Sunday, April 28, 2013

Art Show About Ehlers-Danlos Syndrome to Raise Funds for EDS Center

Super exciting news here...I am having an art showcase in May to raise funds for the new EDNF EDS Research & Treatment Center! The art show will be on May 18th, 2013, 8pm, at Kitamu Coffee in Hilliard, Ohio, and the art will stay up for the remainder of the month. The coffee will be flowing and live music will fill the air. If you are in the area, please join us for the fun! If you live elsewhere, I will be posting the artwork here when the show is over.

In honor of EDS Awareness Month, I will be sharing my art, all modeled after Life with EDS. Being disabled, this project has taken a lot of time and effort, and was only able to come to fruition with a lot of time, patience, and support from family and friends. At the event, donations will be gathered to help fund a new research, training, and patient care facility right here in U.S. - the first of its kind in the world!
 

My goal is to educate people here in Columbus, Ohio, about EDS with the hopes that more people will be diagnosed, strengthening the network of doctors that treat the disorder and helping those who face the uncertainties of living life with an invisible illness. In sharing my art, I am baring my soul in order to help others.

If you can't make the event but want to donate to help fund the new EDS treatment center, please comment below and we will contact you to arrange for your donation. 





Just a few snippets for you...




May 18th, 2013 - 8pm
Kitamu Coffee
3221 Hilliard Rome Rd.
Hilliard, Ohio 43026
(614) 282-4324

xo
dani

Wednesday, March 27, 2013

What Can I Do for May Ehlers-Danlos Syndrome Awareness Month?

A question that comes up a lot when talking about EDS Awareness Month is, "how can I help?" Well, I am here to tell you that any bit of effort you put forth, big or small, will help increase Ehlers-Danlos Syndrome Awareness. Whether it is talking to a friend about what your average day is like living with an invisible illness, or creating a giant event in the community, every single action counts. I am an activist at heart, so getting the word out about things is in my blood. If there is something that needs to be aired, I have no problem standing up and speaking my mind (though, I have terrible social anxiety, so panic attacks can ensue!), holding a sign in protest, or writing a blog post, newspaper entry, or spreading the message via social media. Because of this, I have some tips for you, my readers, for things you can do this year to help all of us. In addition, later this week, I will be writing a post on what I am doing for EDS Awareness Month this year -- and it is big and exciting and involves art!


Proclamations:

First, you could work on getting the Governor of your state to pass a proclamation making May EDS Awareness Month if there isn't already one in place. To find out if there is a proclamation already, check with the EDS Proclamation Facebook Group, as they have all the current info for 2013. You need a Facebook account, then you need to become a member of the group by "asking to join." If you find out there isn't a proclamation in your state, go to your Governor's website and find the link to request a proclamation. For example, Ohio's proclamations are requested through the Governor's contact page. There is a drop-down menu and you click "Proclamations" and then add the text for your state-specific proclamation and your contact information. They will typically follow up with you a few weeks later. Feel free to use the templates I provided and tweak them to represent your state. Also, don't contact Nevada, Ohio, or Oregon with a request - we already have a proclamation for 2013. You are welcome to mail a thank-you letter, though!

Templates: 
Proclamations Requests, Proclamation Text, Thank-You Letters


Proclamations I got passed:
2011: Nevada, Ohio
2010: Nevada




Pass out posters and flyers:

Posters are an easy and effective way to spread EDS Awareness. There are all sorts of places you can try to put up posters. Don't forget to bring supplies with you: tape, scissors, stapler and staples, and pushpins are great to carry in a bag with you! Also, you can bring a copy of the state's proclamation, if there is one, to show the business/organization, or you can bring some info about EDS to show them, like an EDNF MRG (print this, two-sided, & fold it in half - it provides a great overview of EDS)!


There are some really great posters that were designed and made for EDSNC. They are copyrighted, but we can copy and print them in order to pass them out and spread awareness. The best way to do this would be to get the image from here, download it (save it to your computer) and make copies. You could make letter-sized copies or even poster-sized glossy copies. If you need hi-res images, you may need to ask EDSNC how to get them. You also may need to "like" them on their Facebook page in order to view the images. Take the images to an office store to make bulk copies. And, color copies always make a bigger impact than black-and-white. You can make an 11"x17" color copy for 99 cents at Office Max -- it seems to be the perfect size to plaster all over.





Where can you leave the posters/flyers? 
(Bring a copy of your state's Proc with you, if you have one!)
  • Ask your doctors' offices or hospitals if they would be willing to post them in the doc's rooms or the lobbies. Explain to them why this would help.
  • Go to other doctor's offices around your town, even if you aren't a patient, and talk about it with them. Maybe you can even leave copies of the flyers (the general one) in their lobby. 
  • Check with Physical Therapy offices.
  • Your local coffeeshops, delis and grocery stores often have bulletin boards, try there.
  • Talk to friends and family about spreading them around.
  • Some places of employment will allow them to be posted or passed around breakrooms.
  • Pediatrician's offices would be a great place.
  • Try local schools, daycares, preschools, etc.
  • City or Town Halls.
  • Youth facilities, low-income and at-risk youth centers.
  • Community centers.
  • Churches.
  • Pools.
  • Skating rinks.
  • Anywhere else! Just be sure to get permission before posting something.
  • SCHOOLS: Schools are a great place to put up posters. Elementary schools have boards by the office that the parents see, middle and high schools usually have boards up in the halls that the students see, and college campuses have multiple areas that posters would be appropriate. Getting permission from School Districts is a must, so please read the post I did on getting school board approval first. Usually contacting the communications department is a good start. 

  • GENETICS CLINICSEDS Network Cares is asking that everyone try to get 3 posters put up in their local genetics clinic for May EDS Awareness Month. Please call your Genetic doctor or clinic in your area and ask them if they would be willing to put these EDS posters up in their office. If they say yes, ask them how many sets of posters they would like. Each poster is 11"x17". Please send the doctor's name, address, and phone number to EDSPosters@gmail.com. 






Speak at a local venue:

Are you a member of a local church, Girl or Boy Scout Troop (member or leader), knitting club, sports team, or any other community gathering? If so, it might provide the perfect avenue to take some time to talk to your peers about Ehlers-Danlos Syndrome. The first step would be to talk to the organizer of that group and ask if you can have some floor time at your May meeting/event/competition (any month works if you can't make May happen!), and gather up some talking points. Just be candid. You can make small copies of posters or flyers to pass out, if you choose. You can pass out MRGs (Medical Resource Guides). You can have a very intimate conversation, or just gloss over a few major symptoms. If you are school-aged, what about asking your teacher if you could write an essay and read it in front of your english class for extra credit? Whatever you are comfortable with, make it work for you and your setting.

For example, while I don't attend a church, many of my readers do. You can talk to your Pastor and ask them if they would allow you to talk to the congregation for a few minutes after your Sunday service. You can just tell the folks about Ehlers-Danlos Syndrome, why it is important to get diagnosed, what it is like to live with EDS or love someone with EDS, what some of the symptoms are, and explain to them that May is EDS Month Internationally (though not officially recognized everywhere).

While public speaking can be very difficult for some people, myself included, it really can make a world of difference because it allows you to educate a large group of people all at once. Can you imagine if 10, 100, or 1,000 people who didn't know about EDS, all learned about it in 5 minutes just because you were able to talk to them? Knowledge is power and it spreads like wildfire. Don't be afraid to try speaking.
"Speak your mind even if your voice shakes."
- Maggie Kuhn

If you'd like, download EDNF's General EDS Medical Resource Guides (MRG). Don't forget, they are double-sided. If you print them at home, just print on both sides of the paper and then fold in half. It presents like a leaflet and is a great tool to pass out to people when you are talking to them about Ehlers-Danlos Syndrome.







Raise Funds for New EDNF EDS Clinic:

Seriously, a center for Ehlers-Danlos Syndrome! An entire clinic for EDSers...who doesn't want that?! This still gets me giddy every time I think about it!

Photo from EDNF.org
"EDNF's goal: Realizing the dream of physician training, clinical advancement, and research. EDNF seeks your support in endowing a facility that will be the world center for EDS, from physician training and clinical practices to scientific research. The opportunities are boundless; you can help the lives of children and families by advancing the progress towards solving a genetic disorder that affects the fundamental tissue of the body. You have the chance to join us in this life-changing endeavor and improve our future, from site selection through scientific breakthroughs and beyond. This is just the beginning. Our dream doesn't end with one center: we envision a global network of centers working towards a cure"

Hold a bake sale to raise funds:
Check out the recipes!

 Vegan chocolate and vanilla cupcakes with vegan whipped cream, fruit, and cocoa powder.

Seriously, who doesn't like baked goods? Whether sweet or savory, food moves people. Have you seen Instagram, full of people obsessing over their plates? Food is great way to motivate people and a great way to make a few dollars in the name of EDS. Hold a bake sale at your work, school, church, or group/team, and donate the proceeds to EDNF's soon-to-come-to-fruition EDS Center. You can actually donate money specifically to this facility, not just to EDNF. How rad is that?! And, if I were raising funds, I would just make a note that the money had to go toward non-animal tested research or to other non-animal related expenses, like patient treatment, facility supplies, etc.

 





Decorate your car:

Choose to paint your car for the month of May, or get some bumper stickers or magnets. Your car is visible by many people each day, so why not spread the message as you drive?

Purchase a paint marker from a store like Target or an automotive store, or buy bumper stickers from Cafe Press or Zazzle.






Donate Dr. Brad Tinkle's book to your doctor(s):

Dr. Brad Tinkle's Joint Hypermobility Handbook - A Guide for the Issues & Management of Ehlers-Danlos Syndrome Hypermobility Type and the Hypermobility Syndrome is like the Bible for EDSers. It provides comprehensive information on Ehlers-Danlos Syndrome, diagnosis, treatments, etc. It has been the best reading material for an EDSer that I have come across. Plus, Dr. Brad, as he likes to be called, is my doctor and I love using him to help me spread the message about EDS. As you know, educating our doctors is one of the very most important things we can do to help ourselves. Most doctors have only read a paragraph or two about EDS in medical school and never thought about it again -- until they met us. We need to help educate the doctors so they can help treat us properly and so they can help get more people diagnosed. While it is known that about 1 in 20,000 people have EDS, we know it is closer to 1 in 5,000, but those others have yet to be diagnosed. Help them by helping your doctors.


Purchase Dr. Brad's book and give it as a gift to your doctor during the month of May, with a card explaining why you are giving it to him and how much you would love it if (s)he would become more informed. Present it in a way that it makes your doctor feel appreciated and respected, but also that you would like to help them learn a little more in-depth about what an EDSer experiences. Gift wrap (recycled is better!), a bow, and a nice card go a long way in presenting the information without coming across as a know-it-all or rude, even though you know more about your body than your doctor probably does!
Buy the book in the Life with EDS Store!



Contact your local newspapers or TV stations:

In order for news outlets to run a story, something must be newsworthy; there usually must be a local catch and even a local event. Without those, there isn't much for the outlet to write or run a story on. Here is where you come in. If you can create something that has flare, it may be more likely to get coverage. For instance, if you are having a May EDS event, you can contact your media outlets to see if they will cover your event or interview you ahead of time. Just Google to find out the contact number and call the news desk (TV) or editor (newspaper). Alternatively, if you are savvy, you could write a press release and submit that via email or fax.

If you are not doing an actual event for May EDS Awareness Month, you can still try to get something in your local newspapers by writing a Letter to the Editor (LTTE). This is a great way to get Ehlers-Danlos Syndrome in the paper, and editors usually run all sorts of LTTE topics. It is very likely that your letter will end up in your local paper or online.

Template:
Letter to the Editor  
Example of letter that started as LTTE on Washington Times Communities

Article I got in the Nevada Appeal Online Edition in 2011:






Use social media to spread the word about EDS:


If you have accounts on Facebook and Twitter, a very simple and easy way to spread awareness is to dedicate your May feed to EDS. All you have to do is post one thing a day, for the whole month of May, and all of your friends & connections will learn a ton about Ehlers-Danlos Syndrome. If 31 days of EDS is too much for you, or you feel it would be overload on your friends, just pick one week or one day a week to post. If you blog, write a post about EDS. If you have a food blog, for instance, do a post on nutrition for EDSers. If you blog about crafting, how about a post on ways that your artwork helps you feel better? Even a quick tweet about donating to EDSNC or ENDF would help our cause.

Some things you could share on your profile:

Myths & Facts About EDS

Each day you could share one thing EDS; for instance, on May 1st you could post that "People with EDS may have done "party tricks" as kids, twisting themselves into pretzels," and just do one for each day of the month.

You could share links to donate to EDNF, EDSNC, EDS Support UK or any other EDS group.




 
Snail Mail "An Open Letter to those without Ehlers-Danlos Syndrome" to friends and family:


You may have come across this letter on my blog before, but it probably still holds true for you if you have Ehlers-Danlos Syndrome. Snail mail is way more personal than email, so you could print out a copy of the letter, include it in a card, and send it USPS in the month of May to those who you care about so they understand what it feels like to be YOU, living with EDS. To update, the author of the letter is Michael, aka Deaderpool, and I wanted to make sure to credit him. He has written a letter that universally holds true for most of us. If you don't want to snail mail it or hand it in person, email should still do the trick.

Print and mail:
An Open Letter to those without EDS






That's all I have for you. If you think of anything else, feel free to post your ideas below. So much love to all of you, and here's to the best May EDS Awareness Month yet!

xo
dani

Tuesday, March 26, 2013

Proclamations Make May 2013 EDS Awareness Month in Nevada, Ohio, & Oregon!

As you know by now, I strive to get proclamations passed each year by the Governors in states that hold a place in my heart. This year, I got to work early and now have official documents in hand from three states: Nevada, Ohio, and Oregon. Nevada's Governor Sandoval, in my home state, passed the proclamation making May 2013 Ehlers-Danlos Syndrome Awareness Month for the fourth year in a row. Ohio's Governor Kasich passed our EDS Awareness Proclamation for the third year in a row, in the state I now reside. And, for the first time ever, Governor Kitzhaber of Oregon passed the EDS Awareness Proclamation in the beautiful Pacific Northwest! I dedicate the Oregon EDS Awareness Proclamation to my EDS bestie who lives in Oregon, Shellie, and her beautiful family. Tomorrow, I will post info on what you can do locally to help spread awareness and what activities you can organize or participate in where you live. Hope you all are well and having a low pain day! 

Nevada:



Ohio:
 

 
 Oregon:



 xo
dani

Friday, March 15, 2013

New EDS Group in Columbus, Ohio!



The Columbus EDS Support Group just had its first meeting in February. Being in a room with others who have Ehlers-Danlos Syndrome feels so wonderful. Surrounded by people who completely understand what we go through, we were able to share our own stories, compare symptoms, talk about treatments, and discuss increasing awareness in our community. It was empowering and humbling. To see if there is a group in your area, visit Chronic Pain Partners or EDNF.

For those of you in Columbus, Ohio, or the surrounding area, please check out the group's site to get information on our upcoming events and projects.



xoxo
dani

Thursday, March 14, 2013

From Needles to Scalpels...Renal Autotransplantation is a Go!

Acupuncture did not help my flank pain at all (on a side note, it did help my knee pain, but I found it to be uncomfortable and not-at-all relaxing, which was different from most people in the room who were fast asleep during treatments). After a month of needles, it was back to the drawing board. I talked with Urology Doc at The Cleveland Clinic, he confirmed my diagnosis of Loin-Pain Hematuria Syndrome, and we scheduled my renal autotransplantation surgery for August 1st. The surgery will be 6 hours, and I will be in the hospital for a week, then at home healing after that. I will blog about it all when the time comes. After the kidney surgery, we will plan the C1-C2 fusion for my cervical instability.



If you don't remember what LPHS is or you need a recap, along with a detailed explanation of the surgery, visit my other posts:



Hope you are enjoying March! Love and hugs!
Happy World Kidney Day! 

xo
dani

Saturday, January 26, 2013

Update on Acupuncture Trial for Flank Pain

Just wanted to give a quick update on seeing an acupuncturist for my flank pain and for Loin Pain Hematuria Syndrome. As mentioned before, I am going twice a week for one month, to see if it helps my flank pain before I opt to have surgery. I have been three times so far, and I have seen zero results. There has been no reduction in pain whatsoever. That doesn't mean that it won't help, but it certainly hasn't yet.


My acupuncturist was a bit surprised that it isn't helping. She sent me home this last time with needles in my ear; they were placed on kidney spots and pain spots, and were worn for three days and then peeled off. They didn't help either. They did, however, give me a headache. We'll see...still have many more sessions...


xo
dani

Sunday, January 20, 2013

From Nutcracker Syndrome to Loin Pain Hematuria Syndrome & Acupuncture

Nutcracker Syndrome:


I was diagnosed with Nutcracker Syndrome or Nutcracker Phenomenon by my nephrologist a month ago after a CT Angiogram showed signs of a compressed renal vein. He sent me up to The Cleveland Clinic to be evaluated by a renowned kidney and urological doctor. During my appointment, the first of three I had up in Cleveland, the specialist explained that Nutcracker Syndrome is a controversial diagnosis and that there is usually a bit of compression of the renal vein in most people who are thin because there is less mass to "pad" the area. It can definitely cause problems in some people, but because I am having zero symptoms on the left side, we don't need to worry about it. No need for surgery or treatment; unless, in the future there are complications, then we can readdress the situation. That is great news.

 The Cleveland Clinic is ultra high-tech! They have GPS clip-on tags that you 
wear so they can find you when they are ready for you to come back to the room. Crazy!


Cystoscopy:


This, however, left me without an answer to my burning (pun!) question: what is causing my right-sided flank pain? We needed to investigate further. We reviewed the CT Angiogram. We reviewed the previous abdominal, pelvic, and spinal MRIs. Next, we had to check my "plumbing" to make sure everything was working properly. Urology Doc performed a cystoscopy with bilateral retrograde pyelogram. I had to be awake for the procedure and it was uncomfortable bordering on painful, but thankfully only took about 15 minutes. During a cystoscopy, the doctor inserts a microscope up the urethra and looks around the bladder to make sure there aren't any abnormalities or swelling, etc., then he sticks the scope into each ureter (the tube that goes from your kidney to your bladder, one per side), and flushes contrast up the reverse way the urine usually flows (hence the word "retrograde"), while watching this flow via x-ray. When all was said and done, we had two answers and one more theory.



Loin Pain Hematuria Syndrome:


First, my bladder, ureters, and kidneys are all functioning properly with no swelling, stones, or other issues. There is nothing that physically should be causing me any pain.

Second; for years I had many urinary tract problems and sometimes would be diagnosed with UTIs or kidney infections, and other times my tests would come back negative though I was experiencing pain, burning when urinating, etc. This led a previous urologist to diagnose me with Interstitial Cystitis, a painful bladder disorder that results from a too-thin mucus lining in the bladder allowing acidic urine to irritate the bladder walls. It made sense at the time, and I was put on a drug called Elmiron to treat the symptoms. The medicine was very expensive and wasn't keeping me from getting what I call "flares," so I stopped taking it about a year ago. Urology Doc said, though, that I definitely do NOT have Interstitial Cystitis, because the walls of my bladder were perfectly peach in color, with the only red being the normal blood vessels that are in the walls. There was absolutely no irritation or discoloration. So...that leaves me back where I was before the diagnosis: I get a lot of Urinary Tract Infections (UTIs) and have a lot of bladder infections and kidney infections. The good thing, I don't have Interstitial Cystitis (for which there is no cure). The bad, I have no idea why I get so many infections or flares, or why sometimes the tests come back negative though I am in a lot of pain.

Lastly, we are left with a probable diagnosis for my flank pain...(finally!)...a very rare disorder called Loin Pain Hematuria Syndrome.

Let me just say that again: I have a probable diagnosis! We finally found out what is wrong with my flank! Oh my gosh! Jumping for joy!!!


Pain Management Doctor Visit:


After the Cystoscopy, I went to a physiatrist (pain management doctor) that works with Urology Doc to discuss all of the treatments I have tried for my flank pain and to make sure we weren't missing anything. For example, do I have a spine disorder that we missed? Have I tried every treatment option available? Is there a different set of pain meds that might manage the pain better? This appointment went well, I suppose. The pain doctor and I went over everything I have done for my flank pain, listed here:

  • Pain Meds (Opiates and Non-Opiates)
  • NSAIDS
  • Muscle Relaxers
  • Trigger Point Injections
  • Epidural Spinal Injections
  • Nerve Blocks
  • Trial Spinal Cord Stimulator (Neurostimulator)
  • Psychiatric Treatment
  • Chiropractic Care
  • Massage Therapy
  • Reiki
  • Essential Oils
  • Heat
  • Ice
  • Lidoderm Patches
  • Tens Unit
  • Ultrasound Therapy
  • Physical Therapy
  • Back Bracing
  • Volteran Gel
  • OTC Topical Rubs and Creams

The pain doctor said he doesn't see anything else that could be causing the pain and that we have tried everything under the sun except acupuncture. He is a big fan of acupuncture and recommended that I try it for pain control before opting for the only other solution I have been offered: renal autotransplantation, which I will discuss a bit later in this post. So, at this doctor's recommendations, and at the encouragement of Urology Doc, I must seek acupuncture before we move forward or take any other steps.

Urology Doc also will be contacting my psychiatrist here in Columbus, making sure that I am on a good treatment plan and that my pain isn't in my head (oh joy!). He must make sure the pain isn't being caused by my depression, that my flank pain isn't psychosomatic in nature. Let's hope my psychiatrist is on my side on this one. I have run the gauntlet before, even been through the psych workup before the Spinal Cord Stimulator trial to make sure I was stable and these symptoms weren't emotional. I do hope he relays that, but he has only been my doctor for a year, not the entire four I have had the flank pain. I will have to wait to see how their discussion goes. I can't handle another doctor saying this pain is "all in my head" like so many doctors told me before I got my Ehlers-Danlos Syndrome diagnosis.


 Location of my right-sided flank pain (old photos):



What exactly is Loin Pain Hematuria Syndrome and what does all of this mean? What are my options?


Loin Pain Hematuria Syndrome (sometimes referred to as Loin-Pain Hematuria Syndrome, Loin Pain-Hematuria Syndrome, or LPHS) is a rare and not-very-understood disease. According to Wiki, it is "the combination of unilateral or bilateral flank pain and microscopic or macroscopic amounts of blood in the urine that is otherwise unexplained. LPHS is considered a diagnosis of exclusion. The syndrome presents with hematuria (blood in the urine) and flank (a region of the lower back beneath the ribs and above the ilium) pain. The condition is very rare and predominately affects females. Worldwide, only several hundred cases have been reported. 

"The cause of LPHS is currently not known. One theory proposes that it is caused by a thin glomerular basement membrane and red blood cell (RBC) renal tubular congestion that leads to swelling of the kidney and distension of the renal fascia resulting in pain. At this time no cure has been found for this disease. LPHS is a debilitating disease due to chronic pain and the inability to know how to control the glomerular aspect. The pain of LPHS can be worsened by acts as simple as riding in the car and undertaking daily activities. Many people with this disease are unable to maintain employment due to the debilitating pain. The treatment of LPHS varies considerably from centre to centre. As the condition is rare and poorly understood, a widely adopted standard of care is not existent."

To summarize, LPHS is rare, not well understood, the cause is unknown, and the diagnosis is based on exclusion -- meaning the doctors rule out every other possibility under the sun until they are left with only one possible diagnosis: Loin Pain Hematuria Syndrome. There isn't a test they can do to give you a definitive "yes;" the docs must work like sleuths until this is the only answer remaining. For me, the combination of everything the doctors have looked for over the years, the many tests I have done, the giant list of unsuccessful treatments I have tried, and the specialist, Urology Doc, going over every other possible scenario (to no avail) and then checking out my organs and urinary system to rule out any other renal or urological cause, has left us with just one diagnosis: LPHS. I have the intractable, idiopathic flank pain and microscopic hematuria, and therefore, that is the probable diagnosis. With only a few hundred people reportedly diagnosed with this, I can add my name to yet another list of oddities! What is up with my body?! I get all the weird diseases!

So, what am I to do?


I am working with the best team of doctors I could ask for. As I mentioned in the last post about this, the Glickman Urological and Kidney Institute at The Cleveland Clinic was just ranked number one (#1) in the country (USA) for kidney and urological diseases. The Cleveland Clinic is also ranked the number four (#4) best hospital in the country overall. The doctors are world-renowned. I couldn't wish to be at a better place or have better doctors to help me in this journey. I am beyond thankful. We are doing a step-process; I am trying acupuncture first and if it doesn't work, I will be deciding whether or not I want to try the only other recommended treatment - the last resort when pain meds aren't relieving the pain: a kidney autotransplantation.

Maybe acupuncture will help; I don't know. I know I believe highly in Chinese and Alternative Medicine. I am doubtful, because nothing has worked thus-far, but I am willing to give it a chance. Considering my options, and that I am required to by Urology Doc, I don't have any other choice. I am trying to do the "zen thing" and go into it without any expectations, good or bad. I have signed up for twice-weekly acupuncture for one month to see if I notice any help, anything better than the Norco and heating pad.

Renal Autotransplantation:


If the acupuncture doesn't work, I will have to decide whether or not to move forward with the renal autotransplantation. Let me explain to you just what that is:

A kidney (renal) autotransplantation is where the doctor does a kidney transplant, but with my own kidney. They would make an incision (I have the choice of one long one down the middle, about a foot long, or one six inch incision on each side of my body, two total.), remove my right kidney, and sew it into the left side of my body by my left kidney. The "hole" where my right kidney was would be filled with the intestines and other organs, things would just "slide" in place over there, and I would have two fully-functioning kidneys on the left side of my body. The idea being, when you remove the [right] kidney from the body, the one with the pain, you sever all nerves attached to the kidney. When the kidney is replaced in the other side, it now has no nerves, so I shouldn't feel any pain from the kidney. An example of how this works outside of these circumstances; a person who undergoes a complete kidney transplant with another person's kidney, feels no pain in that kidney even if the kidney has stones or other issues. It is because there are no nerves attached to the donor kidney when it is implanted. In the same way, my reattached right kidney would have no nerves and it should "fix" the pain issues I am having.


The doctors at The Cleveland Clinic perform this type of surgery often, for various reasons, most of them not related to LPHS. But, when a patient presents with LPHS and they have tried every other treatment with no success, they perform renal autotransplantation in a lot of those cases. The success rate for the body to receive the kidney and accept it, and have the kidney return to a fully functioning kidney, is about 95%, according to Urology Doc. That is a very high success rate. More than likely, if I had this surgery, I still would have two working kidneys. The tricky part in deciding whether or not to try this surgery lies in the other statistics; there is only a 50% chance that the autotransplantation will relieve the pain completely. There is about a 70% chance it will relieve some of the pain. So, I could go through the surgery, and have very minimal pain relief, or no pain relief at all. There isn't any way to tell beforehand how the surgery will affect me, if it will be successful in pain relief or not. The other thing to take into account, there has been some evidence shown in a couple of studies, that the nerves that are causing the pain could regenerate in the transplanted kidney and I could go back to having the same pain I have now, just in a different spot. This is not-well researched, though, and some doctors believe it is impossible, with others saying it is very likely. Urology Doc told me it is unlikely, but can't be ruled out.

The doctor doesn't think that my Ehlers-Danlos Syndrome will pose too many problems. He is aware of the need for extra anesthesia and we also talked about doing stitches and glue (if we do surgery) like when I had my gall bladder removed, that way we ensure proper closure and healing of the incision. He also doesn't think that there would be any problems stitching the kidney in place, but he will be talking to a connective tissue specialist if we decide to go this route.

An autotransplantation surgery requires roughly a seven day stay in the hospital and one month of being totally out of commission at home, then slowly returning to normal daily function over five or so months. It is a long recovery, and most likely even longer for me because I heal very slowly. The surgery would be performed at The Cleveland Clinic by Urology Doc and his team of experts. If the pain returns, there won't be anything else they can do, and it would just be a waiting game to see if the autotransplant was successful or not in terms of pain relief.

There are some very successful statistics in the most comprehensive journal article on "Renal Autotransplantation for the Loin Pain Hematuria Syndrome: Long Term Followup of 26 Cases," published in the Journal of Urology in 1998. Urology Doc says that not much has changed in the way they do these surgeries over the years, and so the journal article is an accurate representation of what to expect.

 "Abstract:
 "Purpose: We review the long-term results of renal autotransplanation as a form of nephron sparing renal denervation for patients with the loin pain-hematuria syndrome.
"Materials and Methods: From 1985 to 1997, after exclusion of other urological, nephrological and psychiatric causes for severe intractable flank pain and recurrent hematuria, 22 patients with severe debility and heavy narcotic dependency underwent 26 renal autotransplantations for pain control [22 patients, 26 kidneys, because some had this done on both sides/both kidneys]. Postoperative pain relief, narcotic use, level of function in daily activities and status of the autograft were assessed.
"Results: Median and followup was 78.5 and 84.7 months (range 30 to 138), respectively. There were 2 technical failures. Pain recurred within 2 years after 6 procedures, of which 3 resulted in transplant nephrectomy and 3 were managed with a reduced analgesic requirement. Of the 16 patients with a minimum of 5 years followup 12 (75%) were pain-free after surgery with 3 additional patients pain-free after transplant nephrectomy. Overall, 18 of the 26 autotransplant procedures (69.2%) resulted in pain relief, in some cases beyond 10 years, with patients returning to normal daily activities.
"Conclusions: Renal Autotransplantation results in durable narcotic-free favorable results in the majority of meticulously screened loin pain-hematuria syndrome patients. Although some failures, which usually occur within 2 years after surgery, can be expected, autotransplantation is justified as a nephron sparing denervation therapy for select loin pain-hematuria syndrome patients [meaning it is a favorable kidney saving, nerve severing option]. 
"[Mostly] Full Article:
"The loin pain-hematuria syndrome is a rare clinical entity, first described by Little et al, comprising intractable flank and loin pain with microscopic gross hematuria. The etiology is still poorly understood, and the diagnosis is made by exclusion of urological and nephrological conditions. Recent therapeutic efforts have been concentrated primarily on pain management, ranging from percutaneous regional nerve blocks to surgical sympathectomy to open surgical procedures. The latter include renal capsulotomy, vascular pedicle denervation, ureterolysis, nephrectomy and renal autotransplantation. First described by Aber and Higgins in 1982, renal autotransplantation as a nephron sparing procedure has since been reported as having favorable outcomes in the loin pain-hematuria syndrome in small series by Sheil et al in 1985 and 1987, and Chin in 1992. Other reports on successful cases have appeared in the literature. An important reason for preferring autotransplantation to nerve nephrectomy for pain control is that 30% of patients have bilateral, usually metachronous involvement. Although referred to as providing long-lasting effective pain relief, followup of these renal autografts, except for 1 case, has been relatively short at less than 4 years. Concern that pain relief may only be transient following renal autotransplantation has been raised by some groups, with reports of recurrent graft pain within 3 to 4 years. To address the issues of durability of pain relief with autotransplantation and to define further its role in the loin pain-hematuria syndrome, we report the results of 26 renal autotransplantation in 22 patients with a median followup of 78.5 months (range 30 to 138). We also include an update on the 10 patients reported on in 1992, at which time 8 of 9 with minimum followup of 1 year were pain-free.
"From 1985 to 1997 inclusive 64 patients were evaluated as tertiary an in some cases quarternary referrals to 1 of us (J. L. C.) from other urologists and nephrologists with the possible diagnosis of the loin pain-hematuria syndrome. All patients had severe loin pain and 54 had persistent microhematuria including 20% with periodic gross hematuria. Clot colic or severe bleeding episodes did not correlate with the timing or severity of flank pain. Ten patients had no or insignificant microhematuria with intractable flank pain as the only complaint.
"Patients underwent the standard urological evaluation for hematuria and loin pain outlined previously elsewhere. The diagnosis of the loin pain-hematuria syndrome was made only after exclusion of all other urological and nephrological causes for the reported symptoms. A formal psychiatric evaluation was performed when surgical intervention was contemplated, and narcotic dependency and pain management problems were anticipated. In addition, 6 patients had sympathetic nerve blocks as part of the assessment. Of these patients 3 had transient pain relief and subsequently underwent autotransplantation. We did not perform this procedure routinely as a prerequisite criterion to surgery. Surgical candidacy was based on satisfying the 4 criteria of absence of urological and nephrological causes for the symptoms, formal psychiatric evaluation, excluding underlying major psychoses, significant narcotic dependency and significant debility from pain causing disruption of employment and daily activities. 
"The majority of cases deemed unsuitable for autotransplantation were rejected because the degree of pain or disruption of daily activities was judged not severe enough to warrant surgery. Five patients had definite underlying conditions identified during the psychiatric assessment. The patients who did not satisfy all criteria were referred to a pain management specialist or psychiatrist for treatment, whichever was deemed more appropriate. 
"Patients who presented with contralateral flank pain subsequent to the first autotransplantation were reassessed using the same standard investigations and criteria listed previously. The decision to proceed with a second autotransplantation was made independent of the outcome of the results on the first side, although the patients were advised of the increased risk because of the possibility of technical failure leading to dialysis dependency. The same strict criteria had to be satisfied before the second autotransplantation was performed.
"A total of 28 autotransplantations were performed in 15 women and 9 men 21 to 55 years old (median age 36.2) with the loin pain-hematuria syndrome. Two patients with followup of less than 30 months were excluded from the analysis, and 4 underwent sequential bilateral procedures. The standard living related type donor nephrectomy via a flank incision with complete division of the ureter was performed in all patients. An unusual finding encountered in a small number of patients at nephrectomy was an excessive amount of perivascular adventitial nervous and fibrous tissue. A standard Gibson renal transplant incision was used. The usual vascular anastomoses to the iliac vessels were performed (end-to-side anastomosis to the external iliac vein and artery, except for 4 cases of end-to-end anastomosis to the internal iliac artery). The ureter was reimplanted using an extravesical approach in 22 cases and transvesical approach in 4. Postoperative pain control was managed by the pain management team with patient-controlled analgesia using intravenous narcotics, switching to oral analgesia and tapering off the dosage as soon as possible. The psychiatrist and pain management team who had assessed the patients preoperatively were involved in the followup, providing the necessary support.
"Renal autograft function was assessed in the early postoperative period by a nuclear renogram in the first 12 cases, while the latter 14 were assessed with color Doppler ultrasound. Analgesic requirement was recorded before and after surgery, along with the level of daily living function. Updated data on the earlier reported patients were obtained by direct patient telephone interview or through local physicians. The procedure was judged successful by improvement in pain control leading to freedom from chronic narcotic usage, return to gainful employment, normal daily living activities and satisfactory autograft function.
"Results:
"Followup of the 26 cases ranged from 30 to 138 months (median 78.5, mean 84.7). The current status of the autograft and pain control is listed in the table 1. Since patients who underwent bilateral procedures may have discordant results on the 2 sides, the denominator for the success rate was the number of individual autotransplant procedures rather than the number of patients. There was 1 immediate failure in patient 11 with renal vein thrombosis resulting in transient nephrectomy on the postoperative day 2. Left graft ischemia was demonstrated in patient 6 on postoperative Doppler ultrasound, and persistent pain necessitated transplant nephrectomy 2 months later. In 6 other cases pain recurred at the graft site, usually within 1 year. Of these 6 patients 3 have subsequently undergone  transplant nephrectomy and have remained pain-free. Three patients still have the graft in situ, 2 periodically need narcotics and 1 uses nonnarcotic analgesia. The remaining 18 of 26 autotransplantations (69.2%) can be classified as long-term successes, although 3 cases with recurrent pain, reduced analgesic requirements and less severe disruption of daily activities could be classified as partial successes.
"Patients 1, 2, 4 and 6 underwent sequential autotransplantations. Patients 2 and 6 had successful procedures on both sides, although success or failure on 1 side did not necessarily predict the outcome on the contralateral side. Three other patients had undergone prior nephrectomy on the contralateral side elsewhere for pain control and, thus, autotransplantation was performed on the solitary kidney. Histopathological examination of the nephrectomy specimens revealed the expected ischemic changes in patient 11 with postoperative renal vein thrombosis and patient 6 with graft ischemia on the left side. However, the remaining kidneys did not show any consistent histopathological condition. Of 5 kidneys 1 stained positive for the third component of complement. 
"Discussion:
"Since the etiology of the loin pain-hematuria syndrome is poorly understood, numerous medical and surgical treatment modalities have been tried with little success and, thus, most therapeutic efforts are now directed toward pain control. A sympathetic nerve block has been used by some as a preoperative procedure to differentiate visceral pain from nonorganic pain, although with our limited experience we had not found that essential in our management decision process. Attempts at renal denervation have met with transient success, although Andrews et al recently reported cures in approximately 25% of patients with chronic renal pain (not necessarily the loin pain-hematuria syndrome) via surgical sympathectomy. However, a significant proportion of the patients underwent multiple procedures. 
"As a natural extension of the principle of renal denervation, renal autotransplantation has been used increasingly in serving renal function of the involved kidney. The initial reports were encouraging, and early results from our center revealed an approximately 90% success rate. These favorable figures were corroborated by Weisberg et al in their informal survey. However, concern regarding the transient nature of the pain relief derived from renal autotransplanation has been raised by some. As many as 75% of theses patients in these series have had recurrent pain and, thus, doubt has been cast on the long-term benefits of renal autotransplantation for the loin pain-hematuria syndrome. Renal reinervation has been suggested as the mechanism of recurrent pain in the autograft area. Canine studies have documented regeneration of autonomic nerves following renal autotransplantation. Similar findings have been observed clinically by Gazdar and Dammin, who described neuronal regeneration in human isografts and allografts. Since this reinervation process has been noted to extend to the interlobular arteries in some cases within 8 months of transplantation, recurrent pain in the autograft can be expected in a certain proportion of patients despite complete denervation at the time of surgery. The question of necessity to divide the ureter has been raised. We believe that leaving the ureter in tact would, at least theoretically, risk incomplete denervation or encourage reinervation of the autonomic nerves and, this, may jeopardize the success of the procedure. Therefore, our bias is that the division of the ureter may minimize the surgical failure rate.
"Since  the previous report of 1 failure in 10 patients, there has been 1 subsequent failure from that initial cohort. Patient 2 with failure on 1 side has subsequently undergone successful autotransplantation on the contralateral side with followup of 68 months for that autograft. On the contrary, patient 6 who had a successful outcome on 1 side subsequently had failure on the contralateral side, although graft ischemia demonstrated on Doppler ultrasound was likely the cause. Thus, having expanded the original series and extended the followup to more than 10 years in several cases, the current series supports the notion that renal autotransplantation when performed in meticulously selected individuals provides long-lasting pain relief in the majority, although the long-term success rate is closer to 70 than 90% as previously reported. If the mechanism of failure is due to renal reinervation via the iliac vessels, it does not affect all patients uniformly. Pain recurrence appears mostly within the first 2 years after autotransplantation.
"There has been considerable evidence that a significant underlying psychopathological condition exists in these patients as summarized by Kelly. There have been proposals to include psychiatric assessment in the treatment algorithm before surgery. All of our patients had undergone formal psychiatric assessment to rule out obvious psychoses. Despite meticulous patient selection with strict adherence to the defined surgical criteria, there was still a 30% failure rate. Attempts have been made to delineate predictive psychosocial factors for outcomes with autotransplantation. However, it would be difficult to use undiagnosed psychopathology as the explanation for discordant results on the 2 sides in some patients with bilateral autotransplantations. The discordant surgical results of the bilateral cases mend lend support to the theory of neuronal regeneration being the mechanism of failure. 
"CONCLUSION:
 "The loin pain-hematuria syndrome is a complex clinical entity which clinicians have attempted to treat, rather simplistically, with renal autotransplantation. Failures tend to occur within the first 2 years. The mechanism(s) of failure may be equally complex, with psychosocial issues and sporadic renal reinervation being operational. However, in the absence of other viable alternatives, autotransplantation does provide sustained relief and return to normal daily activities in some cases beyond 10 years, in approximately 70% of carefully screened patients."

Okay, now that you got through that long article, I will quickly summarize: There is a 70% chance that autotransplantation will relieve pain. If it doesn't work, it could be due to psychiatric issues or the nerves regenerating on the kidney making it possible to feel the pain again.

There is another article comparing Renal Autotransplantation to Radical Renal Neurectomy and the conclusion is that autotransplantation is much more successful in relieving pain. Also, there were more cases of probable nerve regeneration when the neurectomy was done. A neurectomy is where they open you up and instead of taking out the kidney, which in doing so severs all of the nerves, the doctor just goes around the kidney and cuts all the nerves they can find while leaving the kidney in place. It is a much less invasive procedure, but the problem, said Urology Doc, is that there are many nerves which are too tiny to see and it would be impossible to cut them all without removing the kidney entirely. If they leave any nerves there will still be pain. So, both this article and Urology Doc agree that autotransplantation is much more effective than neurectomy.

I found a nice article by Dr. Marc F. Brazie, Division of Nephrology at University of Maryland Medical Center, Baltimore, that does an interview about LPHS, if you are interested.

Acupuncture:


For now, I am going to acupuncture and will see what the results are. If it relieves the pain enough, I won't need to worry about this surgery. If, however, it does not relieve the pain, I am seriously considering the renal autotransplantation. Urology Doc and I have decided that I will revisit him after this course of acupuncture to reassess.



My first appointment with the acupuncturist at Urban Acupuncture Center was last week. We had a brief evaluation and discussed our goals. We are going to treat the pain in my flank and also, from a Chinese Medicine viewpoint, my liver has a lot of problems, so we will help that as well. The place I am going to has a very unique setting, apparently very common in China, but just picking up some steam here in the states. It is one large room, a community setting, with 12 recliners. All patients are together in the same room having their needles in and relaxing at the same time. They base their fees on a sliding scale, and you decide what you can pay, $15-$40 per visit (plus $10 for the initial consult). Their goal is to provide affordable acupuncture to the community. Those of you who live in Oregon - that is where this got started here in the U.S. There are about 100 of these open space clinics in the states, so you can always look up acupuncture in your area and see if you have one.


According the my acupuncturist, I should see results soon. She said usually people feel some relief after the first visit, although, for me, it just caused a major flank flare-up. I will keep you abreast of the progress in future posts. We have me set up for one month of visits, twice a week.



Alright, I am exhausted and sore from typing, so I will catch up with you all another day. Much love and peace to you. For those of you with flank pain, I recommend talking to your doctor about LPHS. Happy International Day of Acceptance for those of us with disabilities!

xo
dani
 

Wednesday, January 16, 2013

I've Found the Golden Elixir of Life: Saline!

It's been a month of trying IV Saline for POTS, and my doctor and I have reached the decision that the saline infusions are working well and are helping me to feel better and keep my heart rate under control, and that we will pursue a port (portacath) for long-term use. Finally, a treatment that is a success! Saline: you are my fountain of youth, my tonic of goodness, my golden elixir!


My blood pressure is pretty stable with the meds that I am currently on for POTS: propranolol (beta blocker), midodrine, and florinef. The fluids don't seem to reflect much with regard to my blood pressure, as far as numbers go. My heart rate, however, is a different story. I can't keep it under control on a normal day. There are days when it stays in the 70s while doing minimal activity, and that is great, but there are so many other days when no matter what I try, I can't get it below 120. Laying flat on my back and having a 130 pulse makes me exhausted - I feel like I am running a nonstop marathon. The IV saline seems to help this. The idea being, we are tricking my body into thinking there is greater blood volume, so my heart doesn't pump like crazy. After running fluids for about a half an hour, I can see a drastic reduction in my heart rate. What I feel is a bit more energy, like I can breathe easier, I am not as dizzy, sometimes it helps with nausea, and I even notice it helps my GI system. I go from feeling out of breath and sluggish to feeling a bit more energized.

I'd like to reiterate that I do drink a lot of water. I average 64 ounces of water per day, I also have a cup or two of coffee, and sometimes I drink coconut water or juice (rarely), or Pedialyte when needed and I can afford it (stuff is expensive!). I would not account this drastic change to something I could do by just drinking more water. I drink A LOT of water. It never makes me feel this good, nor does it lower my heart rate like the IV Saline.

Right now, we have upped my visits to 3x a week. I have a nurse come out and hook up my IV Saline three days each week. I notice that the earlier I have it done, the better. If I have the Saline hydration in the morning, it helps me to feel better throughout the day; if I do it in the evening, it is almost wasted, as I end up sleeping not long after and the effects don't carry over to the following day. Some days, the nurses can't make it early, so I will gladly take the hydration at night; any is better than none. Once I have my port in place, I will be able to run my own fluids in the morning.

I tend to run about 250ml per hour, so it takes me about 4 hours to run a one liter bag. If I need to be somewhere, I can run it on "open" and be done in a little over 2 hours, or if I want to be lazy and lay around, sometimes I will run it at 200ml per hour, so it takes me 5 hours. It all depends on how I am feeling, how the IV feels (sometimes running it at open or even 250ml is uncomfortable, depending on IV location; it hurts more in my hand than my arm), how I am tolerating the fluids/pressure, and what my schedule is. I have so many doctors' appointments that I have to adjust my infusion schedule accordingly.

My doctor is in search of a general surgeon to install the port. Amazing PCP tends to take a while to do things, so I may have a bit of waiting on my hands, but he does come through. He is teaching me patience! Ha!

Just to get an idea, here are some numbers from random days I got fluids:

Day "A" - Nighttime Run of Fluids

Time:        Heart rate:          Activity:

9:37a         138                    Just got up - feeling very POTSy today, HR stayed this way all day
4:35p         93                      At rest - heart rate is too high, keeps jumping between 90 and 140
6:39p         89                      At rest - heart rate is still too high
7:18p         93                      Starting fluids - let's see if we can get the heart rate to drop!
7:36p         95                      Running fluids
7:52p         86                      Running fluids - heart rate starting to drop
8:09p         83                      Running fluids
8:56p         80                      Running fluids
9:18p         82                      Running fluids
9:52p         76                      Running fluids - still dropping
10:23p       78                      Running fluids - I am feeling much better at this point
10:32p       73                      Fluids done - So, I dropped 20 points. If I could have run these
                                            earlier in the day, I could have had my heart rate reduced and
                                            felt better all day.

Day "B" - Early Morning Run of Fluids

Time:        Heart rate:         Activity:

6:19a         102                    Just got up - resting heart rate of 102, too high
6:46a         78                      Been running fluids for about 20 mins, heart rate drastically dropped
7:04a         83                      Running fluids
7:50a         76                      Running fluids - big drop
8:13a         69                      Running fluids - keeps dropping!
8:39a         67                      Running fluids - feeling great
8:51a         68                      Fluids done - Here's the amazing part: After this run, I left and went
                                            to the doctor's. Afterward, I stopped and got groceries, then
                                            came home and had enough energy to put them away.
                                            Whoa! That is HUGE for me - I never have enough energy
                                            to do multiple things in a day, nor am I able to sort through
                                            my groceries when I get home. This was profound.
                                            I continued to feel good until the late afternoon, then my
                                            heart rate went back to its normal high. So, the effects lasted
                                            many, many hours. 


Hope this helps some of you - maybe you will decide to give IV Saline a try. It never hurts to talk to your doctor. I am so thrilled I decided to push for this, and I couldn't be happier with the results! Don't forget to read my guide on talking to your doctor about IV fluids.

Love and gentle hugs!
xo
dani