SMA: Neuromuscular clinic (September 3)

A few days after Khloë was discharged from the hospital, we went to our second Neuromuscular Clinic appointment. We met Dr L, an orthopedic surgeon, who was very friendly and great with Khloë. He liked that her feet aren’t terribly formed yet and he wrote up a prescription for Ankle-Foot Orthotics (AFOs). There’s an ADP form that goes with the prescription as well, which means we will only pay 25% of the cost.

In case I haven’t explained what ADP is, it’s short for Assistive Devices Program, and it’s for residents of Ontario. Once approved, ADP pays 75% of the cost of the device. In this case, the AFOs. We are awaiting ADP approval for the suction machine and feeding pump. Khloë’s equipment is very expensive! Another program we are hoping to be approved for is ACSD (Assistance for Children with Severe Disabilities). If we get approved for that, then ADP will actually cover 100% of the cost of the devices I listed! ACSD is based on our income.

We also saw the physiotherapist (not much to remark) and Kellie, the dietician. Kellie is looking into Pediatric Vivonex, an elemental formula, for when Khloë turns 1 in December.

Dawn, our nurse coordinator, gave us the CoughAssist machine to take home. She suggested we have Khloë play with the tubing and mask to get used to it first. Not sure what happened to the pulse oximeter we were supposed to be given… Both of these pieces of equipment are loaned to us from the Ventilator Equipment Pool at no cost.

Next we saw Dr MacL, a different respirologist. He doesn’t think Khloë needs BiPap just yet, but he wants to do an overnight pulse oximetry at home to get an idea of her sleeping O2 sats. We professed interest in the BiPap because we know Khloë will most likely need the extra breathing support when she gets sick.

And lastly, Khloë was assessed in her car seat with the pulse oximeter attached to her toe. We were trying to see if her blood oxygen level was different while upright. The OT still doesn’t want to help us with getting the car bed from FSMA Canada.

Later that afternoon Jordan and I met with a genetic counsellor. We found out that we are indeed carriers of the SMA gene. No surprize there! In order for Khloë to have Spinal Muscular Atrophy, both of us had to be carriers. The counsellor drew our family tree, explained a few things about autosomal recessive genes that we already knew, and said that she would be mailing a letter to us that we could give to family members. The letter would explain our family history of SMA and would encourage family members to get tested to see if they are carriers of the gene that has changed our lives!

Khloë: 9 months old (40 weeks)

Wow, she’s getting so big!


40 weeks (September 9)
40 weeks (September 9)

So that shirt she’s wearing? It’s size 2T! Must be made small though; her other T-shirts are 12 or 18 month.

Lots of appointments and meetings this week. Here’s what went on:

Tuesday: We met Leona, a dietician with CCAC, for the first time. We talked about Khloë’s current diet and I explained that we would be switching to an elemental formula when she’s a year old. Jordan and I have discovered that any dieticians we deal with are trying to get us to feed our daughter as if she was a child with normal muscle mass. She needs less fats and proteins than a “normal” kid. There aren’t any documented studies on SMA and nutrition. I have spoken with other parents who have been feeding their SMA kids special amino acid formulas, baby foods, and breastmilk and we plan to do the same; but to get the dieticians on the same page is a hard task! These parents are not considered “professionals” to them. She had a portable scale with her and weighed Khloë at 16 lb 13 oz and she measured 28″ long.


9 month collage
9 month collage

Wednesday: Mary demonstrated some infant massage techniques to use on Khloë’s legs. She loved it! Mary lent us a Tumble Form chair to use for Baby Girl, as well. We also discussed the latest car bed issue–that CHEO doesn’t want to deal directly with FSMA Canada, but Khloë’s neurologist is willing to write a prescription for the Hope car bed and EZ-on Vest.

Thursday: A military chaplain came to our house and spoke with us about Khloë’s condition and how it will relate to Jordan’s work. He assured us he’s there to advocate for us and will do his best to make sure we don’t fall through the cracks, so to speak.

Sunday: After church, we had a delicious lunch at Ken & Sandra’s. They let us bring Khloë’s stroller inside and park it in the kitchen. It’s too awkward to hold her on our laps while eating since she needs extra support, and the stroller in the carriage position is perfect for her to lay flat and play in.


Sweet grin

Swing swing swing!

Monday: Taunia (Occupational Therapist) and Christine (Physiotherapist) from CCAC stopped by. Taunia gave us a larger Tumble Form chair. I attempted to feed Khloë while sitting in that chair but she started choking. The recline position is just not right. Taunia also gave us a few ideas for messy and sensory play. Christine just observed Khloë and made sure we were still comfortable doing chest therapy. Later in the afternoon, Kathy (Case Coordinator from Infant & Child Development) met with us for the first time. Her role is to help us with applications, find relief workers, and to offer any other kind of help we need. We had a great conversation with her. She walked us through the application for Special Services At Home, yet another program of funding we would like to be approved for.

SMA: Khloë’s gastrostomy surgery

On Wednesday, August 27th, Khloë was scheduled for gastrostomy surgery. The surgery was planned proactively because we know that eventually, as part of the progression of Spinal Muscular Atrophy, she will lose her swallow and require a new way to be fed adequately.

We found out Tuesday afternoon the date and time of the G-tube surgery. Luckily we had most of our stuff already packed. Before we left, we received a couple phone calls: the first one changed the surgery time from 8:30 am to 10:45 am in order to snag a room in the ICU, and the second was just to confirm we understood Khloë’s fasting guidelines. When we arrived in Ottawa, we checked in at Roger’s House and spent the evening playing with Khloë and watching Netflix.

The following morning we were up early. With a surgery time of 10:45 am, Khloë began fasting at 6:45 am. We headed over to CHEO to register Khloë and then waited around in a special room where no one is allowed to eat or drink. The movie “Frozen” was playing for the few kids in there. Even though she was starting to be hungry, Khloë surprized me and didn’t fuss too much.

Finally, at 10 am, we were seen by a nurse. Khloë weighed in at 15 lb 13 oz. We dressed her in a pair of pink striped infant hospital pyjamas. She was then given a dose of Tylenol. The surgeon, Dr Bass, came in and spoke with us about the surgery. I gave Dr Bass the SMA protocols for surgery; he actually read them over and did his best to set our minds at ease that everything would be fine. Dr Silver, the anesthesiologist we had seen on Friday, also popped in to see us. By 10:30 am we were being led to the OR and had to say goodbye to Khloë. It was so hard to hand her over to the nurse. I gave her lots of kisses and told her we would see her when she woke up. She had no idea why Mommy was handing her over to a perfect stranger, but she wasn’t too upset about it.


Hospital pyjamas (iPhone)

A nurse brought us to the waiting room. I asked if there was a room I could go to in order to pump breastmilk privately. I had a cooler bag and ice packs with me and a single electric miPump. I left Jordan in the waiting room and followed the nurse to the Ronald McDonald ICU waiting room. I was given a special pass to use the waiting room. Inside there was a Lactation Room with bottles for milk storage. I used that room numerous times that day!

After the longest hour and a half of our lives, Dr Bass came to get us at 11:55 am. He assured us that surgery had gone smoothly and so far Khloë was doing well after extubation. He brought us to the Ronald McDonald ICU waiting room and told us to wait there; we would be allowed to see our daughter in about 45 minutes, or when she woke up.


Sleeping angel (iPhone)

When we were finally allowed to see her, an hour later, she was still sleeping peacefully. She had opened her eyes a bit earlier and cried a little before going back to sleep, one of the nurses told us. Seeing her face after surgery was heart-wrenching; she looked so angelic but I still felt terrible that she had gone through such a surgery. I wanted to scoop her up in my arms, but couldn’t. Lots of different wires were attached to her. I noticed that her O2 saturation level was hovering around 96 and not her usual 98. Throughout the afternoon her sats would vary from a low 92 to a mid-range 96. I hoped it was just an effect of the surgery.

When Khloë opened her eyes and saw Jordan and I, she pouted and cried, but quickly stopped, smiled, and fell back asleep knowing we were right there with her. Off and on she would wake and sleep. Around 5:15 pm she was moved to another ICU room where she and I would spend the night.


After gastrostomy surgery

Eating the pulse ox cord

The RN assigned to Khloë’s care, Karen, was amazing! We loved her. She was friendly, helpful, and chatty. She even advocated for us when we were getting concerned about Khloë’s continued fasting–it’s dangerous for an SMA child to fast longer than 6 hours, and 8 hours was really pushing it. Muscle breakdown occurs much quicker in SMA kids and I was worried she would lose some of her strength if she fasted too long. Dr Bass said that normally a gastrostomy patient wouldn’t be given any food, other than glucose through IV, for 24 hours after surgery; however he would allow Khloë to be given breastmilk 8 hours after the surgery.

At 7:30 pm, just shy of a 13-hour fasting period and many conversations with Karen about the importance of giving our daughter nutrition as soon as possible, we were given the go-ahead to feed Khloë expressed breastmilk orally, by syringe. All she managed to drink was 10 ml, a ridiculously tiny amount, but it was something. She was tired and didn’t want to spend the energy trying to drink from a syringe.

Jordan and I left her in the care of the night nurse and walked over to Ronald McDonald House to make dinner. Once we saw Khloë after surgery, Jordan had moved our belongings from Rogers House over to Ronald McDonald House. We made Kraft Dinner and chicken noodle soup.

At 11:30 pm, Baby Girl managed 30 ml of expressed breastmilk (EBM). It took an hour and a half to get that much into her; syringe-feeding is difficult! I could tell she was hungry but the doctor didn’t want me to give more than that amount in case her stomach expanded too quickly. I continued to ask if I could nurse her, but because there is no way to know the exact amount she would drink that way, Dr Bass said “Not yet”.

By early morning, I was finally allowed to breastfeed! My little girl was starving–and probably thought she was never going to get to nurse ever again!–and she drank for 25 minutes straight.

We left the ICU on August 28th and were moved to another wing. The nurses we had were great, but the ward was very noisy and Khloë (and Mommy) had a hard time sleeping. We hoped to go home as soon as possible because it was Labour Day weekend coming up.


Doing great after surgery (iPhone)

Hospital set-up

My little trooper

Her IV was removed August 29th since she no longer needed the extra glucose. The extra fluid was causing her diapers to leak everywhere. She had begun to feed by G-tube the night before. I would breastfeed her and then we would give a 2 oz supplement of EBM by G-tube with the Kangaroo feeding pump. She tolerated her new feeding schedule very well.

After a frustrating discussion with two dieticians and a meeting with Dr Bass, we came up with a temporary diet solution. The main concern was that Khloë’s weight was down compared to our previous Neuromuscular Clinic visit; she could not be discharged until she gained weight. We decided that I would allow Khloë to nurse and then tube-feed 2 oz EBM every 3 hours. While she was being fed by G-tube, I pumped milk with the hospital’s Medela. Overnight she was tube-fed Enfamil A+ formula for extra calories. My heart broke a little when we started that first formula feed, but I understood the benefits.

By August 31st, her weight had gone up to 16 lb 5 oz. The general surgery team was satisfied with the weight gain and discharged Khloë into the care of Rogers House. Our rental Kangaroo Joey enteral feeding pump and supplies arrived and we moved out of CHEO and over to the relaxing, stress-free atmosphere of the family suite at Rogers House. It sucks that we were stuck in Ottawa over the long weekend, rather than with family in Kingston as we had originally planned, but there was no point going home since Khloë had Neuromuscular Clinic in a few days’ time. We were also given a few cans of Enfamil A+ concentrate to get us through the long weekend!


Chillaxing at Rogers House

Happy girl

Before we were allowed to leave, we had to be confident with changing Khloë’s G-tube dressing and with enteral feedings. I think Jordan was much more confident than I was, but the hospital environment was stressing me out too much to really feel like I knew what I was doing. As soon as we were in our new location, though, I was able to set up feeds by myself!

We are so thankful that our daughter got through her first surgery with no ill-effects! She has so many people praying for her and sending positive thoughts; we appreciate this so much. Khloë is such a sweet, special girl. Anyone who meets her instantly falls in love! In the words of the anesthesiologist, who made a special visit to see Khloë before discharge, our little girl “captures hearts”.

Khloë: 9 months old (39 weeks)

Khloë turned 9 months old the day after her “week” began:


39 weeks (September 2)
39 weeks (September 2)

The majority of this week was spent in Ottawa at Rogers House for recuperation. The palliative care home always treats us well and everyone is so loving toward Khloë. We cared for her ourselves but the nurses still popped in during shift changes to say hello and to see if we had any questions. Jordan and I realized that once we were away from CHEO our stress levels dropped!


Purple girl

Playing with pearls

We came home Thursday and it was such a relief! Khloë must have missed all the familiar things of home because she had the most wonderful smiles for everything. When I put her on the change table, she looked up and gave a big grin to the photos of her cousins above her head.

Oh, and my little girl has her first tooth!

You're just as sane as I am. — Luna Lovegood (H.P.)