By Glen Gardner
Many of us DO experience arthralgia, the pain, swelling and inflammation of rheumatoid arthritis (not to be confused with osteoarthritis).
We have had a lot of people complaining of pain in feet and\/or hands due to cryo lately. I’m getting the impression that people are often not sure which is which.
Cryoglobulinemia is clearly and easily described by Meltzer’s triad having core symptoms of arthralgia, purpura, and weakness.
Many of us DO experience arthralgia, the pain, swelling and inflammation of rheumatoid arthritis (not to be confused with osteoarthritis). This very often starts in the feet and ankles. Cryoglobulinemia is a vasculitis, and it can cause circulation problems in the extremities, which also causes pain and swelling. On top of all of that, about 70% of us also have problems with neuropathy, usually in the feet, sometimes the hands as well, but it can happen elsewhere too. (I have neuropathy it the left side of my face, for example). I have had issues with neuropathy in my feet, and that has slowly improved since I have been in remission. Even my face is better and is now mostly just annoying instead of painful.
About 25% of people with cryo have symptoms that are identical to rheumatoid arthritis. In very few cases it also includes erosion of joints, just like severe erosive rheumatoid arthritis. This means that sore, stiff, swollen feet, hands, joints are to be expected for many of us when flaring.
A flare is more complicated than just inflamed joints. There is vasculitis which can cause circulation problems in the hands and feet, and it will cause inflammation and swelling there too. At the same time, the breakdown products of the immune system reaction to cryoglobulins can lead to damage to the myelin coating in the very small nerves in the hands and feet, which results in neuropathy Some of us will experience damage to larger nerves in the lower legs which can result in “foot drop”, a partial paralysis of the foot and lower leg (I have had this before). Fortunately, IF the flare is halted, the myelin coating on the nerves can eventually regenerate and things improve. But if the cryo is not kept in remission, the neuropathy can only get worse, and likely won’t be getting any better. Even if remission is established, the neuropathy will continue for months or even years before it has healed sufficiently for things to improve.
Neuropathy commonly causes a tingling sensation, sometimes sharp pains like a pinprick, or an itchy sensation that is somehow impossible to scratch in the right spot… sometimes one can have odd sensations that are hard to describe. At times it can be downright maddening, and at other times it is not so bad. It varies. Some people with cryoglobulinemia have neuropathy as the only presenting symptom.
Arthralgia… this term is used to describe arthritis-like pain, stiffness, and swelling. Among those who have cryo, some of us never really get this, others of us are plagued by it. Like rheumatoid arthritis, it basically involves stiff, sore, inflamed joints, usually starting in the feet and ankles, and many report that it seems to work its way up the body as a flare worsens. There is always some swelling, and it is always symmetrical… that is, if the left ankle is affected, you can expect that the right ankle will also be affected. One interesting indicator is that when the hands and fingers are affected, the very last joint in the fingers is never affected by the swelling and stiffness.
Vasculitis: You are likely to have a rash when flaring, but some of us never have a rash, at all. But purpura, and echymoses are commonplace, and in some cases, you might have necrotic skin lesions, skin ulcers, and even gangrene that develops rapidly. You can expect to have some swelling in the feet and lower legs at times. It varies a bit, but the basic problem is inflammation of small and medium-sized blood vessels.
With the above in mind, it can be hard to sort out what is causing pain and swelling in the extremities because we can have all of these things going on at the same time. With that in mind, we need to pay attention to our bodies and learn to understand exactly what is paining us.
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!
Cryoglobulins: Understanding the pieces, the pipe and the reason to “Stay Warm”.
Cryoglobulins: As the name implies and science tells us, exposure to cold causes cryoglobulin precipitation, which thickens our blood. But which then reabsorb when our body feels itself warm enough, restoring normal viscosity. Viscosity is important. It affects flow. Think pouring water vs. pouring honey. Thicker blood means reduced flow. Reduced flow means less oxygen available to brain, muscles, organs etc.
The Size of the Blood Vessel Matters: Capillaries and small veins may have normal flow with normal blood but may be too small for “thicker” or cryoglobulin clogged blood to pass through. Because of friction, any pumped fluid in any pipe will be stationary at the inner wall of the pipe, effectively “coating it”. And reducing the bore. This is particularly true of small bore size and liquids like blood which are composed of many different size and shape cells. Also the roughness of the pipe wall will affect build up.
Cryoprecipitation: Cryoglobulins also like to clump together and can block the blood vessel or the pipework. So basically, struggling on without sufficient regard to “warming up opportunities” implies ongoing un-reabsorbed Cryoglobulins and ongoing fatigue, aches and pains, ongoing lining of vessel walls impairing their access to blood and possibly no flow in smaller veins.
The first doctor I saw about having cryoglobulinemia said “Stay Warm.
“Stay warm” is rather under stating the issue I think!
Perhaps he should have said:
“Avoid body heat loss but if that fails a quick overheat followed by a suitable period of comfortable warmth will restore normality and avoid the repercussions of thick blood”!
For those of you saying “ increased pressure gives more flow and what about my high blood pressure?” Blood pressure is mostly controlled by pipework dilating or constricting, not heart rate! This information surprised me too! Ultimately, taking the time and effort to enable Cryoglobulinss to reabsorb might be beneficial in all sorts of ways.
My Personal Experiment: Over the last 18 months I’ve been taking my blood oxygen saturation readings when feeling ill after a cold exposure. They have consistently read quite low compared to when well. And the worse I feel the lower the oxygen saturation, which (to me) confirms the thick blood/slower flow theory. These days I don’t dress to avoid cold exposure, more just to enable a steady body temp that might give my Cryoglobulins a better chance of reabsorbing, rather than being provoked, throughout the whole day.
Just an update about my recent Cryo Flare. I hope this helps others to better understand how Cryo affects some of us. If you missed my post, I shared with the group that my docs agreed that my cryo is in remission as long as I continue my Rituxan Treatments for maintenance. My Cryo test has come back NON-Detected for over a year and my blood work is just SHOCKING to see so many NORMALS. One doc actually had to check the name to see if these were my results because they were so incredible NORMAL.
It’s taken me since 2009 to get to this point. I had many years of fear, depression, anxiety, feelings of isolation and facing what I thought was a death sentence. Once my team of docs, along with myself, started understanding how Cryo works in my body we gradually found out what my triggers are, what the signs of my Cryo becoming active are, how much cold prevention I need to be aware of and what steps to immediately take before things get out of control. We try to be 10 steps ahead of those Cryo Critters. Well maybe not 10.
Now, for this weeks update. I also posted last week about a flare I had on one of our threads when visiting a museum and suddenly the AC came on blasting icy cold and I didn’t have my bag of Emergency clothes with me. My Cold Freezes or Cold Soaks, as Glen Gardner calls them, start with ice stinging, painful ears and eyes. I usually cover them up immediately to see if I can stop the Freeze from going any further. Next, I start getting a headache and feeling like I’m going to fall over or pass out. By this time I know I’m in trouble. I ran out of that place as fast as I could but it was too late. Since I wasn’t home I resorted to covering my mouth and nose and breathing as fast and heavy as I could to help heat up my blood and help to get it flowing. When that didn’t work, I put my coat over my head and my mouth and nose down my shirt to heat up my body. Along with these actions, I started walking as fast as I could. After about 45 minutes I started to feel like I was starting to thaw. This is a very frightening experience even though I’ve had this happen to me many times. It’s on the edge of feeling like I can’t undo what is going on. But when the thaw starts to hit I know I’ll be OK for now. (As a reference to this happening, this is my blood coagulating and reducing its normal flow).
Later that night my rash broke out on both of my legs. I haven’t had a rash showing like this in about 18 months. I’m not really sure how long, to be honest. It was both disappointing and scary. It’s hard to find the happy medium between, believing all is well and not sitting in fear that something might happen! It’s the Cryo Roller Coaster Ride that isn’t so thrilling.
Now the point of all of this is that even though your test comes back Non-Detected, Cryo can STILL be active in your body, so I have discovered the hard way. All the antibodies can’t be picked up in the blood test, so I’m told. Some of the patients in our Cryo support group have told us that their doctors won’t treat them because of this. NOT GOOD! Also, some patients receive results with Trace or 0 % of Cryocrits and also are NOT treated by their physicians because of this result. This is a big issue to get past with your docs because they just don’t know enough about Cryo but who really does. Scary isn’t it? Doctor, Dr. Ferber from Cooper Health Systems in NJ, seems to understand Cryo quit well and has given me back my life. It’s hard to believe that our bodies can make such miraculous recoveries. Of course, I have learned to live with modifications and continued Chemo (Rituxan) treatments but I’m back enjoying life again.
Please do learn to be very aware of your body and what your triggers are. Understand your blood work and all of your test results. Be proactive and assertive with your care. Educate your doctors. It’s our job if you want to learn now to Live With Cyro.
I’m fortunate to have a doc that believes in both Cold Prevention and Treating the Symptoms.
When I went to see my Oncologist, Dr. Ferber today, this is what we observed;
- All my blood work looked better than it did since 2009.
- I am feeling great. No fatigue, no major body pain but some joint and back pain which may or may not be RA or Cryo. I have clear thinking, kidneys are good, heart and lungs are all stable. Neuropathy is the same but I can live with it. I look healthy. Yeah!
All seemed to be good except my voice problems which may or may not be related to Cryo. BUT, my RASH (Purpura) appeared and I experienced a Cold Freeze last week! Roller Coaster here we go again.
Todays Appointment, 1 week after the Flare.
We WERE going to consider cutting back my Rituxan treatments from every other month to every 3 months because I was doing incredible well.
What to Do now that I had this unexpected Flare?
- Do we treat the Symptoms or wait until we see a change in my blood work?
2. If we wait will my kidneys start to decline, will my hemoglobin decline, will I continue to have these Cold Freezes? I’ve already had kidney and heart failure This is a huge concern for us.
3. If we wait, how will I handle the AC with the outside temps getting hotter?
4. It’s obvious that AC is still a problem considering the Cold Freeze of last week.
5. Test is NON DETECTED but obviously enough is present to cause a flare even if it is minor. We both agreed that this was unexpected from my previous experiences with Cryo.
We both agreed that it is in my best interest to still stay with this month’s infusion to hold off any complications that might occur. As you can see our decision was based on Symptoms. Unfortunately, not all doctors agree with treating patients when just symptoms are present. Obviously this is an area where we need to spread as much information as we can to both our doctors and other patients.
Dr. Ferber concluded today with the same statement he has told me over and over again. “Some patients have a high Cryo count and have NO Symptoms and some patients have a NON Detected and have a serious case of Cryo”. I asked him if he knew of any documentation or studies on this and he said there was one from many years ago. I asked him if he would find it for me to share with all of you and to share on our website. He said, “Of course, it is very important that patients receive treatments or Cryo can get out of control and doctors need to know about this information that appears to be contradictory. As soon as I receive the paper I’ll post it for all of you.
Sorry for this long post but I do think it will help some people especially our new Cryo folks. Stay Warm. AC is brutal. Don’t forget to touch your nose in AC. If it’s cold you are too COLD.
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 why 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 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!
Eileen M. Propp, Ph.D. CoFounder
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