Living with chILD
Living with chILD
Different forms of chILD require different treatments and support. The goal of treatment for chILD is to relieve symptoms, provide support to maximize growth and development, and to prevent exposure to preventable illnesses that could make the chILD syndrome worse.
Our bodies are fueled in part by the oxygen in the air we breathe. When we breathe it into our lungs the oxygen crosses from the lungs into the blood stream. The blood then takes it to all parts of the body. Our body cells “eat” oxygen and “spit out” carbon dioxide. This carbon dioxide travels back through the blood stream, crossing from the blood stream into the lungs, where it is breathed out. chILD can get in the way of this process. Some children with chILD have to work harder to breathe. Things like eating, crying and playing can be extra tiring. When children do not have enough oxygen for a short time, it may not harm them. They may have no effects you can see and they may not look different. Sustained low blood oxygen levels, like below 88 percent, can hurt the body. The heart gets larger than normal because it has to work harder to keep oxygen in the body. Some children with chILD need extra oxygen to help them breathe better, grow, and prevent heart strain.
Oxygen is a medicine. If your child needs extra oxygen, the doctor will prescribe the lowest amount that can be used safely. Keeping blood oxygen saturation levels (“sats”) above 92 percent helps ensure that the body has the oxygen it needs. Children with pulmonary hypertension need their blood oxygen levels kept at 95 percent or higher. Some children only use oxygen when they sleep or exert themselves. Others use it all the time. Sometimes the amount of oxygen needed changes as the lungs change.
If home oxygen is prescribed, a DME vendor will give the needed supplies. These might include an oxygen source that stays in your home, an oxygen source you take on the go, an oxygen delivery system (like nasal cannulas), swivel connectors, tubing extensions and water bottles. The DME vendor should have 24-hour service in case something breaks or you need help.
There is no single treatment for chILD and each patient will require a personalized treatment regimen. Some common treatments for various forms of chILD include:
Bronchodilators: If your child has reactive airways (asthma-like symptoms), treatment with bronchodilators, inhaled steroids, or both may help. Bronchodilators help relax the muscles in the airways to improve breathing right away. Inhaled steroids help decrease swelling in the airways over time. They do not improve breathing right away. These drugs are often given using an air compressor and nebulizer (from a durable medical equipment [DME] vendor) or using a metered dose inhaler (puffer) and spacer (from a pharmacy). Both the drugs and supplies need a prescription.
Mucus Clearance: If your child needs help clearing lung mucus, there are many options:
- Chest Physical Therapy (CPT or Chest PT) involves putting your child in different positions and clapping your hands on the chest and back over each of the lung areas.
- Positive Expiratory Pressure (PEP) Therapy employs cycles of abdominal breathing, breaths through a PEP device and coughing or huff coughing.
- Intrapulmonary Percussive Ventilation (IPV) involves breathing treatments (using a mask, mouthpiece, or tracheostomy tube) using a machine that gives mini-bursts of mist and air.
- High Frequency Chest Wall Oscillation uses a machine that sends air pulses to the lungs through tubing attached to a chest vest or wrap. There are even more options than these. Your health team can help you choose the best option for your child, help you get the needed supplies and teach you how to give the treatment.
Synagis: This is an antibody used in high-risk children to prevent severe cases of respiratory synctytial virus (RSV), a common virus that can be damaging to the lungs of chILD patients. Children who receive Synagis may still get RSV, but they will not get as sick as if they were not treated at all. This is an expensive treatment and is only approved by insurance under specific circumstances. If your chILD is under 3 and at high risk, your doctor may order this for your child.
Intravenous immunoglobulin (IVIG): Some forms of chILD are involved with immune responses. Some forms of chILD will require regular IVIG treatments to stop the progression of fibrosis in the lungs.
Anti-inflammatory drugs (steroids, cytotoxic drugs, immunosuppressive drugs): Some forms of chILD respond very well to steroids while others do not. Steroids and immunosuppressive drugs can have many side effects. Be sure to consult with an experienced chILD physician about the use of these types of medications in your chILD.
Transplants: Patients with certain types of chILD may be candidates for a lung or heart-lung transplant. Transplants are more often used with adults than children; but, for some forms of chILD, lung transplants are the only treatment that works. Click here for more information on transplants.
A common problem in chILD is poor growth, mainly in younger kids. Growth and lung function are closely tied. It takes more work to breathe with unhealthy lungs, so children with chILD may need extra calories. A visit with a dietitian trained to work with kids with lung diseases is a great first step in learning your child’s unique needs. The dietitian will review your child’s height and weight, past diet, calorie and protein needs, and growth and eating problems. From there, a care plan can be made that will best support feeding and growing.
Children have poor weight gain when they can't eat enough by mouth to meet their needs. Tell your child's doctor about any feeding problems your child is having. Some babies, like those with breathing problems, struggle to suck and swallow. Breathing and eating at the same time is hard work! Your dietitian can help you decide if a different bottle nipple might help. Give short rest breaks. If the doctor has ordered oxygen during feeding, use it!
Another common feeding problem is gastroesophageal reflux (GER). Food travels from the mouth, down the esophagus (the tube that leads from the mouth into the stomach), into the stomach. When food or liquid in the stomach backs up into the esophagus, it is called gastroesophageal reflux (GER). Many babies have GER, since the muscles that close the opening from the esophagus into the stomach are still weak. In children with breathing disorders, GER can be made worse by the forceful movements of the diaphragm and abdominal muscles that occur with labored breathing. There are drugs to treat GER, but sometimes surgery is needed.
Even with careful feeding and supplementation, some patients will require a feeding tube to meet their caloric needs. Click here for more specific information on feeding, feeding tubes and GER.
Because kids with chILD often need to be watched closely by many health team members, the challenge of travel is often faced. If your child uses a lot of home care supplies, travel can be tricky and may take more than one adult to pull off. Even a trip to the store can seem like a risky, all-day event! Click here for more travel tips.
Since chILD is rare, taking a child to the emergency room (ER) can be tricky. Most doctors don’t know a lot about chILD. If your child is sick, it might be hard to recall the details a doctor needs so having a current and complete Go-Binder with your child’s history and any information you have on their diagnosis is a must! Be prepared to educate the physicians and nurses about your child’s disease. Whenever possible, go to a hospital where your pulmonologist is affiliated and have the Emergency Room personnel contact your normal physician as soon as possible.
Some ERs don’t have infant- and child-sized supplies. If you have a small child that needs oxygen or breathing treatments, have an extra nasal cannula or aerosol mask in your Go-Bag. Also have extras of the supplies that are unique to your child (like extra MIC-Key extensions) and your special formula (which the hospital may not have on hand). Think about bringing splints to the ER to cover IVs so your child cannot pull them out.
What Is chILD
Living With chILD
Acute Interstitial Pneumonia
Alveolar Capillary Dysplasia
Neuroendocrine Hyperplasia of Infancy
Pulmonary Interstitial Glycogenosis