Chest infections and breathing issues are the most frequent problems that require urgent treatment. Anyone with SMA who becomes unwell, should have a plan of action that any medical team can follow. This plan should be agreed between the doctor and adult while they are well, or between the doctor and parent while their child is well. Children should be involved in the discussions if they have an appropriate level of understanding. There should be a written record of this in the form of an ‘Emergency Healthcare Plan’. The aim is to empower adults and families to state their wishes and improve communication between them, urgent care teams and long-term care specialists.
The Emergency Healthcare Plan (EHP) or ‘Illness Plan’ should be written with the medical team. It should cover information about:
- What the warning signs or indications would be that would mean the person with SMA should be taken to hospital.
- Which healthcare providers should be contacted in case of an emergency.
- Preferences around breathing management including your views on different ways of supporting breathing. Respiratory support may be delivered non-invasively using a mask (NIV), or in the short-term ventilation may be delivered via a flexible plastic tube into the windpipe (intubation). Longer term an opening can be created at the front of the neck so that a tube can be inserted into the windpipe to help with breathing (a tracheostomy).
- Any neck and jaw mobility problems and limits.
- Techniques used for clearing secretions, including how often.
- Nutritional and fluid needs during illness.
- When and which antibiotics should be used.
- What action has been agreed and will be taken if resuscitation is required.
Where possible, local emergency medical services should be contacted in advance to discuss any specific needs and what equipment is used at home. In an emergency, you should go to the closest hospital. Wherever possible, the equipment used at home should also be used, even if this is in an ambulance that is well equipped. Sometimes non-sitters and sitters may need to be transferred between hospitals as they should be cared for at a specialist (tertiary) centre that is equipped to look after them. The clinical team responsible for their long-term care should always be notified about the illness.
Breathing assessment and support is the most important issue during an emergency. To address this and other key emergency care practices, the authors of the SoC advise medical teams to consider the following:
- Management should include early use of NIV and clearing of secretions before giving oxygen.
- Oxygen alone should not replace NIV and should only be added if oxygen levels in the blood remain low while NIV is being used and secretions are being cleared as described in the EHP. Only then may oxygen be added at the lowest flow required to optimise oxygenation, and then slowly stopped during recovery from the illness.
- Carbon dioxide levels should be monitored by either a blood test or transcutaneous skin probe during the time oxygen is being given.
- If it looks like intubation is required take into account the views of the patient or if the patient is a child they will consult with their parent/legal guardian.
- If intubation is no longer needed and the tube is going to be taken out, the lungs should be fully inflated and oxygenation optimised beforehand. NIV should be used as transitional support following extubation.
- Children, young people and adults should only be given antibiotics if there is a specific cause of the illness such as sepsis or a chest infection.
- Urgent care teams should review symptoms on admission, predisposing factors such as recent surgery, and nonrespiratory causes of sepsis e.g. urinary tract infection, skin infection etc.
- If there is a need for an anaesthetic, guidance in Section, Anaesthetics should be followed
It is essential that emergency care includes giving fluids early and that hydration, levels of salts and minerals, kidney function and glucose levels in the blood are monitored. Nutrition with protein should be given within six hours of becoming ill and there should not be long periods without food. Special attention should be paid to swallowing during an illness due to the risks of inhalation of food or liquids into the lungs (aspiration) (See Section, Nutrition, Growth and Bone Health).
Shortly after admission, discussions should start with the team as to what goals need to be achieved and what plans need to be put in place to make it safe and manageable to go home. Discharge planning should set goals with you, the hospital team, and the primary healthcare team. Physical and occupational therapy, speech therapy, psychosocial and palliative care services can all help with recovery from illness and efforts to maintain abilities.