Immunisation in

Sickle Cell Disorders

Dr Nick Jackson
Lindsay Randall
Barbara Bailey-Squire
Dr Matt Howard-Jones

Coventry
 A sad story
2001: 25-year old Nigerian man comes to
UK (leaving wife & one child in Africa)
2004: found to be sickle screen positive
before an op.
Diagnosed to have Haemoglobin SC dis.
No history of any sickle problems:
No pain, No eye or hip problems.
Hb 14.5 g/dL. No retinopathy.
Asymptomatic Haemoglobin SC
(2004 )
Immunisation history – vague; ? NIL since
childhood.
Antibody titres:
– Pneumo: 58 U/ml (‘Normal’ adult titre > 20)
– Haemophilus influ b 0.53 mg/L (optim >1.0)
Hib vaccine given during 2005
Job: Security Officer
Annual reviews: 2005, 2006, 2007: well
 The story continued
January 2006: assaulted leaving work:
# humerus, radial N palsy  off work 1 yr

(Discovered during audit)

July 2007: Unwell 2 days  agitated
confused. Diagnosis: ‘sickle cell crisis
with hepato-renal failure due to sepsis’.
CT scan: meningeal enhancement; high
WBC
 Sad Story – the end
Lumbar Puncture: Gram +ve cocci
CSF & blood culture grew Streptococcus
Pneumoniae (aka Pneumococcus)
CXR – Normal (no pneumonia)
Patient deteriorated despite appropriate
antibiotic therapy; renal & hepatic failure,
falling conscious level, DIC. (ITU etc)
Died 3 days after admission
 Lessons from this case
Pneumococcal meningitis/sepsis is a
devastating illness; often fatal even when
diagnosed quickly
Haemoglobin SC disease can be
associated with overwhelming sepsis
(Not just Haemoglobin SS)
Antibody levels are not useful in
determining whether to vaccinate
(Latest UK Guide no longer recommends)
 Audit Immunisation status in
Sickle Cell patients: Why?

New Guidance in UK, Green Book 2006

Perception of poor compliance, despite

reminders and letters from clinic

National screening programme for sickle:

raison d’étre is to protect newborns
 Vaccines
PPV (Pneumococcal Polysaccharide Vaccine;
Pneumovax II); 23 subtypes covered. Only from
age 2 years. (1992)
PCV (Pneumococcal Conjugate Vaccine;
Prevenar); 7 subtypes. From age 2 mths. (2004)
Hib (Haemophilus influenzae b); 2 mths. (1992)
MenC (Meningitis C). From 3 mths. (2001)
Influenza (viral) (annual).
 UK Green Book 2006
Pneumococcus MenC & Hib
Under 2 years 3 x PCV 4 x Hib; 3 x MenC
(UK Routine schedule)
2-5 yrs, 1 x PPV 1 x MenC/Hib
fully vaccinated
2-5 yrs, not 2 x PCV, 2 x MenC/Hib
fully vaccinated then PPV
> 5 yrs, previously 1 x PPV 1 x MenC/Hib
vaccinated
> 5 yrs, not prev 1 x PPV 2 x MenC/Hib
vaccinated
Boosters PPV every 5 yrs MenC every 5 yrs
 Audit Setting & Methods
Coventry 300,000 population
16% non-white (mainly Indian subcon; 3% black)
Neonatal screening since 1998
Formal universal antenatal screening since 2004

Audit carried out: April & May 2008:

– Patients identified from local database
– Immunisation status – written evidence in GP
notes/computer (CNS’s visited all practices)
– Took account of d.o.b. : e.g. PCV only applicable if
born since Jan 2000
Audit Standards = Green Book
Evidence for Compliance:
Pneumococcus: primary course or booster
within last 5 years
Meningitis C: primary course or booster
within last 5 years
Haemophilus influenzae b: if ever had
complete immunisation (as child or adult)
Influenza: immunisation within last year
 Patients (n= 58)
33 adults ; aged 17 – 63 yrs

25 children ; aged 4 mths – 15 yrs

34 HbSS 22 HbSC 1 HbSOArab

1 HbSDPunjab
 Children (n=25) Bacterial
Fully immunised
14 (56%)
(Pneumo, MenC, Hib)

Pneumococcus 18 (72%)

Meningitis C 20 (80%)

Haemophilus influenzae b 20 (80%)

 Adult (n=33) Bacterial
Fully immunised
4 (12%)
(Pneumo, MenC, Hib)

Pneumococcus 7 (21%)

Meningitis C 6 (18%)

Haemophilus influenzae b 5 (15%)

 Influenza immunisation
Adults Children
(n = 33) (n = 25)

Influenza (within last year) 4 (12%) 2 (8%)

Influenza (ever) 6 (18%) 2 (8%)

 Comments
Compliance is poor; worse in adults
Children under 9 yrs – 60% complete
Children over 9 yrs – 40% complete
Young children benefitting from routine UK
immunisation since 2004 to include
Pneumo, MenC and Hib.
Influenza seems to have been forgotten!
 Compared to others?
UK HPA: 1999-2005 survey of ‘at risk
gps’:
– Pneumo immunisation in 15.9% sickle cell pts
(Coventry: overall 43%)
NCEPOD 2004-06:
40 deaths in adult sickle cell pts
– Pneumo 32% (Coventry 21%)
– Hib 23% (Coventry 15%)
– Men C 19% (Coventry 18%)
– Influenza 6% (Coventry 12%)
 Strategies that don’t work
Telling a patient at an OP appointment

Writing letter to GP

Copying letter to patient

Depending on clinic attendance !

 Our ongoing strategy
Haemoglobinopathy Nurse Specialists
– Maintain database of immunisation status of
all known patients in Coventry
– Liaise with GP’s (building close links)
– Help to bring the patient to the GP
– Negotiating if they can given the vaccines in
patient’s home
– Raise awareness via local patient groups
 May - Nov 2008 : 26 adults
9 had left the area

7 non-responsive to attempts to contact to

discuss

10 seen (2 currently pregnant)

– 7 letters to GP
– 7 received PPV, Hib and MenC vaccines
– 7 received Influenza vaccine
May - Nov 2008 : 27 children
4 had left the area

3 (parents) non-responsive to attempts to

contact to discuss

20 seen & discussed immunisation:

– 10 letters to GP
– 7 received PPV, Hib and MenC vaccines
– 7 received Influenza vaccine
– 1 has changed diagnosis !
 Conclusions
Encapsulated organisms (esp Pneumo) are
killers for sickle cell pts (and SplenX thal pts!)
Routine UK children immunisation covers the
appropriate organisms (except influenza)
Many older children (?esp those coming in from
abroad) and adults are poorly protected
CNS’s have a key role in keeping records,
liaising with GP’s, and chasing, ?in giving?
National database may have a role in tracking