Medical Emergencies (Adult) - Sickle Cell

Situations when a person with sickle cell disease needs to be seen by a doctor straight away include:

Fever Temperature 101°F or 39°C or higher
Headache Severe headache, dizziness or stiff neck
Breathing difficulty Pain or trouble breathing
Abdominal pain Severe pain or swelling of the tummy
Colour Very pale palms of the hand, lips and inner eyelid
Spleen Sudden enlargement
Penis Painful erection lasting for more than two hours
Change in behaviour Person appear confused or drowsy or unable to speak
Fits, convulsions Body spasms and loss of consciousness
Weakness This may affect one side of the body or both and may include inability to walk

In these situations the GP should be contacted immediately but if there is difficulty contacting the GP the person should be taken to the local Accident and Emergency department straight away, either by car if the person can be transported easily or in an emergency an ambulance should be called by dialing 999 and asking for an ambulance.

As soon as the person arrives in the Accident & Emergency department the medical and nursing staff should be told that the person has sickle cell disease so that they are aware of the urgency of the person’s condition.

Some medical problems

This section will deal with medical problems relating to adults specifically, however some of them they may be seen in children. For information on medical emergencies relating to children specifically see Care and Management of your child with sickle cell disease – A Parent’s Guide.

Sickle cell disease is very variable many of the complications associated with the condition may or may not happen to an individual. Sometimes it is possible to manage a medical problem at home with the help of the GP. Sometimes it will be necessary for the person to be taken to hospital.

Anaemia

Anaemia is the hall mark of sickle cell disease however the anaemia is not as a result of iron deficiency necessarily. Because the condition is called ‘anaemia’ people assume that individuals with sickle cell disease are lacking in iron, but the anaemia in this condition is as a result of rapid breakdown and the shortened life span of the red blood cells. Individuals will often feel tired and become breathless on exertion, they may feel faint or actually faint. The anaemia often worsens during an acute illness and an individual may need blood transfusion. The lips, eyelids and palm of the hands will appear pale and if the anaemia becomes worse this areas become even paler still. This is the reason for taking note if a person with sickle cell disease appears paler than what is usual for them as an individual, this may suggest a medical emergency and the person need to be seen by a doctor straight away.

It is important not to self medicate with iron tonics and medications because one can become iron overloaded, which is dangerous for people with sickle cell disease. Iron should only be taken if prescribed by the doctor and only following a special blood test to confirm that the person is genuinely lacking in iron.

Sickle Cell pain crisis

Pain is the most common symptoms of sickle cell disease and is often caused by sickled red blood cells blocking small blood vessels. It can occur in any part of the body. Pain may be mild, moderate or severe. The majority of mild to moderate pain is managed at home successfully with the support of the GP, community or specialist nurse but severe pain may necessitate going into hospital for more specific medical treatment. For management of pain at home see Managing an Illness at Home - Sickle Cell.

Although pain is associated with sickle cell disease it must be remembered that pain is merely a warning sign that something has gone wrong in the body and people with sickle cell disease like anyone else can get pain that is to do with other ailments and nothing to do with their sickle cell disease. For example, pain of appendicitis, general tension headache, arthritis, muscular injury and other forms of pain.

That is why it is important for health care professionals to be told if the pain feels ‘different’ and just as important for the health care professional to listen and take note of the report in order for them to investigate the cause of the pain rather than assume that it is related to sickle cell disease.

Meningitis

This is an infection of the surface of the brain. Meningitis can be caused by different infections. In sickle cell disease, the pneumococcus is one of the commonest causes of meningitis. This is why it is important for people with sickle cell disease to take penicillin daily especially during childhood and whilst still in the education environment including college and university. To confirm the diagnosis blood tests and a lumbar puncture (a small amount of fluid is taken from a space surrounding the spine) are done. If an infection is present antibiotics are usually given intravenously (through a drip) to clear it.

Chest infection/acute chest syndrome

People with sickle cell disease are more prone to infections therefore they get colds and coughs more easily and may get pneumonia which is an infection of the lung.

Sometimes sickling of the red cells can occur in the lung when there is no infection, but the medical signs are the same. For this reason, the term ‘acute chest syndrome’ is used for both conditions. There may be cough, fever, chest pain and the breathing rate may be faster than normal. Pain may also be felt over the back and abdomen. If a person needs to be admitted to hospital because of an acute chest syndrome, a chest X- ray will be taken and usually they will need antibiotics.

The level of oxygen in the blood will be measured, using a machine called an oxygen saturation monitor. It may be necessary in some cases to give oxygen through a face mask and a blood transfusion.

Avascular necrosis of the femoral head (painful hip)

This is due to sickling in the topmost part of the thighbone. Sometimes it is seen on an X-ray just by chance but at other times it can cause a painful hip and a limp. This sort of crisis can usually be managed at home but the GP should be informed of this complication. Pain in the hip may last for several months and it may be necessary to avoid bearing weight on the affected leg. Sadly in the teenage years and young adults the pain can be so severe that it is advisable to stop weight bearing and use crutches to relieve stress on the joint. Sometimes the use of a wheelchair is inevitable and a hip transplant (replacement) may be necessary.

Avascular necrosis can also occur in other parts of the body, for example the shoulder joint or elbow.

Osteomyelitis (infection in the bone)

Osteomyelitis can be difficult to distinguish from a painful crisis but it does not occur very often. In both conditions, there may be swelling and tenderness of an arm or leg and the skin feels hot. In the early stages, X-rays are often normal, and the person may need to be referred to an orthopaedic (bone specialist) doctor to explore the swelling to look for infection and if osteomyelitis is confirmed, Intravenous antibiotics will be given for at least 6 weeks.

Leg Ulcers

People with sickle cell disease may develop leg ulcers following injury and or infection in the lower part of the leg especially the ankle. Because of sickle cell disease there is poor circulation to this part of the body and leg ulcers can be very painful, debilitating and difficult to treat. It tends to occur during the teenage years and in adulthood. Treatment includes treating any infection, pain relief, resting the limb by elevating it when sitting down. Sometimes the leg ulcer requires a skin graft.

t is advisable to wear protective clothing when in situations where lower leg injury may occur, for example, wearing long leg trousers and socks when gardening, to prevent scratches and stings, and when going out in an area where insect bites are common so as to prevent bites; especially in malarial areas of the world.

Where there is injury this must be treated rigorously to prevent a breakdown of the tissues and prevent infection.

Haematuria (blood in the urine)

Blood may be noticed in the urine due to sickling of red blood cells within the kidney. It is usually painless and may last a few weeks. There is no particular treatment and the bleeding will usually stop on its own. There are other causes of bleeding in the urine that are not to do with sickle cell disease so it is important to tell a doctor if blood is seen in the urine. Blood in the urine may be associated with a kidney or urinary tract infection. The doctor will probably ask for kidney scans and urine tests.

Sometimes haematuria occurs more commonly during pregnancy and may be a sign of infection.

Gallstones

When red blood cells are broken down some of it goes towards making bilirubin, a yellow pigment which the body uses for digesting the fat in the food we eat. Bilirubin is responsible for the yellow coloration of the whites of the eyes seen in many children and adults with sickle cell disease. The excess bilirubin may also form gall stones in the gall bladder, a sac which produces bile and lies behind the liver.

Most people with sickle cell disease over the age of ten years have gallstones and these can be shown by doing an ultrasound of the abdomen. Sometimes the stones get caught in the gallbladder tube and cause tummy pain, particularly on the right side of the abdomen. If gallstones start to cause problems it is advisable to have the gallbladder removed, because the stones can sometimes cause a serious infection known as cholecystitis. Not having a gall bladder does not cause any health problem and the individual will still be able to make enough bile.

Priapism (painful erection of the penis)

Priapism is caused when sickled red blood cells block the blood vessels of the penis, causing a hard and painful erection. This can occur at any age and is more common at night or early morning after a long period of being under warm bed clothing. Going to the toilet to pass urine and empty the bladder, having a warm shower or bath often helps to relieve priapism. The person experiencing priapism may need pain killers.

A warm bath or shower and gentle walking to improve blood circulation may help. Sometimes the penis becomes soft again on its own without any treatment. If this happens on a few occasions it is important to let the doctor know because repeated long term episodes can cause long term damage which can lead to impotence and later infertility.

If the painful erection persists for longer than four hours the person should be seen in the hospital because an operation may be needed to flush out the red blood cells that are causing the obstruction.

Applying an ice pack will make the situation worse.

Stroke

A small proportion of people with sickle cell disease have a stroke (damage to a part of the brain), especially in childhood, resulting in weakness down one side of the body. If a person shows signs of a stroke which include weakness of one side of the body or in a limb, inability to raise an arm and hold it there, slurring of the speech or not able to speak, facial drooping on one side, inability to smile properly or an abnormally slanted smile and or not responding when spoken to the individual needs to be seen straight away.

The person may need special imaging tests of their brain and a blood transfusion. Sometimes the weakness does not last very long, especially in children but less so in adults, and goes away by itself within a few hours or days but it is still very important that the person needs to be seen in hospital as a full blown stroke may occur at another time.

Without blood transfusion it is possible that the stroke be more extensive and cause more permanent damage. It is usual for the initial signs of weakness to get better after the child has been transfused. Unfortunately there is quite a high risk that another stroke may occur and to prevent this happening monthly blood transfusions are usually recommended.

It is now possible to carry out an ultrasound test (Transcranial Doppler Scan) to see if a person is having episodes of a stroke. It is recommended that this is done yearly from the age of 3 years and this is now national practice.

Eye problems

Different problems can affect all parts of the eye and can sometimes affect vision. Some hospitals carry out routine eye screening in people with sickle cell disease. If there is complaint of blurring of the vision or pain in the eye, the doctor should be informed immediately.

It may be necessary to refer the person to an eye specialist for an assessment and if necessary treatment will be recommended.

Headaches

Headaches are quite common in sickle cell disease, probably due to an increase in blood flow to the brain. Headaches do not tend to last very long and can usually be treated with pain killers, for example, Paracetamol. If the headaches are very frequent or if they persist for more than two hours at a time it is advisable to inform the GP or doctor at the hospital on the next clinic visit.

However if the headache is severe or the person also has a temperature above 38.5°C, a rash, or is vomiting or has a stiff neck this should be treated as an emergency as these are signs associated with meningitis or other complications.

Prevention

Many health problems can be prevented provided an individual learns about the illness and how it affects them as an individual. They should take all recommended medications and vaccinations and seek prompt medical advice or attention when they are unwell.

Because people with sickle cell disease have periods when they are well they often assume that they do not need to attend clinic regularly, however it is important for their condition to be monitored so that any signs of a worsening of their illness, such as heart and kidney damage which they may not feel physically, can be picked up early, treated and possibly prevented from getting worse.