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Ending Cryoglobulinemia Confusion

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Cryo Confused

I can think of no other experience that brought more confusion into my life than being diagnosed with Cryoglobulinemia. The more questions I asked about my disease, the more questions I found, and very few answers. I wanted to know what to expect and how to be proactive in maintaining my health. Of course I also, hoped for a cure. I had and still have many unexplained symptoms that could be connected to Cryoglobulinemia but none of my doctors were or are willing to make that leap. In fact, I generally have to explain my condition to health care workers and hope I don’t insult their egos in the process. My dream is to find a doctor that knows more than I do about Cryoglobulinemia. I am determined not to give up on that dream.

It’s always important to be a proactive patient and know as much as possible about one’s own body and conditions. In the case of Cryoglobulinemia it is imperative to survival to self-educate. Accomplishing that is not an easy task. There is more information online than there was when I was diagnosed ten years ago but much of it is repetitive and shallow. Practical advice for living with this disease is mostly, unknown to my doctors, at present, and there is little information written about life with Cryoglobulinemia. I know no one else in my community with the disease and there are no local support groups. I remember how overwhelmed and alone I felt being saddled with such a scary diagnosis and unable to find any real answers. The pictures I found on Google of black fingers and toes were downright frightening and I was terrified wondering if that could happen to me. The only advice given me was to stay warm but there were no explanations of what that means for a Cryoglobulinemic.

I am a persistent person driven by a need to understand and some relief from the confusion came when I found an online support group and connected with other Cryoglobulinemics. Fellow sufferers willing to share their experiences became a life-line for me. This is where I learned what the type of Cryoglobulinemia I have could do to my body and what steps I could take to prevent some of the damage. It is the place where I found practical advice on how to live with cryo from day-to-day. I also, found validation and emotional support that makes the burden of my disease easier to bear. I have witnessed how our stories have made an impact (though sometimes it still seems inadequate) on what is known about this disease and the healthcare system’s awareness about Cyroglobulinemia. Patients coming together do make a difference even when we have to join hands around the globe in order to find a few hundred of us. Together we can find better answers and make a difference in the lives of one another. We can bring an end to much of the confusion that comes with the diagnosis of Cryoglobulinemia.

If you are a newly diagnosed Cryoglobulinemic or if you’ve been diagnosed for some time and still feel confused about your disease, the Alliance for Cryoglobulinemia website is the best place to be. The information here is good information from articles, to treatments, to finding doctors, to patient stories and practical advice. Good information and connecting with other patients through a support group goes a long way to lifting the cloud of confusion and empowering Cryoglobulinemics to survive and even thrive.

Validation: Alliance for Cryo Secret Support Group

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secret alliance

Validation is Strength

I am a shy person. When faced with a challenge knowing ‘why’ makes it easier to meet that challenge even if the ‘why’ doesn’t come with a solution. When I was diagnosed with Cryoglobulinemia, that diagnosis became an exception to my rule and left me with a million more unanswered ‘whys’ and no solutions to my many health issues.  The label Cryoglobulinemia came with only one warning: Stay Warm!  My doctor also, told me that cryo had the potential to cause harm in every system of my body and to every organ but he had no idea if it was the underlying cause of my many mysterious health issues or my constant pain. My new diagnosis left me in a state of unknowing fear and dissociation as to why I felt so bad. Even the warning to stay warm was not enough information because staying warm with Cryoglobulinemia is very different from the average idea of staying warm. I was left on my own to become proactive in understanding my rare disease and learning how to take care of myself. Over ten years later, I am still pretty much on my own and still searching for a doctor willing to learn about Cryoglobulinemia.

I have sometimes been criticized for my insatiable quest after the ‘whys’ in life but if it weren’t for that desire, I know I wouldn’t be doing as well as I am and I quite possibly, wouldn’t be alive. Knowledge is power and understanding Cryoglobulinemia is essential for survival. Being frustrated by my doctor’s lack of knowledge sent me digging for information on my own and I soon found that there was very little good information to be found. What I did find was a repeat of basically, three paragraphs that gave me little to no insight into whether my signs and symptoms were related to Cryoglobulinemia or not. The help and information I needed came when I found an online support group and began comparing notes with fellow Cryoglobulinemia sufferers. I met Eileen Propp and she became not only, a source of understanding and emotional support but she gave me the information I needed to associate my symptoms with my autoimmune disease and learn how to be proactive in caring for myself. Others in that group also offered me life-improving insights and the mental/emotional relief that comes with greater understanding. Many years later, Eileen and the members of Alliance for Cryoglobulinemia Secret Support Group members remain as my main source for the information I need to understand my disease. Even now as an un-wanting veteran of Cryoglobulinemia, I would be lost without the strength of validation that I can find only in my fellow Cryoglobulinemia fighters. It isn’t easy to share personal health struggles and it should never be demanded of anyone to do so but I’m very grateful for all who do choose to share. Validation is a sacrificial gift and to all those who have sacrificed to reveal their personal stories, daily struggle with Cryoglobulinemia, I want to say thank you for giving of yourself. You are my heroes and I am forever grateful. In you, I have found some answers to why but mostly, I find the support I need to continue to hope and fight to overcome. There is a great power to be found in the gift of validation.

Make like an Inuit

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make like inuit

Living with Cryoglobulinemia.

I live in the SE of the UK. Fairly temperate. Where snow in winter is rare and in summer maybe high 20’s C. But I’m beginning to understand how people in frozen latitudes survive! It can only be done with 24/7/365 awareness of the dangers around them and for which they prepare. Cold is our common foe. As a Cryo sufferer I too can only maintain a state of wellness by vigilance in not getting exposed to too much cold. Cold which might manifest itself as an almost unnoticed draught indoors, a gentle breeze on a hot summer day or full on winter. Or as a cold drink, ice cream, A/C or the difference in temperature from one room to another.
From a position of being well and able to function normally, one or two small cold washes may not have much initial impact. But without a recovery period, one or two more small cold washes will cause a landslide in my wellness levels. Recovery will take much longer. Days rather than hours.

Wellness means I can run upstairs and down, think straight, have good humor and am pain free. After a Cryo landslide I’m an old man without the energy to roll off the couch to trudge to the kitchen to make a cup of tea. Pain accompanies every move. The Black Cloud of gloom/depression invades my head. I can barely hold a simple conversation, never mind a bit of banter. Panting for hours on end doesn’t help either. It also makes eating a chore.

So I make like an Inuit. Not in furs but certainly with enough layers, head to foot, to keep any chill or draught out. With extra precautions like blankets, scarfs, gloves etc available for emergencies. And I’m always aware of my environment. I balance the probable impact of being outdoors against necessity to be outdoors. But mostly my best survival tactic is ‘Attention to Prevention’. Hoping for a medical solution is valid. Self maintenance is immediate. And much more rewarding than blindly grinding on between Dr. appointments!

Cryo and Arthritis

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Cryo & arthritus

Cryoglobulinemia can cause arthralgia (arthritis-like symptoms), less frequently cryoglobulinemia can cause arthritis which is difficult to distinguish from rheumatoid arthritis This includes the erosive form of rheumatoid arthritis. Just like rheumatoid arthritis, the arthritis caused by cryoglobulinemia requires that rheumatoid factor be present. If you have type II or type III cryoglobulinemia , you are positive for RF, guaranteed… This kind of arthritis differs from the simple arthralgia that some people with cryo experience in that it is always symmetrical (both left and right side joints affected), commonly worse in the morning, and will improve , as the day progresses allowing stiff, sore joints to loosen up a bit. In general terms arthralgia involves joint pain, but not inflammation of the joints. Rheumatoid arthritis involves both pain and inflammation in the joints. Lastly, some people with cryo actually have rheumatoid arthritis AND cryo at the same time! Either way, if you have the arthritis, you can have joint inflammation and a risk for joint damage. You need to talk to your doctor about it. Sometimes milder cases of RA can be treated with aspirin or NSAIDs such as advil or naproxen sodium. In general, forcing the cryo back into remission with steroids or immunosuppressants will also force the arthritis to remit…. but once the joints are damaged, the damage is done and there is no remission from that.

Just remember, that joint pain without swelling, is not likely a significant amount of inflammation. If you are having trouble with RA, then you will most likely have pain, stiffness AND swelling from the inflammation. I have had my ankles and feet affected so badly that I could not get my shoes on. Same deal with my knees, but I don’t put shoes on my knees… tongue emoticon It is always symmetrical, that means that if one knee is hurting, swollen and inflamed, the other knee will be much the same. As far as hands and fingers go, I have problems there too, only one clue that it is RA is that the very last joint toward the end of your fingers is never affected. Basically the symptoms of arthritis caused by cryoglobulinemia are identical to those caused by rheumatoid arthritis.. so I guess we call call it “RA”, even though we might not have RA at all… but then there are some of us who do!

Cryoglobulinemia is my life but Not my Identity

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Rare Disease Day 2016.

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Cold Soaked: Marianne

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Cryoglobulinemia: The Triple Whammy

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Triple whammy

Cryoglobulinemia: The Triple-Whammy!

Cryo has a way of hitting you from three different directions. Usually when we are flaring we only experience one or two of them. But in a big, bad flare it can really hit you hard with all three.

1) Hyperviscosity syndrome: Accumulating cryoglobulins can make your blood a lot thicker if the concentration is high. It can lead to some pretty nasty effects due to circulation being obstructed in the skin, muscles, and various organs, as circulation can rapidly become impaired in the smaller blood vessels. This can cause numerous problems. Hyperviscosity is a particularly onerous problem with type I cryo, but can also happen in types 2, 3, as well as mixed/combined types.

One problem that often occurs with hyperviscosity is damage to the eyes. In particular, the retina and optic nerve. We had a couple of people in this group experience a bad flare with effects on vision likely due to hyperviscosity syndrome last winter. You need to be alert to sudden changes in vision such as blurry vision, flashes of light in your side vision or a sudden increase in “floaters” in your eyes. This is a serious problem that could damage your vision permanently in hours if not treated right away, so be alert.

2) Autoimmune Vasculitis: Cryoglobulinemia is a vasculitis affecting small and medium blood vessels. Cryoglobulins are unusual molecules. They are large, heavy, and in the immune system they act as both antibodies and antigen. Once cryo starts, there is a tendency for it to not stop. The immune system processes the cryoglobulins and in the process a lot of inflammatory agents and autoimmune components are released which cause inflammation in small and medium blood vessels, and vasculitis results. Rashes, skin lesions, skin ulcers, and symptoms similar to rheumatoid arthritis are not uncommon. The process continues, usually until the cryoglobulins are gone or, more commonly, until the immune system is forced into remission and the inflammatory process is halted with medication.

3) Cryoprecipitation: Once the cryoglobulins have been processed by the immune system, the already large molecules tend to link up vis-a-vis a bond facilitated by immune system compliment molecules. Once this happens the cryoglobulins may rapidly begin to precipitate if the skin temperature drops below the core body temperature. This causes even more problems with outright plugging , and vasculitis in medium and small blood vessels. This will continue as long as cryoglobulins are available if the temperature is allowed to remain below the core body temperature. If cryoglobulins are present and you are not actively flaring, it is still possible to have cryoprecipitation.

Cryo and Mortality

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Cryoglobulinemia and Mortality

This is a topic which I have been avoiding to keep from alarming those who have been newly diagnosed without good reason to do so.

After researching this , and thinking about it for some time, I decided that I should talk about this in a post on the forum.

Based on work done by Claudio Ferri (2004) it appears that the mortality rate for persons with cryoglobulinemia are about 42 percent, possibly as high as 50 percent in the first 6 years after diagnosis. Rossa et-al (2008) included the work of Ferri and others to arrive at similar results.

Rieu et-al (2002) examined outcomes for various comorbid conditions with cryoglobulinemia and determined that a more positive outcome was likely in patients who did not have liver, kidney or lung involvement with cryoglobulinemia.

Later work by Bryce et-al (2006) revisited mortality and observed that for all patients in their study, overall the mean 50 percent survival time was about seven years, and that for patients over the age of 57.6 years, the 50 percent survival time was a little better than 5 years.

In all three of these studies, researchers point out that the outcome is likely to be significantly better if there is no involvement of the kidneys, liver, or lungs. Surprisingly, in spite of the seriousness of potential involvement of these organs, the leading single cause of death in cryoglobulemia appears to be vasculitis.

With a cure for hepatitis C now available and with patients getting treated successfully, I would expect to see the 5 year survivor rates improve over the next few years as the number of people having liver involvement with cryoglobulinemia ought to decline markedly. Unless a breakthrough in treatment is achieved, I would expect to see vasculitis continue to be the leading cause of death in persons with cryoglobulinemia.

ADDENDUM: One thing that should be mentioned is that treatment of cryo has improved a lot in the last 2-3 decades. The expected survival of a person with severe cryoglobulinemia would be a matter of a few months, at most, without treatment.

I just added a citation for Rossa (2008). I had used his information but had not cited it.

B Cell and Cryo

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It has been speculated that B cells are living too long due to apoptosis failing, as they age past their pre-programmed expiration date they start producing cryoglobulins. It is very likely more complex than this, but failed apoptosis (programmed cell death) is one cause for B cell proliferation. However it is unclear if the failure is in the message sent to the B cells to die or if they are ignoring it. One clue is that in the case for HEPC patients is that cryoglobulins will frequently contain fragments of HEPC RNA. Cryoglobulins do appear to function as an immune system component, but they basically trap targeted antigens by snaring then in their spider-web-ish structure and ripping them apart under electrostatic tension. It is sort of like the Borg in Star Trek. They tear things apart and incorporate them into their molecular structure. The down side is that cryoglobulins behave a bit like both antigens and antibodies which results in a runaway immune system when the immune system basically attacks itself. I tend to interpret the cryoglobulins as the immune systems last desperate effort to rid itself on a source of long standing inflammation. This is not entirely true but it helps me to visualize it. Also, one thing that I suspect may be true is that in cases of non-HEPC cryo, one may benefit from keeping the immune system suppressed long enough, and deep enough to sometimes be effectively “cured”, or at least permanently in remission. This is also speculative, but the fact that stem cell transplants often “cure” cryo strongly suggests that something along this line is going on.

Vasculitis Patient Powered Research Network.

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The Vasculitis Patient-Powered Research Network is open to ALL United States Cryoglobulinemia patients.  Please Register.

 V-PPRN-Logo-Final-CMYK_clean
The Vasculitis Patient Powered Research Network (V-PPRN) is a collaborative research effort for vasculitis research involving patients as partners in research along side clinicians, researchers, physicians and interested parties.
It is a joint venture of The Vasculitis Foundation and the Vasculitis Clinical Research Consortium. The Vasculitis Foundation is our trusted vasculitis research partner and is the largest private funder of research on vasculitis. The VF collaborate with researchers around the world to fund the most promising studies. We are proud to work with the Vasculitis Clinical Research Consortium to bring you a new vision for vasculitis research with the V-PPRN.
has the ability to collect and securely store patients’ disease experiences, known as patient-reported data, as well as electronic health records to build robust data for innovative vasculitis research. The Network is patient-powered and patients participate as full partners in the work of the V-PPRN from generating a research idea to the summary of results and all the steps in between!

 V-PPRN Mission

The V-PPRN seeks to transform how clinical research in vasculitis is conducted by directly engaging patients, investigators, care providers, and health systems to develop research methods to electronically collect health records and patient-reported data on a large number of patients with various forms of vasculitis.  The goal of the V-PPRN is to conduct high-quality studies that will improve the care and the health of patients with vasculitis by exploring research questions that matter most to patients and advance medical knowledge about vasculitis.

Patient-Engagement in Network Governance

Central to V-PPRN governance structure is the theme of patient leadership, engagement and group consensus throughout all aspects of network design, implementation and governance.  Fully embracing the belief that patients and families should be full partners with investigators and clinicians in research and care, the Co-Principal Investigators include Dr. Peter Merkel, MD, MPH and George Casey, MBA, patient-partner.  The patient-academic-clinician shared leadership model replicates itself at all levels (Steering Committee, Working Groups, V-PPRN Patient Advisory Council) having a mix of stakeholders that include academic health care professionals and patient a