My Medical Care

Articles by Brenda, my nurse

Acute Respiratory Care

by: B.S. Hanson

When respiratory crisis occurs, one must be prepared in advance. One must be prepared to enter the hospital knowing that you, as the parent will likely know more than the medical staff and you can't be afraid to speak up and give your opinion. Become familiar with the techniques that are available before hand so you can offer your opinion. For example, know what BiPAP, vest, intubation, and tracheotomy are so you know what these options are and know what the outcomes are.
Having a folder with you or at beside that lists medications, formula given, and respiratory treatments is needed in the event of a hospital visit. Also included in the folder should be the protocol for intubation/extubation and any other articles you have found. Keep spare paper in the folder to write down questions and thoughts and also have a list of other parent's phone numbers to ask questions and also the doctors who are available to contact in a respiratory crisis (Dr. Bach and Dr. Schroth).
Before respiratory crisis occurs, every parent needs to call their local EMS workers to prepare them for transport in the event of an emergency. Your local EMS workers need to know what supplies and equipment they need to have on hand to successfully transport your child.
When a respiratory crisis occurs, quick action in needed. It is recommended that oxygen is kept at hand along with an ambu-bag. Oxygen can be bled through the cough assist, the BiPAP, or the ambu-bag to assist in bringing up oxygen saturations. A respiratory crisis is usually a sudden episode where the saturations drop below 95% and maintain that level despite vigorous coughing, suctioning, and chest PT.
In the event that transport to a hospital is needed, take all your equipment with you. Many hospitals do not have the In-Exsufflator or Cough Assist on hand to help provide proper care. Also, if your child is on BiPAP, you will have to wait for the respiratory team to find a BiPAP and bring it to your child, which is wasting precious time. Take their BiPAP with you and make sure they are using it while in the Emergency Room. Don't let the staff talk you out of not using it. Be aware of nutritional issues. Regardless of if your child has a g-tube or not, they must not go more than 8 hours without nutrition. Regular IV's do not count as nutrition. TPN or PPN are needed to be ordered right away as it takes several hours to prepare. TPN is total parental nutrition and PPN is partial parental nutrition. If your child does not have a g-tube, then ask for an emergency NG tube so formula can be given safely.
One of the biggest concerns is hand washing. You need to watch every person like a hawk to make sure that they wash their hand before entering your child's room. Lots of hospital personnel forget to wash hand upon entering rooms, so stop them before they enter and have them wash.
Have the hospital staff right your child's routine down in the chart so it is followed as per your instructions. If you do nebulizer breathing treatments every 4 hours then they need to continue to do so in the hospital. If your child is coughed a certain way, with their head to one side or the other, then make sure everyone knows it. If you aren't comfortable with the respiratory therapists doing it, then do it yourself. Don't be afraid to stand up to them and take over if need be. If possible, don't leave your child's beside. Many hospital personnel have no clue how to help your child and will not know what is needed if you aren't there. Take turns with your spouse or other family members. Your child cannot call out for help or use the call assist button to call for help so you must be prepared to be there for them.
Upon entering the hospital have a sheet prepared that lists several specific things. It should have your child's name and date of birth and a brief description of SMA and how it affects your child personally. List past hospitalizations and what special treatments were done or needed. Include length of stay, intubations, and also surgeries done. Have a list of what is needed upon admission including TPN via an IV and also feeding if possible through g-tube or NG tube. Include a list of daily medications with time and route. Also include on the list allergies to any medications and medications that work and those that don't.
In the event that your child needs to be intubated make sure they have the Intensive Care Protocol developed by Dr. John Bach. Make sure they follow it to the letter for successful extubation. Calling him or Dr. Schroth for advice is warranted and highly suggested for success if your hospital isn't familiar with it. For information on the protocol, visit
The overall theme is do not be afraid to stand up for your child's needs. Don't be afraid to second guess the hospital staff; don't assume that just because they have the degree behind their name, they know what they are doing. Many doctors haven't really heard of SMA and aren't sure what will work best so stand up and let your needs be heard.



by: B.S. Hanson

NIV stands for noninvasive ventilation. It is called noninvasive because the ventilation is accomplished with artificial airways. It is a form of care for people with any neuromuscular disorder, including spinal muscular atrophy. NIV involves the use of certain equipment in the daily lives of people to help maintain energy and prevent respiratory distress. NIV methods can be safely utilized on people of any age, including infants.
There are three main goals of NIV, which are 1) Maintain range of motion of the lungs and chest wall to allow normal growth. 2) Maintain oxygen saturations at all times. 3) To assist in airway secretions.
One piece of equipment needed for any SMA child/adult is an oximeter. An oximeter simply measures the heart rate and level of oxygen in the blood. This is accomplished by attaching a probe to one's finger or toe with tape. It is very simple and painless. Normal saturations should be maintained at 95% or above at all times.
The second piece of equipment is a BiPAP (Bi-level Positive Airway Pressure). This piece of equipment often tends to frighten people the most. Common questions arise because any breathing device is often seen as a "last resort" or the "end is near." One must look upon it as a new beginning or a tool for assisting your child for better things. Simply put, a BiPAP is a machine that will assist a child in taking deep breaths. When non-affected people breathe, we inhale and we exhale. During breathing patterns of one with SMA, they can breathe in some, but the exhalation is often impaired. It is like trying to breathe in through a tiny, long straw, not because of tight airways, but because of so much energy is needed to get the air. The BiPAP machine helps by assisting the person to get a deeper breath. Upon inspiration, the BiPAP will recognize the breath and deliver more air to the person until a certain pressure is achieved. When exhalation occurs, the air flow in drops to a low level so Carbon Dioxide is exhaled from the body properly and the air flow helps to keep the lungs and airway open. All of this is accomplished by a simple mask worn primarily over the nose. There are many different interfaces that can be used, depending on the specific individual needs.
When starting your child on BiPAP, there are several considerations and concerns. First of all, starting the BiPAP earlier, the better it will be. Children are often frightened by the machine and the noise, so getting used to it when they are healthy is important. Starting BiPAP during an illness or respiratory crisis makes it harder for your child to get acclimated to it. Start for small periods of time and gradually build up the length of time using it. Your ultimate goal is to get your child to use the BiPAP all night and during naps.
Another important aspect of the BiPAP is the pressures the unit is set at. There are a lot of new terms involved with this unit. When talking about pressures, there is an IPAP on some units (the inspiratory pressure) and the EPAP (the expiratory pressure). In order for the BiPAP to be effective for your child, the span between the two numbers must be at least ten. The EPAP should be set as low as possible, like 2 or 4. If your child has a lung collapse, sometimes, it is beneficial for the EPAP to be 6, as this will help keep air in the lungs and help prevent the lung from further collapse. For example, if your unit goes down to 2, the minimum pressure for IPAP should be 12. There is also a setting for sensitivity; for how hard or easy it is to initiate a breath. A BiPAP unit can have up to three different settings: S, T, and ST (which differ by the manufacturer). A unit set to a "S" mode, is set to spontaneous mode. This means that your child must initiate every breath, so the sensitivity must be set so the child can trigger the machine. A "T" mode is set on a timed mode. The machine gives a breath for a specific number of breaths in a minute and no more will be delivered. The "S/T" mode is a combination of the two previous modes. The machine is set to give a specific number of breaths but the user may initiate breast as needed. Preference depends on the individual child but the timed mode and the spontaneous/timed mode are used most frequently. The spontaneous mode should rarely be used since one of the main goals of the BiPAP is to conserve energy and having to trigger every breath is tiring and using a lot of energy.
One thing that must be watched for is "BiPAP Bites". These are pressure sores from the masks that show up on the bridge of the nose or forehead. There are many different ways to treat these, using a different mask, adding padding like duoderm to the nose and forehead can help, or putting ointment or cream on the area helps. Commonly used creams are pure lanolin, RoEZit, and Carmex, to name a few.
Another part of NIV is the use of a volume ventilator instead of or in addition to the BiPAP. A volume ventilator delivers a set volume of air on each breath. However, there are two new small ventilators that can be used with BiPAP settings: the LTV series by Pulmonetics and the HT-50 by Newport.
The third piece of equipment is the cough assist or In-Exsufflator, manufactured by JH Emerson Company ( The Cough Assists function is just what it says; it is to assist one in coughing. The machine delivers a deep breath of air (insufflations) via a mask followed immediately by a deep exhale (exsufflations). It gives a big breath in and then takes all of the air out, including any secretions in the airway. Simply put, it fills the lungs with air; a big breath, and with the flip of a switch, pulls out the air and secretions easily, effectively, and efficiently. For maximum benefit, the cough assist should be used at pressures of +40 for insufflations and a -40 for exsufflations. Many people start at 20 and work their way up. The cough assist should be done daily at least twice a day to help keep the lungs mobile and to allow the chest wall to expand. When sickness hits, this piece of equipment will be a lifesaver and can be done as often as needed…even every few minutes. A training/informational DVD is available from the company.
The cough assist is another piece of equipment that needs to be started early. One myth about the cough assist is that even if your child is too young to work with it, then it won't be of any benefit to them. It is a fact that even if your child isn't working with the machine or crying, they are benefiting from the chest expansion.
Chest physical therapy or Chest PT can be of great benefit. This involves the use of "cupping" your hands and "clapping" them upon your child's chest. Chest PT needs to be done properly but is easy to learn how to do it. Chest PT can be done along with postural draining, having the child's head lower than their chest, in a reverse inclined position. Using postural drainage however, is contradicted if the child has reflux.
If doing Chest PT, you "clap" or "pound" on your child two to three minutes per side. Coughing and suctioning should be done after each side, to remove any secretions that were loosened. Suctioning should be done especially if your child has lost their swallow. Besides doing Chest PT with your hands, often wands or hand held cups can be used. There are also percussor that can be applied to the chest area to accomplish Chest PT without using your hands.
There is also a Therapy Vest out on the market today that accomplishes Chest PT easily. The Vest Airway Clearance system ( is accomplished by wearing a special vest that is attached to a box and bursts of air pulsate through the vest. The vest can be done in any position and once set up, done independently. Special modifications must be done for infants but it is possible.
One of the last pieces of equipment needed is a suction machine. Most families ask for two. There are stationary units, that are kept at bedside and there are also portable units that utilize rechargeable batteries that can be taken anywhere. The portable suction is extremely necessary, so you can move your child to another room or for traveling. The suction machine is necessary for all type 1's and most type 2 children.
Suctioning can be done in the nose or mouth, and can be done deep or just in the nose or mouth. There are a variety of catheters that can be used for suctioning, based on location of suctioning, child's age, and purpose.
There are hard yankauer catheters that can be used for just suctioning the nose or mouth. For deep suctioning, a longer more flexible catheter is needed. Some parents use a red Bard All-Purpose Urethral Catheter for nasal suctioning since it is more flexible and less traumatic for the child.



by: B.S. Hanson

Aspiration is the inhalation of foreign material into the lungs. Once can aspirate food of any consistency, liquids, or even mucus.
Normally, when a person aspirates, they are able to cough it out and they also know that it went down the wrong way. Children with SMA have a two-fold problem. First of all, they have no muscle strength to cough it out, so help is required. The second problem is most type 1's and some type 2 kids lose their swallow and cannot manage their own secretions. Care must be taken for these individuals as suctioning must happen each and every time before the child is moved. Common signs of aspiration are a wet swallow and cough that can be heard in the throat along with "wet" sounding vocalizations. Aspiration can cause oxygen desaturations and must be removed immediately so complications do not occur, which are respiratory failure, pneumonia, and lung collapse.
Frequently, people aspirate silently with no warning or any symptoms. Some of the chest infections that kids with SMA get are actually caused by a silent aspiration. These kids will need a gastric tube to help prevent respiratory failure and associated problems and so that the child gets adequate nutrition at all times.



by: B.S. Hanson

For the children that completly lose their swallow (majority of type 1's), laying flat and head to the side is important to help avoid plugging episodes. When the swallow is lost - sitting children in an upright position and placing head in a mid-line position can add to secretions pooling in the back of the throat.

Every SMA child is different and can tolerate various degrees of head positions as well as their beds at slight inclines- you will have to find the optimal position for your child.

Turning the child from side to side to back often is highly recommended as long as they tolerate it. The body will change shape if left on one side too long. Some kids do well only on one side so care must be taken if this occurs.

Movement is highly encouraged to develop proprioception in children. Because SMA kids (type 1 and 2) don't walk, their sense of balance is often under developed to not developed at all. The use of swings and movement by "dancing with your child" if possible and help make the ears learn the sense of balance.

Standing can be done by any child (some kids will need BiPAP support) but it is essential for digestion issues and for bone growth and strength.

Getting foot splints (AFO's or DAFO's) can help prevent foot drop early on and allow standing to occur. Using leg immobilizers on the knees can help stretch the knees out and prevent contractures from occurring. Standing is possible with contractures...padding needs to be added to help with the degree of the contracture. Time spent on the stomach is advised, if tolerated. It helps open up the spine and stretch the hips out.

We used to do belly time and I would apply pressure to her buttocks (like I was sitting on her) and then supporting her head and neck, pull her upper body upwards by an inch or two to relieve the kyphosis pressure of the upper body. Another stretch that is benefitial is while laying on the stomach and pulling the shoulders back to help open up the chest area in the front...since most of the day is spent with the shoulders hunched forward. This can also be done on the side, doing one shoulder at a time. Stretching the shoulders and arms is often forgotten but it is beneficial. Try stretching the arms over the head. We put balloons around at all different levels so when stretching, we would hit them away. Stretching was done when all the balloons had been hit away. To stretch out the hip flexors (front of hip area), you put the child on the side of the bed and have on leg hang off the bed and down. This stretches out the front area of the hips, which will allow for better standing!

Daily ROM and stretching is essential for painfree positioning later on.

Try to avoid having the legs in the frog position. Using pillows and socks filled with uncooked rice will help keep the legs in alignment.

Using a back brace (TLSO) can help slow down the scoliosis from occurring. It will not stop it, but will slow it down enough so surgery can occur later.

Providing supportive seating and positioning to prevent deformaties from occurring. Keeping the body in alignment will slow progression of contractures from developing. Having support while in the upright position will often limit a type 2 child from some functions but will prevent rapid growth of the scoliosis curve. Proper strapping and belts are a must for the child's safety for a child of any type. Having a recline or leg elevating function on chairs is recommeded to allow for stretching and relieving pressure and having the body in a seated position for long periods of time.

For children who can swallow, positioning the head midline with the chin slightly elevated will allow for a safer swallow.


Recognizing Respiratory Infections

by: B.S. Hanson

In Spinal Muscular Atrophy, common colds can be quite dangerous for the kids. The key is recognizing infections and distress early. You, in essence, have to learn to see into the future. It actually is quite easy. By using the oximeter day after day and night after night, you will soon notice a pattern that is developing. During healthy times, a child's oxygen saturations are normally between a given range as is their heart rate. This becomes their "norm": what is normal for them and only them. When the numbers aren't in that pattern or normal range, the child is in distress of some type. This disruption doesn't automatically mean your child is sick; it is just a warning. It could mean anything from needing a diaper changed, hunger, constipation, needing to be repositioned to discomfort from teething.
Other early signs are sweating more than normal, flushing, and that the breath rate increases. You will quickly become accustomed to what signal your child gives you for each specific problem.
Another helpful tool is a stethoscope. Buy one early on and practice listening to your child daily. It doesn't matter if you aren't trained in medical techniques; you will notice a change when one occurs.
One of the last things to raise a red flag is secretions. Take note of the color, consistency and amount. If the color changes or is thick and sticky, something could be going on. You will get to know every minor detail of your child's life and will know what to do to mobilize and begin to help your child feel better.