Gwendolyn's machines

June 09, 2008 by Bill Strong

We've had a lot of questions about the machines that Gwendolyn is using to keep her in tip-top health and provide her, in our opinion, the best quality of life possible through the progression of her disease. So, below is a picture of her machine cart and we've described each of them below the picture starting with the small blue machine on the pole in the top left of the picture and moving clockwise from there...

  • Feeding machine ~ Since her G-tube and Nissen fundoplication surgery in mid-April 2008, Gwendolyn has been fed solely through her G-tube directly into her tummy. To accomplish this she uses a Kangaroo Joey enteral feeding pump. The pump is the size of two small video cameras and attaches to a medical pole. Whatever we are feeding her hangs in a disposable bag on the medical pole and the contents are sent from the disposable bag through a thin rubber tube, through the feeding machine, into her G-tube, and into her tummy. This machine is much more user friendly than prior models we've used in the past and has a battery and backpack option which allows us to feed her on-the-go; both great options.
  • Suction ~ Since Gwendolyn cannot swallow and is at extremely high risk of aspirating on any secretions that do go down her throat, diligent suctioning of her mouth, throat, and nose is one of the most important preventative tasks that we perform on a regular basis. Gwendolyn's suction needs depend heavily on her health, but even during perfectly healthy times we need to suction her several times per hour; in not so healthy times it has been as frequent as every 2 minutes. This is the main reason we have nursing help at night. Gwendolyn uses a DeVilbiss portable suction pump that is about the size of a toaster and can be used on battery. For suctioning her mouth or throat we use a long semi-hard rubber tube called a 10 french catheter and for suctioning her nose we use a hard plastic device with a Hershey kiss looking thingy on the end of it called an olive-tip or BBG. Gwendolyn is becoming accustomed to this and actually opens her mouth for us when she really needs suctioning.
  • Pulse oximeter ~ Gwendolyn has a Nonin 9600 pulse oximeter machine. The machine is about the size of three small video cameras and is portable and battery operated. It measures the % of oxygen saturation in Gwendolyn's blood and her heart rate. She wears a small infrared sensor around her toe which attaches to the machine. We use the output from the machine as a warning system to alert us to potential issues that Gwendolyn may be having. But, as we've learned time and time again, at the end of the day it is a machine, it is only a tool, and we've found it is most important for us to know Gwendolyn's specific signs of distress and look to her to tell us when something may be wrong. Currently, Gwendolyn's baseline oxygen % runs between 96-99% and her heart rate between 130 (sleeping) and 140-150 (awake). Gwendolyn wears this at all times, except when bathing.
  • Bi-pap ~ Bi-pap stands for "Bi-level Positive Air Pressure" and is a non-invasive breathing aid that delivers alternating levels of air pressure; higher pressure when Gwendolyn inhales to keep the airway open and lower pressure when she exhales. It is a semi-intelligent machine and senses when she inhales and exhales to closely match her natural breathing pattern -- it does not breathe for her. The bi-pap is one of the most important pieces of equipment that Gwendolyn uses as it allows her to breathe much larger volumes of air with much less energy than without it. The larger volumes of air may also work as a type of physical therapy, allowing her diaphragm to expand and develop more normally. At home, Gwendolyn is using the Respironics Synchrony S/T bi-pap to help her with her breathing. Her machine is about the size of a shoe box and is very quiet. This is nice because the model they use in the hospital is about the size of a Weber barbecue and is much, much louder. She wears a plastic/gel mask that covers her nose and the mask attaches to fabric headgear that fits around her head. A tube attaches to the mask and that tube attaches to the machine. Gwendolyn didn't use the bi-pap until she was about 6 months old, although it is clear to us now that she should have been on it much sooner than that. Since then there have been times when she has been on her machine 24/7. Our general rule of thumb is that she must be on the bi-pap when she is sleeping or napping but can come off of it when she is awake, alert, and healthy enough from a respiratory standpoint to do so. She tells us when she can and can't be off of the bi-pap through her body language and respiratory rate. Currently, Gwendolyn is on her bi-pap for about 18 hours per day and off for about 6 hours and her settings are 14 IPAP, 4 EPAP, 30 rate, 0.9 insp, and room air with oxygen as needed.
  • Nebulizer ~ Gwendolyn uses the PARI PRONEB Ultra II nebulizer. The machine is about the size of three small video cameras and has to be plugged in to operate. The nebulizer is an important part of the respiratory treatment routine we perform on Gwendolyn several times per day and is used to administer liquid medicine in a mist form into Gwendolyn's airways. In times of health we use it several times per day but in times of respiratory distress we start the nebulizer treatment immediately to try and help Gwendolyn clear whatever is keeping her from breathing normally. The machine is kind of like a high powered humidifier. A thin tube connects to the machine at one end and to a small plastic container on the other end. The container holds the liquid medicine. The nebulizer pumps oxygen through the tube, through the medicine in the plastic container, and blows it out in a mist form. We either hold the small plastic container in front of Gwendolyn's nose or attach the small plastic container directly in-line with her bi-pap hose to administer the medicine. There are several medicines (e.g. Albuterol, Pulmozyme, Mucamist) that we have given her at different times through her nebulizer but in general the medicines work to either open up her airways, break down sticky secretions in her lungs, or move her secretions up and out of her lungs/airways. The different medicines are used at different times and frequencies depending on her overall health. Currently, Gwendolyn is relatively healthy and is only being given treatments of Albuterol, a medicine that helps open up her airways, as part of our respiratory treatment routine at least five times per day or as needed.
  • CoughAssist ~ Since Gwendolyn is unable to cough, we use the Respironics CoughAssist machine to help her cough and clear her lungs of secretions. This machine is an important part of our regular respiratory treatment routine and plays a major role in keeping Gwendolyn's lungs clear and, therefore, healthy. The machine is about the size of four toasters, looks like something that would have been cutting edge in the 1950's, and must be plugged in to use it. A tube attaches to the machine and to a thin rubber face mask that fits over Gwendolyn's nose and mouth. The machine forces air into her lungs then automatically pulls that air out, forcing her to cough. To be honest, it's not the most pleasant machine (I actually did it to myself because I wanted to see what she was experiencing -- not fun) but it's very effective. Currently, with every respiratory treatment we are doing three sets of three coughs using 30 lbs of pressure in, 30 lbs of pressure out, 1.5 seconds in, 1.5 seconds out, with a 1.5 second pause in between coughs, on automatic mode.
  • Oxygen ~ Although Gwendolyn is not currently on any oxygen, that can change at any moment and the use of oxygen is an important tool in times of respiratory distress. We have several oxygen cannisters on hand which we can use to give her an oxygen boost when she is struggling. We can administer the oxygen in many ways, but most commonly we attach the oxygen directly in-line into the tube on her bi-pap machine which then delivers it to Gwendolyn as she breathes. When we returned from our most recent hospital visit in late May 2008, Gwendolyn was on a modest amount of oxygen 24/7 but her overall strength and respiratory health since has allowed us to stop the oxygen altogether -- something that we are both extremely happy to see.

There is really only one stroller/wheelchair for Type 1 families: the EASyS by Thomishilfen. As it is a medical device, it is quite expensive but most insurance companies will cover at least a portion. There are two sizes and both expand so each will last for several years. The EASyS can carry all or most of the equipment with some modifications. Click here to read how we modified Gwendolyn's chair. 

{UPDATE} We wanted to leave this post as it is to reflect where Gwendolyn was shortly after her SMA diagnosis to best help newly diagnosed families. Most of this information is still the same and is still a basic guide for anyone just entering this SMA world. As Gwendolyn has grown, her needs and various machine settings have changed a bit. And we now use the Trilogy 100 BiPAP by Respironics which has a much longer battery life. Here is a list of all of her machines with links. If you have any questions, please don't hesitate to contact us. We know how overwhelming an SMA diagnosis can be. 

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