NCEPODA Sickle Crisis.shortversion

8/14/2019 NCEPODA Sickle Crisis.shortversion

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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)

Documents

NCEPODA Sickle Crisis.shortversion