Oliver: 6 months old

Here is what’s been happening in the past few months!

2 MONTHS OLD
December 23 – January 22
– Daddy was very sick so we stayed home for Oliver’s first Christmas instead of going to family celebrations in Kingston
– We went to our church’s Candlelight Service on Christmas Eve
– On Christmas Day we Skyped with everyone at Oma’s
– On December 31st Oliver received his second RSV shot; that evening Uncle Luke & Aunt Katelyn arrived for a 4-day visit
– Oliver weighed 11 lb 14 oz on January 6th, length 23 inches, and head circumference 40 cm; this showed no weight gain since end of December
– Drove to Kingston on January 7th for the weekend; hung out with Oma, went shopping with Aunt Keesje, and Oliver met my Uncle Dale and Aunt Margaret
– Oliver weighed 12 lb 3 oz on January 13th
– January 16th marked one year that my baby girl Khloë has been in Heaven
– On January 21st Oliver received his fourth injection of Nusinersen (IONIS-SMNrx) in Boston with Dr Swoboda; he had the CHOP-INTEND physio test in the morning and injection was scheduled for late afternoon in the OR with sedation; he weighed 12 lb 5 oz, length 23.5 inches, head circumference 40 cm, chest circumference 40.5 cm
– Packed away Oliver’s 3 month onesies and pulled out size 6 month; he was still in size 2 diapers


2 months old

2 months old

3 MONTHS OLD
January 23 – February 22
– Kicking his legs, waving his arms and holding his head up are major milestones
– Oliver was becoming interested in toys dangling in front of him; he smiled and cooed a lot
– Jordan and I celebrated 10 years together on February 2nd
– Oliver weighed 12 lb 9 oz on January 28th; this showed very little weight gain in 18 days and a half inch gain in length; Dr Swoboda recommended a swallow study to rule out swallowing issues
– He started showing signs of silent reflux (twisting and arching while breastfeeding, crying, needing to be upright often); he was prescribed ranitidine
– On February 4th Mary from Infant Development met Oliver; later he received his third RSV shot
– Nanny & Papa visited from February 5 – 10th; Oliver enjoyed having “conversations” with them
– Oliver lifted his head up, for the first time, while propped on a nursing pillow
– Struggled with getting him to take a bottle; he gagged on everything I tried
– By the middle of the month I moved Oliver into size 6 month pants and size 9 month onesies; also switched to size 3 diapers
– On February 15th I woke up in extreme pain from mastitis; spent Family Day at the emergency room to get antibiotics
– On February 16th Oliver had a swallow study at CHEO; he was put in a high chair and fed liquid barium in a squeeze bottle; the x-ray showed no signs of aspirating while swallowing
– Oliver had neuromuscular clinic on February 17th; Dr M showed concern for Oliver’s lack of weight gain and suggested we have a consult with general surgery about getting a g-tube
– We met with Dr Bass (surgeon) on February 18th and talked about g-tube surgery for June


3 months

3 months

4 MONTHS OLD
February 23 – March 22
– Oliver weighed 13 lb 1.5 oz on February 23rd, length 25 inches, head circumference 41 cm
– Started feeding him Neocate Infant formula by syringe as a supplement
– On February 24th he had his fourth month vaccinations
– Tried carrying Oliver around in the Boba 4G to get him used to it
– My mom, sister, and nephew came on February 25th to spend the weekend; we did a lot of cooking and baking
– Oma discovered Oliver was teething by pointing out blisters on his gums
– On February 29th we celebrated Rare Disease Day by wearing out special shirts from Gwendolyn Strong Foundation
– On March 2nd he received another RSV shot
– Switched to CeraVe brand for Oliver’s cleanser and cream; his eczema is so severe that everything causes him to break out and the eczema oozes; after a trip to the doctor about oozing and infected-looking skin, he was prescribed 1% hydrocortisone; Dr K sent off a referral to dermatology at CHEO
– Oliver finally learned how to use a pacifier
– Ordered a seahorse pattern Lillebaby carrier and Oliver was more comfortable in it


4 months old

4 months old

5 MONTHS OLD
March 23 – April 22
– Oliver weighed 13 lb 3 oz on March 23rd; his weight has stopped increasing
– He met all four month fine/gross motor skills
– Uncle Cheepow & Aunt Serena and the girls visited for Easter weekend
– On March 30th Oliver received his last RSV shot of the season
– We bought a used Outlander because we needed a larger vehicle; Jordan plans to sell my car
– Mary worked with Oliver on April 7th; she loaned him a Tumbleform chair
– I bought a used Baby Einstein activity jumper for Oliver
– On April 9th Oliver tried brown rice cereal and pureed sweet potato for the first time
– Oliver started rolling from his right side onto his belly but can’t get his arms out from under him yet
– On April 13th we had neuromuscular clinic at CHEO; Oliver’s occupational therapist supervised him eating pureed food; we also met with his other specialists; he showed off his new skills; had a long conversation with Dawn about Oliver’s lack of weight gain and agreed we needed to move his surgery up
– Oliver weighed 13 lb 4 oz on April 13th, length 25.4 inches
– Oliver was admitted to CHEO on April 14th for failure to thrive; an NG (nasogastric) feeding tube was inserted and he was placed on the waiting list for emergency gastrostomy surgery; the tube made him sound phlegmy and put him at a higher risk for aspirating; I continued to breastfeed before every tube feed
– Oliver weighed 13 lb 6 oz on April 16th
– On April 19th we met the pediatric dietician, Julie, who understood Oliver’s needs in regard to SMA; we moved his tube feeds to every 4 hours
– On the morning of April 20th Oliver had gastrostomy surgery performed by Dr Bass; a PEG tube was inserted; PPN (peripheral parenteral nutrition) was ordered to keep him from fasting after surgery
– Oliver’s tube feeding was scheduled at 75 ml breastmilk every 4 hours at 75 ml/hr and breastfeeding on demand


5 months old

5 months old

6 MONTHS OLD
April 23 – May 22
– On April 25th Oliver was discharged from the hospital and we moved over to Rogers House for the night; CCAC ordered us some enteral feeding supplies and then we headed home the next day
– Applied for Oliver’s first passport
– Oma stayed with us April 29 to May 2nd; she helped cook while we got used to a new routine with Oliver
– Oliver weighed 13 lb 13 oz on May 4th at the doctor’s office, length 26 inches, head circumference 42 cm; refilled ranitidine prescription and he had his 6 month vaccination
– Jordan’s parents arrived May 7 and stayed til May 10th
– Approved for ACSD (Assistance for Children with Severe Disability), but for the bare minimum
– Travel itinerary for Boston was booked; our first time flying for the NURTURE trial
– Oliver enjoyed being able to sit supported on the floor with toys in front of him
– His first tooth finally came through
– We registered for SMA Family Camp Ontario (July)
– I purchased a new stroller, Bugaboo Donkey Mono, for Oliver; insurance agreed to pay for 80%
– On May 16th we flew to Boston; Oliver did great on the plane, although the whole ordeal was stressful for myself; car service delivered us to our hotel
– In the morning of May 17th Oliver had the motor function test (CHOP-INTEND) and in the afternoon the CMAP; Dr Swoboda was very pleased with his progress
– On May 18th Oliver had his fifth injection of Nusinersen with sedation; he weighed 14 lb 2 oz, length 25.9 inches; he passed the neurological exams and that evening we left the hospital to go out for dinner; the next morning we returned to the hospital for one last neuro exam
– On May 19th we flew home
– I can’t keep Oliver from constantly rolling from his back to tummy, he’s seriously working on this skill


6 months old

6 months old

6 months old

I cannot believe my little boy is 6 months old! We are so happy with the progress he has made; he truly is a miracle!

Oliver: Birth to 1 month

October 23 – November 22, 2015
The first month with my baby boy was a whirlwind, to say the least: his early birth, getting used to having a newborn at home, his diagnosis of SMA, and travelling to Boston to get him in the NURTURE clinical trial.

This post is for me to play catch-up!

Oct 24 – The day after Oliver was born, we left the hospital at 9:30 am. The L&D floor was very busy and short on rooms so they wanted us out of there as soon as possible.

Oct 25 – Home visit from Suki. Oliver was slightly jaundiced.

Oct 26 – Home visit from Suki. Oliver weighed 7 lb 8 oz. We gave him BioGaia probiotic drops, as recommended, to help with gas (he’d been struggling at night).

Oct 27 – Noticed that since starting the BioGaia, Oliver’s poops have been even more frequent and burning his bum!


October 27

November 1

Oct 28 – We went to Prenatal Nutrition and brought my mom. Decided to stop the probiotic drops and picked up Sudocrem to help heal his bum.

Oct 29 – Oliver was breastfeeding every 2 to 2.5 hours round the clock. Lots of wet diapers! Suki had the flu so home visit was rescheduled.

Oct 30 – Oliver turned 7 days old. We drove to CHEO and met with Claire (genetic counsellor) and geneticist. Oliver’s muscle tone seemed normal for a newborn. He had blood drawn for SMA testing and weighed 7 lb 12 oz.

Oct 31 – We didn’t do anything special for Halloween but did take some pictures of Oliver in a themed sleeper.


October 28

My mom stayed with us until November 2. I loved having her with us. She made delicious meals for us and changed lots of dirty diapers! She also finished crocheting a beautiful baby blanket and two hats for Oliver.

By the end of his first week, I switched to size 1 diapers rather than buying another box of NB. Some NB size onesies were starting to be too short, as well. Oliver slept the days away, only having a few alert moments. When awake, he would follow my voice with his eyes. At night, he woke every 2-3 hours to breastfeed and then he’d fall back to sleep. I was finally getting some shut-eye!


November 9

Nov 3 – Jordan from “Cole Creations” came all the way from Kingston to do two newborn photoshoots at my house–one for Oliver and one for my friend’s baby girl, Josebelle, who was born five days after my boy. Oliver was 11 days old.

Nov 4 – I had Oliver weighed at Prenatal Nutrition: 8 lb 5 oz.

Nov 10 – Oliver met his family physician, Dr K. He weighed 8 lb 14 oz and measured 21 inches long (same as at birth). Around this time I switched him into 0-3 month clothing and packed away all the NB outfits.

Nov 11 – Geneticist called in the evening to tell us that Oliver’s blood test results showed he had Spinal Muscular Atrophy (SMA).

Nov 12 – I emailed the coordinator for the NURTURE trial (in Boston, Massachusetts) and explained Oliver’s diagnosis and our interest in getting him into the presymptomatic newborn clinical trial for ISIS-SMNrx. Kellie replied right away and Jordan and I immediately FaceTimed with her and Dr Swoboda. We agreed to Dr Swoboda’s suggestion that we get Oliver to her as soon as Monday so that she could assess him and, if he’s accepted, be given his first injection by the Thursday.

Nov 13 – We met with Dr M (neurologist) at CHEO. He examined Oliver and told us that he was displaying some signs already. He was 3 weeks old. I had noticed lessened movement in his arms and legs (only because I was really looking for it) and his inability to pull his head up. Dr M wrote us a prescription for a car bed.


November 2

November 6

Nov 15 – Arrived in Boston at 7:30 pm and settled in at the hotel. It took us two days to drive there.

Nov 16 – We met Kellie and Dr Swoboda and discussed what to expect from the clinical trial. Oliver went through a full day of screening, including an ECG, blood draw, motor function test, and CMAP. We had to wait for the blood work to come back before Oliver could officially be accepted into the study. The following day they had to repeat the blood draw due to the vials clotting.

Nov 18 – Oliver was accepted into the NURTURE trial!

Nov 19 – Procedure day! Oliver was given the drug ISIS-SMNrx intrathecally (administered through lumbar puncture). His back was numbed but of course he still got very upset. No sedation was needed. Jordan stayed with him the whole time while I pumped, since he had to miss a feeding session.


Two weeks old

Three weeks old

Although procedure days will be tough on our little guy, we know it’s the right thing to do. SMA is a terminal disease that takes almost everything away from a child. But this drug can delay or stop progression. Right now we hope for the little things, like being able to hold his head up and kick his legs. We have to make two more trips to Boston in the month of December for two more injections, then we will have a month reprieve.

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 (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.


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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.