This case description concerns a 72-year old woman who has had CRPS type 1 or Sudeck’s Dystrophy since 13 years. This syndrome developed after she fell off a short flight of stairs and bruised her ankle. Initially, pain and swelling developed only on her left foot. After some time however, the symptoms spread also to her right foot. Both feet were swollen, the skin felt hot and was shiny, the toenails dystrophic. All these confirm a typical case of CRPS type 1. The case can also be seen at Youtube.
The pics show her feet before treatment via our protocol and 1 month after treatment. Meanwhie, after 3 months she again is much better.
Development of CRPS in the feet:
She experienced severe, burning pain that made sleeping difficult. The bed sheets caused contact pain and this woman had to cool her feet during the night. As part of the treatment her pain was assessed on a scale of 1 to 10, where 10 is the most severe pain imaginable. Her pain had very severe peaks, but averaged 6-8 when she came in for treatment. Her quality of life had also suffered significantly; she rated this herself as a 4, again on a 10-point scale (where 1 is terrible and 10 excellent).
Additional CRPS in the left knee:
In mid-2012, the patient fell off her bicycle. Due to the severe contact pain in her feet, she hardly walked anymore, but still rode her bicycle. While going out on her bicycle one day, she unfortunately hit an obstacle and fell off the bike. She fell on her left knee.
The CRPS surged due to the fall and spread to the left knee. When she came to our clinic in November 2012, the knee was swollen significantly and the skin blueish-red, as were both feet. It was the presentation of a generalizing case of CRPS. This is a very unpleasant syndrome, where the CRPS spreads to different parts of the body. None of the doctors she had seen knew how to treat this effectively, which is why she came to our clinic.
Many treatments tried against the CRPS:
From the beginning 13 years ago, many treatments and painkillers were tried to treat her CRPS. Treatments to open the blood vessels wider (vasodilatating drugs) had counter-productive effects, increasing the pain and swelling. DSMO cream applied locally on the feet also did not help. Only the drug amitriptyline had positive effects, but limited. Pain remained present at a score of 6-8 and quality of life at 4 (i.e. poor) when we first saw her. By that time she had also visited a well-known specialist in CRPS in an academic hospital int eh Netherlands, who could not do more for her than determine her invalidity score.
She came in to our clinical in a wheelchair, with severe pains and swollen feet and left knee. She felt very bad and was in a downtrodden mood.
Palmitoylethanolamide (PEA) under the tongue (sublingual):
As all usual methods had been tried, we started her straight away on our specific treatment protocol for CRPS. The initial step here consists of the natural compound PEA (a painkiller and inflammation inhibitor) combined with a topical cream of 10% ketamine. PEA was prescribed as the preparation PeaPure, to be taken 3 times per day. The dose form is a capsule of 400mg, that can easily be split and the contents put under the tongue, where the content (a powder) melts. It is expected that in this way a quicker effect is obtained.
After ten days she returned to our clinic. She came in walking, albeit with a cane, and the swelling of her feet was significantly less.
She then came to visit one month after start of the treatment. We then had the conversation together that you can see on video.
The swelling and the pain were reduced by more than 50% after one month of treatment. She also did not need a cane anymore for walking. This was something she had not been able to do in the past 13 years.
CRPS and the rationale for treatment with PEA:
PEA is known as a food supplement and thus easily overlooked by doctors, who mainly get information on drugs from pharmaceutical companies. Yet PEA is a natural painkiller and inhibitor of inflammatory reactions, present in our own body. Judging by the positive experiences of our patients, it is a pity and really a missed opportunity that it is not used more.
Two months later the clinical picture was improved even more: she could ride the bicycle for 20 km/day, could wear socks and shoes again and was a totally new person.
Another option for treatment that is often overlooked is the use of topical painkilling- and anti-inflammatory creams. CRPS is a kind of inflammation, as witnessed by this patient’s red, swollen feet that are also painfully hot. In our institute, we developed a cream containing 10% ketamine that we use in our treatment protocol for CRPS. This cream is gradually discovered and adopted by neurologists and anesthesiologists in large hospitals. One of them recently told me: “when I see a patient with CRPS I always treat them with the ketamine cream; if the pain does not go away with this treatment I doubt if the diagnosis was correct.”
Research into the causes of CRPS:
It is difficult to investigate what causes CRPS in humans. Thus, in recent years researchers developed an animal model to investigate this. In the model, a back leg of a rat is broken and immediately immobilized with a splint. The leg then becomes swollen, red and painful. The rat cannot stand or walk on the leg any longer. This model has helped researchers a lot to gain understanding of the inflammatory process involved in CRPS.
Mast cells: cause of the inflammation in CRPS?
Two experts wrote a comment with this title in the Journal of anesthesiology in 2012. They emphasized the inflammatory part of CRPS and the role of the special inflammatory cell, the mast cell.
But first let’s look at results obtained with the rat model.
The researchers found many indications for an increased and dysfunctional inflammation in the rat hind leg. Inflammatory substances such as TNF-alpha, interleukins 1 beta and 6, substance P were found as well as increased inflammatory factors in the skin, such as growth factors.
One aspect of the research of this group has received increased attention in recent years, the mast cell. The group found increased activity of this cell in their model. The mast cell is a source of many inflammatory substances. Increased activity of the mast cell has also been shown in models with humans. In rats, there is a specific genetic type that is especially sensitive to increases of the inflammatory substance P. In this type of rat, the mast cells will excrete large amounts of inflammatory substances when stimulated. These substances then overstimulate small fibre nerve cells in the skin and other tissues, which causes severe pains. The substances from the mast cell stimulate a specific nerve cell, the peptidergic nerves, causing these to excrete even more substance P, thus causing a vicious circle. In humans, it is supposed that there are people with an inherited, genetic oversensitivity to substance P. Small increases in the level of substance P in these people causes a massive reaction from the mast cells, leading to severe forms of CRPS.
‘Bringing in check’ mast cells in CRPS with PEA and ketamine to reduce inflammation and pain.
The substance PEA was discovered in in 1957. The influence of PEA on mast cells was discovered in 1993 by Professor Rita Levi-Montalcini (a Nobel laureate). Her research showed that many pain syndromes and inflammations react positively to PEA, due to its ‘calming’ effect on mast cells. By now, more than 300 studies have been done into the way PEA works, making PEA the most researched natural substance. PEA is a simple, fat-like substance that occurs naturally in humans and many different species of animals. It is also present in many types of food. It has an important function in normalizing excessive inflammatory reactions. This is why, since its discovery, PEA has been used successfully in the treatment of many types of pain and inflammation (apart from CRPS, e.g. sciatic pain, arthritic pain, diabetic pains, zoster pain, amputation pain, flu and cold symptoms, rheumatoid arthritis, etc.).
PEA is available via the internet as PeaPure, a food supplement produced according to the standards of GMP in the Netherlands. The recommended starting dose is 400mg three times per day. In CRPS it is recommended to open the capsule and put the content (powder) under the tongue. There, PEA will dissolve slowly and thus absorbed directly into the body to exert its effects.
The interview with the patient shows a positive response. Without reported side effects!
After several years of experience in developing our treatment protocol, we believe all CRPS patients should be started on treatment with PEA and ketamine cream as the basis. This should be supplemented with adequate amounts of vitamin D3 (at least 1000IU/day) and vitamin C (at least 500mg/day). In this way many patients can be treated effectively with minimal or no side effects.
The patient needs to come first, not guidelines!
In the last 10 years, medicine has become dominated by guidelines that are more and more treated as law, instead of what they should be: a guide. These ‘laws’ originate from evidence Based medicine (EBM). EBM started was initiated in the nineteen eighties with the aim to base patient treatment more on the evidence available from well-designed studies. In principle this is of course an excellent idea. Unfortunately, though, this noble initiative has derailed into a straightjacket. If a patient comes in with disease a, the doctor initiates treatment A, if not effective replace by- or add B etc. And woe thou if this standard recipe is not followed. This is fundamentally not good as it ignores the fact that large studies show an average effect in an average, non-existent patient. I.e. it ignores the individuality of the patient doctors treat.
We recommend that every CRPs patient is treated as an individual, with all his or her personal factors. Thus deviating from standard guidance may be the best thing to do for the patient. For CRPS patient, we honestly and truly believe that the treatment with PEA and topical ketamine cream is the best starting point, providing effective treatment for many (but not all!) patients with minimal side effects and no interactions with other treatments.