Trainees Report:

We would like to thank Thalassaemia Society of Mauritius (TSM) and Thalassaemia International Federation (TIF) for sponsoring us for two weeks Nurse’s Training at Fujairah Thalassaemia Center under the supervision of Dr Mohammed Naveed and his Thalassaemia team.

It was an enriching and very informative training in which we were exposed to various aspects in the management of thalassaemia patients. During these two weeks it was a great opportunity to learn how patients with thalassemia are managed in a very scientific way using standard protocols by a dedicated team.

In Mauritius we have around 150 thalassaemia/haemoglobinopathies patients. Majority of these are over the age of 15 years. Currently the management of thalassemia is being done in various regional hospitals and private. In the absence of a dedicated Thalassemia treatment Centre, with a clinical haematologist trained in this subspecialty, the treatment and management of thalassemia remains quite varied from hospital to hospital.

The following topics were covered for the two weeks training:

Overview of Fujairah Thalassaemia Centre Beta Thalassemia Disorder Iron Overload Treatment of Iron Overload (Deferiprone) Treatment of Iron Overload (Deferoxamine) Treatment of Iron Overload (Deferasirox) Blood Transfusion Guidelines Blood Transfusion Reactions Bone Marrow Transplant

After our two weeks Nurses Training at the Thalassaemia Center of Fujairah Hospital UAE, we have identified the following problems in Mauritius regarding the Management and Treatment of Thalassaemia patients.

Treatment varies from Doctors to Doctors and hospitals to hospitals.

Treatment guidelines protocol are not being adhered to. Pre Tx Hb still not maintained above 9.5 g/dl in certain

hospitals.

Either patients (Thalassaemia Patients) are being under transfused or Sickle Cell Disease patients/ Thalassaemia Trait with Iron Deficiency are being over transfused.

Iron overload in our patients are not properly managed due to

- Lack of knowledge on doctors side – Calculation of the correct dosage of iron chelators, when to decide whether to use monotherapy or combination therapy.

- We have a limited stock of desferal, kelfer not available, only Asunra available in 400 mg, and 100 mg.

- Liver Iron Concentration (LIC) MRI R2* and Cardiac T2* not done. We rely only on serum ferritin level.

- Limited amount of desferal pumps.

Some investigations that are not available

- Hepatitis B Titre

- Hepatitis C RNA, PCR genotyping

- G6PD Screen

- Vit E and Zinc level

- Bone Densitometry, Vit D, PTH (being done in private only)

Vaccination for certain patients not up to date

Our suggestions:

Revising and updating the existing protocol. This has been carried out by Dr Mohammed Naveed on the 6th December 2019 in Mauritius in the presence of stakeholders. We even present our learning outcomes.

Training of dedicated doctors in the management and treatment of Thalassaemia as soon as possible. The best way is that an experienced Doctor from abroad come in Mauritius and train our Doctors. It will be more like a clinical training. At the same time the expert will assess and make recommendations for our patients regarding their management.

Education of patients regarding their disease, maintaining their pre Tx Hb, vaccination, iron overload and its complications, management of iron overload, compliance to medication self-infusion of desferal. This will be undertaken by nurses and doctors.

Management of liver disease, haemosiderosis, cirrhosis and hepatitis. Availability of Hepatitis B Titre, booster dose hep B Vaccine. Availability of hepatitis C RNA PCR genotyping and treatment of hepatitis C.

Support from Government /TSM for test that are not done in Mauritius e.g. Molecular Genetic Analysis, G6PD, Hepatitis C RNA PCR, Hep B Titre, vitamin E and zinc level.

Managing iron overload in our patients.

Education of Nurses, Doctors, Parents and patients. Drug compliance, Self infusion techniques.

Make sure all the iron chelators are available: Desferal, Asunra and Deferiprone.

Enough Desferal pumps are available.

MRI R2* and Cardiac T2* to measure Liver and Heart Iron Overload.

Blood Transfusion- All patients attending Thalassaemia Day Care Ward to wear the identification bands.- Maintained pre Tx Hb above 9.5 g/dl for Thalassaemia Major patients.- Calculation of volume of blood to be transfused according to protocol- Availability of blood pumps

Since treatment is not standardized in the different hospitals and based on our experience and training at Fujairah Thalassaemia Centre UAE, we would suggest stakeholders to set up a Reference Thalassaemia Centre where trained, experienced doctors, nurses will be able to manage our patients effectively and improve their life expectancy and quality of life.

A reference centre where patients from the four regional hospitals will be assessed by the Multidisciplinary team (Doctors, Nurses, Social worker, psychologist, cardiologist, endocrinologist, gastroenterologist) at least twice a year or when need be and where patients will be educated, psychological needs met, treatment are revised and sent back to their respective hospitals for routine blood transfusion and Iron chelation Therapy.

The reference centre will also be like a learning and teaching centre for patients/parents and Health Care Professionals.

Thanking Dr Naveed and Thalassaemia team of Fujairah Thalassaemia Center for putting their effort in this training. We are looking to put into practice what we have learned and strongly believe that we will bring a positive change in the management of our Thalassaemia Patients.