Priapism (painful erections) associated with sickle cell disease This leaflet explains about priapism associated with sickle cell disease. If you have any further questions or concerns

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Priapism (painful erections) 

associated with sickle cell 


This leaflet explains about priapism associated with sickle cell disease. If you have any 

further questions or concerns, please do not hesitate to contact the doctor or nurse 

caring for you. 


What is a priapism?  

Priapism is an unusual erection of the penis that is frequently painful and distressing. These 

are not associated with normal sexual function or desire.  


The majority of males with sickle cell anaemia (HbSS) are likely to experience a priapism in 

their life time. Priapism is less common in boys and men with sickle cell disease (HbSC) and 

sickle beta thalassaemia (HbSB). 


There are two common types of priapism:  







What is a stuttering priapism?   

The most common type of priapism is a stuttering priapism. It is called stuttering because it 

keeps reoccurring. Stuttering priapism attacks usually get better by themselves, but 

spontaneously reoccur again in a few minutes or within couple of hours.  


Some episodes of stuttering priapisms are not painful; however the longer the stuttering priapism 

lasts the more painful the erections become. Stuttering priapism may go on to develop into a 

more severe attack of acute priapism. 


What is an acute priapism?  

This is a sustained painful erection lasting more than a few hours. This type of priapism will 

often not resolve spontaneously. Acute priapism is normally very painful and is considered a 

major complication of sickle cell disease. It needs emergency medical attention. Patients often 

experience episodes of stuttering priapism before an attack of acute priapism. 


Why does priapism happen?   

Sickled red blood cells block the supply and flow of blood in the penis. Therefore a priapism 

attack is a sickle cell crisis that is localised to the penis. The sickled red blood cells cause a 

disruption to the normal flow of blood within the penis. Consequently the penis becomes starved 

of oxygen and this causes pain and tissue damage within the penis. Normal early morning 

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erections that men and boys experience are an important trigger for priapism in patients with 

sickle cell disease.  


What are the risks from having priapism?  

The tissues within the penis can become scarred and damaged because of frequent episodes 

of stuttering priapism, or, more commonly, following a prolonged episode of acute priapism 

(lasting four hours or more). Scarring and damage to the tissue within the penis can then disrupt 

the normal flow of blood into the penis and this may result in a loss of normal sexual function 

(erectile dysfunction). 


At what age am I likely to experience priapism?  

The majority of patients report they first experience a priapism during early puberty 

(adolescents) but some patients still experience priapism into their 30s and 40s. 


What time of the day or night am I likely to experience priapism?  

Most priapism attacks occur in the early morning and are associated with normal early morning 

erections. Sleep is repeatedly interrupted causing tiredness and this can significantly interfere 

with studies and work. If you are losing a lot of sleep because of priapism please discuss this 

with your haematologist or specialist nurse as they may be able to recommend treatments and 

helpful strategies. 


Why did I not know about priapism?  

Knowledge of this severe complication of sickle cell disease is often very poor. Many patients 

report that they are not aware that priapism is a complication of sickle cell disease. Sickle cell 

patients are therefore not fully aware of the risks associated with episodes of priapism. Patients 

are often reluctant to talk about priapism because of shyness and embarrassment.  


What can be the consequences of priapism?  

Some patients who have had stuttering and acute priapism report difficulties achieving a normal 

sexual erection (erectile dysfunction). Erectile dysfunction is more likely to occur if priapism 

episodes are not treated promptly. Erectile dysfunction can cause severe psychological distress 

and affect relationships. Patients might hide the extent of the problem particularly from nurses, 

doctors, partners and family members because of embarrassment.  


If you feel that you have developed erectile dysfunction it is important that you are evaluated by 

a specialist (urologist) who will be able to offer a number of treatment options. Your GP or 

haematologist can refer you to an urologist. It not advisable that you attempt to treat erectile 

dysfunction yourself as some treatments (including those that you can buy from the internet) are 



What can trigger a priapism?   

There are a number of factors that can act as a trigger for priapism  



  Priapism is commonly triggered by the same factors that cause other types of sickle cell 

crisis and this includes stress, infection, low oxygen levels, dehydration, alcohol, physical 

activity, and hot and cold environments.  


  Early morning erections are an important trigger for sickle cell priapism. Early morning 

erections are normal and are associated with having a full bladder. These normal 

sustained erections normally resolve when you pass urine. However in sickle cell 

patients early morning erections can develop into a priapism attack, particularly if the 

patients fail to resolve them normally by passing urine.  


  Patients with a low oxygen levels when well or a history of breathing problems whilst 

sleeping have an increased risk of priapism.  


  Alcohol and smoking can act as trigger for all types of sickle cell crisis including priapism  


  Patients report that priapism can develop during sex. Having sex can be physically 

demanding and this may trigger a sickle cell crisis including a priapism.  


  Certain drugs and treatments can increase your risk of priapism. Consequently if you 

have sickle cell disease, it is important to discuss new medications or treatments with 

your haematologist. 


  Applying ice or cold water to the priapism will worsen the problem. Remember that being 

cold will trigger all types of sickle cell crisis, so applying ice or cold water to the penis is 

likely to increase the risk of localised sickling. 


How do patients manage their priapism?  

Many patients have developed different strategies for dealing with their own priapism.  


Helpful strategies for dealing with priapism include painkillers, heat packs and the application of 

warm water to the penis. Exercise such as doing squats, jogging, cycling or running up and 

down stairs can resolve a priapism attack. Emptying the bladder during the night to avoid early 

morning erections has been reported as a beneficial preventative measure in some cases. 


Patients often report that the normal painkillers they use to treat their sickle cell crisis are not as 

effective in controlling the pain of priapism. Do not use ice or cold water applications to the 

priapism as this is potentially harmful. Before trying a strategy for managing your own priapism 

it might be helpful to discuss it with your sickle cell nurse or haematologist.  


Here are some helpful tips  


  Treat a priapism as you would a normal sickle cell crisis including drinking extra fluids 

and pain relief. 


  Empty your bladder at night and immediately on waking to help you avoid early morning 

erections that might trigger your priapism.  


  Try exercise strategies to resolve your priapism. 


  Find ways to avoid stress, for example counselling, support groups. 


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  Avoid sexual activity during priapism. Remember priapism will not be resolved by sex or 



  Avoid taking lots of painkillers to manage your priapism; find out about other treatment 






 as these can potentially worsen or trigger 



You must attend your usual A&E urgently if the priapism does not show signs of 

resolving within two hours. 


Seeking help 

Many sickle cell patients report that they would not seek medical attention for priapism because 

of embarrassment and practical difficulties such as getting dressed and travelling to the hospital 

in the early hours with a severe priapism. Many patients are unaware of the treatments 

available and are fearful of about what might happen when they come to hospital and how 

doctors and nurses might react.  


Many patients therefore seek information about priapism from brothers, mothers and other 

males with sickle cell disease. However many of these people may be unable to recommend 

suitable treatments or strategies due lack of knowledge. Sickle cell patients have found it 

difficult to discuss the issue with female health care professionals, partners and friends. Many 

patients are anxious about how their sexual function might be affected in the future.  


Therefore, we’ve put together some helpful information that you will need to know if you are 

coming to the hospital with a priapism: 


  Ask for privacy in the clinic, ward or A&E if you wish to discuss your priapism. 

  Talk about your priapism and treatment options with your doctor (haematologist) or 

specialist nurse or psychologist. 

  Discuss helpful strategies and treatments with your haematologist or specialist nurse. 

  Ask for written information about priapism and treatments. 

  Call an ambulance if you cannot travel normally to the hospital because of the pain of 


  Ask your haematologist or nurse specialist to explain this complication to your family or 

partner if you feel this would be helpful. 

  If you are stressed because of priapism and the impact on your life, ask to be referred to 

the sickle cell psychologist as they are used to helping people live with this problem 

  Trying to find reliable information about priapism in resources such as the internet may 

be difficult and could result in confusion. Therefore, it’s best to discuss this health issue 

with your haematologist,  urologist or specialist nurse 


Are there any treatments to prevent priapisms?  

There are a number of common medicines that treat or prevent priapism. Your haematologist or 

urologist will talk to you about what is the best treatment to manage your type of priapism. It is 

important to understand that painkillers are not a direct treatment for your priapism but will help 

you to manage the pain associated with this complication.  


If you find you are using too many painkillers to manage your priapism it would be advisable 

that you discuss this with your haematologist so that he/she can consider other treatment 

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options. If you are experiencing priapism episodes please mention this to your haematology 

team in clinic as it may be recommended that you should be reviewed in our specialist priapism 



Why and when should I attend A&E with a priapism? 

  If your priapism fails to respond to your normal treatment or strategies at home after two 

hours you should go to A&E.   


  The longer you remain at home with your priapism the more likely damage and severe 

pain in the tissue of the penis can occur. This increases the likelihood of tissue damage 

in the penis and loss of normal sexual function (erectile dysfunction )  


  Wear baggy jogging bottoms or loose underclothing as tight garments might aggravate 

the pain caused by priapism. Loose clothing will help you travel and will help you to 

disguise the nature of the problem when in public places. 


  You must call an ambulance/arrange transport if the pain is severe and the priapism is 

not responding to your normal treatments and strategies. 


  Once you arrive at A&E, inform the medical or nursing staff as to the nature of the 

problem so that they can contact the sickle cell team and urologist. If you feel shy or 

worried ask for privacy. 


  Priapisms that require you to attend hospital will be evaluated by your haematologist and 

a urologist (specialist) and they will recommend treatments which can include oral 

medications that can directly treat priapism and strong painkillers,  


  In very severe acute attacks of priapism the urologist may advise you are admitted for 

injections of treatment directly into the penis or aspiration (removal) of the sickle cell 

blood from the penis.  


  Although some of these treatments sound unpleasant your urologist will try to make you 

as comfortable as possible. It is important to understand that your urologist is trying to 

prevent erectile dysfunction and to treat this severe emergency. 


  Whilst you are in hospital with a priapism you may also receive the common treatments 

that are used to manage a sickle cell crisis and this may include intravenous fluids

oxygen and antibiotics. In severe cases of priapisms exchange blood transfusions may 

be recommended by your haematologist if the priapism fails to respond to treatments 

recommended by  the urologist  


  The earlier you go to A&E, the more likely it is that you will be treated successfully, which 

will reduce the risk of erectile dysfunction







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Pharmacy Medicines Helpline 

Useful contacts for the sickle cell team 

Consultant haematologists – Dr Jo Howard and Dr R. Kesse-Adu, t:020 7188 2741 

Consultant urologists – Dr Majed Shabbir and Dr Z Evangelos, t: 020 7188 0243  

Sickle specialist registrar, bleep 0248 

Sickle senior house officer (SHO), bleep 2283 


Advanced nurse practitioner – Mr Neil Westerdale 

t: 0207 188 2710 / 07770 683 947   bleep 1843. 

Clinical nurse specialist – Mr Luhanga Musumadi 

t: 020 7188 2710 / 07770 678 851   bleep 2256 

Adolescent clinical nurse specialist – Mrs Judith St Hilaire 

t: 020 7188 2710 / 07920 711 266   bleep 2868 


Consultant health psychologist – Dr Nicky Thomas 

t: 020 7188 2725 

Clinical health psychologist – Dr Heather Rawle  

t: 020 7188 2718 

Health psychologist – Mina Abedian 

t: 020 7188 2718 


Haematology Clinic, t: 020 7188 2743 / 2724 

Haematology Day Unit, t: 020 7188 2745 / 2710 

Florence Ward (urology), t: 020 7188 813  


For more information leaflets on conditions, procedures, treatments and services offered 

t our hospitals, please visit





If you have any questions or concerns about your medicines, please speak to the staff caring for 

you or call our helpline. 


 020 7188 8748 9am to 5pm, Monday to Friday 


Patient Advice and Liaison Service (PALS)  

To make comments or raise concerns about the Trust’s services, please contact PALS. Ask a 

member of staff to direct you to the PALS office or:  


020 7188 8801 at St Thomas’  


 020 7188 8803 at Guy’s  



Language Support Services  

If you need an interpreter or information about your care in a different language or format, 

please get in touch using the following contact details. 


 020 7188 8815  


 020 7188 5953 



Leaflet number: 3501/VER2

Date published: March 2015

Review date: March 2018

© 2015 Guy’s and St Thomas’ NHS Foundation Trust 

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