ESP 
Your child’s primary physician will help assess for common reasons that respiratory symptoms develop. A pulmonologist can consider chronic management options. A speech therapist and radiologist can assess for aspiration. A gastroenterologist can determine if a feeding tube might help address feeding, swallowing or reflux problems that may lead to respiratory issues. Complex care and palliative care clinicians can provide expertise if there is declining benefit from chronic treatment plans.
Aspiration and Other Risk Factors
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There are a number of chronic problems that can impact pulmonary function (breathing). Breathing can be affected by common problems, such as asthma, allergies, and by obstructive sleep apnea. Respiratory issues in children with SNI may begin early in their life and for others increase as their underlying condition worsens and the likelihood of aspiration increases. Aspiration puts the child at risk for pneumonia. Interventions for these problems can improve baseline breathing and lessen the risk of an acute respiratory illness.
The Respiratory System
ESP The job of the respiratory system is to bring oxygen in, and breathe carbon dioxide out. In breathing, muscles expand and contract the lungs, the brain transmits nerve impulses to these muscles, and oxygen enters and carbon dioxide exits the body through the blood vessels and lung tissue.
The immune system is part of the response to the foreign material that enters the lungs. To keep lung tissue clear, mucus helps trap debris and bacteria, and tiny hairs called cilia sweep mucus out of the lungs.
The ability to generate a cough will further clear mucus from the lungs. Children with SNI usually have a normal immune system and cilia (unless there is a rare disorder that alters either), but often have other issues that impede the respiratory system.
ESP Aspiration
Aspiration is the passage of food and fluids into the airways and lungs. Mucus, the immune system, cilia and continuous coughing clear the lungs and keep small amounts of bacteria from turning into pneumonia. Healthy people aspirate small amounts of saliva and can have bacteria from the mouth enter the lungs. An impaired cough and less activity with aspiration can gradually and acutely overwhelm these normal responses and cause pneumonia.
Aspiration is often considered if a child has coughing, choking and gagging with feeds by mouth, or if a child develops pneumonia. A swallow study can assess for aspiration. This test will indicate if all liquids are aspirated or only some of a certain consistency, and help determine what your child can and cannot safely swallow by mouth. Let the medical team know if you are concerned that your child was not at their best during the study (for example, if they are recovering from a recent illness) as the test results may be affected. Repeated studies may also be recommended as your child’s baseline shifts. In some children, especially younger infants with or without SNI, and older children with SNI, may not reliably show the classic symptoms (coughing, gagging) with feeds. This is often called silent aspiration and can be detected through a swallow study.
Contributing Risk Factors
The risk of respiratory illness may increase as these problems develop and/or worsen in children with SNI:
- Altered ability to cough and expel mucus from the lungs
- Lack of activity such as walking and running, that would require deeper breathing that expands the lungs and helps move mucus
- Aspiration of food or fluids in the mouth into the lungs
- Gastroesophageal reflux disease
- Constipation
- Obstructive sleep apnea or central apnea
- Neuromuscular (NM) scoliosis
- Seizures
- Dystonia
Function and Equipment
Respiratory function is often monitored with a pulse oximeter. This device fits easily onto a finger or toe, and measures the oxygen saturation or amount of oxygen in the blood. Most people maintain a level of approximately 95% or higher, indicating that the respiratory system is functioning well. A sudden drop in oxygen saturation often indicates a sudden mucus block in the lungs or an acute illness. It is important to establish what is typical for your child and when to be concerned; for example, the oxygen level may drop during and after a seizure, and then improve. The medical team will determine the oxygen saturation level they want your child to maintain, and will tell you when to be concerned about a drop in that level. They will think with you about what equipment might be helpful to use. They may suggest, in addition to a pulse oximeter, a nebulizer machine to deliver inhaled treatments, pulmonary vest, cough assist, suction machine, oxygen concentrator or oxygen, feeding tube with connecting tubes, CPAP or BiPAP, tracheostomy tube, and/or ventilator. This equipment often includes associated parts such as tubing, face masks, syringes and replacement tubes as back-up.
The equipment used can provide benefits for some children over years, but there can be a decline in benefit as there is decline in a child’s overall condition. If acute respiratory illnesses have initially responded well to treatment, decline may be difficult to detect at first. It might only be possible to see significant decline in baseline by reminding yourself of what your child’s days looked like in the past compared to where they are now, and considering how your child is recovering now from each episode of acute illness. Complex care and palliative care teams can help you reflect back and look forward to what this might mean for your child.
Treatment
The goal for children with SNI is to identify and manage these problems, which can help to decrease the frequency of pneumonias. The medical team, including a pulmonologist or lung specialist and complex care clinician, will tailor treatment to your child and make adjustments as needed. Treatments that the medical team may consider include:
- Inhaled steroids to decrease chronic inflammation in the lungs
- Interventions to help clear mucus from the lungs
- Medications to decrease saliva and mucus production
- Nebulized saline to loosen mucus that has become thick and more difficult to clear from the lungs
- A feeding tube if your child aspirates when eating and drinking by mouth
- A specific type of feeding tube if content in the stomach is refluxed up to the airway and aspirated, using a longer tube that goes from the stomach into the small intestines so that feeds go into the intestines rather than the stomach
- Tracheostomy
- CPAP or BiPAP for obstructive sleep apnea
ESP Breathing is of course essential to living, and it follows that pulmonary problems are among the most worrisome ones for parents. Deciding on interventions is extremely difficult when there are no right or wrong answers. Clarifying which interventions have less-clear benefits will allow you to consider the possible impact of whatever decisions you make.
Suctioning of secretions at home is an example to explore. Suctioning might allow your child to be more comfortable–but you might have questions about how much suctioning you will have to do and what will happen if you are sometimes unable to follow through. Talk with the medical and palliative care team to try to strike a balance for you and your child. It might help to know that healthy people also aspirate small amounts of saliva. Discuss the risk of chronic aspiration and the interventions that can decrease this risk and help clear the mucus that develops. With this knowledge you can make decisions that help your child and also allow you to feel more settled. You are not a machine keeping your child healthy. You are providing loving care that should be balanced with reasonable expectations of the care you can give.
The most difficult shift is certainly when the interventions that helped your child in the past become more difficult for your child to tolerate or no longer provide the same benefit they once did. Families describe seeing this change by reflecting back on how their child’s life may have changed. This was described by one family as, “When did we go from assisting our son’s breathing to insisting that he breathe?” These shifts in baseline can happen gradually, often over years–or come on suddenly, especially towards the end of life. As you think about these shifts it can be helpful to ask if the intervention is doing things for your child or doing things to your child.
