Happy Birthday 2nd Birthday Lanadoodles!

Wrapping her Baby Doll up in Tissue Paper!

Visits from Lana, Sarah and Bob are always a favorite of mine. I've been so fortunate to see Lana three times since April, including today, for a little birthday celebration. Lana went right for her present as soon as she walked in the door and it seemed to keep her entertained for much of our visit. We also had some watermelon to celebrate.

Again Daddy! Again!

She's getting so much less shy and starting to recognize her Auntie Em right away when she arrives. Today she had a little baggie with a card on which she had colored in PINK and a new toy for the puppy that she eagerly delivered. She also willingly gives hugs and kisses and lets me help her with things. She says: "Help?" and looks at me with those big brown eyes. (When she opened her present she handed me each little piece of paper as she tore it off so she wouldn't make a mess! Ha! She so does not take after her Auntie Em!)

Lana Gives Hugs and Kisses Whenever I Ask! :)

We finally got a pic of Lana and I together, as well as Lana and Bob. Next time, I'm making sure to get a photo with Sarah, because I love the whole family!

Visit at the Beginning of April. Ladybug Bowl Hat.

We have a tupperware filled with some random items for Lana's visit, including three plastic ladybug bowls, a wooden spoon, a spatula, crayons, coloring books, a purse, and a few other things. When Lana arrives she knows to go right for the box that awaits her arrival. Our favorite is when she wears the bowls as hats. :) But she also makes us all sorts of yummy goodies with her spoon, spatula and bowls.

I'm tired. I need a nap!

She was so sleepy, we got out pillows, teddy bears and my prayer shawl. She got everything organized as if she was going to take a nap. 

End of April Visit. I know how to color now. PINK is a good color!

She looks so grown up with her new haircut and bangs! 

Blessings and Happy Birthday to My Wonderful Niece! I fall more in love with this little girl every time I am with her. I thank Sarah and Bob for their friendship, love and kindness. And I thank them for blessing me with being an aunt to their daughter and making her such an integral part of my life. It is a true joy to be her aunt.

Emily

Comfy?

This is for my paws to rest on, right?

We have no idea why Asher finds putting his paws over the base of chairs, tables, stools, etc. so comfy, but he does. Here he is perfectly content under the coffee table. He's also freshly groomed, so he looks extra handsome (or at least we think so). :)

Hanging Out..

Blessings,

Emily

Cousin Jan! Cousin Jan! Cousin Jan!

Cousin Jan and Me.

I am always SO excited to get to see family. Cousin Jan, the wife of my cousin Mike, was in town for a conference and we got an afternoon visit out of the deal. :) I've known Jan since I was as little girl, since she and Mike started dating and visited us then. We're hoping that next time we get to see each other she is able to come with more of the family, especially her youngest child, Lizzy. 

Thanks for the wonderful afternoon visit, Jan!

Blessings,

Emily

Puppy Countdown!

June 10. Our girl!

Now that you've endured reading two long medical posts, here are some fun puppy photos. The top photo is the most recent photo we have of our girl. Lowchen have small litters, so there are only three pups in this litter. The two girls are going to homes and the boy is staying with the breeder. We finally know, with about 90% certainty, that this is the girl the breeder has chosen for us. She loves to be held and give kisses.

We're less than three weeks away from bringing our girl home on July 3rd! I have narrowed the names down to three, and hopefully as I see her grow more (or when I finally get to meet her), I'll know what her name is meant to be. 

The breeder is great about sending regular stories, updates and photos, and now it's fun to go back and look at all of the photos now that we know which puppy will be ours. I love photo days! :) In addition to connecting with this wonderful breeder, I've now been in contact with another woman who owns one of her Lowchen, as well as the other family who is getting a puppy from this litter. I'm enjoying this community of women and dog lovers!

Thanks for sharing in my joy. I have not felt this joyful or excited for a very long time. I feel like an expectant mother, busy nesting and getting everything I can ready before our girl arrives so that I can spend all of my time with her. :)

Enjoy the photos. It's amazing how quickly they change in such a short time!

Five Days Old. (Our girl is on the left)

Nine Days Old. Our girl is in the middle.

Seventeen Days Old. Look at their pink noses! Mama is ever-so-watchful she can't catch a break for some grooming. :) That's our girl digging in for some food!

Nineteen Days Old. Just the girls (ours is on the right.)

Twenty-four days old. Our girl is in the middle.

Moving day--June 2nd. The puppies move from the whelping box to their new little home.

June 4. The puppies have their first real food--Goat's milk, raw honey, and slippery elm (an herb). Usually this is a messy process, but as you can see everyone was pretty neat and tidy, except the boy got one paw dirty. Our girl is in the front left.

June 8--the puppies have their first exposure to the Lowchen Lounge--the room where all of the other Lowchen in the family live--and go straight for the toys. Our girl is on the left.

Dissonance or Discovery?

Don't blink! The blooms only last a few short days.

Now that you've been brought up to speed on our past explorations of the neck issues, here is the latest on the new testing. So far I have had the thoracic MRI and the MRI in flexion and extension done. Both were completely clear. I will share some parts of email exchanges between myself and Dr. ANS regarding the results, what happened to me during the MRI in flexion and extension, and where we go from here.

First I had the thoracic MRI done with the following results:

Emily: The MRI films arrived late last week, and I have had a chance to review them. They look excellent, with no bulging discs at all, which is impressive, and no areas of stenosis or signs of disease. That doesn’t help us figure out what is causing nervous system irritability, but it does clarify things a bit further, emphasizing that the anatomy looks good and that you are not a candidate for surgery. 

Have I mentioned how much I love this tree?

After the thoracic MRI, most of our discussion has revolved around the neck MRI. Because there are only about nine facilities in the entire country that do seated MRI's in flexion and extension and none took my insurance, we opted to do an MRI lying down in flexion and extension. Here is Dr. ANS's review of the MRI in flexion and extension:

Emily: the MRI arrived late last week, and I had a chance to review it over the weekend. The quality of the film was quite good (often the motion of the positions of flexion and extension leads to blurring, but these images were crisp). The images show no compression of the cord when you are in positions of flexion and extension, and there is ample spinal fluid cushioning the spinal cord in both extremes of neck bending. The cord itself looks fine, and the discs are not bulging, so there just doesn’t seem to be a visible anatomic cause for the symptoms you get. This would suggest that the problems you get with neck movement are originating from the peripheral nerves (which we can’t see well on MRI), especially those emerging from the cervical spine area, rather than in the spinal cord or from spinal stenosis. So, as they might say on Sienfeld: “No neurosurgery for you!”

More!

I didn't go into the MRIs with any expectations of finding something because I've spent 14 years getting negative test results. Even without expectations, when these results came back I found myself confused, disappointed, and generally emotional. This was, as my friend said, a classic case of good news bad news. I don't want surgery. But I do want an answer, darnit! It's frustrating to have such an extreme reaction to the test itself and such major neck pain and not have any evidence of what might be causing it.

I'm pretty much a pro at MRIs and don't mind them, but knowing what flexing my neck backwards can do, I was anxious about this particular type of MRI. You are placed an open MRI. The first scan is done with your neck pressed forward (flexion) and the second scan is done with the neck pressed backwards (extension).  Each scan is about 15 minutes long.

Here is my description via email to Dr. ANS of what I felt like during the MRI:

In flexion: Mostly I just felt a pull from the lower cervical/upper thoracic area like a string through to the front of my chest (common complaint I've mentioned before) and some difficulty getting a full breath. I also had forceful heartbeats.

In extension: It was very difficult to get my neck as far back as they requested and the tech basically forced my neck into position. He commented on how clearly he could see I was in pain. It felt as if the vertebrae were smooshed (good word? ;)) and there wasn't room for them to bend that way. Sort of like an accordian. It was painful, but also just unpleasant to have that sensation that things were being forced and compressed. I did a lot of meditating during this part of the test! Gradually I started to sweat. Then have nausea. Then dizziness. The feelings resembled what had happened to me on the TTT (Tilt Table Test) or what I get when I am in ANS distress. I buzzed the tech to let him know, but we pushed through the last 4 minutes without me fainting.

Blooms.

To help me understand what these results might mean and where we should go from here, Dr. ANS wrote the following. (You can get a sense of why my file folder is so HUGE with all of our email exchanges over the years!)

The MRI results are indeed puzzling, given how much movement causes a symptom flare so consistently. One comment is that the MRI is still a relatively crude diagnostic instrument for whatever it is that you have, which is some kind of adverse tension in the spinal cord as a best guess, but not one associated with obvious anatomic defects. I am not sure how to work on the peripheral nerve aspect of the problem, since we don’t have that properly defined. It will be worthwhile to see the vascular surgeon about thoracic outlet syndrome to see if your presentation and symptoms (especially the excellent description below about how the different flexion and extension postures affected your symptoms) ring any bells for him (or her).

I don’t know what to think about the possible causes of this group of symptoms, but I still think an infectious origin is possible. It just is very puzzling, and I am not certain what we should do about it.

Anyway, let’s see what Dr. [Listener] and the thoracic surgeon think, and then put our heads together again.

:)

Last week, I had an appointment with my PCP, Dr. Listener, and we set up the next phase of the plan. I have an appointment with a neurologist in August for a full neurological exam and review of the MRIs. I've worked with this neurologist before regarding my abdominal migraines and he works well with Dr. Listener, so I feel he will take me seriously. There is also a four page letter in the medical system from Dr. ANS, which all of the doctors take very seriously!

Dr. Listener also knew a vascular surgeon who travels weekly from the main hospital for appointments at the clinic where I see Dr. Listener. Dr. Listener will talk to this surgeon about my case, show him Dr. ANS's letter, and get back to me as to whether this doctor or another surgeon is the best fit for me to be evaluated for thoracic outlet syndrome.

So far I'm not sure if we have more dissonance or more discovery! I'm trying to pace myself emotionally and physically through this process. Maybe we're gradually unraveling things, but it appears to be another complicated process. Most days I try to focus on the now, especially puppy preparation!

If you're still reading these oh-so-compelling updates, I thank you for hanging in there! I thank you not just for reading my blog, but for all that you do to continuously bless my life. If you think of it, send a little prayer up for the doctors who take such good care of me. 

Blessings,

Emily

What the 'Neck' is Going on?

Cherry Blossom: Impermanence. The beauty and transience of life. 

--from The Language of Flowers by Vanessa Diffenbaugh--

Cherry Blossoms

Our cherry blossom tree has always been my favorite tree, and I've grown increasingly attached to it throughout my illness. As I was listening to The Language of Flowers, I learned the meaning of a cherry blossom. Perhaps there is a reason I've been so drawn to this tree all of these years? For some reason the meaning of the cherry blossom felt perfect in the midst of our search for a new answers and new diagnosis, while I also work to focus on and create beauty in my life.

As promised, here is a continuation of the medical journey as of late from the post Out of Dissonance Comes Discovery.

First I want to comment on the outpouring of support and comments I received in regards to that post. Wow! I'm continuously amazed by your generosity of time and love. I will also try to answer some of the questions that arose. I agree with all of you that I have AMAZING doctors on my team, and have now for many years. Having Dr. ANS as my 'point guard' for the past eight years has been an invaluable, incredible and humbling blessing.

Our beloved weeping cherry tree.

I'd like to take a step back in time before going directly to the most recent testing. Since I first saw Dr. ANS in 2004, he has been incredibly tuned in to the neck issues. Over the years we've done brain  MRIs as well as MRIs of my cervical, thoracic, and lumbar spine. We have enough data from these to see that no obvious anatomical abnormalities exist. We also can identify that no significant changes have occurred over the years. Dr. ANS works with many patients whose ANS issues are caused by either cervical stenosis or something called Chiari malformation. He works closely with a surgeon on these cases and my MRIs have been reviewed by that surgeon in the past. He found nothing of note. Originally, when we first looked at my MRIs in 2004 Dr. ANS suspected that my spinal column was slightly narrow, but further investigation revealed this not to be the case. Dr. ANS is one of the pioneers in connecting cervical stenosis and Chiari malformation to POTS.

Blooming.

Over the years we really expected to find evidence of cervical stenosis, given my incredibly strong family history of this. As you all know by now, my mom has severe spinal stenosis in both her cervical and lumbar spine, both of which she has had surgery for. My mother's only sibling, my uncle, has also had surgery for cervical and lumbar stenosis in the same vertebrae as Mom. Lastly, my grandmother had surgeries in the same locations. Even though my symptom presentation differs from that of my family, we truly thought we would find something. Guess what? My MRIs look PERFECT in this department. We would expect by my age I would start to show some evidence of stenosis, since my mom and uncle both noticed symptoms, such as sciatic pain at young ages. 

Love this tree!

When my MRIs all came back negative for stenosis and Chiari malformation, Dr. ANS started searching for alternative answers to the neck issues as well as the ANS dysfunction. Because I had been unresponsive to all of his 'tricks' to improve my health--medications, PT, etc. we pursued the possibility of an infectious base for my problems, such as Lyme disease and its associated co-infections. As you all know, I've been on the Lyme journey now since November of 2005. After 7 years of treatment for Lyme disease (under the care of two different Lyme doctors), and very little if any progress, we decided to step back and see what else might be going on. To be honest, I've never felt sure about the Lyme diagnosis--but because it is a disease that is so nebulous, manifesting itself differently in each person and having no reliable testing--it is hard to feel sure about it. It is important to note here that over the 14 years I have been sick, I have been tested for hundreds of other conditions before we made the decision to treat me for Lyme disease. This testing and search for alternative answers did not stop during the Lyme treatment.

We've had to come at the neck pain using medications for symptom management to decrease the pain, but not solve the problem. I've had significant improvements from using Lyrica, Lidoderm patches, the LDN, Klonopin, etc. In another post, I will explain more about what this neck pain has felt like over the years. We had hoped that treating me for a systemic infection and inflammation would have significantly reduced the neck pain and gotten to the root cause of it. I do think the antibiotics may have helped some, but I also think a lot of the improvement I have had is due in large part to the medications aimed at symptom management.

Canopy of Blooms.

What I do know is that over the years, I increasingly found myself 'not fitting in' with anyone else on the forums for Dysautonomia/ANS Dysfunction or Lyme Disease. I couldn't find a single person like me. Dr. ANS and Dr. Complex have both told me that they do not have another patient like me and have never had someone present the symptom profile the way that I do. This is unbelievable considering that Dr. Complex has been practicing medicine for 50 years now with and deals with incredibly challenging cases and that Dr. ANS has seen thousands of patients with CFS, FMS and ANS dysfunction.  This is what has made solving my case so incredibly challenging to them. We are really having to dig deep for more and more unusual causes of my symptoms. 

At our appointment in February, Dr. ANS told us a story about a young boy who was very sick, but no other doctors were taking him seriously. Certain that something was not right, Dr. ANS kept looking for possible causes of this patient's unusual presentation of symptoms. He finally placed him in a seated MRI with flexion and extension where he found that the small bone at the base of the skull was pressing on the base of the boy's brain stem when he was in extension and he had very little space at all even when his head was positioned normally. Dr. ANS is remarkable in many ways, including his ability to remember each of his very unique clinical experiences and apply them to other patients. He will often say to me: I have one patient who has had success with such and such. And we'll give it a shot. Every detail of his treatment is individualized. 

What I know is that from the time I first became sick in 1998 I kept saying: something is wrong with my neck. How can something not be wrong with my neck? I feel a bit as if we have come full circle in re-focusing our energies on my neck.

To be continued...:)

Blessings,

Emily

Pretty

Mom all dressed up

We're always snapping photos of me and Asher, but not as many of Mom, mostly because she's usually in her 'work clothes' and not wanting her picture taken. She dressed up the other night to an event as "Jeannine's Mom" and I snapped a few photos. This one makes me smile and I wanted to share. :)


Blessings,


Emily

Josie and Bruce: A Lovely Reunion

Josie, Bruce and me

While I try to continue the medical update, I also want to catch up on sharing photos from fun stuff and visitors. I'm way behind! 

I continue to say that Facebook has been such a positive source of connection and reconnection, because here is yet another example of how it has enriched my life. In high school Josie and I were both part of a group called Peer Listeners, a group of students available to our peers to provide a listening and confidential ear. We trained together as a group, which is how I met Josie.

Our experiences training together left an indelible mark on my heart. One day we went for a retreat, during which we engaged in activities such as trust falls, rock climbing, and repelling. Despite her disabilities, Josie found a way to repel that day! I remember standing and watching her, cheering for her and admiring her confidence. I was self-conscious about everything, and I was in awe.

I always wondered how she was doing, we were reconnected through Facebook, started texting regularly, and have now been trying for at least a year to get together for a visit. We struggle with very different disabilities--which presents us with different perspectives but lots of empathy for each other. We're also HUGE dog lovers. :) 

So, Josie and and her husband Bruce made it over for a lovely afternoon visit. They are the sweetest couple--showing each other such patience, appreciation, and respect. We had so much to talk about, poor Bruce didn't get to say much, but he listened patiently (and he wasn't even the Peer Listener growing up :))! Josie, Bruce and I discussed the complications that come with saying a person with a disability is an 'inspiration', so I will leave it that I admire their resilience and their zest for life in the face of challenges. They are wonderful teachers for us all.

Thanks so much for the visit you two! I hope you will come back to meet the puppy! :)

Blessings,

Emily

Out of Dissonance Comes Discovery

Dr. ANS's medical file for me!

Since our visit to see Dr. ANS on February 28th, I've been silent as to what is going on medically. This is in part because it has just been too difficult to find the time and energy to blog, and also because I'm still trying to process all that we learned during those back-to-back appointments with Dr. ANS and Dr. Complex. For those of you who have been wondering where things are at, here's the scoop!


This was my first face-to-face visit with Dr. ANS in 8 years, but as you can see from the photo of my file, our communications via email and phone have been extensive (!!). Here's what we learned during our three hour visit. 

Peony Preparing to Bloom

After spending a great deal of time going over my symptoms, daily activities, etc. Dr. ANS did an extensive exam, including several neurological tests and physical therapy screenings. In the physical therapy screening he found that passive straight leg raises or arms overhead provoke symptoms such as forceful heartbeats. In the neurological screening he found that my Romberg test was positive (falling within seconds and my first positive on this test); he was unable to find a radial pulse in my right arm when it was raised above my head; and when he asked me to lift my hands above my head while opening and closing my fists, I was unable to keep time with him, had slower verbal response, flushing, and sweating. Any type of neck extension provokes symptoms.

Pink Peony

My exam led Dr. ANS to say: This is NOT a normal neurological exam. Something is going on. Pointing to my file, he said he felt that if we looked at the entire eight years worth of correspondence the thing that stands out to him the most is the neck issues. What screams out to me is the neck, he said. Do I have some sort of anatomical abnormality that is causing such constant and persistent symptoms that have been unresponsive to any type of treatment, he wondered? An anatomical abnormality might cause such persistent symptoms because something (whatever it is that is going on) is causing a constant agitation of the ANS, never allowing it to calm down even with treatments aimed at doing so.

Pink Peony

Dr. ANS shared a story with us that he often teaches his medical students. When looking at a case like mine that is difficult to solve, he presents the line from a poem: "Out of dissonance comes discovery." In my case, the question is what doesn't fit with all of his other CFS and ANS patients? What doesn't fit is 1. my neck issues, 2. my lack of lightheadedness compared to other POTS patients, 3. my ANS responses in general. It's at once extremely difficult and incredibly validating to hear Dr. ANS say that I am unlike any other patient he has ever seen.

Pink Peony

Dr. ANS suggested that we pursue the route of searching for a possible anatomical abnormality. This would include a possible instability at the base of my skull or something like thoracic outlet syndrome. I won't go into all of the details of all of these things until I am further into the testing. The proposed testing was 1. Thoracic MRI, 2. MRI of the neck in flexion and extension, and 3. meeting with a thoracic neurosurgeon to investigate the possibility of thoracic outlet syndrome. So far I have had the first two tests done and will share the results in another post, as it is a lot to process.

We also discussed some possible medicine changes that I could make now or in the near future. One option is something called Low Dose Naltrexone (LDN) which is now being used for pain in patients with Fibromyalgia, CFS, etc. It's basically a teeny tiny dose of Naltrexone and was used for opioid addiction. I am trying this medication already. I am tolerating it, but it is too soon to tell if it is benefitting me.

Option number two is a new medication called Droxidopa (a norepinephrine precursor) that has been used in Japan now for many years in POTS patients and will hopefully be approved by the FDA and on the market as early as this Fall.

Since I had seen Dr. Complex the previous day, I wasn't sure how to integrate the two appointments. Later, in a phone consult with Dr. Complex, he was completely on the same page with Dr. ANS, which is GREAT! We are continuing to use the supplements I've been using, but I am not adding anything else in at the moment and am not doing any treatment for Lyme or other infections. I appreciate so much the mutual respect these two doctors have for each other and their willingness to work together. They truly do not put their egos in the way: they are both so focused on just helping me to feel better. What a blessing!

Pink Peony

Dr. ANS spent a lot of time at the end of the appointment discussing my options. What makes Dr. ANS so Amazing is his ability to honor, acknowledge and validate his patients. He said: "I wish I could tell you that one of these things (the MRIs, the thoracic outlet syndrome, the LDN, the Droxidopa) would be a 'slam dunk'. But I can't tell you that." Instead, he encouraged me to pursue each of the options as *I* felt emotionally ready. He wants me to pursue these options at my pace because, as he said, he doesn't want to look back 8 years from now and say: "I wish we would have looked at this sooner." 

He took time to tune into my struggles to feel that my life is meaningful, pointing out the ways in which I am magnanimous with what I do have, and that is the most we can do and be. He commented on my maintenance of a loyal circle of friends and family in spite of my illness, which he says is very unusual. I tend to attribute my friendships to the fact that my friends are, well, loyal and awesome. But he pointed out my role in creating these relationships. This is just a little snippet of what he said. What he said to me was so moving, so personal and so powerful that I will always carry it with me, but I won't share it all here.

In addition, Dr. ANS shared how meaningful it would be to him in his life to get me feeling better and solve this ongoing puzzle. Perhaps he will be called Dr. Amazing from now on?

Hearing that we may be taking a completely different path towards diagnosis was both exciting (that we might actually find an answer) and overwhelming (that 14 years later I'm still in for more tests and more doctors visits in search of these answers). What has helped me through this process tremendously is the permission and encouragement Dr. ANS gave me to take things at a pace that I could manage emotionally. I've been able to balance the pursuit of new answers while doing my best to live well now (aka new puppy!). 

Blessings,

Emily