Some basic research into the disease;
- degenerative neurological disorder
- incurable (death usually within 1 year)
- it is a prion disease – group of progressive conditions that affect the nervous system
- similar to Bovine spongiform encephalopathy (BSE or mad cow disease) which is a type of TSE (transmissible spongiform encephalopathy) – potentially BSE causes vCJD (varient CJD), by eating contaminated beef, as the agent responsible for BSE outbreak is similar as for vCJD
- caused by transformation of normal prion proteins -> abnormal prions
- risk increases with age
- vCJD is more likely to affect younger people, it has a longer illness duration
- iCJD: acquired CJD
- sCJD: sporadic CJD
- symptoms - rapidly progressing dementia (mem. loss/ personality change/ hallucinations) – ataxia (co-ordination dysfunction) -speech impairment
- PRION: -infectious agent composed of protein in a misfolded form – act by causing normal proteins to convert into the disease-associated prion form -causes large quantities of affected cells
- prion exhibits 2 stable conformations 1. native state – water soluble and presents healthy cells (used transmembrane transport/signalling), 2. insoluble and readily forms the aggregates
- mass of misfolded proteins disrupts cells function (nerve cell enzymes cannot digest the new proteins) which causes cell death (cell bursts) – this is how CJD is diagnosed in autopsy
-> the burst cells mean there are many tiny holes and thus the brain takes on a spongy texture
- can be genetic – mutation of PRNP gene (the gene which codes for the prion protein) 5-10% of all cases -> causes alpha helical regions to change to beta pleated sheets therefore cannot be digested (fCJD)
- once prion transmitted, defective proteins invade brain and are produced in ‘self-sustaining feedback loop’
- transmission: -exposure to brain tissue/ spinal cord tissue -blood transfusions (as infectious prions may accumulate in lymph nodes)
- diagnosis: -electroencephalography (EEG) – measures electrical activity along scalp -> would show characteristic ‘triphasic’ spikes – normally associated with wide range of abnormalities eg. epilepsy
-cerebrospinal fluid analysis
-possible blood tests researched a few years ago however the tests didn’t show early stage prions
- treatment: -2011 experimental treatment given but no stopping of symptoms, only slight slowing (PPS medication which is infused into a brain ventricle)
- research: -Kuru tribe Papua New Guinea – ate dead relatives which meant there was a prion disease spread throughout the tribe, this provided information as to the fact that the incubation period of prion diseases can be 50+ years
- research into similarities with other dementias currently going on at Leeds
Again I was struck with the problem I had no clue where to go this morning, however this time I couldn’t feign innocence of not being told, as I had but veryvery quickly and I didn’t know where it was. So using some pretty sneaky detective skills (google maps and the NHS website) this time I managed to get there without help. ‘There’ was the conference centre for a Morbidity and Mortality meeting – a really joyful start to the morning – which involved talking about what had gone wrong with several cases in the cardiology department. This only took about three hours of painstakingly long, but I can appreciate very important, conversations about who was wrong, who was right, who wasn’t there, and even financing and quota management.
Then I went to the A&E department. As Hammersmith Hospital’s A&E was only heart related issues and it being a Tuesday morning, it was relatively quiet apart from a collapsed lady came in having been brought by an ambulance and I got to see what the first hour-or-so of care is like in a hospital. I also got to see a few patient histories being taken and narrowly avoided witnessing a rectal exam that one of the junior doctors thought it would be hilarious for me to see.
Overall it was an absolutely fantastic week and I’m really lucky to have seen so much of what goes on in hospitals and I feel like it’s really given me more of an insight into what the life of a doctor would be like – and I loved it which is always a good sign!
Feeling like we’d seen pretty much everything that was going on in the hospital, I wasn’t too sure what else we were going to do today. However Dr Malik managed to organise our spending the morning with the radiography department. We watched x-ray after x-ray and after a while I felt as if maybe I could have done the job, but then when looking at the pictures I didn’t notice things that were obviously wrong once I’d had them pointed out to me! For example one man had a ‘dropped metatarsal’ meaning one of his toes was lower than the rest – which I couldn’t see until someone actually circled it on a print-out for me. We also saw some CT scans being done and we saw one lady come in who was really disoriented and her brain scan showed quite a large bleed, this time I did actually realise that the big white spot was a little out of place.
In the afternoon we watched … OPEN HEART SURGERY!!
It was an on-pump CABG (Coronary Artery Bypass Graft) operation where a vein from the leg, usually a saphenous vein, and grafted onto the heart to bypass certain vessels that have become narrowed due to atherosclerosis. It increases blood supply to the heart and is used to treat angina. The chest is literally sawed open to reveal the innards of the person lying on the table swathed in blue sheets and tubes. The heart is stopped and the blood is pumped out of the body and oxygenated by a machine instead then pumped back in – this is cardiopulmonary bypass. Seeing the surgery was possibly one of the coolest moments of my life especially as the surgeons were so relaxed, listening to music and even chatted to us about why we wanted to be doctors as they were suturing!
Got up for what seemed like ridiculously early start to watch the ward round to then be informed that that’s actually what time junior doctors wake up at, on a late-start day, JOY! The ward round entailed following about 20 people round all who were more important and more informed than me on work experience but it shocked me that actually all those people were somehow involved in the care of each of the patients. There were multiple docs, some medical students, some nurses … a real assortment. The variety of cases that we saw was shocking too and the drugs that the docs could reel off on demand was crazy! I also learnt about the different issues associated with the actual stay in hospital, as when you’re there and vulnerable there are multiple infections you can get and lots of people suffer from muscle wasting and then have to spend more time in hospital being rehab-ed.
We went to ECG after lunch, electrocardiography (not to be confused with echocardiography!) and saw loads of tests. I even got to feel up some strangers and actually do something, I put an electrode on someone having an ECG! The electrode is a little sticker that goes in special places along the chest and connects to wires to give the red squiggle line diagrams that everyone is so familiar with from House/ Grey’s Anatomy etc. We watched an ETT (Excercise Tolerance Test) that is similar to the stress echo but this time instead of a drug making your heart beat quickly you actually run on a pre-programmed treadmill that tests how many levels you can complete.
Also on the upside we got to go home at 3!
Today was a pretty easy day! Not too much learning but still saw interesting stuff – perfect! In the morning we shadowed Dr Malik in the cardiac outpatient clinic which was cool but what was really shocking was the sheer amount of over 60s that we saw. I think before this week I was pretty naive to the groups that were most affected (affected, effected … I never know!) by cardiac problems but now I’ve realised that it really is an old persons field. Something that was also crazy was we met a man who technically had been dead for 30mins only then for his mates to realise he wasn’t sleeping off an ache but he was having a heart attack!
In the afternoon Dr Malik took us over to the echo place. Echocardiograms are sonograms of the heart (according to wikipedia) and this doesn’t really mean much to me except that it makes an amazing pic of the heart that actually shows you all the valves and walls and the other stuff that’s jammin’ in there. Did you know though that the heart isn’t actually like straight and flat in your chest with all the chambers nice and perfect, it’s actually on an angle meaning that initially when you’re looking at the echos you get really worried that one side is huuge and the other side isn’t really there?! We also watched a ‘stress echo’ of a woman which basically means they give her a drug to make her heart go fast so that they can see if something is going wrong, luckily for the lady nothing was as the doc said it gets pretty uncomfortable if something is. But apparently in the room opposite the one we were in they found a whole in someones heart!
So yeah, a good and informative day that left me looking forward to tomorrow …
Today I started my work experience.
Realising the night before I had no idea where I was going the next morning it dawned on me that I was actually about to start my work experience with the legendary cardiologist Dr Malik at Hammersmith Hospital. After a rushed email conversation at 11pm to the work experience co-ordinators I found out I was to report to ‘Cath Lab A’ at 9am – ‘what even is a Cath Lab?’ is what I asked myself then but now I am proud to admit that I am fully educated (well kinda fully) about, wait for it, Catheter Laboratory A.
Within 5 mins of arriving I was in scrubs in leads living the dream in the lab. We saw angiogram after angiogram and finally the diagrams started to make sense to us, we could actually tell – so we liked to think – if there was a blocked artery or what was going on! Next up was an angioplasty with a stent. Having heard about stents in biology I was super shocked to find out that they actually existed and that they weren’t just another silly school myth! The stent was put in via a catheter in the femoral artery in the groin and it was amazing to see how the cardiologists masterfully manipulated the wires through the maze of blood vessels.
For lunch we went to the hospital cafe which was pretty damn cool as we were still in scrubs so we got a staff discount! Got asked at least 5 times where the loo was and was complained at for the long wait to see a doctor but who cares … the cashier thought we were doctors #livingthedream
After lunch we went and chilled with some junior doctors in one of the wards. This didn’t really entail much other than sitting around and listening to some junior docs discuss what to do with various patients.
So, day one of work experience down and I’m pleasantly surprised. Feel like I’ve learnt a ton and brain is definitely overloaded but happy to say that the scrubs experience can be ticked off the list already!