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: 8 months old (38 weeks)

My little girl:


38 weeks (Aug 26)

38 weeks (Aug 26)


This was a crazy week! We spent August 26th to September 4th in Ottawa while Khloë had surgery and recovered. Unfortunately we got stuck at CHEO over the long weekend and had to cancel our trip to visit family in Kingston.

I’m not going to go into much detail about our hospital visit because I want to save that for a separate longer post.

On the Tuesday of this week, when we finally received confirmation that the G-tube surgery would take place the following day, we loaded up the car with all our stuff–I think we need a bigger vehicle already–and made the familiar drive to Ottawa. Khloë recovered well after Wednesday’s surgery and only had to spend one night in ICU. We had a much longer stay at the hospital because Khloë hadn’t gained any weight since the beginning of July and the dietician wanted to make sure we had a diet plan in place. When she was finally discharged on Sunday, Khloë had gained enough to satisfy the doctors.


Last photo shoot before surgery

Getty Owl bracelet

We spent Labour Day relaxing in our family suite at Rogers House or out for a walk around the neighbourhood. Khloë liked being out in her stroller, in the carriage position, watching the sky and trees overhead. We dressed her in T-shirts and pants rather than onesies.

Update on G-tube surgery

Once again I find myself behind on updates, but I have a really good excuse. We found out on Tuesday of last week that Khloë was scheduled for gastrostomy surgery on Wednesday, August 27th. We packed up the car and drove to Ottawa and have been here for a week now. Khloë was discharged from CHEO on Sunday and transitioned to the care of Rogers House, where we stayed together. There was no point in driving the 2.5 hours home only to have to come back for Neuromuscular Clinic today.

Tomorrow we will be going home, at last! Once we settle in I will prepare updates as quickly as possible. Khloë is doing great and I am looking forward to getting back into our daily routine.

Khloë: 8 months old (37 weeks)

Here we go:


37 weeks (August 19)

37 weeks (August 19)


At the beginning of the week CCAC sent an Occupational Therapist and Physiotherapist to our home to meet with Khloë. The ladies were very friendly and Khloë gave them lots of smiles. I showed the OT the feeding chair and the infant rocker chair we currently use and explained that, although they work fine right now, Baby Girl is growing quickly and will soon be too tall for these chairs. Since she can’t support her head when upright, Khloë needs to be partially reclined when sitting in either of her chairs. These chairs are meant for babies who develop into strong toddlers who can hold their heads up and don’t need extra support. The OT said she would look into finding us a tumble form chair.


Beauty

On Wednesday we went to Prenatal Nutrition. In the late afternoon, Mary from Infant Development came and worked with Khloë for an hour. We agreed that she would benefit from infant massage and we will start that in September.

The most important part of the week was Khloë’s appointment at CHEO on Friday. We met with an anesthesiologist to discuss Baby Girl’s upcoming G-tube (gastrostomy) surgery. It looks like surgery will take place next Wednesday, August 27th, but we must wait for confirmation. In our original discussion with the surgeon, we were told to expect three days in the hospital for recovery and training on how to use the feeding pump. I voiced my concerns to the anesthesiologist about fasting, as it’s not good for an SMA child to go more than 6 hours without adequate nutrition–Khloë will need to fast starting four hours prior to surgery. Other SMA families advised us to have PPN (peripheral parenteral nutrition) given to Khloë, but even though I asked for it and the doctor said she would make a note about it on Khloë’s chart, I doubt she will get it. We were told that it’s not protocol at CHEO to do PPN.

The following quote, taken from the “Consensus Statement for Standard of Care in Spinal Muscular Atrophy”, is on the dangers of fasting:

Spinal Muscular Atrophy patients are particularly vulnerable to catabolic and fasting states [and] are more likely to develop hypoglycemia in the setting of fasting… Thus, it is necessary to avoid prolonged fasting, particularly in the setting of acute illness, in all Spinal Muscular Atrophy patients. Nutritional intake should be optimized to meet full caloric needs within 4 to 6 hours after an admission for acute illness, via enteral feeding, parenteral feeding, or a combined approach as necessary. Prompt postoperative caloric supplementation is recommended to avoid muscle catabolism, particularly in a child with reduced fat store.

As long as the surgery goes well, she’ll be given Pedialyte; if she tolerates that and has been extubated with no breathing issues, I’ll be able to breastfeed. If Khloë is too tired to nurse, I can give her pumped milk with a syringe. I have no idea how long she will end up going without eating if she isn’t given PPN and can’t breastfeed right away.


Outdoor<span class= #1" width="720" height="480" class="alignnone size-full wp-image-3931" srcset="http://kyslota.net/wp-content/uploads/2014/08/102_2014_08_19_9999_67.jpg 720w, http://kyslota.net/wp-content/uploads/2014/08/102_2014_08_19_9999_67-300x200.jpg 300w" sizes="(max-width: 720px) 100vw, 720px" />

103_2014_08_19_9999_84

Catch that tongue

On a happier note, my sweetpea has added the sounds “guh guh” and “ba ba ba” to her repertoire of baby babble. She’s being saying “mum mum mum” for awhile now but usually while upset. She even mouths it without the sound coming out, which is really cute. She also tries to copy me when I make a certain noise with my tongue. It’s hard to describe but she’s fascinated by watching me do it!

Khloë: 8 months old (36 weeks)

I’m totally loving little baby skirts:


36 weeks (August 12)

36 weeks (August 12)


It has been two months since Khloë’s diagnosis of Spinal Muscular Atrophy (Type 1). I never thought that we would get through those months without some sort of emergency popping up, but I am happy to say that our baby girl is doing okay! She can still breastfeed, eat pureed foods, laugh, smile, and play with her toys. That is amazing!


Chapstick lover

Flowers and pearls

On Thursday I spoke with Dawn, the neuromuscular nurse coordinator at CHEO. She gave us the news that Khloë was placed on the emergency elective surgery list. This means that Baby Girl will be getting a G-tube put in within the next few weeks. We don’t have a date yet, but the surgery requires a 3 days hospital stay. We are doing the surgery proactively, as Khloë will eventually lose the ability to swallow. She can still have the G-tube and eat orally.

We had another visit from the nurse this week to finish paperwork.

Khloë weighs 16 lb 4 oz, is 28 inches long, and is still wearing size 3 diapers.