This SLS patient was a 19-year old female with generalized ichthyosis, intellectual disability, underlying hypotonia, and spastic paraparesis. She had bilateral radial head dislocation of the elbows and had undergone hamstring release 16 years previously. An ITB trial 6 years earlier made her floppy and unable to walk, so a pump was deferred. At baseline, the patient had daily physical and occupational therapy and was on oral baclofen 10 mg TID. She was capable of walking 2-3 blocks with her posterior walker and ankle-foot braces, but ambulated with great difficulty and employed a manual wheelchair often. Her increasing tone impeded daily care and hygiene. She was considered for ITB therapy to ease care while weighing the potential for interference with her potential for ambulation.
After a positive ITB trial, she underwent ITB pump implantation. The initial baclofen dose was 39.9 mcg/day. After surgery and rehabilitation, an observable improvement was noted with muscle tone relaxation in both legs, but without lethargy, excessive hypotonia or loss of axial control. She continued to have daily physical and occupational therapy.
Over the ensuing of six months, her ITB dose was increased to 135 mcg/day to progress with gait training, strengthening, and to address residual spasticity. This ITB dose was intermittently too high, resulting in difficulty with gait and hypotonia, causing her knees to buckle, so the ITB dosage was titrated to allow active standing and lower extremity strengthening. A daily flexible dose delivery was eventually programmed. Three years after surgery, her standing dose is 86 mcg/day due to potential drug interaction with an antidepressant (citalopram). At this dose, the patient has a substantial improvement compared to baseline spasticity (see Table 1) and shows no signs or symptoms of ITB over- or underdosage.