My Care



Photos and Descriptions of my Equipment

Corner Seat

This chair has been a fabulous way for Kaitlyn to see her surroundings and not get left out.  The corner chair supports Kaitlynís body from her head to her toes, an absolutely fantastic chair.  It tilts back almost flat and the head rest, lateral supports and seat to back angle are all adjustable.  We have added castors to the bottom so that Kaitlyn can be moved from room to room.  Since she has lost her swallow at 13 months it has become extremely difficult to place Kaitlyn up in her seat, but we are hopeful that as she gets a little older she will again be able to sit up (this seems to be the case for other older Type 1 children).

 

Corner Seat
Max EasyS Stroller/Wheelchair with Homemade Tray for Dora DVD's Max EasyS Stroller/Wheelchair with Homemade Tray for Dora DVD's
Max EasyS Stroller/Wheelchair

In selecting a stroller for Kaitlyn we did a lot of research.  Nothing could compare to the Max Easy S stroller.  It is designed to support medically fragile children like SMA type 1.  It can fully recline, and can adjust to hold larger children.  The vent trays are a must, travel with a Type 1 child is not easy.  This stroller can hold all Kaitlynís machines. 

Max EasyS Stroller/Wheelchair
Gel Cell Battery and Pure Sine Inverter

These are absolutely essential pieces of equipment as we need a way to run Kaitlyn machines when we are out and about and in case of power failure. The cough-assist is the one piece of machinery that is tricky to run. You need a pure sine wave to run the cough-assist. This requires a special type of inverter. The battery isnít as specific, but the lighter, smaller cells are certainly the gel cells. We also have a deep cycle battery and a UPS(which our equipment is always plugged into in case of power failure there would be no interruption in Kaitlynís ventilation). The UPS alone can run our Bi-pap for several hours at Kaitlynís current settings. But the cough-assist can only be used with the pure-sine inverter and the gel cell.

Gel Cell Battery and Pure Sine Inverter
Night Hand Splints Night Hand Splints
Deep Cycle Battery and Battery Charger
Deep Cycle Battery and Battery Charger
Homemade Bath Sling Homemade Bath Sling
UPS Backup Powersource UPS Backup Powersource
Profile Lite (Small Child) Mask with Nebulizer In-line Profile Lite (Small Child) Mask with Nebulizer In-line
Laptop, Microlight Switch and Computer Interface Laptop, Microlight Switch and Computer Interface
Nasal Aire Petite (size E) Interface Nasal Aire Petite (size E) Interface
EZ-On Vest

Kaitlyn lost her ability to manage her own secretions around 13 months of age.  Sitting up on an incline caused her own saliva to choke her.  Kaitlyn couldnít travel in a conventional car seat any more.  We now use the E-Z On Modified Vest and Kaitlyn travels laying flat on the bench seat in the van.

EZ-On Vest

JH Emerson Cough-Assist Insufflator-Exsufflator

Kaitlynís cough is very weak and ineffective and she is unable to clear her own secretions. With a mask that covers her mouth and nose, and mechanical pressures of +40 cmH2O and -40 cmH2O, the Cough-Assist forces air into Kaitlynís lungs, and then sucks the air back out really fastósimulating a cough.  It is because of this machine that we are able to keep Kaitlynís lungs clear. Click Here for More Information on the Cough-Assist

Devilbiss Portable Suction Machine

This machine is very important for Kaitlyn.  The suction machine is used in conjunction with the Cough-Assist and any other time she may need to clear her nose and/or throat.  Because Kaitlyn cannot swallow we use this machine a lot to clear her mouth of excess saliva.  During colds we sometimes have to suction Kaitlyn every few minutes around the clock.

Nellcor Oximax N-560 Pulse Oximeter

Kaitlyn wears her Pulse Oximeter when on Bi-pap, using Cough-Assist, when ill, or while travelling in the van.  This machine is a great tool to aid us in avoiding potential problems, but we do not rely solely on this machine to detect all problems.  We watch Kaitlyn relentlessly on and off her Oximeter.  

Resmed VPAP III ST-A  Bilevel Non-Invasive Ventilator

Kaitlyn began using non-invasive ventilation at ten months of age.  The Bi-pap allows Kaitlyn to take bigger breaths and expand her lungs more fully than she would be able to do on her own.  This gives Kaitlyn a more recuperative sleep and more energy for the following day.  When healthy Kaitlyn uses the Bi-pap only while sleeping, but when sick she sometimes needs continuous support.

Zevex Enteralite Infinity Enteral Feed Pump and Mini Backpack

Kaitlyn began losing her swallow when she was seven months old.  She had a G-tube placed at nine months, followed by a GJ-tube at 11 months.  Kaitlyn now has a G-Button with Nissan and is on continuous feeds for 20 hours a day.


Cough-Assist

The Cough-Assist is a very important machine in Kaitlynís daily care.  Kaitlynís cough is very weak and ineffective to clear her own secretions.  With a mask that covers her mouth and nose the Cough-Assist forces air into Kaitlynís lungs, and then sucks the air back out really fastósimulating a cough.  It is because of this machine that we are able to keep Kaitlynís lungs clear.                           

Kaitlyn uses the Cough-Assist several times a day.  We begin using the Cough-Assist as soon as Kaitlyn wakes up in the morning before she even gets out of bed.  This clears her airway of any accumulated secretions and mucous from overnight.  We then do Kaitlynís chest percussions followed by more coughs.  We do chest physio three times a day, always followed by Cough-Assist. (What the percussions loosen up the Cough-Assist helps to get out.)  When Kaitlyn is ill this machine can be used every few minutes to keep the airway clear.  We also use this machine to clear airways when Kaitlyn chokes on her own secretions. When the child is old enough they can ask to be coughed, for Kaitlyn we use the Cough-Assist to reverse oxygen desaturations due to secretions in the airway.

For Kaitlyn we use mechanical pressures of +40 cmH2O and -40 cmH2O on the automatic setting, with inspiratory-expiratory times of 1.5 seconds each and a pause of 1 second.  We deliver five continuous inhale-exhale breaths, followed by immediate suctioning.  We do five rounds of this each session.  We find Kaitlyn coughs better when a good seal with the mask is achieved and she is sitting up a bit. Supporting her head and neck and tilting her head back a bit to open the airway also helps to get a good cough.  These are tricks that we have learned to help Kaitlyn but every child is different.  It is really important to work with your health care provider when trouble shooting with this machine.  Every child is different, and lots of approaches work!  You need to find what works best for your child.


My Routine

7:00 AM -  Glycopyrrolate  1.4 mL(0.5 mg/mL),  Salbutamol  1.8mL(0.4 mg/mL).  Medications are given via G-Button with a 10 mL warm water flush. 

  • Turn off Kaitlynís feeds in preparation for morning breathing treatments. 

8:00 AM - Kaitlyn is usually awake by this time.  Begin morning breathing treatments.

  • Remove Kaitlynís Bi-pap mask and suction her mouth really well.  Secretions are always much thicker in the morning.

  • Begin with 5 rounds of 5 breaths Cough Assist.  Insufflation pressure +40 cmH2O; exsufflation pressure -40 cmH2O.  Inhale 1.5 seconds, exhale 1.5 seconds, pause 1 second, or slightly less.  Kaitlyn should still be on Oximeter at this point.

  • Always leave Kaitlynís G-Button vented during Cough Assist treatments.  We vent the G-button by attaching her extension tube to a sixty cc syringe hanging above her.

  • Suction out mouth after every round of Coughs.  Suction nose as needed, but should have at least one good cleaning out each time the Cough Assist is used.  This means at least once during the 5 rounds not once every round, unless she needs it.  Lubricate the catheter with saline solution before placing in the nose.  Suction only the nares.  Do not deep suction Kaitlyn as this causes edema and desaturations. 

  • Diaper change and G-Button stoma cleaning.  Coat diaper area really well with zinc cream as Kaitlyn has a tendency to rash.  If her bum is really rashy or red then we use the Critic-Aid Clear.  Clean stoma with sterile saline and Q-tips.  Dry really well.  If stoma site looks red a thin coat of Milk of Magnesia can be rubbed on with a Q-tip.

  • Morning medications:  Valproic Acid  4.4 mLs (50 mg/mL),  Zantac  2.3 mLs (15 mg/mL),  and Carnitine 1.8 mLs (100 mg/mL). Medications are given via G-Button with a 10 mL warm water flush. 

  • Morning chest physiotherapy.  Front upper and lower lobes, sides and back 5 minutes each position.  Chest physio may be done in postural drainage if needed.

  • After chest physio, suction and then move Kaitlyn over to her crib and brush teeth with a couple of drops of Oral-B mouth rinse.  Brush teeth and gums, tongue, inside of cheeks, and roof of mouth.  Suction mouth immediately.  Rinse toothbrush under tap and repeat. 

  • Finish morning breathing treatment with more cough-assist.  5 Rounds of 5 breaths, suction out mouth after every round.  Suction nose as needed; clean out at least once in the five rounds.  Always place Kaitlyn on Oximeter before beginning Cough-Assist.

  • Always vent Kaitlynís G-Button and elevate extension before beginning Cough-Assist.

  • We have not added postural drainage as a part of Kaitlynís daily routine.  But if she were sick we would now do 20 minutes postural drainage, suction, followed by another round of Cough-Assist.  This would be added to every breathing treatment until Kaitlyn was better.

  • After all this Kaitlyn likes to go out and play on the floor with her brothers.  She will tell you ďall doneĒ, ďoutsideĒ, or ďgo...brothersĒ. ROM is worked into playtime.

Note:  Kaitlyn may remain on Oximeter all day, but we prefer to give her a break, as she can never be left alone anyway.  More problems can be avoided by just watching Kaitlynís expressions and learning her signals when something is wrong.  For this reason we watch Kaitlyn continuously when not on Bi-pap and Oximeter. When Kaitlyn is sick she is on Oximeter 24/7! This is important if she should crash.

Between 7 and 9:00 AM-   Whenever there is time, ASAP, mix up Kaitlynís formula for the next twenty-four hours.   Formula recipe is found below. We restart Kaitlynís feeds as soon as morning physio is done, never later than 9:00 AM.  Two hours is the maximum we like to fast Kaitlyn.

9:00   AM-     Begin daily feeds to be run at a rate of 60 mLs per hour and clean feeding equipment.

  • 1 tsp.  Acidophillus dissolved in 5 mLs cool water via G-Button with a 10 mL warm water flush.

  • Wash her 90 degree extension with warm soapy water.  Soak in vinegar and water (two parts water one part vinegar) and then flush really well in hot water.

  • Rinse out and clean Infinity enteral feed bag.

  • Cleaning:  place one drop of dish soap into feed bag with hot water, remove air and replace cap.  Rub sides of feed bag together being careful to get into corners.  Allow to run through line.  Then rinse bag several times with fresh hot water and allow fresh water to run through tubing.  Rinse bag again with one part vinegar to two parts hot water.  Then rinse really well.  Let lots of fresh water run through the tubing.

  • Kaitlynís feeds run at a rate of 60 mLs per hour through her G-Button for 20 hours per day.

  • Add a freezer pack to Kaitlynís backpack.

12:30 PM-    Kaitlyn usually goes for a nap around 12:30 PM, never later than 1:00PM. 

  • Kaitlyn sleeps always on Bi-pap.  Lay Kaitlyn in her crib after changing her diaper.

  • Place Kaitlyn on Oximeter.  Never place Kaitlyn on Bi-pap without Oximeter.  Reset Oximeter default low O2 sat to 94% and default low heart rate to 70 beats per minute.

  • Vent Kaitlynís G-Button and attach to 60 cc syringe.

  • Suction Kaitlynís mouth.  Do a couple rounds of Cough Assist and suction to clear her airway before putting on her Bi-pap mask.

  • Turn on ventilator before placing mask.  Make sure the Bi-pap mask is snug but not tight.  Suction Kaitlynís mouth one last time after the mask is placed.  Raise Kaitlynís G-Button extension and lightly squeeze Kaitlynís tummy to help any trapped air out the tube.  Place Kaitlyn on her side to sleep so she doesnít aspirate saliva.  Make sure that Kaitlyn is high up in the crib.  There should be a soft blanket under Kaitlynís head to support her neck, and protect her ear.  Pull Kaitlynís clothing straight, and make sure she is not lying on any wrinkles.  Place a rolled blanket between her legs and snug up to her bum so she wonít slip down in the crib.  Place a rolled blanket at her back to keep her on her side.  Make sure that her neck and back are straight, and that she is looking forward and not down.  She cannot breathe properly with her chin on her chest.  Put her stuffed bear between her arms to support the upper arm (and for company).  Place her stuffed bunny on its side facing her level with her eyes.  Place Dora or anything else she is asking for where she can see them!!  Cover Kaitlyn with a light blanket.

  • Kaitlyn can be left in her room alone while on Bi-pap with Oximeter on, but she must be checked on every few minutes.  She could be in trouble or crying long before the alarms sound off!  The sixty cc syringe venting Kaitlynís G-button also needs to be checked often.  If air gets trapped in the line the syringe will fill and overflow.

1:00  PM-     Glycopyrrolate 1.4 mLs (0.5 mg/mL),  Salbutamol  1.8 mLs (0.4 mg/mL) 10mL water flush.

1:00  PM-    Turn off formula before afternoon breathing treatment.  Rinse feed bag with hot water.

2:00  PM-     Kaitlyn is usually awake by now.  When awake, remove Bi-pap mask and suction.       

  • Change diaper.

  • Begin afternoon chest percussions.  See morning treatment.

  • Cough Assist:  5 rounds of 5 breaths.  Suction between rounds.  See morning routine.

  • Bring out for more play with the boys.  Lots of position changes, enabled toys and creative play are required to keep this girl busy!  Her portable DVD player is never far. The girl is hooked on Dora! When nothing else works, and her diaper is clean, sometimes she just wants to ďwalkĒ around the apartment on a shoulder.  A change of scenery usually perks her up! ROM a great way to play!

3:00  PM-     Restart Kaitlynís feeds.  Add a new freezer pack to Kaitlynís backpack.

  • Afternoon medications:  Valproic Acid  4.4mL (50 mg/mL),  Zantac  2.3 mLs (15 mg/mL ), and Carnitine 1.8 mLs (100 mg/mL)  given via G-Button with a 10 mL warm water flush. 

5:30  PM-     Bath time!  Kaitlyn loves her bath.  Turn off feeds in preparation for evening physio.

6:30  PM-     Evening chest physio. Followed by Cough Assist, 5 rounds of 5 breaths. See morning routine.

7:00  PM-     Glycopyrrolate 1.4 mLs (0.5 mg/mL),  Salbutamol  1.8 mLs (0.4 mg/mL).   10mL warm water flush.  Restart feeds at 60 mLs an hour to run all night.

8:00  PM-     Bedtime! 

  • Change diaper.  Put on size 6 diaper to sleep.

  • Follow the steps for placing Kaitlyn down for an afternoon nap.

  • Plug in Kaitlynís feed pump, and make sure there is a freezer pack in her backpack.

  • Kaitlyn sleeps with a night light so that we can see to help her in the night.

11:00 PM-   Top off Kaitlynís formula before going to bed.  Check freezer pack and change if necessary.

  • Evening medications:   Valproic Acid  4.4mL (50 mg/mL),  Zantac  2.3 mLs (15 mg/mL ), and Carnitine 1.8 mLs (100 mg/mL).  Medications are given via G-Button with a 10 mL warm water flush.

1:00   AM-   Glycopyrrolate 1.4 mLs (0.5 mg/mL),  Salbutamol  1.8 mLs (0.4 mg/mL).  Medications are given via G-Button with a 10 mL warm water flush.   Diaper change.

From this point on Kaitlyn is left to sleep unless she wakes up fussing.  She is never ignored!   Kaitlyn will only wake up if she needs something.  Usually manually venting her G-button, a position change or adjustment and/or a fresh diaper are enough to send her back to sleep.  Occasionally she needs to be suctioned, or even needs a round or two of Cough Assist to clear her upper airways so she can go back to sleep.  We do not talk to her or play as this is sleep time and we wouldnít want this to change! We help make her comfortable and then immediately go back to bed; she will settle herself, as long as her problem has been fixed.   


My Medications

These are all of Kaitlynís medications, along with a brief explanation of why she is on these medications.

Valproic Acid 50 mg/mL:  dosage 4.4 mL/220 mg  TID (3 times per day)

Valproic Acid belongs to a class of drugs known as histone deacetylase(HDAC) inhibitors. These drugs increase the activity of certain genes in the body. Kaitlyn is missing the gene SMN1 that produces a protein in the body called SMN. A second gene called SMN2, which is almost identical to SMN1, is found on the same chromosome. However, while the normal form of SMN1 produces a full length functional protein, most of the protein produced by SMN2 is truncated and unable to function. The relatively small amount of normal SMN protein produced by the SMN2 gene can reduce the severity of the disease. Therefore, investigators have begun looking for drugs that can increase the amount of normal protein produced by this gene.

They tested Valproic Acid to see if it could increase the amount of normal protein produced by the SMN2 gene. They found that the more drug they gave, the greater the amount of gene activity. The drug also increased production of functional SMN protein. VPA has a mechanism of activation, such that the whole amount of SMN2 RNA is elevated after VPA treatment.

Kaitlyn began taking Valproic Acid at five months of age. She has gained strength in some areas and lost some strength during illness. But overall we feel that she has maintained a lot more strength than other children with Type 1 SMA who are not on experimental drugs.

Carnitine 100 mg/mL:  dosage 1.8 mL/180 mg  TID (3 times per day)

Children with SMA have abnormalities in carnitine metabolism, which can lend to muscle atrophy and progressive weakening. VPA depletes carnitine so Kaitlyn receives supplemental L-carnitine. The recommended dosage is at least 50mg/kg/day (Swoboda).

Zantac 15 mg/mL:  dosage 2.3mL/34.5 mg  TID (3 times per day)

Kaitlyn takes Zantac for reflux. It neutralizes stomach contents making them not so caustic should she aspirate.

Glycopyrrolate 0.5 mg/mL: dosage 1.4mL/0.7 mg QID (4 times per day)

Glycopyrrolate is used to help dry up secretions so that Kaitlyn can breathe without aspirating secretions every few minutes. It makes the secretions she does have more manageable.

Oral Salbutamol 0.4 mg/mL: dosage 1.8 mL/0.72 mg QID (4 times per day)

Salbutamol is a beta2-adrenoceptor agonist and has been shown to improve muscle strength in SMA patients in a pilot trial. Salbutamol increases SMN2 full length mRNA and SMN protein by promoting exon 7 inclusion in SMN2 transcripts. 


Positioning
A Major Cause for Choking & Desaturation

Positioning is one of the most important aspects of Kaitlynís care.  SMA type 1 children have a lot of problems managing their secretions.  The position that Kaitlyn (and most SMA type 1 children) tolerate the best is laying flat with the head turned to the side.  Most children will choke with their heads in a lateral position (looking up at the ceiling).  She can and does sit up, but only for short periods and only while being watched relentlessly as she may need suctioning often and can choke. Kaitlynís most favourite position is on a shoulder travelling wherever she can convince us to go. She canít say many words clearly but we have no trouble understanding go...go...go!

 

The safest position we can put Kaitlyn in is lying on the floor with her head turned to the side.  In this position Kaitlyn can spend hours not needing any, or very rarely any suctioning.

The second safest position is being carried around on a shoulder.  She seems not to have any difficulty handling secretions in this position. She hardly ever needs suctioning in this position.

Side lying is the safest position for Kaitlyn while she is sleeping.  Any secretions produced can be drooled out.  In any other position we have had problems in the past with aspirating secretions.

 

 

 

 

 

Sitting up is Kaitlynís least tolerated position.  She cannot handle secretions as well when sitting up and we have to be very careful in how we position her head.  Suctioning is a must!

 

 


My Diet

Kaitlyn is on an Amino-acid diet.  See Amino-Acid Diet by Mary Bodzo and Letter of Medical Necessity by Dr. John Bach for more information on the Amino-Acid Diet.  
Kaitlyn's current diet is as follows:

  • 2 Packs Pediatric Vivonex by Novartis Nutrition

  • 1 scoop  Nano VM vitamins 1-3 years by Solace Nutrition

  • 1/8 tsp.   Table Salt                       

  • 2000 mg   L-Glutamine                

  • 1 tablet Calcium Citrate(250mg) with Vitamin D3(50 IU)

  • 100 mL  Prune Juice (Not brand specific)

  • 220 mLs   Pear Juice (we use Heinz brand)                                                

  • 7 tbsp.   Pears   (Heinz jarred baby food)

  • 3 tbsp.   Organic Apples  (Presidentís Choice Blended)

  • 520 mLs   Filtered pre-boiled water          

  • 200  mLs Unflavoured Pedialyte to replace lost electrolytes

All these ingredients are added to a blender and blended for a few seconds until well mixed.  This formula is then put into the fridge and used for the next 24 hour period.

This formulation is what works for Kaitlyn now. We have had to add things and takes things away in the past. Whenever we add something new we start out with only a very small amount for a few days to check for tolerance. If she shows any signs of increased secretions, higher heart rate or lower sats then we consider this a reaction and the new ingredient is discontinued. SMA children, in particular Type 1 children, seem to be very sensitive to changes in diet. They are particularly sensitive to high levels of fat, too much protein, milk and soy, and some children canít tolerate high amounts of sugars in the form of juices. The only thing Kaitlyn has reacted to so far are vegetables and added fat (safflower oil and canola oil).

Glutamine is a conditionally essential amino acid and is involved in more metabolic processes than any other amino acid.  Patients with gastrointestinal diseases on parenteral or enteral nutrition have shown a reduction in bacterial infections, and a healthier gastrointestinal tract when taking supplemental glutamine.  Glutamine is the preferred fuel for the cells in the gut, and helps maintain gut integrity.              

Kaitlyn used to have formula back up from her small intestine into her stomach.  Not only should this not happen, but it is extremely dangerous for Kaitlyn. When we switched Kaitlyn onto the Amino-acid diet the back-up wasnít near as bad, but it didnít stop entirely until we researched, networked with other parents, and began supplementing Kaitlyn with L-glutamine.  Kaitlyn is now fed directly into her stomach and so far has no problems with slow gut motility like so many of her SMA peers.  A conservative SMA specialist Dr. Swoboda recommends 200-300mg/kg L-Glutamine. (www.fsma.org)

Kaitlyn gets 200 mLs of unflavoured pedialyte as we believe that she always seems healthier and stronger when she is supplemented with the extra electrolytes.  She loses a lot of fluid through secretions everyday.  She gets an additional 50-60 mLs Pedialyte at bedtime to thin out her formula.

We have recently had Kaitlynís bone density checked, and her levels are low. SMA children, especially Type 1ís, tend to have low density bones and as a result suffer fractures and breaks with very minimal force. Most parents of SMA children choose to supplement their childrenís diet with additional calcium, over and above the recommended RDA. We decided to supplement Kaitlyn before her bones get so weak that they begin to fracture. This wonít prevent Kaitlynís bones from getting weaker, but it may buy us some time.

The only other supplement that Kaitlyn receives currently is her Acidophilus (Dairy  Free).  She receives 1 tsp. (up to 1 1/2 tsp. during antibiotic use) once a day in the morning.  SMA children are known for their yeasty problems.  Smelly fingers and toes, diaper rashes and skin rashing.  Between Kaitlynís fingers and toes, behind her knees and under her neck used to get so raw she would bleed.  We did our best to keep her skin clean and dry but her skin was never very good until we started the Acidophilus.  In most SMA children these problems clear up once acidophilus is begun.  Acidophilus also aids in replacing beneficial bacteria after antibiotic therapy.  We have far fewer problems associated with antibiotic use when Kaitlyn takes Acidophilus.