Wednesday, December 31, 2008



Shira has had 2 major hospitalizations since being diagnosed with SMA Type 1 at 4 months old. The first time Shira was hospitalized was with RSV or Respiratory Synctal Virus at 6 months of age. During the first hospitalization I read doctor Bach’s book Management Of Patients With Neurological Disorders and Non Invasive Ventilation as well as had the Paediatric Intensive Care Nurses, Respiratory Therapist and Physio Therapist train me so that I could deliver the intensive care Shira needed at home. Through the years my skill set has increased from watching other parents via video conferencing as well as the exchange of ideas over the SMA support inc. chat. Shira was recently hospitalized and again I picked up some new skills. I hope these suggestions can help you.

The information below is specific to Shira but as I have learned from so many parents that have had great success caring for their children during times of critical illness and have applied their methods to Shira’s care. By no means do I suggest that this is a complete protocol or the only way of delivering critical care. If you have any suggestions for me please forward those as I am always willing to broaden my skill set in Shira’s care.


Shira is connected to an Oxymeter 24/7 but this is especially true during times of acute illness. Shira’s Oxymeter is set at 70 for the HR low and 175 for the HR high. Oxygen alarm is set at 90 for the the low and no set for the high. The Oxymeter warned us too many times to count in the past. Doctors were worried that we would watch it but I don’t. Remember when the alarm goes off always check the patient first!!! Machines can lie!

Before giving Shira her CPT session!

Before giving Shira CPT I will turn off her food and make sure to aspirate her stomach (remove its contents via 60cc syringe). This will help insure Shira does not throw up during her session and aspirate her stomach contents.

How often should I give my child Chest Physio Therapy or CPT?

In the hospital you will here the doctors and nurses use the term Q2 or Q4 for delivering treatments and drugs. These are the intervals the delivery will occur i.e. every 2 hours or every 4 hours etc.

When Shira is sick with a cold I give her CPT including cough assist treatments every 4 hours and ramp it up to every 2 should need be.

Bipap and CPT: This time around I learned from another parent to leave Shira on bipap while giving her CPT so that Shira doesn’t crash. The other advantage of giving CPT on bipap is that your child will have more inflation and air flow allowing for a more aggressive treatment.

How often should I use the cough assist?

I use the cough assist during every CPT treatment. If Shira is having trouble coming off her bipap during these sessions and her Oxygen levels are low I will bleed in oxygen in line into the cough assist during treatments. The level of oxygen I use depends on how sick Shira is and to what level her Oxygen is. I will also give Shira coughs and vibes (hand technique for shaking the chest to loosen secretions) in between CPT sessions as needed. I have Shira’s bipap on her head so that when the CPT session is finished I just have to pull down the mask over her nose.

Suctioning when and how often?

Conventional medicine suggests that suctioning can promote secretion development. Because SMA Type 1 children have a difficult time swallowing either having an inaccurate swallow or no swallowing capability I say suction as needed!!! Shira has used 10 FR suction catheters for her oral secretions since 6 months of age and 8 FR for nasal suctioning. I only nasal suction when Shira is critically ill otherwise I don’t nasal suction as the nose bleeds easily and can become easily infected.

Thick Secretions: This time around Shira developed intensely thick secretions and I used 12 FR catheters for her mouth and bebonkers for her nasal suctioning. For deep nasal suctioning I used 8 FR. Catheters and I lubricate the catheters with medical sterile lubricant.

Vaso Vagal Nerve Response: When deep suctioning through the mouth it is not uncommon for our children’s vital signs to suddenly drop i.e. heart rate thenO2 and the minute you stop it regain itself. This is known as a Vasal Vagal Nerve Response. Be careful to watch the Oxymeter while suctioning in case this happens and pull the suction catheter to the front of the mouth should it happen and wait for the HR and O2 to regain itself.

Throwing up what should I do?

1) Immediately turn your child on their side and deep suction to the epiglottis be careful not to scrape anything on the way down.
2) Make sure you open their g-tube and let it drain or take a 60ml syringe and aspirate their stomach (remove its contents) so they don’t throw it up and aspirate (breath in ) the stomachs contents.
3) If you are comfortable using the cough assist you can put it on manual mode and time the exhale with your child and give them a couple of big exhales followed by suctioning to remove any stomach contents that are accessible to the suction catheter.
4) I then give Shira some extra coughs bleeding in O2 with the coughs with both inhale and exhale. If your child is really unstable at this point and might throw up again forego the coughs as you don’t want to induce vomiting and the possibility of aspiration.
5) Stomach Contents: These can be re injected into the stomach after aspirating it as it contains the healthy bacteria found in the stomach.
6) Feeding: See Food How Much How Often below

Oxygen. How much how often?

I follow the NIV Protocol recommendation of O2 being delivered at <95%. I’ll add just enough O2 to bring her back to 97% .

Ambu Bag: Should I have one?

I find the ambu bag the most effective way to give restful deep breaths to Shira after a major extreme bradichardia event or mucous plug removal. Again Shira’s bipap mask will be half on with the mask sitting on her head with her face exposed so that I can give breaths. Be careful not to over inflate your childs lungs while using an ambu bag. Again I will run O2 inline with the ambu bag for giving breaths if Shira’s O2 is <95%. The wonderful thing about using an ambu bag is that you can perfectly time the breaths with the patient so that the patient doesn’t have to change their timing which you have to do with cough assist. I strongly suggest learning how to use one of these bags effectively as they are easy to carry with you. I have one with me where ever I go. If you child plugs while on a walk you can use the ambu bag and supportive coughs to remove the plug and keep bagging them until you get to your vehicle where you have cough assist and bipap waiting. Some parents only walk with both bipap and cough assist with them. It always depends on your skill level and comfort zone.

Nebs: How We Deliver Them

Nebulizer treatments are a main part of critical care protocol for respiratory illness. Shira isn’t on any broncho dilators when healthy but in the hospital she receives treatments of Flovent and Ventolin. Sometimes other broncho dilators are used but you will have to decide on which ones depending on illness and severity. The first time Shira was hospitalized we took off her bipap and delivered the medicine through a spacer set up which was very difficult and looking back ineffective when the patient couldn’t breath off of ventilation. This time we delivered the medicine by using the ambu bag, and bagging it in while also running in O2. This was very effective and in my opinion helped shorten Shira’s hospital stay. The less we make our children crash and still deliver affective treatments the quicker they recover from what I experienced this time.

Food: How Much How Often

There are many variations as to what to do depending on what is ailing your child. With Shira I tend to put her on just her vivonex which is double diluted. I then adjust the mixture so that Shira gets 70% of her daily vivonex and 30% pedialyte. It is important to make sure that Shira is urinating and is not becoming dehydrated and that potassium levels are kept up. And again if Shira really isn’t doing well I’ll go into the hospital and have blood work done to make sure all is well. Shira is on a bolus feed so I drop her feeds to a continuous feed. I still make sure I vent and flush Shira at her regular intervals. If Shira has been vomiting I’ll put her on an open vent using a 60cc syringe running her food line into the open syringe.

Bipap: How high do I go with the pressures?

Shira is typically on fairly low bipap pressures to begin with. PICU doctors will increase these pressures and then do blood gas tests to check ventilation. We obviously do not have this option at home. Seriously over ventilating a patient can be harmful especially on the EPAP not allowing enough CO2 to escape so its important to watch your child carefully if you are playing with pressures. Parents that have been at this a long time know the ranges that work for their children in times of good health and in bad. I know for Shira she can handle moving from 14/4 to 18/7 when sick while her back up rate pretty much remains the same. If you are unsure what you are doing consult with your Pulmo doctor.

Hospital: When Do We Go?
When I can no longer keep Shira’s O2 at 95% while giving supplemental O2 I will take Shira in for a mucous swab and an X-ray on her chest. Every family has a different protocol for this but you will know when you feel like you are loosing control. I don’t like to wait to be totally out of control of the situation before heading to the hospital and prefer to be more pro active so if Shira completely crashes I have a team behind me. Again some parents are able to manage severely sick children at home but you need a lot of experience delivering nebs but more importantly a high skill level in reviving your child and removing extreme mucous plugs. I say, “It’s better to be safe than sorry.” Just listen to yourself and follow your intuition.


Shira Update

Well we have been back from the hospital since the 24th of December. When Shira was sick she was on bipap for about 17 days straight which means she became dependent or biapap dependent. Bipap is bi level positive airway pressure and is the type of respirator she uses. This type of ventilation is considered non invasive vs. tracheostomy which is considered invasive because it takes a surgical procedure to introduce the system to the body. Anyways, we left the hospital while Shira still had a little adolectasis in her left lung but she was well enough at that point to be managed at home. Because we have a battery back up system and true sign wave inverter we were able to transport Shira back home while on her respirator. We took an ambulance from the hospital because it is so much easier to load her onto a gurney with all of her equipment, slide her into the ambulance and have the extra 2 sets of hands to bring her into the house. Once home the recovery really started. It was so great to get out of the toxic hospital air and lighting. Our first days at home were spent getting Shira slowly off of bipap and getting her food back up to speed. Shira is fed through a tube in her stomach and has been fed this way since 6 mos. Of age. Shira is now on her regular feeding schedule and volume as well as spending about 6 hours a day off of bipap. Yesterday was our first walk in 22 days which is way too long to be indoors although I remember being inside for more than 3 mos. At a time when Shira was first diagnosed. Shira is a little weaker than before going into the hospital but it is my hope that she will gain back her strength. We have been spending our days playing, learning, Shira is a real sponge. You don’t notice what she knows or doesn’t knows because expressing herself takes a lot of effort but then you ask her a question out of the blue and she gives you the answer or sings something on her own. She knows so much and can almost read. If you just sound out a word she’ll tell you all the letters. Shira loves to finger paint and do all sorts of art work. I am going to build a new repair shop in my basement for repairing musical instruments and making adaptable devices for Shira so that she can do more. We are looking forward to getting back on track with learning how to drive Shira’s power chair. Oh I forgot to mention that Help Fill A Dream approved us to build an accessible playground in our back yard with a special swing for Shira so she can play with Sam and other kids. We are really looking forward to some better weather so that can happen. Also, ChaiLifeline is sending Maxine and Sammy to Disney World in Florida in January on their way to visit Bubbe Ruth in Hollywood Florida. Maxine has a cousin with a little girl slightly older than Sammy that lives near Disney Land so they will get a chance to visit with them. Shira and I are going to start to run again and we’ll see how it goes. I have some foot problems these days due to many years of sports and I’m about 30 lbs over weight so hopefully some weight loss will take care of the foot problems. Shira and I want to try and run at least the half marathon next October again and beat our time of 2:22 but we will train for a full marathon in the hopes of doing that as a fundraiser. I have to thank everyone again for the love and support so many of you gave us while in the hospital. These life and death situations with our kids are gut wrenching and take an enormous amount of energy and concentration to get through. The love, prayers and contact make it a lot more easy to make it through the critical moments. Maxine, Sammy, Shira and I wish all of you a Happy, Healthy, Successful 2009!!!!!!