Central Pain Syndrome (CPS)
Stroke caused neurological damage to the Central Nervous System (CNS). The damage to the CNS causes sensitization to the pain system. The extent of pain and the areas affected are related to the cause of the injury. CPS begins shortly after the causative damage, but may be delayed by months or even years.
CPS can either be relegated to a specific part of the body or spread to the entire body. Pain may be moderate to severe in intensity and is often exacerbated by movement, light touching or temperature changes, usually cold temperatures.
Burning pain is the most common sensation, but patients also report pins and needles, pressing, lacerating, aching, and extreme bursts or constant sharp or unremitting excruciating pain. As a result of the constant extreme pain, blood pressure often rises.
The year was 1994, I was on an important business trip in Toulouse, France. For years, I had been regularly suffering from migraine headaches. My neck would become very tense from the effects of the migraine. To relieve the tightness, I would frequently push my head sideways to make my neck crack. Little did I know how much damage the years of repeatedly cracking my neck did to it. Then, on the morning of June 20, 1994, I found out!
The inner wall of my left vertebral artery partially broke off like a flap. It temporarily blocked the oxygenated blood from flowing to my brain stem causing a catastrophic stroke. The location on the brain stem where the stroke occurred is extremely rare. My doctor had only seen a single similar brain stem stroke in over 40 years of practice. He explained to me that my stroke happened in the brain stem, which is basically a bridge between the brain and the central nervous system. He went on that the brain stem controls all of the mechanical skills areas of the body (breathing, moving, talking, etc). And that my brain’s ability to control the muscles in my body had been severely stunned. I could not move anything below my nose. My whole body became flaccid, like a wet noodle. Oddly, I still had normal sensation over my whole body. I could feel every touch on my skin, including the 6 weeks that I was in the hospital of painful needle pricks necessary for the daily drawing of blood.
I finally came home from the hospital. As time went on, there was little but somewhat noticeable improvement. At two months, the muscle tone in my extremities returned and replaced the flaccidity. After about four months, my leg muscles began to respond.
The quadriceps muscle for both legs woke up first. I had heard that it was common for the larger muscles, like the quadriceps, to recover first. It felt strange that my right and left legs were recovering at different rates, though. The whole right side of my body is still slightly stronger than the left. My right quadriceps consistently lagged the left by at least 10%. My quad muscles were slowly getting stronger. My left quadriceps probably started at about 10% of full strength and very slowly increased to about 40%. The right started at about 5% of full strength and very slowly increased to about 30%. This is where my leg strength maxed out, though.
My hamstring muscles were still very weak. I could feel a faint contraction in the muscles but could not make them move. In other words, if I thought about it and tried really hard, I could make my quadriceps push my legs down to make my legs go straight. But, I could not pull back with my hamstrings and pull them back. Those muscles need to work together, pushing and pulling, in order for me to be able to walk. Unfortunately, because my hamstrings were so weak I could not make my legs move backwards.
Eventually, my quadriceps became strong enough that I could stand with assistance. Every morning, while transferring from bed to wheelchair, my wife would stand me up straight for a few minutes and give me a hug. It felt so good to be able to push myself up to standing position, flex my quads on demand and feel my leg muscles respond. This routine went on for at least the first three years after my stroke.
Recovery from a stroke, after the first six months, is painfully slow. This slow-down in recovery happens to all stroke survivors so it was not like it was happening to just me. Something that concerned me, though, was that as my legs grew stronger I could feel the muscles in one leg better. I could not understand it, my left quad felt light and normal but my right quadriceps felt heavy and dense, like it was filled with lead and weighed 50 pounds. Little did I know that this new sensation would soon become my worst enemy!
Onset of CPS
CPS did not happen immediately following my stroke. The CPS waited almost four years after it. For almost four years, I had a relatively busy schedule seven days per week. Monday, Wednesday and Friday were days for Out-patient therapy – Physical, Occupational and Speech. Although the therapy sessions consumed over three hours on these days, every other second when possible, was spent on the computer. I would sit in my wheelchair usually for as long as I could, sometimes in front of it at least 10 hours per day. Cyberspace became my way of life!
My therapy sessions finally ended after almost four years! It seemed that simultaneously with my therapy sessions ending that the first signs of CPS started. My CPS was not painful at all at first. The CPS began with just a light tingling in my right leg. Shortly thereafter, my left leg started to lightly tingle, too. I was not concerned as I thought the tingling sensation was a good thing. I had heard from my stroke survivor friends on-line that paralyzed limbs often tingled right before recovering. As far as I was concerned, the recovery for my legs had started to progress again.
A month later, my lack of concern with the tingling was quickly becoming a major concern! I was utterly confused about what was happening. It seemed like the tingling in my legs was intensifying! They had developed a pins-and-needles sensation and it felt as though both legs had become severely sunburned. It was extremely painful whenever my legs were touched. Even incidental light touches irritated them. Touching or leg movement caused the combination of tingling, pins-and-needles and severe sunburn. It felt as though my legs were lying in a fire and as if though metal wire brushes were simultaneously poking every square inch of them!
Plus, it felt like an uncomfortable cold fluid had replaced the normally warm blood circulating through my legs. No longer did my legs naturally warm to room temperature by themselves. Light breezes or cooler temperatures caused my legs to easily become irritated, causing the severe sunburn pain with pins-and-needles to flare up.
I had to have a throw blanket on my legs at all times. Having my legs covered by a blanket became a part of my daily attire. The blanket prevented the open air from circulating around my legs, causing them to become irritated. Much like applying pressure on a wound, to dull the ache and pain, the weight of the blanket was soothing and brought comfort! I quickly adapted to wearing a heavy blanket on my legs full-time even in the middle of the summer.
I was determined that just because I now had CPS I would not let it slow me down. My daily activities soon became a routine. Getting ready for the day was the worst! Hygiene involved me getting a bed bath followed by my wife dressing me. During this time, she had to frequently touch my legs, bend them at the knee and turn me in the bed, from side to side, in order to wash both sides of my body and then get me dressed. After I was almost completely dressed in bed, she then had to move me to transfer me to my wheelchair.
This consisted of her picking my legs up and moving them off of the side of the bed so that they were sitting flat on the floor. Then, she lifted my upper body so I went from lying flat to now sitting up on the edge of the bed. Next, she would put my head down by her hip, hold me under my arms and would count to three. On three, it was my job to push with my legs while she pivoted my body from my bed to the wheelchair. This is aptly named the sit-pivot transfer technique. Once I was in my wheelchair, she would pull and tug my sweatpants and get me positioned evenly on my cushion so I was comfortable for the day.
The bad thing was that the intense burning sensation would start with the first touch of my wife’s hand when she started washing me. The burning pain would linger on for almost an hour after the last time she had to put her hands on me to transfer me to my wheelchair. Every day, I had to endure the agony of this for almost three straight hours. It literally felt like my legs were being burned by a blow torch. The pain was gut-wrenching and lingered on for almost an hour until it would finally subside.
What was strange was that if I was not being touched, like when I was sleeping, my pain level was almost non-existent. But, the very second I was touched and moved around in my bed the extreme burning sensation began. I could not continue to live this way! I needed to know what was happening to me. I wanted to do whatever was necessary to make the pain go away.
Searching for a Cure
I started off trying to find out what was happening to me by going to see my General Practitioner. He was baffled and recommended that I see my Neurologist. Several months later, I finally had the appointment with my Neurologist. He was perplexed, as well, and could not identify the problem nor offer me a solution. Over the next six months, I saw several more doctors with no luck. Nobody could identify the source of my pain or provide a treatment plan.
I even tried acupuncture to no avail. Acupuncture treatment was fun! Not! It consisted of the acupuncturist placing 3” diameter suction cups all over my legs. Each cup caused a tiny vacuum on the skin under it, which was extremely painful! I had to lie still with those cups all over my legs for like 15 minutes. I was really mad because I had to endure this pain, plus the acupuncture treatment did absolutely nothing for me!
My wife and I spent almost two years of countless doctor appointments only to learn that they knew little more about this mysterious pain than we did. I was at the threshold of my limits for pain, too. I remember one night, as my wife prepared to transfer me from my wheelchair to my bed I just broke down in tears. I anticipated the intense pain I would have to endure as she moved me to my bed and then had to straighten my legs out. Just the mere thought of the upcoming pain had become too much to bear.
One night, my wife was browsing through a magazine called Physical Therapy. She came across an article about tingling, pins-and-needles and intense sunburn pain. The formal name for this pain was something called Central Pain Syndrome (CPS). We were both so excited because the article described my pain. . .EXACTLY! My type of pain had a name! As she read further, the article explained that treatment should be performed by a Pain Management doctor.
We did not know what a Pain Management doctor was so she got out our pamphlet of approved doctors by our insurance company. She found a doctor named Dr Peter Staats at Johns Hopkins Hospital Pain Clinic. I was so fortunate to have a world known premier hospital only 30 miles away in downtown Baltimore. Three weeks later, I was finally talking to a doctor who understood my problem with the burning, tingling and pins-and-needles pain in my legs.
Dr Staats was a very kind and compassionate man. He listened patiently as I mouthed out an explanation of my pain, while my wife read my lips and turned what I said into words and then sentences. What was cool was that he was familiar with the type of pain that I was describing.
After I finished venting, he explained that my stroke damaged my Central Nervous System (CNS), which was causing my CPS. He further explained that pain is ordinarily a protective sensation that causes a person to move away from its cause, such as withdrawing one’s hand from a fire. In the case of CPS, a source of the pain does not exist. Instead, the sensory pathways within the brain were damaged by the stroke. This stimulates central nerve fibers, which created the perception of pain.
So, what this meant was that my brain stem had been damaged in such a way that my CNS had stopped functioning properly. My CNS perceived the pain, like the burning and pins-and-needles sensation, although the source for that pain did not actually exist. It made sense.
Then, he told me he had bad news and good news. The bad news was that CPS unfortunately could not be cured! The good news was that there were drugs that could help me manage my pain but it was not possible to actually cure me of CPS. He then developed a plan to help me manage it.
The first step was to take the “edge off” of my pain. The pain in my legs was becoming increasingly more intense by the day. The pain would zap me like a high voltage electrical charge wherever my legs were touched. The zap would then morph into severe sunburn on that spot. So, to take the “edge off”, he prescribed a low dosage of a long acting pain killer called Oxycontin.
He then tested my body for spasticity. When he saw how stiff I was, especially my legs and knees, he told me that I might be a candidate for an ITB pump. He said that the pump would be permanently implanted under the skin on the left side of my abdomen. He explained that ITB stood for Intrathecal Baclofen and that the pump would continuously inject a drug, called Baclofen, into my body. The continuous injection of Baclofen would relax the spasticity in my legs. What was even better was that he told me the ITB Pump would also reduce the pain.
I thought it sounded too good to be true! I asked him to tell me more. He then picked up a small circular shiny object from the table in the exam room. The object closely resembled a stainless steel hockey puck. What he was showing me was the demo model of a Baclofen Pump. He explained that the pump’s reservoir only held 40 ml of liquid and that it would have to be refilled by a nurse every two – three months.
The pump continuously injected Baclofen through a space, called the intrathecal, in the spinal cord. A small tube, called a catheter, would be threaded from the pump through the body, around the back and into the intrathecal space.
Because the Baclofen would be delivered directly to the spinal cord, the symptoms could be controlled with a much smaller dose, about 1/300, than is needed with oral Baclofen. The goal of an ITB pump is to control the symptoms and to better reduce the need for oral pain killers; thus reducing their associated side effects.
Depending on the dosage and rate of delivery, he was confident that this would effectively relax the spasticity in my legs and help reduce my pain.
Everything he said sounded good. I was excited and I told him I was ready to proceed. He told me that we had to perform a trial before he permanently implanted the pump. A trial is a standard screening test that is required to determine whether an ITB Pump is a suitable treatment option for a patient’s severe spasticity. In the trial, the patient receives a small dose of morphine injection via a lumbar puncture, which is a minor medical procedure. Then, the patient’s response is evaluated.
So, I scheduled a date for the trial. I waited for the trial date with much anticipation. I thought the trial would be a simple procedure. Boy was I wrong! It required that I be admitted to the hospital for the weekend. Little did I know that the lumbar puncture would be a major source of the problems I next had. My doctor explained that migraine headaches typically occur anytime the lumbar is punctured. The puncture to my lumbar caused a severe migraine headache right away. I have a very small tolerance for a migraine headache and I immediately became nauseous.
That was the worst two days of my life! The migraine headache and nausea traumatized me. I was a different person for several weeks following that weekend at the hospital. I was not motivated to work the usual long hours at my computer every day. Instead, I would just spend the day watching television. My brain felt fried! At least I had passed my trial for the ITB Pump.
A surgery date was scheduled for several weeks later. Finally, the big day arrived! Surgery was scheduled for the early morning hours. My wife had to get up around 2:00 am in order to get herself ready. Next, she started getting me up around 3:00 am. By 6:00 am, we were in route to the hospital for a 7:00 am check in appointment.
Although, my wife and I arrived the required two hours early, we still had to wait for several more hours in a huge Waiting Room. The Waiting Room was partitioned into dozens of smaller private waiting rooms. Each equipped with its own private flat screen television and with several tasteful pieces of overstuffed furniture, much like a den, or family room, in your house. After about three straight hours of watching boring morning shows, a nurse came and took us to my Recovery Room, where we waited for several hours more.
After a briefing of what to expect in the OR by the Anesthesiologist and an OR Nurse, I was transferred from my comfortable hospital bed to a narrow, hard and uncomfortable gurney. Seconds later, we were zooming down the hall and into the Operating Room (OR). The OR was a well lit room, obviously sterile and freezing cold. The room was crowded with people wearing caps, gowns and with masks covering their faces. Immediately, the Anesthesiologist was in my face with a mask telling me to count backwards from 100.
Suddenly, I was being woken up, “Mr. Mallory, Mr. Mallory wake up, the procedure is over”, they were repeating loudly! I hate being awoken after surgery. What an obnoxious way to wake up! I half way opened my eyes and quickly closed them again. The next thing I knew, several nurses were pushing the gurney back to my smiling wife and awaiting Recovery Room. I had to lie there for four hours to make sure I was okay.
It did not matter to me because I was extremely sleepy. A half hour later, I started waking up and slowly opened my eyes.