Implementation of a nurse practitioner policy for the requisition and administration of drugs in a haemophilia comprehensive care centre

Summary The evolution of comprehensive care centres for haemophilia has altered the work of those involved in the care of haemophilia patients. The role of the haemophilia sister has expanded and the concept of the haemophilia nurse specialist has emerged. We describe the implementation of a local policy which has enabled haemophilia nurse specialists to requisition and administer clotting factor concentrates, DDAVP, tranexamic acid and hepatitis vaccines independently, in hospital and community settings. Since the introduction of this policy on 2 August 1994, prescribing practice has not changed in the haemophilia centre, nor has the involvement of medical staff in the care of haemophilia patients. This approach is widely applicable in other centres and should be a logical progression of the haemophilia nurse specialist's role.     

DDAVP in acquired hemophilia a: Case report and review of the literature

One patient with an acquired factor VIII inhibitor is reported in which an acute lower intestinal hemorrhage was successfully managed using Desmopressin (DDAVP). The patient initially had a factor VIII level of 10% with a inhibitor titer of 1.9 Bethesda units. Following administration of DDAVP the factor VIII level rose to 86% and there was a decrease in the number and volume of bloody stools. The inhibitor disappeared following treatment with corticosteroids, however the patient ultimately expired due to complications of ischemic colitis. This case and 21 previously reported cases of acquired hemophilia treated with DDAVP are reviewed. The data support a role for DDAVP in the treatment of non life threatening hemorrhage in patients with acquired hemophilia and low titer factor VIII inhibitors (<5 Bethesda units or factor VIII≧5%).     

Intranasal administration of desmopressin acetate (DDAVP) to hemophiliacs

The intravenous infusion of desmopressin acetate (DDAVP), a synthetic vasopressin analogue, has been shown to cause a sustained increase in all Factor VIII related properties (VIII-C, VIIIR-Ag, VIIIR-WF) in patients with mild to moderate hemophilia and von Willebrand's disease. This study examines the effect of high doses of intranasal DDAVP (0.4 μg/kg BiD) in four patients with hemophilia A and one patient with von Willebrand's disease. In spite of a physiologic response with a decrease in urine volume and increase in urine osmolality, there was no effect on the Factor VIII complex.     

Effects of vasopressin on noradrenaline-induced cyclic AMP accumulation in rat brain slices

Addition of arginine vasopressin (AVP) or 1-desamino-8-D-arginine vasopressin (DDAVP) to rat cortical slices resulted in significant inhibition of the rise in cyclic AMP produced by incubation with 50 microM noradrenaline. A single injection of DDAVP (20 micrograms/rat) produced a reduced response to noradrenaline in derived cortex and caudate slices. In animals pretreated at day 5 of life with IP desipramine and intracisternal 6-hydroxydopamine (6-OHDA), both acute and chronic treatments with DDAVP resulted in a reduction in response in derived cortical, caudate and hippocampal slices. The 6-OHDA pretreated animals also showed reduced open-field behavioural activity after both acute and chronic DDAVP, while animals which were not pretreated responded to acute treatment only. The relationship between the effects of vasopressin on noradrenaline-induced cyclic AMP accumulation and its action on learning and memory is discussed.     

The Release of Plasminogen Activator and Factor VIII after Injection of DDAVP in Healthy Volunteers and in Patients with von Willebrand's Disease

Increases in plasma concentrations of VIII:C, VIII:CAg, VIIIR:Ag and plasminogen activator (PA) were observed in 50 healthy volunteers given i.v. injections of DDAVP (desaminocys¹-8-D-arg-vasopressin). The PA activity reached its maximum immediately after the injection, VIII:C, VIII:CAg and VIIIR:Ag after 3040 min. However, a positive correlation was found when the PA and VIII:C responses in each of the normals were analysed. DDAVP was also administered to 3 patients with severe von Willebrand's disease. 2 of the patients displayed no changes in VIII:C, VIII:CAg, VIIIR:Ag or VIIIR:RCF and there was no increase in PA. The third patient responded with an increase in VIII:C and to a minor degree in VIII:CAg. This patient developed fibrinolytic activity, but in the lower normal range. In 3 other patients with mild von Willebrand's disease DDAVP caused increases in VIII:C, VIII:CAg, VIIIR:Ag and PA. We feel that the combined data may support the concept that one and the same target cell is involved in the DDAVP mediated release of factor VIII related activities and PA.     

IMPORTANCE OF ANTIDIURETIC PROPERTIES OF VASOPRESSIN IN EXPERIMENTAL RENAL HYPERTENSION

1. Homozygous Brattleboro rats with hereditary hypothalamic diabetes insipidus (DI) developed one-kidney, one-clip (1K–1C) and two-kidney, one-clip (2K–1C) Goldblatt renal hypertension. 2. However, the systolic blood pressure (SBP) in 1K–1C Brattleboro rats was significantly lower than in non-DI hypertensive 1K–1C Long-Evans (LE) rats. 3. Treatment with the synthetic antidiuretic analogue of vasopressin, DDAVP (l-desamino-8-D-arginine vasopressin), increased the SBP in the DI rats to the same level as in 1K–1C Long-Evans. This elevation of SBP was associated with increased extracellular fluid volume in the DI rats after DDAVP. 4. The results suggest that vasopressin is not essential for the development of renal hypertension but the absolute levels of blood pressure achieved in 1K–1C hypertension are volume-dependent and thus influenced by the water-retaining properties of vasopressin.     

Urinary kallikrein excretion after DDAVP during lithium treatment

Several factors are believed to contribute to impaired responsiveness to arginine vasopressin (AVP) during lithium treatment. The renal kallikrein–kinin system is reported to antagonize the renal effects of AVP in vitro but there are no reports of its activity during lithium treatment. Urinary active kallikrein excretion in 18 lithium-treated patients was found to be lower than that in eight healthy volunteers. Following intravenous administration of an AVP analogue, des-amino-D -arginine-vasopressin, to the lithium-treated patients, the urinary excretion of active kallikrein was unchanged, despite an impaired urine-concentrating ability. These results suggest that stimulation of renal kallikrein does not contribute to inhibition of the action of AVP, and thus the genesis of nephrogenic diabetes insipidus during lithium treatment.     

Factor VIII Concentrate Prepared from DDAVP Stimulated Blood Donor Plasma

Human fraction 1-0 (AHF-Kabi) was prepared from plasma from blood donors who had received an i.v. injection of DDAVP (0.2 μg per kg b.w.) and tranexamic acid (0.01 g per kg b.w.) 15 min before collection of the blood. The factor VIII preparation from such plasma contained twice as much VIII:C, VIIIR:Ag, and VIIIR:RFC as normal fraction 1-0. Normal fraction 1-0 and DDAVP fraction 1-0 were given to 2 patients with severe haemophilia A. The in vivo response of the DDAVP fraction 1-0 corresponded to the in vitro values. No differences in survival time were seen. Hence, it is possible to produce factor VIII concentrates with at least double the yield by increasing the factor VIII level in blood donors by i.v. injection of DDAVP.