Yesterday was a good day, not anticipated to be as it involved several appointments at Great Ormond Street. As we live in London, albeit in the suburbs, our appointments tend to be scheduled for later in the afternoon, however anyone who knows London traffic knows that it's often easier to drive out of London than to drive around London.
Having picked Daisy up from her school it then took me 90 minutes to drive the 14 miles from school to hospital, 90 minutes of roadworks, delays and diversions with a little girl in the back of the car who doesn't like to sit still for too long and no-one else to entertain her. The only CD in the car suitable for Daisy was the Singing Hands Christmas CD so on a rainy May afternoon, stuck in gridlocked London traffic Daisy and I sang and signed Christmas songs - shall we refer to it as a little seasonal rebellion rather than the actions of a desparate mother?
Of course once I drove around and around the streets of Bloomsbury looking for a place to park which the ever vigilant Camden Council parking posse would approve of, I was late for our first appointment. This was with our Stoma nurse, a very important appointment as with four stomas Daisy needs a lot of help from nurse specialists. I was even a bit late for the second appointment but I always assume the clinic is running late (normally delayed by parents with me with a list of questions) so it wasn't too bad. Appointment two was Orthotics.
A couple of years ago Daisy needed surgery on her feet to lengthen her achilles tendons which had become so tight her foot was held in a permanent tip toe and she had actually lost the ability to walk. She spent 6 weeks in plaster casts after that surgery, normally children can walk when in these casts but this was not the case for Daisy. The anaesthetic caused her bowel to shut down and a three day hospital admission extended into a month as she needed intravenous morphine and ketamine to manage the pain. Needless to say we have to do everything to minimise anaesthetics and surgeries with Daisy as she can scupper best laid plans so we work with orthotics to ensure that now her achilles tendons have been lengthened they stay that way for as long as possible. Daisy wears (or should wear) splints to keep her feet in a good position, she tolerates them for a short time and then the velcro straps on them are pulled off and the splints are thrown across the room. We try and make them more bearable by having splints made up in funky colours and patterns, over the years she has progressed from Teletubbies and Bunny Rabbits to this week's splints - Pink Groovy Chick Girls. She loves them. In fact she doesn't mind an appointment at orthotics because not only did we pick up the new splints, but the Orthotist also ordered her new orthopaedic boots in a fetching shade of hot pint and some funky gaiters to help keep her legs straight when the physios in school work on her hip alignment (also not good, sadly orthopaedic issues are very common in children with Costello Syndrome and cause a lot of pain and discomfort).
So we left the orthotics appointment clutching new splints and expecting a parcel of more exciting goodies to be delivered in a few weeks - a productive shopping trip courtesy of the wonderful National Health Service.
Next stop (via a couple of social visits to various hospital friends) was the hastily rearranged visit to the Stoma Nurse. Helen the welsh stoma nurse is a bit of a legend in Great Ormond Street, what she doesn't know about stomas is not worth knowing! We have been having a lot of trouble with Daisy's ileostomy stoma, she has to wear a stoma bag over it to catch the waste, in Daisy's case because of her malfunctioning small bowel her stoma output is very high and watery which means that bags don't stick to her skin and need changing often, sometimes several times a day. Her mitrofanoff stoma which has prolapsed is very close to the ileostomy stoma and this causes problems getting a bag to fit, on top of all of this her jejenal stoma is also close by and constantly being infected as a result of ileostomy fluid leakage (have a look at the medi speak glossary if all this sounds confusing!).
Again a visit to the stoma nurse is like a shopping trip for Daisy as we always leave with a bag of stoma related goodies to try out at home. Hopefully we can come up with a good solution for Daisy as it is really uncomfortable for her to have her bag changed so often, the skin is breaking down and the stoma fluid burns her skin further.
Next stop was a very welcome rendez-vous with Andy at TPN clinic where we also had arranged to meet Daisy's palliative care consultant, Michelle. Since the Palliative team have come into our lives things have been so much better, their remit is to manage symptoms in order to maximise quality of life for terminally ill children, the work they do is outstanding, they are not going to make children better but they are going to make their lives more comfortable and manageable. We work closely with Michelle to look at Daisy's pain management regimen, on the whole it is working at the moment and we have moved from 5 nights out of 7 of uncontrollable pain to 2 nights, this is as a result of a complex regimen of regular and breakthrough drugs which are all designed to target different elements of Daisy's pain. Often Daisy will become tolerant to these drugs after a while and doses need to be tweaked and changed or the drug swapped with an alternative. We had a particularly bad night with Daisy recently where I had to use every drug in our armoury and we were still not on top of the pain, eventually I resorted to sitting with her and massaging her until she dropped off to sleep. Michelle suggested that as Daisy responds well to massage (but clearly we can't sit up all night massaging her poor tummy and back) that she could try a TENs machine.
I used TENs in my labours with two of my children and found it useful, if only for distraction (except where I dropped the controller at one point and it switched to its highest setting, that was too much distraction!), so we have decided to give it a go for Daisy. She was very interested probably because being a typical Nimmo child it's a gadget! It's just good to have a non-drug based option in the armoury and it's lovely to have a Consultant who tries to think laterally when managing Daisy's pain rather than throwing more and more medication at her which will affect her ability to function during the day.
I always enjoy going to TPN clinic as I get to have an all too brief catch up with other families we have got to know over the years. It was great to see my friend Emma and her lovely family, her son George has so many similar issues to Daisy and her little girl Ava is such a character, Emma makes lovely bracelets and she delivered my order of matching Mummy & Daisy bracelets to clinic for me.
We eventually got in to see our TPN consultant at 5.15, the nutrition team is all assembled at these clinics - The Nurse Specialist, Dietitian, TPN pharmacist, Consultant plus any other interested parties (normally by the time we get to go into clinic people have started to drop out!), and Daisy's Palliative Consultant normally comes in with us too. The team were please that Daisy is maintaining her weight and growth on the TPN. She gets 2 litres of fluids from 5pm to 8am, 7 nights a week. Two of those nights are "lipid" nights when she receives additional fats directly into the bloodstream, the rest of the nights are "Vamin" nights when she receives a balanced mix of intra-venous vitamins and minerals. We also administer a nightly dose of intravenous antibiotics, these help keep the numerous bugs that have colonised her body to a level where they do not cause sepsis, and intravenous omperazole for the severe reflux caused by her deteriorating fore-gut motility. Within her bags of TPN she also has intravenous ranitidine (again for reflux) and iron. Daisy is chronically anaemic, despite receiving maximum doses of intravenous iron every night for over two years. It was decided at clinic to look again at her iron stores as we have no room to give her more iron and while she has probably got a type of anaemia called anaemia of chronic disease the team want to rule out any other causes. The main focus of yesterday's clinic however was to discuss Daisy's next admission which is now planned for early July.
As Daisy is getting quite a few low grade fevers after we access her central line to administer her IVs (probably because it is colonised with bacteria) her consultant decided she should have her line changed next admission. This is a relief to us as it already has a repair in it and is under a lot of pressure being used to deliver a large volume of fluids plus two intravenous drugs daily. Daisy's hickman line is her life line, without it she cannot live so it is worth risking an anaesthetic procedure to make sure it is in the best possible condition than it breaking or the bugs in it growing to a level where the antibiotics we use to manage them no longer work and she develops septicaemia. At the next admission, known among the team as the annual TPN MOT, she will have a bone density scan, her bone density is reduced because of the way she receives nutrition and also as a result of the use of the anti-reflux medication and the risks of her syndrome, she is already classed as osteopenic, having the beginnings of osteoporosis but at the moment this is managed through extra vitamin d and calcium in her TPN, it may be that the next bone density scan shows she will need to have monthly infusions of a drug to strengthen her bones. She will also have a set of scopes under anaesthetic which involves putting a camera into what remains of her intestine to take biopsies as our worries are that it has become inflammed again. Normally this sort of admission is scheduled to take around 5 days but given Daisy will have two anaesthetics during that week we will see...
Our next TPN clinic will be in August following her inpatient stay. The only problem with that is that it is in the middle of the olympics and London will be a no-go zone. As life still goes for families like ours and hospital appointments still have to be made whether or not there is a major world event going on in the capital I am wondering how we are going to manage this appointment, particularly as public transport is so difficult with Daisy (it's not just the wheelchair its all the kit we have to carry in the form of TPN, pumps, spare stoma bags, catheters, feed pumps, changes of clothes, medicines etc etc) so driving is really the only option.
We have a fantastic mobile TPN pump which meant that after the clinic I was able to take Daisy's bloods from her Hickman line, connect her TPN drip and put the pump and bag of fluids into a back pack which hangs on the back of the wheelchair. So with all appointments out of the way, plans made and solutions worked out our day at Great Ormond Street was done (just as the cleaners were arriving at outpatients and the staff were stood with the coats on by the door waiting for us to leave so that they could go!).
Our final stop of the day, with Theo (who had travelled up to meet us after school) was the Lyceum Theatre to see the stage production of The Lion King. We had been very generously given some tickets by the Great Ormond Street Hospital Charity who had organised a block of seats for regular patients at the hospital. Daisy was beside herself with excitement, she loved every minute of the experience, from the moment we entered the theatre, as always she amazed us, she loved the show, despite its 2 1/2 hour running time and we just about managed to keep her untangled from her TPN wires and deal with pump alarms in the dark (always a relief to be in seats surrounded by other hospital patients as no-one bats an eyelid when your child's pump beeps in a quiet bit of the show). The production was sensational, but the best bit for all of us was how much Daisy loved being there and being with us, and most especially with her lovely big brother "Yoyo".
We got home really late, having once again listened to the Christmas CD for the journey home, but she couldn't wait to go to school today, clutching her Lion King souvenir programme and signing that she wanted to see more!
Yesterday was a good day and now my task for today is to order the Lion King soundtrack CD to put in the car for future journeys!
Websites close to my heart
- Shooting Star Chase (Daisy's Hospice)
- Great Ormond Street Hospital Children's Charity
- Torbay Holiday Helper's Network
- Sick Children's Trust
- Daisy's Angels - Facebook Page
- About Costello Syndrome
- Asperger Syndrome
- Caudwell Children
- Together for Short Lives (Children's Hospices in the UK)
- Costello Syndrome Support Group
- Give Kids The World
- PostPals - Putting Smiles on Sick Children's Faces
- Singing Hands (Daisy is their No1 Fan!)
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