The healthcare professional team involved in your child's care is very important and is there to respond to your child's needs. The team should consist of a specialist (expert) doctor with experience of ChILD as well as other professionals relevant to the specific needs of your child.

Not every team will be the same or stay the same as your child's requirements may change over time. Some professionals who may be involved are listed here:

• Local paediatrician
• Paediatric pulmonologist (specialist doctor for children's lung disease)
• Paediatric cardiologist (heart specialist for children)
• Psychologist
• Paediatric surgeon (if a lung biopsy is being considered)
• Rheumatologist
• Immunologist
• Gastroenterologist
• Human geneticist
• Dietician
• Physiotherapist
• Speech therapist
• Social worker
• Nurses
• Play therapist

There are no established treatment guidelines or specifically-approved medications for the treatment of ChILD as they are so rare, though there are some recommended practices for the investigation and treatment of ChILD, though these need to be tailored to each case. Most medications used are well-known and proven treatments in other diseases.

As there are no trials of what is the most effective treatment for ChILD conditions, you may find that children with the same condition have different treatment in different specialist centres.

Babies start to use their lungs to breathe as soon as they are born. The oxygen they breathe in from the air goes into the blood and travels around the body to keep it working well.

In chILD, less oxygen may be transferred to the blood in the lung. The extent of this will depend on how severe their condition is. If oxygen levels get too low, your child may find it hard to breathe.

Some children may start to breathe faster and deeper to make up for their reduced oxygen levels. Others, with more severe conditions, may find this does not give them enough oxygen and they will benefit from supplementary oxygen (also called oxygen therapy) to help them keep their oxygen levels to the required level.

If your child needs oxygen to help them breathe, your healthcare professional will prescribe oxygen and the equipment needed. You may be referred to a specialist nurse who will liaise between you and an oxygen supplier company, or you may need to make these arrangements with the supplier directly.

Everyone on oxygen needs to have their saturation (amount of oxygen in the blood) level monitored periodically (or in some cases continually) to make sure that the levels are adequate.

Oxygen therapy usually brings an improvement in your child's wellbeing.

Oxygen therapy can be provided at nursery or school giving your child more freedom. Depending on the kind of oxygen supply and flow needed, you may need to set up an additional oxygen supply at school/nursery.

If your child is prescribed oxygen to help them breathe more easily and is able to go home from hospital, then arrangements will be made for the delivery of equipment to your home. You will receive detailed information about the safe use of all equipment.

It will be necessary for your child's oxygen levels (called saturations) in the blood to be monitored, often periodically, but for some all the time. Talk to your child's healthcare professionals about whether you will need a monitor at home. You may be given a pulse oximeter which monitors the oxygen levels of your child, so you can make sure they are getting just the right amount of oxygen that they need. These devices have an in-built alarm which is programmed to alert you whenever the saturations fall below a certain level.

Once your child's oxygen requirements have been the same for a while, then you will not need to monitor them continuously. Your doctor will decide whether you need to monitor your child's oxygen saturation levels during sleep. This can be an anxious time for parents.

Your healthcare specialist will advise on the type of oxygen equipment your child will require and some examples are given below.

• Oxygen concentrator: This is the most commonly used equipment for long-term oxygen therapy use. The concentrator sucks in the air around you and filters out all other gases than oxygen. They are powered by electricity so a constant electric supply is needed for them to work. You can also get portable battery-operated systems for use with your child away from the home.
• Liquid oxygen systems: These tanks hold liquid oxygen which is produced when oxygen is cooled to -183 degrees when it changes from a gas to a liquid. They do not need an electricity supply to work and so give your child more freedom to move about. The tank must be stored in a cool, well-ventilated area away from flammable materials (e.g. paint).
• Oxygen gas cylinders: These are usually used in hospitals and are used in the home for getting out and about or as an emergency back up.

Your child will inhale the oxygen through plastic tubes which connect them to the oxygen supply. The oxygen is inhaled through either the nose (nasal cannulas) or the nose and mouth (mask):

• Nasal cannulas (also called prongs): This is a soft plastic tube connected to the oxygen supply which loops over both ears and has two nasal prongs to deliver oxygen directly into the nose. You may need to tape the tubing down securely in smaller children so they do not pull the tubes out. They can be used while your child is eating, drinking and being washed.
• Oxygen masks: These are made of plastic and cover both the mouth and nose and are attached by a piece of elastic around the back of the head. Some children find these more comfortable when sleeping than nasal cannulas but they can slip off easily.

Long-term use of oxygen therapy for full-term babies or children is not considered dangerous. It can lead to the drying out of the nasal mucosa which may result in crusts or nose bleeds. A humidifier attached to the oxygen supply can help prevent this so talk to your doctor if this is a problem.

Long-term use can result in problems for some prematurely born babies so discuss any concerns with your healthcare professional.

Most children only need mechanical ventilation temporarily or intermittently e.g. overnight or during short-term worsening of symptoms.

With very sick children, oxygen therapy alone may not be enough to maintain adequate breathing and they may not be able to eliminate enough carbon dioxide from their blood.

In these cases, the child may need help with their breathing in the form of ventilatory support (mechanical ventilation). There are two types of mechanical ventilation:

Non-invasive ventilation (NIV)

• This involves a specially-designed mask connected to a breathing (ventilation) machine with flexible tubing to improve breathing. It helps by increasing the pressure of air in the lungs to make breathing more comfortable. This can be by continuous positive airway pressure (called CPAP) which is a constant pressure during breathing in and out, or by a background pressure when breathing out (CPAP) and a higher pressure breathing in (bilevel ventilation).

Invasive ventilation

There are two ways to provide invasive ventilation:

• Intubation: usually a temporary tube guided down through the mouth or nose into the windpipe.
• Tracheotomy: usually a more permanent tube that is inserted through an incision in the throat by a surgeon (Ear, Nose and Throat specialist).

Cortisone is the most commonly used medication in children with ChILD as it has a powerful anti-inflammatory effect. Cortisone is a group of related substances collectively called Glucocorticoids (or steroids). The most commonly prescribed steroids are:

• Prednisone
• Prednisolone
• Methylprednisolone

Most high doses of steroids are well tolerated although some side effects may occur.

Not every child with ChILD will need medication. For example children with NEHI (neuroendocrine cell hyperplasia of infancy) usually respond well to oxygen therapy but not to any of these medications.

Hydroxychloroquine is an antimalarial, but is used in ChILD as it is considered to also damp down inflammation.

Azithromycin is the most common antibiotic used to treat children with ChILD. It works both as an antibiotic and is also considered to have mild anti-inflammatory properties.

A lung transplant will be considered in very rare and severe cases when a child progressively deteriorates in spite of medical therapy given and only after all other therapeutic options have been exhausted.

The goals of lung transplantation would be to improve the quality of life for your child and also to give them the chance to live longer than without transplantation.