Winter is here, so stay warm!

Winter is here, so stay warm!     If I may resort to somewhat dark, but relevant humor…

Fall and Winter are the time of year when the people with cryoglobulinemia come out, turn blue, and consume lots of prednisone!  It is getting cooler every day here in Maryland, and today I see the first fallen leaves laying on the ground. For those of us who have cryoglobulinemia it means that many of us have to make some lifestyle changes to get through the winter months without getting sick.               

STAY WARM.

  1. Don’t get chilled.
  2. Limit the time you spend exposed to cooler temperatures.
  3. If you do get chilled, warm up as soon as possible.  See my blog post on what to do if you get cold soaked.

Here are some helpful ideas;

  • Dress in layers
  • Wear gloves
  • Wear a hat
  • Wear insulated underwear.  Also called long underwear.
    • Get the wicking kind if possible to keep you dry.
  • Wear well-insulated shoes or boots.
    • I have some nice felt insulated shoes that I like to wear in the colder months, and some heavy duty felt insulated boots for extreme cold.
  • At work and at home, I sometimes use an electric heated foot pad.
    • It is made by a company called “Cozy” and a search for “heated foot pad” will find it online. These heated foot pads are absolutely wonderful and really take the chill away for me.

In the fall I wear a leather jacket and sometimes wear inexpensive brown flannel gloves. In the winter months, I have a down insulated parka made by Mountain Hardwear, felt insulated boots, and some very nice gloves made by outdoor research… all are hard weather gear meant for hiking or climbing in arctic conditions, and they keep me nice and snug on the coldest of days.     Whatever you do, stay warm!

How I know do I know if a flare is coming?

How I know do I know if a flare is coming???

One common characteristic of cryoglobulinemia is that of periods of remission followed by periods of flaring.

Very often, if one can spot a flare coming early enough and treat it early, many if the damaging effects and a lot of suffering can be avoided by forcing it back into remission with medication (usually high dose prednisone) before the situation gets out of hand.  I am going to outline here what I have experienced with cryo in the hope that it will benefit others. Please bear in mind that each person is a bit different, and that not everyone experiences the same set of symptoms with cryo.

The very first symptoms that I usually get before a flare starts are malaise, myalgia, and fatigue.

Malaise is a general feeling of “not well”. It can be subtle , this feeling will usually persist for a couple of weeks before strong, overt symptoms appear.

Myalgia is muscle weakness. This is generally due to muscles deteriorating due to vasculitis. My blood work might show an elevated C reactive protein and an elevated erythrocyte sedimentation rate at this time. If the muscle loss is significant, I might begin to experience rapid weight loss. Achy muscles are sometimes a problem. I sometimes end up with a few splinter lesions under my fingernails as an early warning that I might flare soon.

Fatigue starts gradually. I feel tired. A little extra sleep is sometimes needed , and I tire easily.


After a couple of weeks of these rather subtle symptoms I begin experiencing mild arthralgia… Achy rheumatoid arthritis-like symptoms, particularly in the morning. Typically my knees and feet are affected first and I am generally a lot stiffer for the first couple of hours in the morning, and limber up a bit later in the day. The swelling and joint pain are always symmetrical, affecting the left and right joints more or less equally, and seems to start at the lower part of the body and works its way up as the situation worsens.

After a few days of mild arthralgia I suddenly find myself faced with a full blown flare and often have severe RA symptoms to the extent that I can not walk or stand without a cane. Sometimes my hands are so stiff and sore that I can’t use them for a while in the mornings. Swelling in my feet and legs can be so bad that my shoes no longer fit. I really need to begin treating my flare with high dose prednisone by this time, in order to avoid further damage to my body. At this point , I begin seeing a few petiachae on my feet, and sometimes my hands, I may develop palpable purpura , or necrotic lesions with dry gangrene. I sometimes experience raynauds’s phenomena, and on one occasion had a remarkably vivid display of levido reticularis. I sometimes develop skin ulcers which take months to heal. Neuropathy seems to be more intense at this time. Treating at this point is a little late, because a lot of damage has been done by the time remission can be reestablished. But treatment is still necessary because in my case the cryo has never remitted on its own. It just gets worse until it is treated.

The thing that is happening is that cryoglobulins are rapidly building up in my blood and the immune system breakdown products causing the vaculitis are increasing. If I wait too long to treat my flare, the cryoglobulin levels will have risen to the point that the vasculitis becomes a process that will continue until all the undesired “wreckage” of immune system components are cleared from my body…. this can take a while, even if I have already started on high dose prednisone to halt the flare. But, if I treat the flare early, I can reduce the amount of damage and misery by quite a lot.


NOTE: Not everyone with cryo experiences symptoms of rheumatoid arthritis, or lesions, skin ulcers, or even a rash. in some cases the sole presenting symptom is neuropathy, while other people only experience a mild rash. It all varies from one individual to the next, so become familiar with your cryo (I am sure you already are) so that you can spot these trends early and hopefully find medical help to halt the flare before it does much damage.

ADDENDUM: The problem with the “prodrome” that leads into a flare is that it is subtle, and unreliable. Sometimes I’ll feel a little tired and achy for a few days, and it just goes away.. no flare happens. I have also had no real prodrome, but have gone straight into a big flare, with little to no warning.
Usually I do have early warning symptoms, and I need to stay alert and aware of my condition so that I can treat the flare as soon as it escalates into a full blown flare… When it does start to flare “big” it is sudden, and it frequently escalates within an hour. A common scenario is that I might be sitting in a chair working at the computer for a while, then discover (when I try to get up), that I am flaring. This frequently happens in the evening for whatever reason… and when it does, I keep my phone and my cane next to the bed in case I discover that I am very ill when I wake up on the morning. More than once I have discovered that I could not stand or walk without my cane when getting up in the morning. Fortunately, I usually loosen up a bit after a couple of hours.

Cold Soaked? Seven Ways to Warm Up.

When it comes to cold weather, if you have cryoglobulinemia, less exposure to cold is best. For many people with type I cryo, their reaction to cold can be immediate and very strong, so great care must be taken to minimize cold exposure. For those of us with type II and type III cryoglobulins it often takes a little longer for us to react to cold, but we still do.. and it often does not matter which type you have when you can eventually reach a point where it seems that any temperature is equally “bad”.

For me, my first clue that I have overexposed myself to cold is usually a mild petichial rash, or raynauds, or levido reticularis on my extremities… usually on my hands or feet. Another clue is that I might suddenly find myself experiencing symptoms of rheumatoid arthritis, particularly stiff and sore knees. At this point ,I know I have done myself harm, and that I need to act right away to recover.

Everyone is a little different.. you are going to have to try some of these ideas to decide what works for you.

This is how I cope with it;

1) GET INDOORS AND WARM UP RIGHT AWAY! For me, I usually do not react to cold instantly, and reacting to cold seems to be the result of the total dose of cold over time. So I need to avoid getting deeply chilled and to warm up fast when I do.

2) sometimes it is a good idea to lay down , and get horizontal , to keep cryoglobulins from pooling in your legs and buttocks after long periods of sitting or standing. Sometimes an hour or so of getting horizontal with my feet elevated a little is helpful for me and will reduce pain and swelling in my feet/legs a bit..

3) If you are sitting, elevate your feet, and use a heated foot pad. This often makes me feel a lot more comfortable, reduces pain in stiff and sore feet and ankles, reduces swelling in my feet and lower legs a bit, and seems to help reduce skin damage from lesions and ulcers a little.
If you have sensorimotor neuropathy, your sense of pain might not be all too accurate, so be careful when using a heat pad to keep your feet warm.

4) If you can, get up and move around once in a while. If you just sit or stay in bed, and you are of the type who has rheumatoid arthritis , or symptoms like RA, staying immobile can be a bad idea. If you feel up to it, get up and move around once in a while to keep your joints limber… usually arthralgia will improve once you start moving around a bit in the morning. It is almost always worse in the AM, but often improves in an hour or two. Sometimes in the evening things can suddenly turn worse. You sort of need to learn when it is a good idea to stop and rest and when it is a good idea to push ahead against all odds.

5) If you are the type who develops palpable purpura or necrotic skin lesions rather easily, you might find it helpful to avoid clothing that puts pressure on your skin, such as tight-fitting socks and pants. I have learned the hard way that sometimes I am better off with no socks on my feet and fewer lesions than with socks and more lesions… I also found that at these times I can easily develop lesions on my skin by simply abrading the skin or by just bumping into things… so be careful and adjust your clothing and your behavior to your needs.

6) if you suddenly find yourself headed into a major flare you might need to call your doctor. For some of us a flare is mostly just discomfort, and for others of us it can be life threatening, so be alert to your condition and act on it early.

7) and lastly, STAY WARM!, dress in layers, use a heat pad to keep your feet warm! Keep your core body temperature up to reduce the possibility of cryoglobulins affecting internal organs.

Three Core Cryo Symptoms: Meltzer’s Triad

Meltzer’s Triad

In 1956 Meltzer and Franklin described arthralgia, purpura and weakness as the three core symptoms of essential cryoglobulinemia. These are generally seen in people who have type II and type III cryoglobulinemia. It is seen in 25-30% of patients with cryo. I just thought I’d share this.

Cryo Symptoms: Points to Consider

Symptoms of Cryo: Points to Consider

Sometimes cryoglobulinemia will present with very few symptoms externally, before you have a full-blown flare. I have listed of a few of the more subtle things, and some of the weird things that have happened to me as symptoms some sort of serve as a warning that I am in the early stages of a flare, others only happen when the flare is a big one.

You also need to be aware that cryoglobulinemia can affect any organ, and any system in the body. Cryo can affect so many parts of the body, but below are what I believe to be the most common aspects of cryoglobulinemia. Keep these 14 points in mind when tracking your own symptoms.

  • Malaise.
    • I sometimes get a general feeling of “not well”, but “not sick” days or weeks before a flare.  Malaise is a general felling of “not well”.
    • Plus after a week or so of a full-on flare it is very hard to remember what normal is, and everything becomes a struggle.
    • It is hard to be happy when you are as sick as a dog, with no end in sight, and not really knowing what bizarre life-threatening medical event might or might not strike you at any time. You sort of go into survival mode.
  • Muscle weakness.
    • Don’t confuse this with feeling tired. You might not be tired at all but your muscles are actually weak.
    • This is because people with cryo often experience muscle degeneration, or myopathy in the early stages of a flare and this means weak, sometimes sore muscles.
    • This takes months to heal after cryo is driven into remission. A good combination of rest and exercise helps a bit.
    • Don’t try to push weak injured muscles hard during a flare or you will just pull a muscle and have more pain. Take it easy.
  • Aches and Pains.
    • General aches and pains, mostly sore muscles…. just kind of achy and nondescript pain for a while before a flare.
  • Fatigue.
    • You simply feel tired. Your body is burning a lot of energy on an runaway immune system You can expect to feel tired.
  • Rapid Weight Loss.
    • Rapid weight loss. You will lose a lot of weight as the flare progresses. Some of it is due to muscle loss, some is just weight loss in general.
  • Splinter lesions.
    • Splinter Lesions under the fingernails are tiny blood-filled lesions under the nail that appear suddenly. They are tiny, long, and thin.
    • They form instantly and it takes months for the nail to grow out enough for them to go away.
    • Splinter.Lesionsplinter.lesions.2
  • Fever
    • Fever of unknown origin: You wind up running a fever with no apparent cause.
  • Janeway Lesions
    • I have had janeway lesions that form right at the corner where the end of the nail meets the skin. These seem to start as a tiny spot that forms suddenly. I generally feel a small, but sharp pain the moment they form due the blood vessels rupturing. Look at the picture above (the white arrow). In my case they usually grow and turn into necrotic lesions as the flare progresses. This results in dry gangrene which takes anywhere from 3-6 months to heal. I have lost the tips of two fingers to these. They start out really tiny… smaller than the head of a pin.
  • Arthralgia
    • Arthralgia is arthritis like symptoms. In some cases people with cryo have rheumatoid arthritis as the primary condition, others have symptoms that seem to mimic rheumatoid arthritis, but without the rapid damage to tissues in the joints. Some never experience this.
  • Raynaud’s Phenomena
    • Raynaud’s phenomena, the patriotic symptom: Sudden changes in blood circulation in the hands and/or feet.  Sometimes the circulation in my fingers is compromised by the cryo.  My fingers may turn white, or blue, and eventually will turn red when the circulation returns. Sometimes the palms of my hands will be a very bright red.
  • Achy Joints
    • Stiff/Achy joints: The RA-like swelling in the joints can cause stiffness and pain.
  • Swelling
    • The RA-like symptoms will cause swelling in the joints. Most notably in the ankles, knees , wrist and hands/fingers.
    • Usually the swelling, stiffness, and pain from RA is symmetrical…. that is if the knees are affected, it is typically both knees, not just one, etc. Another interesting thing is that when the joints in the fingers swell , the very last joint toward the end of the finger is always unaffected… no swelling there.
    • Beyond the swelling caused by RA, you have edema, mostly in the lower legs, feet and ankles. This is caused by poor circulation. It can be to the extent that your shoes no longer fit. It does not feel good, and for me, my feet feel rather awful, I am aware of every bone in my feet at this point, and walking is uncomfortable.
    • Massaging the lower legs helps a little, elevating the feet helps a bit. I like to use a heating pad under my feet to keep them warm in an attempt to force cryoglobulins back into solution. it all helps a bit, but the real cure is to get the cryo back into remission.
    • You also need to know that many medications can cause edema in the feet and ankles. Steroids are known for this and it tends to compound the problem.
  • Skin: skin rash, lesions, necrosis, and other things

    • petechiae
      • A rash caused by tiny blood vessels rupturing and bleeding in the skin causing tiny ( < 2mm) papules to form. These can come and go rather quickly.
    • purpura
      • This is also caused by bleeding in the skin. It is a close cousin to petetchiae , only the papules are larger… from 2 mm up to about 10 mm (dime sized).  See the purpura survival guide in the blog for more info on this. These can be painful and may take weeks to heal.
    • skin necrosis
      • Basically, patches of skin turn black and die. This is “dry gangrene”. It is surprisingly fast, It can be extensive, and can result in very bad things. Small necrotic lesions are sometime misdiagnosed as spider bites.
    • Skin ulcers
      • Open sores that won’t heal. The skin dies and breaks down, leaving an open wound. This often requires the services of a wound clinic. Often on the extremities, usually on the feet, between toes, or on the lower legs, but can appear anywhere. Also lesions in the mouth or in the nasal cavity. They can be quite painful; and take a long time to heal, even with proper treatment.
    • Livedo Reticularis
      • This is a really scary looking event when it happens.  Basically the veins just under the skin will dilate (usually in response to a change in temperature) .  The end result is a sometimes-vivid  spider-webbish pattern of purplish veins in the skin.  It generally appears on arms, hands, feet, and legs but can sometimes completely cover the torso.  It looks really scary, but in terms of cyoglobulinemia, is basically harmless… but is also an indicator that something is going on (namely, cryo). It can come and go in seconds.  Sometimes a nice hot shower will make it go away.
  • Neuropathy.
    • Mostly in the form of paresthesias (unusual sensations) caused by damage to the nerves in the hands, feet , arms, and lower legs.
    • In some cases the only presenting symptom of cryoglobulinemia is neuropathy.
    • In some cases sufficient damage is done to nerves in the lower legs that people experience “foot drop” (loss of motor control) in one, or both feet.
    • I have had a bout of paralysis and numbness in my right foot ‘that has partially healed, but my neurologist observed that I often drop the toe in my right foot, apparently due to a bit of a foot drop. It sometimes makes me stumble when I walk.

B Cell and Cryo

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.

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

Cryo Cloud: Words from Patients

Cryo.cloud.capture.2

Medicine X 2013: Epatient

medicine x

It has been a hard health year for Marianne and I but through it all we have been epatients.  We have tried to educate, engage and evolve along with the challenges.

One of the biggest personal challenges I have had to adjust to is that my health did not allow me to attend Stanford Medicine X 2013.  I too was granted an epatient scholarship to attend the conference, along with 34 other epatients.  I was looking forward to attending but you learn to adjust, adapt and accommodate when you have a chronic illness.  In the 2 plus months prior to Med X, I had a serious lung infection and could not get well.  Larry Chu and I spoke by phone and he assured me that I had to put my health first and miss the conference.  Larry said he would honor my scholarship for next year, 2014; for which I am very grateful.  Thankfully, the mainstage of the conference was livestreamed so I watched from the comfort of my couch.

Medicine X has been an integral part of the evolution of Alliance for Cryo.  I had hoped to network, connect and shake hands with the people I admire so much but the beauty of MedX was that I got to connect with folks via Social Media.  I am still playing catch up and trying to connect with all the amazing attendees.  Social media means so many things but to me it is a tool towards social justice.  I am a longtime activist born and raised in the heart of the streets disability civil rights movement, Berkeley, California.   I grew up knowing something was wrong with my body and identified with people with disabilities.  I learned to sign fluently, using American Sign Language by the time I was 14.  I became a sign language interpreter, Deaf advocate and part of the community trying for social justice. I went to college at the time The Americans with Disabilities was enacted in 1990.

I saw the same trifecta of themes at MED X; activists, advocates, and people with disabilities trying to get access, exercise their rights and share their lives and stories.

  • The new activists: epatients.
  • The new activist location: the internet.
  • The new action: better health care.

The epatients, healthcare providers and researchers I watched were all unique and diverse but united in one thing – improvement in health care with epatients at the heart of it all.

 

Alliance for Cryoglobulinemia

Alliance for Cryoglobulinemia

“I have Cryoglobulinemia?  What is that?  Cryo means cold and globulin refers to immunoglobulins and emia means in blood.  So immunoglobulins in the blood that do not behave when cold.  Cryoglobulinemia is a rare autoimmune disease; you can call it ‘cryo’.”

And the conversation starts from there.  But now, along with my dear friend and fellow cryo patient Marianne Vennitti, we are taking the cryo conversation global!

Yes, that is a big goal but when you are part of a team, passionate and determined to help others it is possible.  I know it is possible because in one year Marianne and I have created Alliance for Cryoglobulinemia, established our mission launched the website, and built up two international support groups.  This blog is about the journey to create an international platform via the internet; a network that connects people with cryo, advocates, researchers, healthcare providers and doctors.  It is not my journey alone, I walk it with many others who have cryo and those that love us.  I am here to advocate, share and write for all cryo friends around the world.

Marianne and I met online.  We talked on the phone, became friends and realized that we both were advocates.  In September 2012, Marianne came to California to attend Stanford Medicine X at Stanford University in Palo Alto, California hosted by Larry Chu, M.D.  She was the recipient of an epatient scholarship to attend the nontraditional medical conference that celebrates and empowers patients.  I live close to Stanford so I invited Marianne and her husband Chris to dinner.  We became a team and co-founded Alliance for Cryoglobulinemia.

It is now October 2013 and our list of accomplishments are growing.  We have so much to share, so many stories to tell, and so much support for which we are eternally grateful.   My goal is to blog a couple times a week to let you know what is happening here in our cryo world.  We are rare but we are not alone. Stay tuned, we look forward to sharing what we have done, will do and hope to do.