Jaw Surgery Was the Biggest Mistake of My Life - Philadelphia, PA

I had double jaw surgery and genioplasty just over...

I had double jaw surgery and genioplasty just over 4 weeks ago and the results have been absolutely devastating. I had a SARPE (palatal expansion) two years ago that went fine so my surgeon assured my the risks with this surgery would also be low.

My surgery ended up taking 12 hours, I needed more expansion than originally anticipated and so my palate accidentally tore during surgery. They had to take tissue from where they could to graft the tear but it wasn't enough so now I have a cadaver graft. I can feel pieces of exposed bone under the graft and yesterday a small piece of bone came dislodged and fell out. I can't rinse my mouth without water coming out of my nose.

They weren't able to get adequate blood flow to the upper gums after surgery and the tissue turned purple. Some of the tissue died and my gums receded so far up that bone is exposed. They're still not sure whether I'll lose the teeth or not. Right now I have to do hyperbaric oxygen treatment 6 days a week and at best I'll need a gum graft and at worst I'll need implants.

I got oral thrush from the antibiotics and I was unable to swallow. I spent 8 days in the hospital and lost about 20 pounds. At my lowest I weighed in the 90s.

If I could take it all back I would. I wish more than anything that I had never done this but now I'm stuck dealing with the many complications.

Post op swelling

This is the swelling about 3 days post op.

One Month Post Op

It's been just over a month and I still have complete numbness in the bottom of my nose, my lower lip and my chin. I have partial numbness in my upper lip. The only pain I have is in my right jaw joint which is severe and has been present since surgery. I also have pain in my right ear. I see the surgeon on Tuesday and I'm hoping he'll remove the splint and some of the stitches.

More bad news

I had my splint taken off today but the surgeon said there's way too much damage to save the teeth. I'll lose all 6 of the front ones and they'll have to take bone from my hip or rib to do bone grafts. The hole in the palate didn't close under the graft so they'll make a prosthetic device to cover that until I can have surgery again. Not the news I was hoping for.

Graft pictures (kind of gross)

During and after failed palate graft

Alar Cinch

Now that I've started to get more feeling back in my upper lip I've noticed that it feels very tight, like something is pulling on it. I can't pucker my lips or smile fully. I mentioned this to my surgeon and he said he performed an Alar cinch to keep my nose from broadening during surgery. I'm going to ask him to remove the suture on Tuesday, hopefully it's a simple process.

Anterior Maxillectomy

On April 10th I'll undergo surgery to remove my 6 front teeth, my anterior maxilla and part of my hard palate. I'll be given an immediate prosthetic to replace the missing bone and teeth and then eventually a more customized prosthetic. In a few months I'll undergo reconstructive surgery by a head and neck cancer specialist to attempt to reconstruct my face and jaw using bone from my hip or shoulder to make me appear as normal as possible. Eventually when the reconstruction is finished they can begin prepping for the implants. This is definitely not the path I expected but, for whatever reason, it was the path I was given and I fully intend to come out of this a stronger person.

Medical Records and Second Opinions

Well the tissue in the anterior segment of my maxilla and palate became necrotic and literally fell off zombie style. Now I only have bone in that segment of my mouth so my maxillectomy has been moved up to Monday the 7th. I tried to get my medical records from the surgeon's office and they gave me a very difficult time. It took over a week and they left out the operating report and the post op X-rays. I had to ask 6 times before I was finally given all the reports. The surgeon assured me that he wasn't hiding anything but I'm finding it difficult to trust him.

I went for a second opinion at Cooper Hospital yesterday and I really liked the surgeon there. He said he never would have performed a 4 piece Leforte, especially on someone like me. He seems confident that he can eventually reconstruct me though. The whole process will take 1-2 years and involve bone and tissue grafting, bone morphogenic protein and implants but at least there's hope!

To sue or not to sue?

When I first realized that the surgery didn't go as planned I knew that there was potential for a lawsuit. The surgeon should have taken more precautions considering my medical history and hyperbaric oxygen should have been started immediately. I didn't initially want to sue though and I expressed this to the doctor. I saw what's happened to me as an opportunity to establish a standard of care that's been missing in the Ehlers-Danlos Syndrome community. I told him I wanted something positive to come from all of this and I wanted him to help spread awareness. He agreed to do a case report and have it published in a medical journal. I hoped that my case would make a difference for other patients with my condition.

In reality the surgeon learned nothing. He had his resident do all the research and write the report. In fact, just the other day he asked me how to spell Ehlers-Danlos. I was appalled. I had given this man an opportunity to not only avoid a law suit but also to make a difference and he was too arrogant to accept it. Instead of using this to become the type of doctor that EDS patients can go to, one that fully understands the risks and therefore the precautions that need to be taken, he decided not to treat anymore EDS patients.

He told me he has no control over whether or not I sue him, and this couldn't be further from the truth. He has all the control. If he had taken some initiative to be an advocate instead of covering his ass, we could both make a difference instead of battling it out in a courtroom.

Speak Like a Human

So I've been really anxious about my surgery tomorrow and I wanted to try to shift my focus in a more positive direction. Yesterday I went to see La Boheme with my aunt and then had an early bday celebration with my family (I'll be in the hospital on my bday). Then today my cousin had a party for his twin girls who just turned 2. It was great to see everyone and just feel sort of normal again.

When I was getting ready for the party today I had some sudden pain and it felt like some of the bone shifted and pushed up into the fistula in my palate. Some of the bone also fell off. I didn't want to call the surgeon since I'll see him tomorrow but the pain got worse so I got a little panicky and finally called.

I got a call back from a first year resident and I tried to explain to him that my situation was complex and I needed to speak to the surgeon. He refused to page him and then told me to either "speak like a human being" or he would hang up on me. Then he hung up on me. I was shocked. Mocking a patient who can't speak clearly after a procedure is unprofessional and incompassionate to say the least. I called back and eventually was able to speak to my surgeon but I can't believe how I was treated by the resident.

I don't think the surgeon understands how traumatizing this whole experience is. I'm doing my best to stay strong but there's so much uncertainty. I need to get myself back into that positive mindset before tomorrow.

Post Op Update

So it's been about a week since I had the maxillectomy and I wish I could say I'm in great spirits but honestly I'm just incredibly angry. Wednesday was my 29th birthday and I thought about what a difficult year I have ahead. The surgery itself went pretty well. They removed all 6 teeth and layers of bone from the maxilla and hard palate. They were able to save some which should make reconstructing easier.

Right now I have a temporary obturator (prosthetic) its bulky and rigid and a different color than my natural gums. It's off center so it looks and feels ridiculous. They put platelet rich plasma underneath it to help stimulate new tissue growth. I'm still in a lot of pain and it's uncertain how things will end up.

The care I received was terrible. I was supposed to start hyperbaric oxygen the day after surgery but between transport and the nursing staff I got there late and missed a half hour of very important treatment time. The nurses kept forgetting to give me my medications and one of them refused to acknowledge when the IV became infiltrated and continued to push phenergan through the bad IV even though I was screaming. The surgeon went away on vacation so I won't follow up with him for a little over a week.

I wish I could be more positive but I don't really have it in me right now. Sorry guys :(

Infiltrated IV

I don't know why the other picture didn't post but this is after my IV infiltrated and the nurse kept pushing phenergan through it. The vein is still hard and painful.

A Little Positivity

There's no doubt that my spirits have been down since my last surgery a little over a week ago. I've been feeling frustrated and angry and it's hard to look in the mirror without crying. I gave myself some time to grieve the situation but I can't stay down forever. In two weeks I'll be cleared to return to work and I'll have to start facing the world with my prosthetic. So, in an effort to focus on the positive things I've compiled a list of things that don't completely suck:

1. When this is over my implants will be whiter and better shaped than my natural teeth.

2. I've started getting pins and needles in my lower lip which is an excellent sign that I may eventually regain FULL feeling.

3. I watched the entire series of Breaking Bad and still got paid.

4. Should there ever be a zombie apocalypse and I become a member of the undead, I'll still have 6 perfectly white front teeth and pink acrylic gums. Plus my face is so plated and screwed together that it couldn't fall apart.

5. Again in the event of a zombie apocalypse, I'll be fully prepared for what necrosis is like AND I've technically already had cadaver skin in my mouth.

6. I'll be able to bite into anything hot or cold without sensitivity.

7. Six less teeth to get cavities in.

8. I don't have to lose any weight before summer now.

9. I've met some really amazing people at hyperbaric medicine.

10. Because I work in healthcare, this experience only reinforces my compassion for my patients. It reminds me that no matter how busy my workday is, every single patient deserves to be treated with dignity and respect.

Obturator

Some of you have asked me to post pics from after the maxillectomy so here goes. This obturator is temporary but it's wired in so I can't remove it.

Two Week Post Op

It's been a little over two weeks since I had my last surgery. I had an appointment with the surgeon and the prosthodontist yesterday. First the surgeon took out the obturator. It was wired in and wire was threaded through my back teeth so the process wasn't fun. I think the sound of the wire cutters was probably worse than the pain though. It took a little while but he finally got it out and was able to see how everything is healing. The good news is that the area is healing pretty well and the exposed bone is about 75% covered with tissue. If it continues to heal then I won't need another debridement surgery and we can move forward with reconstruction sooner but it's still too early to tell.

This was the first time I've had the obturator out since my teeth were removed. I had no idea what the area was going to look like but I knew that emotionally, this was probably going to be the hardest part of the whole process. When he first took it out, it felt really weird/awful, like someone just removed a piece of my face. I couldn't figure out where to put my tongue and all the open space really freaked me out. The surgeon wanted me to rinse but my mouth couldn't figure out how to swish so he ended up cleaning the area with a Q-tip and irrigating it with saline. I still hadn't seen my face yet though.

The prosthodontist came in to take some molds for my new obturator. I really like her. She's so nice and she has such a calming nature about her. I feel like she gets how hard this is in a way that my surgeon doesn't seem to. Anyway, she took a bunch of molds and she also shaved down my current obturator a little so that it's not so bulky in the front. The new one should be ready in about 3-4 weeks. Then she had to teach me how to take the current one in and out since it's no longer wired in. She held up the mirror and told me to apply pressure to the metal clasp part in the back, I did and the obturator popped off. I couldn't look at myself. It was by far the hardest thing I've ever had to do. I put the obturator back in. I started crying and she gave me a few minutes to let everything sink in. She asked me if I was ready to try again, I wasn't but I knew this was something I had to do so I said yes. We repeated the process and I was happy to have the obturator back in. I didn't feel like a whole person without it.

The prosthodontist explained that she wanted me to take the obturator out whenever I'm home and at night. My heart sank but I just nodded. She said that the tissue wouldn't be able to heal with the pressure from the obturator constantly against it. I nodded again but I was horrified at the idea of having to face myself in the mirror every night without the prosthetic. She told me to follow up in a week and then in one of the first shows of sincere compassion I've seen so far, the surgeon put his hand on my shoulder and acknowledged how hard this was. He's said it before but always in a stock response kind of way and never in a way that made me feel like he's actually tried to put himself in my situation emotionally. It made me resent him a little less.

I got home and purposely went to bed late because I was delaying taking out the prosthetic. I finally got brave, went into the bathroom, brushed my teeth and then popped it out. I didn't look at the area. I'm hoping that the shock will wear off and it'll get easier in a few weeks. I keep reminding myself that this is temporary and one day I'll have implants but without knowing for sure how well they'll be able to reconstruct it's hard to rely on that. One day at a time though and if I can get through yesterday, I can get through anything.

Car Accident

Well I was on my way to meet with my lawyer today and as I was driving there my prosthetic came loose and popped off. As I was trying to get it out, traffic slowed down and I hit the car in front of me.

No one was hurt but my car isn't drivable. I had to explain to three police officers what happened, which was humiliating.

Luckily the guy I hit was young and super nice but the whole thing is so much added stress.

No Secrets on the Internet

I made this blog for two reasons. The first is that I wanted to share my story with others so that they can fully understand the risks of jaw surgery. It's a major procedure and I want people to be aware that serious complications are rare but possible. The second is that the process has been incredibly difficult emotionally and writing about my experience is cathartic. It's a chance to vent my feelings in a supportive environment.

I never publicly posted the name of my surgeon because this isn't about him. While I definitely get frustrated with him at times, I'm not trying to bash him either. I had a woman private message me and ask me who did my surgery. She was nervous because she was having surgery at the same hospital as me and wanted my opinion. I told her who did mine and I also told her what I would do differently if I could go back in time and make more informed decisions.

My surgeon informed me that he received a call from this woman regarding my blog. Even though this is a public forum, the information I exchanged with her was done privately and there should still be some level of confidentiality. This blog is no secret, there's nothing on here that I haven't already or wouldn't say to my surgeon. If he asked me for a link to my page I would gladly give it to him. However, I feel like calling him without asking whether or not I'm ok with it first is wrong. I'm still receiving treatment from him, he's still responsible for my care.

I'm more than happy to share my experience, offer my opinion or provide encouragement but please don't take advantage of that.

Being Your Own Advocate

When I had my double jaw surgery I noticed, after a few days in the hospital, that groups of residents as well as my surgeon would come in my room throughout the day to look at my upper gums. I was still in too much pain and too swollen to really look at them myself so I didn't think much about what was happening (I was also full of painkillers.) My surgeon wanted to keep me a little longer and my 2 night stay turned into 8. Around maybe the third day the chief resident mentioned hyperbaric oxygen to my sister but nothing ever came of it. Then around day 5 or 6 the surgeon came in, looked at my gums and said "Oh thank god." He told me that they finally started to turn pink and that he hadn't been sleeping at night worrying about the purple color. It wasn't until that point that I really even realized there was a major issue with my gums, no one had ever said it directly to me. I was on so much medication and I was still so weak that I didn't even fully comprehend what it would have meant if the gums had stayed purple. I was discharged on day 8 and told to follow up in a week.

When I got home I looked at my upper gums and I remember thinking they looked more grey than pink and I started to worry about what that would mean. I also remember the smell everytime I lifted my upper lip. By my one week follow up I thought the tissue looked white (in reality I was looking at bone and had no idea.) I remember the surgeon and the chief resident lifting my lip and saying the gums had receded. I asked what that meant and it was only then that I was told there was a possibility I would need gum grafts or that I could lose the teeth. The surgeon called me at home the next day and mentioned how it was a shame we couldn't do hyperbaric oxygen. He had only ever encountered this situation once before and hyperbaric was able to save the tissue. I asked why it was never done and he told me it was contraindicated because I have glaucoma.

As soon as we hung up the phone, I googled hyperbaric oxygen and glaucoma. I read about studies where it actually helped to lower eye pressure in glaucoma patients. I called the surgeon right back and told him what I found. He wasn't convinced so I called my retina doctor who said that it was fine for me to have the hyperbaric treatment. I had my retina doctor call my surgeon and two days later I started hyperbaric treatment. It was too late though. The tissue was dead, we had waited too long. There were some benefits to the treatment though which I'll write about in my next post.

Much later when I found out I would need the maxillectomy, I found out the surgeon was planning to use another cadaver graft. I had already had one failed graft and I knew with my medical issues, another graft would probably fail as well. I did my research and I insisted on him using platelet rich plasma instead. I felt like my body would respond better to my own growth factor than it would to a donor graft. It's a decision I'm glad I made and I stand by it. I'm healing well now instead of going through the ordeal of another failed graft.

My point is that it is so important to be your own advicate. It's ok to question your doctors. They don't know everything. They're just people and they're not infallible. It's your body and you have every right to do your own research and make your own decisions.

Typos

All of the typos in my blog are driving me nuts! Unfortunately that's a side effect of 2am writing sessions and there's no way to go back and edit. I promise I know how to spell advocate though :)

3 Week Post Op

Today was my three week post op. I saw the surgeon and he said there was a little more healing since last week. He clipped off a few pieces of bone that were sticking out which sounds worse than it really was. I couldn't feel anything when he did it and he seemed really aware of how much the noise freaked me out so he waited until I was ready.

I told him I was concerned about still not having feeling back in my lower lip and chin and about not having full control of my muscles yet. He thinks it's just going to take longer because of my EDS but I'm nervous that it'll be permanent. It's hard when you can't compare yourself to the normal recovery timeline and unfortunately there's no EDS timeline to tell me what to expect.

I've also had a lot of pain and clicking in my right jaw joint since surgery. It's a little better now than it was the first month but it's definitely bothersome. The surgeon said it might be something I have to tolerate for now but that if it continues then eventually they can treat it in a non surgical way (thank god). I can handle it as long as I know there will eventually be an end to it.

I saw the prosthodontist and she had a wax mold of my new obturator for me to try. I was really impressed. This one looks way more natural. It's like the difference between wearing Steve Madden heels and then suddenly putting on your first pair of Louboutins (only way more expensive and much less fun). She said she would put a rush on the obturator so I'll be able to have it in a week which is awesome.

I've gotten used to taking the prosthetic out at night and when I'm home which is something I never thought I would be able to say. It wasn't easy but I knew eventually I would have to face it. Once I really took a look at the area and let myself understand the damage and accept the loss it became less scary. At first it was hard because I would look in the mirror and feel like I was gone and there was this horrible freak that had replaced me. Somehow I've managed to dig a little deeper and remember that I'm still there though and that person in the mirror is only temporary. I remind myself that I may not have a lot of control over my situation but if taking the obturator out will help me heal then I need to do my part, no matter how hard.

I used to have these horrible nightmares about losing all my teeth. I had them on and off for years and I would always wake up in a panick and then feel so relieved when I would realize it was just a dream. This has literally been my nightmare come true but there's something empowering about knowing that I've faced it head on and it didn't break me.

New Obturator

Today was my appointment to get my new obturator. I don't know if I would say I was excited but I was definitely hopeful that this would be an improvement over the old one. I tried the wax mold last week and I was pretty happy with it, so I had good expectations for today's visit.

The prosthodontist must have taken it in and out at least a dozen times to make adjustments and to make sure it fit just right. It felt lighter and less bulky than the old one but it'll take a little while to really get used to. The teeth look more natural and are more centered. I feel like it changed the whole shape of my mouth though. My upper lip all the way up to my nose looks like it sinks in. I hate it. I thought this new prosthetic would make me feel more like myself but I've never felt less like myself.

The doctor assured me that everyone feels that way when they first get a new prosthetic and that it takes a few weeks to get used to. She's so compassionate and it helps make the process a little easier. I definitely feel like today was a huge step back though. I regret this surgery so much. I just want my old face back. My lips were always my best physical feature and now they look thin and droopy. I feel like this surgery ruined my face and even with reconstruction I don't think I'll ever fully look like myself again.

EDS Awareness Month

So much has (and hasn’t) happened in the past few weeks that I’ll try to update without being all over the place. My last post I was upset about my new prosthetic. I seriously hated it. Speaking was so much more difficult and eating became nearly impossible. Any progress I made with drinking out of a cup went totally out the window and I felt like I had taken a huge step backward both with function and with aesthetics. Since then I’ve had the prosthetic adjusted again and I’m so much happier. My speech has improved dramatically, I’m drinking out of a cup like a boss and I’m more comfortable with the way it looks. All it took was for the doctor to add some additional lining so there wasn’t so much empty space.

I haven’t had any positive changes in the muscle tightness in my lower jaw and chin though. I was in tears at my last appointment hoping that my surgeon could explain why this was happening but he didn’t have any answers. It’s gotten to the point where it’s almost unbearable. The whole right side and part of the left side of my chin feels like it’s contracted. I can’t chew at all and I’ve lost even more weight (I'm now under 100). Whenever I clench my jaw I get a sensation that all of my teeth are loose (they’re not) and that the left side of my lip is pulling down. Touching my teeth with my tongue causes discomfort all the way down my jaw and drinking anything cold is totally out of the question. It has me so stressed out and I can’t get an answer as to what’s causing it or if it’s permanent. I’ve hit my breaking point and I really can’t live like this. I’ve dealt with chronic pain most of my life but this is by far the most distressing experience I’ve ever been through. I want to pull my own teeth out and rip my face off, I’m fully aware of how insane that sounds but that’s how intense this discomfort is. The surgeon said he would do some research so I'm praying he comes through.

As far as the bone and tissue healing go, there’s been a little improvement but it’s slowed down a lot. I asked the surgeon if we could do another treatment of platelet rich plasma and another round of hyperbaric and he seemed agreeable. It’s noninvasive and it can’t hurt. The prosthodontist said they would probably need to wire the obturator in again which I don’t see happening. I can’t even touch my teeth with my tongue, there’s no way I’m letting anyone near me with wire because where there’s wire there will eventually be wire cutters and my bizarre EDS resistance to lidocaine and Novocain.

The surgeon and I had a pretty open conversation about my feelings towards him at my last visit. He suggested I get a second opinion because he knows I don’t trust him, so I have one scheduled for next week. I think part of the reason that I get so upset with him is because out of all my doctors, there was a point where I trusted him the most. When you live with a combination of rare disorders, there are a lot of grey areas when it comes to medical treatment. You get misdiagnosed, written off as crazy and shuffled between specialists by doctors who don’t know what the hell to do with you. With all the uncertainty regarding my conditions, this was supposed to be the one thing that was black and white. When people asked me if I was nervous about the surgery I always said no, because I genuinely wasn’t. I trusted him completely. This procedure, unlike everything else in my life, was supposed to be routine. I could sit this one out and not have to micromanage my care, I got to take a break and let the doctor be the doctor. I trusted not only his skills, but his ability to make the right medical decisions for me and he let me down. The decision to change my procedure from a 3 piece lefort to a 4 piece lefort was done after I was already under anesthesia and it’s not something I would have consented to if I had a say.

With the type of EDS that I’ve been diagnosed with, there’s a high risk for vascular complications. At 27 I had a spontaneous hemorrhage in my right eye and things have been progressive since then. I’ve had to grieve the loss of the life I won’t ever get to live, one that involves having children and growing old gracefully. With the acceptance of certain sacrifices you gain an appreciation for the time you do have though. I was willing to commit 3-6 months for this procedure, but not over a year of my life. That kind of time is too valuable when you live with a vascular disorder. As I get closer to 30, so does my risk for a spontaneous arterial rupture and the average lifespan for patients with vascular EDS is only into the mid-40s. What that means for someone with my crossover variant is still unclear, but given a choice, I would have preferred he closed me back up and called it a day. I wanted to spend the last year of my 20s feeling good about myself, traveling and making sure my nieces always remember me as the fun aunt who lets them stay up late and be silly. I’m not just angry about losing my teeth, I’m angry about a risk that was taken without my consent that will cost me over a year of my life, a year I wasn’t prepared to gamble with, a year that was precious to me and to my family and friends.

Hopefully the new surgeon will have some insight into why I still have so much discomfort, and hopefully the clean slate with him will make it easier to have a more positive attitude about my appointments and my future surgeries. More than anything though I really hope that my story can help spread awareness about the serious nature of Ehlers-Danlos Syndrome. Doctors simply cannot continue treating EDS patients the same way they treat everyone else just because we appear healthy. The results of such a cavalier attitude toward the disorder are devastating to the patient. May Is EDS Awareness Month, be aware of rare!

What Next?

I want to thank everyone who's reached out and shown support during this whole process and especially over the last month. I've really been struggling with my surgical complications and with my other health issues since my last update, so I'm sorry for the lack of posts or responses. Even when I'm not feeling up to posting, I still read every comment and message and they really mean the world to me :) It's so incredibly touching to know that my story has reached other people with EDS or others who have struggled with their post op results. Thank you for giving meaning to my journey.

I'll try to recap what's happened over the last month. Not much has changed regarding the issues with the muscle spasms in my chin. My surgeon, like always, had no answers and no suggestions as to what might help. I did get feedback from other doctors who suggested I go back on neurontin and that I could possibly consider botox injections to help relax the muscles. I have a CT scan scheduled for next week so we'll see if that reveals anything. In the meantime this has dramatically impacted my quality of life and I'm so afraid that it will be permanent.

I hit a plateau with the healing so last Tuesday I went for platelet rich fibrin treatment. The process is similar to the PRP I had before, except the end result is a fibrin membrane that the surgeon placed on my obturator. I've kept the obturator in all week to give the PRF a chance to work and I go back tomorrow to see if it helped. It's kind of a long shot but it's a simple procedure and there really aren't any risks so I figured it was worth a shot. If nothing else, it at least satisfied my Type A personality need to be doing something constructive :)

Over the past month I've met with a lot of really talented surgeons from the best hospitals in the area. One thing they all pretty much agree on is that I need to wait until at least October to begin reconstruction. Everyone had a different opinion of how to reconstruct though. These are some of the options:

1. Fibular free flap graft. This involves making an incision from my knee to my ankle and removing most of my fibula along with tissue, an artery and a vein from my leg. The fibula would then be used to reconstruct my jaw and the artery would be grafted to an artery in my neck in order to give the bone graft its own blood supply. The advantages to this would be that the graft would be thick enough to support implants and the graft would be vascularized which would decrease the chances that it would be reabsorbed. The disadvantages are that I would have a huge scar down my leg and a scar on my neck, recovery time would be longer, there's a risk to the donor site, there's a risk of clotting and the graft failing and because of my vascular EDS issues, there's potential for things to go really wrong.

2. Radial free flap graft. This is essentially the same thing as the fibular graft except the bone and artery are taken from the arm.

3. Tongue flap or tongue graft. This was casually thrown out there by one of the surgeons but I shot it down before he even finished his sentence. Basically what this procedure involves is sewing the tongue to the roof of the mouth for 2-3 weeks, allowing it to fuse and then surgically separating the two. My cousin had this done as a child for a cleft palate and it didn't work. He basically spent two weeks in agony for no reason. The advantage to this approach is that it would provide a tissue graft to the palate without causing any disfiguring scars. The disadvantages, well they're pretty obvious lol.

4. A bone graft from the hip or rib and a temporal graft. This would involve taking a section of bone from one of my hips or ribs and then taking a tissue graft from my head. The advantages to this approach are that the scarring would be less disfiguring than the free flap and there's less risk for vascular complications. The disadvantages are that there's a higher chance for reabsorption of the graft, rib grafts are notoriously painful and I would still be left with scars.

5. This option is hard for me to even type without crying and I burst into tears when one of the surgeons mentioned it. Even though I know he was only fulfilling his responsibility to present me with every possible option, it was still difficult to hear and that's the option of never fully reconstructing. Basically they could do minimal reconstruction with a fixed bridge. The advantage to this is that it would involve less surgery, less overall risk and most importantly, less time spent in the hospital and in recovery. The disadvantage is that the cosmetic results would be less than ideal and that I don't think I could ever feel like a whole person again without complete reconstruction.

More important than picking the reconstructive method though, is picking the right surgeon. I've consulted with quite a few surgeons, all of them excellent. One surgeon I really liked but he didn't seem to understand the significance of a vascular EDS diagnosis so he was voted off the island before I left his office. Another one told me "You'll never get your old face back, why don't you look through a magazine and pick out a new face." He also said "We'll it's not like you look grotesque with the prosthetic in." I left his office an absolute emotional wreck and after making a mental note to put him on my list of people to punch should I ever get a free pass, he was also voted off the island. A few local surgeons refused to even take my case and ultimately I was back in the office of the surgeon from Cooper that I really liked before. He brought in a partner of his that does maxillofacial surgery and plastic surgery. This was a combination that really appealed to me. What I liked about these two doctors was that they were both obviously excellent surgeons, but they weren't arrogant. They were both extremely compassionate and willing to research EDS. The fact that they were acknowledging that my EDS was a major factor made me feel instantly comfortable. They also seemed more innovative than other doctors I've met with. They suggested things like BMAC and amniotic stem cell injections. One of them also suggested I try glutamine to put on a little weight and that made me want to jump up and hug him since all my surgeon ever does is suggest I eat scrambled eggs (side note: the mere mention of scrambled eggs is now enough to send me into a full blown rage.) I've put on 3lbs with the glutamine :)

I still have one more surgeon that I have to consult with but so far I think that the best thing for me is to go with a rib and temporal graft with the doctors from Cooper. They also seemed pretty confident that they could fix my nose since it's become super wide after my other procedures. So that was a huge relief.

I would be lying if I said that I always handled my situation with grace. Sometimes I still cry in the middle of the night when I let the gravity of what's happened to me really sink in. I get angry and I yell and I occasionally let regret get the best of me and I come down really hard on myself for making the decision the have the surgery in the first place. One thing I will not do though is ask "Why me?" When I was in hyperbaric treatment I met a lot of really amazing people who had all survived something life altering. Two people in particular helped me through the experience and they both taught me something invaluable. The first is that there are children who suffer horrible things like brain tumors. We should be asking "why them?" If things like that can happen to a child, then what makes me so special? Pain is a part of the human experience, why should I be exempt?

The second thing I learned is to stop asking "What if?" You can drive yourself crazy wondering what you could have done differently. What you have to ask is "What next?" You have to wake up every day and keep moving forward. You have to look ahead at what you can do next to help yourself.

So tomorrow I'll find out if the PRF worked and if it didn't then I'll ask "What next?"

Platelet Rich Fibrin

Tuesday was my third PRF treatment. They seem to be helping which is great because it was such a long shot. I think the plan is to continue them as long as we keep seeing improvement. It took a little pushing but I finally got my surgeon to look into the amniotic stem cell treatment. It's expensive and there's no guarantee that it will help but I'm really hopeful that we'll be able to try it.

I'm still struggling with the muscle issues in my chin. When I smile you can actually see something snap on the left side. You can also feel it if you put your finger over it. The surgeon seemed surprised that he could actually feel it and that it wasn't something I've just been imagining for the last few months. He thinks it's scar tissue and that it could take up to a year to resolve. I'm not buying that explanation at all but there's no point in arguing with him because he doesn't actually care. I have my CT scan tomorrow so we'll see if that reveals anything. I also have two appointments with other surgeons next week so hopefully I can get someone to figure out what's causing all the discomfort. I don't have much faith in doctors at this point though.

My neurologist has suggested that I have eyelid surgery to correct the ptosis in my right eye. As much as I want the issue corrected, my jaw surgery experience has me traumatized and I'm worried I'll end up with some kind of unexpected complications that leave me looking like a cyclops. Seriously, I'll never get another date again with no teeth and one eye. It's not a good look. I guess it would save me money on contacts and eye makeup though. Actually it would save me money on makeup in general because I would just never leave my house again. I'd have to adopt a bunch of cats and live like an agoraphobe. Clearly there's a lot to consider and I'll have to look into zoning ordinances regarding how many cats I can legally own, if Max Azria would design a BCBG eyepatch for me, and whether or not I can get chocolate banana milkshakes delivered before I decide to let anyone near my eye with a scalpel. All joking aside though, I'm not sure I'm ready to deal with eyelid surgery just yet but I'll start a separate review if I decide to go through with it. Hopefully that review will be all about how awesome my plastic surgeon is and not about how a botched eye surgery turned me into an agoraphobic, cat collecting, cyclops recluse.

What Did I Just Agree To?

The head and neck surgeon who recommend the fibular free flap graft sent me to a max face surgeon that he works with for a second opinion. I saw the max face surgeon yesterday and he agreed that the fibular graft was the best option. I was pretty adamant that I wanted the less invasive rib graft but he felt as though it would have a high chance of failing. He did suggest a possible tongue graft but I shot that down again. He also suggested doing another type of tissue graft that would involve leaving a scar on my face (thanks but no thanks).

After a lot of discussion we agreed that he would contact a vascular EDS specialist to see if I'm a candidate for the free flap, my other specialists would be consulted to make sure I have cardiac and pulmonary clearance and something needed to be done about my lower lip and chin (a new med, injections, TENS treatment or acupuncture). I left feeling overwhelmed but still hopeful.

Fast forward to today. I followed up with the head and neck surgeon this morning. He reviewed my CT scan and we talked about what the max face surgeon had to say. In the end he feels really strongly that a vascularized graft is the only option. He said the defect is too big to reconstruct using just a rib or hip. At this point I was willing to reconsider the tongue flap but he said no.

I tried so hard to make it through the appointment without tears but when we started talking about the scars I would be left with I got a little emotional. I would be left with a surgical scar from knee to ankle and another one down my neck. To the surgeon it's just a scar but to me, as a 29 year old woman who's been through facial disfigurement, it's having to sacrifice more of my body, more of myself. It's knowing that even if the surgery goes perfectly, I'll still be left with disfigurement. I look at myself in the mirror every day and I have to force myself to accept my new face, to look past the huge defect where my smile used to be and remind myself that this is not what defines me. I go through this process every single night when I remove my obturator and every single morning when I put it back in. I don't want to go through it every time I look at my leg or every time I want to pull my hair back but can't because I have a scar down my neck.

We scheduled a debridement surgery for September 26th. The plan is to remove some additional bone and possibly repair the hole in my palate. After that heals he wants to schedule the free flap surgery (for those of you unfamiliar with my story this involves removing most of my fibula, some tissue, an artery and a vein from my leg and using it to create a vascularized bone graft).

I left his office pretending I was fine but I really wasn't so I took the rest of the day off work and stayed in Philly. For some reason I felt really alone in the whole process and it hit me kind of hard. I called my dad because he's always my rock and he made me feel a little better. Then in a fairly ironic turn of events I ended up passing an animal rescue so I walked in. There were cats everywhere. I had the thought that my cat lady joke may actually become a self fulfilling prophecy. I filled out an application to foster a senior dog with medical problems. Maybe I felt some solidarity for the dog with one eye lol. Either way I think fostering special needs dogs will be a really positive thing.

It started pouring down rain so I had to walk about 10 blocks back to my car with no umbrella. It felt rather appropriate considering my day but it was also kind of cathartic and I actually felt a little better by the time I reached my car. I'm still letting it all sink in but eventually I have to come terms with the fact that the next step is going to be an incredibly difficult one. In the meantime I'll be sporting a bun and shorts all summer long while I still have nice legs :)

Updated pics

Some of you messaged me and asked me to post updated pics so here goes. These are immediately post op and now at 5 months.

Amniotic Stem Cells

Today was a pretty exciting day. I've been asking my surgeon to try amniotic stem cells for about a month now. He's been less than enthused due to the high cost and the fact that there hasn't been enough research to really know how effective the treatment would be. With a little persistence (and some attitude) on my part, he agreed to look into it. When I went in for my appointment this morning I was really surprised to see that he had taken the initiative to have them delivered. It felt like Christmas, well if Santa was a molecular biologist.

The stem cells are derived from amniotic fluid, so there's no ethical debate like with embryonic stem cells. They're still pluripotent but there's no risk for developing a tumor like with embryonic cells. The ones I was given came from a company called AmnioGenix, based in Tennessee. They came freeze dried in a 1mL vial and had to be thawed out over 15 minutes. I took my obturator out, the surgeon drew them into a syringe and then rather than injecting them, he just squirted them over the exposed bone and tissue. Immediately when he did this a small piece of bone fell off. I know that sounds like a bad thing but it was actually really good, underneath the bone was healthy tissue! The PRF treatments seemed to be paying off and the tissue coverage went from an area of 7mm x 12mm to 10mm x15mm in a matter of two weeks. Anyway, he squirted the stem cells over the area and then I put the obturator back in to try to seal them in. Neither of us had any idea if this was even an appropriate method of stem cell application but it seemed like a decent plan.

What has always upset me most about my situation is the way my post op care has been handled. There was no urgency to try to save the tissue and I never felt like my surgeon went above and beyond to research all the available options that might help me. I often wonder if he would be so lackadaisical if it were him going through this. Today felt like a small step forward in mending a little of my resentment for him though. I appreciated that he had gotten the stem cells and I felt incredibly grateful to have access to a treatment that most people would never get to try. I also felt profoundly thankful to whoever the woman was that made the decision to donate her stem cells.

It was a beautiful day out so I stopped for an iced latte and took a walk down to a used bookstore that I love. I felt close to the old me. I thought about what it was like that first month after surgery. I remember just being in complete agony, unable to close my lips and barely able to stand without help. I thought about my first night home from the hospital, how my sister had stayed with me and I spent hours throwing up in the bathroom with a broken jaw, desperate and in pain and with no fight left. Now here I was sipping a latte with a book in my hand.

I might be a long way from full recovery, but I'm also a long way from that night that I spent in my bathroom, ready to quit and breaking my sister's heart. This whole experience has been an exercise in patience, something I probably needed. Today might not seem like a huge victory, but it sure as hell felt like one.

Jaw Surgery Playlist

I don't actually have anything to update but I was bored so I thought I'd share my jaw surgery playlist, enjoy!

1. "Crooked Smile" - J. Cole ft. TLC
2. "Bigmouth Strikes Again" - The Smiths
3. "Dental Care" - Owl City
4. "Girl, You Have No Faith In Medicine" - The White Stripes
5. "Broken Face" - Pixies
6. "I Wanna Be Sedated" - The Ramones
7. "If You Can't Leave It Be, Might As Well Make It Bleed" - Dashboard Confessional
8. "Novocaine Hurricane" - The Presidents of the United States of America
9. "Dentist" - Little Shop of Horrors
10. "Hospital Song" - Ben Folds Five
11. "Broken Jaw" - Foster The People
12. "Present/Infant" - Ani Difranco
13. "The Not So Pretty Princess" - Jude Christodal
14. "Grillz" - Nelly ft. Paul Wall

And just for fun…
Bonus Track: "Plastic Surgery Slumber Party" - Jeffree Star
HUP

1 out of 5 stars Overall rating
3 out of 5 stars Doctor's bedside manner
3 out of 5 stars Answered my questions
2 out of 5 stars After care follow-up
3 out of 5 stars Time spent with me
3 out of 5 stars Phone or email responsiveness
2 out of 5 stars Staff professionalism & courtesy
2 out of 5 stars Payment process
3 out of 5 stars Wait times
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CHELIE!! So good to read your latest update about the amniotic stem cells! (Many prayers for something on this order have been going up (by me). I AM glad it was the first surgeon finally trying to rescue the destruction. It is not ALL his fault (some blame has to be given to the EDS) but his cavalier way of treating you post-op has been more than a little distressing In fact, it's been d___d upsetting and I have felt your grief for many weeks now. Feeling a little sprig of hope starting. Thinking of you and praying for you...love, Maggie xox
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I think you're giving him too much credit. My EDS diagnosis was clear before my procedure, so his cavalier attitude started long before my complications. He made the decision to change my procedure to a much riskier one after I was already under anesthesia, even with the knowledge that I have EDS. He played fast and loose with my medical consent and then continued to fail me post operatively by not starting hyperbaric oxygen immediately. Yes I have EDS and yes that makes things complicated but ultimately it was his responsibility to factor that into my treatment plan. While I'm happy that he took the initiative to order the stem cells, he only did so because I pushed for it. I have yet to see him do any research or explore any treatment options that I haven't come to him with. I'm working towards forgiving him for my own sake, but I still feel as though he is responsible for what happened to me.
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Hey Chelie, Speaking of a hyperbaric oxygen chamber, do you think continuing with that could still benefit you? With everything your body has going on currently (and in the next few months) I just wonder if that would help your body with healing? If insurance will cover it, and you've got the time it may be something to consider. Every little bit in the right direction can help sometimes. Cheers!
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I definitely think it would still help, my surgeon disagrees but the surgeon from Jefferson thought it couldn't hurt and wrote me a script. The only issue is that I have to go back on disability because treatment is 6 days a week in Philly. I've also developed a cataract from the steroid injection I was given so there's a possibility that the hyperbaric oxygen could make it worse. HBOT will probably be something I always have to do post op though.
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I'm also only allowed so much medical leave at work before they can post my job position so I'm trying to save it for surgery.
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I so wish they were available at more hospitals, but those puppies are hard to find! I don't suppose there's one available closer to either where you live or where you work, so it would be slightly more convenient? The other thing I was wondering about is the protocol. I know there's a standard recommendation, but does it have to be all or nothing? Would there be a benefit to just going once in awhile? Hmm, I don't know...
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We actually have like 4 of them where I work but they're all mono chambers which kind of freaks me out. I'm not usually claustrophobic but I don't know if I could do 2 hours in that thing. HUP is the only place in the tristate area that has a multi chamber. I know when I was going for treatment at Penn they had a 2.5 hour protocol. It took 10 minutes to get down, then an hour of treatment, 5 minute air break, another hour of treatment and then 15 minutes to come back up. The dive didn't count if you had less than 90 minutes of oxygen time. They also want you to go consecutive days with only 1-2 day breaks otherwise you won't see any benefit. It's super time consuming. The first time I did it that was my whole life for like a month. Luckily my insurance covered it because the cost was over $10,000 a day and I had 30 days of treatment. I'm not sure if they would cover it now though.
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Every day for 30 days, yikes. I've only heard of it being used for like 10 or 14 consecutive days. That longer time period would be super challenging. Also, $10,000 daily for 30 days, that's essentially Monopoly money. Shoot. Oh, the cost of health care when you really need it. Anyway, I really hope you had a nice weekend. Cheers Chelie!
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Usually what they do is set you up for 10 sessions and then evaluate you and then add on in 10 session increments. So they wanted me to get at least 20 dives in before they did my second surgery and then another 10 immediately after. One of the women I met there was doing 60 dives for radiation damage after stomach cancer. She was a remarkable woman. Once you get your initial 20 or 30 dives in then it's usually only 10 dives post op. It wouldn't be so bad if you could bring your phone or an iPod in there but you can't take anything that could explode so I read a lot of books and watched a lot of bad movies. I was floored when I found out how expensive it was. I don't think the mono chambers charge that much though. And thanks, my weekend was awesome! I danced my ass off until like 3am that night :) def felt it the next day though lol.
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Thanks its been of great help...somehow i feel not to mess with the way god made me..
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I tend to agree :)
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Dear adi_9833, this is a reasonably brief (for me!) continuation of your discussion re: "messing with the way God made me." Except life on earth has a way of interfering what I believe God wants for us all. I don't think it was HIS plan that caused me to have a recessed lower jaw causing unhappiness with my appearance, and even WORSE, to have adult-lifelong sleep apnea, meaning I could NEVER ever stay awake for longer than an hour in the day. Nor did HE plan (imho) for my first baby to be 9lbs causing all 5 of my short-waisted 5'1" frame to tear 5 out of 5 abdominal muscles, causing a lifetime of looking overweight, even when I wasn't. And of course birth deformities such as cleft lip and palate I cannot imagine you or ANYONE believing that is the way God made those children, not, if there are ways to restore normal fxn and appearance with medical intervention we are interrupting God's plan. I feel 100% certain you agree TOTALLY with this. Not an argument, simply an opinion, hope that's ok with you?? I TOTALLY understand that PS is not for everyone. I actually had NEVER considered it until I recognized in the last two places I practiced PT I could tell everyone thought I was "past it". I could accept that if it were true, honestly. But I would go head-to-head with any new or middle aged PT (other than the exceptions who have not married, devoted their LIVES to research and writing books. I'm not quite THAT vain!!) I doubt anyone reads more than I do, and my patients (at least once a week) ask me, "Why hasn't anyone else told me that? I would have changed _________ (my behavior) if I'd only understood." I believe my face could "get by" as it always has...(which actually is quite young for 64, except for the darned drooping brows and eyelids and fatty changes causing my normal relaxed face to look a bit "forbidding" or "angry") Since I work openly in an intimate fashion as a physical therapist, I actually DO believe improving slightly the face that God gave me that was cute until about 60, is the right thing to do. Hope this just adds to the general PS discussion and in NO WAY offends anyone, that is absolutely not my intent.
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I think the point was that if you're considering surgery for purely aesthetic reasons, sometimes you'll have a better overall outcome by just accepting your flaws and loving yourself regardless. If you end up with severe complications, like I did, you'll wish you just left your face alone. That overbite doesn't seem like such a big deal after you've lost your teeth and part of your jaw. There are always exceptions, especially when the problem is functional, but I think we'd all be better off if we just accepted ourselves more. I'm not against PS, I think if you want it then go for, it's an amazing thing for some people. The problem is when people tie their happiness and their self worth into their surgical outcome.
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@MVPT, understandable :-) I'll be going next week for the braces and let's see what the doc has to say about my dished in lower jaw overbite look..
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Chelie, I was wondering if you have heard or have met with Dr. Diecidue out of Philadelophia? If yes, what was your experience? Dawn Becker
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I've heard the name, I believe he's from Jefferson? I haven't personally had a consult with him though. Sorry, I wish I could be of more help :(
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Chelie, Yes, he is from Jefferson. I was sent to him for what was supposed to be an independent opinion of my current state and surprisingly, he was quite objective. I am hoping that someone can help me regain a normal appearance, so that I don't have to be subjected to comments such as "what happened to your pretty face" My son has difficulty with my appearance because he said that his friends always told him how pretty his mom was and now they say, I feel sorry for your mom. He does not know how to respond to comments such as these and from others, when they ask him what happened to your mom's face. I have been told that since I am unable to use my teeth on the right side, there is a high probability that I will lose them, because I have atrophy on that side.
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Is this from jaw surgery complications?
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Chelie, yes, it was from jaw surgery complications. Three weeks post-op, oral surgeon left the country and never returned. My chin was on the left side of my face, I could not eat or swallow properly, lost 25 lbs, throat displaced to right side, screw protruding from face under eye socket and damage to nasal passage beyond normal repair, no saliva-lived on biotene. Prior to the surgeon leaving the country, he gave me an extremely high dosage of Lorazeapm as I appeared to be suffering from anxiety after the surgery and I had never taken anything beyond an advil, I suffered withdrawals and was hospitalized because dosage was unusually high. I just want to be a little more functional and look normal again. You look fantastic!
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Wow sounds like you've been through quite the ordeal. Can I ask what the original surgery was and where it was done? Your complications sound like they may be beyond what a regular max face surgeon would be able to reconstruct. What kind of corrective surgery are they recommending? If you're looking at Jefferson maybe try their head and neck surgery department? Dr. Krein does major facial reconstruction, I would book a consult with him. As much as I dislike Penn, I have a consult with Dr. Chalian next month, he's supposed to be excellent. I'll let you know how it goes. It really sounds like you should be seeing someone in Otolaryngology. I'm sorry things went so wrong for you, but please know that there is always hope and if you ever need someone to talk to, I'm always here. You WILL get through this!
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Hi Chelie. Lovely to read your latest update. Coffee and a good book, that's sounds like my cup of tea. (Yes, it would be tea for me - being English!) Very interested to hear about the stem cell treatment. Why did they not suggest your own adipose tissue? Have you none to donate, or is their method better in some way? Love your new photos - you look so pretty, you are going to come out of this looking better than ever. You still have a lot more surgery ahead of you and I must admit I was shocked to read about your next op involving your leg. These are big decisions you are having to make, I hope you are able to talk everything through with your family and nearest and dearest, although ultimately I know it's your decision only that matters. I wish you all the best and sending you my warmest wishes. I wish I could meet you to share a latte and a hug, but this will have to do. xx
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Thanks Faye :) I don't know much about adipose stem cells but from what I understand they're adult stem cells. ASCs have a limited ability to differentiate into different cells types. With amniotic stem cells, they're pluripotent so they have the ability to differentiate into various cell types. Both types are immunoprivileged so there's no risk of graft vs host with either. I've done platelet rich plasma and platelet rich fibrin treatments. They seemed to help so my hope is that the amniotic stem cells will be even more effective. Do you know how they harvest the fat for adipose stem cells? I'm afraid I don't have much to donate after all my weight loss. I'm always interested in hearing about new treatment methods though :) Thanks for the kind words and support! I'm incredibly lucky to have a wonderful father and sister as well as some close friends that I'm able to talk through my options with. In the end they're all supportive of whatever decisions I make (and happy not to have the responsibility of deciding themselves lol). I'm sorry we can't share a real latte and hug but virtual hugs are better than nothing! xx
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LOL!  I can't believe there are so many appropriate songs.  I know, like, two of them.  Though at least I recognize most of the artists. :D
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Haha right!? It was way easier to find suitable songs than I thought it would be. Some of them are kind of obscure but I'm into a weird range of music.
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We're huge Smiths and Morrissey fans in this house so that one I know.  Of course the Ramones song is a classic everyone knows, whether they like it or not ;).  But we also performed some songs from Little Shop of Horrors back in junior high so that song I know.  So I guess that's three.
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