Title of Guideline (must include the word “Guideline” (not
protocol, policy, procedure etc)
Contact Name and Job Title (author)
Guideline for Adults with Sickle Cell Disease:
Chronic complications
Directorate & Speciality
Dr Sarah Marie Donohue
Consultant Haematologist
Clinical Haematology
Date of submission
August 2015
Date on which guideline must be reviewed (this should be one to
three years)
Explicit definition of patient group to which it applies (e.g.
inclusion and exclusion criteria, diagnosis)
Version
Abstract
November 2017
Key Words
Sickle cell, avascular necrosis, annual review,
renal, priapism, retinopathy, neurological, leg
ulcers
Statement of the evidence base of the guideline – has the
guideline been peer reviewed by colleagues?
Evidence base: (1-6)
1
NICE Guidance, Royal College Guideline, SIGN
(please state which source).
2a
meta analysis of randomised controlled trials
2b
at least one randomised controlled trial
3a
at least one well-designed controlled study without
randomisation
3b
at least one other type of well-designed quasiexperimental study
4
well –designed non-experimental descriptive studies
(ie comparative / correlation and case studies)
5
expert committee reports or opinions and / or clinical
experiences of respected authorities
6
recommended best practise based on the clinical
experience of the guideline developer
Consultation Process
Target audience
This guideline has been registered with the trust. However,
clinical guidelines are guidelines only. The interpretation
and application of clinical guidelines will remain the
responsibility of the individual clinician. If in doubt contact a
senior colleague or expert. Caution is advised when using
guidelines after the review date.
Patients diagnosed with sickle cell disease
This guideline describes the chronic
complications of sickle cell disease and how
best to manage them
Introduction
As survival of patients with sickle cell disease improves, the
management of chronic complications remains a challenge and requires
a robust multidisciplinary team approach.
Table 1 Chronic complications of SCD
Organ system
Description
Musculoskeletal
Chronic sickle pain, avascular necrosis (AVN) of the long bones, osteoporosis
/ osteomalacia
Chronic renal failure, haematuria, hyposthenuria, priapism, erectile
dysfunction
Sickle eye disease
Cardiac disease and heart failure
Pulmonary hypertension and chronic sickle lung
Ischaemic or haemorrhagic stroke, headaches
Leg ulcers
Genitourinary
Ophthalmology
Cardiology
Respiratory
Neurology
Skin
This document briefly describes management of chronic complications
of:
a) end organ damage related to sickling events
b) end organ damage relating to the effects of iron overload in
transfused patients
c) end organ damage relating to toxicity of chelation therapy
The appendix includes the annual review documents, and schedule of
tests, which document the process of identifying end organ damage and
monitoring effectiveness of management strategies.
Contents
Introduction ............................................................................................. 2
Contents ................................................................................................. 2
1 Annual Review .................................................................................. 3
2 National Haemoglobinopathy Register (NHR) ................................... 3
3 Renal disease ................................................................................... 4
3.1 Routine clinical review ................................................................. 4
3.2 Specific renal conditions .............................................................. 5
3.2.1 Sickle cell nephropathy .......................................................... 5
3.2.2 Hyposthenuria ........................................................................ 5
3.2.3 Urinary tract infection ............................................................. 5
3.2.4 Renal papillary necrosis ......................................................... 6
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 2 of 16
3.2.5 Renal medullary carcinoma ................................................... 6
4 Priapism ............................................................................................ 6
4.1 Background ................................................................................. 6
4.2 Management of acute fulminant priapism.................................... 6
4.3 Management of stuttering priapism ............................................. 7
4.4 Nottingham contacts .................................................................... 7
4.5 Long term problems with potency................................................ 8
5 Orthopaedic / metabolic bone problems ............................................ 8
5.1 Osteoporosis / osteomalacia ....................................................... 8
6
7
8
9
Arthropathy / avascular necrosis ....................................................... 9
Retinopathy ....................................................................................... 9
Chronic respiratory disease............................................................... 9
Cardiology ....................................................................................... 10
9.1 Pulmonary hypertension and other cardiac complications (heart
failure / arrhythmias) .......................................................................... 10
9.2 Other cardiac problems ............................................................. 10
10 Neurological complications.............................................................. 11
11 Chronic Pain .................................................................................... 11
11.1 Choices of analgesia ................................................................. 12
11.2 Non-pharmacological Adjuncts .................................................. 12
11.3 Onward referral if pain relief problematic in community ............ 12
12 Complications of transfusion overload and chelation therapy ......... 13
12.1 Endocrinopathy ......................................................................... 13
12.2 Audiometry ................................................................................ 13
13 Chronic leg ulcers ........................................................................... 14
14 References ...................................................................................... 14
Appendix 1 – Contacts.......................................................................... 15
1 Annual Review
All Patients with Sickle Cell Disease are offered an annual review in the
Hb Disorders Clinic.
2 National Haemoglobinopathy Register (NHR)
All patients are offered registration on the National Haemoglobinopathy
Register.
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 3 of 16
Once patient consent is obtained, data regarding demographics,
diagnosis and treatment will be submitted to the NHR. In a timely nature
their annual review and significant adverse events will also be submitted
to the NHR. Full details of the NHR Dataset including adverse events
and annual review can be found on the NHR website:
http://www.nhr.nhs.uk/information.aspx.
A local database of all haemoglobinopathy patients is also maintained to
aid reporting to the NHR and clinical commissioning groups. For details
contact the Data Manager.
3 Renal disease
3.1 Routine clinical review
All patients with Sickle Cell Disease will be offered a urine sample
screen at each outpatient appointment, (minimum frequency, annual
testing), as well as a blood pressure check. These are to detect
proteinuria as an indicator of renal function, nitrates indicating urinary
tract infection, haematuria and glycosuria. Symptoms of enuresis, and
nocturia and priapism will be reviewed at least annually in patients with
sickle cell disease.
Where urine dip test shows + protein or more, urine will be sent for
ACR/PCR.
Where PCR is > 70mg/mmol in normotensive patients or
>30mg/mmol in hypertensive patients, treatments with ACE
inhibitors should be commenced.
Lisinopril 2.5mg daily increasing to 5mg daily if tolerated (no
hypotension, renal function and potassium levels stable)
Careful control of hypertension is essential to limit renal impairment and
other complications in the long term. The target blood pressure with
patients with proteinuria should be 130/80 mmHg.
Chronic long-term use of non-steroidal anti-inflammatory drugs should
be avoided.
Other medication (e.g. deferasirox) may also contribute to renal
impairment, and appropriate dose adjustment may be needed.
The outpatient clinic provides an opportunity to reinforce the
importance of continuous, regular hydration to avoid precipitation
of crises.
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 4 of 16
Prompt referral to the renal clinic is indicated if there is significant
deterioration in the creatinine level, or eGFR (eGFR <90 ml/min).
Consideration of the use of erythropoietin in patients with SCD will be
made in consultation with the renal physicians. Referral for Urology
review is indicated in the event of recurrent urinary tract infections,
pyelonephritis, haematuria, or complications of priapism
3.2 Specific renal conditions
3.2.1 Sickle cell nephropathy
Affects 5-18% patients
Characterised by insidious onset; usually detected by regular monitoring
as described in Routine clinical review above. Where significant
proteinuria is present, ACE inhibitors should be used in line with NICE
guidance, as described above.
Patients may be treated with Erythropoietin in consultation with renal
physicians, but higher doses may be required. Transfusion may be
required and if on a regular basis, iron chelation therapy will be required
or regular exchange transfusions.
Where renal transplantation is likely, a perioperative plan should be
available in the notes for immediate use (including transfusion plan,
aiming for HbS<25%). Long term hydroxycarbamide or exchange
transfusion should be considered in these patients
3.2.2 Hyposthenuria
Hyposthenuria is associated with nocturia and enuresis. Although it is
more common in childhood a history of enuresis should prompt further
investigation (e.g. overnight O2 saturation).
Both enuresis and nocturia are associated with an increased risk of
dehydration, which can precipitate a sickle crisis. Continued
reinforcement of need for hydration should be given at every
opportunity. Patients should aim for fluid intake of 3-4 L/ day.
3.2.3 Urinary tract infection
Patients with SCD are at increased risk of UTIs due to hyposplenism.
Asymptomatic UTI may be associated with sickle cell crisis. Renal tract
sepsis should be considered in patients with SCD and fever. Urine dip
test at each OPD, with MSU where screen is positive for nitrites or WBC,
is advisable.
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 5 of 16
3.2.4 Renal papillary necrosis
It is due to medullary infarction and it can affect both SCD patients and
carriers. It presents with microscopic or frank haematuria. Treatment is
supportive with maintenance of high urinary flow and blood transfusion if
necessary.
3.2.5 Renal medullary carcinoma
Although extremely rare, it is a rapidly fatal malignancy, as usually
already metastasised at presentation, associated with sickle gene
(carriers as well as SCD). Haematuria should alert for prompt
investigation with renal USS or CT/MRI. Other symptoms include flank
pain, weight loss, abdominal pain and fever.
4 Priapism
4.1 Background
Priapism is defined as a persistent erection lasting more than four hours
and is a medical emergency requiring hospital assessment
Priapism in sickle cell can present at a young age (from childhood) and
is caused by vaso-occlusion caused by sickling in the penis. Lifetime risk
29-42%. Is common in young men with sickle disease but the exact
burden is unclear as research and self-reporting are both lacking. It is
undoubtedly painful and can result in penile damage and impotence
which occurs as a consequence of prolonged attacks.
There are various types of priapism: ischaemic, non-ischaemic and
stuttering (recurrent, intermittent).
Stuttering priapism can occur as episodes lasting less than 3 hours,
often occurring in the morning and recurrent painful can occur with either
on its own or with sickling elsewhere in the body. It can be relieved by
exercise (e.g. running upstairs), a warm baths, but sometimes is so
frequent that medication may be required to lessen or reduce the
episodes.
It is important to identify those affected, usually by direct questioning in
routine clinic visits as it is often a difficult subject for patients to address.
4.2 Management of acute fulminant priapism
 Patients should be assessed as with any other acute sickle
presentation (brief history with particular reference to precipitating
factors, duration, and attempts to relieve; examination)
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 6 of 16
 Adequate pain relief should be given
 I/V fluids
 Blood samples including group and save
 Etilefrine 50mg po should be administered
 Urology opinion should be sought and surgical decompression
may be necessary
 In the longer term long term therapy, hydroxycarbamide,
ephedrine, etilefrine or sildenafil but good evidence for efficacy is
lacking
4.3 Management of stuttering priapism
 Self-help measures are advised (mild exercise, hot baths,
relaxation techniques)
 Medication with ephedrine or etilefrine can be used
 Role of sildenafil and hydroxycarbamide are uncertain but may be
considered
Drugs which may be useful
Drug
Dose
Side effects/monitoring
Ephedrine
15-60 mg po usually at night
High blood pressure – check 2 weekly,
tachycardia
Etilefrine
25mg po at night, if persistent
problems add extra dose of 25mg
around 4-5 pm, maximum daily dose
High blood pressure
Sildenafil
50 mg daily
Headache, diarrhea, dyspepsia, flushing
( Viagra)
Tadalafil
Possible increase in sickle cell crises,
priapism
5mg daily
Similar to sildenafil
(Cialis)
4.4 Nottingham contacts
 Urology SpR on call. Monday to Friday 9:00-17:00
 Further advice could be available from colleagues in haematology
(Dr Donohue x56704 or Haem SpR) and urology (Mr A Bazo
Associate Specialist in Urology Ext 58303)
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 7 of 16
4.5 Long term problems with potency
 Urology referral essential: Mr. Bazo. Associate Specialist in
Urology. Contact Secretary Ext 58303
 May require onward referral to Mr. Summerton at Leicester Royal
as guided by Mr. Bazo / urology team
5 Orthopaedic / metabolic bone problems
5.1 Osteoporosis / osteomalacia
Patients with Hb disorders are particularly prone to metabolic bone
disease. Factors such as diet, sunlight exposure, sex hormone levels
and hypoparathyroidism and expansion of the marrow cavity will all
contribute.
Serum calcium levels must be measured regularly in all patients at least
annually. If below normal range, Vitamin D level must be measured and
replaced. Following correction with supplementation, vitamin D levels
should be repeated, but with a minimum interval of 1 year in view of slow
response to treatments. High doses of Vitamin D may be necessary for
patients with hypoparathyroidism.
Maintenance dose: Adcal D3 ii daily orally or other preparations aiming
for a dose of 1000 units/day.
Patients with severe deficiency may require higher doses.
Alphacalcidol or 3 monthly vitamin D injections may be required in
patients with endocrinopathy, and if standard replacement is suboptimal,
further advice should be obtained from the metabolic bone clinic.
Dexa Scans are appropriate every 2-5 years depending on individual
circumstances. Advocate regular exercise and balanced diet including
dairy products, fish etc where possible. Advice against smoking and
excess alcohol consumption should be given when patients are
reviewed in clinic.
Bisphosphonates should be considered and used with caution, and
should be given alongside Calcium and Vitamin D supplementation as
appropriate.
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 8 of 16
6 Arthropathy / avascular necrosis
Patients with SCD may be subject to avascular necrosis or degenerative
joint disease. Atypical pain, limited movement of a joint must be
investigated with plain X-ray. If changes are apparent on X-ray, referral
to orthopaedic specialist without further imaging is appropriate. If no
changes are reported on X-ray, the MRI may be considered to exclude
early avascular necrosis. Management plan should be defined in
consultation with relevant orthopaedic surgeon. If any intervention is
advised, a perioperative plan must be provided by the
haemoglobinopathy team. Early involvement of the physiotherapy and
the occupational therapy team is essential.
7 Retinopathy
Annual ophthalmology review is advised for all patients with SCD, to
exclude or treat sickle retinopathy. Retinopathy can be classified as nonproliferative (NPR) and proliferative (PR). The distinguishing element is
neovascularisation. Care should be taken, as NPR can be asymptomatic
in early stages. PR can be complicated by vitreous haemorrhage and
retinal detachment.
All patients on regular chelation therapy should attend for yearly
ophthalmology review to exclude toxicity. Urgent ophthalmology referral
is required if any acute change in vision occurs.
8 Chronic respiratory disease
Oxygen saturation should be measured at least annually in the
outpatient’s clinic. Where pO2 is repeatedly low (<95%), hypoxia should
be confirmed by obtaining arterial blood gases. In these cases, and
where individuals have had recurrent chest crises, frequent admissions,
or symptoms of shortness of breath, poor exercise tolerance etc., then
lung function tests should be requested, clearly stating diagnosis and
usual Hb on the request form. Prompt antibiotic treatment of infection
and cessation of smoking should be encouraged. High resolution CT
scan may be more helpful than CXR and 6-minute walk test can be used
as a marker of functional ability.
Obstructive causes and sleep disorders should be considered and
investigated. Obstructive sleep apnoea is usually due to tonsillar
hypertrophy or other causes of deep disordered breathing. It can
precipitate painful episodes and cause neurological events. If concerns
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 9 of 16
the patient should be referred to the OSA unit for further assessment
(i.e. Epworth score, overnight sleep studies)
9 Cardiology
9.1 Pulmonary hypertension and other cardiac complications
(heart failure / arrhythmias)
Affects about 5-30% of SCD patients
Chronic haemolysis releases free haemoglobin which leads to NO
deficiency causing acute and chronic pulmonary vasoconstriction.
Echocardiography should be undertaken regularly in all patients: interval
depends on clinical condition, severity of disease and symptoms. Every
two years in asymptomatic individuals with moderate sickle disorder and
no symptoms. Every year in individuals with complicated history e.g.
recurrent sickle crises, or risk factors for pulmonary hypertension.
1) Clearly mark requests for echocardiography with ‘sickle cell
patient, to exclude pulmonary hypertension’
2) Echocardiography department to do echocardiogram, measure TR
if present and add to RAP (right atrial pressure)
3) The sum of TR and RAP gives RVSP this equates to PA systolic
pressure in the absence of pulmonary valve disease – values
above 30 mmHg are considered abnormal – should be discussed
with cardiology before onward referral to Sheffield Pulmonary
hypertension unit.
Values up to 30mm of Hg is considered normal – if no other
clinical signs or symptoms repeat in 6 months
4) Asymptomatic patients with RVSP values less than 25 mmHg
should be rescreened every 2 years
5) Echocardiographers to comment on pulmonary hypertension when
this is mentioned in request
Onward referral is to Pulmonary Hypertension Unit at Sheffield
Hallamshire Hospital after discussion with Cardiology.
9.2 Other cardiac problems
Include left sided heart disease or diastolic dysfunction. ACE inhibitors,
diuretics and beta-blockers may be used as recommended by
experienced cardiologist, under joint care with the haematologist.
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 10 of 16
10 Neurological complications
Neurological complications such as stroke or TIA should be treated as a
medical emergency. Following acute management and referral to Stroke
team, LGH, or City Hospital Nottingham the Haemoglobinopathy team
must provide a management plan for long term stroke prevention,
including transfusion plan (consideration of exchange transfusion),
antiplatelet or anticoagulation therapy and intensification of surveillance
(BP, renal function) as necessary.
Cognitive problems may be identified by patient, clinician or psychology
team. Annual review will include psychosocial review, offer of
assessment, and referral on for further cognitive investigation by
psychology team as appropriate.
(Leicester Dr Joanne Herdman, Nottingham Dr Jayne Mills)
Patients with new onset neurological problems will be reviewed at the
regional MDT in consultation with an experienced neurologist.
11 Chronic Pain
Pain will be assessed at each clinic. Where there are issues of chronic
pain, pain relief may be offered using WHO treatment ladder.
Psychology support team in clinic will routinely enquire about pain
control and discuss strategies for pain control. Where these measures
fail, referral may be made to the Pain Team. Patients with chronic pain
not controlled by mild opiate analgesia should also be discussed at the
regional MDT.
Chronic pain, either intermittent due to painful sickle cell crises or due to
a complication of sickle cell disease (e.g. avascular necrosis of hip),
occurs in many sickle cell patients. This should be included in every
sickle cell review undertaken. Unexpected or unusual pain should not
be attributed to sickle cell disease but other possibilities should be
sought.
Close liaison with other prescribers (e.g. GP) may be necessary to
check doses and frequency of prescription.
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 11 of 16
11.1 Choices of analgesia
Table 2: Choices of analgesia include
Medicine
Paracetamol
NSAID
Dose range
1g orally qds
Dihydrocodeine
30mg orally every 4-6 hours
In young fit people may need doses
up to 60-90mg qds.
Tramadol
50-100mg orally NOT more often
than 4 hourly (usually not more than
400mg required in 24 hours)
Fentanyl patch
25-100µg/72 hours
Start at lowest dose, assess at 24
hours (oral morphine sulphate
equivalent is <135mg in 24 hours to
25micrograms patch)
Morphine salts
Gabapentin
Also available as lozenge
5-20mg orally every 4 hours
300mg orally on day 1, increasing
every 24 hours if required to max
dose of 1.8g (in 3 divided doses)
Comment
Care with renal impairment. If regular
use, may need PPI cover.
Be aware that this drug needs to
be metabolised to have full effect
and ~25% of the Afro-Caribbean
population don't have the gene.
Better as a regular analgesic as
superior for neuropathic type pains.
Care if patient already on tri-cyclic
antidepressant (added serotonin
effect) and not with MAOIs.
For constant level of pain only. No
use in variable pain. Needs good
skin blood flow to be effective.
Plasma doses of fentanyl reliant on
skin blood flow. Pyrexia will cause
greater uptake.
For constant level of pain only. No
use in variable pain unless
supplemented with other analgesics.
For chronic neuropathic pain. No
good for acute flare-ups. Reduce
dose in renal impairment as renally
excreted.
11.2 Non-pharmacological Adjuncts




Local heat
Massage
Aromatherapy
TENS
11.3 Onward referral if pain relief problematic in community
Applicable to
Check
If still problems
Referral in hospital
service
Those with chronic pain related to haemoglobinopathy
Use of analgesia appropriate
Consider referral to chronic pain team in the community for further
advice, use of alternative therapies
Ask GP to refer to Chronic Pain team
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 12 of 16
12 Complications of transfusion overload and chelation therapy
Patients who are on transfusion programmes (top-up or exchange
transfusion) are at risk of iron overload. Ferritin and LFTs should be
checked every 3 months. Iron chelation should be considered in all
patients on a regular transfusion regimen who have received at least 20
top-up transfusion episodes or have a liver iron concentration of >7mg/g
dry weight. Monitoring with T2* MRI is required to assess cardiac and
liver iron overload.
Cardiac T2* MRI:
- If T2* > 20 msec every 2 years
- If T2* 10-20 msec yearly
- If T2* < 10 msec every 6 months
Please see separate chelation guideline for more detail
12.1 Endocrinopathy
Low sex hormone levels may be a feature of iron overload and should
be corrected as part of the regular endocrine review. Endocrine failure is
a common complication of poor chelation, and screening will be carried
out at regular intervals and always as part of the annual review of
patients on transfusion programmes.
 Thyroid function: TSH every three months
 Reproduction: menstrual and sexual history annually as
appropriate
 FSH, LH annually for females; Testosterone at least annually for
males
 GTT annually. If this is not carried out, then fructosamine levels
and random blood glucose levels when attending for transfusions.
 Calcium and Vitamin D – consider hypoparathyroidism in
refractory cases: may require higher Vitamin D doses
Patients with established endocrine dysfunction will be reviewed
regularly as appropriate in the endocrinology clinic.
12.2 Audiometry
Must be offered annually to all patients receiving chelation therapy to
exclude toxicity (high tone deafness). Referral is made in Leicester by
letter to the Hearing Aid Department, in Nottingham to Karen Dyer in
Audiometry, QMC.
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 13 of 16
Possible complications of chelation therapy should be monitored
regularly. Regular monitoring of the therapeutic index may help to avoid
toxicity. Further information is available on the iron chelation guideline.
13 Chronic leg ulcers
Affects predominantly males with HbSS disease. The mechanism of
ulcer formation is poorly understood but they usually form around the
medial or lateral malleoli. Early intervention is required to prevent
progression to chronic ulceration which is difficult to treat. Elevation,
modern wound dressings and treatment of infections is essential. In
difficult cases, review by the plastic team (skin grafting) might be
needed.
14 References
- Standards for the clinical care of adult’s with sickle cell disease in
the UK, 2008. London: Sickle Cell Society
(http://sct.screening.nhs.uk/getdata.php?id=10991)
- NICE, 2008. Chronic kidney disease: early identification and
management of chronic kidney disease in adults in primary and
secondary care. London: NICE. http://www.nice.org.uk/CG73
- Iron chelation guidelines (hyperlink)
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 14 of 16
Appendix 1 – Contacts
Speciality
Contact ( deputy)
Contact details
Ophthalmology
Miss Lim, QMC
x68485
ENT
Mr Marshall, QMC
x61224
Audiology: Karen Dyer
Respiratory
Prof Brittain, City
Contact via overnight sleep
studies
Prof Hubbard / Dr Baldwin City
78331710 / x57462
Renal
Dr Bebb, City
x56297
Bone disease
Dr Prinsloo, City
x55079
Cardiology
Dr Mathew
Neurology
Dr O’Donoghue City / QMC
x57546 / x65324
Gastroenterology
Dr Teahon, City
x57348
Hepatology
Dr Ryder QMC
x70441
Endocrinologist
Dr Page City
x54161
Obstetrics
Miss Moore + Dr Myers (City
Thurs pm clinic
x58379
Miss Ramsy/Miss Gribbin/Miss
Rutherford/ Dr Donohue (QMC
Tues am clinic)
Orthopaedic surgeon
Mr Manktelow (hips)
x59742
Prof Wallace (shoulders)
x56885
General surgery
Urology
x61924
Via on call
Mr Bazo, City
x55830
Useful Telephone numbers
Dr Forman
x67251
Dr Stokley
x62005
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 15 of 16
Doreen Richards, Haemoglobinopathy
specialist nurse ( Mon Weds pm and Thurs)
x53446 (bleep 780 7972)
Dr Donohue
x56704 (secretary)
[email protected]
x59381
Jayne Mills Psychology
x56448
Sickle cell and Thalassaemia service
0115 8838424
OSCAR
21A Hendon Rise, St Ann's, Nottingham NG3
3AN
0115 947 2718
Guideline for Adults with Sickle Cell Disease: Chronic complications
Author: Dr Claire Chapman, adapted for use at NUH by Dr Sarah Marie Donohue
Approved by: East Midlands Sickle Cell & Thalassaemia Network
Page 16 of 16