The medical world agrees that ‘cold’ causes Cryoglobulins to precipitate within our blood. They also agree that ‘warmth’ enables that precipitate to redissolve. They also agree that Cryo precipitation thickens ones blood and that the precipitate likes to clump together. In the world of pipes and flow, thicker fluids travel more slowly than thinner ones for the same size bore. And muck in fluids tend to coat the pipe inner wall, reducing bore but in the case of our vascular system also denying contact twixt vein inner wall and the blood that helps keep it healthy. Cryoglobulins are not only inclined to clump together but are also ideally shaped for getting snagged somewhere. In the world of our bodies, Cryoprecipitate thickened blood means reduced oxygen-carrying ability. So physically and mentally tired, aches and pains after a cold exposure wouldn’t be a surprise. Nor would mood swings. Or unusual panting after minimal exercise. But while that precipitate is in our system it may be doing other damage. Our vascular system contains some very narrow pipework, very easily blocked. If those Cryoglobulins are loose in our bloodstream, who knows where they might do damage. In defence of cold, the veins in our extremities and skin constrict, further causing problems for thicker than normal blood flow. A short sharp nasty cold exposure can floor me in seconds but it’ll take a day or two of staying comfy warm and avoiding further cold exposure to recover. On that basis, with the demands of daily life it is easy to accumulate a growing presence of undissolved Cryo precipitation which only needs a little more precipitation before something gives. With me that would usually be unconsciousness. So ‘staying warm’ really means either no precipitation going on or that recovery from a previous cold exposure is happening by giving those Cryos a chance to dissolve back into the blood and become harmless again. ‘Cold’ can be as subtle as obvious and is a 24/7 for but the thought of what that precipitate could be doing keeps my motivation high for avoiding cold exposure. Layers and all the rest of the clobber and tricks may be a chore and inconvenient at times but cold exposure means Cryo precipitation and illness. Prevention isn’t infallible but it keeps me well most of the time and holing up for long enough always restores me should prevention fail. Stay warm stay well, get cold get ill is how it works for me. Fitting a life around that is the really tricky bit! Does Cryo thickened blood really carry less oxygen? I decided to test this with a blood oxygen saturation meter. The kind that fit on a fingertip. Lowest reading so far during a particularly awful reaction to cold is under 84% Normally I’m 97- 99%. Stay warm!
For those of you with Cryo and or something else, I only have Cryo secondary to cured Hep C, … this is my experience. Since my HepC (cured) and Cryo diagnosis 10+ years ago I have developed no complications.
I “get cold, become ill, warm up, get better”.
By “get cold” I mean “lose body heat”. It doesn’t need to be cold out for me to lose body heat. It is 25C at the moment and I’m indoors, minimally dressed, healthy and happy. But all that can change by simply turning on a fan for some air flow – it could trash my day. Wind chill isn’t only for winter and gales, it is just as effective in high summer and no breeze.
Only 3 days ago the “shock” of moving from full sun to shade and then staying in the shade for half an hour made me very ill 2 hours later. Ill enough that only this morning, after one totally miserable day and a better one yesterday I’m quite well again. Being fully covered in clothes will largely defeat wind chill as it needs exposed skin to rob heat from. But don’t forget that your lungs are very warm, humid and an ideal situation for body heat loss. Cold air is breathed in, warmed air breathed out. Which means you’ve lost some body heat. And “core” body heat at that! Cold air falls floorwards so be careful about exposed feet, ankles, lower leg.
Body Heat Loss:
I use the phrase body heat loss because my body responds to threats to its temperature control all the time and at sub conscious levels. By the time I’ve associated a little shiver to “am I getting cold?” my body is well on its way to responding as it sees fit. “Become ill” means the onset of aches and pains, can’t think straight, poor humour and unusually massive fatigue after the smallest exertion. If I’ve really over done the “cold” exposure, “feeling like death warmed up with shivering, sweating and grey skin pallor” would be about right. According to my wife, “not a good look”! “Warm up” really means excluding any more body heat loss. Because of the fatigue, a sofa and blanket is all I want and in fact need.
Apart from time of course, Cryoglobulins seem to take much longer to reabsorb than precipitate. So too frequent “cold exposures” and not enough quality recovery/Cryoglobulin reabsorption time between will lead to accumulation of Cryo in the blood and worsening health. “Get better” means back to my usual bouncing 61 year old self. This may only take an hour or two of avoiding further heat loss, sometimes much longer until I’ve no aches, pains or in fact any symptoms. This good health will hold until I next fall foul of sufficient body heat loss that my body responds by precipitating Cryoglobulins in my blood. That threshold of too much body heat loss might be reached by one large, brief dose of cold exposure or an accumulation of small exposures over hours or weeks, summer or winter. Being fully covered in at least one layer of clothes definitely lessens my Cryo susceptibility and helps keep me well.
In my Cryo life, avoiding body heat loss and subsequent precipitation keep me well. Obviously this isn’t bomb proof but warming up with blanket, sofa and enough time always restore me to full health. Another incentive for taking the time to reabsorb Cryoglobulins is that thicker blood has been associated with some nasty health problems. I hope this helps someone, it feels like I’ve written a book!
Is essential cryoglobulinemia real ?
Essential cryoglobulinemia (or idiopathic cryoglobulinemia) is cryoglobulinemia with no apparent cause.
Most of the time, cryoglobulinemia is caused by “something”. Usually it is caused by Hepatitis C. In the Mediterranean region, at least 90% of cases of cryoglobulinemia are associated with Hepatitis C. In the United States, the rate is a bit lower, but has never been clearly defined.
When the association of cryoglobulinemia with Hepatitis C was found, it soon prompted many researchers to declare that cryoglobulinemia is ALWAYS caused by hepatitis C, this assertion is completely without foundation as many persons with non-hep-C cryo do exist.
Today we know that cryoglobulinemia can be caused by other autoimmune disorders, certain cancers and leukemia, MGUS, and a variety of rare, unusual tropical diseases, as well as toxicity from various substances. The list of known causes is long, and growing. In the past, cryoglobulins have been referred to as an “abnormal protein”. it turns out that many people have detectable levels of cryoglobulins but are completely asymptomatic. It may well be that cryoglobulins normally are present in small amounts, in most, or all people. Furthermore there is growing evidence that, in some people things that affect clearance of cryoglobulins may also be a player in cryoglobulinemia…. Cirrhosis caused by a number of different problems such as non-alcoholic fatty liver disease likely have an association with cryoglobulinemia…
Even with that, there still remain a few individuals who have cryoglobulinemia with no apparent cause.
It is very likely that some individuals have a natural tendency to develop cryoglobulinemia, which eventually becomes active when the “right” environmental trigger comes along. It could be a passing virus, or any source of long standing inflammation. It could also simply be a random mistake made by the immune system. It is simply a big unknown, and there is a lot of speculation, and little research on the subject.
The advent of the ability to eradicate hepatitis C brought about a lot of new research into treating cryoglobulinemia. Some gains have been made, but the basic approach to treating cryoglobulinemia remains a “brute force” policy that involves inhibiting the immune system in order to achieve remission.
To-date, little research has been done on essential cryoglobulinemia.The reality is that modern medicine does not know how to identify it, and barely acknowledges it. Yet, people who have it do exist.
In all probability essential cryoglobulinemia is a different disorder than cryoglobulinemia caused by Hepatitis-C, but the medical profession has yet to look into the matter and it does not seem likely that this will change soon.
In the strictest terms, the answer to “is essential cryoglobulinemia real?” is a definite “nobody knows for sure”, but the hard reality that patients with cryoglobulinemia must face is that some of us definitely do have cryoglobulinemia with no apparent cause. Some doctors seem to be aware of this, and others are in denial.
What we need is more research and better advocacy in order to let the medical profession know that we exist, and that we will not be ignored or forgotten.
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 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.
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!
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!
Eileen M. Propp, Ph.D. CoFounder