Care and mobility considerations
Mild functional restriction
People in the followings categories are likely to have minimal or mild functional restrictions only:
- Occasional or infrequent crises only
- Able to manage crises at home themselves, or with some input from the general practitioner or community nurse
- Full recovery after crises and return to normal activities
- Haemoglobin levels within normal range, or mild anaemia only, for majority of time
- Not under regular hospital care
- Sickle cell anaemia without complications
During a crisis a person is likely to be confined to bed for up to a week, and would be expected to return to normal activities within seven to ten days.
People with mild functional restrictions are unlikely to require any long-term help with self-care, nor are they restricted in their ability to walk. Constant watching over does not prevent sickle cell crises occurring, and people would usually be able to seek assistance if needed, when a crisis was developing.
Moderate functional restriction
People in the followings categories are likely to have moderate functional restrictions, in particular as long term complications of sickle cell anaemia develop -:
- Frequent painful crises (2 or more per month) - full recovery between crises less likely and more potent analgesics taken most of the time.
- Persistent moderate to severe anaemia (haemoglobin level - 50% of normal or less 6-8g/dl ) between crises
- Arthritis of the joints of the upper and/or lower limbs and persistent bone pain requiring regular medication
- Avascular necrosis of the hip and/or shoulder joint.
- Regular admission to hospital for treatment or need for frequent regular clinic attendance to maintain stable condition
- People with persistent leg ulcers requiring regular treatment
- Recovering from a stroke
People with moderate functional restrictions may need some help with self-care and preparations of meals. The activities of people with moderate to severe anaemia will be limited by fatigue and shortness of breath, and tasks may take longer to complete. The ability to walk may be restricted due to arthritis of the hip joint and/or other joints in the lower limbs. Painful leg ulcers may impede walking, and anaemia causing fatigue and shortness of breath may also limit the distance covered.
Severe functional restriction
Severe functional restrictions are likely to be present when people are limited by the combined effects of a number of the complications described above under moderate functional restriction. In addition the following conditions restrict capacity:
- Severe arthritis affecting both upper and lower limbs
- Bilateral hip and shoulder deformity due to avascular necrosis, especially if not amenable to joint replacement surgery
- Persistent neurological deficits following stroke or cerebral haemorrhage
- Seizures and epilepsy
- Long term lung damage, including chronic pulmonary hypertension, causing more severe shortness of breath
- Enlarged heart (cardiomyopathy) and heart failure
- Renal failure
People with severe functional restrictions are likely to need help with all aspects of self-care including washing, dressing, bathing, rising from a chair, moving around the house, with the toilet, on stairs, administering medication, maintaining nutrition and fluid intake. Severe lower limb arthritis or long term neurological deficits after stroke are likely to cause considerable restriction in walking, and people with these problems may be prone to falls.
The majority of adults with mild to moderate sickle cell disease have normal cognitive function and are unlikely to require supervision in the home or out of doors. 20% of adults have some level of cognitive impartment related to infarcts; the most likely impairment is learning difficulty. Constant watching over does not prevent sickle cell crises occurring and people would usually be able to seek medical assistance when a crisis was developing.
Amended May 2009
