Our Community and Covid-19
We know that many of you will be very concerned about the pandemic and the implications for your children,
young people and adults with Batten disease. If new guidance is published this will be updated.
We will also post any useful links we find.
Most of our community are at no more risk than anybody else of serious complications from Coronavirus. But some of you will have received official guidance from the NHS that you should ‘shield’ your child. However, due to the way the NHS has identified patients who are ‘highly vulnerable’ the system is not perfect (everything is being done at high speed). Some of our community will not have been identified despite their complex needs. For example, you may have a child who gets recurrent chest infections, is peg fed and has a deteriorating swallow and finds it difficult to clear secretions. This combination of complexity may mean your consultant/GP advises that you shield them for 12 weeks.
The best thing to do is to contact your GP or consultant and ask them directly.
We are not a medical authority but have gleaned some potentially useful tips from other rare disease organisations and parents.
• If you have carers coming to the house, think about increasing your hygiene procedures, washing hands and using hand gel etc more regularly. Think about writing up new tighter guidelines for the household and sticking them up somewhere.
• It may be useful to have a clear idea of what your child or young person’s ‘baseline’ obs are. For example, some young people’s ‘normal’ temperature is lower than others. For some children and young people with rare diseases, their ‘normal’ temperature may be lower than their peers (eg: 35 rather than 36.5). Or their ‘normal’ may be higher than normal. Likewise their oxygen saturation levels may rest at a lower level. It may be useful to do your child or young person’s temperature more regularly.
• Look for local support pages, many areas are doing Facebook supports groups that identify people who are happy to run errands, do dog walks etc.
• Many hospital are cancelling non-urgent procedures and outpatient appointments so it’s sensible to check and discuss any appointments with your medical teams to see if they can be done remotely or postponed.
• For children on Brineura… GOSH understand the importance of the infusions for the CLN2 patients currently on treatment and do not anticipate any changes to this service beyond limiting visitors etc. that are outlined in the guidance here https://www.gosh.nhs.uk/…/coronavirus-covid-19-information-…. This guidance will be updated regularly as things progress. Do contact Laura Lee, our Clinical Nurse specialist for advice and support.
• Think about which medications your family member with Batten disease relies on. Ensure now that you have enough stock of crucial epilepsy and other medications. Check medications are in date. Check that you have enough supplies for gastrostomy feeding for those of you who have children who are peg fed.
Do use us for support and just to talk stuff through (even though we may not have definitive answers). This is the time to pull together as a community.
Click on question below to expand
The BDFA had a call with Great Ormond Street specifically about our families. We spoke with Professor Paul Gissen and Clinical Nurse Specialists Laura Lee and Becky Bower.
• The risk for children with Batten disease
Children with Batten disease without significant respiratory problems (needing oxygen or CPAP overnight, for example) are not thought to be at more risk than other children and young people. Whilst we don’t have Batten-specific statistics, a very small percentage of children were thought to be affected in Italy and even fewer needed medical support. There is no evidence currently that babies are more severely affected. One of the questions was from a parent who has two children with CLN3 with diminishing eyesight and no other symptoms. These children are not thought to have a higher risk than other children. We would advise all families to discuss your own child or young person with your local team who know them well.
As a group, NO. Patients who have severe respiratory or breathing difficulties needing overnight support should self-isolate if this is implemented, at the moment social distancing is recommended. Many young children have recurrent viral infections so this does not necessarily put your child in this group. Individual cases can be discussed with your local team. If your child has seizures that are exacerbated by a temperature or illness again there is no need to do anything differently to what you would do for a ‘normal’ virus. Children who do not have an effective cough or struggle to manage secretions would be at higher risk to be more seriously affected with any respiratory illness. Discuss your child or young person with your local team. This is such a personal decision and we know that many of you have decided to err on the side of caution and shield anyway.
These are hugely challenging times, if you are a key worker and your children are eligible to go to nursery, school or college, and this is something you should consider. This is entirely your choice. We know that many of our families are already under extraordinary pressure anyway, before Covid-19.
No really, no. The whole family would need to keep a safe distance from people, wash hands regularly etc. You are safer staying at home. One person should do key tasks.
The family member with Covid-19 should isolate separately in their own in a separate room if possible. Testing may be available in the coming days or weeks . The whole family needs to isolate for 14 days. Remember, the majority of people will recover well.
You will have to check your agency guidelines. Carers are within the groups of key workers. Again, it is an individual choice. We know that many children and young people need intensive levels of care and families would struggle without support. This is likely to change over the next few weeks.
If you decide that, you do not want carers to come to the house they would still be paid according to their contract. You can implement additional hygiene measures.
We are carrying on as we have before and Brineura is a critical therapy and have no plans to cancel any infusions. In Italy, children have carried on receiving infusions and new patients have started on the therapy.
We hope that newly diagnosed children will also be able to have surgery and commence treatment. Currently, only 1 adult is able to attend the infusion with your child.
Let us know if your child or anybody in your household is showing symptoms. The GOSH team will do their best to move the infusion if necessary.
The government are rapidly expanding the number of tests they are able
to do and are hopeful testing will be available in the community soon. We know there are some of our families desperate to gain access to testing as they have affected family members and it looks hopeful that this will be available next week.
End of life care will still be able to take place at home as far as we know. PPE (personal protective equipment) is available for those working in end of life (this is regardless of whether a child has respiratory symptoms). The NHS are rapidly expanding their intensive care capacity. The appropriateness of intensive care provision is individual for every child, young person and family. We encourage you to have these discussions with your healthcare professionals.
For children and young people already on a palliative care plan and receiving end of life care who contract Covid-19, there may be a difficult decision to make for some parents. But we are not there yet.
Do contact your local paediatric team who know your child well for advice.
The BDFA team
In line with other organisations our support workers Wendy and Cathy will be postponing all home visits and meetings.
They are available on email at firstname.lastname@example.org over the phone for advice.
Wendy is on 07876 712 553 and Cathy is on 07787 755 278.
Our CEO Amanda will also be working at home as she has a young adult who is ‘shielding’. She too is available on email at email@example.com or on 07876 682 589
** Some brighter news **
8th April 2020
The board of the BDFA is pleased to announce an urgent family grant scheme to support families caring for a family member
with Batten disease with the challenges faced due to Covid-19.
We have a designated pot of £10,000 to give out to families in the form of two family grants. Grants of up to £500 are
available for families who face financial hardship that has been exacerbated by the current situation. Grants of up to
£200 are available for all families as wellbeing and resilience grants to help families get through the next weeks and months.
We know that you are all facing a really difficult time emotionally and practically and we hope this grant scheme will
help in a small way to lift spirits and overcome practical challenges for our Batten community.
We are ensuring grants are super easy to apply for and there will be a very quick turnaround.
We will be sending out an email shortly to families we already support with the form attached,
or if you would like us to email you the form directly, please email firstname.lastname@example.org with
your details and the details of your family member with Batten Disease, variant type, age, and
To get the completed for back to us, please edit in word, re-send back to us via email, or print and take a
photo and email that to us. Support workers Wendy and Cathy will be processing applications.
If you would like to donate to help with this much needed cause for our families we would love to
hear from you, please get in contact with us at email@example.com.
Stay safe and well,
The BDFA team