Background

Foods for Special Medical purposes (Phe-free protein substitutes [PS] and low protein foods [LPFs]) are central to successful dietary management of patients with phenylketonuria (PKU). In the UK, PS and LPFs are prescribed by primary care general practitioners (GPs) and dispensed by high street pharmacists or via home delivery companies. As patients and carers commonly reported access issues with PS and LPFs through the National Society for PKU (NSPKU) helpline, the NSPKU collected more information about the extent of prescriptions issues via a questionnaire.

Methods

A cross sectional online survey was carried out consisting of 7 multiple choice and 3 open questions. The questionnaire was available via the NSPKU website over a 9-month period (15th May 2018–31st January 2019). Carers or patients with PKU were invited to participate if they had any problems accessing PS and LPF.

Results

There were 252 responses. 65% for children and 35% for adults, aged ≥18y with PKU. 59% (n = 146/246) of responses described difficulties accessing basic LPF (bread, pasta) and 33% (n = 81/248) PS. 36% (n = 88/248) said problems had re-occurred >1y, and 40% (n = 99/248) for <3 months. 59% (n = 146/246) had supply issues with LPF and 33% (n = 81/246) with PS. 18% (n = 42/234) reported that the local NHS authority had refused, restricted or had a policy to block treatment access; 27% (n = 63/234) cited GPs declining requests or restricting prescription amounts. 21% (n = 49/234) could not access all their products via a pharmacy, 40% (n = 93/234) said their home delivery system failed, and 15% (n = 34/234) of the dispensed prescribed products were out of date, incorrect, damaged or poor quality. For patients and carers these issues caused: stress (87%, n = 210/241); they considered PKU was not taken seriously by professionals (66%, n = 160/241); they reduced patient access to LPF and PS (53%, n = 128/241); and affected the patients' health (53%, n = 128/241).

Discussion

Access to PKU dietary treatment is frequently disrupted for reasons relating to GP prescriptions and unreliable dispensing and delivery practices. These issues require immediate attention by UK health professionals in order to create a fairer and more efficient system for everyone.

中文翻译：

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英国苯丙酮尿症患者的处方问题。

背景

特殊医疗用途食品（无 Phe 蛋白替代品 [PS] 和低蛋白食品 [LPF]）是苯丙酮尿症 (PKU) 患者成功饮食管理的核心。在英国，PS 和 LPF 由初级保健全科医生 (GP) 开具处方，并由大街药剂师或通过送货上门公司分发。由于患者和护理人员通常通过国家北大协会 (NSPKU) 帮助热线报告使用 PS 和 LPF 的问题，因此 NSPKU 通过问卷调查收集了有关处方问题程度的更多信息。

方法

进行了一项横断面在线调查，包括 7 个多项选择和 3 个开放式问题。该问卷在 9 个月期间（2018 年 5 月 15 日至 2019 年 1 月 31 日）通过 NSPKU 网站提供。如果 PKU 的护理人员或患者在访问 PS 和 LPF 时遇到任何问题，他们将被邀请参加。

结果

有 252 条回复。65% 为儿童，35% 为成人，≥18 岁患有 PKU。59% ( n  = 146/246) 的回复描述了获取基本 LPF（面包、意大利面）和 33% ( n  = 81/248) PS 的困难。36% ( n  = 88/248) 表示问题再次出现 > 1 年，40% ( n = 99/248) 表示问题再次出现 < 3 个月。59% ( n  = 146/246) 有 LPF 供应问题，33% ( n  = 81/246) 有 PS。18% ( n  = 42/234) 报告说当地 NHS 当局拒绝、限制或制定了阻止获得治疗的政策；27% ( n  = 63/234) 表示全科医生拒绝请求或限制处方金额。21% ( n = 49/234) 无法通过药房获取所有产品，40% ( n  = 93/234) 表示他们的送货上门系统出现故障，15% ( n  = 34/234) 的配发处方产品已过期、不正确、损坏或质量差。对于患者和护理人员，这些问题导致：压力（87%，n  = 210/241）；他们认为 PKU 没有得到专业人士的重视（66%，n  = 160/241）；他们减少了患者获得 LPF 和 PS 的机会（53%，n  = 128/241）；并影响患者的健康（53%，n  = 128/241）。

讨论

由于与 GP 处方和不可靠的配药和交付实践有关的原因，获得 PKU 饮食治疗的机会经常被中断。这些问题需要英国卫生专业人员立即关注，以便为每个人创建一个更公平、更有效的系统。