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uk different to usa? |
Hi all...
maybe a stupid question, but hey here goes, Ive obviously been trawling the internet for any signs of hope for our newly diagnosed son. I was checking out youtube and noticed theres alot of American SMA type 1 videos on there but the children in the videos are around 4 years of age. My question..Do the USA and UK have different grading? Weve been told our son probably wont reach his 1st birthday and he's type 1..but some americans with type 1 are 4 years old...whats the difference?
Dean |
| Posted: Thu Oct 23, 2008 6:17 pm |
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Re: uk different to usa? |
hi dean, I did the same when Toby our second son was siagnosed with the condition during my pregnancy and yes in america there are many children who live longer with type 1.
one girl I know of is Lizzy and she is now 5, i think and it was her grandma who gave us all the information for Non-Invasive ventilation and the amino acid diet. Non invasive ventilation is basically use of a BI-pap machine, this helps expand the lungs to fullest capacity and can help reduce co2 levels making them more comfortable, co2 can cause disturbed sleep and headaches and therefore a grouchy baby, bi pap reduces this. eventually a cough assist machine could be brought in if and when your child is old enough and a tough regime of physio.
the amino acid diet comes in the form of neocate and is made up the same way as infant formula, this is not scientificly proven to to help but hey if it can't hurt and it can help what is the harm. it apparantly reduces airway secretions and can improve muscle tone, i have to say we put toby on it his muscle tone improved a bit but his airway secretions didn't change.
babies with SMA should also weigh at least 3 centiles in weight below a normal baby would and they certainly don't need the calorific content in their milk that a normal baby would, this is because they don't burn their calories so neocate LCP would be more advisable with a vitamin supplement but speak to your dietician. extra fat stores can be create extra unwanted and uneeded weight making it harder for the chest wall to work.
I don't know where in the country you live but we were reffered to the royal brompton and harefield hospital in london who are respiritory specialists and they gave us all the information and advice we needed.
Alot of other children in America have a trachaeotomy and a vent, this is not something I was happy with for Toby or Jake but then quality of life is down to a persons happiness and that is the most difficult decision us SMA parents have to make.
the age is also partly determined by which group of muscles are affected more severly, anyway whatever you decide to do time is of the essence with type 1 babies.
could you tell me what treatments you have in place at the moment and how old your little boy is.
if I can help in any other way please just shout and I will be happy to, if I can.
take care
kathryn |
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Re: Re: uk different to usa? |
Hi Kathryn
thank you for your reply, our son is now 3 months old. He is being fed via a nasal gastric tube through a electrical flowcare and SMA high energy. We live in Birmingham and are waiting for an appointment at the Birmingham childrens hospital. Our son is given physio twice every day and has a massage every other day. Those are the only treatments we have inplace at the moment.
Do we need others?
Thank you
Dean |
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Re: Re: Re: uk different to usa? |
Dean,
I just wanted to say hi to you, and i hope your little boy is doing well at my time of writing. Our son Jacob was under the care of Birmingham Childrens Hospital and they were absolutely fantastic with us as a family. The consulatant that we saw was so compassionate and thoughtful to our situation and very approachable for guidance on care, medicines,equiptment etc.We recieved our diagnosis on the 1st Nov 07 at the hosp and stayed there for 2 wks on the wards and the nurses were fantastic too.The ONLY thing i struggled with is to get a dieticaian appt cos the waiting list was ages, and its really important to get the right advice from them because some where like BCH would be one of the first to know new nutricianal (cant spell!!) milks that would benifit your son at the appropiate age and weight for him. it was 14 wk wait then, but as time has passed it may well have changed by now. Any how i hope this has helped a little, and wish u the best with your appointment, they are very experienced with specialist conditions there.
Nicky. |
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Re: Re: Re: Re: uk different to usa? |
Hi Nicky
we have been appointed a dietician but not from BCH..from sandwell hospital. My main concern and worry is that unfortunatly "specialists" may never have treated a baby with type 1 SMA before and so its just a stab in the dark as what may be best for him. Its so frustrating.
Dean |
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Re: Re: Re: Re: Re: uk different to usa? |
when u get up to BCH, try to push for a referal to their dietician dept, or ask for their opinion with your diagnosis and what they recommend. its nothing against the local Hospital of Sandwell, nor our local Hosp for that matter but all you want is the best advice from the most experienced specialist. I do sometimes wonder when your put on a waiting list for appts, that maybe that medical person doesnt quite grasp the severity of sma type 1 and getting the right treatment plan in place asap is crucial.BCH is one of the best in the country and once you are refered, and get a consultant, they will be be able to guide u to what you son needs. Could u get in contact with your local PCT and community team for advice on nutrician and equiptment? they may have some ideas too. if i can be of any use just ask or email me. I hope this helps u a bit, Take Care,
Nicky. |
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Re: Re: Re: Re: Re: Re: uk different to usa? |
hi dean, there are things like bi-pap that can be put in place which may help your son, I have now lost two boys to this condition, one at 7 months going down the route of pallitive care and toby recently at 11 1/2 months using NIV (non invasive ventilation), this was as a result of collapsed left lung due to an untreatable bacterial infection.
I live in poole in dorset and when I asked about bi-pap we were referred to london, they regularly deal with babies with SMA and they know exactly what it is, for all the best advice for your son I would urge you to be referred to them or another hospital with knowledge on the condition itself.
my local hospital was great and understanding but they did not have the knowledge of this condition, half the time no-one had heard of it and i had to explain it to them. I can only let you know what we did for Toby so here goes.
the list of equipment Toby had at home was:
1. oxygen concentraters, 2 piggy backed in the lounge so that we could reach 10 litres of oxygen if required and just the one in the bedroom.
2. 2 back up cylinders of up to 12 litres through each incase of power failure and the concentraters could not be used.
3. 4 ambulatory cylinders for going out
4. 2 sats moniters, 1 as backup incase 1 stopped working
5. 1 portable nebuliser with salbutamol and saline nebules
6. 2 suction machines, one for home and one for travel with a varying degree of catheters, we were also taught how no nasal suction.
7. 2 bi-pap machines one for upstairs and one for downsatirs and 2 back up batteries incase of power failure.
8. his pump for his his feed.
then we had his prescription feed and medicines.
1. started with infitrini but he put on too much weight with that so we changed to neocate low calorie.
2. vitamin supplement to complement his milk
3. glycopyrronium bromide solution, for help with thickening secretions we found it better than hyocine patches.
4. septrin profilatic antibiotics taken on alternate days.
5. salbutomol and saline nebs to help open airways and mobilise secretions plus when really bad DNASE nebs to help break down bacteria.
then of there was the physio after the nebs and play.
if this sounds like your list then you ok but also keep in mind this is what Toby had towards the end of his life and things gradually got put in place as and when he needed it, brompton were great in that it was better to have these things in place before we actually needed them so they kept a regular check on him.
nicky is very right in that that time is so important and I as I am sure she can't stress that enough.
we had Professer Andrew Bush who's area is pediatric respiratory support and has done a powerpoint presentation on options for type 1 respiratory support, if you google it you will find it.
there is also alot of info on families of SMA or www.fsma.org i think, of the care choices for SMA type 1 babies, click on the link for sma information on the grey bar and then on medical issues on the orange side abouth 4th or 5th one down and then on chest care.
on a lighter note, i'm sorry if it all seems alot of info to take in but having now lost two babies in two years and seeing the difference with Toby, i like to pass on that info. whether or not you take it and use is entirely up to you so I don't want you to feel like i'm being pushy there are no right or wrong way to treat this terrible condition, the best way is the way that is best for you and your family, also each baby is so completely different in that different muscle groups are affected and this will also plays part in how yur little one is treated and will be affected.
If you need any more help, advice etc please don't hesitate to ask. |
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Re: Re: Re: uk different to usa? |
| hi, sorry to post again but have just read that you son is fed high energy milk, do you know why this is? |
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Re: Re: Re: Re: uk different to usa? |
Hi milly
Our Dietician has put him on SMA high energy because of the weight that he was loosing... still waiting now to see if his weight has stabilised.
Dean |
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Re: Re: Re: Re: Re: uk different to usa? |
hi Dean, this is also the mistake i made with Toby, we think they should gain weight at a normal rate forgeting that our babies are not so to speak 'normal' if you get what i'm saying.
nothing about them is 'normal' in terms of their whole physical development.
say a 'normal' baby has length on the 50th centile, this means ideally their weight should also be around the 50th centile and so on with their head circumference.
a 'normal' baby would have muscle tone and muscle weighs alot heavier than fat so therefore this would be correct however with a baby with SMA their weight to be normal would actually be less than what you would 'normally' see for a baby the same age due to lack of muscle tone.
now i am no dietician but it makes perfect sense now that a baby with SMA does not need extra energy or calories as this will turn to excess fat as they can't move to burn off the extra calories, as it did with Toby and can cause weaker movements just because they have more fat to move. this is why we put him on neocate too, if the body takes more energy to digest food it has less energy to move. there is no proof that neocate works but i personally found that Toby's movement improved and so did his carers.
long story short, it is normal and understandable for an SMA baby to lose weight, it is a good idea to keep an eye on it but just as we do not expect them to walk, talk, crawl, feed the same as a normal baby we also should not expect them to put on weight at the same rate.
did you read my post before this one?
kathryn |
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Re: Re: Re: Re: Re: Re: uk different to usa? |
Hi
yes i did read it..and all of this makes for very interesting reading. About a week after our son was but on high energy he seemed to start sleeping alot more, maybe this has something to do with him being more tired dragging his limbs around.n Our dietician is due to visit this week so i will quiz her as to how much she knows about sma and the benefits and downfalls of this high calorie meal. Will keep you posted.
Dean |
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Re: Re: Re: Re: Re: Re: Re: uk different to usa? |
yes please do keep us informed, there is some info on the diet if you wish to speak to the dietician armed and ready the link is:
http://www.smasupport.com/diet.htm just copy and paste it into your browser and have a read. |
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Re: uk different to usa? |
Hi Dean
Mollie was also prescribed SMA High Energy milk as she wasn't gaining weight. Mollie actually fell below the 9th centile. Once she reached the 25th centile her milk was changed to a different one with less calories. Mollie's dietician was excellent. Although she had not specifically dealt with a baby with Spinal Musclar Atrophy before she had had experience with other neurological disorders which meant that she knew that Mollie shouldn't put too much weight on. We had the local health visitor come to see Mollie once a week to weigh her to keep track of her weight gain.
Mollie had Non Invasive Ventilation at night time but this was only introduced a month before she passed away. We did Sats testing on Mollie on a regular basis and her results were good until she was 10 months old and so we requested the ventilator which was provided immediately.
In my opinion the babies/children in USA appear to be living longer due to having a trache. This is not something that we wanted for Mollie. |
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Re: uk different to usa? |
In the USA, I get the impression that parents get what they want - life at any cost (including Tracheotomy)... you get what you pay for. In the UK, I believe families have a much more varied experience, and are not initially as informed (that's not a swipe at parents - that experience of being one!)
I guess we are lucky, Cariad is now 5, in school and has a team of people that help out. She has bipap at night (since 6 months old), is ng fed (she doesnt want a gastro-tube yet), is suctioned regularly and has a 'achieva' bed at school, a buggy that goes horizontal (wheelchair services have just finished building an electric version), potty seat, slings to help her move her forearm, cough machine (for those blocked moments) and a range of antibiotics and other medicines that help.
I am not too familiar with other cases of SMA type 1, as we have been pretty insular, but I dont think planning longer term is a problem if you can get access to the right package. However, I do say this with the caveat that I am only familiar with my own experience.
Wish you all the best
Jez Howat |
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