Oliver’s trip to the Aviation Museum


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The above is a super adorable picture of my clone babies! 🙂

On June 7th we arrived in Ottawa at CHEO in time for Oliver’s appointment with General Surgery. We waited an hour before we were called in; they were running behind. The g-tube nurse was training another nurse so the trainee did the tube change. She removed Oliver’s temporary PEG tube and inserted the measuring tube. Oliver was not impressed with this at all; he got very upset and I felt bad, but the tube switch needed to be done now that the stoma was healed. I did my best to comfort him and rocked him to sleep while Jordan went down to the pharmacy to purchase a new Mic-Key button (12 French, 2 cm). Over $200 later, the button was inserted and we were finished for the day! The nurse showed us how to do the insertion so that in 6 months’ time we can do it ourselves. I’m actually nervous about that, as we never did a tube change with Khloë.


Superman Baby

Mic-Key button

We stayed overnight at Rogers House, the palliative care facility. The following day, after packing up, we drove over to Ottawa’s Aviation & Space Museum and spent a couple hours wandering around there.


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Aviation

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Aviation Aviation

The museum was a nice way to end our trip to Ottawa!

SMA: Oliver’s diagnosis

In the evening of November 11th, at a couple days before he turned 3 weeks old, we received a phone call from the geneticist that Oliver was diagnosed by blood test with Spinal Muscular Atrophy. The test showed he was missing the SMN1 gene and had two back-up copies called SMN2. Most likely he would be a Type 1, like his big sister.

We had to make a decision very quickly.

While I was pregnant, someone from an SMA support group contacted me with information about a clinical trial called NURTURE, a phase two study sponsored by Biogen for presymptomatic newborns diagnosed by blood test. This particular study wasn’t available in Canada yet but the closest centre was in Boston, Massachusetts. (Phase three trials of the same drug on babies with symptoms have been ongoing for awhile with very good results.)


Oliver's first visit to meet family doctor

Oliver’s first visit to meet family doctor


The next day, after we had digested the news and decided that we were willing to do anything for our tiny baby who was quickly losing the use of his legs, I sent an email to the study coordinator at the Boston site. She replied fairly quickly, stating their interest in Oliver, and we FaceTimed with her and Dr Swoboda almost right away. I was surprised–and relieved–that they made contact so quickly.

After an hour long discussion with Dr Swoboda (neurologist, SMA specialist) about my pregnancies, both babies, and aspects of the trial, Jordan and I decided it was in Oliver’s best interest to get the drug ISIS-SMNrx.

The doctor told us that it was imperative for her to assess Oliver as soon as possible. We already had an appointment for him to see Dr M, neurologist, at CHEO on Friday. She said that if we could be in Boston by Sunday, she could asses him Monday, November 16th. If Oliver qualified, he would receive his first intrathecal injection by Thursday. Could we do it? With Jordan on parental leave, YES!

As of now we have already completed three trips to Massachusetts General Hospital for the NURTURE study. After two injections, Oliver started to show more strength! By the time we arrived for his third on December 17th, Dr Swoboda was impressed with the progress he had made in the last two weeks.


Baby boy

In a short period of time Oliver’s disease had progressed rapidly. He couldn’t hold his head up or move his legs very much, and only used his forearms rather than his whole arm. With two doses of SMNrx under his belt he began holding his head up again, although for 5-10 seconds at a time, began moving his legs more, and lifts his whole arm up and keeps them held to his chest when rolled side to side. It’s such a huge difference and we are so happy he’s getting this drug!

Oliver’s next injection is at the end of January. Now that we are done travelling for a little while, I will try to write some updates about our precious baby boy who is 8 weeks old now!

The birth of Oliver Kaleb Azariah

While Skyping with my mom on October 22, I felt some mild cramps. I didn’t think much of it, but I did mention to her that it felt like period cramps. Later that night I felt them again but a little stronger and they continued until 4 am. I realized they were contractions because they were regular. I tried to get some sleep.

By morning (October 23) I felt that I had had some rest. Jordan went to work but was supposed to come home at lunch time to take me to my midwife appointment at 1:30 pm.

At 11:15 am I went to the bathroom and lost my mucous plug–so gross! Fifteen minutes later, contractions started up again, but stronger than the previous nights’. They remained strong and steady at 4 – 5 minutes apart and 30 – 45 seconds long. After an hour of this, I texted my mom to let her know. I wasn’t sure if I was really in labour… October 23 marked 38+4 weeks. I called Jordan and told him he should come home.

By 1 pm mom decided she would start driving up from Kingston because she really wanted to be a part of Baby Boy’s birth. At that point I called my midwife and told her I was having regular contractions and asked if she wanted me to go to my appointment. She told me to stay put and she would come to check my progress at 2 pm.

Suki arrived around 2 pm. She checked my cervix and did a membrane sweep. Only 1-2 cm dilated! She also confirmed I really was in labour and offered me the use of a TENS machine to manage the pain. I figured I would give it a try. She decided to go home to rest and told me to head to the hospital when the pain is getting too much to handle, and to call her when we leave.

By 3:30 pm the contractions were pretty intense and I had to really focus to breathe through them. They were coming 3 – 4 minutes apart and about 1 minute long. Another hour later, I decided we needed to get going! I knew we had a 40 minute drive ahead. My mom just happened to pull in our driveway at that time too, so she hopped in our car and came with us!

We arrived at the Pembroke hospital and Jordan grabbed me a wheelchair. We had to make a stop at registration but quickly headed up to Labour & Delivery. Suki hadn’t arrived yet, but the on-call obstetrician Dr M showed me to a delivery room. A nurse came in and hooked up the belly monitors.

My midwife showed up at 5:30 pm and checked my cervix: 3 cm dilated. At this point we discussed what I wanted for pain relief; I was really struggling with the contractions and was already feeling tired. We agreed an epidural would be a good idea and she went to talk to Dr M, as an epidural means a transfer of care to an OB.

Around that time a nurse came to start an IV and take my blood. I was GBS+ which meant I needed a round of antibiotics (clindamycin). A fluid bolus was also administered for the epidural.

Two hours later, at 8 pm, feeling like I was gonna die from the pain, the anesthesiologist arrived! Suki joked to Jordan, “Here comes the man your wife is going to want to marry!” And boy was she right. 😂 He explained the risks of an epidural to me, I replied “Let’s do this!”, and he had that epidural in super fast!

My body slowly began to go numb; he said it would take about 20 minutes. Both my legs were completely frozen, but I could wiggle my toes. I couldn’t feel the contractions except in a small fist-sized area on my lower left side.

Dr M checked me at 8:30 pm and voila, 6-7 cm! Finally my body was doing its job and dilating my cervix. The catheter went in at this time, as well as a scalp monitor for the baby. Baby Boy was so active during labour that the belly monitor was having trouble picking up his heart rate so I agreed to the monitor being attached to his head.

Fully dilated by 10:10 pm! This was it! Soon we would meet our little boy. Even though I had been transferred to the OB, Suki still caught the baby. At 10:42 pm, Oliver Kaleb Azariah was born and placed on my chest. He was completely covered in vernix! We waited for the cord to stop pulsating, then Daddy cut the umbilical cord. And guess how much my little guy weighed–a whopping 8 lbs! He was 21 inches long and had a healthy cry. Head circumference was 35.5 cm.


Birth collage

I was so astonished to have birthed an eight pound baby!

After Oliver was cleaned up and weighed, I let him breastfeed. He knew just what to do, my little milk guzzler.

We were discharged the next morning and brought our sweet little boy home. 😀

Second trimester: Weeks 26 – 27

Week 26 (July 27)
It was a HOT week! I don’t remember the heat bothering me that much when I was pregnant two summers ago; maybe I’ve just forgotten! A few nights of the week I wasn’t able to sleep much, even with two fans pointed at me and no blankets on. It was so sweltering hot on Wednesday that Jordan and I decided to hop in the car, blast the A/C, and drive to Pembroke for chocolate milkshakes and ice cream from the dairy. Meals have been a bit tricky because I didn’t want to turn on the oven. We’ve been living on cold salads and barbecued hamburgers or sausages, but one day I did make a creamy ranch chicken in the slow cooker. Oh, I finally pre-registered at the hospital I’ll be giving birth at.


Weeks 26-27

Week 27 (August 3)
Some websites consider this third trimester, but to make it easier I’m going to use 28 weeks as third trimester. My hips and pelvis are aching more, a sign that baby and belly are growing and that I’ve pushed myself too much for one day. In preparation for my mom’s upcoming visit, I did a lot of house cleaning, which exhausted me. I had one really bad night with SPD (symphysis pubis dysfunction); anytime I moved I would get shooting pains in my pelvis. Jordan had to help me to the bathroom in the middle of the night because I literally couldn’t stand up without collapsing.

My mom drove up on Saturday and we spent the weekend cooking yummy meals. She made my favourite mushroom risotto one night. She had a blast taking care of the chickens, constantly checking on them, feeding them, and collecting eggs. I think she enjoyed herself!

Timeline of Spinal Muscular Atrophy

Every child diagnosed with Spinal Muscular Atrophy, regardless of type, shows symptoms differently. Khloë was diagnosed with Type 1 at six months of age. Type 1 is considered the most severe form of SMA. This timeline will be kept updated to show how her neuromuscular disease progresses, the milestones that she reaches, and anything that is important.


Timeline of SMA

{Last Updated // February 2, 2015}

December 3, 2013: Khloë is born at 7:22 pm, delivered by midwife and obstetrician. She is two weeks early, weighs 7 lb 4 oz, and is 19 inches long. Khloë is perfectly healthy!

January 3, 2014: Khloë turns 1 month old!

January 16, 2014: Check-up with family doctor.

February 3, 2014: Khloë turns 2 months old!

February 4, 2014: Check-up with family doctor. Dr K is concerned that Khloë is “floppy” but suggests we give her more time to develop. She receives her two-month vaccinations and weighs 11 lb 13 oz.

March 3, 2014: Khloë turns 3 months old!

March 12, 2014: Check-up with family doctor. Khloë shows improvement in head control, which satisfies Dr K, but she is still “floppy”. Hydrocortisone cream is prescribed for eczema. Khloë weighs 13 lb 12 oz and is 24.5 inches long.

March 30, 2014: Khloë is sick with her first cold.

April 2, 2014: Khloë has a chest x-ray which shows her cold has progressed to bronchiolitis. She is given an oral steroid to help with breathing. Her illness lasts approximately 10 days.

April 3, 2014: Khloë turns 4 months old!

April 15, 2014: Check-up with family doctor. Khloë has not progressed any further with head control and we are getting worried. Dr K sends a referral to a pediatrician in Ottawa. Khloë receives her four-month vaccinations and weighs 14 lb 4 oz.

May 3, 2013: Khloë turns 5 months old!

May 14, 2014: Mary (Infant & Child Development) visits with Khloë and expresses great concern about her hypotonia (low muscle tone) and lack of reaching milestones. She will continue to work with Khloë.

May 15, 2014: We meet Khloë’s pediatrician, Dr I, for the first time. She sends a referral to a neurologist at CHEO. She also prescribes clotrimaderm/hyderm cream to treat the yeast rash under Khloë’s chin and on her left wrist.

May 21, 2014: Khloë sees Dr M, pediatric neurologist, at CHEO. After running a few tests, including an EMG, he tells us he suspects a neuromuscular disease called Spinal Muscular Atrophy. Khloë has blood work done to confirm.

June 3, 2014: Khloë turns 6 months old!

June 9, 2014: Check-up with family doctor. She receives her six-month vaccinations and weighs 15 lb 7 oz. Length is 27 inches.

June 10, 2014: Khloë is officially diagnosed with Spinal Muscular Atrophy (Type 1).

June 23, 2014: We meet with Debbie (Occupational Therapist) at CHEO. Khloë eats pureed baby food for the first time! We also tour Rogers House, the palliative care facility located behind the children’s hospital.

July 3, 2014: Khloë turns 7 months old!

July 5, 2014: We celebrate Khloë’s 7 month “birthday” with a friends and family BBQ at the cottage!

July 7, 2014: A care package from CureSMA (Canada) arrives for Khloë. It’s filled with fun toys, a Radio Flyer wagon, sheepskin, and much more!

July 8, 2014: We discuss gastrostomy surgery with Dr B at CHEO. Nothing is scheduled yet.

July 9, 2014: Khloë meets her team of neuromuscular specialists.

July 12, 2014: Khloë’s first trip to the beach.

July 19 – August 5, 2014: Khloë travels to Bar Harbor, Maine and visits family in New Brunswick.

August 3, 2014: Khloë turns 8 months old!

August 7, 2014: We meet with our Care Coordinator from CCAC to discuss Khloë’s current and future needs for nursing, etc.

August 9, 2014: Candles are lit for SMA Awareness Month.

August 11, 2014: A CoughAssist machine and pulse oximeter is ordered for Khloë after a visit to CHEO. She weighs 16 lb 4 oz and is 28 inches in length. Khloë visits the Aviation & Space Museum!

August 22, 2014: We meet with an anesthesiologist at CHEO to talk about her special needs during surgery.

August 27, 2014: Khloë has gastrostomy surgery. We spend Labor Day weekend in the hospital and at Rogers House for recovery.

August 28, 2014: Khloë has her first tooth!

September 3, 2014: Khloë turns 9 months old!

September 16, 2014: Check-up with family doctor. Dr K prescribes mupirocin for the infection on Khloë’s wrist. She weighs 17 lb 3 oz and is 29 inches in length.

September 18, 2014: Khloë is fitted for AFOs.

September 20, 2014: Illness strikes again! Khloë is sick with a cold. Her illness lasts a few weeks.

September 24, 2014: Khloë has a chest x-ray which shows her cold has progressed to pneumonia. Dr K prescribes amoxicillin.

October 3, 2014: Khloë turns 9 months old! We meet with Khloë’s neuromuscular team for clinic at CHEO and receive the CoughAssist and pulse oximeter.

October 9, 2014: Khloë starts receiving 5 ml of sambucus (black elderberry extract) through G-tube daily.

October 13, 2014: Khloë’s first Thanksgiving!

October 15, 2014: We meet with Khloë’s neuromuscular team for clinic at CHEO. Dr K (respirologist) is very concerned with the fact that she is still sick and her low o2 sats; he orders a chest x-ray which shows pneumonia has not cleared. He insists she needs to be admitted for BiPap trial ASAP. Khloë is admitted to the hospital the following day but cannot trial BiPap until an ICU room becomes available. She is prescribed amoxicillin-clavulin to treat pneumonia.

October 17, 2014: Khloë begins BiPap trial in the ICU at CHEO. We are there for 6 days until finally she is discharged into the care of Rogers House. After two more days there, we go home! Pneumonia has cleared and o2 is back to normal. Final BiPap settings are 18/8.

October 22, 2014: Khloë’s temporary G-tube is replaced with a Mic-Key button.

October 28, 2014: Khloë receives the flu vaccine.

October 31, 2014: Khloë’s first Hallowe’en!

November 3, 2014: Khloë turns 11 months old!

November 5, 2014: We receive the Hope car bed, donated by CureSMA (Canada).

November 7, 2014: We start giving Khloë breathing treatments every morning and evening (suction, chest PT, CoughAssist).

November 21, 2014: Khloë receives RSV shot #1 (Synagis). She weighs 19 lbs.

November 27, 2014: We begin the lengthy process of purchasing a medical stroller for Khloë.

December 3, 2014: Khloë turns 1 year old!

December 6, 2014: Friends and family celebrate Khloë’s first birthday with a Frozen-themed party.

December 9, 2014: Check-up with family doctor. Khloë receives her twelve-month vaccinations and second flu shot. She weighs 19 lb 8 oz.

December 12, 2014: Khloë receives RSV shot #2 (Synagis). She weighs 20 lbs.

January 3, 2015: Khloë turns 13 months old!

January 7, 2015: Khloë receives RSV shot #3 (Synagis). She weighs 20 lb 4 oz.

January 12, 2015: After a 9-1-1 call, Khloë is rushed to our local hospital for a chest X-ray and observation. She is then sent by ambulance to CHEO and is admitted for aspiration pneumonia. She also struggles with mucous plugs.

January 15, 2015: Just after midnight at CHEO, Khloë’s airway is blocked by a mucous plug during a CoughAssist treatment. Her oxygen level and heart rate plummets dramatically and she goes into cardiac arrest. Twenty-five minutes later she is stabilized and intubated.

January 16, 2015: Khloë receives a CT scan which reveals significant swelling in her brain. She no longer has any responses. At 9:25 pm, surrounded by Mommy and Daddy and close friends/family, our baby girl takes her last breath.

January 21, 2015: We celebrate the beautiful life of our sweet Khloë during the funeral service at Real Hope Christian Assembly.