we've got signatures, help us get to 1,000 by January 1, 2014
Hi everyone! First off, if you are taking the time to read this then I would like to send my utmost and sincerest gratitude. This matter is one that is especially personal to me and many others who live with Klippel-Trenaunay Syndrome. Under the "petition" tab, I will explain why it is imperative to lobby for change in regards to the mental health component of KTS at the VAC clinic at Children’s Hospital Boston. If successful in implementing this change, than it will hopefully extend over to other hospitals nationwide that treat KTS.
At birth, I was diagnosed with Klippel-Trenaunay Syndrome. For those of you who are not aware as to what Klippel-Trenaunay Syndrome is, I will offer a semi-brief yet coherent explanation. There are three main characteristics of KTS, including 1) a port-wine stain (red birthmark) 2) abnormal overgrowth of soft tissues and bones & 3) veinous malformations. A port-wine stain can quite often have small red blisters that open and break easily. This is indeed dangerous, as these act as portals for bacteria to enter and cause infection such as cellultis. Cellulitis is quite common among Klippel-patients, as the lymphatic system is not able to clear out the bacteria the way in which a normal, unimpacted lymphatic system would. Therefore, the bacterium becomes stuck and there is lymphedema along with internal bleeding from abnormal blood vessels. One should always seek immediate medical attention if presenting symptoms of one as they can progress quite rapidly. These infections can then evolve into sepsis, an infection of the blood, and eventually lead to death if not treated properly.
While infection is a major threat to Klippel Patients, there are myriads of others, too. Due to deep malformations, there is increased risk of developing a deep vein thrombosis (DVT); this DVT can then travel throughout the bloodstream and land in the lungs. At this point, it becomes a life-threatening clot known as a pulmonary embolism. Often, but not always, Klippel presents itself in one leg-region. Due to the overgrowth of bones and soft tissues beginning in infancy, the impacted limb can feel painful, heavy, and even have reduced movement. Such overgrowth can also allow one limb to grow longer than the other. If not treated within a very specific time period, the patient will be forced to deal with this size discrepancy for the remainder of their life. If one leg were to be longer than the other, this would be a major hindrance to walking properly.
Anyone who has dealt with chronic pain knows that it is indeed a vile force to be reckoned with, as numerous medical journals and patient reports suggest. Did you know that the emotional side-effects of chronic pain can actually make pain worse? Fatigue, anger, depression, anxiety, and stress are now known through research to interact with chronic pain in a series of complex ways. An example of this is the body’s decrease of natural pain killers. Not to mention the fact that relentless pain can suppress the immune system.
A multitude of Klippel patients are faced with the burden of chronic pain; chronic pain is physical discomfort that lasts for at least a period of six months. While infinite chronic pain sufferers in the world are indeed adults, this is not always the case with KTS; a great deal of KTS child patients deal with chronic pain from an extremely tender age. As WebMd states, “chronic pain can be mild or excruciating, episodic or continuous, merely inconvenient or totally incapacitating.” While KTS manifests itself in each patient differently, the vast majority of patients experience a reasonable amount of intrusive chronic pain on a daily basis despite the use of pain meds and other available techniques. Klippel is indeed so remarkably rare and under-researched that Klippel specialists themselves are sometimes unsure of how to treat such unremitting pain. It is vital to note that with chronic pain, signals of such pain remain in the nervous system for weeks, months, or even years
In 2008, Science Daily wrote an article entitled “Chronic Pain Harms the Brain.” Please read an excerpt from the article, below:
In a new study, investigators at Northwestern University's Feinberg School of Medicine have identified a clue that may explain how suffering long-term pain could trigger these other pain-related symptoms.
Researchers found that in a healthy brain all the regions exist in a state of equilibrium. When one region is active, the others quiet down. But in people with chronic pain, a front region of the cortex mostly associated with emotion "never shuts up," said Dante Chialvo, lead author and associate research professor of physiology at the Feinberg School. "The areas that are affected fail to deactivate when they should."
They are stuck on full throttle, wearing out neurons and altering their connections to each other.
What made this study so significant from the others? It was the “first demonstration of brain disturbances in chronic pain patients not directly related to the sensation of pain.”
Please take a look at another excerpt from the article, below:
Chialvo and colleagues used functional magnetic resonance imaging (fMRI) to scan the brains of people with chronic low back pain and a group of pain-free volunteers while both groups were tracking a moving bar on a computer screen. The study showed the pain sufferers performed the task well but "at the expense of using their brain differently than the pain-free group," Chialvo said.
When certain parts of the cortex were activated in the pain-free group, some others were deactivated, maintaining a cooperative equilibrium between the regions. This equilibrium also is known as the resting state network of the brain. In the chronic pain group, however, one of the nodes of this network did not quiet down as it did in the pain-free subjects.
This constant firing of neurons in these regions of the brain could cause permanent damage, Chialvo said. "We know when neurons fire too much they may change their connections with other neurons and or even die because they can't sustain high activity for so long," he explained.
'If you are a chronic pain patient, you have pain 24 hours a day, seven days a week, every minute of your life," Chialvo said. "That permanent perception of pain in your brain makes these areas in your brain continuously active. This continuous dysfunction in the equilibrium of the brain can change the wiring forever and could hurt the brain."
Chialvo hypothesized the subsequent changes in wiring "may make it harder for you to make a decision or be in a good mood to get up in the morning. It could be that pain produces depression and the other reported abnormalities because it disturbs the balance of the brain as a whole."
He said his findings show it is essential to study new approaches to treat patients not just to control their pain but also to evaluate and prevent the dysfunction that may be generated in the brain by the chronic pain.
This study was published Feb. 6, 2008 in The Journal of Neuroscience. So what does that mean for us Klippel Patients? Is all hope lost? According to recent studies, we should not give up hope just yet. Some even go as far as to say that the action we take today could change the way in which we function tomorrow. What kind of action are these researchers talking about? One of these actions is proper meditation, while others include receiving help in the form of cognitive-behavioral therapy.
At 18-years old, I had the pleasure of writing an article for Children Hospital Boston’s own Thrive magazine, in which I detailed parts of my life with KTS. I also had the privilege of filming a three-minute documentary in conjunction with the article. The objective of this project was to show a well-adjusted young adult flourishing in the real world despite the serious complexities of KTS. Inside, however, I was crying for help. Deep down within, depression and anxiety had set in; yet I was too afraid to say anything. After all, the psychological team at Children’s Hospital Boston said I was “the most well-adjusted patient they had ever seen.” Who would want to argue with that? The only reason they said that, however, was because I led them to believe it. I was too frightened to admit that I was indeed collapsing mentally, and the general
psych. team Children’s had sent in knew nothing about this condition in which I permanently suffered with. It was as though I was teaching them, unfortunately. Perhaps had they been more knowledgeable on KTS, they would have seen past my vicious façade and broken down my walls to the point where I felt comfortable discussing my true feelings. There is a bleak certainty that all born with KTS will at some point experience intense feelings of hopelessness and anxiety at some point throughout their lifespan
Later on in the year, after writing the Thrive article, I had a mental collapse due to years of pent up emotional trauma resulting from my KTS. Finding doctors and psychologists who were able to a) accept my insurance and b) successfully treat chronic illness patients with such rare conditions was extremely difficult. The Children’s Psych Team recommended a few “qualified” professionals but many of them were not able to make sense of my condition. At first, I was diagnosed with severe depression and OCD. However, the doctor in whom I saw admitted he had little idea as to how to treat a patient with such a rare condition. My next doctor said the same thing; he too was not sure if he could do me justice given his inexperience with this condition. By this point, I felt so incredibly misplaced within the world and as though my mental state had been compromised permanently. My Klippel pain was worsening, and there seemed to be no doctor who felt competent enough to even treat me.
Refusing to give up, however, I continued on to another doctor who mistakenly diagnosed me with bipolar disorder. My psychologist at the time disagreed with the diagnosis in which I was given, but no antidepressant had helped much thus far so I was willing to take a shot with the mood stabilizers in which the doctor provided. I was provided an extremely high dosage of lithium and another mood stabilizer, which caused me to have the most erratic year of my life emotionally. This particular doctor confused my “acting out” from this condition with the symptoms of an actual bipolar patient. She, like the rest of my previous doctors, had never heard of KTS before. Like most of my sessions with the prior doctors I had seen, I was teaching her about my condition. She was dumbfounded, and admitted that she felt the situation was indeed very complex given the rarity of my condition.
Still, she insisted that I was bipolar. Upon being hospitalized for cellulitis after my new diagnosis of bipolar, many of my doctors were absolutely appalled. “These people are mistaking a young adult trying to adjust to live with a severe chronic illness to that of an actual bipolar patient. She has never displayed any actions symptomatic of bipolar in all her time here, and that has been a lot. She is simply not bipolar.” My other doctors echoed this sentiment, and were not pleased with how my psychological care was being handled. Several of these doctors had known me for at least nine years, some even since birth. I trusted their opinion more than anything, but once again was confused as to where this left my psychological health.
While in hospital, I was weaned of the mood stabilizers but kept on antidepressants to treat my deepening depression and OCD (no one had ever contested the OCD diagnosis). Children’s seemed to be out of ideas, and that is when my Pediatrician’s office stepped in and took control. The social worker at the office found me a wonderful place in which the psychologist and psychiatrist work in unison (albeit after much work and several hours of research). There is open communicated between all three of us, and each of them have a great deal of experience working within several hospitals throughout the country. At last, I feel as though I am being treated properly although I do still struggle greatly with depression and anxiety. These doctors, however, are helping me to implement techniques in my life to deal with this condition in a viable way and are knowledgeable in treating rare chronic illnesses.
The harsh reality is that it took me five years to obtain proper guidance within this field. Throughout that time period I had frequently had thoughts of commiting suicide, and often avoided social situations due to my OCD. Some days, I was so bent out of shape that I was not able to make it off the couch. No one facing such excruciating symptoms from their Klippel should have to endure the emotional struggle in which I did finding proper psychological care.
Since the Thrive article, I received hordes of emails from copious sufferers and their families. It was so delightful to be able to speak with others in similar situations. I then started my first ever KT blog, KT Fabulous, in which I chronicled my past and present with this condition. Again, this brought about a considerable amount of patients and their families to reach out to me, often from a diverse network of backgrounds. Due to serious chronic pain issues and other life stressors, I ended that particular blog. A few months later, however, I had the chance to be a featured speaker at the biennial KTS Conference in Minnesota. Minnesota was undeniably a life-changing experience; being in the same place at the same time with patients, their families, and medical staff was so unbelievably surreal. I remember the day of the conference, the room itself was overflowing with emotion. There was so much said, yet so much left unspoken.
I watched parents of young ones weep as they sat through the various presentations in regards to physical symptoms of KTS and, for the first time ever at a KTS conference, the emotional side effects. The emotional toll in which Klippel can have on a person has been widely ignored universally; as even mental-health professionals are unsure of how to deal with such a rare species of patients. While substantial amounts of mental health networks exist for those suffering with the likes of other diabolical illnesses among the likes of cancer, diabetes, etc., Klippel continues to go unaddressed in this regard.
Shortly following the Minnesota Conference, I started another blog entitled Kisses for KTS. Having now been three years older than when I had worked on the Thriveproject with CHB, I had found a more confident, fearless voice. This voice was not scared to speak of matters concerning mental health matters in relation to chronic illness and, even more specifically, KTS. To date, one of my most popular self-written articles on the blog is “Depression: Dying on the Inside.” There is a reason this article resonated with such a massive amount of sufferers, and that is because they too were going through/ had gone through the same thing.
In all my years interacting with Klippel Patients and their families, I’ve gathered an abundance of knowledge in regards to this condition. Mostly, however, it has been mental health knowledge in which I have gained from speaking to the patients directly. Every patient has suffered emotionally in some way and been unable to cope for a period of time – many have sought help, but were unable to find adequate professionals who understood the propensity of their medical plight (much like myself). In result, many have resorted to self-harming, drugs, eating disorders, and/or a place of mental agony in which their lives are ruled by fear, depression, and even anger.
I asked a prominent figure within the KT Community, Melenne Dickinson Finger (who runs k-t.org), to write something in regards to this petition as she too feels the mental health aspect of Klippel is often ignored. Here is what she had to say:
“Complex disorders like KT/CLVM are best treated using a holistic approach. Multidisciplinary clinics use hematologists, interventional radiologists, surgeons, and other specialties needed to manage these lifelong conditions. It is equally important that they recognize and place emotional health in that first tier of treatment, adding psych services to the core team. Quality of life is immensely important for the chronic patient. The need to educate chronically ill patients to deal with the emotional fallout of their disease is as important as applying the science of medicine to their condition.”
This now brings me to the Vascular Anomalies Center at Children’s Hospital Boston. First off, let me say that this is an incredibly competent, mind-boggling staff that has saved my life several times in the past. Not only do they know their medical material, but they know it well and are experts at their craft. About once a year, a Klippel patient will visit the VAC clinic and meet with several physicians throughout the course of a few hours. This includes, but is not limited to, an interventional radiologist, general surgeon, orthopedic surgeon, dermatologist, and someone from hematology. In total, the VAC team is comprised of 25 employees – all in which are physicians meant to address the physical abnormalities of KTS. Yet, there is not one psychologist on board – if need be, follow this link and look at the VAC staff list for yourself (http://www.childrenshospital.org/centers-and-services/programs/o-_-z/vascular-anomalies-center-program/meet-our-team). To have a psychologist on board familiar with the ins-and-outs of Klippel-Trenaunay Syndrome is imperative to ensure the all-around wellness of those suffering with this condition.
This psychologist would not be meant to take the place of the social worker currently aboard the VAC team. Brooke Corder, said social worker, plays a vital role in helping patients coordinate proper care and other imperative tasks such as accommodating travel for patients who live far away. However, as Mellenee mentioned above, there needs to be a professional solely trained in educating chronically ill patients about how to deal with the fallout of this condition. This professional could help in patients to implement techniques in their daily lives that could help vastly improve their quality of living.
My proposal is to find a psychologist who is well-versed on chronic illness, inform the heck out of them about the ins-and-outs of this condition, and bring them aboard. Since the patient will be meeting with several doctors during their VAC appointment anyways, have them meet with the psychologist. Dependent upon the age or preference, this meeting may be with the patients alone, with the parent(s)/guardian(s) or both.
There is no cure for KTS at present and treatment remains symptomatic. KTS is a progressive disorder, and complications can very much so be life threatening, as mentioned above.
Children’s Hospital Boston was ranked #1 in 7 out of ten specialties by U.S. News & World Report. It is now time for them to step up to the plate and be the pioneers of mass change in which could save several-thousand patients. As a doctor once said to me, “what good is it for a child to be physically healthy if they are so incapacitated by depression that they cannot move out of bed?” For many of us with KTS, this statement hits close to home as we have been widely-ignored over the years by the mental health community ignorant of our situation.
This is not a problem unique to CHB by any means; I have spoken to many from the famed Mayo Clinic and other hospitals of high stature who have noticed the same problem within their own institutions. My hope is that if Children’s takes this exigent step in the right direction, other institutions that treat Klippel will follow suit. As a community of adults well versed in the mental challenges younger children with Klippel will eventually face (if the system remains unchallenged), don’t we have a responsibility to be pro-active? Let’s fight for adequate care together, care that entails being well physically, mentally, and emotionally!
If you would like to join me in helping to bring this change to the VAC clinic at CHB, please sign the petition below and share it on your social media! I cannot do this alone, and to ensure the best all-around health for Klippel patients, this is a step that must be taken to ignite change. – Arianna
“A journey of a thousand miles begins with one small step.” – Old Chinese Proverb quote
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