Baclofen Pump Survey Results

Note: The Baclofen Pump Survey was an informal feedback survey taken by individuals with PLS or HSP who have used the Baclofen Pump. There is no way to know how these results relate to the entire population of those with HSP or PLS who have used the Baclofen Pump. Results should not be considered any type of recommendation. Please consult your doctor.

There were 31 respondents. Of the 31 survey respondents:

1. Eighteen reported their diagnosis as HSP, ten as PLS, and three as unsure of their diagnosis

2. Over 60% of the respondents are 45 years old or older

3. 57% have used the pump for 4 years or less

4. Four have had their pumps turned off

5. One had the pump removed

6. The main reason for getting the pump was to cut down on the sedative effect of oral Baclofen

7. Two-thirds of the respondents spent less than 4 days in the hospital after the surgery with an average of another 3 days of bed rest at home

Results reported:

1. All respondents reported spasticity in their legs. Most of them experienced a moderate or significant reduction in spasticity with the pump.

2. 13 respondents reported moderate or severe spasticity in their arms. 9 of them reported moderate or significant reduction in spasticity with the pump.

3. 13 respondents reported moderate or severe spasticity in their bulbar muscles. 6 of them reported moderate or significant reduction in spasticity with the pump.

4. Most people with spasms reported a moderate or significant reduction in spasms with the pump.

5. Most people with pain reported a moderate or significant reduction in pain with the pump.

6. Other benefits that stood out included reduced clonus, improved quality of sleep and enhanced self-esteem.

7. Several respondents experienced difficulties with the catheter or pump turning.

8. There were some initial difficulties with some regarding getting the proper dosage, nausea and spinal headaches.

9. A small portion of respondents reported some later problems with moderate discomfort and limited mobility in the area.

Many respondents completed summary statements, listed below. Please note: There has been no attempt to evaluate, organize, or edit them.

1. I will be going on a drug holiday, hopefully to "reset" my CNS. I've developed a tolerance for the Baclofen. Overall I'm extremely pleased with the choice to get the pump.
    

2. It was turned off once for testing desired by the neurologist. After the turn-off, I could hold a cup and drink by myself...could be understood in my speech and generally felt more alert and better. I had life threatening withdrawal due to the dr. turning it off cold turkey with no titration or periactin since my dosage was so small he believed I would not experience withdrawal...boy! Was he wrong!!!

   My life was destroyed by the pump. I know now that other medications such as zanaflex should have been added to the oral baclofen. The program at UAB needed numbers, so I was told there was no other way to relieve the pain and spasms. I was mobile when I entered surgery for pump installation and came out immobile. Life for the last three years has been a living hell. My brother has the same disease and is controlled by oral medication. He has not yet had the pump, and even thought is nine years ahead of me in the disease, is in far better condition.
    

3. I've only had the pump 4 months and am still in physical therapy as well as working on getting the correct dosage. You didn't have a category for "worse" since surgery and I have a number of things that I put in that category. I can't tell yet if it is only temporary as I relearn how to walk. In particular my balance is much worse and my legs feel very weak, however both my doctor and physical therapist feel I now have more strength and I can do higher weights on them than I used to. I walk slower and now use a cane and this was not the case before. Also going up stairs is much more laborious. Going down has always been difficult. In many ways my self-esteem has gone down, both because I was expecting a more 'miraculous recovery' than what I got, and because it is much harder to walk at the moment. I didn't use to take so much effort when I could use the spasticity. My legs do not turn in the way they used to, I can stretch them further, and I look much steadier when I walk and not as 'funny looking'. However, my legs feel very unsteady to me. My guess as to what’s going on is that I've had HSP for over 16 years and they just need to learn how to work again. If the medication is too low, I default to the spasticity. When it is higher, I struggle to walk and get around and must use a cane. As my physical therapist says, "my spasticity is my cane with the lower dosage." My current dosage is 110 mg.

   I still have the restless legs at the end of the day and my legs get very jumpy. I wouldn't say the pump has been successful, but I'm not yet ready to throw in the towel. I am considering turning the pump off for a week or so this summer when I'm not working so I can really judge what it has helped and what it hasn't. Thanks for taking the time to do this.
    

4. Pain is a consequence of improper use of muscle, which is a consequence of spasticity. It is my belief that as a consequence of being much less spastic, I can achieve proper use of muscle, and as a consequence eliminate pain. It won't happen quickly. With regard to "benefits", distance walking, climbing stairs and lifestyle activities have become more difficult in the short run. It is my belief that these will all improve in the long run.
    

5. At time of surgery I was not told that I could not/ought not go into hot tubs/saunas, do deep sea diving, or go into high altitudes (e.g. rock climbing).

   I had a revision done 8 months later because the pump itself rotated and pulled catheter out and catheter was wrapped around the pump like a yo-yo. The rehab hospital I had pump originally done at could not do the revision because of Medicare issues. I was out of work for 2 weeks when I could have been out of work for just one had I known this in advance and gone directly to neurosurgeon who did the original implant.
    

6. I had taken oral Baclofen in two different trials, but had no significant results and noticed an extreme tired with the oral dosages. I am unsure of these past dosage rates. Some others on the HSP list suggested Mirapex and Klonopin for the nightly spasms and I currently take 1.5mg of the Mirapex and 0.5mg of Klonopin. This combo taken at 9PM has greatly reduced the evening spasms for me and even a FULL night’s sleep. Surgery for my first pump was at the Mayo Clinic (Rochester, MN) in 1995 and second pump in 2000.
    

7. If I didn't have the pump I don't believe I would be able to work full-time. I'm tired at the end of the day, but with the oral medication I was drowsy all the time. My pump is set at 500 ug a day, but I do supplement it by taking 20 mg of oral Baclofen at night. I could have the nurse/doctor program a bolus for night, but this also works, and I can control it my use. It took awhile to find the right dosage, but the pump was worth it. I wouldn't have the mobility I have now if I was taking only oral medication. I would recommend the pump to anyone who still has problems when taking oral medication. At my present dosage, I only have to get the pump filled every two months by the nurse, and she comes to my home.
    

8. Some areas were not filled in, as I do not have that information available right now. I am on my second pump, the first was juvenile size and now I have an adult size. It is refilled about every 6 weeks and I would never be without it as long as it is functioning. I love my pump. I have had several major problems and surgeries but that seems to be behind me and all is functioning well. It is worth it in the end. I may not walk significantly better, but certainly feel more comfortable because I have it.
    

9. When first implanted, the medication was much more than I needed. Concentration: 1000 with 200 ug a day. Severe dryness occurred. I was drinking about a gallon of water a day. Thick mucous blocked nasal sinus passages. I visited MA Eye and Ear Clinic and found no relief. It occurred to me that it might be the baclofen concentration that was causing the problem. I had the pump turned off for a week and restarted and reprogrammed. Now I use the 250 concentration with 20 ug. a day. also I had a severe rash or itch on my arms.

   The baclofen relaxes the muscles in lower body; consequently urinary incontinence is a problem. Also sometimes I have a rash on left arm and shoulder and have to apply a cream. Before implantation of the pump, I used to take several aspirin every day to reduce the leg pain. Now, I have no leg pain, but have back stiffness. The leg muscles are somewhat stiff, and I arise from a seated position slowly. I do not have spasticity can stand tall and straight.
    

10. After the planting of the pump, there was no improvement, so dosage was increased periodically assuming that we would find the right dosage and get the expected improvement. The opposite result happened, and, after many increases, stiffness increased I went from cane to walker and off baclofen, started using zanaflex. It was felt that baclofen was not the drug for me. Pump was not turned off or removed, still in place but battery is dead.
     

11. Have had pump for four months, but am still working on correct dosage! Probably my fault - I kept asking for increases & went up to 210 mcgm/day - profound weakness. Now down to 84 mcgm and am asking for another 10% decrease. I want this to work!
     

12. Cannot see any improvement from pump. My walking today is not as good as before pump. This is perhaps a catch 22. Would my walking be even worse if I didn't have the pump? Cannot seem to get correct dosage. Have gone up, then down and now going up again with new doctor. Trying botox next week to try and straighten toes.
     

13. I had my pump put in, in Oct 2002 we are still trying to find the right dose. I could not lift my head because of pain and nausea for 5 weeks after my surgery.
     

14. Implantation of the pump brought immediate relief from drowsiness caused by oral baclofen and reduction in spasms, pain. Although my symptoms have worsened in the last 6 mos., dosage changes have eased that.
     

15. I can tell some horror stories about my pump. Since it was implanted 10/93, I have consistently had problems, mainly with the catheter. Three months after the initial surgery, the catheter got pinched and cut upon entering the spinal chord, requiring surgery. I guess I should have known, but no one ever told me that there was a piece of catheter inside my spinal chord. I found out when it happened a second time, in Sept. of 2002. Up to 10/95, the pump was working fantastically well.

    I was at 600 mcg/day, continuous flow. I could walk short distances unaided, needing my forearm crutch instead of a cane) for long walks. A bad auto accident 10/15/95 changed everything. My body quit responding well to the baclofen. Suddenly, my legs would no longer hold me up. A kink developed in the catheter, which caused the flow to be blocked until enough pressure built up to push everything thru at once, wiping my legs out. After the 3rd time, surgery was finally performed, but I wasn't responding well. In the spring of '01, a kink developed again, and a pump study performed caused a baclofen overdose that almost killed me. I was in a coma for 24 hrs, and on life support for 2.5 days. My docs wouldn't believe there was a problem, and while they tried to figure things out, they put saline solution in the pump and I went on oral meds- Zanaflex, baclofen and neurontin. Those worked for about a year, but on 6/4/02 I had a new pump put in to replace the 2nd pump put in 10/98, after the battery went out on the first one. On 7/30/02, my docs finally believed me that the problem was with the catheter, and a new one was put in. It got pinched and cut by my vertebrae, and on 10/15/02, a new catheter was put in. Right now I'm recovering from total right hip replacement surgery performed on 12/26/02. My HSP has significantly affected and lengthened my recovery time, including 6 days post surgery in the hospital, and 11 days in an acute care rehab facility. Believe it or not, that's the Reader's Digest version.

    I have lots more I'd like to say, but it appears I'm running out of room. I would be delighted to speak to anyone about my thoughts and feelings regarding the pump. I am really glad you're doing this survey. I responded to one sent by Medtronic several months ago, but, not surprisingly, I have gotten absolutely no response whatsoever, not even an alleged newsletter they said would be forthcoming.
     

16. I have a very rare side effect of burning buttocks, which limits how much I can let the doctors turn up the dosage of Baclofen. Other than that I have had fairly good success. The pump has managed to keep me ambulatory somewhat.
     

17. I have just gotten worse. If I knew what I know now, I would not have had the surgery. The drug has really affected my quality of life.
     

18. After about two years of having the pump I am considering having it removed. I have been suffering from chronic constipation as a result of the Baclofen. We have been lowering my dose. The dose is currently at a point where it does not seem worth it to keep the pump implanted. At my highest dose (660 micrograms daily) I experienced significant benefit from the pump. However, I would have a bowel movement about once a week. Therefore we have been lowering my dose.
     

19. I developed meningitis after its removal. Also walking was more difficult with the pump. The legs were very weak. The pump also did not stay in place. I was very frustrated and disappointed with the pump. My condition actually worsened significantly while on the pump and after its removal. I went from walking with a cane to using a walker or wheelchair and being in constant pain.
     

20. At time when battery (unit) needed replacing, I decided to try just saline for a period to see what difference it made. After 60 days I decided there wasn't enough difference in the spasticity to go with a replacement and had it taken out and went back on oral baclofen.

    In trying to reach elevated levels of baclofen through pump we found I developed bladder problems (unable to void) due to the baclofen levels relaxing the bladder and not allowing it to squeeze. This was an ongoing challenge to find the highest dosage that I could still void normally.

    Pump removed twice, I had MRI's done. (Current pumps can be left on during MRI's.) Pump was replaced about a year ago due to its age.
     

21. Baclofen pump has been somewhat helpful and generally hassle-free for me. On 3-4 occasions, the medication has run out sooner than expected and I experienced withdrawal symptoms, which involved major spasms that were alleviated by refilling the pump. As my PLS has progressed, the pump has become less effective. But it still helps keep me loose enough to move around.
     

22. It did not improve my spasticity in my legs nor did improve by ability to walk. With higher dosages I had increased muscle weakness until I felt like I could not stand so I had the pump removed.
     

23. Would recommend this therapy to anyone considering it. I believe my continued walking is directly attributable to the pump.
     

24. Main reason was that trial helped speech and reduced spasticity. My condition continues to deteriorate and I have continued to have frequent increases. My biggest disappointment was I have never reached my test level results.
     

25. I was originally disappointed in the pump because I wanted 100% of my mobility back and did not get it. Things such as clonus, spasms, and being able to at least sleep a little were better. It is when the pump medication gets low that I realize how much the pump does for me. My legs shake so bad that the cane is no longer enough to keep me up and going and I need a shoulder to hold on to. I don’t walk well or quick but I know I would not walk at all without it.
     

26. To clear my length of time in the hospital up, the longevity was due to having fusion surgery done on my spine at the same time. And on the pump, it was not worth the effort, limitations, fears of falling and catheters dislodging, nor the high cost, based on the improvements I felt it brought, or rather did not bring. 

    It affected many areas of life with it. Not actively sexually, but do know this would interfere with the enjoyment based on where it was implanted. Could not use a seat belt while driving because it pressed on the area and was uncomfortable. If an accident occurred I fear it really jolting the pump and perhaps causing a catheter problem. My medical insurance company dropped the hospital that covered me and I had a real problem getting care for it.  None of the hospitals in the immediate area knew anything about it....!?!.  What would that mean if I was traveling and no other facility could help if there was an emergency. On top of all this, it malfunctioned. It stopped giving medication and if it did this, could it reverse this and give too much. I called the Medtronic people and they had no answer for this. I just feel the juice was certainly not worth the squeeze. I was very disappointed with its performance and problems.

    GenComments: On the screening, I believe no one was sure of the effects. If someone has more weakness than spasticity how would they know that more baclofen could make things worse and your legs could become like rubber.  There is not enough knowledge about each form of HSP to put such an intrusive thing in your body without knowing what your form of HSP you have and is it more spastic or more weakness. This is very important and no one has come across that magic formula yet. It's great for some but perhaps their progression is not as bad or has stopped and the pump is just doing as well as oral may be doing. It's too hard a call to make to give it my recommendation for anyone with this rare, complicated, unknown disorder.

    Thanks for the opportunity to discuss my experience with its affects.