Iron Overload in Thalassaemic Patients

It it a need for patients with thalassaemia to be transfused with blood at least once a month especially when they are of thalassaemia major which is the severe form that need blood tranfusion.

And, sadly to say; there is no medication in this world that can be given without any side effect. Though blood tranfusion helped them to prolonged their life as campared to those in the ancient time, it brought up another problem for them - Iron overload.

Those excess iron as a result of repeated tranfusion couldn't possibly be excreted by our body, hence starting to accumulate in our body in the form of ferritin. When in excess, this will lead to a condition called as Haemosiderosis and Haemochromatosis.

Moreover, this excess iron tend to accumulate in the vital organs such as the heart (heart failure/cardiomyopathy), liver (hepatic cirrhosis), pancreas (diabetes mellitus) and endocrine organs (hypopituitarism and hypogonadism).

Therefore, iron chelation therapy is the choice of options in order for them not to get the side effects from repeated blood transfusion. Usually, iron chelation therapy is only needed after 20 transfusion have been done as each unit of blood tranfusion will give them an excess of 200-250mg of iron.

There are 3 form of iron chelating therapy available :

Desferrioxamine

- subcutaneous injection via an infusion pump (5-7x per week) for 8-12 hours
- indicated for those > 3 years old, ferritin > 1000 ug/l after 10-20 tranfusion
- promote urine excretion
- s.e = skin rashes, yersinia infection (fever, abdominal pain & diarrhea)
- cost - RM 500-1000

Deferiprone

- oral tablet form trice daily (tds)
- usually use in combination with desferrioxamine
- chelate iron from heart also
- s.e = agranulocytosis, joint pain,nausea, diarrhea & elevated liver enzymes

Desferasirox (Exjade)

- once daily dose tablet (OD)
- excrete iron via defaecation
- s.e = nausea/vomiting/abdominal pain, rashes,increased creatinine and liver enzymes


Other options available would include Splenectomy and Bone Marrow transplant

Spleenectomy

- when there is hypersplenism, large spleen or excess haemolysis
- indicated for those > 5 years old, transfusion of more than 200-220mls/kg/year.
- pancytopenia
- s.e = increased susceptibility to bacterial infection (meningococcus/ penumococcus/haemophilus influenza b) - need vaccination 4-6 weeks before surgery
thrombocytosis (use aspirin)

Bone Marrow Transplantation

- ideal choice of treatment (cure rate > 80%)
- allograph transplant from HLA-matched siblings
- s.e = infection, graft rejection and graft versus host disease
- greater chance of success rate if compliant to iron therapy, < 2cm of hepatomegaly and no portal fibrosis

Thalassaemia Awareness Talk in HTJ

Attended a talk organized by Negeri Sembilan Thalassaemia Association in conjunction with a pharmaceutical company for lay person on thalassaemia last Saturday which was rather interesting. An one day whereby they educate lay person about the hereditary disease. The programme lasted from 9am to 2pm and was started with short talk from HTJ Pengarah and Head of Department of Paediatric in HTJ.

Amusingly, it's all communicated in Malay language which i think most of the talkers find difficulty with when they have long communicate in English ever since they joined the medical profession.

There was a talk on the overview of thalassaemia disease which they keep on emphasizing that it's not infectious followed by another talk about the management, working opportunity and issue in pregnancy.

Of course, the most interesting part would be from the pharmaceutical company where they have 2 short video summarising the effect of iron overload and the life of thalassaemic patients after they found out the oral solution instead of the injection. undoubtterly, the cost was not shown, just to terrify the public. haha

The lousiest part? it is a talk by the personnel from government working resources. Basicly, she was just not prepared for this talk. She gave a talk on how to get a job for a normal person without any consideration for thalassemic patients who would require more holidays for their blood tranfusion. Bad day for her, as she actually manage to trigger the rage from some of the patients. How impressve...

Anyway, we were glad to have the free guideline for thalasaemmia which would be very useful for us. Though the breakfast and lunch was provided, it was not really a good meal as there was a lot of people attending the talk. Once you are late in queing there goes the 15 minutes just for that.

Gynaecological Posting

As far as it's concerned me, gynaecological(gynae)posting is really hectic that i hardly can make out some time to read. What am i busy with? Oncall almost every other day from 5 to 11pm. Worst of all, we need to stay in the ward from 5 to 7pm when it doesn't make sense at all as it' s the visiting hours for family members.

What are we supposed to do there? standing there and blocking the way when there is already too much crowd? This is a real crap... When someone mentioned that houseman is also there, i really wanted to just slap that ill-looking face. The houseman need to be there for the patient to take care of them. What are we as a tiny little creature in the ward can provide? Not even information when what we know is actually not much...

Perhaps they have misunderstood the real purpose of being in the ward, which is for us to learn. But learning has to be an initiative for everyone and not covering the ward full of patients for the sake of covering. We are there in the ward to learn about managements of patients and not to learn to cover ward for the sake of fearing some lecturer who would come to the day the next morning asking about the patients.

This week has been a little bit crappy due to some unreasonable people who just wanted to cover themselves up. Anyway, i do hope gynae posting for another 3 weeks to be fun and interesting that nobody else ruin it.