Dystonia Awareness Month

As September is Dystonia awareness month, I’ve been sitting back and thinking about the past 3 years; all I can say is holy s*** this has been an adventure. There have been many obstacles I’ve faced, and have beaten the odds more than anyone imagined. Being told numerous of times that I wouldn’t be able to walk again was the fuel to my fire to proof everyone wrong that I will be able to walk again. More importantly, I proved to myself that if you want something badly, you will do whatever it takes to make a dream come true. My dream came true; I am walking on my own. 

This has been one of the hardest journey's I’ve encountered, and I refuse to give up. My motto has always been, "losing is not an option". The jigsaw puzzle is being pieced together, and there is a clearer overview for why my legs became deformed and spastic. I didn’t want to give up finding the root cause, as my gut was screaming something wasn’t right; I knew there was more to the story, and soon the next chapter will begin. This chapter will be focusing on my spinal cord, and how there is a strong believe this is causing the Dystonia.

Fighting to be your own advocate is easier said than done, and I will admit it isn't easy. Throughout my journey living with Dystonia, I have learned how to be my own case manager, and speak up for what I need. It was hard in the beginning, and within time, it became easier. It can be hard to speak up for your personal needs, and it is okay to ask for assistance. Let's be honest, humans in general don't know everything. One of the ways we learn is by asking others who may have expertise about the topic; if they don't know the answer, there is a possibility that individual can steer you in the right direction. There are many forms of support systems; family, friends, and colleagues, are just some of the examples of support systems you can rely on. When times become difficult, use your support team; these individuals will help you navigate the resources you need to be your own best advocate. There was some wise words I have learned, and anyone can implement it in their lives; you are your own best advocate.

My legs 3 years later

For the past few weeks, I‘be been working on a painting reflecting the last three years. This was an emotional, yet empowering piece to conceptualize and bring to life. I think back to my medical journey, as it was a dark time for me the first two years. Most appointments I would leave crying, feeling down and hopeless. When I learned about all of the surgeries I was facing, I felt defeated. I became angry at myself, frustrated with providers, and my guard was up. I lost confidence in myself, and I stopped believing in myself.

I had a difficult time putting into words all the emotions I have been feeling for the past three years, and the best way to express my feelings, was through painting. I found painting as my form of communication and expression.

5 surgeries later, and my lower extremities are looking good. The pins will be removed in less than 2 weeks, and I will begin relearning how to walk, using specialized braces and forearm crutches. Out of state specialists will be joining my medical team, as there has been a high suspicion I was born with a spinal cord disorder called Tethered Cord Syndrome. TCS is when the tissue of the spinal cord is tethered to the tail bone. This is a condition that is closely associated with Spina Bifida, and most of the time, TCS doesn’t show up on imaging.

When I think of TCS, I visualize my spinal cord as a rope, tied to my tail bone, and slowly it’s freying. When i move my back or legs, I feel the rope pulling harder, making my legs extremely spastic. I painted the rope wrapping around my legs to symbolize my spinal cord controlling the spasticity in my legs.

I was referred to a neurosurgeon, who specializes in EDS and Tethered Cord Syndrome; usually EDS is correlated to TCS. At my first appointment, I learned the terms spasticity paralysis. Spasticity paralysis is when specific muscles are continuously over-contracting. Spasticity is caused by damage to the brain or spinal cord. The continuous contractions I experience are involuntary, meaning I can’t control when these occur.

This is just the beginning, and we are making progress finding the root cause. I feel a sense of closure, knowing answers are developing. I’m ready to close this chapter in my life, and begin the next one.

How I called my Doctor ‘Kramer’ During My Surgery

I’ve always believed laughter is the best medicine. To me, laughing helps distract me from the fear and anxiety. I am feeling. I was first diagnosed with Complex Regional Pain Syndrome (CRPS) when I was 11 years old. CRPS is a neurological disorder that affects the sympathetic and autonomic nervous systems. Often CRPS develops in a limb, after a trauma to the body, and it impacts everyone differently. It is categorized as the most painful condition on the McGill Pain Scale; it is rated higher than amputation or having a child naturally.

During my junior year of college, my pain doctor suggested a spinal cord stimulator trial to see if this was a good fit for me. This is an implanted device that sends electric currents to your spinal cord, to help treat certain pain conditions. Any time I undergo a medical procedure, my family would spend the evening with me the night before to keep my spirits up. For some reason, I have always thought my pain doctor resembled the character Kramer from Seinfeld. The night before my surgery, the discussion of my doctor’s resemblance to Kramer entered the dinner conversation. I remember my brother asking me if I had ever seen “The Junior Mint” episode. When I replied that I hadn’t, my brother shook his head in disappointment, and told me I had to watch it that night. Long story short, Jerry and Kramer observe a surgery being done in the hospital’s teaching theater. Kramer drops a junior mint from the viewing gallery, and it lands in the patient’s body.

The next day I had the procedure, and everything went well. I remember waking up in the recovery room and looking over at my dad, who could barely contain his laughter. I asked him what was so funny, and he responded, “You called him Kramer.” I didn’t believe him, and told him he was lying. His next response was the icing on the cake. “No, you called him Kramer, and you told him to not drop a Junior Mint in you.” Let’s just say I woke up pretty quickly from anesthesia realizing I just called my pain doctor Kramer.

When I went back to school after my procedure, I was telling this embarrassing moment to my roommate, and she was in tears laughing. She majored in graphic design, and was always on her laptop working on different branding projects. She was working on something and started laughing. When I asked her what was funny, she told me that I’d see soon. Twenty minutes later, she designed, printed, and put together this white box. On the box was the Junior Mint logo, with pictures of both Kramer, and my surgeon’s face. As we were hysterically laughing, she commented, “You know you are giving this to your doctor.” I told her there was absolutely no way I was bringing that box to him.

Well, the box somehow landed up in my bag, and it came with me to the appointment. I was having an internal panic attack as I had no idea if he would bring up the subject first. The appointment lasted 30 minutes, and I thought I was in the clear as the Kramer discussion didn’t come up. As the doctor was wrapping up, my dad chimed in, and mentioned I had a gift for him. At that moment, I thought I was about to piss myself. I looked over at my doctor and said, “I’m sorry for calling you Kramer, and telling you not to drop a Junior Mint in my back. When I told my roommate this story, she felt it was necessary you had this Junior Mint box to remember this moment.” He looked at me, and started hysterically laughing when he saw the box. His comment was priceless, and something I wasn’t expecting. “That’s what you were trying to say! You kept saying something about Kramer and a Junior Mint, and I had no idea what you were talking about!” Seven years later, I still call him Kramer, and he still has the Junior Mint box I gave him.

What it feels like to sit in pre op once again

As I walk into the pre op room at Faulkner Hospital, I’m greeted by a familiar face who states “onto another surgery?”  I smiled and said “4 surgeries later, were finally tackling my right side”. She is the sweetest secretary, and last time I saw her, we were discussing about a company that will deliver edible raw cookie dough. I was in heaven eating that, and I thank my friend Kerry for sending me such a thoughtful surprise. 

I’m sitting in the same chair I was last time, and everything still smells the same: sterile. I’m looking at my phone, and talking with my friend who accompanied me, and I noticed I felt a sense of ease; my right foot will be tackled in 7 days, and I can close this chapter in my life.

As much as I don’t want to undergo the pain with the recovery, I am ready to get this done. I have seen how far I have come with my left leg, and the pain will be worth it when I’m able to stand on my right foot without my toes being contracted. I often think about how I want to celebrate having my feet again when these surgeries are completed. For me, I want to feel the sand in between my toes. 

Going through surgery is never easy, and I remember being terrified going through this not even a year ago. I had so many thoughts and emotions going through my head, but the biggest thought was “how am I going to cope if the surgeon couldn’t save my foot?” This time around, that concern that was clouding my brain has diminished. Am I scared, absolutely. Who wouldn’t be, as surgery can be nerve wracking. The primary difference between this surgery and my first surgery, is I feel confident. I have faith in both my surgeon and myself that things will go smoothly.  I am going into this surgery knowing I will wake up with my toes. 

It can be hard to open up to others

The last couple of weeks I can tell I’ve been isolating. I always have people around me around the clock, and it’s hard to have alone time. My alone time is at night, and many times I want that time for myself. I want to be independent, but it’s difficult when I need help with basic tasks. I have a hard time opening up and telling others how I truly feel, as I don’t want to be a burden. My health issues is the main thing that is going on in my life, and it gets tiring talking about it. Who wants to hear about ongoing medical issues that may bring your spirits down? There are many things that I have going on that are non medical, and at times, it is difficult embracing these things. 

I have a large support system, but it’s difficult to reach out and say I need help. Some days are easier than others, and I’m doing the best I can to open up. At times, I’m afraid to tell someone how I truly feel, as I’m sensitive to others who may have opposing views. 

Currently, I am trying to be as independent as I can, even if it may not be the safest decision. In my eyes I see it as ‘let me enjoy it until it’s taken away from me’. It’s difficult to transition from walking, to being wheelchair bound for more than 3 months. Yes this is only temporary, but it is still hard for me to accept that I have to do this. 

Art has been my outlet, and I’m trying to push myself daily to do some form of it. I’ve finally gotten back into painting, and I can’t allow myself to take a break from it again. For a period of time, I had lost my drive to create. Lately I will put music on, and paint for 4+ hours at a time. For me, painting is an outlet; I feel completely calm, and I am focused on the process of creating. 

I want to be the best version of myself, and that’s a work in progress. My top priority is to work on loving myself for who I am, and accepting my new norm. There is no quick fix for this , just continuing to do my best everyday. 

Surgery Numbah 5: Getting Closer to My End Goal

The next surgery has been scheduled, and it is hard to explain how I feel; the surgery will be done within under a month, and the surgeon will be focusing on my right foot. The game plan for this surgery, is the same as the left foot, and I am still facing the same chances of amputation. Even though the left foot surgery was successful, the surgeon has made it clear he is unsure if he can make that happen again. The game plan will be to sever the tendons of every toe, remove bone from each toe, and perform a fusion on my left joint where the big toe is. The plastic surgeon will be in the operating room as well, as the surgeons' are keeping a low threshold for amputation. The surgeon has explained if there are issues with the skin, blood flow, and more, he will amputate half of my foot right then and there. Currently I have 3 toes that have been breaking down, to the point skin is missing. I am keeping my spirits up that I will have my toes back, but I am being realistic that there is a good chance I could lose half of my foot.

 The range of motion in my left ankle looks great, and we are all estatic with the progress that has been made. I attend outpatient physical therapy two times a week, and continue at home physical therapy 2-3 times a day. Daily I set myself goals, and I push myself to accomplish them, even on the days I want to curl up in a ball and cry. I am making sure to listen to my body, and allowing myself to rest when my body demands it. Rehabilitating my leg is my full time job, and it is rewarding as well as exhausting. I have found ways to implement physical therapy exercises wherever I am, including walking the entire grocery store several times. I have learned simple errands we take for granted, have become rewarding in my eyes.

I was fitted for a specialized brace, known as an AFO. An AFO is a customized brace that supports limbs that may be weak, or assist limbs that are positioned in abnormal positions due to muscle contractions; currently my ankle is still struggling with muscle contractions, and has a permanent foot drop. I'm at a big risk of my left ankle becoming stuck in a permanent contracture again. If this occurs, amputation will be necessary as I do not have any other options to reverse the damage. When my foot isn't in the AFO splint, my foot flops down and locks up in muscle contractions. To prevent my ankle going back into a fixed contracture, it has been recommended to wear the AFO at all times, with exceptions for showering and physical therapy. What has been difficult is my leg is continuing to fight the brace. My heel is unable to sit properly in the AFO brace, and it continues to slide up and out of the brace. Envision pushing down on the gas pedal in a car, but instead of releasing off the pedal, it is stuck pushing down; that is what my ankle does daily. There have been multiple times my leg has gotten out of the brace, and there has been an incident where my foot was forcing itself out of the brace. During that occurrence, I was unable to put a finger in-between the strap of the brace and my foot/ankle. The surgeon and my physical therapist were notified, and they were confident this was from the dystonia. I met with the brace clinic, and learned that I am running out of options for how to keep my ankle immobilized. I will meet with the brace clinic again next week,  and further adjustments will be made to the brace. My surgeon has referred me to a colleague who specializes in neuromuscular diseases, and this provider was consulted originally for my first left foot surgery; the fun fact about this physician is he was the one to suggest the surgical method to save my foot originally. Botox is being considered again for my right leg, and further discussion will arise about what options I have left for my left leg.

I am overwhelmed with emotions, but there is a relief that I am in the home stretch. It is draining to go through the grieving emotions, but it doesn't feel as intense as a year ago at this time. I am practicing self love and self care, and have been reaching out to family and friends for support. I continuously keep myself busy, between doing work for the RSD association, and creating art. When I start feeling down about the situation, I look at my left foot, and wiggle my toes; I smile every time I see them, as I am lucky to still have them. Making the decision to undergo this extensive surgery again hasn’t been easy. Signing paperwork allowing my surgeon to make the decision to amputate is difficult, but I know he will make the best judgement. When it comes to RSD/CRPS, any surgical intervention that is necessary has to be thought out in depth. The more trauma my body encounters, the chances of the RSD/CRPS spreading increases. Every person who has RSD/CRPS responds differently to medical and holistic approaches, and its crucial to figure out what works for you before undergoing any procedure. For me, I respond well with Ketamine, and medical marijuana; my treatment team is insisting both of these approaches are incorporated in the recovery process, and I was hoping they would prescribed frozen yogurt, and chocolate as well (a girl can dream, right?).  In my eyes, there are to many pros that outweigh the cons, and it is completely worth undergoing this procedure; I am not giving up my goal of being able to walk.

7 day hospitalization, and I have never been so happy to be home

As I am finally laying down on my bed, I can take a deep breath, and snuggle into my own bed. This was a terrifying 7 days, and and some of the admission I don't have recollection due to the amount of narcotics benzodinpines, and muscle relaxers to try and control the pain. What is difficult with CRPS, it tends to not respond to every medication or treatment method, and you have to try every option in hopes for relief.  Often patients are told ‘this is all in your head’, and it can take an emotional toll on you. Growing up I was often told the phrase ‘suck it up and live’, so I often ignored the pain and continued to do the things I loved. CRPS acts like a monster, and only responds to when things are his way. But that is how I think of the disease. It has a mind of it's own, and you have to find the right cocktail to make the CRPS have a nice buzz going to make him happy. What s tricky, is every person responds differently. CRPS is categorized as a neuromuscular disease. My case of CRPS may look different than some of my closest friends as we have similar symptoms, but are affected differently with comorbidity conditions. Think of a snowflake; there isn't an identical one. The same theory applies to CRPS. I will never know what it feels like to be in their shoes, and vice versa. But what is amazing about what you meet someone with a chronic illness, there can is an automatic connection. You may not be friends with them, but you will feel respect for them because you understand that they are fighting a difficult battle.

On Tuesday November  28th, I went for allergy testing, as I was having anphlactic reactions to cherries. I still laugh at the idea of being allergic of being not being able to eat it because it is so random. But for me, it severe, to the point I stop talking. The way you are tested is by a skin test, and I was reassured that there was little risk of going into anaphylactic reaction. Oh haiiii I was that girl that had to be the in the little risk category. The test revealed so many allergies, that my body went into heigh wire not only with an allergy attack which shocks your body. With CRPS its shocks your body to the extreme. Your body is extremely sensitive, especially to trauma. The trauma could be minor, but it could piss off the CRPS monster. It is not uncommon for one to go into a flare, after having multiple traumatic events occur.

I have learned how to identify which symptoms are related to CRPS, and the same with Dystonia, but this was a completely different kind of pain. The pain was going up my left flank, and down my groin and abdominal area. I had been having cramps for a couple of weeks, but I didn't think much of it. By Thursday evening I was hunched over vomiting and having the chills from the pain. I was struggling to urinate, and could feel so much pressure, along with shooting pain that would come and go. I was hunched over, and any time I moved I was in tears. My dad and I went to an urgent care, and they sent us immediately to the local hospital as the physician felt I had a kidney stone.

When I got to the closest ER, we tried to rule out a uti, but I wasn't able to give a sample. A cat scan was done as there was questioning about a kidney stone. The results came back negative, and I was discharged. I emailed my pcp, and got in to see her the next day. After I caught her up with my symptoms, she was concerned about my kidneys, bladder, and spine. She wanted me to be seen in the ER that was affiliated with the partners systems, and she didn't want to wait. She sent me to Faulkner.

When I was at Faulkner, I was greeted by a triage nurse who's voice was familiar. When I saw her face, I realized it was someone I grew up with in first grade, and we danced together. It was a little embarrassing to tell someone you know and haven't seen in years about your medical stuff, but it almost felt comforting when you're trying to remain calm. I'm thankful she was there, and she is truly an amazing human.

It will take time for the effects of the medications to get out of my system, and it has caused some unpleasant side effects that affected my internal organs. I was loosing sensations in areas of my body that was frightening, and this left the doctors nervous that neurosurgery may have to get involved. The doctors knew I was going to be admitted, but there was debate if I should be treated at Brigham and Woman's as I have specialists there. I remember crying as I was frustrated, and just wanted to go home. I remember my friend who was my triage nurse pop into my room right as she was about to head home off her shift. I caught her up with everything as tears are rolling down my eyes. She gave me the biggest hug, and reassured me that she was here if I needed anything.

It was recommended I be transferred to Brigham and Woman's. After being in the ER and having another cat scan performed, and was brought to a room three hours later. When meeting with the specialists, they were confident my SCS cords had migrated, and it was stimulating my abdomen and bladder. The down fall was they do not do work with the manufactuer that makes my specific stimulator. If this is what was occurring, then I was facing neurosurgery to correct the misplaced leads in my spinal cord.  I don't remember much of this stay as I was heavily sedated with every form of oral and IV pain medication. I was unable to void, and would need to be straight cath'd every 6 hours

My pain doctor was contacted and a plan was made to be discharged Monday morning, and I would see him in clinic. As my dad and I listened to the nurse explain the discharge instructions, we were speechless. "If she is continuing to retain urine in 24 hours, go to the emergency room; Just not this hospital. When I was discharged I was retaining to the point of having severe abdominal and back pain. The only comfortable way to lay was in the fetal position. If this is what it feels like to have kids, I am not doing it!

When I was discharged, my dad drove me to my pain doctors office, my pain doctor was confused with what was going on, and why I was hunched over crying. We had explained what had happened at the previous admission/discharge and his response was priceless: "wait they discharged you and you can't pee. How the hell am I going to fix that?" He told us that he received a phone call that I needed an urgent appointment, but there was no communication about the situation. When I usually see him in a good mood and we talk about life, and this is the first time I saw him fuming and flustered about the situation. I've been seeing him since I was 19, and I call him Kramer as he looks like a mix of Kramer from Seinfeld and Al Pacino. Kramer explained that there was no way my leads could have migrated as they have been implanted for 7 years, and the scar tissue healed it to be in the proper places. He felt the hospital discharged me because it wasn't their programing they use, and there was nothing else they could to do to help. This was the best thing that could have been done. I described my symptoms of the severe amount of pain I had in my back,He was concerned that one of the discs was pressing or causing issues with my bladder. He admitted that my back issues were brushed under the rug and something was actually going on. When Kramer looked at my CT scans and reviews, he revealed that I had degenerative disc disease, and two herniated discs at my L4 and L5.  911 was called, and I was shipped off to Beth Israel as we were fearing my spine was compressing my bladder.

When I got to the ER, more tests were performed, and I was admitted. Neurosurgery was consulted and they said my spine wasn't pressing on anything. I had every medication in my system, but the two important medications were never provided for a week; these medications are for my anxiety, as well as my ADHD. When I am not on this meds, it is extremely noticeable; not only do I see a difference, but others can as well. These are two meds that should never be stopped cold turkey, and going through a traumatic event is probably the worst time these medications were stopped. I was a hot mess. I remember being in the bathroom trying to urinate, and if I couldn't I would need to have a foley to help. I called my therapist and bawled my eyes out of how scared I was. My mind was racing endlessly. There was so much unknown, and I was scared shitless. The neurologist that was assigned to my case suggested I should see the same neuropsychiatrst that first diagnosed me with a conversion disorder. This is a sensitive subject, and I have trust issues with doctors after what happened in the past. I have had so many doctors who have not believed my pain is real, and that has left me feeling guarded when I work with providers. Having flashbacks and severe anxiety without your medication is horrible. My head was spinning in every direction, and I felt depleted physically and mentally.

I met with the pain team on Wednesday, and it was agreed to stop any opioid pain medication and muscle relaxer that was being pumped into me. The doctor asked me what I use for pain control at home, and my response was medical marijuana and ketamine. We were talking about how beneficial these forms of treatment can be, and I told him about how much ketamine infusions helped me during college. The hospital did a way with infusions years ago, and it had to be rare circumstances to have it done on an inpatient level. The goal of the infusion was to reset my nervous system, and get it to a manageable level, so I could go home. After the physician spoke with another colleague who works closely with Kramer, it was recommended a 12 hour low dose ketamine infusion be administered. It was a difficult night as I was bombarded with visual and auditory hallucinations, as well as paranoia. The nurses taking care of me were fantastic, and were so kind and compassionate reassuring me I was  okay. The nurses acted very quickly when the side effects were increasing, and the doctor responded as quick as he could. Within minutes I remember a nurse holding my arm reassuring me I was okay as she pushed a sedative to calm me down. I remember crying to her and saying thank you.

When I woke up in the morning, my infusion had a few hours left, and I felt so much better. I was in a complete haze, and the pain was tolerable. The pain doctor came in, and he sat down next to me and we were shooting the shit cracking jokes about the side effects I experienced, and was curious about the hallucinations I had and what was it that triggered them. He explained he expected me to be affected by the side effects, and he talked about how he made a very short clear message warning my nurses that this most likely would happen. I thanked him for making this infusion possible and we suggested that my upcoming surgeries Ketamine continues to be involved for pain management along with non-opoids medications, as I am hyper sensitive to opioids causing internal organs to not work properly or at all. These doctors were truly wonderful, and they both went over and beyond to make sure we could figure out a plan. You could tell from his mannerisms that he cares for his patients and wants to make a difference. After talking for a bit, he had a shit grin on his face wanting to know the details of what I could remember from the hallucinations. He had me hysterically laughing at what I could remember, and it is true when they laughter is the best medicine.

Before I was discharged, one of the pain doctor's asked if I was in counseling, and I was open and told her and her colleague I go weekly. It was recommended I increase my counseling sessions to a couple of times a week, as it may be beneficial to receive some extra support. As each day has passed since I was discharged, I am remembering more of what occurred, as the medication is slowly getting out of my system. It has been quite the rollercoaster, and I'm worn down physically, mentally, and emotionally. Since being discharged, I have been forcing myself to keep busy and moving continuously as I know the minute I stop to rest, the emotions take over, which is usually at night. It has been difficult to accept what I can't control, and learn to adapt, particularly obtaining to my health. I have been doubted for so long that my pain isn’t real, and I at times doubt myself that I’m over exaggerating about the intensity of  the pain. I always wonder how can I suck it up a little more, and I’m realizing I can’t act tough anymore. I will always be strong, but I need to be open with myself and others about my fears with RSD and Dystonia.

I am starting to reach out to others about what has happened, as I know this is the time I need some extra support. My dad, brother, and sister in law were unbelievable during this time, checking on me daily keeping my spirits up, and being by my side through it all. I’m grateful for every person who checked in, even if I couldn’t respond back. Just knowing the amount of support I have, makes me realize I’m not alone in this journey. All the nurses I had were wonderful, and I have so much respect for anyone who is in the health care profession. I'm thankful that the pain team at Beth Israel for all they did, and advocated for what I needed.