Showing posts with label Mitrofanoff. Show all posts
Showing posts with label Mitrofanoff. Show all posts
Take time to smell the roses
When I walk home with Jules, my 9 year old son, from school we pass a house which has roses in the front garden. These are not the weedy, insipid offerings you see on garage forecourts, these are big, blousy, fragrant roses - the old fashioned ones I remember from my childhood. And more often than not I stop to smell them. Just have that moment, breathing in their heady scent, before the chaos of the evening descends.
Costello Syndrome Curve Ball
Daisy has Costello Syndrome - a very rare genetic syndrome with around 250 reported cases worldwide. It's caused by a sporadic mutation of an oncogene. An oncogene is a gene that causes cancer, children with Costello Syndrome have a 15% increased risk of developing cancerous tumours, we live with this risk every day. This week Daisy nearly became one of the costello statistics.
We have been concerned for a long while that we might have been missing something with the pain Daisy gets from her bladder and abdomen, the protocol for children with Costello Syndrome is that they should have regular ultrasounds to check for tumours, and with any unexplained pain or lumps there should be a high index of suspicion for tumour development. So last Tuesday I took Daisy along for her screening ultrasound to our local hospital, Theo was off school that day, recovering from one of his migraines so I brought him along too to distract her.
We call ultrasounds "jelly on the belly" and over the years Daisy has had hundreds of them and is very used to them, we go along to outpatients, say hello to the staff and hope to see the nurse we always refer to as "JennyfromXray", we have a picture of Jennyfromxray with Daisy as she had her first ever ultrasound at only a couple of days old.
We have been concerned for a long while that we might have been missing something with the pain Daisy gets from her bladder and abdomen, the protocol for children with Costello Syndrome is that they should have regular ultrasounds to check for tumours, and with any unexplained pain or lumps there should be a high index of suspicion for tumour development. So last Tuesday I took Daisy along for her screening ultrasound to our local hospital, Theo was off school that day, recovering from one of his migraines so I brought him along too to distract her.
We call ultrasounds "jelly on the belly" and over the years Daisy has had hundreds of them and is very used to them, we go along to outpatients, say hello to the staff and hope to see the nurse we always refer to as "JennyfromXray", we have a picture of Jennyfromxray with Daisy as she had her first ever ultrasound at only a couple of days old.
Not Waving But Drowning
Yes, I am still here, I know that it's been a while since I updated but as you can guess from the title of this blog, life has just been a bit too overwhelming to find the time/motivation/energy to fire up the laptop and write about it, but I think I have my blogging mojo back and in the interests of ensuring that the world understands what it is like for families like ours it's time to pick myself up and get the words down..
I hardly dare to write this but it's been two months since Daisy's last emergency hospital admission. Two months where well meaning people who don't know my world equate no hospital stays with Daisy being well. If only this was the case, no hospital stays are because every day is a battle for us to keep her out of hospital. Not being in hospital does not mean that she is better, it means that bar a life threatening emergency there is nothing else they can do for her. And actually the definition of life threatening emergency seems to be stretching a bit now, a couple of years ago we had to take Daisy to hospital if she spiked a temp or had positive cultures, now we judge whether this is a "manage at home" situation or a "call 999" one. The last admission was for intravenous pain relief but since then Daisy has been on various intravenous antibiotics at home to manage various bacteria which colonise the bits of plastic in her body. Now together with the TPN drip we put up every night and which provides her nutrition into her blood stream, we also draw up at least one antibiotic - a few weeks ago it was meropenem, now it's augmentin, IV antifungals are being mentioned too.....in addition to this, because of her worsening foregut motility and severe reflux (we are not talking vomiting here, we are talking gut renching, blue in the face wretching and projectile vomiting of luminous yellow bile...) we also administer anti-reflux medication intravenously. Drawing up an IV is not like pouring out a medicine, each iv has it's own way of being prepared - some (if we are really really lucky!) are ready mixed, most need mixing with something else - saline, water or some other solvent. They need to be drawn up, measured, air bubbles dispersed and administered. Sometimes as a slow push, sometimes as an infusion via a pump and always with a saline flush between each medication. So another compromise we have had to make to keep Daisy out of hospital and with her family is to add intravenous medication into our daily routine. But it is working and she is definitely better on IVs than she was off them. It's just sometimes it would be nice just to be Daisy's mum, sometimes it would be nice to say "tell you what let's not bother with putting the TPN on tonight, let's go out to the park instead", sometimes it would be nice not to have to change her catheter knowing it's going to make her cry or drag her away from her toys to change her stoma bag because the contents are leaking all over her clothes and burning her already sore skin....sometime there are so many things I would like to do rather than be Daisy's nurse but this is the pact that we have made...for Daisy to be here with us, for Daisy to be enjoying life then this is how it needs to be.
It's just sometimes when I have a brief moment and allow myself the really think about the enormity of the situation I realise how unfair it is, on Daisy, on us, on her siblings. Why does she have to have this rare, sporadic syndrome? It's nothing to do with one of us carrying a rogue gene, or our age or race, just one of those, chance in a million (make that 28 million) occurences that could have happened to any of you. So why then having this dodgy gene mutation does she then have to have intestinal failure meaning that she relies of chemicals and machines and plastic to live and then the final cruel blow - why does her bladder then decide that it's going to join the party too? That's just not fair.
It's the ripple effect that's tough too - Daisy has three siblings. These four children have one shot at childhood and while we constantly tell them there is no such thing as a normal family, a perfect life, I feel guilty at how sometimes I rely on their help as I change stoma bags and catheters and run through TPN when there is no other adult around to help me. Ask any of my children about TPN, Hickman Lines, Mitrofanoff stomas, gastrostomies and jejenostomies and they will confidently tell you all about them, probably more confidently than a junior doctor. They seem oblivious to the physical manifesations of Daisy's disease and see her simply as their sister and she in turn has them completely and utterly wrapped around her little finger. Daisy rules the roost at our house, and given her larger than life personality I think she would still rule the roost despite all her medical problems.
But for all of us, while we present a facade of coping those feelings of drowning are bubbling under. All three of my older children are receiving emotional support - one from CAMHS (child & adolescent mental health services) , two from the hospice and young carers groups. Andy and I attend counselling sessions. These sessions have given me time to reflect and take stock and think about me and what's important. I felt that because I am intelligent, articulate, willing but I think above all, so desparate to have Daisy at home and keep the family together, I was being laden with more and more work and responsibility and risk to manage this. I felt like I had been thrown into a lifeboat with a load of supplies and set adrift left to fend for myself while everyone waves from the land. But the thing is, I am not waving, I am drowning - I am drowning under the overwhelming workload that comes with looking after a chronically sick child, trying to be there for the other children and listen to their problems, trying not to get angry with Theo's asperger's outbursts and meltdowns and reminding myself it's not him, it's the autism, drowing under the paperwork and bureaucracy that comes with my world.....and it seems to me that the more that the world thinks you are coping, the less likely it is to check if you really are.
So when I stood back after a recent long, all day appointment at Great Ormond Street and took stock of all the addtional meds and IVs that we were being asked to add in to our already packed daily routine and I had an epiphany and I realised I could just say no. So I did. It was not about putting Daisy at risk, it was about ensuring that overloading me (because remember it is mainly me, Andy works long long hours to keep a roof over our head) did not put Daisy at risk. This was a really liberating feeling because suddenly the wheels of support starting to turn and a better, more manageable plan was formed, a plan that could be managed at home taking into account the workload and Daisy's needs. Since that time I have been on a bit of a roll, realising that I don't have to do it all. I have stood back and taken a look at my life and what is important, for me the most important thing is my family and my marriage. Since having Daisy I have gradually acquired other projects and interests to take the place of the career that I had to give up when she was born, these things have started to take up more and more of my time away from the family and I have realised this is not what I want, so taking advice from the great Homer Simpson I have asked myself "Can't somebody else do it?" and I have discovered that I am not infallible, the world will not fall apart if I withdraw myself from a voluntary group or committee, there will always be enthusiastic souls like me who are happy to take on more than the lion's share of service to the community!
I have not given everything up though, I have made room in my life for something that is just for me, long distance running. I have run on and off since I was twelve, I ran a marathon at 13. I may not be fast but I have the mental and physical stamina which ensures that I can run and run. And recently this is what I have been doing, I plug in my music and run, morning, evening, it does not matter, running is my prozac, it's my time out away from everything where I can meditate to the repetitive rhythm and escape into music and thoughts. Running keeps me physically and mentally strong to keep going, because I have realised that life with Daisy is not a sprint, it's not a marathon, it's an ultra marathon, iron man, triathlon, decathlon endurance event and she, and the rest of the family, needs me to be up to the challenge.
So I have regained my mojo and with it normal (maybe a bit more streamlined and focussed) service has resumed.
Surgery Plans
Oh my goodness, a whole month has flown by and I have not updated this blog - big apologies!!! I think I'm going to cut and paste some of my statuses from Daisy's Angels (www.facebook.com/daisysangels) from now on so that those who don't use facebook can still see what is happening in our lives...
It's been a funny few weeks, lots of real progress in terms of plans for Daisy, more emergency trips by ambulance and time at our local hospital and we are even managing time at home - for the first time in years I'm actually getting to spend part of the summer holidays with all the children under the same roof.
The biggest news is that we had our much anticipated meeting with Daisy's surgeon at Great Ormond Street. This was the meeting to discuss whether or not a colectomy was an option for her and whether he was even prepared to carry out the surgery. Andy and I had talked and talked about this prior to the meeting, veering from being 100% sure we wanted to go ahead to deciding that it was not an option as there were no guarantees. In the end meeting with the surgeon reassured us of the safety of performing the surgery, he will not perform a full proctocolectomy as this is just too big for anyone let alone Daisy, instead he will take out most of her large bowel which will hopefully massively reduce the colitis symptoms she experiences every day. It will also mean that she can come off the drugs which supress her immunity and render her at risk of infection and we can treat the colitis more conservatively. This will not be the only surgery Daisy will have at the time, Daisy's Urology surgeon will also perform surgery on her bladder at the same time to help manage the symptoms of it's deteriorating function. Initially the plan was to form a vesicostomy, a stoma, from the bladder into her abdomen to allow urine to drain out into a nappy. After much consultation and discussion however everyone agreed that this was not an option for Daisy, a little girl desparate to get out of nappies. Instead along with the colectomy surgery Daisy will have a Mitrofanoff procedure where the appendix which will be removed as part of the colectomy will be used to form a channel from her bladder to her abdomen allowing us to catheterise her intermittently. Individually these surgeries are big, performed together on a girl who is not in optimum health they are huge. She will need to spend some time post operatively in intensive care and it is likely that we will be looking at another prolonged stay in Great Ormond Street, however our hope, which comes with no guarantees, is that once she has recovered from the surgery, we can reduce some of the drugs she is on which have such awful side effects, her pain may be more manageable and she will be less susceptible to the infections which often lead to emergency trips to the hospital. Making the decision to go ahead with the surgery was the hardest decision we have had to make for Daisy - we don't know if it will make things better or worse but the main thing that swung it for us is that she or we can't keep going on as she is.
The pain she experiences, mainly at night, is getting worse - she now has regular doses of morphine plus ketamine and then when this doesn't work sedating medicine to help her sleep. We are seeing a big deterioration in her health, her hair is falling out, she is getting tireder. But her lust for life is immense - she managed very few days in school this year but every single day was packed to the brim, she won a medal in sports day, had fun times with her friends, learned new skills. She is vocalising more and more, singing away to herself in bed and her hands never stop signing. She has so much she needs to do and this is why we made the decision to go ahead with the surgery - desparate times call for desparate measures so although it doesn't have any guarantees to take the pain away we just hope that it will keep her hospital trips down and improve her quality of life so that she can enjoy the things she loves in life.
In the past month we have called 999 three times, each time Daisy has spiked high temperatures and developed sepsis, mainly from the bugs (ecoli & candida) which are permanent residents in her bladder. I used to drive her to hospital myself when she became ill but she is going downhill so quickly now we have been told not to risk it, so now the whole neighbourhood knows when Daisy has gone back into hospital. But as quickly as she goes downhill, Daisy can bounce back once she has had enough doses of IV antibiotics. So the moment she can come home we have been out and about having fun as a family. We visited Peppa Pig World earlier this week - Daisy was beside herself with excitement, as everyone who knows her knows, she adores Peppa Pig, almost as much as she loves Mickey and Minnie.
We are keeping everything crossed that we can also manage a trip to Devon towards the end of next week, Daisy will be able to meet her new cousin and we will be able to have a lunch to celebrate my mother's 70th birthday (Daisy will be in Great Ormond Street when it is Grandma's birthday so we are having an early celebration). We have been given a date of 9th September for Daisy's surgery, this gives us a few more weeks of family time and also hopefully gives us a chance to be out of hospital in time for Christmas. We are in Daisy's hands as always but have learned flexibility and whatever happens we will adapt to make the best of the situation.
It's been a funny few weeks, lots of real progress in terms of plans for Daisy, more emergency trips by ambulance and time at our local hospital and we are even managing time at home - for the first time in years I'm actually getting to spend part of the summer holidays with all the children under the same roof.
The biggest news is that we had our much anticipated meeting with Daisy's surgeon at Great Ormond Street. This was the meeting to discuss whether or not a colectomy was an option for her and whether he was even prepared to carry out the surgery. Andy and I had talked and talked about this prior to the meeting, veering from being 100% sure we wanted to go ahead to deciding that it was not an option as there were no guarantees. In the end meeting with the surgeon reassured us of the safety of performing the surgery, he will not perform a full proctocolectomy as this is just too big for anyone let alone Daisy, instead he will take out most of her large bowel which will hopefully massively reduce the colitis symptoms she experiences every day. It will also mean that she can come off the drugs which supress her immunity and render her at risk of infection and we can treat the colitis more conservatively. This will not be the only surgery Daisy will have at the time, Daisy's Urology surgeon will also perform surgery on her bladder at the same time to help manage the symptoms of it's deteriorating function. Initially the plan was to form a vesicostomy, a stoma, from the bladder into her abdomen to allow urine to drain out into a nappy. After much consultation and discussion however everyone agreed that this was not an option for Daisy, a little girl desparate to get out of nappies. Instead along with the colectomy surgery Daisy will have a Mitrofanoff procedure where the appendix which will be removed as part of the colectomy will be used to form a channel from her bladder to her abdomen allowing us to catheterise her intermittently. Individually these surgeries are big, performed together on a girl who is not in optimum health they are huge. She will need to spend some time post operatively in intensive care and it is likely that we will be looking at another prolonged stay in Great Ormond Street, however our hope, which comes with no guarantees, is that once she has recovered from the surgery, we can reduce some of the drugs she is on which have such awful side effects, her pain may be more manageable and she will be less susceptible to the infections which often lead to emergency trips to the hospital. Making the decision to go ahead with the surgery was the hardest decision we have had to make for Daisy - we don't know if it will make things better or worse but the main thing that swung it for us is that she or we can't keep going on as she is.
The pain she experiences, mainly at night, is getting worse - she now has regular doses of morphine plus ketamine and then when this doesn't work sedating medicine to help her sleep. We are seeing a big deterioration in her health, her hair is falling out, she is getting tireder. But her lust for life is immense - she managed very few days in school this year but every single day was packed to the brim, she won a medal in sports day, had fun times with her friends, learned new skills. She is vocalising more and more, singing away to herself in bed and her hands never stop signing. She has so much she needs to do and this is why we made the decision to go ahead with the surgery - desparate times call for desparate measures so although it doesn't have any guarantees to take the pain away we just hope that it will keep her hospital trips down and improve her quality of life so that she can enjoy the things she loves in life.
In the past month we have called 999 three times, each time Daisy has spiked high temperatures and developed sepsis, mainly from the bugs (ecoli & candida) which are permanent residents in her bladder. I used to drive her to hospital myself when she became ill but she is going downhill so quickly now we have been told not to risk it, so now the whole neighbourhood knows when Daisy has gone back into hospital. But as quickly as she goes downhill, Daisy can bounce back once she has had enough doses of IV antibiotics. So the moment she can come home we have been out and about having fun as a family. We visited Peppa Pig World earlier this week - Daisy was beside herself with excitement, as everyone who knows her knows, she adores Peppa Pig, almost as much as she loves Mickey and Minnie.
We are keeping everything crossed that we can also manage a trip to Devon towards the end of next week, Daisy will be able to meet her new cousin and we will be able to have a lunch to celebrate my mother's 70th birthday (Daisy will be in Great Ormond Street when it is Grandma's birthday so we are having an early celebration). We have been given a date of 9th September for Daisy's surgery, this gives us a few more weeks of family time and also hopefully gives us a chance to be out of hospital in time for Christmas. We are in Daisy's hands as always but have learned flexibility and whatever happens we will adapt to make the best of the situation.
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