The Story of Shannon Curley

5/7/93 – 5/31/15

Shannon’s story as told by her mother, Karen Curley:

Shannon Marie Curley was born May 7th, 1993, to my husband Dennis and I.

I was already blessed enough to be able to carry and give birth to two beautiful children, Dustin who was 9 years older, and Stacie who was 6 years older than Shannon. We thought we were finished having children, and, ironically, just weeks before we knew we would have a third, we had given away the last of all of our baby stuff (…. and I was thin again!) And so, for me, realizing that there was something so special about this surprise, about her… I nicknamed her my ‘Angel’.

As a youngster Shannon was a very vibrant and capable athlete, being the ‘top pick’ by coaches in our hometown, happily participating in many sports such as swimming, soccer, hockey, softball, little league baseball, basketball and football. She also enjoyed riding bikes, skateboarding, playing games, fishing, boating, quading and dirt biking, and many other activities with all of her friends; as well as being an ‘A’ student and receiving the president’s award in the sixth grade. She was, however, accident-prone and seemed to struggle with recurrent injuries. She acquired a few undesirable nicknames over the years, because no one could comprehend why she was always getting hurt. The trips to the ER started at a young age and many sprains, breaks, infections, pains, surgeries, etc., would soon become characteristic of her life.

Shannon and her Dad

A baseball injury initiated her right knee pain at about the age of 10, leading to a preliminary diagnosis of ‘condromalacia patellae’ by her primary care pediatrician. She did months of prescribed physical therapy, while continuing to play sports and pushing through her pain, but to no benefit. I found out (fortuitously) that her initial MRI scan had shown 2 masses behind the right knee; and after pursuing that finding, we were referred to a tumor specialist at Yale. The masses turned out to be lymph nodes that were swollen and inflamed, but no one had any idea why. The doctor at Yale surgically removed them, and their proximity to the nerve is what was believed to have started the interminable nerve pain of RSD/CRPS (reflex sympathetic dystrophy/complex regional pain syndrome) that she was diagnosed with in 2005.

The doctor that diagnosed her was the only one in our area who presumably knew anything about RSD at that time; so I felt compelled to bring Shannon to a professional who I had hoped would care for her and guide us through this excruciating nightmare. Within a short period of time, Shannon began showing signs of RSD in the opposite side of her body, in her left wrist. This was back when some doctors believed RSD could only affect one side of the body; (although, I actually did at that time, find evidence online that this was not true.) That pain doctor advised me to put her in a psychiatric facility because he diagnosed her with ‘conversion disorder’ and I was the accomplice with ‘munchausen by proxy’. In other words, she and I were making it happen….. for some sort of remuneration apparently.

We were shocked and devastated. Nothing could have been farther from the truth. I refused to follow his advice and told him I would be finding a new doctor for her. The next day I received a call from him stating that he had turned me over to the Department of Children and Families (DCF) for ‘neglect and abuse’. In retrospect, this was the beginning of the end for Shannon. Our entire family had to bear with this torment for a couple of years on top of everything else, our privacy invaded, many accusations made, labels solicited, threats to take custody of her, and some incidents with a few deceitful people within the DCF system; not to mention we also had the added expense of hiring a lawyer for this whole ordeal. The DCF issue was, however, concluded, the conversion disorder and munchausen diagnoses were dropped, I was absolved of any neglect or abuse, and the judge told DCF that they were making fools of themselves pursuing this case. I remember the judge asking to be quoted as saying, ‘in order of importance on a scale of one to ten (ten being the least important), this case is a 100’!! There was so much emotional damage done to Shannon in the wake of this doctor’s poor judgment. Although, the DCF issue was dropped, the label of ‘conversion disorder’ seemed to follow us everywhere we went in this state. Consequently and regrettably, an accurate and detailed diagnosis wouldn’t come for Shannon until almost ten years later when I took her to Maryland to see Dr. Fraser Henderson.

The RSD/CRPS diagnosis in 2005 would be the beginning of years of anguish, numerous additional diagnoses and persistent debilitating symptoms for Shannon. She would endure countless frequent and drawn-out hospitalizations and lengthy recovery periods, more than 30 surgeries, 2 spinal stimulator trials, severe complications/adverse reactions and toxicity from medications, several episodes of sepsis (and septic shock), relentless pain, contractures in her legs and hips, sleep disturbances, subluxations, loss of consciousness, seizures, gastroparesis, poor immune function, concentration and memory deficits, and on and on. She went through years of physical, occupational and psychological therapies, and she spent a length of time at 2 different pain rehabilitation facilities. She had been in and out of a wheelchair, needed a walker and been on crutches for years. There was a time when I thought I would never see her walk without some kind of assistance again. She endured numerous tests and invasive procedures, torturous desensitization therapies, scans, lumbar epidural steroidal injections, and been seen by various specialists throughout the years, but no satisfactory answers or treatments had ever been offered.

She tolerated the judgment, ridicule and criticisms of peers (also teachers and office personnel) in school as well as some ‘friends’ and even medical professionals who did not take the time or have the compassion to truly ‘listen’ or even begin to understand what she was experiencing on a day to day basis. I was told by several professional people in the hospital setting (including an ethicist and an insurance advocate) that I should just accept my daughter’s situation and stop trying to find answers. Shannon was utterly disrespected and humiliated at a local hospital that I took her to for years, accused by some doctors and nurses as ‘wanting’ to be sick and purposely ‘making’ herself sick, a merciless and effortless response entertaining their own lack of competence or even any inclination to ameliorate.

Shannon and her brother

In June of 2013 I took Shannon to Maryland, where she had diagnostic upright, weight-bearing MRI and CT scans done of her cervical and lumbar spine, and was then seen by Dr. Henderson. We had finally gotten the answers we had long prayed for and that explained perfectly what had been going on in Shannon’s body for so many years. She had exhibited many revealing symptoms, even as a young child that were indicative of EDS (the diagnosis that came too late); and although, we appreciated and welcomed this long awaited revelation, I was so disillusioned by a ’health care’ system that had put so much credibility in diagnostic scans that are not 100% accurate and many times are misinterpreted, rather than authenticating and recognizing Shannon’s symptoms in combination with our family history, all of which pointed to the diagnosis she had finally received; and all of which we had repeatedly requested and even demanded since the beginning of this hell, that they be ruled out.

WE knew for years that Shannon had a tethered cord…. and chiari….and high intracranial pressure…. but we were thoughtlessly disregarded over and over again. This is the effect of medical professionals projecting their own lack of love and compassion, and even divulging their proclivity for some sort of dominion, onto the people who are at their mercy. There were few doctors who ever said such wonderful things like, ‘you know your body better than anyone else’, or that gave Shannon and I any credit for OUR expertise living this experience from the inside. Our very worthy perspective was shrugged off subsidiary to judgements made, which were characteristic of the illusion of understanding a circumstance you have not lived. You can be formally educated all you want, but until you have ‘lived’ through something like this, please, don’t impersonate someone who ‘knows’.

Shannon was given several new diagnoses as a result of our trip to Maryland. We came to believe that the CRPS was actually suggestive of what was likely the root cause of most of the issues Shannon had been dealing with – Ehler’s Danlos Syndrome. There is no cure for EDS, so Shannon still faced a future of enduring challenges from this obscure disease. She was also diagnosed with intermittent pituitary deficiency, cervical-cranial instability (atlantoaxial rotatory instability), spina-bifida occulta, tethered spinal cord syndrome and Chiari malformation, all of which are linked to EDS.

In August of 2013 Shannon underwent a decompression and fusion surgery to address the Chiari malformation and volatility in her cervical spine. Dr. Henderson states, “remember that every function in the brain, every fiber in the brain, is modulated through the brain stem. And Chiari, ultimately, is a disorder of the brain stem.” The Chiari along with the cervical-cranial instability (and the tethered cord) were constricting her brain stem. Shannon had the tethered cord release surgery shortly thereafter, but she had been tethered for so long that there was permanent damage done to her spinal cord. Later that year, she would also have a peritoneal (VP) shunt placed for extremely elevated intracranial pressure.

Over the last several years of Shannon’s life, her health deteriorated with even further complications, to the point of facing tremendous exhaustion, discouragement, frustration, apprehension and sheer lonesomeness, with infrequent spurts of energy enabling her to participate in some events (always paying for it later, however). In 2015 Shannon decided she wanted to try more surgeries back in Maryland. Unfortunately, a month and a half after we got to Maryland for testing and appointments, etc., she had a stroke in the beginning of March, the day before my birthday; and she had to be put into an induced coma for intractable dystonias. She would spend the last 3 months of her life in the hospital down there, in and out of comas three times and withstanding more surgeries; but it was ultimately too much for her. Her previous misdiagnosis, and this negligence on the part of the medical community, had complicated things tremendously; and the toll it took on her body from so many years of interminable agony and heartache, was just too much for her to bear anymore. Shannon and I talked a lot about her illness, its ramifications and even her death throughout the last years of her life; and even though she was setback and devastated by the tragic death of her brother, Dustin, in 2010, she fought even harder to live because she didn’t want our family to be torn apart any more than it already was. She was a trooper, and she survived for much longer than I can even wrap my head around. She hung in there for twelve years from the time she was first tormented by physically painful symptoms in elementary school.

I admit, I have my crushing instances of feeling like Shannon was unquestionably ‘shit on’ by life. It was all so unbearable. She had ‘moments’ of quality in her life, and she couldn’t do most of the things that the rest of us just take for granted… simple things. She deeply wanted to finish school, have a job, have children. She wanted friendships and to be included in some way; she wanted to feel loved and lovable. She didn’t want her mom taking care of her, even though she genuinely appreciated my support.

I want people to commemorate my Shannon for the legacy she has so unwittingly left behind. She helped put EDS on the map with her years of suffering, in contribution to raising awareness, to supporting discovering more effective and appropriate ways to diagnose and treat this disease. Shannon had a lot of wisdom for her young age; she loved music and she wrote and spoke beautifully. She was a warrior and a collaborator; she could be dynamic, and she wasn’t afraid to speak up or call you out on your nonsense. She had a charming and generous heart of gold. She was talented and creative. She was musically inclined, playing the clarinet for four years. She loved doing crafty things, and had a passion for watching movies. Summer vacations up in New Hampshire with family and friends were her most favorite past times. She loved staying in hotels even though there were times when she wasn’t able to even leave the room. If we were at the beach, I would open the slider so she could hear and smell the ocean, which she loved. She adored children and they were drawn to her. She had an affectionate weakness for all animals, and their conscious and benevolent treatment. She rescued our little Ronan, a male white terrier who is still keeping us company today. She was a natural born comedian and a great friend to those she loved. She had soft velvet skin and a lovely smile. She was awe-inspiring, encouraged greatness and even fostered strength in those who suffer from the same disease.

Shannon and her sister

If she had had her health, I can just imagine what she would have mastered in this life. She made a difference even in the short 22 years that she was here. I am hopeful that her story will benefit and incentivize breakthroughs in the subject of EDS and other chronic invisible illnesses. I honor and respect my sweet Shannon for courageously battling this disease and hanging in there for much longer than she ever thought she could. I acknowledge and bless her for donating her brain to research on this disease; and I am grateful for the privilege of having had her in my care, and for the pleasure of being her mom and best friend. She is most definitely one of my heroes, having taught me so powerfully, how to become a better person. Dennis, Stacie and I miss her deeply, though there is some comfort in knowing she is no longer suffering, and she accompanies my son in paradise.

We love you so much, my sweet Shannon Marie, and we thank God for you and all you have done to promote excellence in this world. It is clear to me now, why I nicknamed you my ‘Angel’.

I love you forever my sweet.