I had double jaw surgery and genioplasty just over...
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.
One Month Post Op
More bad news
Medical Records and Second Opinions
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?
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
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
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 :(
A Little Positivity
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.
Two Week Post Op
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.
No one was hurt but my car isn't drivable. I had to explain to three police officers what happened, which was humiliating.
Luckily the guy I hit was young and super nice but the whole thing is so much added stress.
No Secrets on the Internet
I never publicly posted the name of my surgeon because this isn't about him. While I definitely get frustrated with him at times, I'm not trying to bash him either. I had a woman private message me and ask me who did my surgery. She was nervous because she was having surgery at the same hospital as me and wanted my opinion. I told her who did mine and I also told her what I would do differently if I could go back in time and make more informed decisions.
My surgeon informed me that he received a call from this woman regarding my blog. Even though this is a public forum, the information I exchanged with her was done privately and there should still be some level of confidentiality. This blog is no secret, there's nothing on here that I haven't already or wouldn't say to my surgeon. If he asked me for a link to my page I would gladly give it to him. However, I feel like calling him without asking whether or not I'm ok with it first is wrong. I'm still receiving treatment from him, he's still responsible for my care.
I'm more than happy to share my experience, offer my opinion or provide encouragement but please don't take advantage of that.
Being Your Own Advocate
When I 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.
3 Week Post Op
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.
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
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!
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
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?
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 :)
Amniotic Stem Cells
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
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
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).
Second Thoughts and a Seventh Opinion
When I first met with the reconstructive surgeon from Jefferson and he suggested a fibular free flap, alarms started going off in my head. I only half listened to what he was saying because I was picturing myself ending up with a below the knee amputation from some never before seen complication. I remember interrupting him and telling him there was no way I would agree to something that involved moving arteries. My gut was telling me to run out of his office while I still had both legs intact. I had no doubt that this man was an excellent surgeon, but he very obviously was not factoring in my medical history and I wasn't about to make the same mistake twice.
After the initial shock wore off and I consulted with another surgeon, I began to accept that the free flap was the only option left. I was absolutely terrified but I did my best to put on a brave face for my family and friends. Outwardly I tried to be as optimistic as possible. I made jokes about being able to get a prosthetic leg that you can still wear heels with, but I wasn't telling anyone that I was quietly putting together a will and an advanced directive. Something inside me made me truly believe there was a good chance that the surgery would go wrong and I wouldn't make it off the operating table. It seemed like so much more than my body would be able to recover from. I tried repeatedly to shake the feeling and to tell myself I was overreacting but I just couldn't. I started looking for information in the EDS support groups and that's when I came across stories of people who had coronary bypass. Even though this is a completely different surgery, all the vascular EDS patients that I spoke to who had undergone bypass said they had major complications that caused their arteries to dissect or shred. Some of them made it and others, whose stories were told by family members, weren't so lucky.
I was left to face the impossible. Do I refuse the surgery and live the rest of my life feeling like less than a whole person or do I take a chance at reconstruction knowing that I may never wake up from surgery? There were so many factors to consider and so many questions I had to ask myself. What kind of life would I have if I chose not to have surgery? Would I be strong enough to endure a lifetime without reconstruction? There was still a chance that the surgery would go well and I would be able to have a normal life. Could I really put what I wanted ahead of what's best for the people that love me? Would it be selfish of me to take such a big risk? I felt overwhelmed and the stress of trying to stay strong in the face of so much uncertainty was leaving me exhausted and feeling incredibly alone.
At the request of my original surgeon, I had set up an appointment with a reconstructive surgeon from Penn. I knew he had a great reputation, but they all did and that meant nothing to me. I had no interest in having another surgery at that hospital, especially not one as major as a free flap. The day of my appointment I met with my surgeon first and it took all of my energy and self control not to lose it on him. I left and went over to the reconstructive surgeon's office. After waiting in the exam room for almost two hours my temper started to get the best of me. I hadn't slept in over 30 hours, I was angry and resentful and I made up my mind to just leave without having the consult. I had wasted enough of my life waiting around for arrogant doctors. I was about to walk out when the surgeon finally walked in. He was really friendly but I was already out of patience and in full on bitch mode, he never had a chance.
I knew I was coming off as despondent and short but I was tired of doctors. I prepared myself to answer the usual questions and to give the usual explanation of EDS and then he said something I never expected. He had already spoken to an EDS specialist and he felt as though it was best to involve someone who knew more about the disorder than either of us. He thought a fibular free flap was too drastic and then he mentioned how I'm still young and I probably want to be able to wear shorts and dresses. He wanted to wait a few months to see if I could heal more and he thought there may be a possibility that we could use a donor graft. I was completely taken off guard and I burst into tears. I had been so focused on bracing myself for things I didn't want to hear that it never even occurred to me that he might say EXACTLY what I wanted to hear. It was like my mind had completely forgotten how to process good news. I had a million questions but I couldn't stop crying long enough to articulate any of them. I must have looked like a crazy person.
I made an appointment to follow up with him in two months. I don't know if I can trust him or not but he's the first doctor to offer me any hope of getting through this with a minimally invasive approach. He's also the first one to display an understanding of what a huge emotional impact additional surgical scars would have. I made the decision to cancel my scheduled surgery at Jefferson and I've taken the free flap off the table for now. I've always tried to maintain faith that there would be a light at the end of the tunnel but this was the first time I could really picture it. I'm still not comfortable having another surgery at Penn, but I don't have to make any decisions for at least two months and that's a huge weight off my shoulders. I feel like I can finally breathe for the first time in six months.
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
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.