Hua Zhao ,Png Zhao ,Yan-Ni Wang ,Yu-Lin Guo ,Rui-Hong Wu

aNursing Department,The Second Hospital of Shanxi Medical University,Taiyuan,Shanxi 030001,China

bDermatology Department,Shanxi Provincial People's Hospital,Taiyuan,Shanxi 030012,China

cTaiyuan Health School,Taiyuan,Shanxi 030012,China

dDepartment of Rehabilitation Medicine,The Second Hospital of Shanxi Medical University,Taiyuan,Shanxi 030001,China

eDepartment of Hematology,The Second Hospital of Shanxi Medical University,Taiyuan,Shanxi 030001,China

1.Introduction

Hemophilia involves a potentially life-threatening tendency to bleed after trauma to any part of the body,with joints and muscles being common sites.Hemophilia is currently incurable,and patients must depend on infusions of clotting factors throughout their lives.1Transient treatment in hospitals cannot guarantee effectiveness for controlling the disease,thus,home therapy is particularly important for hemophiliacs.2

Home therapy for hemophilia refers to the prevention,evaluation and treatment of bleeding that occurs at home by the hemophiliac and/or their family members.Home therapy is helpful for early treatment of bleeding and reducing pain and the risk of deformity and hospitalizations associated with complications.3,4

The Shanxi Hemophilia Treatment Center(HTC)was established in 2009,and the National Health and Family Planning Commission of China established an information management system for hemophilia cases and 11 branches in Shanxi Province.The hospitalbased HTC managed the records of hemophilia patients and a total of 494 patients with hemophilia were registered by the end of 2015.

The Shanxi Province HTC conducted home therapy training for hemophilia from April 2012 to September 2014 with 165 hemophilia patients and 178 family members.An investigation conducted by the Shanxi Province HTC in October 2015 showed that only 34 hemophiliacs had continued to undergo home therapy.The reasons that others failed to continue with home therapy require urgentexploration to provide a foundation for formulating targeted interventions by the HTC in Shanxi Province.

2.Methods

2.1.Research methods

We adopted purposive sampling for interviewing 17 hemophilic families who did not continue home therapy from 11 branches across 11 cities in Shanxi Province in accordance with the principle of data saturation.There were eight hemophiliacs and nine family members.The inclusion criteria were patients or family members who had received home therapy training for hemophilia at the Shanxi Province HTC,age＞18,excellent comprehension and communication,and informed consent.Participants with mental diseases or participants who were unwilling to cooperate were excluded from the interviews.Two physicians and one nurse from the Shanxi Province HTC were also selected for interviews.Data were gathered between December 2014 and December 2015.

We adopted a qualitative phenomenological approach to carry out the semi-structured,in-depth interviews.The researchers developed the interview outline after consulting an expert from the World Federation of Hemophilia(WFH)and conducted preliminary interviews with two interviewees.

2.2.Data collection and analysis

The interviews occurred in a single room in the Shanxi Province HTC and were recorded after obtaining the interviewees'consent,with brief transcripts taken in the interim.The researchers conducted one-on-one in-depth interviews with each interviewee,who was interviewed 2-3 times across a period of 1-2 weeks.Each interview lasted for 23-60 min.Particular techniques were occasionally used to elicit truthful and comprehensive opinions,attitudes and understanding from the interviewees,while suggestive hints were avoided.The interview records were numbered sequentially within 24 h after the interview,e.g.,hemophiliacs:P1-P8;family members:F1-F9;doctors:D1-D2;nurse:N1.The transcripts of both language-and non-language-based information(such as intonation,stress and pause)were analyzed according to Colaizzi's seven steps.5Statements of particular signi ficance were extracted from the transcripts after careful reading,and repeated statements were encoded.

3.Results

3.1.Topic 1.Home therapy was generally approved

The interviews indicated that families of patients with hemophilia generally approved of the bene fits of training for hemophilia home therapy.F1 said that his 12-year-old son knew how to handle emergency situations after receiving home therapy training.P2 also thought that he had learned to judge the bleeding after training instead of ignoring the numbness in his legs,which was probably the precursor to bleeding.F5 thought that home therapy facilitated early treatment.He was not experienced enough with bleeding the first time his child grew a permanent tooth,and he had to rush to the hospital for treatment.This time,he injected two shots of drugs to his son at home when faced with the same situation.P1 considered that home nursing allowed him to control his life better.He brought the drugs and previously participated in the patients'association.F8 stated tearfully that her son once thought she was to blame for his disease,but now she feels that he trusts her after treating him twice at home,and their relations have relaxed very much.

她笑了笑，招手把本派新生叫了出来，他们离开队伍，很快消失在前方无边的黑暗里。目送最后一个身影离开，我看着剩下的人。大部分新生都来自无畏派，因此这时只剩下了九个人：当然，我是唯一的无私派转派生，没有友好派，其余几个来自博学派，令人吃惊的是还有诚实派。一直以来，不是诚实最需要勇气吗？但他们为什么转派？不得而知。

3.2.Topic 2.Concerns about the safety of home therapy

3.2.1.Lack of professional knowledge and home therapy skills

Most patients from the Shanxi Province HTC did not have an implantable venous access device.The construction and maintenance of intravenous infusion access had prevented these patients from persisting in home therapy.F1 stated that it was cruel to inject drugs into his son even after being taught by the nurse.P6 thought that professional injection was difficult to master.

Calculating the drug dose also restricted home therapy.F8 said he was not sure when to treat because it may impact the condition.P3 was also uncertain about the drug application dose after bleeding.Safe management of the clotting factor also made families of patients with hemophilia afraid of home therapy.P4 said he was always worried that a drug reaction may occur during home therapy.F2 also said that she was afraid that the poor preservation would affect her husband's treatment.

3.2.2.Lack of confidence in carrying out home therapy

The interviews revealed that long-term hospital treatment resulted in a lack of con fidence in home therapy in families of patients with hemophilia.F3 stated that when his child cried from seeing a large hematoma,he wanted to rush to the hospital where he felt at ease.F7 thought that even though home therapy can be carried out immediately,he still felt scared about its effectiveness.In addition,the lack of supervision and guidance by the medical personnel made the families of patients with hemophilia anxious about home therapy.P8 stated that they got into a muddle using home therapy every time.F6 stated honestly that he lacked con fidence without the guidance of professional personnel.

3.3.Topic 3.Imperfect social medical security system

3.3.1.Limited access to therapy

Approximately 90%of hemophiliacs had little income due to their disease.Clotting factor products in Shanxi Province mainly comprise imported recombinant clotting factor and domestic plasma-derived clotting factor.Recombinant clotting factor is restricted in Shanxi Province due to its expensive price(it has not been enrolled into the medical insurance system),even though it is common.6P3 said:“The recombinant factor is too expensive to afford.”In contrast,plasma-derived clotting factor is cheaper;however,it has been in short supply in China due to a lack of fresh plasma.P2 stated that plasma-derived clotting factor could only be bought from the HTC because they received treatment there.The interviews revealed that hemophiliacs could barely receive timely treatment and that the treatment protocols,dosage and course were also inadequate.P1 admitted that he was reluctant to use the drugs for minor bleeding,so he stopped it by using conservative treatment.

3.3.2.Underinvestment in the social medical security system

The high cost of treatment means that the majority of families of patients with hemophilia in Shanxi Province are experiencing catastrophic medical expenses.Hemophilia was enrolled in the major disease medical coverage in China in 2012.However,recombinant clotting factor has still not been enrolled in medical insurance reimbursement in various cities in Shanxi Province.P4 expressed:“I have to pay for nearly 30%of the expense of the plasma-derived clotting factor,while recombinant clotting factor is all at our own expense,”In some regions of Shanxi Province,hemophilia was not included within the outpatient reimbursement scope,and patients could only be reimbursed for inpatient and not outpatient treatment.The instant settlement compensation service on discharge was implemented in 2015 and prevented patients from going back and forth for drug reimbursement,but it also led to only inpatient reimbursement.A member of P7's family stated that they could get more or less reimbursement for hospital treatment,but they have to pay for home therapy themselves.

Inadequate medical insurance for hemophilia leads to patients'lack of con fidence in the insurance system,especially in rural areas.P8 said:“I would rather use the premium submitted each year to buy the clotting factor.”P3 also complained: “Reimbursement can only be conducted in the designated hospital,which we are far away from,and it is quite inconvenient.”Commercial insurance companies impose arti ficial constraints or refuse insurance for hemophilia.In addition,the social security system in rural areas of Shanxi Province is incomplete,and social assistance cannot guarantee comprehensive coverage.

3.4.Topic 4.Inadequate continuous nursing of hemophilia

3.4.1.Inadequate comprehensive treatment of hemophilia

Hemophilia is a rare disease with complicated diagnosis and management.Apart from clotting factor infusion,hemophiliac families encounter various other problems.7P1 said:“No matter what problems I encounter in the hospital,the personnel can help me,but I have no idea of whom to ask for help during home therapy.”Hemophilia information management centers at provincial levels have the highest diagnostic and treatment levels for hemophilia in China.However,even these still failed to provide comprehensive management.D2 said:“At the beginning a person has the integrated team member,who has also been trained and assigned.However,when the patients have questions in the absence of the assigned member,we are helpless because the other personnel are unprofessional.”

A long-term relationship between the comprehensive care team members and the patients/family members can prompt patients to obey medical advice.However,as P2 emphasized,it was difficult to meet with doctors during home therapy.Home therapy must be closely supervised by the comprehensive care team and can only be carried out with trained patients.However,no effective cooperation had been established between the HTC and the hemophiliac families.The hemophiliac families stated that a lot of information was required for home therapy,but only two interviewees said they would call to consult the medical personnel.In addition,there were no full-time hemophilia doctors or nurses in the Shanxi Province HTC.P8 stated that they did not know how to contact professional medical staff if they had any questions about home therapy.N1 admitted that a lack of full-time nurses leads to non-continuous and non-homogeneous training.D1 also thought a professional nurse was necessary because it was helpful for patients to be instructed.Moreover,the short-term concentrated training offered by the HTC could hardly achieve the anticipated goal.P6 stated:“I still want to continue studying after the short-term class.”

3.4.2.Deficiency of hemophilia-associated managerial knowledge in community hospitals

Although some patients lived close to a hospital,they still chose to consult at the HTC farther away.The major reason was that the community hospitals lacked hemophilia-associated knowledge.P8 stated that their community hospital had never heard about the disease.P9 said that most community hospitals were reluctant to tutor them on how to inject.Community hospitals could not give instructions to patients regarding hemophilia home therapy.F4 stated:“If the community hospital close to my home could instruct us,I would feel better about home therapy.”P10 said that if the nearby hospital could assist them with problems,they would not have to go to HTC.

4.Discussion

4.1.Improving the understanding of families of patients with hemophilia in respect to home therapy

The cognitive bias of hemophilia families regarding home therapy derives mainly from a poor understanding and previous therapeutic experience.Medical personnel should advocate and popularize home therapy and supply training and supervision for home therapy.8They should also share positive home therapy experiences with other patients via peer education,which has gained consensus in multiple countries.

4.2.Exploring localized home therapy for hemophilia

A multidisciplinary comprehensive management approach guarantees the recognition of home therapy for hemophilia.9All members of the core team should possess professional knowledge and experience.However,such comprehensive management approaches should be implemented according to the current conditions in Shanxi Province.We proposed adopting a “step-by-step”strategy.Essentially,we established a primary comprehensive care team,a small home therapy center,and then gradually incorporate other departments to re fine the small-center structure.

The interviews suggested patient concerns about the safety of home therapy and a high-risk in adopting untrained home therapy.The training method of HTC currently entails centralized teaching,but different backgrounds among the educated population are not fully considered.Notably,it is also paramount to establish a home therapy-assisting nursing team to help them execute home therapy.10HTC should re-evaluate the treatment techniques of the patients and family members at intervals to guarantee the continuous and safe implementation of home therapy.In addition,home therapy should be evaluated in accordance with the evaluation criteria for effective home therapy proposed by the WFH.11Hemophilia nurses could then treat the patients and families who had carried out better home therapy as peer educators and conduct peer education focused on experience-sharing.Peer-based home therapy education may compensate for the lack of support from the formal health care system and therefore may be an appropriate approach to promote in China,especially in areas comparable to Shanxi Province.12

4.3.Coordinating medical insurance plans and increased treatment accessibility

A systemic evaluation of the economic burden of hemophilia in Mainland China indicated that approximately seventy percent experienced an obvious economic burden,which was documented by treatment status in Shanxi Province.

Medical insurance reform in China aimed to coordinate various medical insurance plans to reduce the portion paid by patients.By the end of 2015,critical illness insurance covered all the basic medical care in insured urban and rural populations.An improved critical illness insurance system will be established in 2017,which will effectively prevent catastrophic medical care expenditure.Such a medical insurance system would make it possible for hemophiliacs to receive assistance from all parties systematically.In 2015,Shanxi Province took various measures to fight for more drug assistance projects.

4.4.Accelerating provision of speci f i c care for hemophiliacs while gradually building up a continuous nursing service network

Professional hemophilia nurses play major coordinating roles in the multidisciplinary management of hemophilia.13However,both the hiring and skill levels of nurses involved in hemophilia care need to be promoted.In the meantime,the quality and extent of nursing supervision for home therapy remains finite.The accessibility of professional hemophilia nurses could accomplish homogenous home therapy training,ensuring that hemophiliacs can obtain comprehensive nursing support.14

The interviews revealed that most hemophilia families who did not continue with home therapy lacked continuous professional guidance and supervision.Furthermore,the available community's medical care lacked a good understanding of a long-term care perspective for hemophilia.The diagnosis and treatment system for hemophilia should be increasingly improved,centered at the provincial HTC and based around community health service centers.15The provincial HTC is responsible for training and nursing of hemophiliacs at more community hospitals.

5.Conclusions

Hemophilia care in China has made satisfactory progress over the past 20 years under the assistance of the WFH.However,despite the fact that patient survival rates have improved markedly,home therapy for hemophilia in Shanxi Province remains in a preliminary stage.Treatment conditions are unsatisfactory when compared with the standards in foreign countries.Inadequate family and social support systems have hindered the implementation of home therapy for hemophilia.More research is vital to establish a system of home therapy suitable for hemophilia families in Shanxi Province as soon as possible.

The current study only focused on families of patients with hemophilia in Shanxi Province,China and thus the conclusions were inevitably biased.Future studies should aim to support the qualitative data by an in-depth analysis of quantitative data in order to further our understanding as well as identify appropriate topics for additional study.

Conflicts of interest

All contributing authors declare no Conflicts of interest.

Acknowledgements

We wish to thank the patients,their families and the clinical personnel who participated in this study.

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