Good-bye Port Pie! - Haemophilia Foundation of New Zealand Inc.

Saturday Morning Sessions
Susan discussed the following potential problems at birth in
detail:
1. Intracranial haemorrhage
2. Subgaleal haemorrhage
3. Bleeding from blood vessel trauma
4. Bleeding from circumcision
5. Bleeding from umbilicus
6. Other
The recommendations for management of possible and known
carriers of haemophilia A & B are as follows:
• Consider pre-conceptual genetic counselling
• Genetic counselling to discuss antenatal diagnosis and gene
mutation analysis
• Referral to an adult haemotologist
• Factor 8 levels to be measured at booking,28 & 36 weeks,
during labour and prior to any invasive procedures
• Arrange for provision of recombinant factor for the mother if
necessary
• Ideally, carriers be delivered in centres with access to an
“adult” haemotologist, a high risk obstetric unit and neonatal
unit, a “paediatric” haemotologist, a specialised anaesthetist,
a blood bank with Factor, and a laboratory to measure factor
levels.
Susan stressed that regardless of where delivery occurs;
communication between the entire health professional team
involved is important and a delivery plan should be established
in advance. Although the safest method of delivery for an at-risk
babe is controversial, there is an over-riding principle - deliver
by the least traumatic method!!! Carrier mums with factor levels
less than 50% should be kept in hospital for 3 days as they are
at increased risk of postpartum bleeds (more than 500 ml)
Some of the recommendations for the management of a baby
born to a possible or known carrier of haemophilia are as
follows:
• No foetal scalp electrodes to be used or foetal blood sampling
performed
• Cord blood to be collected
• Consider venepuncture to confirm initial result
• Give vitamin K to prevent Haemorrhagic Disease of the
Newborn
• Circumcision not to be performed until status determined
Giving a dose of Factor8/9 at birth to all boys born to known
carriers has been advocated by some but is not widely practised.
There is a possible increased risk of inhibitor development from
early exposure to factor 8.
Giving a dose is recommended if the baby had a traumatic birth,
there is an obvious bleeding problem, the baby is very ill at birth,
or intracranial haemorrhage (ICH) is suspected. Imaging of the
head should occur if the babe is symptomatic of ICH, had a
traumatic delivery or is premature.
13 CONFERENCE SUPPLEMENT, December 2007
We thank Susan for this very informative talk on a topic that
nearly everyone in the room has had or will have personal
interest in.
Menorrhagia: best care and practice
- Dr Julia Phillips, Wellington Hospital
Menorrhagia (excessive or prolonged cyclical menstrual blood
loss), is a major health problem that often goes unrecognised by
patients and doctors alike. It affects 5-10 per cent of all women
at some time in their life. It is the cause of a reduced quality of
life, iron deficiency, gynaecological surgery, lost work time and
increased health costs.
The definition of “heavy” is: over 80 ml blood loss per cycle;
blood loss for more than 7 days; or heavy menstrual loss that
has an adverse effect on daily life.
Julia explained the different ways of measuring blood use and
posed the question - is 80 ml a useful criteria when more than
half the women referred with menorrhagia had less than 80 ml
loss.
Congenital bleeding disorders are found in up to 20 per cent of
women suffering menorrhagia. The most common of these is
von Willebrands disorder, in which approximately 90 per cent of
women are affected by heavy menstrual bleeding.
Non-surgical treatment options for women with menorrhagia
include: intranasal DDAVP (desmopressin), antifribrinolytics
(tranexamic acid), COCP (combined oral contraceptive pill), 21day
POP (progesterone-only pill) and the mirena intrauterine
system. Surgical treatment options are endometrial ablation
and the totally invasive hysterectomy. Both of the latter cause
infertility.
Julia concluded by saying both the definition and recognition of
menorrhagia could be improved; it is important to consider age,
childbearing status, and preference in terms of efficacy, sideeffects
and need for contraception in selecting treatment options;
and this condition is ideally managed by a multidisciplinary team
including a gynaecologist and haemotologist, in a comprehensive
care centre.
Lorraine Bishop - Porter and Robyn Coleman from the Southern Region.
Saturday Afternoon Sessions
Scientific and psychosocial snapshots -
new initiatives and progress
By Sandra Poff
I felt very privileged to attend the Conference held in Canberra.
I attended as a delegate of the Southern Branch and as the
mother of a two and a half year old with severe haemophilia.
I attended five sessions on the Saturday including “Scientific and
Psychosocial snapshots - new initiatives and progress”.
The measurement of physical activity in boys with
severe haemophilia
- Brendan Egan, Royal Children’s Hospital, VIC
(Co-Authors: Dr Chris Barnes, Dr Bev Eldridge and
Dr Rory Wolfe)
Brendan Egan reported on a study he had conducted with
colleagues. It involved a study of 17 boys with severe haemophilia
wearing a device called a PAL-1 for 4 consecutive days which
measured uptime (time spent in an upright position).
None had inhibitors present, all bar two were on prophylaxis
and only 11 were actually monitored for the full 4 days (monitor
failed to record/kept resetting itself in some instances). One
presumption made was that the activity levels of all boys were
comparable. No consideration was given to individuals who had
target joints or other such issues.
Historically, evidence has indicated that boys with haemophilia
are not as fit and active as their peers and in some cases
overweight. This could be a consequence of the idea that
physical activity in boys with haemophilia was discouraged 15
years ago or also as a result of many lost days for exercise
due to reoccurring bleeds in some children. Today, however,
the benefits of exercise, particularly weight bearing exercise is
promoted in all circles.
One of the conclusions reached from this interesting study was
that boys with severe haemophilia are as active as their nonbleeding
disorder peers.
The intent is for an updated version of the PAL-1 to be employed for
studies that will also be able to measure intensity of exercise.
Workshop for young women and bleeding disorders
- Colleen McKay, Haemophilia Foundation of New Zealand,
Outreach Worker for the Southern Branch
In her role as Outreach Worker for the Southern Branch of the
Haemophilia Foundation, Colleen was keen to promote and
discuss issues for young women with bleeding disorders. She
organised a workshop weekend (Young Women’s Weekend
Workshop, YWWW) for women from the ages 13-30 that have
von Willebrand Disorder or who carry the haemophilia gene. 22
women attended, 18 who carry the haemophilia gene, 4 with von
Willebrand Disorder and 3 with both.
The three main objectives of the workshop were:
• To provide participants with information and education, thus
empowering participants to make informed choices for their
health care now, as well as preparing them for the future
Australia and New Zealand Social Workers Group - can you spot yours?
• To provide the opportunity for participants to work through the
issues associated with their condition
• To develop a sense of community within the group, so that
participants and other young women could form a support
network to help them to encourage each other as they overcome
the barriers created by their bleeding disorders
The workshop consisted of information and education on:
• Genetics, the basics of von Willebrand Disorder and
haemophilia
• Reproductive choices for women - Dr Mark Smith
• von Willebrand Disorder - Ally Inder discussed the types,
differences, signs/symptoms and how to understand laboratory
test results
• Bleeds, treatments, products and protocols
• Dentists - how to keep a healthy mouth/information pertinent
for those with bleeding disorders
• Natural options that may help symptoms
Excellent facilitated discussion groups where held and attended
by the women on lifestyle issues such as “Disclosure” - who to
tell, when, how; and “Quality of Life” - where to find information/
help about reproductive decisions
The programme was interspersed with a recreational programme
that included a movie, a competitive quiz night, karaoke,
aquacise, laser strike, bead making and pampering sessions.
Also a contact list for those attending was circulated for that
invaluable peer support.
Participants were asked to evaluate the workshop as this was
the first time such a weekend had been held for women with
bleeding disorders.
All education sessions ranked highly and special interest
appeared to be in the one on reproductive choices, vWD and the
facilitated discussion groups.
December 2007, CONFERENCE SUPPLEMENT 14