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anyone know someone with HSP?

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  • anyone know someone with HSP?

    I have hereditarty spastic paraparesis, and can't find anyone else on other boards who has the disease. Anyone here have it?

    Basically, HSP is a neurodegenterative disorder that you are born with, but get symptoms for during your late teens/early 20's. It causes your upper motor neurons to degenerate over time. Eventually, it affects your ability to walk and you have to use a wheelchair (this is where I am right now). It also affects your digestive system, bladder/bowels, you get spasticity/spasms a lot, and abnormal gait (I walked on my toes since I learned how to walk, and now I have to use AFO's to keep my feet flat on the ground). Its a rare disorder that only affects about 1-2% of the US Population, according to a website I read.

    Jessie

  • #2
    Hsp

    Hi Jesse:
    There are approximately 20,000 people with HSP in the US. There is info. on line and other support groups tailored for those with HSP. It is rare but work is being done to find a cure. I have the pure form of HSP. I have a baclofen pump but also take oral valium to help settle the rouge spasms. It can also cause pain which I am battling right now. I do not remmber seeing your name at the HSP support groups but if you take a look they are not too hard to find.
    Just a bit of info on me. I have AFO's to help with foot drop but hate wearing them. I have a baclofen pump and had three revisions last year to fix a kinked cath. Now this year I seemed to have developed a sort of weird reaction to the intrathecal drug. I can't be increased more than 5% or I suffer with pain and spams-no doc can give me a reason why and I go to a research facility where a lot of pump work is done. It does help and does not make me tired like the oral drug. I walk with a cane 90% of the time, but have forearm cutches and a rolling walker for bad days. i also just got a scooter since I know someday I will probably need it since the diesase is progressive.
    I have gone on a bit. Hope you find support. Maybe you have found the other groups and I have skimmed over a bit too quick.
    Best to you. Willow5

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    • #3
      Thanks Willow for the information on HSP. I met one man in his late 40's about 8 years ago with it. He needed a wheelchair at that time, also had spasms but no pain complaints I remember. He testified before our state assembly on the SCI Research bill and I lost his info and he hasn't contacted me since the signing ceremony in 2000. He did bring up that it is listed in the NORD so you might try searching there. NORD = National Organization of Rare Diseases
      Courage doesn't always roar. Sometimes courage is the quiet voice at the end of the day saying, "I will try again tomorrow."

      Disclaimer: Answers, suggestions, and/or comments do not constitute medical advice expressed or implied and are based solely on my experiences as a SCI patient. Please consult your attending physician for medical advise and treatment. In the event of a medical emergency please call 911.

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      • #4
        Hi willow and jessie-

        I was not diagnosed with HSP...but with PLS --> Primary Lateral Sclerosis (I believe there is a site that works on both HSP and PLS). I think our symptoms are similar.

        In any case, just wanted to say hi.

        wheelie
        "Unless someone like you cares a whole awful lot nothing's going to get better. It's not." - Dr. Seuss

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        • #5
          Originally posted by jessie.gray
          I have hereditarty spastic paraparesis, and can't find anyone else on other boards who has the disease. Anyone here have it?

          Basically, HSP is a neurodegenterative disorder that you are born with, but get symptoms for during your late teens/early 20's. It causes your upper motor neurons to degenerate over time. Eventually, it affects your ability to walk and you have to use a wheelchair (this is where I am right now). It also affects your digestive system, bladder/bowels, you get spasticity/spasms a lot, and abnormal gait (I walked on my toes since I learned how to walk, and now I have to use AFO's to keep my feet flat on the ground). Its a rare disorder that only affects about 1-2% of the US Population, according to a website I read.

          Jessie
          Hi Jessie,
          Sorry, I've only just noticed this forum/your post.
          I was diagnosed with HSP approx 7 years ago, and while it is progressive, people with HSP are not 100% destined for a wheelchair. The symptoms can manifest at any age, from childhood to middle age onwards. I'm lucky in that I've been told by a senior neuro that my HSP is unlikely to get much worse (if any) than it is now at 48 years of age. I'm now registered disabled, tho' I am still relatively mobile, I just need a cane to get about these days. There are roughly 26 known neurological disorders, all lumped together under the name HSP, hence the difference in 'some' symptoms from person to person.
          You might want to join the Yahoo HSP support group http://health.groups.yahoo.com/group/HSP_friends/ Pehaps not the most active of sites, but considering how rare HSP is, that's understandable. There's lots of useful info there, and it's always nice to post and get replies from people who understand what HSP is/can be like.
          Personally, I still dispute I have HSP, I have certain symptoms that simply don't fit, but my present neuro maintains I do have HSP, and says it's also likely I have a secondary, albeit idiopathic, neuro disorder.

          Ian

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          • #6
            Hi Wheelie,

            New member here. I too have PLS, not too many of us around, and yes mine too was confused with HSP/FSP as they are quite similar I'm told.

            I'm looking into a baclofen pump. I have a few questions and post here earlier. Know much about them?

            Thanks,

            Mike

            Or I guess I should ask of everyone PLS, HSP whatever, about a baclofen pump.

            I'll be seeing a pain management doctor 2/1 in regards to a baclofen pump. My neuro says that getting a baclofen pump is the best chance I have of staying able to walk, the little that I do, I already use a wheel chair most of the time.

            My biggest question, concern, is will I feel the pump and will it bother me?

            I'm rather on the slim side. 5' 11", but only 155. I stil try to do a lot of streching and sit ups. I have a bad back, all the sitting I do, so my chiropracter encourages me to do sit ups and back strengthening exercises as much as possible. I was a nationally ranked triathlete 15 years ago, and I love working out, except that now days my Primary Lateral Sclerosis, my spasicity and mobility don't allow me too. But I would if I could.

            I assume that my mobility and walking may very well improve with the pump and if so I'd like to be able to start working out to some extent, but will I be limited in working out, weigts, stretching, stomach and back exercises?

            Thanks for your help,

            Mike
            Last edited by Mike Herman; 01-22-2007, 09:39 PM.

            Comment


            • #7
              Mike, you are new to our forums, so you may not be aware that we ask people not to double post the same information or questions in multiple forums. You can find information about your quesiton from your original post in the Care forum. Your previous duplicate post here was removed.

              (KLD)
              The SCI-Nurses are advanced practice nurses specializing in SCI/D care. They are available to answer questions, provide education, and make suggestions which you should always discuss with your physician/primary health care provider before implementing. Medical diagnosis is not provided, nor do the SCI-Nurses provide nursing or medical care through their responses on the CareCure forums.

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              • #8
                I have HSP too!
                Hey Jessie I noticed your posting and wanted to reply. I have autosomal recessive hereditary spastic parparesis and have also been interested in finding out if there are many other people with a condition similar to mine.
                I was diagnosed about eight years ago and am seeing Doug Kerr at Johns Hopkins. Presently, I also use a wheelchair, have crutches and walkers, AFOs and wear Dyna Splints for the spasticity in my legs at night.
                Cheers,
                Chuck

                Comment


                • #9
                  Hsp

                  Hello,

                  I am also afflicted w/HSP, my Dx was almost 5 yrs ago. My other posting gives more details. On a long & winding road w/my 3rd pump in 4 yrs- currently having all sorts of problems trying to diagnose what I know is a leak, but every scan/procedure tells a different story. Past the pt of frustrated!

                  Serendipity
                  *Serendipity*

                  Comment


                  • #10
                    Originally posted by *Serendipity*
                    Hello,

                    I am also afflicted w/HSP, my Dx was almost 5 yrs ago. My other posting gives more details. On a long & winding road w/my 3rd pump in 4 yrs- currently having all sorts of problems trying to diagnose what I know is a leak, but every scan/procedure tells a different story. Past the pt of frustrated!

                    Serendipity
                    Hi Serendipity You might want to start a thread or PM SCI-Nurse "EMK." From the response below, he or she obviously is very experienced with baclofen pumps. Best of luck:

                    /forum/showthread.php?t=4720

                    Rob,
                    I am currently managing 85 patients with baclofen pumps and can give you my thoughts on your situation.
                    When patients present to us with symptoms such as yours, we would do the same thing....increase dose. A 10% increase is very conservative however, and up to 30% is very much within reason. However, when someone like yourself has had several adjustments and has little benefit, I think it is time to do a diagnostic dye study. Fractures and kinks don't always show up on plain xray. I hope you have a pump with a catheter access port. This is the best way, in my opinion, to do a dye study. Once you access the side port, if you can't draw CSF back easily, you already have your answer. The catheter needs to be replaced. Often the problem is right in the pocket and can be fixed without having to re-open the back incision. If CSF freely aspirates into the syringe, then injecting contrast (dye) may show a small hole or fracture in the catheter.
                    When you have your pump refilled, is the amount which is aspirated from the reservoir equal to what the computer says it should be? More drug in the reservoir than expected can indicate that the drug isn't being delivered correctly and points toward a kink in the catheter. If everything checks out with the dye study, then I would bolus dose with 50 mcg through the pump. This is the same as the screening trial dose and you should see a significant change in your tone. If that is successful, then you probably should be brought up by larger increases than you have been. I have several SCI patients who are over 1000 mcg/day and need this much to maintain. The other thing which works very well for some people is intermittent bolus doses. We had a young woman who didn't seem to respond to increases no matter what we did. We had done all the dye studies and everything checked out fine. We then put her on bolus doses every 4 hours and her tone has been under control ever since. We usually take 60 - 75% of the current dose and divide that amount into 4 - 6 doses over 24 hours.
                    If you have a dye study, make sure the team that is doing it has some experience, or has the Medtronic clinical specialist there to guide them.
                    These are some of my thoughts. I certainly hope you can get this sorted out. The pump is such a wonderful therapy for spasticity when it is working the way it is intended. Let us know how things turn out. (EMK)
                    Please donate a dollar a day at http://justadollarplease.org.
                    Copy and paste this message to the bottom of your signature.

                    Thanks!

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                    • #11
                      Thanks rdk! I guess it's obvious I'm new here. I'm sure I'll learn my way around soon...
                      *Serendipity*

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                      • #12
                        Hi All,

                        This *latest* post in this thread may be of interest for those curious about genetic testing for HSP and are US based http://health.groups.yahoo.com/group...s/message/5953

                        Ian

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