Do long-term day treatment clients benefit from supported employment?

1998 ◽  
Vol 22 (1) ◽  
pp. 24-29 ◽  
Author(s):  
Edward L. Bailey ◽  
Susan K. Ricketts ◽  
Deborah R. Becker ◽  
Haiyi Xie ◽  
Robert E. Drake
Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 2141-2141
Author(s):  
David C. Dale ◽  
Audrey Anna Bolyard ◽  
Tracy M. Marrero ◽  
Mary Ann Bonilla ◽  
Daniel C. Link ◽  
...  

Abstract Abstract 2141 Cyclic neutropenia (CyN) is an autosomal dominant and sporadically occurring hematological disease first described in 1910. Cyclic neutropenia is usually diagnosed in children before age 2 years based of regularly occurring fever, mouth ulcers, and recurrent skin or respiratory infections. In affected families, recognition is generally earlier than for the sporadic cases. Serial neutrophil counts usually show periods of very severe neutropenia (ANC< 0.2 × 109/L) at three week intervals and an intervening peak ANC less than 2.0 × 109/L. A reciprocal monocytosis often occurs during the neutropenic period. Mild anemia is common, particularly in patients with recurrent infections. Cycling of blood neutrophils is more apparent in young children than in adults, who often report severe childhood symptoms ameliorating near puberty or soon thereafter. Through the Severe Chronic Neutropenia International Registry and Repository- Seattle (SCNIR-Seattle) we have prospectively studied the natural history of 348 patients with a clinical diagnosis of CyN, 181 sporadic cases and 167 patients from 36 families whose pedigrees illustrate autosomal dominant inheritance. Sequencing results for the gene for neutrophil elastase, ELANE, are available for 119 of these patients including at least one member of 18 families. We have prospectively followed all of these patients for up to 25 years. Patients with typical, 21 day oscillations of blood neutrophils almost always have mutations of ELANE, most frequently mutation in exons 4 or 5. Neutropenic relatives (ANC < 1.5 × 109/L) of an index case of CyN with an ELANE mutation uniformly have the same ELANE mutation, even if they have few symptoms and the neutropenia is not severe. Patients with severe congenital neutropenia and CyN may also have the same or nearly identical mutations; thus the diagnosis of cyclic neutropenia can not be made solely from mutation analysis. Since 1987 we have treated cyclic neutropenic patients with G-CSF; some patients have been on G-CSF on a daily or alternate-day basis for 25 years. For the population of 209 treated patients enrolled in the SCNIR and treated for a median of 14.8 years, the median daily G-CSF dose is 2.1 mcg/kg/day. We have observed that the dose established during the first year of treatment can be adjusted for body weight and usually maintained indefinitely. Adverse effects are infrequent, particularly with daily or alternate-day treatment, although development of osteopenia remains a concern. A few patients who have transiently interrupted G-CSF; almost all have resumed G-CSF because of recurrence of fever, mouth ulcers, infections or fatigue. We are currently aware of only two CyN patients who have discontinued G-CSF treatment for greater than 2 months without recurrence of symptoms or returning to G-CSF therapy. In this population, there has been no hematopoietic stem cell transplantation for treatment of CyN and no G-CSF treatment failures. 15 deaths have occurred: 1 from ovarian cancer, 2 related to discontinuation of G-CSF, 2 from cardiac causes, 3 from homicide or accidents, 5 from complicated illnesses with multi-system problems. One closely followed patient never treated with G-CSF developed CML and one patient treated long term with immunosuppressive drugs and G-CSF developed AML. Other than this case, there have been not CyN patients developing AML in more than 5000 years of patient observation. Clostridial sepsis, the frequent cause of death in CyN patients in the pre-G-CSF era, has not been observed in CyN patients on G-CSF. These long-term observations serve to characterize cyclic neutropenia is a distinctive hematological disorder, very responsive to treatment with G-CSF and without recognized risk of evolution to AML. Disclosures: Dale: AMGEN: Consultancy. Boxer:Amgen: Equity Ownership.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 548-548 ◽  
Author(s):  
Elena Santagostino ◽  
Christine Voigt ◽  
Denise Wolko ◽  
Grace Cole ◽  
Yanyan Li ◽  
...  

Abstract A fusion protein genetically linking recombinant human coagulation FIX with recombinant human albumin (rIX-FP) has been developed with an improved PK profile, thus improving hemophilia B treatment by allowing less frequent dosing. Two Phase 3 studies (CSL654-3001 and CSL654-3002) were completed. CSL654-3001 study evaluated safety and efficacy of rIX-FP for prophylaxis treatment (PT) of every 7-, 10- and 14-day and on-demand (ODT) of bleeding episodes in 63 previously treated patients (PTP), 12-61 years of age with hemophilia B (FIX ≤ 2%). Subjects in the on-demand arm received only ODT for 6 months and then switched to every 7-day PT. Subjects in the prophylaxis arm received every 7-day PT, and eligible subjects switched to every 10- or 14-day PT for approximately 12-18 months. Annualized spontaneous bleeding rates (AsBR) were compared between ODT and PT periods (in on-demand arm), and between 7-day PT and 10- or 14-day PT (in prophylaxis arm). CSL654-3002 study evaluated safety and efficacy of rIX-FP for weekly prophylaxis regimen in 27 previously treated patients younger than 12 years with hemophilia B (FIX ≤ 2%) for approximately 12 months. Annualized spontaneous bleeding rates (AsBR) were calculated. The median annualized spontaneous bleeding rate were all 0.00 for all treatment interval (7-day, 10-day or 14-day) and in both studies age groups (1-11 years and 12-65 years) during the two completed phase 3 studies. Seventy-six subjects from both studies continued their prophylaxis regimen in the on-going extension study. In addition, subjects (including children), switched to longer treatment intervals of 10-day, 3 times per month or 14-day or lowered their weekly prophylaxis dose. Nine subjects switched to 21-day treatment interval with 100 IU/kg rIX-FP. As of 28 July 2015, at least 50 subjects (PTP) had achieved 100 EDs without developing an inhibitor to FIX or antibodies to rIX-FP. The long term safety and efficacy of rIX-FP will be presented. This presentation includes the new information regarding the change to longer than 7-day treatment regimens in the extension study, among those subjects (1-61 years of age) that previously participated in the lead in studies. Conclusion: The Prolong - 9FP clinical programdemonstrated the clinical efficacy of rIX-FP for routine prophylaxis every 7-, 10- and 14-day treatment intervals. Routine prophylaxis once every 21 days may be effective in preventing bleeding episodes in a selected patient population. In addition, rIX-FP demonstrated favorable long-term safety and tolerability. Disclosures Santagostino: Novo Nordisk: Speakers Bureau; Bayer: Speakers Bureau; CSL Behring: Speakers Bureau; Baxter/Baxalta: Speakers Bureau; Pfizer: Research Funding, Speakers Bureau; Biogen/Sobi: Speakers Bureau; Biotest: Speakers Bureau; Kedrion: Speakers Bureau; Octapharma: Speakers Bureau; Roche: Speakers Bureau. Voigt:CSL Behring: Employment. Wolko:CSL Behring: Employment. Cole:CSL Behring: Employment. Li:CSL Behring: Employment. Jacobs:CSL Behring: Employment.


2001 ◽  
Vol 52 (3) ◽  
pp. 351-357 ◽  
Author(s):  
Deborah R. Becker ◽  
Gary R. Bond ◽  
Daniel McCarthy ◽  
Daniel Thompson ◽  
Haiyi Xie ◽  
...  

1997 ◽  
Vol 18 (4) ◽  
pp. 194-196 ◽  
Author(s):  
John Kregel

Supported employment is an established approach to community-based employment that has provided many individuals with significant disabilities an opportunity to choose between a lifetime of low wages in segregated sheltered workshop settings and the challenges of a real job in their local community. As a service-delivery strategy that embodies the principles of individualized, community-based support services and consumer empowerment, supported employment has become the preferred employment alternative for large numbers of individuals previously excluded from work opportunities (Rusch, Chadsey-Rusch, & Johnson, 1991; Wehman & Kregel, 1994). The purpose of this article is to summarize the present status of supported employment, discuss the major accomplishments of the initiative, and identify those issues that must be addressed in the future to allow the program to achieve its long-term goal of promoting the economic independence and community integration of individuals with significant disabilities.


1981 ◽  
Vol 61 (s7) ◽  
pp. 469s-471s
Author(s):  
I. Szám ◽  
J. Holló

1. Twenty patients with essential hypertension were treated with guanfacine given in single daily doses of 1–5 mg over a period of 24 weeks. Compared with the initial values at the end of the first wash-out period, there was a significant decrease of blood pressure and heart rate. The most common side effect, dryness of the mouth, usually disappeared after 8–10 weeks of treatment. No changes in laboratory values were seen. In the post-treatment placebo period there were significant increases in blood pressure and heart rate compared with the last readings during the treatment period. However, these never exceeded the pretreatment values. 2. In a second trial guanfacine (1–5 mg daily) was abruptly discontinued in 11 patients after 6–20 weeks' treatment. Blood pressure was measured twice a day, in lying and standing positions, during the 4 days before abrupt withdrawal of guanfacine and for 7 days after discontinuation. Clopamide was given concurrently to two patients, and this was continued after withdrawal of guanfacine. Only in two patients did the blood pressure rise to values above the initial levels (30 mmHg systolic and 10 mmHg diastolic), but no clinical symptoms were observed during the withdrawal. A transitory increase of heart rate of between 10 and 30 beats/min was observed in five patients after abrupt discontinuation of the drug.


1998 ◽  
Vol 22 (1) ◽  
pp. 30-33 ◽  
Author(s):  
Daniel McCarthy ◽  
Daniel Thompson ◽  
Susan Olson

2013 ◽  
Vol 26 (3) ◽  
pp. 263-266

The aim of the present study was to assess the impact of combined 14-day treatment with mianserin (10 mg/kg) and simvastatin (1 or 10 mg/kg) on selected biochemical liver and kidney parameters in rats (AST and ALT activities and the concentrations of AFP, total protein, urea, creatinine and ß2-M). The results showed the increase in both transaminases activities, creatinine concentration and the decrease of AFP, total protein and ß2-M concentrations. The results indicate that 14-day combined administration of mianserin with simvastatin negatively affects the liver functioning. The observed changes in kidney biochemical parameters may suggest a risk of renal dysfunction during long-term combined treatment with these drugs.


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