| ObjectiveHcmophilia is an X-linked disease charactcrized by the lack of factor VIII(F VIII) or factorIX(FIX) which is inheritable, congenital and lifc-long discasc without a cure at the momcnt. Affected individuals are vulnerablc to rccurrent blecding episodes which are painful, disabling and potentially life threatening. The prevalence of hemophilia is about five in 100 000 population with no difference in regent and races. The conception of Hemophilia Comprehensive Care is that an organized multiple health carc team provide treatment and nursing for people with hemophilia (PWH) and his family, which include homc care, health education, social support, management. In USA and UK, PWH who received hemophilia comprehensive care delivered by HTC (Hemophilia Treatment Central, HTC), their health quality have been improved dramatically. There are scveral notable difference. The average days spent for inpatient care were reduced from 9.4 to 1.8, The average days lost from work or school were reduced to 33%, the overall cost of patient care per year declined to 30%, from＄15800 to＄5932, the number of unemployed adults decreases from 36% to 12.8%, and the CDC documents a 40% decrease in mortality. In developed countries, 90% of the patients are diagnosed, the average diagnosed age is 0.83, the replacement treatment began at 1.5. The children and adolescents with hemophilia less than 15 years old were not chronic arthropathy cause by bleedings,. Life expectancy is about 60～65. The life quality was not difference to normal people.In our country, due to socioeconomic situation, there is not a health care system for PWH, almost HTC have not a comprehensive care team, and there is not a guideline for Chinese PWH in treatment and nursing. The quality of life is far away from developed country's. 2006, Tianjing Institute of Hematology and blood diseases hospital report that there are only 5000 PWH from 11 hospital been diagnosed, belonged to the estimated number 7%. The report revealed that almost PWH have not been diagnosed, and have not received health care. Most of them were silk and disabled, and in the situation of pain and bleedings and high mortality rate. The situation of children and adolescents with hemophilia were not optimistic. There are significant delay in diagnosis from initial symptoms of patients, diagnosed ages was 5.2 and the treatment start at 4-5, and most of them received inadequate treatment which lead to high prevalence of joint deformity and disability.This research is based on the PWH and their family, by conduct a survey of Children and Adolescents with Hemophilia about treatment and nursing situation, according developed country comprehensive care experience and Chinese situation, to set up a hemophilia comprehensive care to delivery the low-dose replacement treatment for children and adolescents with hemophilia comprehensive care。The purpose is to research the hemophilia comprehensive care model conduc(?)ed by hemophilia nurse and doctor cooperation and to educate the patients and family, improve the self-care skill and the treatment outcome, decline the bleed and costs, improve the quality of life.Relying on the cooperation with WFH, the research was conduct in two part: 1. the first one part of the study was performed by sending questionnaires to Children and Adolescents (4～18) with Hemophilia in China to asking for treatment and nursing situation include the epidemiological and clinical information on all haemophilia A or B patients they have ever managed. Information enquired included patients' demographic data; severity and type of hemophilia; maternal carrier status; use of prophylactic factor replacement; development of inhibitor, HBV, HCV or HIV infection and any other significant complications. Definitions of clinical status were specified in the questionnaire FISH. 2. According the result of the part 1, we carry out the low-dose replacement treatment for children and adolescents with hemophilia comprehensive care to educate the patients and family, improve the self-care skill and the treatment outcome, decline the bleed and costs, improve the quality of life. Subject 4～18 children and adolescents with hemophilia and their family.Methods1 The first stage: the survey has been finished in 7～8. 2006.1.1 The questionnaires design:〈the questionnaires of treatment and nursing situation of Children and Adolescents (4～18) with Hemophilia〉is base on international paper investigation and〈Administering intravenous therapy to child in the community setting〉, and modified by professor in nursing, epidemics, statistics, homologies and the adults with hemophilia. Assess Treatment including initial age, diagnosed age, replacement started age, bleedings/year, lost days from school, overall cost, joint and muscle deformity. From the use of RICE, medicine storage, medical disposal, assess the bleeding severity et al to evaluate home care status. The FISH been used to evaluate the joint status.1.2 the study was performed by sending questionnaires to Children and Adolescents (4～18) with Hemophilia in China to asking for treatment and nursing situation in the past lyear during 2006.7～8. The total questionnaires are 349 and the response are 247(70.7%),the valid are 215(80.04%)2 The second stage: The Research of Low-dose replacement regimens comprehensive care model for Children and Adolescents with Hemophilia2.1 According the record of patience and family's supplement, we investigate situation of treatment and nursing about the 4～18 years old PWH in Guangdong province·using self-compare trial design, eliminate 4 patience who have sever bleeding in the past half-year and lpateince who have inhibitor. The total number of the patient is 37 who are in research list. After 6 month comprehensive care, there are 30 patience follow the trial. To compare the data before and after comprehensive care, we discussed the success and defeat.2.2 The Low-dose regimens replacement comprehensive care model for Children and Adolescents with Hemophilia: Relaying on Guangzhou HTC, we set up a comprehensive care team comprised by hematologist, hemophilia nurse, physical therapist and laboratory technician. Hemophilia nurse is the core in the team and commitment majority of the treatment and nursing including home care, infusion teaching, education, coordinate et al. the family and patience received low-dose replacement which is the team members' order base on the patience clinic performance and income. Hemophilia nurse support the family during the patience's different status how to nursing and treat the child, to prevent majority bleedings, save the concentrates and decline the cost, and improved the quality of life.Result1The fist part:1.1 There is no significant difference in age, weight, severity in 4～18years old PWH between city and rural area.Total patience are male, average age is 12.0, average weight is 40.6kg. hemophilia A 182(84.7%), Baseline factor levels18 were mild, 81 were moderate, 49 were severe and 34 unclear, hemophilia B 30(13.9%), 2were mild, 17 were moderate, 11 were severe. Other hemophilia were 3(0.4%). 123 were living in city and 92 were coming from rural area, There is no significant difference in age, weight, severity in 4～18years old PWH between city and rural area.1.2 Insurances and lower-incomes were the major barrier to hamper treatment and care of PWH, worse situation with PWH in rural area.The average family income were 2253.8(150～40000)yuan/month, about 15% families' income were lower than 1500yuan; 90% patience have no health insurances; 42.7%family report that they can only afford minimal medical cost, 7.4% were completely can not afford the medical cost. There are significant difference between city and rural area.1.3 This is significant delay in diagnosis from initial bleeding symptom of patience.The average initial bleeding symptom of patience in the 215 is 1.4 years and the diagnosis age is 2.59 years, this is significant difference between diagnosis age from initial bleeding age. The initial joint bleeding were 3.23 and the replacement start at 3.74, this is also significant difference between replacement start age from initial joint bleeding age. It reveal that 4～18 PWH received treatment is late, and the patience coming from rural area is more later.1.4 Treatment outcome from 4～18 PWH is worse than other countries, and high incidence in complication.1.4.1 The choice of treatment: because of economic situation, the choice of treatment in patience is different. 60.1%city patient choice on-demand therapy while 50% rual area patience is occasionally treat the bleeding.1.4.2 The choice of medicine: most of patience choice concentrates, while 31.2% use cryoprecipitate, plasma and blood1.4.3 There are more bleedings and lost days from school in 4～18 patience in china, the treatment is inadequate than other countries. The average bleedings per year are 27.2, average visit clinical are 10.3 / year, the days lost from school are 68.9days/year, the in-patient days are 5.64day/year. The consume of the concentrates is obviously inadequate which is 250 U/kg. The whole year medical cost are 11840yuan/PWH. There are significant difference between city and rural area. The situation in rural area is worse.1.4.4 44% family report that their children have sever bleeding history including intracranial bleeding, gastrointestinal bleeding, muscle bleedings et al.1.4.5 In our series, 3 PWH HBV infection, 12 HCV infection, 4 HIV infection while 100 no infection and 89 had not test virus screening.1.4.6 In our series, there is high incidence in complication of hemophilia. The incidence of chronic arthropathy is 61.4%(132PWH). The joint most often involved are knee, elbows and ankles. The rural area patience suffers from higher incidence than city patience. 58.6% joint restrict, 27.9% muscle heamatomas, 8.8% nerve injury.1.4.7 FISH: Only 22.6% patience's score are normal, more than 70% are lower than 27. the city patience's score is higher than rural area.1.5 The PWH and their family lack of education about hemophilia, so that the home care quality is terrible.1.5.1 Most of PWH and family know the reason and the inherent regulation about hemophilia. Only 24.5% received hemophilia health education, 66.5% report that they do not know how to assess the severity of bleeding. Only 30.8% take part in home treatment, and 24.3% are monitor by HTC. 24.3% family will director his child to take exercise regularly. 146 patience used to take pain killer, but 37.0% was wrong. Almost family know how to store the medicine, but only 22.5% know how to keep the medical disposal. Only 67.5% family have the treatment record, the city patience's situation is higher than rural area1.5.2 Use of RICE: in our series, almost family have no idea about RICE, and 18.5%can complete the RICE, and most of them just take rest and ice. the city patience's situation is higher than rural area1.5.3 Home therapy: in our series, only 60 family take part in home therapy. The others have not because:①81.4% due to the difficulty of IV②42.8% due to the lack of support from health care③32.4% due to lack of concentrates④32.4% do not know how to calculate the concentrates according bleeding;⑤10.3% due to economic situation or scare of infection.1.5.4 Exercise: because of cause bleeding and no knowledge about physiotherapy in hemophilia-exercise, only 24.3%children exercise regularly and 34 choice walking, 8 choice swim and bicycle.1.5.5 Preventive care: 25.0% will restrict the hemophilia child all activities while little family take the fight preventive care such as wearing protection when sports and activity.2. The result of low-dose factor replacement comprehensive care for Children and Adolescents with Hemophilia:2.1 Patiente characteristic: 37(4～18 years old ) PWH in the research trail. After 6 month comprehensive care, there are 30 patience follow the trial. Baseline factor levels were reported of patients among whom 2 were mild, 12 were moderate and 10 were severe and 6 were unclear. The average first initial bleeding age were 1.4 and average diagnosis age were 2.6. This is significant delay in diagnosis from initial bleeding symptom of patience. The first joint bleeding was at 3.3years, and average replacement start at 3.9. among 30 patience, 21are living in city, while the others living in rural area. 8 have medical insurance, average family income were 3190 yuan, and 50%fmily report that they only can afford apart of health cost. late HBV infection, 4are HCV infection, no HIV infection. 3 PWH have sever bleeding history.2.2 The comparison between comprehensive care before and after2.2.1 reatment and medicine: after comprehensive care, more family choice more positive treatment and more safer medicine.2.2.2 Out come of the comprehensive care: in the trail 30 PWH have better outcome such as bleedings decline from 15.6 to 10.6, visit clinical decline from 6.9 to 3.5, the lost days from school decreased from 26.4 to 14.8, the dosage of concentrates increased from 168 U to 216 U but the overall cost was not increased significant. This is revealed that the medical fees been better used, but the treatment dosage of concentrates was not adequate yet.2.2.3Complication of hemophilia: there is not significant difference between before and after mainly because of the trail follow time only 6 month.2.3 Home care situation comparison2.3.1 The home care quality have been improved significantly. All of the PWH and family know the cause and the inherent regulation; more parents can assess the bleedings(13:24), more family take part in home treatment(9:19), 28 PWH take exercise regularly, all family take medicine correctly, 19 family treat the disposal in right way and no sharp injury, 30 PWH have complete and accurate record of treatment and care.2.3.2 Use of RICE: 30 PWH and family have better comply with orders. All of them can use RICE after bleeding.2.3.3 Preventive care: no family restrict the hemophilia child all activities while more family take the right preventive care such as wearing protection when sports and activity. 2.3.4 Home therapy: 19 PWH and family take part in home treatment. The average ages is 12.7, weight is 44.6kg, 2 is mild, 7is moderate, 8 is sever and 2 were unclear. The data suggest that bleedings, clinical visit, lost day from school have significant decline, the use of dosage has increased, but the cost was not increased.During the home therapy, there is no sever bleeding, infection, sharp injury, 2 PWH have mild allergy reaction.Conclusion1 Care for haemophilia patients is costly with a significant proportion of expenditure being spent on factor concentrates. As these essential medications are not provided and the whole payment depend on the PWH family, it would be reasonable to assume that most haemophilia patients in china will not received the adequate treatment and care. The basic education was not delivery and diagnosis was delayed and home care situation was terrible. There is high incidence in hemophilia complication.2 The most important measure to improve quality of life for PWH is to build up a comprehensive team and delivery the affordable low-dose replacement and basic comprehensive care. low-dose replacement comprehensive care conducted by HTC will resave the factor concentrates, declined bleedings, and get the better outcome in stop bleeding. The low-dose replacement comprehensive care for hemophilia joint out need follow up long-time.3 Improving the home care level, starting home therapy is the fist step for developing country to improve PWH quality of life. The health education will modify the care in home, better their self-care skill. Let them feel more confidence. Moreover, the PWH and family are monitor by HTC and PWH will live in a safer environment. They can receive treatment intime, and can live a normal life.
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