Screening for sickle cell and beta thalassaemia

Sources of support

Many people turned to their partner and their family as their first source of support. As one woman said, 'My Mum, she's been my rock. She calls me two, three times a day… If I didn't have a religion, or I didn't have a supportive family, then maybe I would have just ended the pregnancy.' (See also 'Values and religious beliefs' for more discussion of the strength many people get from religion).

But not everyone found it easy to draw on family support. Sometimes this might be simply at a practical level. Some people had arrived fairly recently in the UK, and had few family members here. Several talked about telephoning or e-mailing family back home, but sometimes found it hard to explain their concerns.

Professional learning: In the UK their only close family is his mother. His wife rang family back...

Professional learning: In the UK their only close family is his mother. His wife rang family back...

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Father' For our purpose, it's just the baby and, you know, I mean you just sort of don't want the baby to live a life sort of like that. Because it's, you know, our perspective life is about fun and, you know, and doing things really. We just don't want it to, to live like that. Because in that situation I think - another experience we have is that because we, the family's very small. It's just me and my wife and my mother, so we don't have that extended family network to support the baby. Can you imagine when we died, you know, who's going to, where's his or her networks going to be? And if you're carrying a sickness, you know, who's there for them? So it's that sort of thing that we can't, we can't sort of take the chance with.

Did either of you talk to family members while you were waiting for the results to...?

Father' All the time, yeah, she'd be phoning Vietnam all the time, to her sisters and her mother. And I'd be talking to my friends basically. Again talking to people in Vietnam they just think, they just never, they can't visualise it, you know? It's just totally unknown to them. So I think in South-East Asia this sort of condition is, it's not widely known, do you know what I mean? And so over there it's just, it's a bit strange to have this sort of problem. But I think the problem we had talking to people as well is that too much knowledge is quite - you know what I mean? And I think there's probably a couple like us in Vietnam somewhere, who probably have the condition but have just gone ahead with it and had a healthy baby and just never realised there was a potential risk, I think. So all these tests bring out all these new scenarios, you know, and it put pressure on. Whereas sometimes not knowing it, just going ahead with it is, might be good, you know what I mean?
 

A Portuguese couple described their experiences of arriving as new immigrants and finding themselves homeless and isolated when she was already pregnant. Discovering their baby was a sickle cell carrier and trying to understand the consequences was for them one more hurdle to add to their existing difficulties with language, housing, employment and understanding the system. 

She was 7 months pregnant when they arrived in the UK. A stranger helped them find accommodation....

She was 7 months pregnant when they arrived in the UK. A stranger helped them find accommodation....

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But did you do a test when you got pregnant?

Mother' No. I was 7 months' pregnant when I came to England. No, we only did the test when the baby was born. We stayed in the hospital almost a month, and he (the baby) had blood tests every day, we did not know why. They were always seeing what the problem was. They were constantly doing tests, tests, tests and then, ah, after two months they said they were undecided whether it was a blood disorder, or if it was a kind of anaemia, but they explained more or less what it was.

Did you do the other tests, the tests that one does normally to see if the child has another disease?

Mother' Yes, I have done the tests in Portugal, and I went regularly for doctor's appointments and they did not discover anything. I mean, my food intake was very weak in vitamins and they discovered that I had a bit of anaemia, but it was enough to eat etc. to do the necessary things, and nothing would happen, but I had anaemia during pregnancy. Then, when I came to England 7 months' pregnant, in the beginning , when I wasn't used to the food and the country, my nourishment got worse and worse, and I think my anaemia got worse too. It was very bad when I came here when I was pregnant and I wasn't used to things here, it was very difficult.

It was difficult to get used to the house, to the work...

Mother' I did not like the food, I had a lot of stress.

Life here is not easy.

Mother' Especially in the beginning.

But you were not working at the time, were you?

Mother' No, I was just pregnant.

There are a lot of people that neglect themselves when in situations of stress, don't they?

Mother' Sometimes it was just simply not having enough conditions, wanting to eat this and that and not having any money. I had to eat a lot of vegetables and fruit, this and that, and as I was alone I neglected myself more. I should have been able to cook or to have better conditions, but the fact is that when I came to England I had nowhere to stay. I could not do everything that I wanted.

So when you came to England you did not have a home? 

Mother' No. A friend of mine was coming to pick me up at the airport and he did not come. I was left with nothing. We were left with nothing. The first night we spent at a hotel and the rest, in the next three months we stayed at the house of a stranger.

Father' That supported us

Mother' See, it was her brother coming down the road, and he said " Look some Portuguese people traveling" and we - he speaks Portuguese, we then told him what had happened and he let us stay in his house, and then we went to his sister's house, and then when we had better conditions we came here.

Father' We were very lucky. Because we could not even pay the hotel. We even thought that we had paid the hotel another 4 days. We left the hotel and went to buy something to eat and when we arrived we could not see our suitcases. 

Mother'

One woman had to rely on her parents for support in caring for her son with beta thalassaemia major, because it took several years to sort out her husband's entry visa to the UK from Bangladesh. She drew support from her local specialist nurse and other parents she met at a support group, but it was a lonely time. The couple feel the immigration authorities need to be more sensitive to the needs of families with a sick child.

Professional learning: The mother had to care for her son on her own until her husband's visa...

Professional learning: The mother had to care for her son on her own until her husband's visa...

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English translation, video and audio clips in Sylheti.

So how much trouble was it for you for 8 yrs?

Father' For my wife?

yes

Father' My wife of course had a lot of trouble and I was always worrying, and concerned while I was in Bangladesh about my wife and child, about how they were coping, with this illness, care of my child, or how they were eating and living. So I was always in a bother, concerning this, and I felt weak mentally, and when I couldn't get a visa, I was more broken mentally, however, Allah helped me' Then suddenly, a letter came. In my country, I used to teach in a Madrasa (Islamic school), taught for 9 yrs.

Really, mashallah. 

Father' Yes. I tried to keep myself occupied, I taught about 300 - 350 children in a school. I was teaching in an Arabic schools, so praise be to Allah, I was OK. I thought that whatever problems Allah puts you in, through patience I should accept it and have patience. Everyone should do this. But I would like to propose something here through you, 

Yes

Father' that for the guardians of these patients, if the wife's in the country and the husband is in Bangladesh, or husband in this country, wife is in Bangladesh, then this will at the end come to a resolution or permission will be given where each other's identity or legitimate married life is in place' So in this situation if there are immigration or visa problems then these should also be addressed and someone should be given the responsibility. The 9 years that has taken is very bad mentally and weakening.

Mother' This is very stressful for a person.

Father' This can affect other people's lives. You just mentioned what does the society say? In these situations the society says lots of things - for example, someone has seen this lady always on her own with a small boy in the hospital and clinic. And there is no male guardian. Then this lack can destroy someone's mentality, it's enough to do this. I am requesting to do something so this doesn't happen to anyone' Do you understand? And the truths about these situations should be upheld and people who work in social services should have a lot of input. This will help both the patient and the their carer. 

Mother' (Coughs), Sorry

Sure, definitely

Father' Today you have come here and taking our interview, this will help another patient so this will be fruitful and if another person is mentally weak but through this interview if they feel stronger then this is also helpful to the patient. And the patient's guardian, the parents or whoever, will also feel mentally strong that there was someone else in the same situation who accepted this medication and have recovered. So these people will get the mental strength to go through and reap the benefits. I hope your work brings success and thank you for it.

Inshallah. Do you want to say anything else, sister?

Mother' No, what else to say.
 

Although most people felt strongly that family support had been important to them, a few wanted to keep things to themselves. For example, one woman said, 'You don't know to what extent you'd have wanted people rushing round to see how you were anyhow, because it is a very personal thing. Another woman whose daughter has sickle cell anaemia commented that sometimes family members said the wrong thing. She and her partner found it difficult to support each other as well.

Professional learning: It was hard to support each other. She did not want her family's pity. [Ils l'ont trouvé difficile de se donner le soutien mutuel. Elle n'a pas voulu la pitié de sa famille].

Professional learning: It was hard to support each other. She did not want her family's pity. [Ils l'ont trouvé difficile de se donner le soutien mutuel. Elle n'a pas voulu la pitié de sa famille].

Age at interview: 40
Sex: Female
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English translation:

Yes. Life changes straight away, even between the two partners, because you feel guilty. You have the impression that it's because of you that this child is ill. And that's very hard. And that's hard because to start off with you don't talk about it. That was certainly our case. We each stayed in our corner and thought about it, 'What have I done? Should I have kept him? I didn't know. Is it my fault?' And he was exactly the same, must have been saying the same things to himself. And straight away that brings tensions between the couple. But you don't express that because you're afraid of hurting the other person. And we, in our case at the start we really couldn't find a means of communication. So it was each one for themselves. We suffered in silence. And at the same time we loved, there was this child who was there that we loved a lot. But personally we were suffering, because, especially when he started having his first crisis, which he had at 9 months, which is early. So all of a sudden then we were really confronted by the illness, and we were more focused on the child. You forget yourself because you want to give everything you can to the child. So we forgot about ourselves. And then it was a lack of communication and that created lots of tensions. That's on the family side, between the mother and father. 

At the level of other close family, it was more pity, and we didn't need that. 'Oh, it's hard. Oh, the poor things. The poor little child'. You hear that once, twice and afterwards, well, okay, you try not to talk about it to people around you. So for a second time you close in on yourself. 

French original:

Oui. La vie, elle change déjà entre les deux partenaires, parce qu’on se sent coupable.  On a l’impression que c’est à cause de nous que cet enfant est malade, et ça c’est dur.  Et ça c’est dur parce qu’au départ on n’en parle pas.  Nous, ça a été notre cas, chacun reste dans son coin et on cogite.  ‘Qu’est-ce-que jai fait ? Est-ce-que j’aurais du le garder ? Et on ne savait pas, et c’est ma faute.’ L’autre aussi pareil, devait se dire pareil. Et du coup ça amène des tensions dans un couple, mais on ne s’exprime pas parce qu’on a peur de blesser l’autre. Et nous, dans notre cas au départ on n’a vraiment pas trouvé un ton de communication. Donc c’était chacun pour soi, on souffrait dans le silence. Et en même temps, on aimait, il y avait cet enfant qui était là qu’on aimait beaucoup mais personnellement on souffrait, parce que surtout quand il a commencé à avoir sa première crise, qu’il a eu a neuf mois quand-même, ce qui fait tôt, alors du coup là on était vraiment confronté à la maladie, et on était plus focalisé sur l’enfant. On s’oublie, parce qu’on veut donner le maximum à l’enfant. Nous-même on s’oublie, et après c’était un manque de communication, et ça fait beaucoup de tensions -  ça c’est du côté familial, entre le père et la maman.  

Au niveau de la famille proche, c’est plus de la pitié et on n’a pas besoin de ça.  ‘Ah c’est dur, ah, les pauvres, le pauvre petit.’ On entend ça une fois, deux fois, et après bon ben on essaie de ne pas en parler autour de soi, donc encore une deuxième fois on encaisse pour soi. 

 

For her, professional support from her local specialist counsellors and the Sickle Cell Society had been hugely important and had helped her accept that it is not just the child who needs support but also the parent. She found it easier to talk one-to-one than in a big support group.

Professional learning: The support from local counsellors and support groups has been outstanding...

Professional learning: The support from local counsellors and support groups has been outstanding...

Age at interview: 40
Sex: Female
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English translation: 

And coming back to England, what do you think of the support which exists or doesn't exist at the hospital?

No, I think here they're, how shall I say it? not a hundred per cent but they're at the very top. I can't find the word. Because I was so looked after myself. At the start it was just you, the mother, and you, you do everything to protect the child. All your attention is for the child alone. But since I came here I've learnt that we too, we mothers need attention. And there is some really extraordinary work going on here. For instance, by the Sickle Cell Society. And it's really splendid. It brings tears to my eyes, because the people are so good.

French original:

Et retournons ici en Angleterre, comment est-ce-que tu as vu le support qui existe, ou qui n’existe pas dans ce cas, à l’hôpital ?

Non, je crois qu’ici, ils sont, comment dire, pas 100% mais ils sont au top.  Je ne trouve pas le mot, parce que j’ai été tellement pouponnée moi-même.  Au départ c’étais juste toi la maman qui fait tout pour protéger l’enfant, pour - toute ton attention n’est que sur ton enfant. Mais depuis que je suis venue ici, j’ai appris que nous aussi les mamans on avait besoin d’attention. Et ça il y a un travail extraordinaire qui se fait ici par rapport à la Sickle Cell Society, et vraiment c’est grandiose, ça me donne même les larmes aux yeux parce que les gens sont très bien.


 

Many people echoed this praise for specialist counsellors, and felt the counsellors went out of their way to help. It was important to people not only that counsellors were well-informed and skilled in explaining complex information at the time of screening and diagnosis, but also that they could be phoned to answer queries and provide reassurance in the longer term. Being able to get quick advice was especially important for parents of children affected by sickle cell disorders or beta thalassaemia major when they are learning how to care for their child.

Professional learning: The first person she turns to with any queries is her specialist sickle...

Professional learning: The first person she turns to with any queries is her specialist sickle...

Age at interview: 36
Sex: Female
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I think I've always had good information and support at every, well, at every stage at least as far back as I've been dealing with this situation. Well, I think I've had a wealth of information. Well, I know I know where to go, I know who to call, I know where to get my support from, so in all that's been very satisfactory to me.

And with your daughter, when she, when you discovered she was, she had sickle cell anaemia, were you referred to a specialist counsellor or who came to advise you?

Yes, yes. There was, we were invited to the hospital to see the specialist counsellor.

And do you have regular contact with the counselling services or'

Yes, yes we do. Even for the, even for the most, for the most un-useful issues [laughs]. I mean, I jump on the phone, I phone her, and if she's not there, she's always calling me back. If she can't deal with it, referring me to the right authorities. 

Did you talk to the specialist counsellor this time around about the screening issue?

Yes. She was the first person I called. And I told her, 'This is my situation.' And she arranged the tests then for my partner straight away. It only took about a week. And she arranged for, she phoned the doctor in the hospital, my daughter's consultant and arranged everything through him. So we were first invited for the consultation to talk about it, and given information about it, then after that we got the appointment for the CVS. 
 

Some people were also very pleased that the counsellor could speak to them in their own language, including a Mirpuri-speaking mother whose counsellor came with her to every CVS appointment. For others (including a Thai speaker and a Portuguese speaker) communication had to be in English and it sometimes took them a while to understand. 

A number of people found it very helpful to attend support groups run by counsellors at their local sickle cell and thalassaemia centre. Just being able to talk to other mothers was comforting, and some people had also developed their own informal networks with other mothers. People attending support groups also found it helpful to have invited speakers, information videos and talks from the counsellors. 

Professional learning' The support from her local sickle cell centre has kept her going. It helps...

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Professional learning' The support from her local sickle cell centre has kept her going. It helps...

Age at interview: 33
Sex: Female
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You mentioned the, the support from the Sickle Cell Centre - what other sources of help and support have you had?

Every month we normally go there. Once in a month, every Wednesday in a month, we go there and then the ladies they talk to us, you know, what to expect. Sometimes we watch videos. And then they talk to us what to expect or how to realise it when your child is having a crisis, that kind of thing.

Do you talk much to the other mothers at the group? Is that helpful?

Yes, yes. I think it's helpful, you know, your going there and then knowing that you're not the only person having that problem, you know. And then also there are people who come there and then they talk to us about how they've been able to deal with their problem, with their grown-up children, how they were able to deal with the problem, and that kind of thing. So I think it's, they've been very supportive, yeah.

I'm very happy about the care and the support, you know, they've given me. I think I'm very happy about it. That is what is keeping me alive, you know, now. Always reassuring me that there is hope, you know. There are medications, there are support groups and that kind of thing. So I just want to thank all the health professionals involved in my care and my baby's care. We are very grateful. They should continue with their good work.
 

The bond established with counsellors can be very strong, and one woman said it was difficult to adapt to a new counsellor when the one who had supported the family at the time of her brother's death had left. They were not very happy as a family with the advice given.

Professional learning: She felt the specialist counsellor had not advised them very well. The...

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Professional learning: She felt the specialist counsellor had not advised them very well. The...

Age at interview: 32
Sex: Female
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Would you have felt, if they'd phrased it differently and said, you know, 'There is a choice and you might want to think about termination' would you have felt happier with that?

Sister-in-law'  Yeah.

You wouldn't have minded them mentioning that as a choice?

Sister-in-law'  Yeah.

Even if it wasn't something that -

Sister-in-law'  Yeah.

-that your family would want?

Sister-in-law'  It's up to the individual really. If they think they've been given a choice, that is different. But if they think you're being told what to do, that's totally different.

Have you ever, the family, raised it with the professionals involved and, and tried to point these things out to the people who counselled you?

Sister-in-law'  I had a word with the previous counsellor. She was, she was there with us all through the death and everything, and then she kind of left soon after. But it's that bond you had with her. And then she suddenly left. But they don't have, they don't have much of a choice because they're just telling you what the doctor's telling them. So that's really it. [baby rattle] It's a 9 to 5 thing really, isn't it? They're there, they're doing their job. They're kind of bringing it across to you of what you need to do, and how you need to do it, and then they're going to go home.
 

However such criticism was very rare. The great majority of people felt well supported and thought it was good to know there was someone there for them, even if they did not feel they needed help at that moment. 

Professional learning' In her current pregnancy, it was nice that the sickle cell counsellor...

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Professional learning' In her current pregnancy, it was nice that the sickle cell counsellor...

Age at interview: 35
Sex: Female
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I just felt, 'Oh, it's actually nice of them to have remembered that okay, yes, this lady's pregnant.' Because I think it was passed on from my clinic or something that, OK, I was pregnant, and obviously they knew I have a child who's SC [has haemoglobin SC disorder] and I'm AS [a sickle cell carrier]. So I just thought, 'Well, it's good to know that, you know, somebody cares out there." I think that was, I think that was nice, just to have that. Obviously I didn't think about it when I was pregnant that, "Oh, I'm going to need some sort of help." Because I knew that, 'OK, yes, I know what I have to go through', you know. But it was just nice to have that letter sent to me, so you know that somebody actually is concerned, and there to help you if you need it, so it was nice. It was nice.
 

One at-risk couple were pleased that the counsellor respected their wish not to have any diagnostic testing. She called them after they had made this decision, and the mother said, “She was very nice about it, and she was like, you know, people's faith had actually pulled them through. She respects my view and she wishes me good luck.” 

Last reviewed December 2018.

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