Definition

According to Dr. Charles V. Burton, Senior Medical Director of the Institute of Low Back and Neck Care, Minneapolis, adhesive arachnoiditis represents the most severe form of an inflammatory disease process involving the pia-arachnoid membranes of the brain and spinal canal. The most common causes of incapacitating, clinically significant, adhesive arachnoiditis are foreign substances introduced into the subarachnoid space (e.g. for myelograms and epidural injections); trauma due to motor vehicle accidents; spinal surgery; infections (meningitis).

Arachnoiditis manifests with chronic intractable pain as well as many sensory, motor, autonomic and systemic symptoms. Arachnoiditis can progress to paraplegia; other complications include Cauda Equina Syndrome (CES)- loss of bladder, bowel and sexual function); syringomyelia (cavitation in the spinal cord); arachnoid cysts in the spinal canal. It is, in essence, a form of spinal cord injury for which there is presently no known cure.

Does it really exist?

Despite being listed as a recognized disease by the National Institutes of Health (NIH)and the National Organization of Rare Diseases (NORD), to many physicians arachnoiditis is an unknown condition. To some who have been informed about it, there is debate that it even exists.

Attempts to discount arachnoiditis as an entity do not take into consideration that in some instances, the essential problem may be nerve root damage rather than scarring. The presence of scar tissue in the spinal canal is not always associated with symptoms and primary nerve root damage may not be demonstrated by available imaging techniques.Furthermore, there is no specific diagnostic marker for the entity arachnoiditis nor for nerve root damage.

Diagnostic challenges

Due to some physicians lack of knowledge, skepticism or disbelief about arachnoiditis, patients may obtain incorrect or non specific diagnostic labels for their symptoms. Among these are FBSS (Failed Back Surgery Syndrome), Chronic Pain Syndrome (of unidentifiable origin), Sclerosis, or Fibromyalgia. A significant number of patients have been reported as presenting with anxiety, hypochondria or malingering. Other conditions (such as fibromyalgia or depression) may co-exist with arachnoiditis and a diagnosis may be overlooked due to overlapping symptoms. As a result, obtaining a definitive diagnosis may be a challenge for both the physician and the patient.

The most reported symptom

Albert Schweitzer, medical missionary, having observed many suffering people concluded, "Pain is a more terrible lord of mankind than even death itself." Arachnoiditis sufferers are well acquainted with this unwelcome lord. The major symptom they report is severe chronic intractable pain, usually in the lower back, legs and feet initially, and characteristically described as burning. Later it becomes more widespread and is experienced variously as sharp, stabbing, clawing, twisting, repeated electric shock sensations. Light touch, such as clothing touching the skin, can cause a very unpleasant type of pain (dysesthesia).

Though meant to function like a fine-tuned instrument, the nervous system of one who has arachnoiditis becomes permanently dysfunctional. Nerves in the injured areas release chemicals that signal the brain by a complex two way communication system which acts to transmit messages. In arachnoiditis sufferers the messages that something is definitely wrong continue without letup.

In cases of acute pain, patients are generally able to cope knowing that in time the pain will cease. Chronic arachnoiditis pain is very different in that it continues endlessly, disrupting the lives of sufferers and their families.This is well described in the book "The Management of Pain" by Dr. John J. Bonica who stresses the fact that chronic pain becomes a disease in itself that requires treatment.

Other symptoms

Other sensory symptoms are numbness, loss of heat sensation, tingling, "pins and needles", and intolerable itching. It is a challenge for patients to describe some of the bizarre neurological sensations they experience. Such descriptions as "my body is short-circuiting", " I have vibratory sensations like a cat purring that move around", "I feel as if water is trickling down the side of my leg" are three examples.

Muscle disturbances may include spasm, cramps, twitching, involuntary jerking, and weakness.

There may be headaches, poor concentration, memory impairment, vertigo, visual problems and insomnia. Excessive fatigue is common and very debilitating.

Autonomic nervous system function can be affected - there may be swelling of the hands, feet and legs, flushing of parts of the body, cold blue fingers (Raynaud syndrome).

Physical, mental and emotional stress, prolonged sitting and standing, and weather changes, can exacerbate the symptoms, but often there is no obvious cause for what appears to be an acute flare up of the chronic inflammation resulting in a flu-like illness lasting from days to several weeks.

Only the patient really knows what is experienced. Family members and carers living in the same house may also be able to understand to some degree.

Harmful changes

Entrapment and distortion of blood vessels within the spinal canal result in diminished nutrients and oxygen being delivered to the spinal cord and nerve roots. Lack of oxygen to nerve tissue is known to give rise to constant and incapacitating pain as well as progressive neurological impairment.

Compression of nerve rootlets also causes damage to nerve tissue resulting in neurological impairment which will become irreversible especially if calcification develops within the scar tissue. In the lower part of the spinal canal (cauda equina) loss of bladder and bowel function can result.

This represents only part of the destructive array of problems that develop. Progression may be slow, developing over many decades, or it may develop with dramatic swiftness with rapid progression, even to the point of paralysis.

Mild verses severe

Though a diagnosis based on what is seen on MRI films may indicate that the arachnoiditis is of a mild nature, the experience of pain may be disabling to the sufferer even in the early stages. Therefore the tendency of some physicians to categorize pain by intensity from mild to severe based on the stage of arachnoiditis visualized on MRI can lead to under treatment of pain. It is imperative for doctors to understand that the degree of pain may not correlate with what is visualized on MRI. The patient is the best judge of the degree of pain that they are experiencing.

Autoimmune theory

It is not known why some people develop symptomatic arachnoiditis and others who may also have evidence of scar tissue do not. An auto immune dysfunction has been suggested as a possible reason for this difference and could also account for the widespread nature of the symptoms which are similar to those of Multiple Sclerosis. Systemic toxicity from agents used for myelograms and epidurals could contribute to the multi-system manifestations.

Finding a doctor

For those who have a documented diagnosis of arachnoiditis there may be a long and frustrating search to find someone who understands it and knows how to treat it. In addition, among doctors who do have knowledge about the disease, there may be reluctance to treat the pain with opiods because of concerns that the patient may become addicted or build up a tolerance to the drug. Also, doctors may fear censure from regulatory agencies. This has motivated some doctors to refuse to prescribe narcotics at all or to prescribe an inadequate dosage that does not effectively control the pain. Many patients have thus suffered years of needless pain and disability while searching for a compassionate physician willing to provide available and effective pain management.

Opioids

In his article entitled "Chronic Opioid Therapy for Persistent Non-cancer Pain: Can we get past the bias?" reported in the Bulletin of the American Pain Society, Vol. 1, No. 2, 1991, R. K. Portenoy reported that recent studies document that addiction of chronic pain patients treated with opioid medications is indeed rare yet this fact is not known by many physicians. Another source, Bedard, in a Fact Sheet on Chronic Non-Malignant pain (CNP), July 15, 1997, writes that : Unrelieved pain has many negative health consequences including ...increased stress...water retention...delayed healing...impaired immune system and gastrointestinal functioning; decreased mobility; problems with appetite and sleep, and needless suffering. CNP also causes many psychological problems such as feelings of low self esteem, powerlessness, hopelessness and depression."

Fibromyalgia is frequently diagnosed after some time in Arachnoiditis patients. This is possibly yet another late effect of CNP.

In response to a growing awareness of the benefits of opioids in pain management, physician understanding about the difference in the effects on body chemistry of chronic pain patients and drug addicts is increasing. A chronic pain patient may become dependant on the narcotic medication for pain relief. That is very different from becoming addicted. As doctors are learning not only how to diagnose and treat arachnoiditis but also to understand the dynamics of chronic pain and the beneficial affects of opioids for their chronic pain patients, misunderstandings related to addiction and tolerance in chronic pain patients are being corrected.

Regulatory agencies are beginning to provide legislation authorizing physicians to prescribe narcotics to appropriate patients. This is helping to restore many patients to an improved quality of life. Addressing the pain with adequate and effective pain medication is helping to reduce the suicide rate.

Treatment

Treatment involves finding the most effective relief for pain and other symptoms. Pain clinics are helpful in managing pain. Internal medicine, family practice and physical medicine doctors are also treating arachnoiditis sufferers. Because of the varied patient responses to medications, physical therapies and other non-medical therapies, individual programmes must be designed. Some patients are helped by surgically implanted pumps for direct delivery of medication into the spinal canal, or by spinal cord stimulators.

Despite the increase in understanding about chronic pain and the availability of effective medications, there have been a significant number of patient reports indicating that their doctor attributed much of their pain and suffering to psychological causes. This has happened despite documentation of arachnoiditis in their medical records and scientific information describing the cruel and intractable nature of the disease process. The patient may be referred to a psychiatrist or encouraged to go home and learn to live with the pain. To deny potent and effective pain relief to such patients abandons them to a life sentence of unending pain, discomfort, and progressive disability. There is no way a person can remain stoic in dealing with untreated intractable severe nerve pain.

Compassion

IHJ Bourne, MD in the Journal of the Royal Society of Medicine, Vol. 83, April 1990, states the following in the conclusion of his article "Lumbo-sacral Adhesive Arachnoiditis: a review:

"The relentless and progressive pain syndrome of arachnoiditis is taxing to the patient's morale. In many instances doctors, relatives, and friends fail to realize that the pain can be as bad as terminal cancer, without the prospect of death to end the suffering. Well-meaning enquiries as to whether there is any improvement with the implication that there must inevitably be improvement, since it is not cancer, is distressing to the patient. There are sympathetic doctors, relatives, and friends who expect the patient to be brave, stoical, and cheerful. In the end, the patient yearns for less exhortation and more compassion. Compassion is an important consequence of comprehension of the existence of arachnoiditis."

Other effects

Secondary effects of arachnoiditis are the economic, social and political consequences. The disability is permanent and progressive. The ability to work decreases until careers come to a halt and mutual support becomes the only form of "work" possible for many. This is costly to society in terms of lost productivity, medical expenses and social benefit payments as the majority of these people are disabled during their most productive years.

Appearances are deceiving

In addition to their experience of pain, arachnoiditis sufferers are often misunderstood ("But you look so well") or cruelly misdiagnosed. Perhaps lack of understanding and sympathy is due to the belief that arachnoiditis is a rare disorder, or that it is simply a variant of back pain which is experienced by everyone at some stage of their lives.

A not so rare disease

Despite reports that arachnoiditis is a frequent cause of so-called failed back surgery, it is difficult to diagnose; improved diagnostic methods are urgently needed. It seems that statistical surveys are not conducted in any country so the true incidence is unknown.

An appropriate name

It is hoped that a more positive approach to the condition known as arachnoiditis will be developed. Perhaps using a more acceptable diagnostic label would help. For example, Spinal Cord Injury with Chronic Intractable Pain has been suggested as being a more appropriate term to cover the wide range of signs and symptoms. Their symptoms might then become the main issue rather than the actual cause, and this, after all is where medical management plays the most important role. Since a cure is not known at this time, the emphasis should be on improving the quality of life for the patient.

Perspective

The prognosis for arachnoiditis sufferers may appear grim. Yet it need not be all doom and gloom.

We are all familiar with the Serenity Prayer:

God grant me the Serenity to accept the things I cannot change, the Courage to change the things I can, and the Wisdom to know the difference.

Sufferers must accept that there is no known cure. There are many things that need to be changed to improve the situation as a whole and we must assist in any way possible in order to make such changes a reality so that we can derive some benefit and to ensure that prevention of this disease is achieved.

Fostering change

The key words for change are Acceptance, Education, Responsibility, Rehabilitation, Support, Research, Prevention, Choices and Quality of Life.

Acceptance - the medical, pharmaceutical and political authorities must accept that this condition exists and that it is largely the result of medical interventions and the use of substances which are injurious to the human body.

Education - Medical schools and teaching hospitals must introduce adequate education and training on the subject for health providers.

Support groups are important resources of information which is available to Arachnoiditis sufferers, their carers, the general public and any health providers who are interested. Patients should be partners with their doctors in the management programme. In order to make informed choices they need to learn all they can about the disease and treatment options. The Internet is also a valuable resource for those who have access to it.

Responsibility - The next step is for doctors to take responsibility for making a correct diagnosis as well as providing regular monitoring of patients' progress and appropriate management of their symptoms and disabilities.

Rehabilitation - Rehabilitation at spinal injury units should be freely available for all Arachnoiditis patients, providing full assessment by a multi disciplinary team, followed by a programme to assist in achieving the best quality of life, for each. Regular follow up would be ideal.

Support - Support groups are very important for education, sharing experiences, boosting morale, etc. They should be assisted at government level financially, and by the medical profession with the appointment of expert medical advisers.

Research - Research into all aspects of this disease is needed.

Prevention - This requires the collaboration of politicians, the medical profession, and pharmaceutical companies. The general public can be involved by being adequately informed about the causes of arachnoiditis so that informed decisions can be made if they are faced with any procedures that might put them at risk.

Choices and Quality of Life - Even when one's hopes, dreams and ambitions are shattered by this disease, there is a choice about how to react - whether to become a victim, allowing the situation to take control, or to take charge oneself and use the experience creatively for some meaningful purpose. This may well have to proceed through the stages of the grieving process-Denial, Anger, Grieving and Adaptation. Knowledge is a vital ingredient in the Adaptation phase. It empowers one in the rebuilding of a new life pattern. While the previous mosaic of one's life can never be restored, the pieces can be used to create a new design. We need not be defined by our disease, instead we can incorporate it into a new self image, realistically keeping our goals and aspirations within the boundaries of the levels of pain and disability. We will then have reached beyond Adaptation to Renewal.

Those who take up the challenge will discover that a good quality of life is possible when the focus is not on personal pain but on the positive opportunities arising from even the most adverse circumstances.

If you are able to identify with any of the above then this website is for you! ASAMS is about Caring and Sharing. You are not alone!