Adjusting.

Dealing with EDS has been a constant fight between my mind and my body. My mind wants to do everything a healthy person can do and more. Iv'e always been a crazy workaholic. Working 2-3 jobs at a time since I was 16. Now that I am 21 I am working 3 jobs and with the holiday season I have been working mandatory over time. Working 60+ hours hasn't ever been fun, but I've always been able to suck it up and listen to my mind over my body. After my hip surgery I slowed down a little, but it wasn't until my most recent hand surgery that I realized I need to start listening less to my mind and a little more to my body. I focus so much on trying to be "normal". Not letting my illness control me. Not letting my life be effected by my illness but in reality, of course it is going to change the way I live my life. The only thing I am going to do by listening to my mind instead of my body is cause myself more pain. I push myself to the point of exhaustion and so much pain that the thought of moving brings tears to my eyes. I've had mornings where I know that I have to get up and go to work, but it is so physically painful and my body is so exhausted that I end up staying in bed the entire day instead of working. Iv'e been so stuck on fighting. Fighting the pain. Fighting the exhaustion. Fighting to be normal. Now its time to give in a little. Not worry so much about what people will think of me if I give myself a break. Sometimes you get tired of fighting. Sometimes you have to give up your pride to do whats best for yourself. I am not a "normal" healthy person, but that does not make me weak. It does not mean that I am not strong. It does not mean that I am lazy. It just means that I have to adjust my life and live it the best way that I possibly can.  

Good Days

Today I woke up at 5am to go to work. Normally waking up at 5 and functioning is a task for me. I try and keep my eyes open while I drive to work. I get to my desk and find myself trying everything to keep my eyes open. I feel like this throughout the day. Foggy and exhausted. Not today. Today I woke up and went to work and was alert and awake. Yeah my body was hurting and I had a long day ahead of me but it was the first day in months that I actually felt somewhat normal. With the good days come the bad. Unfortunately the feeling of being normal didn't last the entire day. My body finally gave in to the pain. Although the good day didn't last very long, these are the times that I look forward to. Those few hours of feeling normal is what gets me through the months of bad days. 

A Mothers View

Maile ~ Ehlers Danlos Syndrome

The journey to diagnosis of Ehlers-Danlos Syndrome has been not only long, but extremely frustrating.  As a young child Maile enjoyed participating in a variety of activities. She danced, played soccer and gymnastics. She was very coordinated and unusually flexible. When Maile was 5 she became very ill. She was pasty white, bruised and lethargic. Her pediatrician ordered lab work and diagnosed her with leukemia. She was sent to Primary Children’s Hospital for bone marrow aspiration to further diagnose and specify the type of cancer, and determine treatment options. I remember that day as if it were yesterday. I remember the fear as I sat in the waiting area with other parents and their children. Overcome by emotion I went to the bathroom, shut the stall door and cried. The results of the biopsy came back and proved to be inconclusive. The doctors were unsure, but decided on the diagnosis of Juvenile Rheumatoid Arthritis.  She was prescribed medication to help with inflammation. The pills made her stomach painful, yet did not help with pain. The doctor had no explanation for her reaction to the medication. 

Then came the painful and twisted pinky toe, attributed to arthritis, and unresponsive to prescribed anti-inflammatories, acetaminophen, ibuprofen and ansaids. Maile continued to play soccer, and at the end of each game experienced a great deal of pain and fatigue. She retired to her bed and a heating blanket for the remainder of the day.  Soon to follow, Maile quit gymnastics because of pain and exhaustion. Then came the string of fractures and multiple bruises. Her doctors labeled her as “active and accident prone.”

In middle school her symptoms and pain increased. There were multiple symptoms and multiple doctors. Same response, “you are just weird”. She was unable to sleep, frustrated with medical care and intense pain. As a mother it has been difficult to watch your daughter in pain. Feel helpless, sad, and frustrated. Shoulder surgeries and playing tennis with a dislocated shoulder. Her surgical scars widen and atrophy. She heals slowly. Pain and numbing medication affords no relief. 

While playing on the high school tennis team, Maile suffered a dislocated shoulder. This was an outpatient surgery and she was sent home that same day.  Her pain was so intense we ended up in the emergency room. The doctors were shocked at the amount of pain medication that did not touch the pain. She was visibly in shock. After her second shoulder surgery she was admitted to the hospital.  Again she went into shock due to pain. The anesthesiologist said he had never seen anything like it before. He had given her so much medication and it wasn’t touching the pain. 

Maile had her follow up appointment with Dr. Metcalf. While comparing her surgery shoulder with her nonsurgery shoulder, the supposedly good shoulder dislocated. It was at this point that Dr. Metcalf suspected the injury was due to an underlying problem, a collagen deficiency disorder. We were sent to see a rheumatologist at McKay-Dee Hospital. Dr. Shelby Dames, who was condescending, and accusatory. She to Maile that “yes she was flexible and had hyperextension of her joints, but so do a lot of people”. She proceeded to tell Maile that the pain was in her head and she needed an anti-depressant. It was unbelievable how rude she treated Maile. Needless to say this was devastating. There were no doctor appointments for some time after that. 

Maile’s hip was causing her pain and the quickest appointment she could get was with Dr. Blackham at McKay-Dee Hospital. He barely examined Maile and told her she had snapping hip and referred her to physical therapy. Tres Ferrin, physical therapist at Stewart Rehab identified Maile’s hip dislocation and talked with her about Ehlers-Danlos Syndrome. Maile had an appointment with Dr. Scharmann at McKay-Dee Hospital. He recognized the symptoms and put the pieces together. Maile was diagnosed with Ehler’s Danlos Syndrome. From there we began the process to contact Ehlers Danlos specialists in Seattle Washington, and were put on a wait list. Meanwhile Maile’s hip became so painful it was difficult to walk. She saw Dr. West at TOSH and he recommended surgery to loosen the iliotibial band that was catching on the bone. She came out of anesthetic in shock again. Dr. West listened to us and gave her a muscle relaxant that helped calm her body shakes. 

  

Maile’s hand warranted the next surgery. She saw Dr. Gowski at TOSH for her thumb with joint collapse. Pre-operatively we discussed her reaction to pain post-operatively and her need for a muscle relaxant to help with the shock symptoms. Maile came out of surgery and again was shaking all over and shocky. I explained to the nurse that she needed a muscle relaxant Dr. West ordered. The nurse blew us off and said shaking was very common when coming out of surgery and walked out. She soon returned when the monitor visualized her elevated and climbing heart rate.

In the midst of all of these incidences Maile had stroke like symptoms with loss of eye sight. McKay Dee Emergency Room would not address the urgency and sent her to an ophthalmologist. There was concern of a detached retina and stroke and it wasn’t until I demanded to see the Emergency Department Manager that she was seen. Eight hours later, and no answers she was sent home. 

Maile has had nosebleeds, temporomandibular joint dysfunction, inability to sleep and severe exhaustion. She experiences dizziness, impaired vision, memory loss, speech difficulties and passing out. 

After 3 years waiting to see a geneticist, Maile was seen at Primary Children’s Hospital by a new doctor, Dr. Ball. He spent three hours with us. He listened, he validated, he explained, examined, empathized and understood. Other medical providers that have been amazing are; Dr. Julian Allred, Neurologist at the University of Utah, Darin Stratford, OT at Stewart Rehab, and Sheila Savaiinea, massage therapist.

Maile has an amazingly positive attitude which most definitely affects her health in a positive manner. She lives with pain every day of her life, yet continues to live her life and bless her family and friends with her humor, love and laughter. I am grateful to those that care for her, understand and believe her pain and encourage her to keep moving forward. 

                                                                                                                                     

My Story

When I was younger I used to do Gymnastics, ballet, soccer, basketball and softball. I noticed that I didn't have as much energy as the other kids. I was constantly spraining my ankles and wrists and was always covered in bruises. I ended up quitting everything and just stayed in bed because I was always tired and in pain. Walking became painful. Going to school became painful. Functioning became painful. After many doctor appointments I ended up at Primary Children's. They thought that I had Leukemia. They took bone marrow and tested it. The doctor told me it was not Leukemia and that my blood work didn't make sense and that he had no idea what was going on. We settled for the diagnosis of Juvenile Rheumatoid Arthritis. Once I joined middle school my symptoms became worse. I was constantly in pain and doctors just looked at me and said "I don't know. You're just weird". I never got any answers as to why I was feeling like this. In High School I joined the tennis team. My Freshman year I stepped wrong on the court causing a slipped disc, doctors couldn't explain how simply stepping wrong could cause that. My Sophomore year I hit a ball and dislocated my right shoulder. They tried to pop it back in but it wouldn't stay. I met with multiple surgeons in Ogden and no one knew how to fix it. I finally found Dr. Metcalf at TOSH. He told me that my labrum was shredded and that I needed surgery. I had surgery in Dec 2010. He told me that with normal tennis injuries like this there was less than 10% chance that my shoulder would come out again. I was cleared to play tennis my Junior year after months of physical therapy. I made it through my entire Junior year season with little problems. A few months later I was putting my backpack on and my shoulder popped out through the back. I went back to Metcalf and he told me I would need another surgery and that I would not be able to play tennis after that. I played my senior year with my shoulder dislocated and help together by KT Tape. I got my second surgery in Dec 2012. He drilled in to my bones and put anchors in to provide more stability. Normally after a shoulder surgery you are in a brace for 4-6 weeks. He put me in my brace for 12 weeks because he wanted me to have more stability. During one of my follow up appointments he went to compare my range of motion and ended up popping out my other shoulder. He then realized there was something bigger going on than just a tennis injury. I went and saw a Rheumatologist at Mckay Dee Hospital. Dr. Dames examined my joints and we found that my joints were all loose and hyper extending. She then began to tell me that all of the pain was in my head and that I was just flexible and that I was being a hypochondriac. She tried to put my on an anti depressant. I knew my pain was real. After that I didn't go to doctor appointments for a while. I was tired of being told that I was crazy. That it was all in my head. That I was a depressed hypochondriac. It wasn't until almost a year later that I ended up seeing Tres Ferrin, a physical therapist because my hip was dislocating. He told me that he had read about an illness called Ehlers Danlos and that my symptoms reminded him a lot about it. I went and saw Steve Scharmann and he did the beighton test and I scored 100% on it. That day I was diagnosed with Ehlers Danlos Syndrome. At first I was relieved that I had a diagnosis. I wasn't crazy! I wasn't making it up! But with this diagnosis came very frustrating and complicated doctor appointments because very few doctors know about this illness. I was in and out of the ER for fainting and neurological problems that presented like a stroke, but doctors had no answers. All of these scary things were happening to my body and no one knew why. They all just said "I dont know enough about Ehlers Danlos to know what this could be". It's frustrating because you are supposed to trust doctors with your care. How do you trust someone to care for you when they have to go in the back and google EDS? In Oct of 2015 I had a hip surgery done by Dr. West at Tosh and has a hand fusion done in August of 2016 by Dr. Gowski. While I am still trying to find answers for my neurological problems and while I have run into a lot of doctors that do not know anything about EDS, I have found some amazing people that have helped me. The most important thing to me right now is patient care. More doctos need to be educated. More doctors need to be able to have the education and ability to help people like me, because we cant do this on our own. A huge thanks to all of the amazing people that never doubted me when I said that something was wrong. Thank you for believing me and educating yourself on EDS and helping me manage this illness and live my life the best I can. 
Dr. Ball- Geneticist at the U
Dr. Scharmann-Sports Med at Mckay Dee
Tres Ferrin-Physical Therapist at Mckay Dee
Darin Stratford-OT at Mckay Dee 
Sherri Duft-Physical therapist at Sundance Physical Therapy
Clay Sniteman-Physical therapist at Sundance Physical Therapy 
Sheila Savaiinaea-Massage therapist
Dr. Metcalf-Shoulder surgeon at TOSH
Dr. Hugh West-Hip surgeon at TOSH
Dr. Gowski-Hand surgeon at TOSH

Yesterday I had someone come up to me that noticed I had posted something about EDS and asked what exactly it was and what damage it caused. This is the exact reason I post about EDS. Awareness. I want people to know the name. I want people to know the symptoms. I want people to know the dangers of having this illness. I don't post for attention or sympathy, I post in hopes that people read it. My hope is that some day in the future I will be able to say "I have Ehlers Danlos Syndrome" and I wont get the response "what's that?".

Hand Surgery Update

After spending 1 week in a splint, 6 weeks in a cast, and 3 weeks in a smaller cast I am finally cast free! X rays show that I am not completely fused yet but the hardware does not show any signs of rejection. Still have a lot of pain and swelling and pretty much no function in my thumb but everything surgically looks good. Now its time to be patient and get used to having a fusion.

BEFORE SURGERY 

                                                      AFTER SURGERY

I Am Not Invisible

When people see me they don't know I am sick. They don't see all of my pain and struggles. They see what appears to be a normal healthy 21 year old. When you have an invisible illness you hear things from people like "Well you certainly don't look sick". Although at times I am happy that people cannot tell that I am sick, sometimes it is hard to be doubted and questioned when you do explain to someone that you are sick. To not have your pain, suffering and struggles recognized but questioned is almost unbearable. The feeling of having someone doubt you and tell you that "You can't be that sick if you look completely normal". I shouldn't have to look sick for people to believe me. It makes you feel alone. Not only do I get this from friends, family, co workers and strangers, but I also get it from doctors. Physically I look healthy, doctors see someone that has muliple health issues that don't make sense and an extreme level of pain and they automatically think you are a hypochondriac. I have had multiple doctors tell me that it was all in my head and that I just needed to take an Anti Depressant. I knew my body better than any doctor I saw. I knew there was something wrong, but because physically I appeared normal I was constantly misdiagnosed. I have had medical problems since I was in Kindergarten. I was in and out of the hospital and was just told that I was weird and they didn't know what was wrong with me. I did not get diagnosed until I was 19, after I had already had two shoulder surgeries. I am now 21 years old. I have had 2 shoulder surgeries and have anchors drilled into my bones to keep my shoulder from dislocating. I have had 1 hip surgery where they had to cut into my IT band so that when my hip dislocates the IT band doesn't get caught on the bone. I have had 1 hand surgery where they fused together my bone because my joint collapsed. My illness way be "Invisible" but maybe people aren't paying enough attention. 

How invisible is it really?

I May Not Tell You

Losing Yourself

Having a Chronic Illness or Invisible Illness is a lot harder for people to understand. When you see me you don't see my pain or my struggle. You see my smile not the tears that I am holding back. People say "you don't look sick" or "you are too young to be like this". It makes you feel alone. It makes you feel like a burden to those around you. It makes you feel guilty. Guilty for not being able to do the things you want. You have no control over your body and it is terrifying. The pain doesn't go away. You start off having more good days than bad, and then the good days become less often and you find yourself trying to remember what its like to have good days. What it feels like not to have pain when you walk. Not to be exhausted every moment. You find yourself slipping away from who you used to be. You become the pain. You lose yourself.

Geneticist appointment

For over two years I have been working with my doctor to try and get into a specialist in Seattle. Dr. Ball, who is from Washington and is a geneticist that specializes in EDS came to the University of Utah to work. I got an appointment with him and was "officially" diagnosed with Ehlers Danlos. With EDS there are many different types. I was diagnosed with Classical EDS. Although I already knew my diagnosis, it was different hearing it from a specialist. It made it more real. There was no question that I had EDS and it was like I had to find acceptance in my diagnosis again. He went over all of the possible risks and symptoms with me and was very patient while I explained everything that was going on with my body. If I have learned anything it is that no one knows your body more than you and it is so important to pay attention. After listening to my symptoms he realized I may have more than just the EDS. He thinks there is a very good possibility that I could have MCAS-Mast Cell Activation Syndrome, which can be common in people with EDS. Along with Chiari Malformation and Postural Orthostatic Tachycardia Syndrome. He gave me a few referrals to different specialists and recommended that I get further testing done to make sure I do not have the Vascular EDS. Dr. Ball was amazing. It was such a great experience to speak with someone that knew SO much about EDS.

Mark Madsen Bill

Today I went to the State Capitol and learned how to lobby. With this bill coming up its important to try and talk to as many people as possible. It was an exhausting day but really cool. I'm so happy that we have Sen. Madsen fighting for us and giving us hope. It was really difficult to hear another person who is voting say that he is completely aware of what EDS does to your body and how cannabiss can help EDS patients and so many other people. He said he will still choose to vote against the bill like he did last time because he doesn't want people taking advantage to get a high. He had his mind made up and made it clear that nothing would change that. It's heart breaking to have someone say that to you. This is the only hope some people have. Opiates don't always work on people. If there's the slightest chance at getting any relief from pain why wouldn't someone want that? We aren't taking advantage. We don't want to get high. We want a life. We want relief from pain. We want a quality life. We are patients. Not criminals. We need help. 

Medical Marijuana

As many of you know, I am a firm believer that marijuana should be legalized for medical purposes. Living in Utah has been extremely frustrating lately. I feel like people here are more closed minded compared to other states. We aren't asking to make it legal for everyone to just get high all day, we are asking for help. We are asking for hope. Hope for relief. Relief from this horribly painful and crippling medical condition. I'm not asking for everyone to change their views or how they were raised, I'm asking to being a little more open minded about something that can help someone and their quality of life. It makes my heart hurt that someone actually booked Enedina Stanger because she used marijuana to help her live. I know so many people that sit around at home and smoke marijuana in front of their kids just for fun. And here we are making someone who already has a difficult life even more difficult. She is an amazing person dealing with a horrible illness, this is the last thing she needs. Help me and others get Mark Madsens bill passed this month so that Enedina can come back to Utah and get the help she needs and everyone that is struggling and could benefit from this can get some relief.

Classical EDS

There are many types of EDS. I was diagnosed with Classical EDS. Classical EDS is characterized by highly elastic, soft, and doughy skin; unusual scarring; and loose joints. People who have Classical EDS have smooth, stretchy and fragile skin. They are easily bruised and their wounds often split open and heal slowly. We have abnormal range of motion and are more likely to sublux, dislocate and sprain. It can also cause cardiovascular and neurological issues. Problems also caused by EDS include; bleeding, double jointed, heart murmurs, muscle weakness, muscle pain and physical deformity. I am in the process of being diagnosed with Postural orthostatic tachycardia syndrome (POTS). It is a form a dysautonomia. This causes an irregular heart rate, fatigue, dizziness, headaches, heart palpitations, nausea, exercise intolerance, diminished concentration, shaking, fainting, coldness and pain in the extremities, chest pain and shortness of breath. POTS is definitely not uncommon in EDS patients. The shortness of breath, neurological symptoms, chest pain, fainting and heart palpitations have caused me many visits to the ER in the past year. Living with EDS and the pain and complications that come with it will be my biggest obstacle in life. I have to find a way to fight through the pain, deformities and depression. I wont let EDS win.

Living life like a zebra

For those of you who aren't as familiar with EDS, the zebra is our adopted mascot. When people go to med school they tell their students that when they hear hoofbeats that they need to think of horses not zebras so that they don't misdiagnose people with rare diseases. Zebras seem to get forgotten in the medical field. We are rare. But we still exist. 

How it started

Two years ago I was diagnosed with Ehlers Danlos Syndrome. Growing up I always had weird medical problems that no doctor could ever figure out. I was just called "the medical mystery" or doctors would say "she's just weird, we don't know how to fix her". I was always really frustrated growing up, I knew there was something wrong but no one ever believed me. I was told by doctors that my symptoms didn't make sense and that I was making it up to get attention. When I was in 10th grade I had to get shoulder surgery because my shoulder was dislocated and wouldn't go back in. In 2012 the same shoulder dislocated through the back. I then had 6 anchors drilled into my bones. I had hip problems for years so I finally went to a doctor. He told me he didn't see anything wrong with it and sent me to a physical therapist for snapping hip syndrome. The physical therapist told me he didn't agree with that diagnosis because I wasn't able to do any of the exercises. He said that I reminded him of someone he knew with a rare medical condition called Ehlers Danlos. I had never heard of it. I went home and researched it and it was like all of the puzzle pieces finally came together. I then got a confirmed diagnosis in 2014. At first I was almost relieved. I didn't feel crazy anymore. I always knew something was wrong with me but now the doctors finally knew what it was and knew I wasn't making anything up..not that I didn't enjoy being called a hypochondriac. Then once the relief wore off I fell into a depression. This syndrome is horrible and painful. My joints  slide in and out, my muscles feel like knives are being shoved in them, I have no energy. I can sleep all day and still have no energy. I feel like it started to take over my life. I became someone completely different. I feel like I lost myself through all the pain. The simplest things are so hard:getting out of bed, showering, making it through a day at work. It's hard to imagine living a life filled with pain that no one can stop. My fingers started to become disfigured, I have had two shoulder surgeries and a hip surgery. I fee like I am falling apart everyday. This is not just a syndrome that causes you to be flexible. It is painful, crippling and extremely hard to cope with. Every day I struggle to do normal things. Every day is hard. Every day is a struggle. Every day is a fight against my body.