The Changing Needs of Hemophilia Patients Through Different Life Stages

Alison Street, Megan Walsh and Penny McCarthy Ronald Sawers Haemophilia CentreThe Alfred Hospital Melbourne October 2011

Topics to be covered

Infancy The early years School Adolescence/transition Early adulthood/career choices Aging issues

Infancy

Diagnosis

• With family history Diagnosis at or before birth

• No known family history Diagnosis is often delayed .

Diagnosis usually occurs before 12 months of age with the average age of diagnosis being around 6 months. Only 15-30% of newborns with an inherited bleeding disorder present with bleeding in the neonatal period

Infancy, family and clinician awareness

• Cephalohaematoma

• Bleeding post heel prick, venepuncture (circumcision)

• Excessive bruising, muscle or joint bleed

• .

•

Infancy - Issues for Family

A diagnosis of Haemophilia can affect the whole family

Parents Siblings Grandparents and other

relatives

How do we support the family after diagnosis?

Education on bleeding disorders, including the genetics of the disease

Attention to psychosocial care, family, community and professionals

Networks for new parents

The Early Years, a time of uncertainty

• Fear in parents and families for the next bleeding episode and treatment

• Associated costs and disruption to family life

The Early Years

Painful procedures and multiple venous access attempts can become a barrier to families and boys seeking and participating in optimal management of their condition.

The Early Years

• If product is available regular replacement therapy (prophylaxis) may commence after 1st or 2nd joint bleed

• Infusaports may be inserted to reduce anxiety and fear but are not always needed

• Home treatment gives some degree of control and “normality” and allows boys to be involved in their own treatment from an early age

School Years • The child needs to develop motor and social

skill along with his peers

• This is often difficult for parents, teachers and others who look after the child, who are often fearful of bleeding disorders and need information

• It is important that professionals support the family in encouraging the child in progressive independence

Promoting benefits of Sport and Exercice for

• Enhancing rehabilitation for muscle strength and endurance and joint stability

• Improved confidence and self esteem

• Greater opportunities for socialization both during sport and leisure activities

• Maintenance of appropriate body weight

What Sports ?

Factors influencing a persons choice of exercise include:

-Age

-Frequency and sites of bleeding

-Existing muscle or joint damage

-General body build

-Facilities available

-Preferences

Recommended Sports

Most people with Haemophilia can participate in:

- Swimming

- Bicycling

- Tai chi

- Walking

Rehabilitation, Sport and Exercice – The Physical Therapists Role Advice on rehabilitation after a bleed Examination and documentation of

musculo-skeletal status Suggestions for physical activities

including sports participation Liaison with community physical

therapists and others

Adolescents- their issues

• Preparing for the privileges (and responsibilities)of independence of adulthood

• Learning to “put life first” and not let the bleeding disorder control their life

• Not wanting to appear different

• Challenge advice including management of their disease

Adolescents - Issues for Parents to discuss

• Difficulty with “letting go” – over protection by parents when boys undertake risk taking behavior

• Uncertainty about disclosure of bleeding disorder outside the family (not wanting to be different or compromise employment or social opportunities)

• Less control over son and his environment

Adolescence - Health Professionals Role

• Start treating the adolescent as an individual, separate to his parents

• Support parents in letting go and setting boundaries

• Start preparation for transition to adult centre early

• Communication-what works?

Don’t forget about women

• Who may suffer from a bleeding disorder or be carriers

• Also need to be identified and counselled

• Additional issues of menses and pregnancy which need support of gynecologist and primary care physicians

Adulthood and advice on

• Career choices (education gives better range of options)

• Possibility of physical work

• Support with work /study issues

• Disclosing Haemophilia outside family

Ageing

• Many people with Haemophilia especially with access to safe, adequate supply of product are living well in to old age and can expect similar if not equal life expectancy compared with the rest of the population

• A persons quality of life in ageing will be influenced by their life time experiences of health, socialisation, education and employment

Chronic disease management

Ageing includes risks of

• Cardiovascular disease, diabetes, hypertension and cancer

• Prostatic disorders

• Osteoporosis• Impaired balance and falls

Ageing and Mobility

• Impairment worsened with arthropathy, reduced balance and increased risk of falls with subsequent risk of injury

• Affects independence and ability to get to appointments/social activity etc

• Important to assess for and provide mobility aids

Ageing and pain management

• Pain affects mobility and sleep

• May require medications which can further affect mood, balance and coordination

• Chronic pain can lead to depression, social

isolation etc

Ageing- the health professionals role in education

• In support of carers too

• To maintain healthy exercice regimes (including aerobic, strengthening and balance)

• About regular general health checks and men's health screens..NO SMOKING!!

• To other agencies/specialists on care of patients with bleeding disorders undergoing procedures

Recently published and at www.wfh.org

Summary 1.

Many issues arise over the lifespan of a person with a bleeding disorder both for the person and his family. It is a family disease

“Normalizing” childhood and adolescence allows boys to develop self esteem, to form social networks, and ultimately learn how their disease fits into their life

Summary 2

Exercice and physical fitness are important in the life of person with a bleeding disorder from childhood to old age

A satisfying family, social and working life with healthy development and ageing are possible with good planning and support

ReferencesEgan, Brendan

Boys will be boys –A guide to sports participation for people with haemophilia and other bleeding disorders,2005

Jones, Peter

Living with haemophilia, 5th edition 2002

Mauser-Bunscoten, Evelien et al

Aging With Haemophilia-medical and psychological impact ,2007

Dolan Gerald

Haemophilia and Ageing, WFH monograph 2011

Resources

www.wfh.org