Case 73 – update 1

Thanks for all your suggestions so far. When the patient was seen for the first time the following was established:

History:

She is now age 10 and moved to the UK from west Africa a few months ago with her parents. She has a cousin with sickle cell disease but has no siblings. 

She has not had any previous chest problems apart from a course of antibiotics for a chest infection a few years ago. She does get pains in her legs but never needed hospital admissions for pain.

Since her move to the UK she has had a few episodes of leg pains intermittently. These settle with rest and paracetamol plus ibuprofen. She has had a few days off school because of this. Her mum feels the cold weather is to blame.

She has never had blood transfusions.

Her mum is unsure if she has been vaccinated.

No concerns with her development. 

She has never had TCD measurements. 

 She has no sign of puberty at present. 

She has no symptoms of picca,enuresis or dyspnoea.

On examination:

She is between 9th and 25th centile for height and 25th centile for growth when you plot it on a growth chart.

Her blood pressure and pulse are normal for age. She has oxygen saturations of 97% on air. 

Her chest is clear, heart sounds are normal and she has no palpable spleen. 

Investigations:

Her urinalysis is normal.

She has a baseline echo arranged given slightly low oxygen saturations this shows no signs of pulmonary hypertension. 

She has a blood film that was reviewed and showed prominent sickle cells and Howell jolly bodies. There were a few target cells but not the predominant feature.

Her Baseline Hb is around 90

HPLC confirms Hb SS. This sample was sent to another lab for confirmation as this was her first attendance to the hospital.

She has a high retic count and normal ZPP.

She has blood phenotyping given the possible need for transfusion. This includes full ABO, Rr,Ee,Cc,Ss (U type if s neg ),Fya,Fyb,Jka,Jkb typing. Genotyping for Rh group could also be considered as variant RhCE is more common in these patients.

She has a full viral screen performed.

Management:

You introduce her to the haemoglobinopathy specialist nurse and she is going to have a home visit and discuss the national haemoglobinopathy register with the family. 

You prescribe penicillin V and folic acid. 

You speak to her GP regarding need for repeat prescriptions for penicillin and folic acid.

 You also arrange catch up childhood immunisations and inform them of the need for 5 yearly pneumococcal vaccinations, annual flu vaccinations and arrange for hepatitis B vaccines. 

Her TCD is booked for next week and is to be performed annually. 

You are feeling rather pleased that you remembered to cover so much in your first meeting. 

A week later……

You get a call from the TCD list saying that the patient has a Right MCA velocity of 210 msec

What is she at risk from?

What evidence supports management in this situation?

Should we transfuse her? If so how?

What requirements should be met as a minimum if she is to have a blood transfusion?

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