Three month old diagnosed with BWS

[hroussel]

Hello! I'm hoping to connect with others that have children with BWS. My 3 month old was diagnosed six weeks ago. He has macroglossia, hernia, and ear pits. We had a feeding, swallow and sleep study done. His sleep study results were not good. His tongue is obstructing his airway so now he needs a tongue reduction. I'm terrified. Wondering how other surgeries have gone. He will probably have his soon. We meet with the doctors tomorrow to discuss. He also has some mild curving of his ureters, also know as pelvocalietasis. Wondering if others have the same issue. Looking forward to connecting,.

[Songbird63 Administrator BWS Support]

Where do you live? In the UK all BWS children with macroglossia are treated at Great Ormond Street Hospital. There is a lot of info about it on our website, bws-support.org.uk. My son had surgery there at 20 months. He is now 15, very handsome and has perfect speech. It's important that your son is assessed at a craniofacial centre for children that is familiar with the condition.

Have not come across the problem with ureters before. But we do know that some kidney problems such as cysts and medullary sponge kidney can be associated with BWS.

Most problems with BWS occur in early childhood and with good management the vast majority of children grow up without any problems. Try not to loose the joy of these early wonderful months with your son because of fear and worry. Take care and come back and ask anything you would like to.

[hroussel in reply to Songbird63]

I'm actually in Milwaukee, WI, USA. I'm really interested in understanding why doctors opted for a TR instead of a tracheostomy. Our ENT and pulmonologist seem to be leaning that way and it terrifies me. I think the TR was standard. They felt it would be best. They don't feel the TR would completely relieve the obstruction. So confused and so many questions. We have another sleep study to see how he does on his side and on his tummy. In the last study he had 89 episodes.

[Songbird63 Administrator BWS Support in reply to hroussel]

A tracheostomy is a big op and a big decision, it takes away the child's ability to vocalise, and can stay in place for many years. It also limits activities they can do etc so is only generally done if there is no other option and the child is at significant risk if it isn't done. However, if it is needed it usually has a dramatic impact in improving the child's respiration etc.

Children generally recover very well from TR surgery, with no damage to speech or feeding.

It sounds like you are seeing the right specialists so I would trust in their advice. It may be worth asking them whether they have treated other macroglossia cases though. And why they feel the TR may not relieve the instruction. Are there other issues etc effecting her breathing. Don't feel bad asking lots of questions, it's what we have to do as parents, and good paediatricians and specialist should be more than happy to answer them.

[hroussel in reply to Songbird63]

The ENT and pulmonologist treating him felt the tracheostomy would be more effective than a TR. He had 89 events during his sleep study so it was pretty severe. They sound as though they aren't convinced the TR would completely relieve the obstruction. I'm just sick about this. We have another sleep study on 8/6 to monitor him on his tummy and side. If there is significant improvement we may be able to avoid surgery.

[Songbird63 Administrator BWS Support in reply to hroussel]

With that many episodes in the sleep study is sounds as if her respiration is severely affected . But there must be some other obstruction/problem other than just macroglossia if they don't thing the TR would solve things.

I can understand that it is a really stressful and difficult time for you, and that it must be hard to cope knowing that your daughter has so many episodes when she is asleep. As I said before if the specialists feel that a tracheostomy is the way to go then I would trust them. I have seen many children have a trachy and although it's a big step and learning to manage it can be hard, it does bring peace of mind and some normality to life when you adjust to it. and the children usually cope very well and their growth improves as their respiration becomes more efficient.

I do hope you can manage to find some time to enjoy your daughter amongst all the difficulties you are facing. Please let us know how things go and feel free to come back at any time for support, or even just to offload how you are feeling. Thinking of you.

[hroussel in reply to Songbird63]

Thanks so much for the support, it is much appreciated. I'm curious, of the trachy cases you we heard about, was it permanent or did it come out after they got a little bigger? They said his adnoids and tonsils were fine and it's just the to gue causing the obstruction. I'm really hoping and praying that as he grows it wiont be necessary. He's really such a good natured baby. Through every procedure he has amazed me!

[Songbird63 Administrator BWS Support in reply to hroussel]

The trachys stay in for as long as the problem causing the obstruction is there...so it depends on why it was put in in the first place. In some children it is taken out when they grow and the problem diminishes or surgery corrects the problem and it is then taken out. It is only a permanent solution in a small minority of children with major issues.

I would ask the team looking after you what experience they have of TRS because if it's just the macroglossia causing the breathing difficulties then I would have thought surgery would solve this. It's important your son is reviewed by a multidisciplinary peadiatric craniofacial team who are experienced in this condition. This team should include a speech and language specialist, and an orthodontist as well as a craniofacial surgeon.

Take a look at our website for information from Great Ormond Street Hospital about this, as you may find it helpful.

[hroussel in reply to Songbird63]

Thanks again!!!

[BobBaker Administrator BWS Support]

If you live in the UK I can put you in touch with other parents fo BWS children who live near you, with their permission of course. Do let me know if this would be helpful.

Best wishes

Bob Baker

[hroussel in reply to BobBaker]

Thank you. I'm in the US. I appreciate the offer though!