Intrathecal Baclofen Pump Journal
by Bob G.

This page represents an ongoing journal of Bob's experiences with the Intrathecal Baclofen pump as treatment for spasticity for HSP. Bob is 51 and has had symptoms since childhood with a more rapid progression since age 30. The SPF conducted a Baclofen Pump Survey and 31 individuals responded to share their experiences. See Survey Results.

Bob ended his journal in February 2006, after chronicling his experience for five years.

Feb 2001
On Feb 6, 2001, I had my screening trial for ITB therapy. Prior to the trial, I had tried the oral drugs and had the same experiences that others, on this site, have reported--minimal change, if any, in spasticity, and severe drowsiness.

I arrived, was taken to a room, temperature, blood pressure, etc. Dr. Smith stopped by at 9, said "hello", came back at 10, tested me for spasticity, had me lie on my side in fetal position, injected an anesthetic, and injected the baclofen. He came back a little after 12, and tested me for spasticity. The baclofen had worked as anticipated and I was told that I could leave whenever I was ready. I left at 3:30, with still a lot of residual weakness. The strength and the spasticity came back entirely early the next day.

While with me, Dr. Smith made it clear what difficulties I would be facing when using the baclofen pump and the limits of what I can expect. He wants me to come back to him and definitively assert that I want the pump implanted.

May 2001
The next step was to schedule implantation. The date was April 11. I was admitted to the hospital for pre-op testing on April 10. I went into the operating room around 11am on the 11th. The operation was done and I was discharged on April 12.

Walking was tough. I needed my cane for both balance and support. At home the next morning, I had intense pain in the back of my right leg. I got nowhere with the offices of the neurosurgeon or the neurologist because it was Good Friday. So, it was rest until Monday. The pain subsided.

This wasn't what I had expected, because the neurologist told me that I would be ready to return to work on Tuesday. I thought that the weakness was a consequence of the surgery and that it was a matter of waiting to heal.

One pleasant note was that when I laid down and put a pillow under my leg at the knee, as the pain subsided, I felt an exhilarating feeling on my shin, like someone was slowly stroking it with a feather.

Another pleasant sensation was sleeping all night without being summoned to urinate. That has been an annoying issue for the last two years.

Monday, I called the neurologist's office about the pain. The sent me to my general practice physician. The prescription was sodium naproxin and rest. OK.

That Friday, I went to the neurosurgeon's office and had the staples removed. I was told that I should return to work the following Monday. (I teach in a high school).

Monday, I returned to work. It was really tough. I knew that I couldn't put weight on my leg for significant periods of time, my balance was bad and walking was strenuous, but I figured that this would be a temporary price that I would have to pay.

Thursday, two weeks and a day after the operation, I saw the neurologist who was surprised to see the difficulty that I had walking. He then tested me for spasticity and everything became clear. What had happened was that he had intended for my initial dose to be 50 micrograms per day. That was not communicated to the neurosurgeon who set the dosage for 100 micrograms per day. What had happened was that my spasticity had nearly been eliminated, which made walking strenuous and painful. My dosage was reset for 75 micrograms per day.

That's what's in me now. Walking is still strenuous, but it is not painful. Balance is still difficult. I still use the cane for nearly everything. The bladder issue is at some half-way point. I am not summoned to urinate as frequently as I was before I had the pump, but it is not back to "normal".

I shall stay at 75mcg/day. My goal is to be able to walk from my doorway to my automobile without the cane by the end of July. If I can do that I'll stay at that dosage. If not I'll ask to be dropped to 60 mcg/day.

Physical therapy starts tomorrow.

Aug 2001
Unfortunately, in mid-June, the catheter which connects the pump to the spine came out of the spine. I knew that something was wrong because all of a sudden the walking became more sure-footed and bladder issues started to recur as well as difficulty falling asleep because of the buzzing in the legs. Last Wednesday, August 1, the surgeon opened up my back and reinserted the catheter, hopefully more securely. Before surgery, he told me that the probability of the catheter coming out was very small in the first place. Now that it is in more securely, the probability of it coming out again should be very close to zero. The dosage was set to 50 mcg/day which is what it should have been back on April 12.

What have I learned that I can share? First, make sure that if you go with ITB therapy, stay close to home. I live near White Plains, NY. The doctor who recommended the pump is in Staten Island, NY, an hour and a half away. I would have hated making all those trips, so I'm glad that I found a local neurologist that knew something about ITB therapy.

Second, I've gone from no baclofen to 100mcg/day to 75mcg/day to none to 50mcg/day. Each of us is different. For me I have three spasticity measurements, walking, bladder control, and ease of falling asleep. With regard to walking, I walk best when I'm spastic because that's how I've learned to walk. The more baclofen, the more strenuous walking is. Hopefully with physical therapy, walking will become less strenuous as I build the proper muscle. With regard to bladder control, which only became a problem two years ago, there is no problem at all at 75mcg/day or more, and now, at 50 mcg/day, it's just a small issue. The same is true with falling asleep.

Thirdly, recovery isn't only nerve and muscle. There is also something intellectual involved. In the days immediately following the revision of the catheter, I was summoned to urinate, when there wasn't much in the bladder. I realized that one part of the brain was telling me to urinate and another part was telling me that with the baclofen, I really didn't need to urinate. So, I took my chances. Eventually the part of the brain that tells you that you have to urinate learns that it was reacting incorrectly and changes it's behavior. Similarly, a little at a time, falling asleep is getting easier. Walking may turn out in similar fashion, but over a much, much longer period of time.

I'll comment on physical therapy in another journal entry.

November 2001
This is my fourth entry to my intrathecal baclofen therapy (ITB) journal. To recap, I had the screening trial on February 6 of this year. the pump was implanted on April 12. The catheter came out of my spine in mid-June. It was put back and secured on August 1. My current dose is 60mcg/day.

One of the things to consider is getting the reservoir in the pump refilled. I thought that this would be a tremendous hassle, with me schlepping every three months to my neurologist's office. It turns out to be much easier. Since most people who use the baclofen pump are less mobile than I am, there exist infusion services which come to the patient's home and drain and refill the reservoir. Without the paperwork, it's less than a half hour.

That's the good news. I am also lucky enough to be in a health plan where these services are provided, so it's just a matter of scheduling. I've read postings here on the list where people have to deal with paying for this service. I don't have a clue how much it cost, but it must be expensive. So it pays to make sure that you have a way of paying for infusion as part of the overall cost structure of having the pump.

Another thing to consider is physical therapy. When the spasticity is greatly reduced, the patient is greatly weakened, because he now has to use undeveloped muscles. To develop these muscles you need physical therapy. Once again, I am fortunate to have a medical plan which pays for forty visits per year. (The previous terms were 25 per year, and when I reached visit 20, the terms were renegotiated up to 40). But that may not be enough. I will be covered for the rest of this year, but at the rate of two visits per week, I will use up my forty visits in the first twenty weeks of 2002. I will have to think up some alternative way of doing things and when I do I'll report it here. The cost of physical therapy and the time for the visits and the time needed for home exercise must be considered before having the
pump implanted.

Now, about the weakness. I teach mathematics in a public high school. Teaching, and all the preparatory and administrative running around, involve a lot of time on one's feet. It was tough before I had the pump. It is much tougher now. Because I am slower, as I am learning to walk with different muscles, and relearning how to keep my balance, I do less than I did before, and use much more energy doing it. At the end of the day, I find that I have accomplished much less then I used to and that I am feeling physically exhausted. The students are doing poorly as a consequence and I am feeling demoralized. I have brought this to the attention of my supervisor, who has brought it to the attention of the principal. I have a meeting with him on Friday. I'll leave you in suspense until my next journal entry.

February 2002
In my last journal entry, I described how the weakness, resulting from the fact that I need to use muscles that are only poorly developed, was effecting my work performance. (For those who want to see my prior journal entries check out sp-foundation.org). I teach math in a high school. My supervisor, the principal and I met in early November. My wife suggested that I take a medical leave of absence for the purpose of rehabilitation. I have 126 cumulative sick days. It was agreed that with my neurologist's blessing I would take the leave. The neurologist wrote the appropriate note. On the Tuesday following Thanksgiving I started my medical leave.

The first thing that I found out was how incredibly fatigued I was. I made certain that I did two hours of quality exercise each day, at 8am and 2pm, but apart from that, I spent 20 hours/day lying down around 16 of them sleeping. That lasted for about the first two weeks in December. Then I started to lie down a little bit less and to sleep a little bit less. Now I sleep about 12 hours/day.

Another issue was pain. Because I had been leaning on a cane, which I held in my left hand, my left hand, arm and shoulder and my neck were in pain. Also, because the left leg was bearing the rest of the weight, I had pain in the lower back on the left side. The pains in the arm have slowly subsided as a consequence of taking naproxen sodium, of not being on my feet, and of massage therapy. Here in the beginning of February the pain in the arm is gone. The lower back still hurts.

But there's another element to the pain issue. In order for the pain to not return, I need to walk without the cane. I walked without the cane until December 1999. At that time I did not have the pump and was spastic. (I started using the cane because I slipped on the ice and tore a muscle in my right thigh, and I wanted to take the weight off that leg). Now I need to be able to walk without the cane, with the pump and much less spasticity.

Part of being able to walk is strengthening whatever it is that I need to strengthen. For this I put my complete trust in physical therapists. My exercise routine is comprised of different things that physical therapists told me to do. As stated above, I have exercised religiously twice a day. At the beginning of December my physical therapist told me that we had gone as far as I could go until I got my ankle-foot orthotics (AFO). Coincidentally, we had arrived at the last PT session that the health plan would pay for.

I had worn orthotics beginning somewhere around 1991, until spring of 1999, when my physiatrist felt that my spasticity was "fighting" the brace. Screws kept coming out. The instep strap would work its way loose, etc., etc. He put me in orthopedic shoes. Last summer, when the physical therapist recommended AFO's, I wasn't going to do it until I got the blessing of the physiatrist. The physiatrist wasn't aware that I had the baclofen pump implanted. When he saw the consequences of my having the pump, he wrote a detailed prescription for the physical therapist and for the orthotist. It took until early January for me to get the orthotics, properly adjusted. Now I was ready to resume PT, but had to wait another week for my first appointment.

So, during the period from about Dec 10, 2001 to Jan 20, 2002, I'm home from work on medical leave, I'm not going to physical therapy, I am exercising two hours a day and resting the remainder. The mind has had enough rest and it starts getting restless, and I'm thinking about becoming more knowledgeable and more "activist" in my rehabilitation.

I know that (1) I am much less spastic than I was, (2) the muscles that I need to use are weak, (3) muscles that I used when I was spastic are abnormally strong, and (4) my method of walking is a bad habit. How do I change? There is really no roadmap, that I am aware of, that tells us how to do this. The HSP resources on the web don't have much about rehab. Where do I go?

There was a show on PBS' Scientific American Frontiers having to do with spinal cord injury, which had I thought might be useful and I had purchased the videotape. I watched it a segment at a time and went back and forth to the PBS website to find out more. I followed their hypertext links to other sites and I threw names and phrases into search engines to find out more.

What did I find? I found that my (or should I say "our") rehabilitation is on the cutting edge of science. At the November HSP conference in Philadelphia, I asked Dr. Fink the question stated earlier, or something roughly approximating it. His answer was, to the best of my understanding, to develop the muscles that promote hip flexion, and that everything else takes care of itself because the stepping mechanism is programmed into the spine. If you properly stimulate an unconscious cat's spine, its legs will move as if walking indicating that the walking mechanism is not stored in the brain. A serendipitous discovery at the Miami Project to Cure Paralysis led researchers to believe that the same is true with humans. Since then, they have been experimenting with trying to wake up or restimulate this "central pattern generator". However, the difference between us and spinal cord injury patients is that they were walking just fine and then all of a sudden they weren't. We, as our spasticity worsened, learned to walk to accommodate that spasticity. We learned how to walk "incorrectly". Isn't that incorrect walking pattern the one that is programmed into our central pattern generators?

PBS also had a show on Nova about the work of Dr. Ramachandran. This brought me to the library where I borrowed his book "Phantoms In The Brain". While there, I borrowed John E. Dowling's "Creating Mind". Ramachandran mentions a stroke patient who smiles when a friend enters the room. When the same friend asks him to smile for a photo all he can generate is a contorted mouth. Why? Because the first smile is generated by the inner ganglia of the brain which was not damaged. The "volitional" smile, for the camera, is generated by the motor cortex, which was damaged. Now, when at PT, the physical therapist asks me to walk on a treadmill with my foot landing heel first, I can do it 100% of the time as long as I am thinking about it. The minute I think about something else, I land toe first. Aren't I the converse of the stroke patient? So maybe there are two parts of the nervous system involved in gait control. The central pattern generator maybe isn't the only thing involved. Isn't my gait training like a teenager learning how to ice-skate? Does that learning take place in the central pattern generator or somewhere else? Dowling says that motor learning is programmed in the cerebellum (for things like riding a bicycle or rowing a boat). No matter where the learning is though, I am not learning from scratch. I need to break bad walking habits developed over the last fifty years. How is that done? Nobody seems to know.

May 2002
In my last journal entry, I had reported that at the end of November, I had started a medical leave of absence from my teaching job for the purpose of rehabilitation, that I had resumed wearing AFO's and that I had resumed physical therapy. It is May and I am still on my leave of absence. My goals were to eliminate pain, build strength, improve my balance, improve flexibility and develop stamina.

The pain dropped by 60% in the first month, and at the end of January I was dealing with only one-tenth of the pain that I had at the end of November. The pain was gone from my left hand, arm and shoulder. (This pain was due to leaning on my cane). The pain that lingered was in my lower back on my left side and was much less than it had been. I was confident that in due course it would be gone. That didn't happen. During March, there was increasing pain in my left quadriceps, the pain in the lower back increased, and I started to develop pain in the left hip flexor. This kept going on. I discovered part of the reason for the pain in the quadriceps. At physical therapy I was working on a jogger, a cardio-vascular exercise machine. I discovered that using it the way I was using it was counter-productive to my goals and general health. I found that if I concentrated on putting the weight on my heels as I stepped, rather than on the front of my feet, that my gluteal muscles and my hamstrings were getting the intended workout. In my spastic gait pattern, because the gluteal muscles and hamstrings are weak, the wait falls on the front of the foot, the quadriceps does the work that the glutes and the hamstrings should be doing, and gets developed, and also gets tired and sore. Once I discovered this, I changed what I was doing and concentrated on putting the weight on the back of my foot. The pain in the quad didn't get worse, but it didn't get any less. Then, fortuitously, I found out what else was contributing to the pain. I had read that lying on one's chest while sleeping is bad for the back. I cannot lie comfortably on my side. So, I have been laying on my back. But if one spends the entire night on one's back, night after night, he is constantly stretching the quad and it never relaxes. I started sleeping with a pillow under my legs and within days, the pain in the quads went away. I am still suffering with pain in the left lower back and left hip flexor.

In the beginning of December, I was discharged from physical therapy, being told that I had done all the strengthening I could do until I got my AFO's. I continued my home exercise routine, got my AFO's in early January and returned to physical therapy in mid-January. I had been mislead. When I came back, I found out how weak I was. I also began to address the issue of range of motion. I got on the right track with more stretching and strengthening, but I began to realize the magnitude of the task that lie before me. By the beginning of March, I had made significant measurable progress in terms of strength and range of motion, but there was so much more to do. There were muscles that I didn't have a clue how to use or how to develop, for example, muscles that are used to move the pelvis. Also, when it comes to walking, I may have increased my hip flexion and my dorsiflexion, and I may have strengthened my gluteal muscles and hip abductors, but turning that into walking involves learning how to put weight on one foot at a time, how to land heel first, how to get you leg to move without circumduction (when it seems like the thigh is welded to the pelvis at the hip) and many other things. Because there wasn't improvement in functional areas, I was discharged from physical therapy in early April.

The balance issue is still unresolved. There is little point in working on flexibility until I have balance.

I joined a health club. The exercises that I learned while going to physical therapy I do on alternate days at home. They take two and forty minutes, net of breaks. On days when I don't do the home exercises, I go to the health club, working on an EFX machine, and various Nautilus and Nautilus-like machines.

When I realized that strengthening, all by itself, was not going to bring me to stepping, I needed to learn how to change physiological behavior. I have started working with an Alexander Technique teacher, whom I now see once a week. Where I was doing my physical therapy, they started to include proprioceptive neuromuscular facilitation, which I thought was great. But then they slammed the door on the treatment because my stepping hadn't improved quickly enough.

Right now, I am unhappy. I haven't lost any pay, because of all the accumulated sick leave that I had. But if I had to return to work tomorrow, I would be feeling that I have accomplished very little. The pain would probably come back. I would not have the time or strength to do all the quality exercise I do now. I would make best efforts, hoping that if I couldn't further improve strength and range of motion, that at least I wouldn't go backwards. I will have to return in September. I do not know where my rehabilitation will be then. I am not optimistic.

What does this mean to people who are considering having the pump implanted? It means that the rehabilitation strategy should be planned in detail with a neurologist, a physiatrist and a physical therapist. It should begin with an assessment before the pump is implanted, to see what kind of work can be done at that stage. It should include an education in the relevant aspects of physiology. It should include training to develop a kinesthetic sense, so that a person can learn to understand what his body is "saying" to him. (This has been a major shortcoming in my rehab.) An effort should be made to develop muscle, where needed, where the muscle can be isolated. I have found that if we ask a muscle to do a task, and the muscle is too weak, the central nervous system will find another muscle to do the task. In so doing we develop the wrong muscle and worsen our condition.

Once the pump is implanted and spasticity is reduced, another, more thorough assessment should be done. Something that hasn't been done with me, but may be considered, is experimenting to find what baclofen titration would be optimal for rehabilitation. The rehabilitation strategy should be modified. In addition to customary strengthening and range-of-motion work, the following should be considered: Proprioceptive Neuromuscular Facilitation, Neuro-Developmental Treatment (Bobath concept), Alexander Technique, working with body-weight support and/or with functional electrical stimulation. Add to this list anything that you have found that I don't know about, maybe Dr. Taub's strategies with stroke patients at UC, Irvine, for example.

Also, when we find out what works and what doesn't, let's share the information. Your eyes are probably tired from reading this message, so 'bye for now.

Aug 2002
I have now had the pump working without interruption for a year. I am a teacher, who had a lot of accumulated sick time and was able to take the period from Thanksgiving, 2001 to the present off for rehabilitation. I needed it. Anyone whose job is somewhat physically demanding, or who is raising children, will find the rehabilitation work nearly impossible.

One positive development is that I found a professional who has taken a "personal" interest in my rehab. She is an Alexander Technique teacher. I recommend Alexander Technique, but I don't recommend anything exclusively.

Alexander Technique attempts to make the client aware of misuse, and aware of alternative ways to do "ordinary" tasks. The teacher also brings in things outside of AT to help in my rehabilitation. This work is all being done outside of managed care and I am being charged a reasonable amount of money.

Managed care physical therapy has its limitations. Even though a physical therapist may take a personal interest in a patient, one hour per week just isn't enough, and the PT is constrained by the way the managed health care machine operates. Because it is very results-oriented, it favors short-term solutions, which in my case, would be counterproductive in the long run. Alexander Technique calls this end-gaining.

But the point to be made is that it is necessary to find someone who takes a personal interest in you, be it a neurologist, a physiatrist, a physical therapist, or whoever, and one may probably have to go outside of managed care to find such a person.

Another positive development is that, at long last, I have developed and improved my kinesthetic sense. For years my massage therapist has been saying "Listen to your body". I would grunt to the affirmative, while thinking to myself that I can hear my body talking, but I don't understand what it is saying. When I felt something I didn't know whether I had hurt myself, or whether I was in the process of hurting myself, or was sore from working too hard, or just getting a good workout.

I have started to gain an understanding. It has taken months of rehab work, but things are starting to click in this regard. Had I had this sense at the outset, I would have made fewer mistakes in my rehab program. A kinesthetic sense is to physical rehabilitation as learning to read is to academic education.

A symptom of HSP that for me is profound is the way that the thighs seem to be welded to the pelvis at the hip, and that the torso is used to lift the legs. I am breaking out of this.

I walk with two canes. Walking with one or with none looks more impressive but it was doing more harm than good. My hip flexor was in perpetual pain. With two canes my entire foot lands on the ground, 95% of the time, with heel strike, followed by weight distributed over the arch and the fifth metatarsal, passing on to the front of the foot and the toe, whence it is lifted. The right arm goes forward with the left foot, and the left arm with the right foot. The spine is lengthened and there is a feeling of broadness across the back from shoulder to shoulder. This is all part and parcel of getting the legs to move independent of the torso. I have been doing a lot of work on the proper way of sitting down and getting up from a chair.

This is all working well because most of the time that I move I make conscious efforts to move correctly. This will be hard to do working in front of a classroom in September. When not thinking about what I'm doing, I will lapse into bad habits. How will this be avoided? I was introduced electronically to a professor of physical therapy, who I met this past May at the Connecticut Connection. She suggested to me that I teach from a scooter. I am on the path to obtaining a scooter or a motorized wheelchair, not for the purpose of mobility, but for the purpose of not developing bad habits. I will report on this in a future journal entry.

My baclofen titration has remained level since January at 70mcg/day. Things are working well with the AFO's. Because plantar flexion is limited with the AFO's, but is necessary for the swing foot to land properly and take weight, I no longer wear shoes with an ordinary heel. Rather, I am wearing New Balance sneakers. They work wonderfully.

November 2002
I am a teacher and I went back into the classroom at the beginning of September. After dealing with some technical problems, I started teaching from a power wheelchair, using a laptop computer and a projector to "write" at the board. It worked fairly well until the laptop was stolen. Now I am writing at the board with chalk, while seated in the wheelchair. It's not as effective, but until the administration replaces my laptop, or gives me a secure place to put my own replacement laptop, that's the best they are going to get. I also have two classes of less-blessed students. For them, the wheelchair is a distraction which takes them off task, but if the wheelchair weren't there, they would find something else to take them off task.

When not in the classroom, I am still walking using two canes. I am still receiving instruction in Alexander Technique (AT). My exercise routine includes a walk each day except Wednesday and Thursday, twice a day on Saturday, in which I concentrate on AT principals and things I have learned about stepping. Twice a week I go to the gym, spending thirty minutes on the Stair-master and about thirty minutes on Nautilus and similar machinery. Three times a week I do an hour's worth of exercise I learned in physical therapy, and three times a week I do an hour's worth of AT work.

Progress is slow, but every once in a while there is a surprise, when something is all-of-a-sudden easy. Usually I cannot replicate the task with the same amount of ease, but I am confident that some day I will. My main learning task now is to get the limbs to move independently from my torso, and independently from one another. Little by little, I am succeeding.

February 2003
As I suspected, it is difficult to keep up the rehab regimen that I had given myself while on medical leave. Fortunately, I am not sliding backwards, but am making slow gradual progress. I go every Wednesday for my Alexander Technique (AT) training. I recommend it highly to those who, like me, are trying to learn to move without spasticity, after being spastic most of their lives. Every Monday and Friday I go to the health club, working on the StepMaster and on the Nautilus machinery. Every Tuesday and Saturday, for a little more than an hour, I do exercises that I learned at Burke Rehab, modified by AT. Every Thursday and Sunday, I do AT work for about an hour. I would like to walk every Tuesday, Thursday, Saturday and Sunday for about forty minutes, but snow and below freezing weather discourage that.

Hip flexion and ankle flexion have improved noticeably. Glutes and hamstrings are stronger. The top of the spine is being held in the proper position. I sit with my feet flat on the floor. The pelvis isn't held as far back as it was. These and other things keep me encouraged to continue.

I have read selected portions of "Principals of Neural Science" by Eric Kandel, et al, in an effort to find out how the brain learns new muscular behavior. To sum it up in three words, with minor oversimplification, they are "practice, practice, practice".

I can't close this journal entry without comment about the annoyance of getting the right baclofen dosage. It seems that neurologists want you to be just on the brink of comfort without actually getting you there. I've been there, but after a period of time the dosage needs to be adjusted upward, and asking the neurologist to prescribe that, is like a Jew of ancient times asking Jesus of Nazareth to touch him and heal his malady.

May 2003
I have continued the rehab regimen that I had given myself. During the winter months, the cold and dampness gets into the bones, joints and muscles, which makes the workouts harder and compromises the quality. As things get warmer and dryer, I believe that I will make more progress.

With regard to the strategy of keeping the baclofen dosage steady and treating spasms with Valium and urinary urgency with Ditropan XL, it kind of works. I take Valium very seldom, maybe once a week. I'm taking 5mg of Ditropan per day. It works, but it's not "comfortable".

Last year, my neurologist, Dr. Smith, had casually recommended that I see a physiatrist who specializes in neuromuscular rehab. During the winter, I asked for his name and phone and called and made an appointment. I saw Dr. Ragnarsson in March. I had expected him to make recommendations with regard to physical activity. His recommendations were to do a lot of walking, which I do, to lose weight, which I could have told myself, and to supplement the baclofen with botox injections, which surprised me. He was about to refer me to a colleague. Because of transportation issues, I told him to forward his recommendation to Dr. Smith and we would look for someone closer to home.

Dr. Smith was as surprised as I was. He thought that it made more sense into increase the baclofen than to get the botox injections. I said that I wanted to follow Dr. Ragnarsson's recommendation so that I could hear his next recommendation. There is no one locally that could give me botox, so Dr. Smith referred me to the same doctor that Dr. Ragnarsson was going to refer me to, Dr. Simpson.

I made an appointment with Dr. Simpson. I had to make a generalized appointment with him before he would administer botox. When I went to the appointment, Dr. Simpson agreed with Dr. Smith, that I should increase the baclofen rather than get botox. So, I shall contact Dr. Smith, get my baclofen dosage increased, and then make an appointment with Dr. Ragnarsson and get his next recommendation.

Note from Bob a couple of weeks later: I made a journal entry earlier this month. I reported that using Ditropan XL to control urinary urgency was not comfortable. I also stated that I would be increasing my baclofen dosage. Two Thursdays ago I did that.

Last Tuesday, I woke up feeling sluggish. As I walked to my car, I wasn't able to raise my legs to step as much as I had been doing. At 7:55, I told my supervisor that I was not feeling well and might not make it through the day. At 8:20, I told her that I wasn't well enough to teach at all and was taken to the health office where I rested until I felt well enough to drive myself home. I did that but on my way from my car to my condo unit I urinated in my pants. To move the story along and leave out unpleasant details, at 5:00 I was in the hospital.

My family practice doctor came to the conclusion that the cumulative effect of my usage of Ditropan since February and the increase in my Baclofen dosage had the effect of relaxing bladder muscles to the point that I couldn't use them to eliminate urine, nor could I use them to retain urine. I was put on a catheter until the effects of the Ditropan wore off to the point where I could control my urination to some degree. I stayed in the hospital until it was demonstrated that I could control my urination well enough to function in an ordinary manner. I was discharged on Friday.

It was decided that I would stay off Ditropan and instead take Hyoscyamine on an "as needed" basis.

One moral of the story is to not hand-wave seemingly minor side effects of a drug. A second moral is to be careful with different drugs being used simultaneously. Maybe that is why Dr. Simpson did not want to augment the Baclofen with Botox injections.

August 2003
My last entry told of my bladder control problem as a consequence of taking Ditropan and getting an increase in my Baclofen dosage. Since discharge from the hospital everything had gone smoothly until last week. More about that later.

I have had no feedback from Dr. Simpson about my blood test (for the spastin and atlastin mutations) nor about the MRI's. About the former, I really don't care. About the latter, I'm assuming that no news is good news.

I had a second appointment with Dr. Ragnarsson. The work I am doing with Alexander Technique has his blessing. I should continue wearing ankle-foot orthotics. He had no other recommendations.

Progress is very slow. When this all started, I wasn't aware of how messed up I really was. Each movement seems to be factored down to smaller movements and I need to learn each of these.

The school year ended. My wife and I took a vacation trip. When I came back I resolved to put in extra rehab work. I started doing roughly four hours per day. I gave myself Saturdays off.

Last Wednesday, there was a crisis. I had nearly finished my work and took a break, so that my wife and I wouldn't be in each others way. At a certain level, I started to black-out. I was playing solitaire, so I was there cognitively. My wife left for work. I tried to resume my exercises, but I couldn't. I was very weak, and I was spastic. I lied down on the sofa. I thought that I had had a minor stroke. My brother's father-in-law and my cousin's husband each had one in the recent past.

After a while I had the urge to urinate. I got up on my canes to walk to the bathroom. It was extremely difficult. I couldn't urinate. I went back to the sofa. Now I thought that maybe the catheter had come out of my spine again. After a few hours, the urge came again. This time, I crawled to the bathroom. I was able to get something out. A few hours later, the urge came again. I crawled to the bathroom. This time I was able to eliminate.

I waited until my wife came back from work. After spending a total of ten hours on the sofa, I was able to get up on my canes and walk, with difficulty, to the table. After tea, a sandwich, and conversation. I got up, ambulated to the bathroom and brushed my teeth, put on my pajamas and went to bed. I woke up Thursday morning and I was 90% recovered. Friday morning I was 95% OK. Now I am feeling fine. Nevertheless, I have an appointment with my neurologist to talk about what happened.

Now I am limiting myself to three hours of work per day.

November 2003

The blood test that was taken in May indicates that I have a gene mutation that causes HSP.

The sudden spasticity event that took place in late July, was related to the urinary crises I had in May. I had a smaller such event in August and yet a smaller one in October. My neurologist thinks that it's primarily a urinary problem, so he referred me to a neuro-urologist. He examined me and said that the urinary problem was just a manifestation of my upper motor neuron problem and sent a report back to my neurologist.

I also decided that I wasn't going to return to that eminent physiatrist in Manhattan. I made an appointment with a less eminent one here in White Plains, mainly for documentation purposes. (The physical therapist, who gave a presentation at the SPF conference, made recommendations, which needed prescriptions.) She agreed with the physical therapist's recommendations. She examined me. We had a pleasant conversation and I am very pleased. I'm going to try teaching on my feet using a Lofstrand crutch, rather than teaching from the wheelchair. I am also getting a new ankle-foot orthotic, to take advantage of my increased ankle flexion.

My Alexander Technique lessons continue. Slow, but steady progress. I now have sufficient flexibility that I can sit on the floor with my legs crossed, like a boy scout by the campfire. I haven't been able to do that for at least ten years.

Bye for now. I'll be reporting back again in due course.

February 2004

As mentioned before, I earn my living by teaching.  In order to avoid getting into bad habits during my rehabilitation, I decided to teach from a wheelchair beginning in September 2002. In November 2003, I tried teaching my first two periods each day using a Lofstrand crutch, rather than a wheelchair. It has worked. I am now trying to do that during my last two periods also. It is working. One of the long-term benefits of the Alexander training is that I am "taller" than I was before I had the pump implanted. At the blackboard my eyes are just below the top frame of the blackboard. I was never at that level before. I will try to become independent of the wheelchair before the end of the academic year in June.

Flexibility has been steadily improving, as has strength. I used to wake up frequently with back pain. That is gone. However, if I sit down shortly after I get out of bed, and then get out of the chair, the back pain manifests itself. The next major category that I want to work on with my Alexander teacher is balance.

I will continue to keep you informed.

May 2004

Further to my last entry, I am no longer using my wheelchair while teaching. I use a Lofstrand crutch in my left hand. For something that involves only a few steps, I don't use the crutch at all.

I also have been mentioning improvements in ankle flexion. This has led to a modification in my orthotic braces. When not in the braces I tend to walk on the "outside" part of the sole of my foot. A wedge has been put on the braces that lifts the outside parts of my feet. What this has led to is slightly improved stability, and to a change in the muscles exercised when I work out in the gym or when I am walking.

There is also something else new brewing, which is a consequence of my improvement in hip flexion. I will post that in my next journal entry.

August 2004

Fellow Spastics,

Herein is my latest entry to my intrathecal baclofen journal, which I have been making entries in since February, 2001. 

In looking at my other journal entries, I have not been making note of my baclofen titration. To review my earlier experiences, when the pump was implanted in April, 2001, the neurosurgeon errantly set the dosage for 100mcg/day. It was adjusted to 75/day. The catheter came out of my spine in June, 2001 and was put back in in August, the titration set for 50/day. That was too low and it was reset to 60/day. During fall 2001 and winter 2002, I was perpetually asking for more baclofen and was given grudgingly small increases. I now have sort of a system when each time my pump is refilled, which is every three months, I get an increase of 12mcg/day. I am at 156/day now.

I use three things to measure my baclofen craving -- clonus, spasms when trying to fall asleep at night, and bladder control. Clonus seldom happens, although it can be set off at times, when a doctor or a physical therapist wants to set it off. I have been given 5mg diazepam pills to take for the spasms. Prior to July 6, I had to take the pill very seldom, once every three weeks. (More about July 6 in the next paragraph.) Bladder control is the most annoying of these things. It seems that my neurologist wants me to be just on the brink of comfort, without actually being there. I haven't had any crisis since last reported in this journal.

Just prior to the May 1 SPF conference in Tarrytown, my neurologist, Dr. Smith, who was a speaker at the conference, asked me to come to his office. It was time for a pump refill. I had phoned in a request for an increase in the baclofen titration. This time the doctor wanted to see me. While I was there he observed the dramatic improvements that I have accomplished in terms of range of motion. He said that the next thing I should consider was reconstructive orthopedic surgery and he referred me to a surgeon. I went. The surgeon's idea was a variation of what Dr. Smith had suggested. I went to a physiatrist for another opinion. She had nothing against the idea. Surgery was scheduled for July 6.

I went. In my right foot, the Achilles tendon was extended, some relatively unimportant tendon was taken out and transplanted as a dorsiflexion tendon, and my fractured fifth metatarsal, which never would have healed had I remained spastic, was put back together. I've been in a cast ever since. It's coming off on August 13. X-rays indicate everything is healing as anticipated. When the cast comes off I'll be like a six-year-old opening gifts on his birthday. I'll report back with a journal entry shortly afterward.

September 2004

Herein is my latest entry to my intrathecal baclofen journal, which I have been making entries in since February, 2001. That was when I had my screening trial. 

In my last journal entry, I reported that I had surgery to have a tendon lengthened, another tendon transferred, and an old fractured bone put back together in my right foot. My foot came out of the cast on August 13. Everything has resulted as expected. The only surprise for me was the swelling of the foot. I couldn't put it in a shoe. It's in a FoamWalker now.

I had maneuvered to get myself into Burke Rehab here in White Plains as an inpatient, after the cast came off, for the purpose of getting physical therapy from a physical therapist who was designated by the American Physical Therapy Association as a "certified neurological specialist". Burke has a high reputation for neurological rehab. However, I went there as an outpatient after I had my baclofen pump implanted in 2001, and I wasn't completely satisfied because it didn't seem to me that the therapy was directed to my specific needs. That's when I went to APTA's website and found out about certified neurological specialists. There are only two in Westchester County NY. They both work at Burke and serve inpatients exclusively.

I went into Burke on August 16. I spent the entire day in bed, having conversations with the doctor, the nurse, the social worker and others. I was given my daily schedule. (Burke works like a finely-tuned Swiss watch. Every person has his or her specific duties. Everyone is on task at any given moment.) I was in the west wing of the second floor, which is purely for patients with spinal maladies (and what a range there was!). Each day I went to a 45 minute class with my fellow 2 west residents. Each day I had a 45 minute session with my physical therapist; I had a 45-minute session with my occupational therapist; I had a 45-minute session with a lesser physical therapist from the pool; I had a 45-miute session with a lesser occupational therapist from the pool. The routine started August 17.

I met the PT who is a certified neurological specialist. She is the supervisor of the group. She assigned another PT to me. She told me that this PT was as knowledgeable as she was, but simply didn't have the credential. I was satisfied. The good news was that I was seeing a knowledgeable PT. The bad news was that I was seeing her only 45 minutes per day, on only seven days, and we couldn't work much on gait because while the FoamWalkers might be good on carpet or pavement, they are slippery on vinyl, and vinyl was all there was at Burke. The pool PT's and the OT's were good, but they weren't teaching me anything new.

The main objective at Burke is to turn inpatients to outpatients. So, I went through that process and was discharged on August 26. However, my PT gave a specific list of PT's to the outpatient PT scheduler, so that I would get someone who met my needs. I saw my outpatient PT for the first time this past Wednesday (Sep 1) and am satisfied with the first session.

While I was at Burke, the swelling in my foot went down sufficiently so that my orthotist, who has some working agreement with Burke, was able to come over and make a mold for my brace. I can't wait until that comes.

Except for the swelling and the scars, my foot looks like a normal foot, and I can get it to step on the ground like a normal foot does. The trick will be to get it to step that way without thinking about it.

Lest I forget, there's another chapter in the Baclofen titration story. I'll leave that for another journal entry.

May 2005

I had thought that at last I found the Baclofen titration that I would stay at. My neuro went along with my request to aggressively step up the Baclofen titration in August and September of last year. I felt that I had reached the right level at approximately 250 mcg/day. Bladder control was better than satisfactory. Balance was improving as a consequence of the Baclofen and learning how to use my new right foot. This lasted into February.

Then things slowly began to deteriorate. I attributed it to cold and damp whether, and to the fact that, in trying to rehabilitate my right foot, I was putting the wrong muscles to work (a mistake that's easy to fall into). April came. The weather was less cold and less damp, and things didn't improve. Then I came to the conclusion that the deterioration was a consequence of spasticity rearing its ugly head.

I asked my neuro for a 24mcg/day increase. He agreed. Things improved significantly, but I wasn't back to where I was before the deterioration started. I went back to my neuro's office to ask for more. I explained that one of the first indications that the spasticity had kicked up was pain in the left foot and in the right knee. He told me that the pain may have caused the spasticity, or that the spasticity may have caused the pain. There was no way to tell which came first, the chicken or the egg, hence he wasn't going to authorize another increase in the titration. I know that I need it.

Once I have the reconstructive orthopedic surgery done on the left foot, scheduled for June 26, I won't be smashing my fifth metatarsal any longer. Hence no pain there. The pain in the right knee is, in part, a consequence of walking without my AFO's and in part a consequence of using the wrong muscles to "wave my foot". (Part of the rehab work for last summer's surgery is to put my right leg on a yoga block and wave my right foot in the same manner that a model may wave her hand on a float in the Rose Bowl parade. I should be using only ankle muscle. The temptation to use other muscles in the lower leg and the knee is sometimes overwhelming.) So, those sources of pain will be eliminated and I can raise the titration with no argument.

'Bye for now until my next journal entry.

November 2005

Fellow spastics,

In my last entry I mentioned my reconstructive orthopedic surgery.

Physical therapy has been working fine. I do not wear AFO's during physical therapy. I hope that I will be independent of them soon. The main issues are building strength and improving balance. What I am most proud of at this moment is that I can get up from a chair without leaning on canes, or the arm of the chair, without using my arms and upper torso for balance. In fact, I can do it with a tray in my hands and a glass of water on the tray.

A new left AFO was made. I decided to not wear the AFO's on Saturdays in October, and my first entire day without the AFO's was October 1 at the SPF conference. So far so good. I have decided not to wear AFO's on Saturdays and Sundays in November. I'll see what happens.

Now the discouraging news. In the past I have reported on days when my legs feel very heavy. It has happened once every three or four months since that crisis I had back in the spring of 2003. When I have reported it to my neurologist, he acts as if I am fabricating a problem that doesn't really exist. It happened again October 15, a Saturday. My legs felt like I had sixty-pound ankle weights on each of them. I went to my family practice physician. We ruled out some alternative diagnoses and agreed that it was most likely a neuro-muscular issue. The physician tried unsuccessfully to reach my neurologist. I found a way to reach him on Sunday. (On Sunday, as in past experience, the weakness was gone.) There were two hypotheses either my baclofen titration should not have been increased, or I had a urinary tract infection. I was to see his associate on Monday. On Monday, I woke up with the weakness. My wife took me to see my neurologist's associate, who agreed to decrease the dosage, and the nurse took care of that. On Tuesday there was no weakness. On Wednesday, I returned to work. On Thursday, while at work, between 8:30 and 9:00, I became suddenly weak. I left work, got into the car, called my neuro on the cell phone, and made an appointment to see him that afternoon. At last we was going to see that I wasn't whistling "Dixie". He examined me and went to his urinary tract infection hypothesis. I was catheterized, although I didn't think I needed to be. It was in the early evening and based on the appearance and odor of the urine he concluded that I had a urinary tract infection and wrote a prescription for an antibiotic. The next day, Friday (no weakness), I called the nurse to find out what the actual analysis of my urine indicated. She told me it was negative, but that she had to wait for the cultures. She would know about that on Monday. I called Monday. The nurse told me that the cultures were negative also. Now what do I do? We'll talk to Dr. Smith tomorrow (Tuesday) and see what he says. He calls me on the phone and tells me to go in for an MRI. Next available appointment was Wednesday, November 2. I returned to work Wednesday, October 26. MRI was done as scheduled. My appointment with Dr. Smith is on November 8.

I will keep you posted. People who have met me will tell you that generally I have a positive attitude. I am very discouraged now. I have made significant progress because I have worked hard to make it happen, but what's the point, when at any random time I can have serious weakness and no explanation for why.

December 2005

Fellow Spastics,

In my last entry I mentioned episodes of weakness in my legs. The MRI didn't show anything that would explain the weakness. So, my neuro recommended a spinal tap. When analyzed, the protein level was above the normative range. It was above by a small amount which meant that nothing was wrong. Such protein levels exist when there is something foreign, like a catheter, in the spinal column. That was the last diagnostic tool my neuro had.

Then, this past Wednesday, I had another episode of weakness. I put myself in the emergency room. My doctor wasn't on duty, but one of his colleagues was. After examining me, and looking over my records, and speaking with me, she said that she believed that I was suffering from exhaustion, that I was simply asking too much of myself. A contributing factor may be that my baclofen dosage is too high. It's the same recurring dilemma. Hereditary spastic paraplegia minus spasticity equals hereditary paraplegia. We rely on tone as a substitute for muscle, but we have to diminish spasticity in order to build muscle. I want to do everything I can to build muscle and be less reliant on tone. I am probably pushing it too far.

The other issue is the day-to-day demands of earning a living. I am seriously concerned that my ability to earn a living is being severely diminished. I will speak with a social worker to find out what my alternatives are.

At the neuro's suggestion, I went to my family practice physician. I doubt that anything will come of it, but I will report back.

February 2006

In previous entries I have been discussing episodes of extreme weakness. These episodes have been happening more often and have been more extreme. To review what has already been stated, my neurologist thought that I was dealing with a urinary tract infection. I wasn't. He thought that my baclofen titration was too high. He reduced it. The next morning the soles of my feet were tingling. We restored the baclofen titration to what it had been. My feet are still tingling. He ordered a spinal MRI and found nothing that could explain the weakness. He ordered a spinal tap and found nothing that could explain the weakness. He concluded that the weakness was not a neurological problem and referred me to my general practice physician.

I saw him. We ran a variety of tests and found nothing. He believed that the issue was neurological after all and sent me to another neurologist.

She ordered a brain MRI and found nothing. She conducted an electromiogram. Something was not right in my legs, which led to blood tests and an electromiogram of the arms. Those tests all came up negative. The last possibility was that I had a spinal dural arteriovenus fistula, which required a somewhat exotic angiogram. The result came up negative again.

There is no explanation for what I have, but both neurologists agree that it is independent of my hereditary spastic paraparesis. So what have I been told to do? I've been told to reduce my baclofen dosage, which I believe is counterproductive, to get physical therapy to reduce a growth in my lumbar spine which should have been causing back pain, but hadn't until recently, and to return to work on a part time basis, which also seems cuckoo. I will do what they say.

At this point it seems that this journal will have no further use to you, my audience, because I have a unique disability which clouds the consequence of using intrathecal baclofen for HSP. I will publish a closing statement tomorrow.

February 2006 - closing statement

Brothers and sisters,

This is the concluding entry to my intrathecal baclofen therapy journal.

It's been a little more than five years since I was tested with the bolus dose of baclofen to see if I would benefit. In my last entry, I pointed out that my path now becomes separate from everybody else's, so here is a good time to give my conclusions to those who are considering this therapy.

First, don't consider it unless you have a good health care plan or are wealthy. The cost of the pump and surgery, the follow-up physical therapy and the infusion service is tremendous. I have been blessed with decent health insurance.

Second, don't consider it if you don't have a significant other to help you through it. There will be a sudden drop in functionality. Someone else will have to do some of what you have done around the house. Hopefully some of that functionality will return. I was looking forward to being able to take out the garbage. I guess that will never happen. My wife, Ida, has been a blessing to me.

Third, don't consider it unless you can still do your job with diminished functionality, or can get a different job. Same as the previous paragraph. I am a teacher. This has truly been a difficult experience. Americans with Disabilities Act accommodations are done in a tokenistic, begrudging fashion. For accommodations, your employer's attitude is more important than any statute. Some jobs simply aren't adaptable to one's decreased functionality, even if it is only temporary.

Fourth, don't consider it unless a physician, or a physical therapist, or a nurse, or some other medical professional takes a personal interest in your treatment, unless you yourself are a medical professional. I feel that I have been short-changed here also. Medical and physical therapy services have become very procedural in this era of managed care. I frequently feel like an automobile in a shop rather than a person. Luckily my Alexander Technique teacher took a personal interest, and it helped immeasurably. Go outside of managed care if necessary and if possible.

If you can still consider IBT after all those caveats, be ready to dedicate at least an hour per day every day to exercise. Unless you build strength, decreasing spasticity is pointless. Unless you are willing to develop new motor behaviors, building strength is pointless. It will take time. Hopefully you won't crash into a brick wall like I have.

Where would I be now if I hadn't had the pump implanted? If I would have followed the same pattern as my father, I would have become more spastic to the point of having to use a walker, or maybe be wheelchair bound. I would have lost bladder control and used the system my father used when that happen to him. I would not have been able to keep my job and would be living in the poverty of disability insurance. I might be there soon.

Has it been worth it? Borderline "yes". The feeling of accomplishment at various stations on the road to rehabilitation was wonderful. The devastation now is crushing.

One last comment. I have heard others say that they will go to intrathecal baclofen once they reach some disability treshhold. That's nuts! A person needs to be pre-emptive. The sooner one gets started the better.

May God be with you.

Bob Gustafson
 

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