Screening for sickle cell and beta thalassaemia

How well do people understand the tests offered?

A few people we talked to had actively requested carrier screening, either before or during pregnancy, but most were offered it automatically. For professionals, this raises the question of whether people understand the tests and what the results might show, and whether they have given informed consent. 

As is often the case with antenatal serum screening tests, many of the people we talked to saw screening for sickle cell and beta thalassaemia as just another routine blood test in pregnancy and did not expect any bad news. As a result, most paid little attention to what the test was for. Not surprisingly, those with a family history or other prior knowledge of the conditions tended to be more aware of the significance of the test. 

Several people felt with hindsight that they had not been fully informed about the test, or prepared enough for the consequences of a 'positive' test result. A few people believed it was the law that they had to have the test, although in fact everyone has a right to say they don't want to be screened. 

Professional learning: He felt screening in pregnancy was just a routine part of care. He would...

Professional learning: He felt screening in pregnancy was just a routine part of care. He would...

Age at interview: 29
Sex: Male
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I'm interested in sort of what, what made you both willing to have screening in the first place. Was it something you hadn't thought about at all and it just seemed like everybody did it?

Well, I know that is the norm in this country, that once you are pregnant, all pregnant women have to take the screening. So, well, when her GP actually said she was going to take the test this day or that day, I didn't think of even saying, 'Oh no, don't go.' It's, I think it's I took it as normal, you know, a normal thing. So I never thought of not going. Never. Yeah. I never thought of it. I think it's a normal, normal thing. But to tell me to go for the screening without the pregnancy, then I don't think I would have gone, because I always think I'm strong, I'm not sick. I don't even know my GP, after four years of being in this country. I've got a GP. I know his name, but I haven't seen him personally, because I hardly fall sick. I'm always there. So to tell me, 'Go and take the tests', I don't think I would have gone. But because of the pregnancy, I know it's a general phenomenon, so she has to go, and that's it.

Did she ever think of saying no, or -?

No, she didn't. She didn't, because she just went, yeah.

So that was the main thing that made you both - it was just the fact that it was a normal part of care?

Yeah.

Footnote' often people do not realise that being a carrier does not affect your general health and you may have no idea you are a carrier unless you are tested.
 

Professional learning: She did not know what all the blood tests in pregnancy were for. It did...

Professional learning: She did not know what all the blood tests in pregnancy were for. It did...

Age at interview: 22
Sex: Female
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So did, when they took all these blood tests, did you know what any of them were for?

No, they just took them. They didn't say, they just wanted to check I think the iron levels and things like that, and that's how it came about. But they did take more blood than usual on one occasion. I didn't know why, but they just had to check everything, but it was alright.

Have you ever looked back over any of the stuff in your notes, did, did it say anywhere in the written information this is what they were doing, as far as you know?

No [pause] no.

So this was a complete out of the blue shock?

Yeah, definitely.

And did you know anything about thalassaemia?

A little bit, because they do put a little booklet in about it, but you know they don't exactly tell you you're going to be tested for it. As far as I was aware, anyway [laughs].

No, I mean I must have seen about twenty different midwives. I didn't always see the same one. And to be quite honest, you know, I mean I - even though it's not about this - they didn't, they don't really explain too much. They just get you in and get you out again, then they tell you things when they've already done it without asking, sort of thing, so you know [laughs].

Has that happened on other things as well?

Oh yeah, definitely.

What kind of other things?

Just explaining about the pregnancy, you know. No-one sort of told me any classes or anything like that I could go to. They just sort of get you in and get you out again, really, which hasn't been very good [laughs].
 

Professional learning: At first they did not take carrier screening seriously. They'd never heard...

Professional learning: At first they did not take carrier screening seriously. They'd never heard...

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Were you aware when, when you had all the routine blood tests that this was one of the things being tested for?

Father' No. Well, there was a leaflet, but it just never - no one, I don't know anybody who's gone through it, do you know what I mean? And there's nobody I know who had this sort of problem. So I just, I mean in the pack there's so many - there was a leaflet but I just didn't take it seriously. I didn't understand. 'It couldn't happen to us' you know what I mean? Because we don't smoke, we don't drink, so, you know, we live a very healthy lifestyle. So you just can't think, 'It couldn't really happen.' So yeah, the implication of it all didn't really, didn't really hit us at all.

Was that true of screening for other things as well, I mean, like Down's syndrome?

Father' Down's we understand, because Down's we can see and we've seen it in other people, so we understand that. But this thing is just completely - and the thing is I think the most traumatic aspect is that we're born with it. It's not something that we, not because of our lifestyle, but it's because we're born with it. And we thought, you know, we might be a bit unlucky really to, for both of us to have it, really. And it's strange really, because within the Vietnamese community in [city] people were having children without a problem at all. And we felt a bit weird really that we had this problem.

Footnote' often people do not realise that being a carrier does not affect your general health and you may have no idea you are a carrier unless you are tested.
 

Professional learning: She was willing to go along with whatever tests the NHS offered, but she...

Professional learning: She was willing to go along with whatever tests the NHS offered, but she...

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They didn't really tell me much. They just told me they were going to check for glucose levels, blood group, AIDS test, so it wasn't really like 'Glucose level' this is what it means', you know. It was just first lines, and 'We're checking for stuff', and that was it. She said, 'Oh, two weeks time you get your result. If there's anything's wrong we'll call you.' So that's it. So to them there was something wrong, that's why they called me.

And looking back would you make the same decision again, or would you actually say, 'No, I don't want-'?

[Laughs] if I knew they were going to do blood group and all that. But anyway it is, it is the policy. You know, the bible, the bible says give Caesar what belongs to Caesar. So I'll give Tony Blair what belongs to Tony Blair. So if, if the NHS says they're going to check for blood group, AIDS test, who am I to say, 'No'? I will go and do all their stuff. I'll make them happy, respect their views, you know? I'm not going to say, 'Oh, NHS is wrong.' Uh-uh. I respect their views, you know? If God thought there was, he was just going to be healing us without doctors, he wouldn't have made doctors. So I really, really respect their views. I respect everything they tell me. That's why I go for their blood group, I go for their scanning, I go for all the stuff they want me to do. So I would have gone. Because that - it was mandatory, and I'd have gone. 

Footnote' no-one has to have screening if they do not want to - it is a choice. Blood group is different from being a carrier or having the disorder.
 

Newborn tests
The introduction of universal newborn screening as part of the heel prick test raises important questions for professionals around informed consent. Some parents having newborn screening already know they may be at risk - for example couples who know they are both carriers but decline antenatal diagnosis because they know they would not consider a termination. Some women who are carriers and whose partners have been unwilling or unable to be screened may also decide to wait until after the birth to find out if the baby is affected. Generally speaking, people in this group have already been counselled during pregnancy. They have actively consented to newborn tests and understand what the results may show, even though getting the results can still be a shock. (See 'Newborn screening after screening in pregnancy').

This is very different to people who may never have heard of the conditions and have no reason to believe they or their child will be affected. Most people whose newborn baby is screened will get a negative result and staff will not want to make them needlessly anxious. However, for the few people who are given a positive result, lack of understanding of what they are being tested for and its implications can add to their shock and anxiety. Some people thought it was compulsory to have the tests, while others just accepted it as a routine part of care.

The NHS Screening Committee has produced a booklet ‘Screening tests for you and your baby’ that should be given to all pregnant women by their midwife or GP and it gives information about screening for sickle cell and thalassaemia and how it is tested for in early pregnancy and in newborns..

Gaining the consent of their partners to be screened was a concern for many of the women we talked to. Some men (for example Interviews 01 and 13 above) were very willing to be screened, but several were reluctant, or were unavailable, perhaps because they were out of the country for long periods, or because the relationship had broken up. Some men (and some female partners) found it hard to believe they might be a carrier if they were always fit and healthy. (See 'Explaining genetics and risk' for information and video clips about the importance of explaining how recessive inheritance works). Professionals may also find it helpful to read 'Partner carrier screening'.

Professional learning: They had the heel prick test believing it was compulsory. The results took...

Professional learning: They had the heel prick test believing it was compulsory. The results took...

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Had you, either of you heard of the condition before?

Father' No. I've heard of sickle cell but not actually haemoglobin E. It was all new to us, a bit out of the blue. 

So tell me what happened from, from sort of the heel prick [Baby noises]. Did you know what they were testing for when they did the heel prick?

Father' No, not at all, it was something they said everybody was having it done. They said it was a fairly new thing but it had to be done. Obviously you've told us that we didn't have to have it done, but at the time we weren't led to believe that. We were told it was all part and parcel of it. They did it. The next thing we had a letter from a lady in [city], not even the midwife didn't know at the time we were told. We had the letter and then obviously we got in touch with the midwife, and then she'd heard at the same time. 

So nobody actually rang you and talked you through it? It was just a letter?

Father' Just a letter. Yeah, just a letter.

What was that like getting that letter?

Father' It was a bit of a shock, because obviously it said that perhaps if we were both carriers we couldn't have any more children, or if we did there's more of a risk. 

Footnote' no-one has to have screening if they do not want to - it is a choice. Being a carrier does not prevent you from having further children, but this couple would not have wanted to try for another baby if they had discovered he was also a carrier of sickle cell or beta thalassaemia. (If both parents are carriers of haemoglobin E, however, there is no risk to the child's health).
 

Professional learning: After a difficult birth, she did not pay much attention to the heel prick...

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Going back to sort of the heel prick, and what you understood about what they were testing, I mean, what information did they give you about the heel prick? [dog scratching] Can you remember much about what you had?

Mother' No. Because we didn't have really too good a time in hospital after.

Father' No, because you had a rough time, didn't you?

Mother' So it was all, it was all a bit of a . . .

Father' We just took it that that was just part of the normal having a newborn baby. That, you know, that was just a treatment they do as a standard.

Mother' So they --

Father' They take bloods, do the tests. And then while they were doing that we were more concentrating to get, get the wife --

Mother' But she had quite a few, she had a couple of heel pricks because she was jaundiced. So they needed to, I knew that it was, it's, they were looking for the - is it the clotting? She had two or three, didn't she? She had the normal one for the testing, and they kept coming back because she was jaundiced and she was [laugh]

And you, did you have a rough birth then?

Mother' Yes [laughs].

Right. So your mind is on other things completely than the detail of what medical tests are being carried out?

Mother' Yes. Well, yeah, I mean wanting to tend to her, and I ended up having an emergency caesarean. So the shock of having to have that, not being able to move for a couple of days after, the baby being jaundiced, picking up an infection, having high blood pressure, and then finding a heart murmur in her wasn't really a [laugh] very first good week.

Father' Within the first three days or something, wasn't it?

Mother' So we didn't, it was all a bit of a blur. The sort of, it was a shock when the letter come through the post. Because I think it was just one thing out of the week after she was born was we didn't give a second thought. We knew obviously that they'd come and they'd done the test. But with everything else that went on in hospital - I mean we probably, we probably did have leaflets. And I think if we'd had had a nice delivery and sort of, you know, a nice couple of days, then I don't know, it might have been different. But we, it's just one thing we just didn't give a thought to. It's just something that hospitals do, mainly for their records, not for anything that - unless there is a problem obviously that we need to know about.
 

Professional learning: She remembers the midwife taking some blood samples from the baby, but she...

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Professional learning: She remembers the midwife taking some blood samples from the baby, but she...

Age at interview: 35
Sex: Female
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And did you know that he was being tested for sickle cell?

Well, I don't think as such I did, but I do remember the midwife came, took some samples of blood tests, you know. Whether she mentioned it or not I can't remember, but I know she did do some tests. You know, and she said that we would get the result after some time. And I think we were leaving for Nigeria anyway, so we wouldn't have gotten the results. So I got, the results came here. That was when they now phoned us in Nigeria. So I wasn't sure what kind of test they were doing, but I just knew that, well, they have to do it, so.
 

Of course, people may well have been given much more information than they recall. As the parents in Interview 26 point out, just after the birth they had other priorities and it was difficult to absorb information on newborn screening. Similarly the parents in Interview 13 above knew they had had a leaflet about antenatal carrier screening, but in the excitement of a first pregnancy they found it hard to grasp its relevance for them.

The NHS Sickle Cell & Thalassaemia Screening Programme has also introduced the world’s first linked antenatal and newborn screening programme. They are striving to link results from antenatal tests taken by parents-to-be with their baby's test results which should help provide better and more efficient care. By following people through the screening process it provides the NHS with an automatic check that no-one will slip through the net and that procedures have been followed properly.

Last reviewed December 2018.
Last updated
September 2015.

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