Lethal form of spinocerebellar ataxia type 7 with early onset in childhood.

Progressive cerebellar ataxias are well-known hereditary neurological disorders. Among them, spinocerebellar ataxia type 7 (SCA7) is inherited as an autosomal dominant trait and is ascribed to the expansion of a CAG trinucleotide repeat within the ATXN7 gene. An anticipation phenomenon can occur during paternal transmission and sometimes is responsible for a severe infantile form. The specificity of SCA7 is the retinal involvement with retinitis pigmentosa and cone rod dystrophy. We describe a familial form with two siblings who died of a severe infantile form. Diagnosis was made in their father, who had a recent history of macular atrophy and presented with gait disturbance thereafter. Retrospectively, substantial triplet repeat expansion was confirmed in the two affected infants. These infantile forms are rare and difficult to diagnose in the absence of suggestive family symptoms.

Strategic antibiotic decision-making at the formulary level.

The proliferation of antimicrobial agents introduced for the treatment of increasingly complex infectious diseases, coupled with the obligation to provide the best possible medical care in keeping with hospital fiscal constraints, has created a need for strategic decision-making about antibiotics at the formulary level. The following experience at the Hartford Hospital, a 1,000-bed tertiary-care facility, represents an example of an antibiotic management system based on a multidisciplinary approach that utilizes infectious disease physicians, clinical microbiologists, hospital epidemiologists, and pharmacists.

Achieving rational therapeutics through consensus-based protocols and follow-up.

In this exclusive Hospital Formulary interview, three key members of the therapeutics committee at Hartford Hospital, an 861-bed, private teaching hospital, discuss some of the techniques that they have used to ensure the development of a sound and effective formulary system within their institution. The committee emphasizes a policy-making approach that incorporates, up front, the expertise of key clinicians together with the best available information from the medical literature. Once committee policy has been set, implementation of drug use guidelines is largely automatic, and compliance is carefully monitored and reviewed.

Developing staff into clinical practitioners.

Developing clinical skills in pharmacy staff is a process that can enhance the services of a pharmacy department and improve the professional development and interest of the staff. Staff should be included in the development of a clinical philosophy and a training and ongoing development program that will achieve the desired goals should be planned. An MBO process may help to track the individual pharmacist's progress. Knowledgeable, motivated staff are the key ingredient in achieving success in providing pharmacy services.

Applying for a certificate of need for unit dose and i.v. admixture services in a Connecticut hospital.

The process of obtaining regulatory-agency review of a proposal for unit dose and i.v. admixture services is described. In a 901-bed community hospital in Connecticut, a pharmacy department proposal for instituting unit dose distribution and expanding the i.v. admixture service was supported by the hospital administration and included in hospital budget projections. A state body that regulates hospital revenues, the Commission on Hospitals and Health Care (CHHC), rejected the proposal for these changes in pharmaceutical services. The pharmacy and hospital administration subsequently petitioned the Health Systems Agency in that jurisdiction for a certificate of need (CON); the procedure required that the application also be simultaneously submitted to CHHC. Implementation of the program over a three-year period was proposed. The CON application was submitted in July 1981; it required detailed information from pharmacy, nursing services, and hospital administration. During the nine months following application for the CON, further questions were asked and five hearings were held. In April 1982, CHHC approved the program and the capital expenditures but did not approve the proposed 10% increase in drug revenue needed to support the services. The hospital ultimately committed funding for implementation of the program over three years. The net result of the application procedure was a delay in implementation of expanded pharmaceutical services.

Regional disaster planning for hospital pharmacies.

The development of a disaster plan for hospital pharmacy services in Hartford, CT, is described. In June 1978, directors of pharmacy from 12 hospitals in the Hartford, CT, area began a project designed to ensure an uninterrupted supply of drugs and an adequate supply of pharmacy personnel during and after any natural disaster. The project initially involved standardization of components of the individual hospital pharmacies' disaster plans. A questionnaire was then completed by each director of pharmacy regarding hours of operation, telephone numbers, and pharmaceutical products usually stocked (including blood derivatives, radiologic-contrast media, and intravenous solutions). Information on inventory levels was collected later. Pharmaceutical manufactures and wholesalers in the region were contracted, and a list of routine and emergency telephone numbers was compiled. The disaster plan was completed in July 1979. The plan has been tested in a mock catastrophe drill and one natural disaster that caused relatively few injuries.

Training hospital pharmacy technicians: joint effort by departments of education and pharmacy.

The cooperative development and initiation of a one-year pharmacy technician training program is described. The hospital's pharmacy and education departments formulated goals and objectives for the didactic and clinical areas of the program and developed methods to evaluate student competency and the program itself. The training for first class of students consisted of three months of didactic instruction, one month of laboratory experience, and eight months of on-the-job training. The program includes an observation of medication administration which is designed to increase the student's awareness of the entire medication process.

Expansion of pharmacy services in a mental health center.

This article describes a system redesign initiated at the Connecticut Mental Health Center which enabled the pharmacists to release approximately 20 hours per week. This time was reallocated to improve and expand pharmacy services. Also described is the effect of this system improvement on physicians, clinicians, and patients and on the ability to identify first-stage noncompliance in patients who are part of the medication maintenance program. The value of system redesign in improving and expanding services in an era of cost containment is discussed.