Abstracts

For patients treated with intrathecal baclofen (ITB), inability to obtain timely refills or occurrence of a pump malfunction increases the risk for baclofen withdrawal, which can be deadly. Even minor illness may trigger increased seizures or pain in these sensitive patients. Our clinic has developed a rescue protocol, consisting of a diazepam rectal gel/oral baclofen combination, to ensure that caregivers have effective means to handle pump emergencies. This study summarizes our experience with this emergency treatment plan. All patients with ITB received the diazepam rectal gel/oral baclofen rescue plan. Charts were reviewed to identify patients who used the plan. Eighty patients have ITB pumps; 4 patients experienced pump refill or failure emergencies. Patient 1, a 19-year-old man with cerebral palsy (CP) and spastic quadriplegia, acutely developed signs of baclofen withdrawal, including generalized rash, marked increase in tone with extreme spasticity, and respiratory distress. During a 3-hour trip to the hospital, his symptoms worsened despite treatment with 20 mg oral diazepam. Examination revealed that the baclofen level was too low in his pump. During a subsequent incident of baclofen withdrawal, treatment with 15 mg diazepam rectal gel/10 mg oral baclofen resolved the symptoms. Patient 2, a 9-year-old girl with static encephalopathy with CP and a seizure disorder, had experienced recurrent pneumonia. Her mother was concerned about potential respiratory difficulties associated with oral diazepam; diazepam rectal gel/oral baclofen were prescribed as rescue treatment. A subsequent episode of extreme spasticity and 3 consecutive seizures resolved with 10 mg diazepam rectal gel. Patient 3, a 24-year-old man with CP with spastic quadriplegia, complained of feeling tight and experiencing excruciating pain after pump replacement. Diazepam rectal gel (20 mg)/oral baclofen (10 mg QID) controlled his severe spasms while diagnostic tests were performed; results indicated that his pump was incorrectly programmed and contained insufficient medication. Patient 4, a 15-year-old boy with sacral agenesis and spastic paraplegia, was prescribed oral baclofen and oral diazepam for emergency use; however, the medication was misused by his caregiver. Diazepam rectal gel was prescribed for emergencies, and this situation was resolved. The diazepam rectal gel/oral baclofen combination provides effective seizure and pain relief for patients with baclofen withdrawal. It offers distinct advantages as a rescue treatment: it is not associated with respiratory compromise; it may be administered by nonmedically trained caregivers; it is supplied in premeasured doses and is unlikely to be abused; and it is rapidly absorbed and has a more rapid onset of action than orally administered diazepam. (Supported by Xcel Pharmaceuticals)