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
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.
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
13 Apr 2014
2 months post
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 :(
13 Apr 2014
2 months post
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
16 Apr 2014
2 months post
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.
17 Apr 2014
2 months post
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
24 Apr 2014
2 months post
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.
25 Apr 2014
2 months post
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
26 Apr 2014
2 months post
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
27 Apr 2014
2 months post
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.
30 Apr 2014
2 months post
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
30 Apr 2014
2 months post
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.
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
20 May 2014
3 months post
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!
30 Jun 2014
4 months post
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
10 Jul 2014
5 months post
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?
15 Jul 2014
5 months post
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 :)
18 Jul 2014
5 months post
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
22 Jul 2014
5 months post
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
24 Jul 2014
5 months post
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
How I Cope
This isn't really an update but I've had quite a few people who've had post op complications message me and ask me how I cope with my situation and where I get my strength from. I am by no means an expert in coping (just ask my surgeon) but I'll share what works for me in the hopes that maybe it can help someone else.
I am no stronger than anyone else. I have always been the type of person who feels everything deeply. I have moments when I am completely overcome with sadness and with pain over what happened and over all the things in my life this surgery has cost me (my relationship, friendships, an awesome job opportunity, and my former face to name a few). I still have nights where I cry myself to sleep, even at 5 months post op. I'm not immune to anger, pain or sadness but I'm also not afraid of them either. I allow myself to grieve the things I need to grieve. I let myself experience all of my emotions with my whole heart.
What has helped me is learning how to compartmentalize these feelings. I don't let my negative emotions that relate to my surgery take away from the rest of my life. I wake up every day and make a decision not to let yesterday's pain ruin today. I don't let it take away from the love I have for my family, my friends and for myself. I don't take it out on the people that are close to me. I let myself love, laugh and feel happiness with the same intensity and entirety that I allow myself to grieve. I remind myself to be grateful for what I do have and I continue to have hope.
I am a survivor, not a victim. I don't feel sorry for myself, I never ask "what if?" and I never let myself believe that life dealt me an unfair hand. Life isn't about fairness the same way it isn't about only experiencing things that make you feel good. I choose to have the perspective that life itself is a gift, learn what you can from the bad experiences and use them as a way to appreciate all the good.
With a fibular free flap surgery in my near future I'm afraid of complications to my leg. I think about what it would be like if I lose function or if the incision doesn't heal and I can't do the things that I love, like yoga, running and dancing. So I do yoga on the beach, I signed up for a 5k this month and I went out dancing all night last weekend because right now I can. That may change after my next surgery and if for some reason I lose function in my leg I don't want to end up staring at my closet wishing I had gotten one last wear out of my favorite little black dress. I want to look at it and remember the night I stayed out until 3am, drinking too much champagne and loving my life. I want to get my best time yet on a 5k and I want to execute a perfect firefly pose because I don't want to have regrets.
Something awful happened to me but that doesn't mean I have to stop living my life. I can guarantee that if you wait for everything in your life to align itself before you start really living, you'll be waiting a long time.
It may sound trite and cliche but it's what I have found works for me and most days I find peace (and when it doesn't work, I just call my friend and we make ridiculously inappropriate jokes at the expense of a certain surgeon until we both laugh so hard we cry).
23 Sep 2014
7 months post
I went back and forth for quite a while about whether or not to post this update. It’s deeply personal and not directly related to my physical recovery. Ultimately I decided that emotional recovery is just as much a factor in this process as physical recovery and I feel as though it’s important for people to understand just how far reaching the emotional fallout of this surgery really is. There isn't a single aspect of my life that hasn't been impacted.
If you’ve been following my story then you already have an idea of how difficult this journey has been for me. It took me months to get to a point where I could face myself in a mirror and not cry, and even still I have days where I fail at that. I’ve had to learn to accept my new face and the changes and limitations it has imposed upon my previously happy life. I have persevered and endured seven months of physical and emotional hell and I have made an effort every single day not to let it beat me. I often feel like I was ripped out of my own life and shoved into someone else’s, like these experiences were never meant to be mine. They are mine though, however surreal they feel and I am determined to make the best of them with as much grace as I can muster.
I’m at a point now where my face looks more normal, I’m eating again, my weight is stable and I’m laughing and feeling more like my old self every day. I was starting to feel like maybe I made it through the worst of it and I was finally coming out the other end. I even thought maybe I was ready to try dating again. I was tentatively trying to piece my life back together and return to something that felt close to normalcy. I met a guy and there was something about him that made me feel like it was ok to put myself out there again. He was funny and sweet and he seemed like the kind of person who had enough depth to understand that while this surgery is such a huge part of my life right now, it’s only a small fraction of the person that I am.
I was completely open with him about everything. He had a ton of questions and I answered them all honestly because I’ve never been the type of person to hold anything back. I expected him to run the other way, and to be honest I wouldn’t have blamed him if he had. What I’m going through isn’t easy and most people would be overwhelmed by it. He didn’t run though. For the first time since my surgery I felt like someone was able to see past my situation and really like me for me. He made me feel comfortable and strong and like I didn’t need to be embarrassed. For the first time in seven months I felt happy and excited about the future. I remembered what it was like to be a normal girl who had butterflies over a cute guy, finally I was able to shift the focus off of my own tragedy and back onto my life.
I was on my way to dinner with my family to celebrate seven months post op when he texted me to let me know that it was in fact too much for him and he was sorry and hoped I didn’t think he was a jerk. It probably goes without saying that I absolutely did think he was a jerk, not because he was overwhelmed by what I had going on, that part was completely understandable. He was a jerk because he had pretended to be able to look past it just so that he could pry into my life out of some morbid curiosity. I had never had anyone make me feel like such a sideshow freak. I wanted to cry but if I started I knew I wouldn’t be able to stop and I still had to make it through dinner.
I managed to make it through dinner with only slightly watery eyes. I lied to my family and told them my contacts were irritating me. By the time dessert came I wanted to bawl into my bread pudding but I took a deep breath and forced myself to hold it together. I wanted my family to see how much progress I had made, how much like my old self I was. I wanted to be strong for them and I wanted them to feel like things were better. The drive back from the restaurant felt like the longest hour of my life. Tears started to slip out and run down my cheek and I knew I wasn't going to last much longer. I needed to get home so I could cry and write and get these feelings out. I could feel a lump forming in my throat and I wished I was anywhere but in a car full of people who so desperately wanted me to be happy.
As soon as I got to my car I cried. I cried the kind of full body, ugly face cry that you can only do alone or in front of your sister or best friend. He wasn't interested in me, he was curious about my story and I was foolish enough to mistake that for him accepting my situation. How could I have let myself believe that anyone would ever want me as damaged as I am? I wasn’t even crying over him really, we hadn’t known each other long enough for me to be upset about it not working out. I was crying for all the things this surgery has cost me and for all of the things I was just realizing it would eventually cost me. I cried for the life I knew I was never going to have, for every time I've had to look in a mirror and feel like less than a person, I cried for the happy ending I was stupid enough to believe in. I cried with every piece of my soul that night until I was out of breath and my eyes were swollen shut and something inside me finally gave out.
He had used the term “bad timing” so many times that I kind of wanted to punch him in the face. What he was actually saying was “I like you but you’re not worth waiting for.” I had never asked him to go through this with me and I had never looked to him for emotional support but somehow he made me feel like my experiences were a burden on him and that made me realize that I would probably never find someone who was able to see past my surgery regardless of how strong or confident I am. It also made me realize that guys like him are a dime a dozen but there are very few people who could go through what I have gone through and manage to keep their head up and their heart still open.
I always try to measure my progress against where I was immediately after surgery rather than where I was before it. It makes my goals and expectations more realistic and it gives me some perspective on how far I have come. To me, I see myself as so far from the person that was lying in a hospital bed unable to speak and I often forget how far I really am from being normal. This experience has made me realize that other people judge me by how far I have left to go and not how far I have come.
My brief venture into the dating world left me disheartened and with a brutal dose of honesty. I can't control how other people see me or how they choose to determine my worth. It will probably be a long time before I try dating again but one thing that I know with complete certainty is that I wasn't the one who wasn't enough, he was.
A Shift in Priorities
20 Oct 2014
8 months post
Last week I had my two month follow up with the reconstructive surgeon from UPenn. I went to the appointment hopeful that we would be able to set a date for my next surgery which I thought would be happening sometime next month. My last appointment I wasn't in the best of spirits but I felt like things would be more positive this time, however that positivity dissipated after waiting over an hour for the surgeon to come in the room. I think maybe I have some PTSD because sometimes just being in that hospital is difficult and the longer I wait the more emotional and agitated I become. After examining me he said he wanted me to make a follow up appointment in two months so we could reassess. He went on to explain the importance of healing in a rather condescending and patronizing manner. I was livid. I had taken off of work, driven into the city and waited over an hour for him to tell me absolutely nothing. He could have easily ascertained the same information from my surgeon who I had seen less than a week prior. I asked him how long he thought it would be before I was ready for surgery and he wouldn't commit to a timeframe but said it could be an additional year or two. I felt like I had just had the wind knocked out of me. He then told me that being ready emotionally is just as important as being ready physically. He made me feel as though he was judging me, like I wasn't strong emotionally and I resented that and him.
Today marks eight months since my initial surgery, now I am looking at possibly 1-2 more years before we can do reconstruction, an additional waiting period for that to heal, ortho again and then eventually implants which take 7-10 months. This process could drag on well into my 30's. Why was I led to believe that this would all be complete within a year or two after the first surgery? They obviously knew it would take much longer but instead of being honest about the process they waited for me to figure it out on my own (which seems to be common practice among the doctors at this hospital). I couldn't get out of the office fast enough. I had just made it to the elevator when I started to feel hot and I couldn't catch my breath. I felt like I needed to get outside before I suffocated. I stood there in the rain having a full on panic attack. I was hyperventilating so in an attempt to calm myself I sat down on a bench not realizing it was covered in water. The back of my scrubs were immediately soaked which sent me over some type of proverbial edge. For the first time I truly just wanted to give up. The thought of having to go through this process for years was more than I could bear. I was fighting a battle with no tangible end and it was exhausting. I didn't want to wake up another day and face myself without reconstruction. Though I've worked hard to make myself understand that my appearance does not define me, when I'm sitting at home, by myself at night without my obturator in, I can't help but feel less than whole. There is a piece of me, of my face, that is missing and it feels truly awful. I feel trapped, unable to eat, drink or even speak properly without the help of the prosthetic. It's as though I'm claustrophobic in my own body, in my own face and it absolutely takes its toll psychologically.
The following day I drove back into the city for an appointment with a facial pain specialist from Jefferson. Since my surgeon has made no attempt to help treat the tightness and discomfort in my chin, I've been seeing a different doctor. Initially he prescribed a low dose of nortriptyline but it didn't seem to make much difference and the negative side effects far outweighed any improvement I was seeing. He switched me to Topamax so hopefully that will result in some improvement, if not I have to try at least one more medication before we can move on to Botox injections. My appointment with the Jefferson doctor made me realize how much more comfortable I felt being in a different hospital and it helped to shape my opinion of what I should probably do next. I've been putting off the decision to sue for quite some time but I think now it may be something I need to seriously consider. Perhaps the best thing for me would be to seek the cost of past and future medical expenses so that I can pursue better care.
Throughout this process I've only really wanted two things out of my surgeon. The first and most important is that he spread awareness about Ehlers-Danlos Syndrome so that this never has to happen to anyone else. It has been eight months and I have never once walked into an appointment and had him tell me about something that he's done to help raise awareness for the disorder. I doubt he knows any more about it now than he did eight months ago and as far as I know, the case report was never even written. He has no desire to advocate and even though he has firsthand experience with just how devastating the complications from EDS can be, he feels no responsibility to pass that knowledge on to other surgeons or to other patients. I find his complete lack of interest and concern not only an insult to myself, but a complete injustice to patients everywhere. My life has been altered, permanently and immeasurably by this man and he hasn't made any effort to understand that or to prevent it from happening to another person and that's not something I'm willing to overlook.
The second thing that I expected from my surgeon was that he do everything possible to make sure I have a full recovery. He has never come to me with an idea for something that he thinks might help with healing or reconstruction. It's always me researching things and then trying to convince him to try them. Even now, I feel as though I have to fight him to continue with plasma treatments and he still doesn't provide them with any consistency. He has refused additional hyperbaric treatment and he basically wasted an opportunity to use stem cell therapy by simply squirting them over the exposed bone rather than injecting them into the surrounding tissue. He makes no effort to research anything that might help me in my recovery, not even supplements. He continually shares stories with me about vacations he’s going on or places he’s had dinner and it’s as if he’s purposely rubbing in the fact that he gets to go on living his life while mine is at a standstill because of him.
This man has given way less than 100% since before my surgery even happened. The entire situation could have been avoided with better planning, more research and respect for my right to informed consent. After the surgery I was denied hyperbaric treatment because my doctors were misinformed, having that treatment in time could have potentially saved the tissue and prevented everything that happened after. There has consistently been a lack of effort and a substandard of care on behalf of the surgeon, the residents and the hospital.
When I thought that reconstruction would be complete within a year or two, a part of me wanted to wait to pursue a lawsuit. If there was a possibility that they could make every effort to put me back together in a way that I could live with, then perhaps a lawsuit wasn’t necessary. Now, with the knowledge that this could drag on for years to come, what happens if I’m not happy with the final result? It will be too late to seek legal action. What if they can’t fix my nose with a simple alar cinch and I end up needing rhinoplasty? Who absorbs that cost? If my surgeon and the hospital refuse to put in their best effort now, how will my care be affected when they know I no longer have to option to sue?
What I have been doing for the last eight months is prioritizing the interests of a person who rarely seems to prioritize mine. Why should I worry about his feelings or his career when he’s not concerned with how his decisions have impacted the quality of my life, how they have stolen valuable time that I will never be able to get back. What I want is a doctor who is willing to try everything possible to help me get better. I shouldn't have to be continually pushing him to try treatments that he should be pursuing on his own. It only causes me more stress in an already stressful situation. I want a doctor who will research new treatments and provide me with every possible benefit. It's become obvious that this surgeon is not willing to advocate for me, but that doesn't mean I won't advocate for myself. I may not be able to put this situation behind me just yet but what I can do is put him behind me and move on to another team of doctors who will make my recovery a priority.
13 Nov 2014
9 months post
Thanks to everyone for the recent positive comments and messages :) Unfortunately due to some complications from a recent unrelated surgery, I won't be posting for a while. I appreciate all of the support and encouragement I've received from the RS community and I'll be back as soon as I can
One Year After My First Surgery
Thank you to everyone for all of the positive comments while I was gone. I had quite a few complications from my eye surgery and needed some time to focus on getting better and to learn to accept my new visual limitations. The past few months have been difficult but I've been working hard to get back to myself.
There have been a lot of new developments with my jaw surgery plan so I'll try my best to cover everything in this post. I am no longer following with my initial surgeon and instead made the decision to follow with the surgeon from Cooper. I've always felt comfortable with him and I trust him to make the right decisions. The last time I saw him was in December and he advised me to consult with a doctor at the University of Miami who specializes in bone morphogenic protein. On January 20th I flew down to Florida for a consult with Dr. Marx and his surgical team. I was very impressed with the whole team, they seemed both competent and compassionate. They suggested a three step surgery process. The first surgery will be on March 18th and I'll go in for debridement of more bone and the removal of some of my titanium plates. Approximately two months after the first surgery I will undergo a second procedure where they will take a tissue graft from my thigh (ALT graft) and use it to reconstruct my palate. Three months later I will undergo what will hopefully be the final surgery which will involve taking bone marrow from my hip and using it to create BMP to hopefully grow new bone. It should take about six months for the new bone to form and then I can undergo the final step of implant placement. I will be following with the surgeon from Cooper between surgeries and the entire process should take about 18 months.
This strategy seems like a better alternative to a fibular free flap but I would be lying if I said I wasn't still terrified. The graft they will need to do will leave me with a scar the length of my thigh as well as possible scars on my face. It's a sacrifice that I should never have had to make and to me it means having to give up even more of myself because of someone else's mistakes. I wish that there was another way, one that would leave me feeling more complete at the end of this journey but unfortunately this is just something I will have to come to terms with.
The plan to remove more bone during the first surgery means that the defect will have to get worse before it gets better. I keep thinking back to the first time I had to face myself without the obturator and knowing I'll have to go through that process all over again hasn't been easy. Every time my face changes it's difficult to feel like I'm not losing my identity. I just have to find a way to keep reminding myself that it's still me and that I will overcome this.
It's been a year since my first surgery and I haven't regained normal function in my lower lip. I still have the discomfort in my lip and chin and I still have a great deal of numbness. I've been seeing a facial pain specialist and we've tried different combinations of meds but without much success. Tomorrow I'll see him again for Botox treatment so hopefully that will help, if it doesn't then hopefully it will at least keep me from getting wrinkles :)
I want everyone to know that even though things have felt so difficult lately, I still haven't given up hope. I still try to find some joy and happiness even in the worst of days. I followed through with my goals of running a 5k again, getting a few last wears out of that LBD, rescuing some very deserving dogs and traveling. I'm sure that during the next year and half life will hand me a few more downs than it will ups, just as it did this past year but that just means I have to cherish the good moments even more.
Pre Surgery Feelings
Yesterday I met with a surgeon from Cooper who's going to be involved in my follow-up care. After he introduced himself he asked me the thing that every surgeon asks when I first meet them, "What is your desired outcome? What are your expectations for a final result?" This question used to make me cry because the answer was always "I want my face back. I want to look like me again. I want back what was taken from me." Now that I'm somewhat of a consult veteran, I know how to shelf my emotional response and give a more practical one. "I want to look as normal as possible. I want full reconstruction with tissue, bone and implants."
The realization that I would never look or feel like myself again was a slow one. Right now when I have the prosthetic in all I can feel is plastic where the roof of my mouth, my teeth and my gums should be. It feels foreign and uncomfortable. When I take the prosthetic out I feel exposed bone, raw tissue and a huge empty space. Either way is a constant reminder of what I've lost. When I meet new people I feel like I'm pretending to be a normal person who hasn't suffered a major trauma. I have to fight my urge to run away before they have a chance to figure out that what they're looking at is the illusion of a complete person. I have to constantly force myself to be brave and to keep my head up.
With my next surgery coming up so soon I've been dealing with a lot of feelings that I thought I had already overcome. I still actively try to do things that will make me happy and that will give me purpose but I always wonder how my old self would be in the same situation. I picture the person I used to be having the same experience, how much more carefree that person would be and it's like I'm experiencing the world in third person all the time. I resent that I have to work so hard at finding peace and happiness when they used to come so naturally.
I'm doing some traveling before my next surgery and instead of being excited about my trip, I've been having panic attacks. Even though I have wonderful, supportive friends, I worry about them seeing me without the prosthetic for the first time and how they'll react. The only person who has seen me without it, besides my doctors, is my father and it was only once. Logically I know that my body went through a trauma and that this was not my fault but it's still hard to reconcile that knowledge with the humiliation and shame of missing teeth. It's hard to imagine anyone else being able to accept something that I'm still struggling to accept myself.
I can't begin to describe what a violation of self this process has been and continues to be. There aren't words to convey the grief, fear, humiliation and pain this has caused. I know that in order to move forward I have to allow the surgeons to remove more of my jaw. The rational part of me knows that this is a step toward physical healing even if it feels like a huge emotional setback. I know that I will overcome and persevere just as I always have because that's who I am but it doesn't make it any easier to take this next step.
Three Days Post Op
I just wanted to give everyone a brief update. Wednesday was my debridement surgery. I was discharged from the hospital yesterday and I'll be staying in Miami for follow up until the 25th. Unfortunately they had to take more bone than they originally anticipated and I woke up in recovery missing an additional two teeth. The shock of losing additional teeth combined with PTSD from my previous surgeries as well as a lack of family support has made this situation particularly difficult. I'm still coming to terms with everything but I'll give a full update when I'm back home and feeling more up to it.
Finding a Balance
There are times when these updates seem to write themselves and then there are times when facing my situation feels so impossible that I can't even bring myself to think about it, let alone write it. Those are the times when it takes every piece of me to get up in the morning and keep going. Those are the times that I lose my fight and allow my grief to take over. My last surgery was incredibly hard on me emotionally and it took a while before I was able to cycle through the grief and get to a place where I could give an honest update.
When I first went to Miami for my surgical consultation, I was impressed with the team of doctors I met. They seemed capable and compassionate and I felt like my concerns were heard which helped to instill a sense of confidence in these new doctors. We spent two hours discussing my case and in the end it was decided that a fibular free flap would be too risky because of the possibility that I could lose my foot from surgical complications. They proposed a series of three surgeries to remove the damaged bone, restore the soft tissue and then eventually to create new bone growth. It felt like the most logical and sensible strategy and I decided to put my trust in this new team and move forward with them.
Two months later I arrived in Miami for the first of my next three surgeries. I felt like I was dealing with a completely different team this time. The lead surgeon mentioned that he was still considering the fibular free flap that we had previously ruled out, the attending physician didn't seem to understand my complex medical history and no one could seem to keep my drug allergies straight. I went into surgery nervous and afraid that I had made a huge mistake by coming to Miami.
When I woke up in recovery the first thing I remember is feeling intense pain in my face and throat. The anesthesiologist had stopped by and told the nurse to give me pain meds. When the doctor left, the nurse refused to administer them, saying I didn't need them. The next thing I remember is the taste in my mouth. They had gotten glue all over the bottom and front of my prosthetic and it tasted like paint thinner mixed with blood. The nurse would't allow me to have water or ice so I began trying to scrape the glue off with my finger to get rid of the taste. That's when I realized two of my molars were missing. Panic began to set in while I tried to figure out exactly what had happened during surgery. The removal of additional teeth had never been discussed and after everything I had already been through I couldn't understand why this surgeon would pull them without so much as a warning. I asked to speak with him but was told he was already in another surgery, this seemed unlikely considering it was already almost 7pm and I began to get more and more upset with the lack of information I was being given. I wanted to know why the teeth had been removed, why it was never discussed beforehand, why a new obturator hadn't been prepared and how long I would have to wait for a new one to be made. Were they planning to send me home with missing teeth? Twenty four hours seemed like an excessive amount of time to have to wait for answers.
I wished that my dad or my sister were there to help me get through this. Instead, my mother had come with me. We've never had a particularly close relationship but I thought maybe this would help to mend that a little, I was wrong. She left shortly after I was out of recovery and I was left to deal with the emotional fallout on my own. Later that night one of the assisting surgeons came into my room to explain what happened during my surgery. He said that they didn't plan on removing additional teeth but the bone above them had lost blood flow and they weren't able to save them. I understood that they did what needed to be done, and I tried to explain to him that I wasn't upset about them removing the teeth, I was upset about having to figure it out on my own while in recovery. My past surgical experiences have left me with what my therapist calls a negative schema. A schema is a mental framework that helps you organize information so that it can be interpreted quickly and easily. We create schemas automatically so that we can better understand the world. In my case, the schema I've developed for doctors and medical procedures is one of mistrust. Feeling as though I'm not being heard when it comes to my medical treatment, triggers an anxiety reaction similar to PTSD. I appreciated that the assisting surgeon came to talk to me, he seemed to have a level of compassion that I wish all doctors had, but what I needed was for the lead surgeon to understand that I couldn't feel confident proceeding with the rest of the surgeries if I didn't feel as though he had respect for my right to informed consent.
I requested a patient care advocate several times but was never appointed one. The following night the lead surgeon finally came to speak with me. He was dismissive and condescending and he seemed almost amused by my anxiety. He refused to send me for hyperbaric treatment and told me that he "wasn't going to allow me to direct my own care and that if I didn't like it I could find another surgeon." I felt like I had made a huge mistake trusting this man with my medical care. By my third day in the hospital I asked to speak with the floor supervisor because I hadn't been receiving any of my daily medication or breathing treatments. They were never ordered and eventually I was discharged with no post op instructions except to follow up in the clinic the next week. On my discharge papers they had stated the reason for my stay as a broken arm.
Things with my mother remained tense and I ended up having to drive myself from the hospital back to the hotel. When I got back to my room I realized my mom had been smoking in it and the smell immediately made me nauseous. All of the clothes I had left in there, the sheets and the pillows smelled of cigarette smoke. Instead of getting some rest I had to pack all of my stuff and go down to the front desk to request a new room. This caused a major argument with my mother and she disappeared for a few hours. When she came back she told me she was going back home and she left the hotel and went to the airport. It was three days after my surgery, I had just been discharged from the hospital and I was alone in Miami but at least I could finally get some rest. Later that night my sister texted me that my mom was stuck at the airport and couldn't get a flight home. I ended up having to drive an hour and a half out to Ft. Lauderdale to pick her up. It was past midnight when I finally got back to the hotel, I was physically and emotionally exhausted. The stress was too much and I broke down and cried until my eyes swelled shut. The next few days were incredibly difficult but I was able to face time with my dad a few times a day and helped me get through it. My grandmother lives a few hours away so she and my uncle came to visit and it was a nice break from all the tension with my mother.
When I went for my follow up appointment they unwired my obturator. It was hard to face myself with an even larger defect but I wasn't going to allow anyone to see me cry. I managed to hold it together through what felt like the longest four hours of my life. The prosthodontists tried to adjust my old obturator and add additional teeth to it. For some reason they couldn't figure it out and they had bent the wires so badly that it kept falling right off. I left with a poorly constructed and very uncomfortable version of my former prosthetic. I called my prosthodontist in Philly but it would be two weeks before she could see me and then another three before a new obturator would be ready.
I flew home the next day and made an appointment with the surgeon from Cooper. I didn't want to continue with the Miami team and Dr. Smith was the only surgeon I trusted to manage my care. I told him what happened and asked him to recommend someone else to do my reconstruction. Dr. Smith has always been incredibly kind and he takes the time to really listen to me and make me feel comfortable. I can tell that he always has my best interests in mind and he makes me feel more like a family member than a patient. In the end he suggested I continue with the team in Miami and as much as I didn't want to agree, I knew he was right. The lead surgeon had a terrible bedside manner and our personalities definitely clashed but I couldn't deny that he's an excellent surgeon. I was healing well from procedure and ultimately that was what mattered. I may not like him on a personal level but I do have respect for his surgical skills. So how do I get over my mistrust and have faith that he's the right person to handle my reconstruction? I still don't know but I've been working hard to find my emotional footing and that's really all I can do.
When you go through a trauma the line between reasonable grief and depression can become blurry. It's difficult to tell how many tears are too many and it's even more difficult to know who you can trust when medical mishaps have become your standard of care. I've become used to my doctors not fully understand EDS and so I've been forced to become hyper vigilant over my medical care. I will never stop advocating for myself but I do have to find a way to put some faith in my medical team. It's not an easy balance to find but I'm confident that I'll get there eventually. In the meantime all I can do is take a deep breath, hold my head up and move forward.
But You Look Fine: What I Wish People Would Understand
The other day I had someone tell me that I should just be happy that I didn't lose both my feet, that I look okay and so I should be grateful. It struck me as the type of thing that only a person who has never experienced a personal tragedy or physical trauma would say. What I tried to explain to this person is that trauma is an individual experience. There will always be someone, somewhere in the world, going through something worse but that doesn't make my suffering any less real or valid. Am I happy to have both my feet? Of course. At one time I was also happy to have a jaw though. If you ask a person with a below the knee amputation if they're grateful to have a jaw they would probably look at you like you were insensitive and completely out of touch, the same way I'll look at you when you tell me to be grateful for my feet.
I was relaying this story to my friend Sarah and this was her take on the matter: A person with a prosthetic leg looks fine when they have pants and a shoe on over their prosthesis. They're able to walk, run and sometimes even do things like ski or snowboard. You wouldn't look at them and right away assess that they were missing a leg. That doesn't make their loss any less real. In much the same way I "look fine" while wearing my obturator but just because my loss isn't immediately evident, doesn't mean it isn't there.
What people can't seem to understand is that there is now a part of my face that doesn't feel like my own. There is never a moment when I don't feel the plastic inside my mouth or the nerve damage in my face. Having an injury or pain that involves your face is, in some ways, much worse than an injury to a limb because you can't distance yourself from it. You can mentally try to separate yourself from your arm or your leg, you can sometimes manage to shift your focus elsewhere. An injury involving your face however, is constant. Trying to separate yourself from your face is nearly impossible. Every time I eat I'm reminded that I cannot bite into anything or chew properly. Every single night when I go to bed I have to remove my prosthetic and face a defect that never should have been. Even the good days will inevitably end with me having to look in the mirror and face a person I no longer recognize as myself.
"Can't you just stay like you are and not have any more surgery?" It's a question I get asked pretty frequently and one that never fails to try my patience. Every time I answer it I have to start by taking a deep breath and reminding myself that the person asking doesn't have a clue how deeply this process has impacted the very core of my being. When people look at me I'm wearing a prosthetic and so I appear normal to them and based on their perception that seems like it should be enough. What people fail to take into account is that the prosthetic is a facade. They don't realize that I feel incomplete, that I have to remove it every night and put it back in every morning. That I cannot bite, or speak properly. That my ability to communicate, to eat, to drink all depends on a foreign object. That my body recognizes the object as foreign and it often causes me to gag and choke and that as amazing as my prosthodonist is, it's still uncomfortable. People don't understand that I lost a piece of myself and no prosthetic can ever replace that. I need reconstruction to help me feel whole again, so that my ability to function in life isn't forever tied to a medical device. For me to feel free and whole I need my reconstruction to be as complete as possible. I manage to keep going only because I have hope that what I feel now is temporary. Right now I'm getting through with the prosthetic but I don't want to spend the rest of my life just trying to get through, I want to be able to put this behind me and that's impossible with an obturator as a constant reminder of what happened.
The fact that I'm able to conceal the physical deformity with a prosthetic while I'm in public doesn't make it any less real or present in my life. I start and end each day by having to look at my missing upper jaw. I feel a surge of panic every time I have to yawn in public because my obturator slips down and I'm terrified people will see it. I spend an incredible amount of time obsessing over my face, avoiding certain words that are difficult for me to say or foods that are too hard to eat in front of other people. I have to cut my food into small pieces and people look at me strange when I use a knife and fork to eat a sandwich. I can't blow my nose. I have a hard time drinking out of a glass because of the nerve damage in my face and I end up spilling whatever I'm drinking down my chin. It impacts nearly everything that I do.
"There are moments when you realize nothing will ever be the same and time is divided into two parts- before this and after this." I never really understood this quote until after my jaw surgery. My life after surgery is no longer the same as it used to be, I'm no longer the same and I'm not sure I ever will be again. As I've said many times before, this surgery has taken so much from me. What I haven't ever said is that there are times when it has even, if only for a moment, taken my will to live. People read my story and they think that I always have a positive outlook on my situation but what they don't realize is that it takes work to be an optimist. It takes an incredible effort for me to find happiness when before it was so natural. For the most part, when I write my updates I do so when I've gotten myself to good place emotionally. No one takes a picture of themselves crying at 3am. You won't see any selfies of a person staring down a pill bottle with the hashtag #todaystheday, but those moments happen even to the strongest of us. We do our best to focus on the happy moments, no matter how fleeting. We take pictures of ourselves laughing and smiling because those are the emotions and the moments we want to cling to but it doesn't make the dark times any less present. We use our experiences to try to help and inspire others because having purpose makes the pain more bearable.
My strength doesn't come from constantly maintaining a happy outlook. It comes from facing even the darkest of times head on. It comes from taking the hits and choosing every time to get back up. I make a decision every single day to keep fighting, to search for happiness and to try to create joy from sorrow but it isn't always easy. I take trips and spend time with friends and family because if I didn't then my grief would consume me. I push myself forward even in the worst of times because that's when it's the most crucial to do so. What separates grief from depression is a person's ability to keep going in spite of everything. The pressure to overcome is sometimes overwhelming and it creates a feeling of isolation while I try to battle my demons and get to a better place but I do it because it's important for me to create as many happy moments as possible so that I can cling to them when I want to give up. I do it because I want to teach my nieces that giving up is never an option. I do it because wallowing in my own grief feels selfish and unfair to the people I love. I do it because I owe it to myself to see what tomorrow will bring and mostly I do it because I need to see something good come from all this.
Finding a Way to Move Forward
Many of you have messaged me and asked me to update on what's been going on the last three months. There's honestly not much to report. I haven't had any additional surgery since my procedure in March. Physically things have healed well but emotionally I haven't been able to take the next step. Full reconstruction is what I want more than anything in the world but it's incredibly difficult to come to terms with the things I have to sacrifice to get to that point.
Taking the next step means agreeing to let doctors mutilate another part of my body. It means allowing them to cut into my leg and giving up another piece of myself. This would be hard even if I had full trust in my surgical team but it becomes nearly impossible when I have none. After what happened in Miami, I don't feel like the surgeon fully understands EDS or the increased surgical risks for EDS patients. How do I willing let another surgeon that I don't trust cut into my body? How can I forgive myself if I let him operate and his lack of awareness costs me my leg, or even worse, my life?
I feel paralyzed, unable to be completely free and happy in my current situation but terrified to take the next step for fear I'll end up even worse. All I want is the one thing I can never have, to go back to my old self. To keep my body intact. To walk away from this restored and without scars. I know these are things I need to find a way to overcome but how do I go into this knowing that I will never be the same, that I'll wake up even less myself than I am now?
I keep praying that another option, a better option, will present itself but there isn't one. The life I have now isn't something I'd wish on anyone but I've managed to find just enough happiness that I'm terrified of losing it. At some point I know I have to move forward. I have to eventually take that leap and hope that the sacrifices I make will leave me feeling more whole in the end. Logically I know that I need to take the next step but emotionally I feel frozen in place.
People expect me to always be brave and strong but that's an unrealistic standard to uphold. You can't go through something like this without moments of vulnerability and uncertainty. Right now my whole world feels so fragile that the slightest upset could cause it to shatter and I know enough to know this isn't the right time for more surgery. I'm working hard to get to a better place but it takes time. Thank you to everyone who has reached out and shown support and encouragement. I'm beyond grateful for the RS community and all the strength I continue to find here.