Sometimes Blood is Thinner than Water...

      THIS IS A VERY LONG BLOG, AND IT IS IN DEPTH AND CONTAINS A LOT OF INFORMATIONAL LINKS. (I would ask you actually take the time to look at the links of the things I provided for reference here, so you can get an understanding of what I mean in this post)

     What does a person do when they are sick with a rare, life threatening disease, and their own family won't help with a benefit or setting up a benefit fund at least, because they don't think your illness is that serious because they never heard of it? Already I was accused of lying about being in school (because I have missed a lot of class thanks to a flare up of the MPA), lying about being sick (because there is lack of data on MPA, and it is not out there in awareness like cancer, ms or the like), and being selfish (when there are people with more serious diseases like cancer who need help.) I am just shaking my head. At this point I am unsure where to go with this. My medical expenses are growing, but I can't do what I need to do thanks to insurance not covering these things, and I am behind on bills and living expenses cause I can't work right now. So...I just don't have the things done that could help me live a healthier more productive life, and I don't take the treatments that could help me more because I can't pay for the effects of them. I am always 2-3 bills behind every month, because let's face it...disability payments don't always cover everything.

     Anyway, because of this, I asked my mom if she could help me set up a benefit fund at the credit union so I could have an event to maybe help defray some of these costs incurring, and to open up my options with treatments so I am not so limited by my insurance limitations. I get met with the above excuses. I slightly envy those whose family rallies around them when they are stricken with a terrible disease. You are lucky to have them. Appreciate them because some people don't have that. I will figure something else out I guess. Maybe I will get lucky and some other family will step up and care enough to help out with this. I am just stunned right now. If anyone has suggestions as to how I can get a fund set up so I can move forward with an event for medical and living costs because of this dumb illness, please let me know. My only other option is to sell all of my dad's stuff, but I do not want to do that, to lose the only stuff I have of my dad who is no longer with me. I know he would have stepped up to help me. I wish he was here now so I could talk to someone in the family who would not judge me
    
      Being called selfish because I do not have a disease like cancer...well yeah I have to take chemo for the rest of my life with NO CHANCE of a permanent remission. At least with cancer (most of the time) there is hope, support, awareness, and people understand. Rare diseases such as my Vasculitis (Microscopic Polyangiitis), you are on your own. . Blah sorry for the downer blog, please keep in mind, the only way I can find others with this disease is online, and most are on Facebook  so I blog to them as they have been through it and get it too. I am working on a website where we vascies can meet up and talk about these issues without fear of being judged by others as whiny or downers. We aren't. We just wish people would understand that just because you haven't heard of a disease, it is not less deadly than one you hear of every day. 

     Fact is Cancer is horrible and it can and often does kill. So does vasculitis, which is in my case a systemic disease (meaning any part of my body can become involved. *Think of stave 4 cancer that has spread through the entire body and is shutting down every organ a person has, rendering them unable to function..MPA is like stage 4 cancer* My blood vessels are being attacked daily by my immune system, so they inflame, break, and tear. Some stop working, so blood flow stops going to skin, stomach, kidneys, lungs, brain...etc. I am at risk of spontaneous intestinal bleed outs, spontaneous lung hemorrhages, rapid kidney failure and more.) Both cancer and Vasculitis is treated similar, with harsh chemotherapy drugs, infusions, and high dose steroids. Yes the doses are different, but the amount of time you are on it is longer with disease like mine than with cancer. Cancer there are rotations of treatment. Blocks of weeks or months of treatment before they stop and hopefully it was enough to put that nasty cancer into remission. With MPA, you are on chemo drugs, sometimes for the rest of your life every week, month, or day depending on type (oral, IV, injection, etc). Sometimes there is a thing called plasmapheresis which basically is them filtering your blood out of your body, separating the plasma from your blood and returning the blood to you minus the part that is harming you. Because people do not seem to understand this disease, and how it is as dangerous and horrible as cancer, I am going to put out there some things we go through. And please don't think I am minimizing cancer, I am not. In fact I aim to show that it is wrong to say one disease is worse than another, when, in fact both kinds of disease are horrible and I wish for cures for MPA, Vasculitis, Cancer, and more. I simply just want to show the people who say that "at least I don't have cancer" that my disease is as serious, and I can die from this as well.

EXAMPLES OF TYPES OF DOCTORS PEOPLE WITH VASCULITIS HAVE TO SEE REGULARLY ARE:

• Rheumatologist  
• Infectious Disease (ID) Specialist
• Dermatologist 
• Pulmonologist
• Nephrologist
• Neurologist
• Cardiologist 
• Otolaryngologist/Ear, Nose & Throat Specialist 
• Gastroenterologist  
• Pain Management Specialist 
• Physiatrist/Rehabilitation Physician 
• Reproductive Endocrinologist
• Sleep Specialist 
• Oncologist
• Immunologist
• Hematologist
• Ophthalmologist
• Urologist

This by no means is a definitive list, as there are other specialties and sub specialties for each unique case. For example a Vasculitis patient that has a stroke might need Intensive Care and Occupational Therapy while another might not.

SOME OF THE MEDICATIONS VASCULITIS PATIENTS NEED TO TAKE DAILY/WEEKLY/MONTHLY ARE:

Chemotherapy Agents:

• Methotrexate (MTX)
• Cytoxan (Cyclophosphamide)

Anti-Immune Agents:

• Imuran (Azathioprine) 

 Infusion Medications:

• Rituxan (Rituximab) 

Corticosteroids:

• Prednisone
• Prednisolone

Medications Still Being Tested/Experimented For Vasculitis (But are being used) are:

• Kineret (Anakinra) 
• Remicade (Infliximab)
• Humira (Adalimumab)
• CellCept (Mycophenolate Mofetil) 

Again this is not a complete list of drugs we have to take. There are many other drugs used to treat the symptoms, and complications from each organ problem, medication effect, and more.

PROCEDURES VASCULITIS PATIENTS ENDURE FOR TREATMENT:

• Apheresis (most commonly Plasmapheresis)
• Angioplasty
• Dialysis
• Transplants (Kidney, Lungs, Liver, Eyes, Heart, Skin etc)
• Amputations
• Skin Grafts

Again not a complete list, but these are the most common people face with vasculitis. 

Now for some of the fun things Vasculitis Patients go through (Taken from Hopkins Vasculitis):

The symptoms of vasculitis depend on the particular blood vessels that are involved by the inflammatory process.
Different types of vasculitis involve blood vessels in characteristic locations throughout the body. For example, Giant Cell Arteritis typically involves the medium– to large–sized blood vessels supplying the head and neck, but rarely involves the blood vessels of the kidneys. In contrast, Wegener’s Granulomatosis (GPA) frequently involves the kidneys, very often the lungs, and almost always the upper respiratory tract, but rarely blood vessels to the brain. As depicted in the image below, Buerger’s disease involves the fingers and (toes). Gangrene can result from a profound lack of blood flow.

Different types of vasculitis have characteristic (localized) patterns of blood vessel involvement.  However, vasculitis is a systemic illness. Thus, patients with vasculitis feel sick. They often have fevers, weight loss, fatigue, a rapid pulse, and diffuse aches and pains that are difficult to pinpoint. It has been said that vasculitis is a “hurting disease”, because it is so commonly associated with pain of one type or another: pain from a nerve infarction, pain from insufficient blood to the gastrointestinal tract, pain from skin ulcers. In some cases, however, identifying the source and underlying cause of the pain is extremely challenging.  In addition to these diffuse, poorly–localized “constitutional symptoms”, vasculitis may involve virtually every organ system in the body.

What organ systems may be affected?

• Skin
• Joints
• Lungs
• Kidneys
• Gastrointestinal tract
• Blood
• Sinuses, nose, and ears
• Eyes
• Brain
• Nerve

It is important to note that not every organ system will be affected in every patient. The pattern of organ involvement (and symptoms) is unique to the individual, as well as the type of vasculitis (category).
A variety of rashes, the most classic of which is “palpable purpura” –purplish–red spots, usually found on the legs. These spots can usually be felt by the examiner’s fingertips, hence the descriptor “palpable”. The image below is a classic example of palpable purpura. These lesions result from the leakage of blood into the skin through inflamed, damaged blood vessels. They tend to occur in “crops”.

Repeated bouts of purpura may lead to hyperpigmented areas of the skin, as shown below.

Symptoms range from full–blown arthritis to aches in the joints without obvious swelling (arthralgias).
Pictured below is an example of Henoch-Schönlein purpura: cutaneous vasculitis manifested by palpable purpura and arthritis (note the right ankle swelling). The diagnosis was confirmed by a skin biopsy, with immunofluorescence positive for IgA deposition witin blood vessel walls.

Cough (particularly coughing up blood), shortness of breath, a pneumonia–like appearance to a patient’s chest X–ray, lung “infiltrates”, and the development of cavities in the lungs.
Below is an image of a CAT scan of the lungs of a 73 year–old woman complaining of constitutional symptoms, shortness of breath, and bloody sputum. The patient also had glomerulonephritis, a positive P–ANCA, and antibodies to myeloperoxidase. The diagnosis of microscopic polyangiitis was made.

Eleven days later, as the patient’s symptoms worsened, a chest X–ray confirmed progression of her lung hemorrhage. The X–ray shows fluffy infiltrates in both lungs, representing bleeding from damaged capillaries.

Red blood cells (usually invisible to the naked eye), clumps of red blood cells (known as “casts”, also invisible to the naked eye), and loss of protein in the urine. May lead to renal insufficiency and the requirement of dialysis. Depicted in the figure below is a single glomerulus (the filtering unit of the kidneys; each kidney has approximately 1 million glomeruli). This glomerulus is involved actively by an inflammatory process, particularly evident in the bottom of the figure.

Abdominal pain, bloody diarrhea, perforation of the intestines.

Anemia (low hematocrit or red blood cell count) and/or a slightly elevated white blood cell count.

Chronic sinus congestion and “infections” that persist for longer than they should; hearing loss; inflammation of the nasal septum, sometimes resulting in a perforation or collapse of the bridge of the nose, as shown in the picture below.

May affect either blood vessels to the eyes, causing the sudden loss of vision, or small blood vessels withinthe eyes, leading to retinal problems, thinning of the sclera (the white part of the eyes), inflammation within the eye’s different chambers, and conjunctivitis (“pinkeye”). Pictured below is an example of retinal vasculitis in a patient with systemic lupus erythematosus (lupus). The white areas represent regions of retinal infarction caused by vasculitis.

Headaches, strokes, changes in mental status, difficulty with coordination. Below, a magnetic resonance (MR) imaging study of the brain in central nervous system vasculitis demonstrates an intra–cerebral hemorrhage (bright area).

Shooting pains in the arms and legs, numbness, and asymmetrical weakness (i.e., weakness that involves one side of the body more than the other).


A LIST OF THE DIFFERENT TYPES OF VASCULITIS:

Largest arteries: aorta and major branches:

• Giant Cell Arteritis
• Takayasu's Arteritis
• Aortitis in Cogan's Syndrome
• Aortitis in Spondylarthropathies
• Isolated Aortitis

Medium-sized arteries :

• Kawasaki Disease
• Polyarteritis Nodosa

Small and medium-sized arteries:

• ANCA-Associated Vasculitis
• Granulomatosis with Polyangiitis (former name: Wegener's Granulomatosis)
• Microscopic Polyangiitis (THIS IS THE TYPE I HAVE)
• Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (THIS IS NOT RULED OUT AND A VERY REAL POSSIBILITY) 
• Primary Angiitis of the Central Nervous System    

Small arteries: 

• IgA Vasculitis (Henoch-Schönlein)
• Cuteaneous Leukocytoclastic Angiitis (Vasculitis related to rheumatoid arthritis, systemic lupus erythematosus and Sjögren's syndrome.)
• Cryoglobulinemic vasculitis
• Anti-GBM disease (Goodpasture's)
• Hypersensitivity Vasculitis (Drug-induced vasculitis)

Arteries and veins of various sizes:

• Behcet’s disease
• Relapsing polychondritis

 This is by no means a complete list. There are vasculitis that happens due to viruses, cancers, and other diseases. 

     As you can see, these diseases may be rare, but they are NOT mild. The ARE deadly. And YES they ARE as bad as cancer, but what makes them slightly worse is the fact nobody seems to care or understand them. If you have cancer, your family and friends will be there to support you, throw beniftis, help out. They understand when you can't go anywhere cause you are sick. With vasculitis, what you hear from patients every day (including myself) consist of, it is all in your head, if you just got out and excersized more, at least you don't have cancer, it can't be that bad, you don't look sick, quit feeling sorry for yourself, if it was so dangerous why haven't I heard of it, and you are making a big deal out of nothing. It sucks. It does. So, while I am not trying to compare cancer as a disease to vasculitis as a disease (as they both can kill and you go through hell treating them both), I am comparing people's reactions and willingness to understand and help you. Google Cancer Patient Foundations you get About 293,000,000 results. Google Vasculitis Patient  Foundations you get About 581,000 results. And MOST of them are repeating the same three websites of information. You see my point?

     We as patients NEED to fight this illness. More importantly we all need to do what cancer patients did, what breast cancer patients did, what MS patients, AIDS patients, Autism patients did...we need to educate. Contact your local media and ask if they would do a story on your illness for Vasculitis Awareness Month. Take advantage of rare disease day. Host educational events. Go to health fairs and give out information. Contact clinics and hospitals and ask them to start a support group, or to host an educational symposium. The reason they have all the press is they fought long and hard for it. We need to do the same. If you are a friend or family member of someone with Vasculitis...speak up. Wear the Vasculitis awareness bracelets. Get a vasculitis awareness ribbon for the car. Get a button for your bag or lapel. (ask me how to get them and I will give you directions where to get them) If your loved one asks for help putting on an event, help them. The other disease patients couldn't do it alone. They got help form friends and family, and got backing. If we want the same care about our disease, to save lives, to make ours easier, and so no one else has to be diagnosed via autopsy like in man vasculitis patients cases, we NEED to stand up and be heard. 

     I would like to say that those who do care, thank you. If any of you are willing to help me set up a benefit fund and event so I can cover medical and living costs while I fight for my life, please get a hold of me. You can find my information on my blogger profile, or ask for it on here and I will give it to you. And next time you judge, or say "at least it is not cancer" think about what you are saying. Pinning one disease over another when they are equally deadly and both very hard to get treatment for, you are only causing harm to the individual. Shaming does not make a person heal and trust me we are down enough because our bodies have us trapped. Try picturing your body as a prison that you can't escape. You can't do anything you love any more. Then maybe you will see it. Thanks :)