Those of you who know my youngest daughter, Shannon, are aware that she has struggled with her health for the last almost 10 years (half) of her life. In this commentary, you will get only a glimpse of the torment she has undergone and what has lead us, finally, to what we hope will change her life. As a youngster Shannon was a very vibrant and competent athlete, being the ‘top pick’ by coaches in our home town, 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 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 sustain frequent 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. 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 is believed to have started the RSD/CRPS (reflex sympathetic dystrophy/complex regional pain syndrome) that she was diagnosed with in 2005. RSD/CRPS is a chronic pain syndrome characterized by severe and relentless pain. It is a malfunction of the nervous system that causes the nerves to misfire, sending persistent pain signals to the brain. The McGill pain scale ranks CRPS pain higher than that experienced by cancer patients. This diagnosis would be the beginning of years of anguish and additional diagnoses for Shannon, through countless hospitalizations and lengthy recovery periods, more than 20 surgeries, 2 spinal stimulator trials-one ending in septic shock-(the spinal stimulator is a device that is placed adjacent to the spinal cord within the spine to help alleviate pain) , severe complications/adverse reactions and toxicity from medications, pseudo-tumor cerebri (high intracranial pressure that mimics a brain tumor and has left her with damaged eyesight), palinopsia, excruciating contractures in her legs and hips, severe spasms all over and in her body, passing out, dizziness, balance problems with frequent falling, tremors, seizures, numbness in her extremities and other areas, debilitating migraines and high pressure headaches, chest pain, asthma, chronic fatigue, fibromyalgia, sleep disturbances, changes in her hearing (loud internal noises), speech and swallowing, over sensitivity to smells, light and sound, gastro paresis, ulcers, POTS (postural orthostatic tachycardia syndrome), dysautonomia, trigger finger, kidney stones, several port placements and at present a central Hickman line in her chest for IV fluids and medication that she receives daily (she is hooked up to an IV pole up to 8 hours a day), subluxations of various joints (her right shoulder has been dislocated since January of this year from a car accident she and I were in, and needs surgical repair), periods of extreme nausea and vomiting with rapid weight loss, numerous infections with slow healing (including several occurrences of septic shock), depleted immune function, poor circulation and blood clots, malabsorbtion issues, concentration and memory deficits, drop foot, scoliosis, anemia, electrolyte imbalances, chronic appendicitis, multiple cysts on her left fallopian tube, pancreatitis, insulin resistance, osteoporosis and many other personal matters. Chronically, Shannon suffers from some of what are called ‘invisible’ illnesses, meaning you can’t see them…like you can ‘see’ a broken leg so you know someone is hurt just by looking at them. Typically, Shannon will look fine to others, but in reality she is always in pain and feeling very ill and/or depleted. She has been through years of physical, occupational and psychotherapy, and she has spent a length of time at 2 different pain rehabilitation facilities. She has 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 has endured numerous tests and invasive procedures, torturous desensitization therapies, scans, lumbar epidural steroidal injections, frequent hospitalizations- some lasting up to a month at a time, and been seen by various specialists throughout the years, but no satisfactory answers or treatments have ever been offered. She has 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 have not taken the time or had the compassion to truly ‘listen’ or even begin to understand what she has been experiencing on a day to day basis. I have been told by several professional people in the hospital setting (including an ethicist and an insurance advocate) that I should just accept my daughters situation and stop trying to find answers. Shannon has been utterly disrespected and humiliated at a nearby hospital that I took her to for years, accused by some heartless doctors and nurses as ‘wanting’ to be sick and purposely ‘making’ herself sick, seemingly because that is a much easier response than to acknowledge their own lack of a resolution. There have, thankfully, been a handful of people who have always supported Shannon, and genuinely cared for her emotional fulfillment, loving her as a valued and vital human being. Over the last several years, Shannon’s health has 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), but for the most part she is now confined to bed. Shannon’s brother Dustin’s tragic death in 2010 continues to be devastating for her on every level. I am a member of several online support groups with affiliates from all over the world that share a lot of the same types of issues Shannon and I deal with on a daily basis. One group in particular has provided us with invaluable validation and information that has recently directed us to a specific neurosurgeon in Maryland, Dr. Fraser Henderson. In June, Shannon had diagnostic upright, weight-bearing (makes sense right?) MRI and CT scans done of her cervical and lumbar spine , and was then seen by him. We have finally gotten the answers we have long prayed for and that explain perfectly what has been going on in Shannon’s body for all these years. Although, we appreciate and welcome this long awaited revelation, I am so disillusioned by a ’health care’ system that has put more credibility in diagnostic scans that are not 100% accurate and many times have been misinterpreted, rather than authenticating and recognizing Shannon’s symptoms in combination with our family history, all of which point to the diagnosis she has finally received; and all of which we have repeatedly requested and even demanded since the beginning of this nightmare, that they be ruled out. Bear in mind that there was a time not long ago, when many disorders were identified without the use of such scans. The new diagnoses that Shannon is facing are as follows. We believe that the CRPS is actually suggestive of what is likely the root cause of most of the issues Shannon has been dealing with, Ehler’s Danlos Syndrome. EDS is a very serious connective tissue disorder that causes weakness and increased elasticity in the skin, joints, muscles, ligaments, blood vessels and visceral organs. Normal connective tissue supports, connects or separates different types of tissues and organs of the body. As you can imagine, a defect in the formation of connective tissue can wreak havoc, resulting in damage, injuries and serious pain anywhere in the body. There is no cure for EDS, so Shannon faces a future of enduring challenges from this obscure disease. She has also been diagnosed with intermittent pituitary deficiency, temporomandibular joint syndrome, cervical-cranial instability (atlantoaxial rotatory instability), spina-bifida occulta, tethered spinal cord syndrome and Chiari malformation, all of which are linked to EDS. For those of you who would like to read more about the devastation associated with Ehler’s Danlos Syndrome, please go to Ehlers-Danlos National Foundation ednf.org. Awareness most definitely needs to be raised about this under diagnosed condition. Shannon will need at least two very serious surgeries to get her started on the road to recovery, and to hopefully make her vision of living a prolific and contributing life, a reality. Her first surgery is scheduled in Maryland, with Dr. Henderson, on August 20th and will be 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) are constricting the brain stem; and one of her arteries in there (which feeds the brain) actually gets pinched off when she turns her head; also, crowding in this area blocks the flow of her cerebrospinal fluid. This first surgery will involve permanently removing a section of bone at the base of her skull, extracting the cerebellar tonsils, which are herniating into the foramen magnum and in consequence crowding the brain stem; and then fusion of the C1 and C2 vertebrae in her neck to help establish stability. A piece of bone will be extracted and harvested from Shannon’s hip, which will be used in combination with the bone dust from the removed skull bone (he makes a kind of glue) along with screws and plates to fuse her neck. We also expect that additional surgeries will be necessary in the future as instability commonly increases below the previous fusions. Google the brain stem and you’ll get an idea about how critical it is, and how many functions it has in your body, it’s absolutely incredible. I’m sure you can understand why Shannon is just a LIL nervous!!! The second surgery will be scheduled for sometime at least 6 weeks after the initial one, and it will address the tethered spinal cord syndrome. Shannon’s spinal cord is being pulled downward at the lumbar region of her back, and is also partly responsible for the brainstem being pulled down and compressed. It is a recognized fact that tethered cord in the EDS patient is not consistently visible on an MRI scan, because of the abnormal connective tissue in the filum terminale. (I will go into more detail about the tethered cord syndrome as we approach that surgery.) Also, the Ehler’s Danlos Syndrome complicates these and all surgeries, making it extremely imperative to have a doctor like Dr. Henderson, who has studied extensively on these matters, and has generously dedicated many skillful years to their diagnosis and treatment, as well as tirelessly educating others and raising awareness about these ‘not so rare’ conditions. I have created this site to keep those of you who know and love Shannon, in the loop. I will be updating as we go along, posting pictures when possible of Shannon’s journey, and hoping that you will keep her in your heart and in your prayers. Please join me as I envision her life turned around for the better, her days full of happiness, comfort, peace and a simpler existence, her heart full of gratitude for a new easier way of living, her dreams come true (like finishing school), and her gladly sharing and promoting the unique wisdom she has gathered from all these years of hardship. Thank you for loving my Shannon.
Posted on: Tue, 20 Aug 2013 00:34:29 +0000
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