Utilizing combination intrathecal baclofen and analgesic medication to manage spasticity and pain in patients with pediatric-onset disability: Case series.

PURPOSE: The use of intrathecal medications for the management of spasticity and various pain syndromes in the adult population has been previously reported. However, no evidence-based guidelines currently exist in the pediatric population. This case series presents patients (n = 8) with pediatric-onset disability who underwent placement of intrathecal baclofen pumps initially for management of severe spasticity. Despite titration of dose and use of oral analgesia, their uncontrolled pain persisted. Each patient was transitioned to a combination of baclofen and analgesic intrathecal therapy. The outcome in pain improvement and quality of life, as reported by patients and/or caregivers, were retrospectively reviewed. METHODS: Retrospective review of the background and decision-making process regarding transition to combination intrathecal therapy identified patient selection characteristics. Each patient and/or their caregivers completed a survey regarding improvements in pain, spasticity, function, and quality of life following initiation of combination intrathecal medications. RESULTS: Survey results revealed improvements in functional and pain assessments after initiation of combination baclofen and analgesic intrathecal medication. Patients and caregivers reported decreases in pain and oral spasticity medications. CONCLUSION: Use of pumps with antispasmodic and analgesic medication for combination intrathecal medication delivery should be considered in the management of patients with childhood-onset disabilities who have both severe spasticity and pain.

Medicine shouldn't be this hard: The intersection of physician moral injury and patient healthcare experience in pediatric complex care.

Dr. Jay Neufeld's story in If I Betray These Words is a detailed account of one physician's catastrophic journey through moral injury when caring for pediatric patients with complex medical conditions [1]. Many clinicians may recognize Jay's journey in their own experiences, but what deserves parallel consideration are the journeys of patients and families when they are accompanied by physicians at risk of moral injury. This case study illustrates the tight link between drivers of physician moral injury and patients' negative healthcare experiences. These include (1) decisions directed by health insurance regulations and prior authorizations; (2) the electronic medical record (EMR); and (3) healthcare systems focused on revenue generation.

Paradoxical reaction to increased doses of intrathecal baclofen in a patient with Leigh syndrome.

In clinical practice, intrathecal baclofen (ITB) therapy is used to control spasticity. After initial placement of the ITB pump, clinicians incrementally increase the dose until effectiveness in alleviating spasms and spasticity is optimized. However, this case describes a 4-year-old male with Leigh syndrome who developed a paradoxical worsening of spasticity and pain with incremental increase of his ITB pump. In this rare genetic disease with a poor prognosis, an ITB pump was trialed and implanted and titrated upwards with initial improvement. However, his spasticity and pain then began to worsen with each dosage increase. Subsequently, his symptoms improved significantly when the dose was weaned. This is the first case that describes this paradoxical reaction in a pediatric population and discusses recommendations about how clinicians should safely titrate the pump for patient care.

Superimposed Guillain-Barré Syndrome (GBS) in pediatric hypoxic brain injury.

BACKGROUND: Hypoxic brain injury results in severe disabilities that require extensive acute inpatient and outpatient rehabilitation to promote maximal functional and cognitive recovery. Brain hypoxemia can result from a multitude of causes, including but not limited to cardiac arrest, drug overdose, and/or shock. While recovery from a hypoxic brain injury alone can be challenging, dealing with concurrent debilitative diagnoses such as Guillain Barré Syndrome (GBS) further complicates the recovery and rehabilitation course. CASE REPORT: The current case study highlights the acute inpatient rehabilitation course of a 16 year old male who presented with cerebral hypoxia secondary to strangulation and subsequently developed GBS. Physical examination of the patient upon rehabilitation consult was inconsistent with a purely hypoxic brain injury, including the absence of rectal tone. This prompted further potential spinal cord injury evaluation and work up, with diagnostic testing confirmatory of GBS. RELEVANCE: This case is important as, to our knowledge from literature review, the first known documented instance of hypoxic brain injury complicated by GBS. Moreover, it highlights the importance of identifying all potential causes of functional disability, particularly when presented with physical exam findings inconsistent with chief diagnosis, in order to maximize functional recovery and rehabilitative gains during acute inpatient rehabilitation.