somehow. And this blog became my diary, my journal,  my confidante, my support, my sounding board, and now a platform to share our experience and our hope with others.

The purpose of having a month of awareness for EDS is to help others learn about it – to share information about symptoms and treatment, to reach out and support those who live with it and to offer hope, along with reality. To communicate: You are not alone. I remember so vividly reading the horror stories and feeling my heart shatter as I faced my daughter’s uncertain future. I remember clinging to the few hopeful stories I found, desperately clinging to the faint hope they offered.

And, it has been my greatest privilege to offer that hope to others: Life with EDS can be good. Research and treatments are improving the lives of those with EDS and the future gets brighter with every day that passes, even for those suffering the most. Life with EDS is worthwhile and beautiful.  There is a truth and beauty and grace that can bloom in the midst of suffering; you can be happy even living with EDS. Life with EDS is ok. You will be ok. Really.

So, share your knowledge or soak it up, whichever you need to do. Use this month as a path to greater knowledge and let us all spread the word about EDS. We may not be able to cure it, but we can fight it and, together, we can beat it.

Iberogast

Our GI doc was extremely excited about a “new” medication last time we saw him and wanted Em to try it. In actuality, this med is not new – it has been well known and widely used in Germany for decades but has only been available in the US for a few years and has largely flown under the radar here. Apparently he went to a conference recently and became a devout believer in Iberogast – the scientific studies and positive results won him over and he is recommending it to his patients.

I was more than happy to try this herbal concoction instead of another anti-depressant which had been suggested previously but I just wasn’t sure how Em was going to view it once she had it in her hands. From the research I did as we were waiting for it to be delivered, I knew it was going to have a strong herbal taste and I had little faith that she was going to be willing to take it: It is hard to take something so strongly flavored when you are so nauseated. However, I also knew there would be a good chance that if she would actually take it, it just might help significantly.

She turned her nose up and gagged and spit it out on the first try, although it truly wasn’t that bad and she had herself convinced it was going to be horrible. It sat on the shelf for a couple weeks before she tried it again.  The second try didn’t go much better than the first.

We did eventually find a couple important clues to being able to take it. One is to take it in something the flavor doesn’t fight, something that works with the herbal taste and not against. She also eventually found that the key was for her to put the drops in herself so she could see just how little actually is used. (There might have also been a little bribery to convince her to take it, she is not above being bribed with a new pair of shoes she wants. Although, even with the blantant attempt at bribery, she also needed cajoling and gentle encouragement from various family members to give it a try.) She has been drinking  it in hot tea – she is slowly working up to the full amount (20 drops) one little drop a day. At this rate, it might be next year before  she ever gets the full dose…

However, the good news is, even with a partial dose, she is seeing a big difference in her GI symptoms. She doesn’t hurt as much, she can eat more. She is actually feeling hunger for the first time in a long time – she is unfamiliar with the sensation and perceives it as painful most of the time. And inconvenient because hunger needs to be addressed.

Another interesting side effect seems to be a calming effect and an almost immediate relief of anxiety. Again, it is hard to tease out the effects of the Propranolol on her anxiety and the effects of the Iberogast. In reading this page about Iberogast it is clear that one of the ingredients has a calming, sedative effect so she probably is feeling calmer when she takes it. Having the constant pain and nausea relieved probably doesn’t hurt either.

Anyway, once she actually tried it, she found it is pretty amazing and is hooked.  She is working up to 20 drops, 3 times a day – before meals. She is up to 15 drops, I think. It does seem to be having a significant impact and that is a welcome change. It is a natural, herbal based supplement – so while there are no side effects like we might see with pharmaceuticals, one does need to be aware of the ingredients. If you are allergic to or sensitive to any of the ingredients, you would want to steer clear of it. Otherwise, it seems to be a pretty mild, very effective way to address the pain and suffering that come along with the GI symptoms of EDS. My biggest concern about it, at the moment, is that Em has had a mast cell reaction to tea in the past and that seems to be the only thing she can tolerate the Iberogast in at the moment. But, I do think that she is seeing such an improvement  that she will find a way to take it should she end up unable to tolerate the tea.

Iberogast is rather inconvenient to incorporate into life – I can’t really imagine her being able to take it if we went out to eat or ate anywhere but home. She is able to make some tea and take it with her in an insulated cup but I don’t know how practical that is in all situations. However, I think if we were to be out and about, she might be able to just take a peppermint oil capsule in those situations and it might be enough to get her by if the Iberogast is unavailable or unpractical.

We really are at the beginning of figuring out how to use it and make it part of her daily life but it definitely seems to be worth the trouble. I would highly recommend getting a bottle and giving it a try if you have troublesome GI symptoms. It seems like a pretty easy and effective way of addressing the symptoms and the trade off of minor inconvenience is worth it.

If you take already take Iberogast, what do you take it in? What are the best ways to hide the taste for someone who can’t tolerate strong flavors (or is just freaked out by the idea of it)? What are your experiences with it? Comment to share your experiences.