As many that read my posts will know, the child was receiving fairly abysmal tertiary care for the last 3 or 4 years prior to our move in July 2019. Even as we ran screaming for the hills, they managed to massively cock everything up by not treating an ongoing infection for which we had positive culture, and also completely failing to refer her to a new team. By the time we could finally, officially get her on the RBH’s caseload and seen the infection had been running rampant for almost 6 weeks, she’d been coughing up blood on the daily for 3 of them, and her fev1 was down to 46%. Her new consultant pretty much admitted her there and then, and we now know that everything I’d been told about her lungs not being particularly damaged, and that her low lung function couldn’t possibly be accurate, was - as suspected - all complete rubbish. They couldn’t have got it much more wrong if they’d tried, and the only reason for getting it so wrong was arrogance and an unwillingness to consider any opinion other than their own, even when other medics had openly communicated concerns.
Fast forward almost 12 months and I get an email from one of the nurse specialists this afternoon: don’t suppose you have a copy of her genetics, do you? I suspect this has come up now as a result of the Trikafta/Kaftrio news, as her genetics mean that she’s going to be one of most likely only a small handful of kids on their caseload that isn’t eligible. At the Past Centre That Shall Not Be Named, of a caseload of around 260 kids, she was one of only 3 that didn’t carry even one copy of F508del, which is the genetic mutation that most precision meds like Kaftrio are designed to correct. Although, initially, they insisted there were only two on their caseload, and the nurse actually started scrabbling through Bod’s file in front of me in an effort to confirm I must be wrong about my child’s genetics. (Spoiler alert: I wasn’t). Anyway... it now transpires that the old centre have never sent them an official genetics report. As I never got written confirmation of her diagnosis or her genetics, just a verbal one at 5 days old, I don’t have any indisputable proof as to what her genetics are, which means the Brompton now don’t either, and as the old team have consistently failed to respond to requests for information to date - like, oh, you know, blood results, culture history, and sensitivity profiles for a fairly sick child - I wouldn’t be at all surprised if they decide the only option is to re-run the genetic screening when she goes in mid-August. It’s of no consequence, nothing will change as a result, other than the child having a complete set of notes with fully dotted I’s, but it’s the principle of the flipping thing. A clinical diagnosis of cf requires both mutations being identified and a sweat test. They informed me last year that they’d lost the original sweat test result from when she was 8 weeks old, so we had to get that repeated a couple of months before we moved, and now I’m wondering if they also lost her genetic screening profile. Why else would you not send definitive proof of a diagnosis when transferring a child to a new team? I suspect the only reason they even realised about the sweat test is because she was moving and they needed to include it on the referral letter.
What a terrible indictment on the medical profession Charlie. It makes my blood boil to think they just needed to do everything there way without taking your views on your own daughter into consideration. Unbelievable.
Im glad you have now got a good team supporting you and your daughter. It helps when you feel the medical team are on your side and not fighting you every step of the way.
Maybe now you have moved it would be a good time to put in a official complaint. If the problems you had arnt flagged up they just keep being repeated. And unfortunately others will continue to be let down by the bad team. Eather way hopefully you are in good hands at the Brompton.
I am so sorry to read of your terrible experiences, and I wish for you some level of peace through finding trust that the team giving care are actually doing their job and on a most simplistic level doing the tests needed and keeping the results properly and acting upon them.
It is very difficult to put that anger and regret at the enormity of the consequences of arrogance and incompetence behind you. I hope you find a way at some point - I sought professional help to find ways to cope.
Terrible tale. But I am afraid I am not surprised. My own tale is no where near as serious as your daughters but time and time again doctors ignored test results because they had already made up their minds that there was nothing the matter with me. Eight consultants said there was nothing the matter with my lungs. And they were wrong. As a consequence I have become very interested in cognitive bias in the medical profession and the resulting misdiagnoses. I will PM you as it may interest you to see some work that is being done in this area.
I remember reading in a textbook, when studying health psychology, about a doctor patient meeting:
Doctor: I see you have two boys.
Mother: No, a boy and a girl.
Doctor (insisting): No, it says here, two boys.
I've come across appalling arrogance in the medical professions too, and it has done me some damage, though not as much as you, Kate. or Charlie_G's bod. And yes, I have complained, because how else are things going to change.
Please do PM me Kate, as I’d be extremely interested. We were very unlucky with the care we both received where we moved from. I think the issues with me stemmed to some extent from prior mental health history in my teens that I stupidly revealed when asked about medical history during my first episode of arrhythmia. I’ve found there’s this really bizarre belief that anyone who has ever been severely depressed or anxious categorically must be a hypochondriac, even though I’d been well for a number of years and often scolded by my brilliant GP for not seeking medical advice when I should have done. To give you some examples:
I was told for two years that my severe headaches and gradually increasing inability to walk when Bod was a toddler was all in my head. Well, they were right, it was, just not the way they meant: by the time I got the correct diagnosis and was listed for brain surgery for a rare, congenital malformation, I could barely walk at all even with two walking sticks and was in agony 24/7. They said that they felt the surgery would help my headaches, but although my other symptoms were classic of my diagnosis, the severity of the problem didn’t match to the severity of what I was experiencing, so the word psychosomatic was bandied about even to my face until they saw me able to not only stand upright, but also walk up and down stairs completely unaided just 4 days post surgery. My neurosurgeon always took me seriously after that, and ten years on we now know that what happened with me is quite common for the condition.
After my second bout of surgery for that a couple of years later, I presented in A&E 8 days post op and 2 days post discharge with all the classic symptoms of meningitis including the most excruciating headache I’ve ever experienced ever, which is saying something when I’ve been living with migraines and chiari pain for years. They gave me morphine, and because that rapidly worked to stop my agonised moaning, immediately proceeded to interrogate me about any drug abuse history I might have. I arrived in the department at 9am, was transferred by emergency ambulance to the operating hospital at 3pm having had some bloods done and been rammed full of tramadol that did absolutely nothing, but nothing else. Was finally seen by a consultant on the neuro ICU at 8pm, throwing up and barely able to see straight, and told that they were certain it was entirely post-operative pain and I was over-reacting, but that they would do a lumbar puncture ‘just to prove it’. Puncture was done at 10pm, a full 13 hours after first going to A&E, and at 2am, a very sheepish looking consultant came in to tell me my white count - which should have been no more than 5 - had come back at over 2000 as a result of bacterial meningitis and I would be staying with them for a while. Spent almost a month there on IV antibiotics. That was at the same hospital providing Bod’s tertiary care, and all I can say is arrogance seemed to be a recurring theme in both paeds and adults.
The local services, including a rheumatologist, completely missed my inflammatory arthritis for 10 years, instead constantly asking about my stress levels whenever I went with joint swelling and/or extreme fatigue and telling me how draining it must be caring for Bod on my own when she was so ill. The end result of that is that instead of only having issues with a couple of joints, by the time I got diagnosed in Jan of this year following our move, it’s now rife in 9 and my lower spine.
On one occasion I was left sitting having a confirmed cardiac episode in the waiting area of A&E for 40 minutes only to then have to rush me into resus when they did finally triage me, even though the bloomin’ paeds on call had phoned ahead whilst staff were wheeling me over because I’d been taken ill with Bod on the ward for IVs. The stupid thing is that the department was empty, they knew what I was there with, and a walk-in with a cut to his hand got seen before I did!!
As you can probably tell, I have a lot of stories for myself in a similar vein, those are actually just the ‘highlights’. The good news is that where we are now is leagues away from all the hospitals where we were, and Bod does at least seem to be getting the care she needs. The issues with her old consultant seemed to at least partly stem from him also deciding I was an overly anxious father, and once he’d drawn that conclusion, there was no going back. Prior to him joining the team, though, she’d been a nightmare for 6 years. He hadn’t seen all the drama in her first year, the times she’d had full on emergencies on the ward and been rushed off to PICU or HDU, the endless head scratching or clinical trials because they didn’t really know what was going on or what else to try. At the point he took over, after years of joint effort and hard work she was stable and actually doing well, and then he started making unilateral decisions about her care and it all went to pot again. I’m there going something’s not right, he’s going I think she’s pretty good, only for it to turn out she had a massive lower left pneumonia that subsequently took a month in hospital to treat. She spent 5 months out of 12 in hospital, yet he acted like that was perfectly fine, and in actual fact was entirely unnecessary, as if I’d somehow forced multiple different doctors to admit her?! Our local consultant was brilliant and very experienced, he used to be a cf reg at a tertiary centre, but his view counted for absolutely nothing despite tertiary saying how much they trusted and respected him. Emails with concerns were ignored. Requests for her to be seen at tertiary were often ignored. He always said to me that if I said I was concerned then he got really concerned because he’d been through so much with us and knew that I’m not one to fuss for no reason. When well the local team would go months without hearing from me, the problem was that she was rarely well, in part because tertiary wouldn’t listen to anyone. At the end, he was pretty much beside himself because he agreed we absolutely needed a CT scan, we needed an LCI, and he expected them to be abnormal, but although he could request them, tertiary had to agree them and they just wouldn’t because they’d (incorrectly) decided for reasons best only known to them that she was fine. My last conversation with him was the week before we moved. Bod was a week out of tertiary, who had discharged her coughing up green, losing weight, with positive microbiology that hadn’t been covered for and generally in worse condition than on admission, and he just apologised profusely and told me to get her to the RBH as quickly as possible. He felt like he’d let us down as much as the tertiary team had. The hardest bit of it all, though, was that that one opinion at tertiary poisoned all the other relationships there. Where other team members had previously taken us seriously, over time they were influenced by this consultant’s views, including the nurse specialists who had dealt with us since birth. When a new consultant joined the team - from the RBH, ironically enough, who sang her praises when we first arrived there - it was almost like she took his view as gospel, and immediately treated us the same way. I strongly suspect he’s a very difficult man to work with, so wonder if that may be part of it: staff adapting to survive, particularly after he was made the clinical lead a couple of years prior to us leaving. In any event, we’ll always be left wondering if things might have been different for Bod if they’d just briefly considered they could be wrong, because we now know they were wrong about absolutely everything and then some, and we’re the ones that have to live with the consequences of that.
Charlie_G my heart goes out to you. I pray things look up for you with the new team and that someone in it maybe consults with your GP who seems to have a better handle on things. An over the phone conversation with the GP and the new consultant might be better as then the GP could say things that he might not want to write down.
Even as a teacher I see parents or students sometimes being put into a box and then "colleagues' take on the beliefs of other colleagues. One teacher had a student for two years and complained that the parent was "over anxious" (It's not only doctors who give that label). After the first week the parent came to me with a nervous tension and sounding very scared at even approaching me. She was concerned about her son. I had tested them that week and was able to reassure her that, "Yes, she was right. He was having difficulty and I was putting him in for special needs so that he would get extra help." This parent and I got on fine. She wasn't "over anxious". Her anxiety was real and exacerbated by the fact that she was treated like an idiot and not listened to. Another personality might have moved schools or seen the principal. This lady was intimidated by a very forceful personality (the teacher) who was also in a position of power.
I find that sometimes it doesn't help to move as the assumptions of the previous lot come with you in their notes. It only helps if the next person is open. Maybe write down everything as it happens. Often the doctors who make assumptions are never told that their assumptions were wrong. Someone from another shift comes in and they don't hear what happens. Ironically also the angrier and more frustrated a person is - the more they think it's anxiety. I've had to try and keep my cool many times - but haven't always quite succeeded.
I still smile however with enjoyment when I think of my sister (after 24 hours of no sleep) yelling at the top of her voice, "Well if all you're going to do is kill my mother, then I want an ambulance to take her home now". The words were well deserved and true. She had just started a drip when it came out in a move between wards. The doctor had refused to put it back in and give her fluids. (she was also "Nil by Mouth") until a Speech Therapist could see her in 24 - 48 hours. He was the same doctor who had changed her original active therapy in emergency to nothing with the change of shift. I believe he was giving her the "Liverpool Care Plan". We got the ambulance within the hour when we had been told it would take 24 hours and Mum did well at home with antibiotics from a home visit doctor. My sister did what I've wanted to do for years. However I still practice control as I don't want to be kicked out - as we luckily were! Although they never expressed it as such.
All of this simply confirms my lifelong frustrations with the medical fraternity and you know how I feel for you. My only thought is that as they got so much wrong, they may have made a mistake with the genetic profiling too. Maybe a fresh look at it all might result in the discovery of the mutations which she needs to have to access all new drugs going forewatd.🤞🏻
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On the one hand, that would be great given the spectacular results some people are getting with Kaftrio. On the other, given our carrier results came via a different service antenatally, unless they also spectacularly cocked everything up, a different profile would mean either the ex cheated on me and the Bod isn’tmine or one of us has got cf and doesn’t know it yet, so....👀😬😂
Still, as you say, given our experiences to date, it might still be worth doing anyway.
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I have a quiet hope (probably useless) about kaftrio. I was talking to my consultant about the possibility of its eventual use for us bronchs with no genetic mutations. Asking actually if the present restriction is more due to money rather than efficacy. As in her position she has to be circumspect in what she says, she trotted out the usual answer that no testing on ncfbe done so far but when they can get money hopefully it will be. I hope that this may be a marker to its eventual adoption for use in cf and non cf where those two markers are not present. The cf community have so much money and influence that hopefully they will push for its testing and adoption for cf patients like bod.x
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At the minute, we know within the cf community there’s going to be a minimum of 10% of the cohort who aren’t eligible. We also know that number will ultimately be higher: it’s contraindicated in liver disease, and we know from the existing precision meds and the clinical trials for Kaftrio itself that a percentage of those started on it will find they’re unable to tolerate it. I think the number will probably be under 15% in the end, but that’s about 1500 patients. Less than a third of that group will be children. The prevailing concern is going to be cost, there’s no two ways about it, but if you have the younger cf cohort no longer having the kind of lung disease and chest issues that they typically have done (and the norm already is for many children not to require IVs for the first time until they’re at least 9 or 10 due to the other general improvements in treatment over the last 30 years), and the significantly increased, perhaps even to the point of being almost normalised, life expectancy that will likely follow with that in time, I think there will be much greater scope for ncf bronch to finally get the attention it deserves, because the two conditions will be more on a par than they’re considered currently. I don’t truly understand the science enough to comment on whether there’s any scope for Kaftrio to work in the absence of all known mutations, but I wonder if a study with any ncfb patients that are listed as carriers might be an easily justifiable route into trialling the Vertex meds, as well as an interesting way to at least part test the hypothesis that it’s part of a cf spectrum rather than being a distinct condition. The HIT CF study currently running may lend weight to something like that if it ultimately determines that any of the drugs have impact for a wide range of patients even in the complete absence of F508del.
We’re about 6 years behind where we should be with researching modulator use in the 10%. It’s almost been an afterthought, and the cf trust keep banging on about how no one has been left behind, blind to the fact that however unintentionally, the 10% have been left behind, they’re just too out of touch to see it. There was a blog post the other week from the clinical lead at the Trust who quite openly said that the reason we hadn’t been left behind is because they’re doing lots of work on antibiotics and other generic treatments for existing issues that will benefit everyone regardless of mutations, as if that was somehow comparable to a drug that in some cases has led to a 50% increase in fev1, but mean averages at 14%. I was gobsmacked, but hardly surprised having watched the gulf steadily widen for the last decade, and realise this is how many bronch patients such as yourself have probably been feeling for decades. The 10% are a middle tier emerging between cf patients with F508del and non cf bronch patients in a fairly obscene and unnecessary hierarchy of needs. There are a couple of non mutation specific therapies to correct CFTR function currently going through phase 1 clinical trials, but I’m not holding my breath. I also won’t get excited about the HIT CF stuff until the phase 1 outcomes become available towards the end of next year and they’re positive.
in reply to
Thank you Charlie for that very detailed and interesting response. Unfortunately it does seem that the publicity grabbing fact of help for some patients leaves others - the 10% and ncfbe out in the cold more and more. People are only interested in the good news and it is easy to hide those who are being ignored. All we can do is keep fighting, keep nagging. At least little one is with the best people now and I do hope that develooments for her do come along sooner than later.
Sorry to read this but not surprising sadly but how frustrating for you. Maybe this fresh start could be the most positive thing for her and together with the other aspects of her life that are now coming together, she may get the best treatment possible from now on. I just hope past mistakes in her treatment haven’t caused her any lasting harm.
Yes the medics can get it wrong but to get it very wrong and not listen -there's no excuse. Wishing you and Bod all the best with your new team. x Anita
Although my daughter was never as ill as your child I too suffered from doctors who ignored my input. For the first 4 years of my girls life she was ill. No doctor or health visitor listened. I changed doctor and Wow! what a difference.
Suddenly my daughter was getting the treatment she needed.
In the new health centre I had two doctors who saw my child and cared. Such questions from the doctors as "In what way is your child different to her norm" (OH bliss not comparing her to a text book) and to the child "How are you today? I'm so sorry the last medicine didn't work. There were two I could have tried and I'm really sorry that I chose the wrong one for you. I'd like you to try the other one." (Showed her the pages in the book where the medicines for her problems were listed).
I do hope things improve for your child's care.
Btw the result of the first four years of bad care resulted in my daughter having terrible weak teeth, as the dentist said "You can see how ill she was by the weakness in her teeth I think she'll need dentures by 30" She's mid 40's and they look like an old woman's from a poverty srticken era, thin enamel so yellow/brown, chalky patches and fillings where they crumbled. A small price considering if I had not changed doctors she could have died)
What another terrible tale. And yet sadly common. Again my daughter did not have the serious problems that yours or Charlie's Bod did but three times I was right and the doctors were wrong. And the first time it could have been life threatening. I knew she had appendicitis but the doctor insisted it was cystitis, and treated her with antibiotics. It did clear up the appendicitis but I knew it would crop up again and it did, this time the doctor took it seriously but there were complications because of the previous neglect. When will they learn to listen to the patient and listen to the patient parent? We know our own bodies and we know our children.
My younger daughter had appendicitis. That was overlooked by everyone until she nearly died. However in our defense she was atypical. All we knew was on a Tuesday she said she felt sick. 13 yr old. Sent to bed with a bucket and a drink of water. Sure enough she was sick that night and spent the next day sipping water and sleeping on and off. Thursday she was dripping about doing nothing but looking washed out. Sent back to bed with water, was offered toast but refused. Overnight Thursday to Friday she was very ill but didn't wake anyone. Friday morning I woke early to a foul smell and a skeletal child with 'fish' eyes who was too weak to stand.
I had no phone but got a neighbour to call the doctor, explaining it was urgent. The receptionist just said a 13 yr old been sick.
I was about to go to the neighbour to call an ambulance when the doctor arrived.
Daughter blue lighted to hospital and appendix out It bust in the operating theatre.
There was proof it had an old but healed abscess.
When asked about symptoms, pains etc my daughter said "No, only the stabbing pains in my belly button but everyone gets those"
The worst of this is it’s all avoidable by resourcing. When my son had a pain down one side, my daughter who’s a physio said it could be appendicitis so he went to the doctor who was able to scan it there and then at the surgery and he was in hospital 2 hours later having his appendix out. He lives in Switzerland. Why can’t it happen here? It would make GP services more streamlined and effective. If that had happened here he would have probably been fobbed off with some medication or told it was indigestion or ibs.
The problem with that is my daughter didn't tell us about the pain until after her operation. She was never a one to make a fuss, even falling of a skateboard and skinning nearly half her face she didn't cry.
If I'd known about the stabbing pains I'd have acted much sooner as I know stabbing pains in the belly button are a sign of appendix problems.
I asked among my friends who all said at 13 they wouldn't have worried about a child feeling sick and like me sent them to bed with water.
A painful read, totally understand your frustration and fury. Absolutely appalling non-care of your precious child, a whole chunk of her life. I hope they're ashamed & also praying that the unnamed centre isn't in Buckinghamshire !
It’s ok, Peege, to the best of my knowledge, Bucks is fine and certainly nowhere near the Centre That Shall Not Be Named 😁
I honestly don’t know that the specific consultant involved is capable of shame, he’s just that arrogant and narcissistic. Sad though it is, I do occasionally fantasise about what his reaction might be/have been if anyone from the Brompton ever spoke to him about Bod, though. Something along the lines of ‘blimey, that kid you sent us last summer was a mess, wasn’t she?’: he always had a terrible time schooling his face, particularly if caught out or surprised, and I would just love to see his reaction to being told how wrong he was. Ideally, if there is a God out there, at the national, annual conference whilst surrounded by his peers all suddenly looking very interested by what’s being said. Not that I’m vindictive in the slightest, of course 👀😏
I'm wondering if you can do reviews for consultants &/or hospitals like we can for GP surgeries so it's out there in black and white. We can only hope that karma will have him.
Im surprised you have stayed sane Charlie and can only hope that now you have moved things will change and allow you to get your sanity back, lets hope the Brompton have a better medical team and are willing to listen to you , you have a wealth of knowledge and know what you are talking about so at least wont be fobbed off. My family have suffered so much over the years with doctors getting things wrong that I find I wont take anything that I think maybe wrong. Good luck to you x
In my parents generation, doctors were God. You did what they told you and you never questioned them.
Things have changed!!!
You question and if you don't like the answer, go to another doctor.
When my children were little, I had a great doctor. He said: Who would know more about whether your child is sick (or that something wasn't right) than that child's parent. He would listen to the parent.
Charlie it makes me so very sad to hear how you have both been so short changed in the past by worse than useless clinicians. It is scary to think that people are still being treated by these people.
Best decision you ever made to move. You are an extraordinary man and dad and I hope from the bottom of my heart that both your future dealings and treatments with the medical profession are second to none.
I was so sorry to read all your story. I can only repeat what cof-drop has just said to you. Hoping things will be better for you both now in your new location.
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