The Empowered Patient's Guide to Complex Regional Pain Syndrome (CRPS)

Complex regional pain syndrome (CRPS) causes pain that can be severe, constant, and completely perplexing. In the worst cases, CRPS causes the loss of mobility and function. If you develop pain in the aftermath of an injury or trauma that lasts longer than six months, seek your doctor's counsel. CRPS just may be the most misunderstood and misdiagnosed of all the chronic pain conditions. 

Complex-Regional Pain Syndrome (CRPS), an enigmatic and rare disorder, can develop in the after math of an injury or trauma. It's described as severe, burning, and persistent pain the usually affects a limb, the hands or feet.

 

SO, WHAT IS CRPS/RSD EXACTLY?

Complex regional pain syndrome or CRPS is an unsual type of chronic pain that typically affects one limb—an arm, hand, leg, or foot—and frequently occurs after an injury or trauma.

Here's the interesting thing. The injury or trauma doesn't have to be major, like surviving a serious car accident or getting hit in the face by a fast-flying baseball  Yes, impact like this can trigger CRPS but it can also develop from things like stepping on a nail or having surgery to remove a bunion. It's also possible to develop CRPS from a dental infection, a shot in the arm, or something as ordinary as stress.

In some people, CRPS causes skin coloration changes in affected parts of the body, temperature differences (hot or cold limbs for example), and the loss of movement or function in a foot or the fingers.1

There are two categories of CRPS: type 1, previously known as reflex sympathetic dystrophy or RSD, and type 2, previously known as causalgia. The difference between these two types is whether nerve damage is involved.

In CRPS-I, there is no evidence of nerve damage. In CRPS-II, there is evidence of nerve damage. If you are diagnosed with CRPS, chances are you most likely you will have type 1. About 90% of people with CRPS have type 1. 1

Overall, CRPS is not a common condition. Studies show an incidence rate of 6.28 per 100,000 person-years when combining types 1 and 2.2 This means that, on average, six people will be expected to be diagnosed with CRPS out of 100,000 people observed annually in the United States.

CRPS is more common in women —affecting women three times more than men.2  Although CRPS can occur at any age, it is rare in elderly people and children under 10 years, it peaks at the age of 40, according to the National Institute of Neurological Disorders and Stroke.2  Other data shows it most frequently occurs in people aged 61-70 years.

If you have CRPS—regardless of having type 1 or 2 type—your symptoms may present similarly and be treated in the same way.3

THE DETAILS: HERE'S WHERE CRPS/RSD GETS MORE COMPLICATED

Why people develop CRPS is not clear. For more than 90% of people who develop CRPS, it is triggered by a trauma or injury such as a fracture, sprain, soft tissue injury (like a burn or bruise), or surgery. Even having a limb in a cast can trigger it for some people.

But the majority of people with similar injuries or traumas do not experience CPRS. So why some people develop CRPS while others don't is largely a mystery. 

What is known is that CRPS is an abnormal neurological response. Just as some people have an abnormal reaction to certain food which leads to food allergies (think nuts or dairy), CRPS is an abnormal reaction to an injury or trauma.1

Although less typical, some people develop CRPS after having a stroke, heart attack, or other condition. Perhpas the most confusing part of CRPS is that the pain persists for months or years after an injury has healed. The pain can also have the following distinctions:

  • It's greater than would be expected from the injury (also known as hyperalgesia)
  • CRPS can cause you to become more sensitive to a pain stimulus (hyperesthesia)
  • CRPS  result from a non-painful event (allodynia)  

Physicians and neuroscientists do not know why a person’s nervous systems malfunctions and broadcasts fierce pain signals. In fact, there is usually a lack of a specific nerve injury (or trigger).

Despite its mysterious attributes, few physicians doubt that CRPS is a real and difficult condition.  As a chronic pain syndrome, physicians are increasingly aware of and educated about ongoing research that continues to highlight the important role of the brain in processing sensations like pain and changes in the brain that can lead to an abnormal perception of pain. 4

Unlike in the past when some chronic pain conditions were dismissed as psychological, the newer understanding of the brain’s involvement in processing pain has shed light and helped to destigmatize conditions like CRPS that have been difficult to understand.
 

CRPS SYMPTOMS: INTENSE PAIN 
 

The main symptom of CRPS is continuous and intense pain in the feet, legs, arms, or hands. The pain gets worse over time, and frequently spreads to another extremity or area of the body. Patients often describe the pain as a burning sensation, or a “pins and needles” sensation.

Typically, there are three stages of CRPS:6

  • Stage 1: Lasting from one to three months in which symptoms include severe burning/aching pain, changes to skin (temperature, color, texture), sensitivity to touch, muscle tremors/spasms/jerks, swollen and stiff joints, and rapid growth to hair and nails.
  • Stage 2: Lasting three to six months in which symptoms progress. Pain intensifies, changes to nails and skin continues, joint stiffness and swelling increases, and pain may spread to unaffected limb.
  • Stage 3: Chronic phase that can develop up to a year after initial onset of CRPS and can last for years or become permanent. Pain may be constant or intermittent, atrophy can develop with loss of function to the affected limb.

CRPS CAUSES 

Medical experts have not yet uncovered the cause of CRPS.Some of their theories include:

  • The neurochemical norepinephrine over-activates pain sensors for no apparent reason, or as an overreaction to an injury
  • Inflammation damages small-fiber nerves
  • The immune system is somehow triggering the pain mechanisms  

Other experts believe there may be a number of connected events happening concurrently to see off the pain response in CRPS. 

HOW TO DIAGNOSE AND TREAT CRPS/RSD 

Diagnosing CRPS is challenging and often made by excluding other conditions. A thorough physical examination of signs and symptoms is the mainstay of diagnosis, and your doctor may suspect CRPS if you show a combination of symptoms listed above. Often it will require observing these symptoms over time to make the diagnosis, with moderate to severe cases of CRPS appearing in 1 to 3 months.

Other tests may also be given to help rule out other conditions that may present with similar symptoms, such as Lyme disease, arthritis, small fiber polyneuropathies, generalized muscle diseases, or a clotted vein. The key feature that distinguishes CRPS from these other conditions is an injury to the area in which you are experiencing pain.1

Other tests that may be used for diagnosis include:

  • Bone scans and x-rays—identify early bone deformities
  • Skin sensitivity tests
  • Thermography—measures changes in blood supply and skin temperature
  • Nerve conduction tests–observe nerve behavior
  • MRI–usually 2 or more to monitor changes in skin, muscles, blood vessels, and other tissues

If your primary care doctor suspects you have CRPS, you will probably be referred to a neurologist for further workup and treatment. Often you may be treated even before a definite diagnosis is made as early treatment is important to alleviate symptoms.

Does CRPS Heal?

In most cases, CPRS is a permanent condition. When CPRS is diagnosed and treated early, there is a chance it can disappear permanently. Nevertheless, with most patients the goal is to reduce the pain and slow down the symptoms. Learning how to cope with some pain and learn to live a full life with some symptoms is key to successful management.5

Doctors Who Treat CRPS/RSD

If you or a loved one are living with CRPS, a neurologist or neuropsychiatrist needs to be the doctor who manages your CRPS care. You may also add a pain management specialist, rheumatologist, and other key specialists to your CPRS team.

University medical centers and large teaching hospitals are often the best place to access this team of specialists.5

CRPS TREATMENTS

Early treatment is critical to relieve the pain caused by CRPS and to delay damage. Although there is no cure for CRPS, there are a number of treatments. The National Institute of Neurological Disorders and Strokes describe a number of treatment options:1

These include the following:

  • Physical therapy. Engaging in an exercise program is important to keep your painful limb moving and improve blood flow, as well as maintain and improve limb flexibility, function, and strength.
  • Rehabilitation and occupational therapy. Techniques can be used to rehabilitate nerves to the affected limb to prevent or reverse changes to how your brain processes chronic pain.9 Occupational therapy can help you learn new ways to do everyday tasks.6
  • Psychotherapy. Getting help for the emotional and mental challenges of living with chronic pain is important. It is not uncommon for people with CRPS to develop depression and anxiety, both of which can increase your perception of pain.
  • Medications.  A number of drugs are used to treat CRPS and may be effective particularly if used early. There is no one drug or combination of drugs that works for everyone, and not everyone will be helped by drugs. Types of drugs used to treat CRPS include:
  • Bisphosphonates (eg, high dose alendronate or intravenous pamidronate)
  • Non-steroidal anti-inflammatory drugs (NSAIDS) (eg, ibuprofen, naproxen, and aspirin)
  • Corticosteroids to treat swelling and edema (eg, prednisolone)
  • Botox injections
  • Opioids (eg, oxycodone, morphine, hydrocodone)
  • N-methyl-D-aspartate (NMDA) receptor antagonists (eg, ketamine, dextromethorphan)
  • Topical creams or patches (eg, lidocaine)
  • IVs and Interventional Pain Management. If your pain is not helped by drugs or other treatments, your doctor may talk to you about a number of interventions used to block pain through one of the following techniques:1,10
  • Sympathetic nerve block
  • Surgical sympathectomy
  • Spinal cord stimulation
  • Neurostimulation
  • Intrathecal drug pumps

Emerging Therapies
Several novel therapies are being investigated that may be helpful, including intravenous immunoglobulin (IVIG), graded motor imagery, and ketamine.

Of these, ketamine is emerging as an effective treatment options for some patients.11,12  A recent study that looked at a number of clinical trials found that ketamine infusion provided short-term (less than 3 months) pain relief.13

WHY YOUR MENTAL HEALTH IS IMPORTANT WHEN LIVING WITH CRPS/RSD

Like all chronic pain conditions, living with CRPS is not easy. The physical stress of living with pain can place an enormous stress on your emotional and mental health. For some people, this may lead to problems with depression or anxiety.

If not recognized and addressed, these mental health effects can further increase your perception of pain.

It is very important to talk to your doctor about any mood changes or mental stress you are experiencing. You may benefit from talking to a therapist or pain specialist to better understand the body and mind connection in chronic pain, and learn some self-help tips that can go a long way to ease your perception of pain.14

SELF CARE

There are a number of things you can do to take care of yourself to help you manage the symptoms of CRPS, including the mental and emotional challenges discussed above. Many of these self-care strategies are the same ones that help with overall physical and mental health. These include:16

Eating a healthy diet. Eating a healthy diet made up of fruits, vegetables, whole grains (like brown rice), lean proteins, and healthy fats (like olive oil) is good for overall health. Avoid or reduce your intake of foods and drinks high in sugar or salt, including processed foods (like cookies, chips, processed meats, etc) and sweetened drinks like sodas.

Exercising regularly. Movement helps to improve muscle strength, reduce pain, and improve mental and emotional well-being.

Getting sufficient rest: Establishing a regular sleep schedule can improve the quality of your sleep. Going to bed and rising at the same time, sleeping in a cool and dark room, and avoiding too much stimuli close to bed can help establish a healthy sleep schedule.

Having a positive attitude: Developing and maintaining a positive outlook is key to dealing with stressful events and other conditions such as PHN. HUH? A positive attitude focuses on your strengths instead of your weaknesses.

Overall, taking care of yourself is about making time for yourself and is one of the best things you can do to help cope with CRPS. Along with developing the healthy habits described above, it is also important to take time to engage in activities that bring you pleasure such as reading books, watching movies, creating art, or writing in a journal. 17

CRUSH THE STIGMA

People experiencing a chronic pain condition, such as CRPS, may feel judged or misunderstood by others. According to the Institute for Chronic Pain, many patients with chronic pain feel they are being judged for having a condition they didn’t choose to have or for the way they are coping with the condition. People may say things that make you feel guilty for living with HPN, such as “It must be all in your head” or “I have back pain too but I still go to work…Why can’t you?”.18

It is important not to buy into these judgements or the guilty and hurt feelings they inspire. You can help yourself and others with CRPS by simply acknowledging what you are experiencing is difficult.

Also helpful is understanding how pain, especially persistent pain, works in the body—primarily through changes in how your brain processes pain.

See for example, this simple description of pain https://www.youtube.com/watch?v=C_3phB93rvI . Many people don’t understand chronic pain and the more you become educated yourself about chronic pain the better you can help educate others.

WHAT CAN I DO RIGHT NOW?
 

You can begin to help yourself right now to manage the challenges and stress of HPN by eating better, engaging in relaxation activities, improving your sleep, moving more, and maintaining social connection with others.

Engaging in physical activity, even taking short daily walks, will help you feel better. You also can help yourself by learning about pain and how it works in the body.

Once you learn the large role played by the brain in how you experience pain, it can help you take more control over your pain and become less fearful of it.

For more information on the pain experience, check ou t this video: https://www.youtube.com/watch?v=Zv6RPoVZx9M.

FAQS

What is CRPS/RSD?

Complex regional pain syndrome or CRPS is type of chronic pain that typically affects one limb, such as an arm, hand, leg, or foot, and usually occurs after an injury. There are two types of CRPS: type 1, previously known as reflex sympathetic dystrophy or RSD, does not involve nerve damage whereas type 2, previously known as causalgia, has evidence of nerve damage.

How do you prove you have CRPS?

Diagnosis of CRPS usually is done through a physical exam and history that may include watching symptoms over time such as pain, swelling, redness, changes in temperature and hypersensitivity. Tests may also be given to rule out other conditions with similar symptoms, such as Lyme disease.

What are the stages of CRPS?

There are three stages of CRPS. Stage 1 lasts from one to three months during which people can experience a range of symptoms including severe burning/aching/pain, skin changes, sensitivity to touch, and rapid hair and nail growth. Stage 2 lasts two to six months during which symptoms progress and pain intensifies. Stage 3 is the chronic phase  that can develop a year after initial onset of CRPS and last for years or become permanent during which pain may be constant or intermittent.

How long does CRPS take to heal?

Most cases of CRPS are permanent. If diagnosed and treated early, CPRS may disappear permanently but in most patients the goal is to reduce the pain and slow down the progression of symptom severity.

 

Updated on: 02/16/21
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