A Parent’s Guide to Birth Related Brachial Plexus Palsy (Erb’s Palsy)
Having seen a huge number of families where one of their children has Birth Related Brachial Plexus Palsy (BRBPP), and being a parent myself, I have a significant insight into what you are experiencing:
It is a difficult period where it seems very few people seem to be giving you the full picture and there appears to be a great deal of uncertainty and not insignificant disagreement between what various involved professionals are telling you.
I hope we can help you through this but giving you a clear impression of what has happened, what can be done and what can be expected in the future. Not all of this is possible the first time we meet because it is often necessary to examine your child on a number of occasions and often to also to look at the results of a special test called neurophysiology which gives us a more in-depth assessment.
The condition of Erb’s plasy or BRBPP is rare and because of this not many general paediatricians or obstetricians have seen many cases. It is only those that specialise in this condition who see many and thus have a significant understanding of the whole area. It is for this reason that you may have been told (in good-faith) some part-truths and opposing opinions.
BRBPP is a hugely varied condition; some children recover very well, some quickly, some less so. Some children are very badly affected but this number is very small indeed. Many of the young people under my care with BRBPP are highly functioning adolescents and young adults – many excelling in sport and academia and business. BRBPP has certainly not been a handicap to them.
There are many options available to children now that in comparison with years ago and the outcomes have improved but the impression in many peoples mind is that the outcomes are still poor. I have for example now a great experience in nerve transfer reconstruction in BRBPP for shoulder elbow and hand. I have seen wonderful improvement in utilising Botulinum toxin for a problem called co-contraction which we are not recognising in more and more children.
At the Royal National Orthopaedic Hospital (RNOH) we are the largest unit treating BRBPP in the country and have collaborations with other national and international units. We have a great team of physical and occupational therapists, neurophysiologists, psychologists and surgeons. Our Stanmore Surgical team boasts specialist paediatric anaesthetists (with paediatric HDU back up) and a whole team of paediatric ward doctors, plaster technicians. Our unit’s secretarial team are the organisational hub to keep all of this team functional and organise your journey through the process with great efficiency and commitment.
You will have access to all of this expertise through the NHS PNI unit at the RNOH, or privately. I will be there as the conductor of this orchestra to ensure you get a clear message of our opinion and to answer your questions (don’t feel bad bringing a long list of questions to the consultation, many parents do and it means you don’t leave thinking – oh! I forget to ask that)
So the usual course of events is that we meet for the first time around 2-3 months (it depends a little on your child’s injury and who refers you and also tends to depend on where in the UK you are coming from. At this appointment I will talk to you about what has happened up to that point and examine your son/daughter then we can talk about the injury and I can answer most of the questions you have. Often at this point the prognosis is not completely clear but we can discuss the various options. Then we will arrange neurophysiology testing (at Great Ormond St Hospital) to occur at age 3 months and a time to meet again to review this information and tie this in with a repeat clinical examination. At this point we have enough information to reliably predict the natural history (what is likely to happen with out treatment) and thus to determine if surgery can offer an improvement upon what will happen naturally.
Surgery can be to the Nerves in the neck, or to the shoulder, the arm or the hand. Each has a specific time period where it can be of benefit. I will talk you through these options as each becomes applicable.
I hope this has contributed a little more to what you know about the condition you are soon to become an expert in. It is also important to recognise that making these decisions about your child’s health are very difficult. Harder even than making the same decision about your own health. I will be here to support you through this process as will the rest of the team. No decisions will ever be forced upon you or rushed and I am entirely supportive if you wish to pursue a second opinion.
Please read the other posts on this subject for further specific information.
What happens now?
After your first consultation I hope you have left having had most of your questions answered and if the answer was not available that day at least the reasons were discussed. When you leave the clinic there is nothing more to do: My secretary at the NHS PNI unit (firstname.lastname@example.org) or my private PA (Robyn- NerveInjuryLondon@gmail.com) will arrange your visit to Great Ormond Street hospital (GOSH – Great Ormond St, London WC1N 3JH) and the follow-up appointment where we will again examine your child and discuss the results of the tests.
We try to arrange these on the same day – particularly for those of you from further afield (NHS patients from the South West and Peninsular, the Midlands, Wales, Northern Ireland for example and International Private patients).
If you have any questions on any of this please contact the PNI NHS unit on 02089095447 or my Private PA: Robyn on 07545684524.
Primary nerve surgery means surgery to improve the outcome from the nerve injury in the neck (the supraclavicular brachial plexus). The operation is undertaken if we think that the outcome after surgery will be better than if we left things alone. This decision is a difficult one to be completely sure on. We have shown that our method of treating BRBPP relying on both clinical examination and neurophysiology is around 97 percent correct but it is impossible to know for certain what the best decision is. What I am certain of is that we should use as much information as possible to inform my opinion on what is best – by that I mean examining your child on at least three occasions before surgery (at least two in clinic and one on the morning of surgery) and to use the data from the study of the nerves and the muscles. Many around the word to not agree with this and rely just on clinical examination. There are many reasons why there is much disagreement between different surgeons across the country and across the world but I believe this approach gives me the best method of offering your child the best chance of gaining function.
When I have offered you my opinion that your child would benefit from surgery to the nerves in the neck it is the important to recognise firstly that nerve surgery is not like wiring up a lightbulb – nerves are not like electric wires. It is more like a hosepipe: imagine you have a long garden and you turn the tap on (this is the surgery) you don’t see water out the end of the hose immediately. Instead the water has to creep down the hosepipe – this is just like the nerves regrowing. It is a while before you see water flowing from the end of the pipe and on to the roses, even longer before the roses bloom- In nerve regeneration the nerves reach the muscles and then it takes a while for the muscles to then start working. So it is very rare (but happens in some kinds of nerve injury) that function returns quickly after surgery. It often take many months even a year until we see the benefit from surgery.
The other thing I need mention is that surgery has risks. It may sound obvious but in the process of explaining the pros and cons (consent process) it is important that we all understand what the pros and cons are. I don’t want to alarm you (and much of this conversation is best held face-to-face to allow you to make your own mind up) but there are a number of risks which occur occasionally –
- infection – around 1-2% rate (that is to say a chance of 1 in a hundred or so) this might mean that antibiotics are required or occasionally that we have to re-open the wound and wash away the infection.
- bleeding (again around 1%) a little occurs by the very nature of surgery but we are very careful to keep this to an absolute minimum, occasionally there is significant bleeding and very occasionally a blood transfusion may be needed.
- no improvement from surgery. This is hard to put a risk on – we really don’t know for sure and never really will but there is a chance that your child might after having surgery still only get as good as they would have done without surgery. It is important you understand this idea – we will never know how they would have done as we chose to undertake surgery and we accepted these other risks – but we will never know for sure if the prediction for the likely outcome without surgery was improved upon by deciding to operate.
- ‘theoretical risks’. Here are then the ‘theoretical risks’: think about travelling to the clinic in London, you arrived with no problems but you are aware that you could have been involved in car accident or tripped and twisted your ankle: this is a risk like the risk of bleeding or infection. You are also aware that a meteor could fall from the sky and hit you – you know it could, but the chances are so small you don’t considered it a worry. That is the same with these risks: they sound so very worrying but the chances are so small it is enough just to admit they could happen. I will mention these risks when we talk so we can discuss them together.
Making these decisions about your child’s health are very difficult – we all recognise that. Harder even than making the same decision about your own health. I will be here to support you through this process.
The shoulder is the most affected part of the arm in BRBPP. The C5 nerve root controls the shoulder and it is the most damaged in the vast majority of cases. Even though the nerve injury is a single event, its effects are seen throughout growth.
Why is this?
The seriousness of the injury to the nerve roots is a range – most severe at C5 least at T1. This imbalance in the nerve injury produces an imbalance within the muscles C5 is responsible for moving the arm out to the side and external rotation of the shoulder too (see pictures). These movements are overpowered by the muscles (from C6/7/8) that move the arm in to the side and internal rotation.
This imbalance means that with growth the shoulder joint deforms. The humeral head (the ‘ball’ of the shoulder) can flatten and become trianguar rather than spherical. Growth is unbalanced and the rotation of the growth of the long part of the arm bone (humerus) can be affected (torsional deformity). Also the growth of the socket of the shoulder joint (the glenoid) can be affected as well. This is called glenoid dysplasia- it is where instead of developing as a nice saucer shape to contain the ball of the shoulder it has a flat area where the shoulder can slip out of joint. These are all parts of a condition called shoulder dysplasia where the ligaments and muscles tighten around the shoulder and the joint starts to part dislocate (subluxation) or fully dislocate. This is a progressive condition worsening over time. We think it can be improved in some cases with physiotherapy to stretch the tissues but sometimes surgery is needed.
The inability to turn the shoulder out (external rotation) leads to the arm being held in a ‘trumpeting’ position with the elbow held out from the side. When trying to bring the hand to the mouth. When the shoulder dysplasia progresses even the ability to move the shoulder to the side (abduction) can be lost. Then the arm is held turn in, sometimes with the elbow almost facing forwards and very little movement or function possible with the affected arm.
There are a number of problems that can occur due to BRBPP. The most common is a problem with the range of movement in the forearm. This can mean that the arm cannot rotate to bring the hand to face upwards (supination) or to bring it to face the floor (pronation). Functionally it is more important to be able to turn the hand to the floor as most of the things we do in life are in this position or midway between the two. Thus often we have to turn the arm (a pronation osteotomy) surgically. This involves a scar on the palm side of the forearm and a plate and screws (these are often left inside for life). After surgery there is need for a period of six weeks of plaster casts or splints.
The next most common is an elbow fixed flexion deformity – this means the elbow is unable to straighten. When this is mild it is not a problem but if it worsens then it can cause a problem and is certainly a cosmetic problem as well. We have moved away from surgery for this problem and now use a cast brace (a plaster cast) which stretches the elbow straight over weeks. Think of it like dental braces for the arm.
Wrist surgery – some children have a weakness in lifting the wrist or fingers – this is called a wrist drop or finger drop. In many cases this improves over time but in some in continues and surgery can be used to help. This is called a tendon transfer. This means a number of scars on the arm and six weeks of plasters and splints after surgery. After this period the improvement is clear immediately with the hand and wrist working well immediately.
The hand is only very infrequently affected in BRBPP. When it is there is often a severe sensory and motor coordination deficit as well as the motor loss. Sometimes however there are focal losses of function which can be improved with nerve or tendon transfers.