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Rationale for the study
Changing pattern of care in the UKDeveloping protocols
New interventions
High birth incidence Latest newborn screening figures predict between 1:2000
and 1:2500
High rate of admissions 13,000 finished episodes in London 2005-06
Quality of autopsies
No previous national audits of deaths with
casenote review
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Study overview
All haemoglobinopathy deaths in the UK over a
2 year period
Data collection period 1st January 2005 31st December 2006
ICD-10 codes D56 (Thalassaemia) and D57
(Sickle Cell Disease) Anywhere in diagnosis or cause of death
Adults and children
Hospital and community deaths
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Inclusion criteria
Patients with the following haemoglobinopathies
included:
Sickle cell anaemia (HbSS) Sickle haemoglobin C (HbSC)
Sickle haemoglobin D (HbSD)
Sickle -thalassaemia (HbS Thal)
-thalassaemia major
-thalassaemia intermedia
Exclusions Patients with traits and no clinical
symptoms
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Study aims
Primary aim: to collect information on the care
received by haemoglobinopathy patients who die in
order to identify remediable factors in the care ofthese patients.
Issues addressed included: The organisation of outpatient management
Experience in managing haemoglobinopathies Pain management Mortality associated with surgery Patterns of clinical pathology The availability and use of local guidelines and protocols
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Data collection
Semi structured questionnaire 3 sections The patients regular management
Final clinical episode Organisation of care
Last 6 months medical notes GP/specialist centre notes
Hospital casenotes
Autopsy reports (if available)
Guidelines and pathways local and national
Expert opinion from advisors (assessment form)
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Number of cases identified
Denominator: 47 questionnaires; 55 casenotes
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Thalassaemia and sickle cell traits
Key finding: A disproportionately large number of cases with
thalassaemia trait and sickle cell trait were reported to NCEPOD
when the trait was not relevant.
Recommendation: In our multi-racial society, it is essential that
all doctors should have a basic understanding of thalassaemia
and sickle cell trait. (General Medical Council)
Recommendation: Sickle cell trait and thalassaemia trait shouldrarely be included on the death certificate; and if included this
should only be after review by an individual who has experience in
haemoglobinopathies. (Pathologists)
However, sickle cell trait is not always benign
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Sickle cell trait case study 1
A young patient with sickle cell trait presented with
haematuria and other kidney problems. Investigations
showed that they had metastatic renal medullarycarcinoma. The patient subsequently died.
The advisors were of the view that this rare type of renal
carcinoma is virtually restricted to those with the sickle
gene, particularly HbAS, HbSC and occasionally HbSS.
The prognosis for this carcinoma is very poor, and survival
is less than one year from diagnosis.
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Method of identification
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31/55 patients male
6 paediatric cases
Age range 1 to 77 years
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Age of patients at death
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Place of death
Hospital deaths most common 41/55 died in hospital
14/55 in community; 3 within 14 days of discharge Of these 3, cause of death unknown in 2
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Patterns of death - overview
Thalassaemia patients (n = 7)
Adult sickle cell disease patients (n = 40) HbSC patient subset (n = 10)
Paediatric sickle cell disease patients (n = 6) later in presentation with paediatric management
Sickle cell trait patients (n = 2) not included in
this section on main patterns of death
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Thalassaemia patterns of death
7 patients with thalassaemia died 3 with thalassaemia intermedia (ages 39-58)
4 with thalassaemia major (ages 19-32)
2 died of complications of thalassaemia 1 with cardiomyopathy
1 with mucormycosis (co-morbidity diabetes)
Other 5 patients died of non-related causes 2 died of cirrhosis
1 of carcinoma of the lung
1 with possible sepsis
1 with no documented cause of death
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HbSC adult patient subset
10 patients with age range 26-74 years
Only 2 had causes of death attributable to sicklecell disease Intracerebral haemorrhage
pulmonary embolism
5 patients died of unrelated causes
1 each with brain tumour, diabetes and advanced HIVdisease 2 patients with severe systemic lupus erythematosus
co-incidental/pathogenic link?
3 with unknown cause of death
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Vaccination status
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Vaccinations
Key finding: The clinicians managing the patients in this study
only reported 15/47 as having received pneumococcal vaccine.
Recommendation: There needs to be clear recording of
vaccination status by default; liaison between primary and
secondary care is needed. (Primary and Secondary Care Trusts)
Recommendation: As a minimum, the Department of Health
guidance regarding vaccination and prophylactic antibiotics should
be followed in order to prevent sepsis from hyposplenism.
(Primary Care Trusts)
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Final clinical management
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Final clinical management
NCEPOD looked at the following aspects of management
in the final clinical episode:
Stroke Acute chest syndrome Pulmonary embolism & hypertension Sepsis Iron overload and cardiac deathRenal failure
When sickle is not the main problem Surgery and peri-operative care PregnancyPaediatric patients Nursing care Other issues protocols/education
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Stroke
Stroke was the commonest complication, leading to death
in 9 patients
All the formally diagnosed cases in adults werehaemorrhagic May have been some ischaemic cases, but this was not
possible to determine
No guidelines for the prevention of stroke in adults at
present, as it is less tractable, but there are for children
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Acute chest syndrome (ACS)
2 cases where ACS was listed as primary cause of death
3 further cases where it was likely contributor or part of
multi-organ failure
No documented cases in children, although a recognised
cause of death in paediatric patients
Recommendation:Acute chest syndrome is a major
cause of morbidity and mortality in patients with sickle celldisease. Management of patients with this complication
should be according to local protocols and early advice
from specialists in essential. (Primary and Secondary Care
Trusts)
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Pulmonary embolism & hypertension
2 patients died of massive pulmonary embolism Both diagnosed at autopsy
Advisors commented on potential difficulties associatedwith diagnosis
Especially chronic progressive lung disease
Recommendation:Chronic sickle chest disease is an
expanding, complicated area and requires more careful
correlation of pre-mortem clinical, physiological and imagingdata with autopsy pathology. (Clinicians and Pathologists)
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Renal failure
People with sickle cell disease are known to be particularly
prone to developing kidney failure
May be due to damage to: the renal glomerulus by the sickle process sickling in the medullary vessels papillary necrosis infection the use of non-steroidal anti-inflammatory drugs
No one dying in hospital was recorded as having renalcomplications of sickle cell disease as a cause of death
one patient with chronic disease allowed to die at home one patient in which renal failure potentially overlooked
Care associated with examples of very best and very
worst care
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Renal failure case study 12
Young patient admitted with breathlessness, anaemia and urea of
27 mmol/L.
Comment in notes that serum creatinine should be measuredbefore decision made re: renal failure
no evidence that this was followed up
Breathlessness diagnosed as ACS review by chest physician doubted this; suggested fluid
overload again, no follow up of renal function
A little later, patient died of cardiac arrest blood gas sample potassium result led to a diagnosis of
hyperkalaemic cardiac arrest
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Case study 12 advisors opinion
The advisors considered this to be an unexpected death.
There was a review of the case, but no-one appeared to
have noted the initial high blood urea resulted.
It was evident that the patient has had renal failure on
admission which was not reconsidered.
The focus appeared to have been on the chest problems
and adequate attention was not paid to the completepicture.
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When sickle is not the main problem
Key finding: Patients with sickle cell disease can have
other conditions which can be misattributed to sickle cell
disease.
Recommendation: All staff should be aware that people
with sickle cell disease re subject to the diseases that other
patients suffer from as well. If there is uncertainty as to
whether the problem is sickle cell related, advice should besought from an experienced clinician. (Primary and
Secondary Care Trusts)
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Surgery and peri-operative care
9/41 patients underwent surgery
Seniority and experience of surgeon and anaesthetist considered to be sufficiently experienced in 4 cases insufficient data to judge in remaining 5 cases
7 patients experienced peri-operative complications considered to be avoidable in 4 cases
worsening liver failure and ascites, massive cerebral
infarct, massive bleed and post-operative sepsis
Key finding: There were fewer surgical cases submitted
than were anticipated. NCEPOD is also aware of the
ongoing study of pre-operative blood transfusion.
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Organisation of care
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Seeking advice case study 20
A specialist registrar made the decision to stop the warfarin
in a young patient with a history of pulmonary embolism
who was undergoing eye surgery for cataract. There wasno consideration given to starting heparin post operatively.
The patient developed a post operative pulmonary embolus
and died.
The advisors believed that there should have been a
haematology consultant involved in the complicateddecisions around the patients anticoagulation therapy.
There ought to have been a way around this problem of
prophylaxis.
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Clinicians - recommendations
Recommendation: Patients with sickle cell disease or
beta thalassaemia major should be managed by, or have
access to, clinicians with experience of haemoglobinopathymanagement. (Primary and Secondary Care Trusts)
Recommendation: All haemoglobinopathy patients
should have a named specialist, ideally a haematologist,
responsible for their care. The haematologist must have an
appropriate level of expertise to care for the patient orshould make links with appropriate experts. (Primary and
Secondary Care Trusts)
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Available guidelines
47 questionnaires were returned, representing 26 different sites
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Death certification and autopsies
NCEPOD reviewed death certification and autopsy
diagnoses.
The following are addressed in this presentation: Advisors agreement with cause of death Incorrect death certification Autopsies
number of autopsies
example of an excellent autopsy example of the failure to perform histology
Key findings and recommendations
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Death certification
The cause of death was determined from the casenotes or the
clinician questionnaire.
Of the 43 cases where the cause of death could be determined,the advisors considered 22 to be correct
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Incorrect death certification
Reasons for incorrect death certification included: Failure to include sickle cell disease on the death certificate Incorrect ordering of contributing factors
Failure to include operation details
In the following cases, there was a misunderstanding of
the correct way to complete a death certificate:1a Sickle cell disease
1b Severe haemolytic anaemia
1c Cardiorespiratory arrest
And this was not restricted to clinicians, a pathologist
wrote: 1a Cardiomegaly1b Sickle cell disease
2 Hepatomegaly
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Autopsies
21/55 patients had an autopsy of which 20 were coronial
In 5 cases where no autopsy was performed, the advisors
believed that an autopsy was necessary to confirm thecause of death
15 autopsy reports were returned to NCEPOD
The overall standard of autopsies was poor, although
there were some excellent reports (case study 21) Of the 13 adult autopsy reports, the advisors:
Agreed with the autopsy diagnosis in 4 cases Disagreed with the autopsy diagnosis in 3 cases
Could not make a definitive decision in 6 cases
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Excellent autopsy case study 21
A young adult suffered a pulmonary embolism secondary to
deep vein thrombosis. Full histology was undertaken which
documented the bone necrosis present and the changes ofpulmonary hypertension, both significant problems clinically.
In addition, toxicology was undertaken as the death
occurred soon after administration of diamorphine. A
therapeutic level of diamorphine, in keeping with the
administered dose, was confirmed.
The advisors reported that clinico-pathological correlation
went on to describe that deep vein thromboses and
pulmonary embolisation were not common problems in
sickle cell disease patients.
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Histology case study 24
A middle-aged patient was admitted with painful sickle crisis and
was being treated as per the hospital protocol. All observations
were normal. The patient suffered an unexpected cardiac arrest.
On morphological appearances of the kidney alone, with noconfirmatory histology, the pathologist gave the cause of death as
renal failure due to papillary necrosis. The bladder was full of
urine, and throughout the admission the renal function was
measured as normal.
It was the opinion of the advisors that the patient could not have
died from renal failure secondary to papillary necrosis with
investigations showing normal electrolytes and renal function only
a few hours prior to death. The real cause of death may have been
acute chest syndrome.
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Specialist autopsy pathology
Pathologists and clinicians have problems in correctly
correlating the sequence of events that lead to death
Royal College of Pathologists guidelines (2005) to aidprocess
Key finding: Deaths in sickle cell disease patients were
not well evaluated and depicted.
Recommendation: Cause of death in sickle cell diseasepatients must be better evaluated, whether by clinicians
reviewing the records and writing a death certificate or by
pathologist performing an autopsy. Clinico-pathological
correlation is critical in this complex disease. (Clinicians and
Pathologists)
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Summary and comment
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Summary and comment
Throughout this presentation, the following have been
addressed:
Monitoring patients Clinical networks Development of national guidelines Pain control Autopsy evaluation and causes of death
and..
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National database
In nearly half of cases, the actual cause of death was
debatable or unknown
If this small series is representative, it amounts to a wake-up call to the haemoglobinopathy clinical community: less is
known about severe complications than previously thought
Prompts conclusion that a national database is needed
Recommendation: A national database of patients withhaemoglobinopathies should be developed and maintained,
to include standardised information on death, for regular
audit purposes. (Department of Health)