The Disguised Monster Within

The story of Vanessa

After struggling many years with an unknown autoimmune disease, tons of misdiagnosis, and thousands of dollars spent on treating symptoms, I was finally diagnosed with PBC (Primary Biliary Cirrhosis) and HE (Hepatic Encephlopathy) last year. Not only has getting diagnosed proven to be a nightmare in rural areas, but now even finding experienced medical professionals willing or experienced in the disease is at nightmare in itself. It is a huge misconception that liver disease only effects the liver. Liver disease effects the whole body systemically bringing on new disease daily & challenges. One of the biggest challenges has been the lack of training, experience, & knowledge of HE. Contrary to many beliefs, HE is not a psychiatric illness, but rather the livers inability to remove toxins from the body allowing them to build up in the brain & affect the patient neurologically. Healthcare professionals are not trained in PBC or HE enough. Many do not even know what those acronyms represent nor do they know how to treat the patient or symptoms, or even misdiagnose the patient. HE is the biggest misunderstood side effect of liver disease. It is nothing short of hell for many of us sufferers. Because of its neurological nature, it causes slurring, stuttering, uneven gate or dizziness, loss of memory, forgetfulness, irritability, agitation, rage, abrupt changes in personality & behavior, loss of cognitive ability, unexplained loss of time, unconsciousness and even coma. For a patient suffering from HE, the stigma is ongoing, often misdiagnosed as Bipolar, Mania, Depression, etc… It is very hard to diagnose unless the medical professional is familiar with the disease. It seems medical professionals are ONLY trained to monitor ammonia levels which are NOT a true indicator of symptoms. Clinical symptoms must be assessed along with the patients response to drug treatments such as Xifaxin and Lactulose. Often times, patients are misdiagnosed of having mental illness when in fact they are having an HE episode which can be triggered by stress, dehydration, medications, infections/illnesses etc… Another common misconception is that HE is ONLY occurring in patients with end stage liver disease. This is wrong. Patients with liver disease depending on the type can begin to experience HE immediately. Many years ago, patients with liver disease were often placed in asylums because no one understood and assumed they were crazy. Unlike mental illness or other types of dementia or alzheimers etc, HE is treatable and symptoms clear with treatment. Symptoms also almost immediately disappear upon liver transplant. While there is a wealth of information available through the web, the liver foundation and online support groups, it seems that many patients find themselves isolated and alone with this disease because of the lack of family and friend support mainly due to lack of understanding and research on how to help the patient. Patients suffer even more when medical professional are also unproperly trained to identify the episodes and simply write them off as being stressed, depressed or bipolar. The disease PBC itself only affects approximately 9000 in the USA & over 90% are women while HE can effect anyone with any type of liver disease, but due to the rarity of PBC, finding qualified health care is a monumental, expensive, frustrating feat for even a patient not suffering from HE. Resources are scarcely limited & treatment in rural areas carry even a lower prognosis than those patients living in suburbia areas. After suffering many years, it was myself that advocated my own care, my own research, made my own appointments, my own referrals and demanded further workup. I was told many times, it was ‘all in my head’, i was anxious, depressed, had mental illness etc…I have personally hand delivered liver biopsies, underlined and highlighted to different doctors with all barely reading saying your liver is fine! while indeed it was not! Then after making my own referral, getting a diagnosis, my doctor apologized for her ‘missing’ the diagnosis and thanked me for my relentless efforts. However, she then admitted that the diagnosis and treatment was ‘over her head’ and that she could not help me further & did not have time to research the disease. All the while driving thousands of miles each week in attempt to reach specialists who would know about the disease. The Obama Care initiative is not Universal. It will not allow me to go state to state because many institutions do not accept the types of insurance states have implemented. While the idea of Obamas plan was in our best interest, physicians have figured out that they can simply deny a certain plan and get around the system leaving even more problems with treatment opportunities for me. I have not been able to get out of state to seek specialists at the Mayo Clinics etc because they do not accept my states plan. I was even told I would be denied transplant without certain coverage. There are no cures for this disease; only transplant, There are only a couple medications proven to treat the disease to possibly slow progression, but now pharmaceutical companies have increased the price of these meds in some cases by 60%. Having such a rare disease is nothing short of a nightmare in many cases. It effects everyone differently. Some never have symptoms then others like me live a daily life of challenges with the disease. It is hard being your own advocate as well when HE is symptomatic. There has got to be more training for rare disease, more continuing education requirements for doctors as well as nursing professionals. It is unfortunate that we live in America and have to go through this lack of support while other countries have the availability with them. My concern and my passion for speaking out is because unlike breast cancer, this disease doesn’t have a huge pack of wolves to howl and create awareness. It is the few of us that have to stand up. This disease typically shows occurrences in daughters and sisters. I have two teenage daughters, and I do not want them to have to go through this if they are ever positive for the disease.