Develop a Team 

Most importantly, be sure you choose a health insurance plan that has access to a strong neurology department. Then, having a team on your side is the key to doing well mentally and physically with HSP.  While engaging with family and friends are valuable, so are your relationships with your medical team.  

Part of your medical care team could include: a Primary Care doctor, a Neurologist, a Physiatrist or a doctor of Physical Medicine and Rehabilitation, a Spasticity Specialist, a Neurological Physical Therapist, an Occupational Therapist, a nutritionist, and perhaps even an acupuncturist and/or a massage therapist. 

Having someone assist with your mental health is also a great resource for people with HSP.  Having a psychologist who specializes in neurodegenerative diseases or perhaps a neuropsychologist could be helpful.  On an independant avenue, if you want to improve your resilience skills on your own, try Dovetail Learning for free.  It is an independent way to learn 6 skills including breathing mindfully, letting go, finding gratitude etc. to help ground yourself and strengthen your well being and resilience.  Another avenue that some people find value in is listening to others.  I do not have TicTok, but I can still view Alessandra Notto.  She posts clips about foot drop, fatigue, sleep, blood plasma, and some personal issues while having MS.  Her resilience is inspiring on a personal level.  So, there are a few practical choices for some mental clarity.

Visit a Physiatrist

A physiatrist is a doctor specializing in Physical Medicine and Rehabilitation (PM&R), focusing on injuries and illnesses affecting the muscles, bones, and nervous system to improve function and quality of life. They diagnose and manage pain, support recovery from injuries, and design comprehensive treatment plans that may include physical therapy, medication, and various medical procedures.

I can speak from personal experience. My physiatrist diagnosed my issue within minutes, sent a referral for a nerve ablation, and I was on my way. After treatment, I felt better than I had in years. She also referred me to a PM&R spasticity specialist, which opened even more doors for managing my care.

I once listened to an SPF Talk where a sweet lady said, "Everyone with HSP needs to see a physiatrist." I couldn't agree more.

Several of the injection treatments I reference stem from the SPF Talk, Spasticity Management with Dr. Bianca Martinez MD, FAAPMR, which I highly recommend. Dr. Martinez spoke about helping a patient with HSP through a 'multi-pronged treatment approach' — and that concept is truly crucial. Because HSP involves several things happening simultaneously as it progresses, each element deserves its own thoughtful attention and treatment.

Overactive Bladder for Women and Men

There are several options for overactive bladder remedies, starting with pelvic floor exercises and the Mayo Clinic's lifestyle changes. For women who are postmenopausal, hormone replacement therapy (estradiol and progesterone) can help, or estrace cream used twice weekly. For men and women, the primary prescription treatments include anticholinergics (e.g., oxybutynin, solifenacin) and beta-3 adrenergic agonists (e.g., mirabegron).

There are alternatives to discuss with your specialist.  Some treatments for overactive bladder for women and men include:

• Botulinum toxin A injection into the bladder wall.  It can be done under a local anesthesia.  There is a small risk of passing urine afterwards, thus requiring a willingness to catherize. The effect usually lasts 6-9 months and can be repeated.

• Sacral nerve stimulation involves inserting an implant that stimulates sacral nerves which control the bladder and bowel function.  It requires general anesthesia, as it is a more invasive procedure.  It is generally done in two steps and requires life-long folllow-up.  The effectiveness of this technique is stronger than for Percutaneous Tibial nerve Stimulation (PTNS) in the long term (see below).

• Clam cystoplasty operation expands the bladder using a bowel segment.  The goal is to reduce the effect of bladder muscle contractions.  It is a major operation that requires life-long catheterization and follow up is performed as a last resort.

The nerves of the sacral plexus regulate bladder and rectal function. Percutaneous Tibial Nerve Stimulation (PTNS) uses an acupuncture like needle inserted just above the ankle to deliver gentle electrical stimulation that modulates the sacral nerve plexus.  Treatment typically consists of 10 weekly sessions, each lasting about 30 minutes. Approximately 66% of patients experience significant symptom improvement.  You will need a referral to a Urology or OB/GYN department (many treat both women and men). If you have Kaiser San Diego, you could try Dr. Jasmine Tan-Kim in San Marcos. I am not aware of anyone with HSP who has tried PTNS. I considered it, but chose hormone replacement therapy instead. PTNS may be a good option when HRT is discontinued after age 60.

Injection Treatments for Spasticity

Spasticity injections are more successful when they are aligned with regular neuro physical therapy.   The benefits of intensive physiotherapy are:  spasticity reduction (by using FES to manage stiffness), improves mobility and function (restore walking ability, balance, and coordination), increases strength, pain management (from muscle stiffness and joint issues), preventative care (specialized exercises for gait training to prevent falls/reduce risk) and improves quality of life.  There are also follow-up home exercises aligned with your treatment.

Botox - (botulinum toxin) helps manage HSP by temporarily relaxing muscles, reducing spasticity, alleviating pain, and improving range of motion in the legs.  Muscles that are targeted with Botox will improve muscle tone.  Botox helps with stiff calf muscles, leading to improved mobility, gait velocity, and reduced spasms.  Botox improves:

• Spasticity/muscle tone:  improvement in muscle tone as well as gait velocity and distance for HSP was significantly reduced after Botox treatment.

• Pain and quality of life: Lessening pain improves quality of life

• Fatigue (non-motor benefit):  Fatigue scores  were significantly reduced after Botox injections, which is notable because fatigue is one of the more debilitating non-motor symptpms of HSP.

Botox combined with intensive phsiotherapy can lead to improvement in spasticity and quality of life in patients. Physiotherapy helps maximize the window of reduced spasticity that Botox creates.  There are HSP limitations worth mentioning.  The spasticity is essentially doing two things at once:  causing problems (scissoring gait, stiffness, pain) and providing a kind of passive stiffness that keeps the legs from buckling.  So, Botox can lessen spasticity in the muscles affected by the Botox, making more prominent the underlining muscle weakness.  That is why intensive phsiotherapy is so important - taking advantage of strengthening the underlying muscles to replace the weakness.

A couple of recommended doctors who do Botox for people with HSP are Dr. Christopher Bo at Tri-City, and Dr. Kyle Ryan at Rady Children's Hospital Rehab Medicine SD.  (He sees adults at a Children's Hospital.) 

Cryoneurolysis uses extremely cold temperatures (with nitrous oxide) to create a controlled “ice ball” around a targeted peripheral nerve, such as the common peroneal nerve or one of its branches. The freezing causes degeneration of the axon while preserving the surrounding connective tissue structure. This temporarily blocks the nerve’s ability to transmit signals that contribute to muscle overactivity (spasticity) or pain.

When applied near the common peroneal nerve—where it wraps around the neck of the fibula—the treatment can reduce over-firing in the muscles it supplies. In cases where spasticity contributes to foot drop, this may help decrease muscle stiffness and improve positioning or gait mechanics. The effect is temporary because the nerve gradually regenerates, with its benefits typically lasting from the 3 to 6 month range.

Cryoneurolysis is a newer treatment, so it is more difficult to find a physician who performs it in the USA.  A couple of doctors at UCSD do cryoneurolysis.  UCSD has a strong Neurology department. I looked for cryoneurolysis for HSP and found Dr. Sheng Li – Houston, Texas

• Dr. Li is a physical medicine & rehabilitation physician at TIRR Memorial Hermann in Houston -contact (800) 44-REHAB (73422).

Some other doctors who do cryoneurolysis are at Harvard Spaulding Boston,  University of Kentucky Lexington, and Atlanta Georgia. 

Medicare does cover cryoneurolysis! Have a look in your area.

Chemoneurolysis is a nerve block using phenol to destroy the peripheral nerves in the upper and lower limbs that are innervating the overactive muscles resulting in muscle relaxation and reduction of contractions.  Phenol is a targeted chemical denervation which can be used to treat multiple areas - without the same cumulative dose limitations of botox. Phenol acts immediately where botox usually takes several days for it to take effect.  In addition,  phenol 's effects tend to last longer than botox.  Phenol lasts for approximately 6 months or longer in many cases.  It's also more affordable than botox.  Not as many doctors preform Chemoneurolysis as do botox, so have patience finding a provider.  Try UC San Diego Health, Scripps Health, or other specialized pain/neurology clinics which have comprehensive pain management and neurology departments. 

Chemodenervation with botulinum toxin is injected directly into the spastic or overactive muscles; disrupting the release of acetocholine at the juncture between the nerve and the muscle.  By doing that, it destroys the periphreal nerves that innervate the muscles.  This reduces muscle contractions and relaxes the muscles.  It avoids systemic effects as it is confined to the spacific area rather than being absorbed into general circulation affecting the entire body.  The brand names for this procedure are Botox, Dysport, Xeomin, and Myobloc.  Botox takes 3-4 days to take effect.  It peaks in 2-3 weeks and lasts for 3 months.   It is approved for the spasticity in the arms and legs, but doctors may inject other spastic muscles which have been found to be successful.  It can be difficult to find the right muscle, and the right dose, so you may need patience to find its success.  Some people try once and give up.  It can take more persistance than that.  Botox can also be used for an over-active bladder.

Surgeries

Locations for insertion of the Baclofen Intrathecal Pump: At UCSD, Dr. Joel Castellanos or Dr. Timothy Furnish do the Baclofen Pump trial/test dose via lumbar puncture. Baclofen pump implantations are typically done by the neurosurgery team at UCSD  with Dr. Sharona Ben-Haim or Dr. Khanna.

Kaiser San Diego only does the Baclofen Intrathecal Pump under special circumstances - but Kaiser Los Angeles does it more regularly.  The Los Angeles spasticity team evaluates clients for an intrathecal Baclofen trial with Dr. Tong Jiang, Dr. Saumya Gill, or Dr. Paul Kazimiroff.  Los Angeles Baclofen Pump implantations are done by Dr. Victor Ping-Yang Lo.

Your help is needed!  

Please tell us: What medical injections or surgeries you have tried to lessen the severity of HSP symptoms in the trunk and lower body? How successful was it for you? Which local doctors did you see, and at which San Diego locations did you receive treatment? By sharing this information, we could potentially help others with HSP identify possible treatment options and connect with specialists sooner.   

 hsp-sandiego@sonic.net

Future considerations not yet FDA approved for spasticity/pain:

• Hyaluronidase - an enzyme that breaks down the hyaluronic acid which accumulates in paretic muscles and causes muscle stiffness without causing the muscle weakness commonly seen in botox.  Hyaluronidase is currently a delivery facilitator used with other drugs to help them spread and absorb in tissue.  UC San Diego Health Neurological Institute has neurology specialists who manage complex conditions (e.g. spasticity).  So they may have this as an option in the future.   

• Trenibotulinumtoxine- TrenibotE has a faster toxin onset and shorter duration (2-3 weeks) than Botox, Xeomin, Daxxify.

• DaxibotulinumtoxinA - Daxxify is a neuromuscular blocking agent similar to Botox with a longer duration than Botox.  It does not use human albumin which contributes to its having a longer duration of effect.  It is approved for cosmetic reasons and for cervical dystonia but not spasticity nor chronic pain.  Phase 2 trials were completed for adult upper limb spasticity and it is awaiting FDA approval.