17/04/2012
The Haemophilia Project for KwaZulu Natal
Persons with Haemophilia have a bleeding tendency that is inherited, life-long and which affects
male children. The gene carrying the disorder is carried on the mother’s “X” chromosome. There
is a deficiency of a single factor (coagulation protein) which results in the failure of the blood to
clot. The common type (80%) of Haemophilia is Haemophilia A in which there is a deficiency of
factor VIII and the less common type (20%) is Haemophilia B in which Factor IX is deficient.
When there is a small cut or injury the bleeding in these patients does not stop because of the
missing factor. Unless this factor is replaced bleeding continues. Replacement of the factor at the
time it is needed allows the blood to clot and bleeding stops.
Many patients do not get the factor replacement in time and this results in severe swelling of
joints and muscles and bleeding into other sites. Bleeding into the skull or the abdomen is
potentially fatal.
Many patients have to travel long distances to receive treatment and this causes complications.
Nurses and doctors need to know about haemophilia to correctly manage these patients.
Common signs of a bleeding disorder includes:
- prolonged bleeding after circumcision;
- bleeding from the umbilicus;
- excessive bruising;
- bleeding from the mouth and nose;
- prolonged bleeding following minor trauma;
- bleeding into joints and muscles;
- prolonged bleeding after tooth extraction and surgery;
- family history of bleeding (brothers, male cousins, maternal uncles).
If the disorder is not very severe the diagnosis is missed and complications occur when the
patient is injured.
A project to improve care of persons with Haemophilia (Road to Haemophilia Health Project –
KZN) commenced on the 16 April 2009. The project was run by staff from the Departments of
Paediatrics and Haematologyat UKZN and the co-ordinator was Dr R Thejpal from the
Department of Paediatrics. The other staff involved in project activities include Dr N Rapiti and
Professor VB Jogessar from the Department of Haematology, Dr Y Goga from Paediatrics and
Sisters P Nkosi and NE Madonsela from the King Edward VIII clinic.
The main objective was to improve the care of Haemophilia in the Province and to increase
awareness of Haemophilia and its treatment among health workers and the public. One focus of
the project was the development of 2 further Haemophilia Treatment Centres (at Grey’s hospital
in Pietermaritzburg and at Ngwelezane hospital near Empangeni) and another was the focus on
education.
The Training of Haemophilia Nurses was one of the successes in the project and in July 2009
there were 4 KwaZulu Natal nurses trained via the project (by the South African National
Haemophilia Nurses committee) in Port Elizabeth. In October 2010 the project hosted a
Provincial Haemophilia nurse training workshop. At this workshop there were 25 nurses from
Provincial hospitals trained to be Haemophilia Nurses. In addition, we were able to
accommodate 5 other nurses from other provinces in South Africa.
In 2011 the project enabled 3 additional nurses to be trained at the National Training workshop
which was held in Nelspruit (Mpumalanga). Ongoing training also occurred at the Education day
of the Medical and Scientific Advisory Committee (MASAC) meetings.
The project successfully hosted 3 physiotherapy workshops focusing on Haemophilia Care and
over 100 physiotherapists and physiotherapy assistants participated. The main focus in the latter
2 workshops was for staff in public hospitals.
Medical staff Training was concentrated at the treatment centres at Grey’s and Ngwelezane
hospitals. At Ngwelezane there were 2 Haemophilia Orientation workshops and one at Grey’s
hospital. In addition, there were presentations by the haemophilia nurse and doctor in the Jim
Muller symposium at Grey’s hospital.
Patient Education and increased public awareness is an important component of improving care.
The project has assisted with patient newsletters, input by the treating staff (nurses and doctors)
on radio stations (Ukosi FM and Lotus radio) and a SABC television documentary. Some of
these activities peak around World Haemophilia Day (17 April 2012). At each patient AGM
there is a special focus on patient education; in the first meeting the focus was on recording of
treatment and keeping of the bleed chart and at the subsequent AGM the focus was on genetics
and there was a short physiotherapy session.
Camps for persons with Haemophilia have been held in 2010 and 2011. In the first camp (about
25 participants) the project made educational input (talks and supervision of product infusion).
This camp was primarily organized through the “ROCK” haemophilia group. A second camp
was arranged through the project and there were 26 persons with haemophilia who attended. At
this meeting special attention was paid to teaching home treatment and self infusion (using
mannequins bought through the project). Many of the participants learnt to mix and administer
their own medications.
The project has printed/distributed educational material for public awareness as well as other
sectors. These include copies of talks for workshop; physiotherapy booklet; haemophilia updates;
Haemophilia Treatment Guidelines; and other educational brochures. Through the project we
have translated and printed (with permission from the World Haemophilia Foundation) the
booklet Haemophilia in Pictures into Zulu.
The physiotherapy booklet has also been translated into Zulu.
An objective of the project was to improve the registration of patients on the Haemophilia
Register. At the time of the start of the Project there were about 500 patients on the Province
register. Since then patients have been verified and the register has been updated. The current
number (April 2012) on the Register is 666 patients.
The 2 treatment centres are functioning well and with the staff on site trained clinics will be
sustained ensuring easier and quicker access to care for patients. There are now “Haemophilia
doctors and nurses” at these hospitals.
Although the project has completed its 3rd year and is now completing project tasks the related
activities will continue through some of the achievements of the project.
Dr R Thejpal
Head: Department of Paediatrics and Child Health
Nelson R Mandela School of Medicine
Cell: 0825624491