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, and perhaps even an Occupational Therapist, a nutritionist, an acupuncturist and a massage therapist.  Having someone assist with your mental health is also a great resource.  Having a psychologist who specializes in neurodegenerative diseases or perhaps a neuropsychologist could be helpful. 

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 will reference below come from the SPF Talk, Spasticity Management with Dr. Bianca Martinez MD, FAAPMR. Dr. Martinez spoke about helping a patient with HSP through a 'multi-pronged treatment approach' — and that concept is truly crucial. Because as HSP progresses, it involves several things happening simultaneously, and 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 controls 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.  

Cryoneurolysis

Watch Dr. Winston explain his experience contributing to the development of cryoneurolysis on patients with spasticity.  Cryoneurolysis is a mini-invasive procedure to reduce spasticity, improve movements, restore function, and reduce pain.  See before/after results of cryoneurolysis on his patients.  Some patients claimed cryoneurolysis was life changing and a few patients had tears of joy!  Dr. Winston's videos show the Iovera needle in the cryoneurolysis procedure. 

Cryoneurolysis uses extremely cold temperatures (of 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 yet it preserves the surrounding connective tissue structure. This temporarily blocks the nerve’s ability to transmit signals that contribute to muscle spasticity and/or pain.

When applied near the common peroneal nerve—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 lasting in a range from the 6 months to even over a year.  

Cryoneurolysis is a newer treatment, so it is more difficult to find a physician who performs it for spasticity.  Get clarification on your call.  

Cryoneurolysis in southern California- unclear if it is for spasticity:

• Dr. Brian Ilfeld in the Department of Anesthesiology at UC San Diego (9500 Gilman Drive, La Jolla) is a leading researcher and practitioner of ultrasound-guided percutaneous cryoneurolysis. He's one of the most published experts on this procedure in the country. 

• Dr. Rodney Gabriel does cryoneurolysis also at UCSD in La Jolla.  

• Dr. Gary Briskin, DPM, FACFAS, and colleagues at the University Foot and Ankle Institute offer cryoneurolysis, with multiple locations across Southern California.  UFAI may be reached at (855) 872-5249.

• Dr. Bob Baravarian — A foot and ankle specialist in LA who performs cryoneurolysis is reachable at (855) 557-5400.

Cryoneurolysis in America:

Dr. Winston mentioned that he certified many American doctors with cryoneurolysis including doctors at: Harvard Spaulding Boston,  University of Kentucky Lexington, and Atlanta Georgia. I also found, Dr. Sheng Li is a physical medicine & rehabilitation physician at TIRR Memorial Hermann in Houston Texas.  Contact (800) 44-REHAB (73422).

Search by Iovera:

You may search for a doctor who performs cryoneurolysis by looking up providers that use the Iovera device. Iovera maintains a directory of certified providers offering FDA-cleared cryoneurolysis for knee, hip, shoulder, spine, and surgical pain relief, searchable by zip code. They claim to have the most comprehensive and up-to-date directory at: iovera.com/find-a-doctor

Once you identify a provider, call their office to confirm whether they perform cryoneurolysis specifically for spasticity — such as in the hamstrings and calves — or other areas relevant to your needs. Keep in mind that cryoneurolysis is not exclusively used for spasticity, so it may take some time to find a provider experienced in cryoneurolysis for your needs.

Good news- Medicare covers cryoneurolysis! 

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 Intrathecal baclofen pump trial and implantations: 

• At UCSD: the ITB pump trial with the lumbar puncture is done by Dr. Joel Castellanos or Dr. Timothy Furnish.  ITB pump implantations are typically done by the neurosurgery team with Dr. Sharona Ben-Haim or Dr. Khanna.

• For Kaiser patients: Kaiser San Diego does not seem to do Intrathecal baclofen pump.  Perhaps get a referral out for it.  Kaiser Los Angeles does it more regularly.  The Los Angeles spasticity team evaluates clients for the ITB pump trial with Dr. Tong Jiang, Dr. Saumya Gill, or Dr. Paul Kazimiroff.  Los Angeles ITB Pump implantations are done by Dr. Victor Ping-Yang Lo.

Your help is needed!  

Please tell us: What successful 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 HSPto  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 short duration of 2-3 weeks.

• 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.