Mama and Dad

I wrote this yesterday but had trouble with the photo. Mr. Houdini (above) did not cooperate last night and both IVs were eventually removed after D'Arcy had had enough of holding Ben down. We told them the hand IV wouldn't work (he also had one in his foot). We told them he had a phobia. We asked for sedation. We told them it would be bad. But the bad had to happen before it came out.

Surgery number two

This is a photo I took of Ben waiting for his surgery this morning. For a brief moment he forgot about it and laughed at the movie he was watching.

He didn't look anything like this in the recovery room. He lost blood during the surgery and was white, puffy and fragile. Luckily things improved and he avoided a blood transfusion.

The surgeons discovered that part of his hip bone had broken at the place where the screws came out of the hardware installed two weeks ago. Apparently Ben's bones are so soft and weak they feel like an elderly person's.

They put in a new plate and wrapped him in a non-removable fibreglass leg and body cast.

From my earlier posts you'll remember that when he first had hip surgery two weeks ago he was in a removable, two-piece clamshell-like cast. The cast wasn't seen as essential, but it now is! Because of the weakness of his bones we can't risk another failure so he'll be in his cast for six weeks.

We had a dramatic but relatively painless descent from our house on the hill this morning.

Last Thursday when Ben was taken back to hospital because of acute pain, the EMS folks placed him on what's called a scoop stretcher, which is two aluminum boards with side handles. They snap together under the patient. This was used because a regular stretcher couldn't be carried up and down our 29 steps.

It was incredibly painful for him to lie on that hard surface, strapped down, and by the time he got to hospital he'd reached his limit. He was lying on his stomach but they wouldn't let him roll over and wouldn't transfer him to a padded stretcher that was standing right beside him.

The orthopedic clinic was bursting at the seams with waiting kids and parents and he screamed while the person at the desk did papework for his x-ray. Then he was wheeled to wait in the x-ray hall but again was not allowed to be unstrapped. He continued to cry and thrash around hard against the straps and metal that bound him.

The x-ray showed a screw was dislodged and my husband is convinced that Ben damaged the hip hardware during that fiasco.

When it was decided Ben would have the revisionary surgery today, I was petrified that the same EMS folks would insist on transporting him on a scoop stretcher. I talked to the dispatchers at length about my concerns.

Our experience this morning was like night and day. When they arrived, the paramedics questioned why the other folks had put Ben on the scoop and instead suggested a canvas material that acted like a hammock when carried by metal rods that were threaded through its sides.

These two gentlemen were so gentle with Ben. As they positioned him on the canvas, to our surprise, five giant men in overalls trooped upstairs and into the bedroom. Were they part of some backup EMS team?

Six men carried Ben down our stairs and at the bottom the hammock was placed on a padded stretcher. D'Arcy accompanied Ben in the ambulance and I followed behind.

I didn't realize who these burly guys were till I drove up the street behind the ambulance and saw a fire truck parked there. Unbeknownst to us, the firefighters had been called in!

As we drove along a busy Toronto street during rush-hour, I realized the ambulance ahead had its hazard lights on, and was going at a snail's pace. A couple of times it swerved way into the oncoming lane, as if to pass an accident, but as I followed I realized the driver was dodging potholes. A number of times it pulled off to the side of the road. Was the driver trying to get my attention? Was something wrong?

Soon I was four cars ahead of it and I began to worry that perhaps Ben was screaming bloody murder because he hadn't been able to have his heavy-duty pain meds on time (we were told the last dose could be given at 6 a.m.). At a red light I put the car in park, got out and ran the few cars back to the ambulance with the narcotics in my hand. I was stunned when the paramedic rolled down the window and explained that Ben was just fine. He was driving that slowly on purpose. He didn't want Ben to feel the bumps.

As soon as we got to the surgical unit they put Ben on a padded stretcher and instead of lifting him up to extricate the canvas material he was lying on, they slid out the rods and said Ben could keep the canvas. They didn't want to disturb him. They also left Ben wrapped in an orange EMS sheet and blanket (we had mentioned earlier that Ben's favourite holiday is Halloween).

Tonight in his room on the unit Ben was sleeping and his face still seemed puffy, but his cheeks were pink.

He managed to crack his eyes open for a second and signed "When off?" as in "When can I take them off" about the two IVs and, of course, the gigantic cast.

It's so hard to see him go through this again and wonder what he's thinking inside. He refused the sedative we hoped would make him drowsy before going to the OR so I again donned the infection-control "bunny suit" and went in.

I liked the anesthetists so much better this time – they really cared. Still, it wasn't easy, Ben cried and I wondered about how forcing these procedures on him was breaking his spirit.

I pulled my first all-nighter in years last night: Ben was in terrible pain. He had been weaned to tylenol and by the time I realized he was crashing and went to get the strong pain medication filled, it was too late.

At one point last night Ben started thrashing around in his cast, trying to roll onto his affected leg. We were frightened he would hurt himself and decided to remove the cast. I neglected to mention in my previous posts that Ben didn't need to be in his body cast medically -- for the purpose of bone healing -- but to prevent him from placing any weight on his affected leg. The surgeon made the cast a removable, clam-shell one and told us if we could be sure he wouldn't put weight on his leg, we could take it off.

Ben's pain continued without the cast and as the early morning hours ticked by I started agonizing over whether removing it was the wrong decision. I spoke to the orthopedic resident on call, but despite his reassurance I was fit to be tied. I e-mailed the surgeon, and imagine my relief when I received this message back from him at 9 this morning:

I was so relieved, I cried, and I suddenly felt capable that I could support Ben through the pain. It was natural that he had great pain (not a reflection of anything we were or weren't doing), and hopefully it would get a little better, everyday. We were acting in Ben's best interest when we removed the cast because we were afraid he would hurt himself.

I went for a walk with my younger son and it was bright and sunny and everything was in bloom, particularly the hydrangeas. I felt like I had been touched by grace.

My son Ben had his hip and knee surgery today and I'm very grateful to be on this side of it! Here's a picture of him after he was taken from recovery up to the room he'll be in for a few days.

Barb's daughter Annie (above) was born without the brain and heart defects common in Trisomy 13, but died at 80 days in 2005 after being rushed to a children's hospital in respiratory distress. Following her death, Barb acquired Annie's medical records and learned a "not for intubation" order had been written without consent. “This discovery was like the first domino in a long line of questionable events that left us unclear as to whether our daughter’s death was preventable.” Determined to change what she believes is systemic discrimination against treating children with certain genetic conditions, Barb shares Annie’s story at health-care conferences and ethics talks, with medical and law students, in medical journals and through her work with Patients for Patient Safety Canada.

Me: When did you learn Annie had Trisomy 13?

Barb Farlow: After the 22-week ultrasound, the geneticist said "this is Trisomy 13, 18, or something equally lethal." We were told she would likely die before birth or in the delivery room. Initially, the research we did suggested these babies were ‘incompatible with life.’ We were confused because it seemed that many of the anomalies they had could be fixed. When I was six months pregnant we found Living with Trisomy 13, a web site for families. We came to know many living children and their families and discovered these children were very special and loved, that some did benefit from medical intervention, and that while the experience was challenging, it was life-changing for the families.

Me: How did you decide to continue with the pregnancy?

Barb Farlow: It wasn't a process, it was immediate. By the time of the diagnosis, I had grown to love my baby and felt very protective of her. I realized the odds were against her even coming to term, but it didn't mean I would take steps to end her life. The geneticist said “We don’t do surgery on these kids” and an obstetrician later told us: “You don’t crack the chest open for these little kiddies.” That prompted us to meet with staff from three departments at the children's hospital to ensure that if Annie needed life-saving surgery, she would be eligible for it. We were told she’d be treated like any other child. We knew surgery might not be indicated for her – that it might be too burdensome or not in her best interest, but we didn’t want the door closed on account of her genetic condition or disability. We wanted full information about the benefits and risks of any treatments so we could make good choices.

Me: What were your hopes and dreams for Annie?

Barb Farlow: We wanted her to have a chance. We wanted her to survive as desperately as any parent wants their child to survive. We were well aware of the disabilities and challenges she would have. My husband had taken an indefinite leave from work so that we could both devote ourselves to Annie and our other children. We wanted Annie to have comfort and happiness and we knew that through loving her we were going to learn so much. With Annie, we took nothing for granted and lived each day to the fullest. One clear, hot night, when everyone was asleep, we took Annie outside and looked at the stars together. We viewed life differently when Annie was born. Her first smile was like magic.

Me: How was Annie affected by Trisomy 13?

Barb Farlow: When she was born, we learned she didn't have the brain defect or the major cardiac condition they predicted. She was seven pounds, with Apgar scores of eight and nine out of 10, which was amazing. We were more shocked than anybody. She wasn't blind or deaf, which was a possibility, and she didn't have any major structural deformities in her organs. As we were rejoicing about how well Annie was doing, a medical fellow asked my husband: "Do you really want to treat this child? She's going to have these disabilities." He suggested she shouldn’t be treated, irrespective of her physical status. It was like a slap in the face. Annie was treated in hospital for six weeks for low blood sugar and received excellent supportive care at that time.

Me: How did Annie die?

Barb Farlow: We only recently received the medical certificate of death and it lists respiratory distress that began one month prior to her death. When we took her to the outpatient clinic two days before she died – because she was having trouble breathing and her face was beet red – we were told it might get better on its own and sent home with no tests and no measure of comfort for her distress. Earlier on, a pulmonologist had suspected she had a tracheal disorder, but she was never tested for it. When she was rushed to hospital in acute distress for a tracheal assessment, we were told her trachea was fine, but she had pneumonia. She didn’t. We later learned that throughout her life, doctor after doctor ignored critical indications of impending respiratory failure. Meanwhile, we were led to believe that Annie was receiving full care.

Me: How are children with serious genetic conditions viewed in our culture?

Barb Farlow: Especially with the financial challenges in our health system, I think there's an element of anger similar to how some people feel when a person is obese or a smoker and needs expensive medical treatment. There's the perception: "This is a choice, a life choice, we have ways of preventing these things." I think there are many in health care who see these children this way – as something we can avoid and have the ability to avoid. There's a perception that if a child isn't perfect, it's cruel to bring them into the world. It's expected that the vast majority of women are having testing and will terminate for these conditions. I think it's a challenge for health-care providers to have compassion and see value in our children when it's a road they wouldn't have taken themselves. Especially when there are limited resources and they're already short of NICU beds.

Me: What about in the community at large? Did you feel supported as a mother who chose not to terminate a child with a genetic condition?

Barb Farlow: I had a sense that most of our friends and neighbours wouldn't understand our choice, so during the pregnancy I confided in only a few close friends. It's unfortunate, because a person needs a lot of support at that time, but the support doesn't exist. When you terminate in our system, you're given a gift package – with hand-knit booties, a swaddling blanket and a kit for making footprints. There are support groups and psychological counselling and it's all there to help you go in that direction. If you choose not to terminate, you're on your own, with some of your friends and relatives thinking you're crazy.

Me: How has the rise of prenatal testing influenced public perceptions about children with genetic conditions?

Barb Farlow: I believe it creates the perception that we have a cure for these conditions. There’s a sense of “I’ve done all my prenatal screening and testing, I have a right to a perfect baby.” I’ve heard from genetic counsellors that more women are terminating for less serious conditions like cleft lip and palate, or club feet. I think we need to review whether the training doctors receive allows them to counsel in a neutral and balanced way. I found an educational presentation online created by Ontario’s Fetal Alert Network that included a photo of a stillborn child with a severe deformity related to Trisomy 13 placed next to a picture of a one-eyed Cyclops from Greek mythology. This was a terribly dehumanizing picture. When a family doctor has a patient whose fetus has been diagnosed with Trisomy 13, what picture will come to his mind? Annie had an extra finger on both of her hands. But she wasn’t less human.

Me: You’ve spent the last three years sharing Annie’s story across North America and further afield. What do you hope to achieve?

Barb Farlow: I hope I’ve opened the eyes of health-care providers to how a family makes decisions and how they feel. I hope I’ve held a mirror to them to reveal how their actions or inactions were perceived by a family. I think there’s a group-think approach in health care to issues related to disability. Everybody does what everybody else is doing and no one thinks for themselves. I believe a lot of denials of care are tacit and “let’s all do this because we think everyone is doing this" and based on the assumption: "These kids are hopeless and no one wants them.” I hope I’ve changed that, and allowed providers to start thinking about things in a different way, so they can stand up and speak for what they believe is right.

We know that our decision was not a common one, but we believe parental involvement and consent in treatment decisions are critical. I think there should be a requirement that children with complex conditions have a clear treatment plan documented in their file that includes the parents’ values and wishes. The issue is consent, and if the line is crossed now, on this genetic condition, what other conditions will be crossed? Down syndrome is also a genetic condition in every gene, and we don’t call it lethal. Sometimes children with Down syndrome have things that need to be fixed, and we fix them.

Me: What advice would you give a parent who’s chosen to bring a baby prenatally diagnosed with a genetic condition to term?

Barb Farlow: Number one would be communication. It’s important that the doctors know you’re aware of the challenges of the medical condition, that you’re realistic and realize you might have to make difficult decisions. Emphasize that you want to make decisions based on medical reasons and not quality-of-life reasons, that you’ve accepted the disability. It’s helpful if you connect with other families of children with similar disabilities prenatally, so you can say you’re aware of what you’re getting into and you’ve made the commitment.

Ask a lot of questions about tests and their outcomes and continually restate your position regarding the level of care you want. You may be afraid to ask questions because you’re afraid of the answers. It’s natural, as a parent, to be afraid of bad news. But you must ask anyway. After Annie’s death I was asked by the chief of a hospital department why I didn’t challenge the doctor at the outpatient clinic we took her to when her face was beet red and he sent us home. I wouldn’t have dreamed of challenging a doctor at that point. We trusted them.

At the international level, groups for Trisomy 13 are trying to get a short medical primer developed that lists the various associated conditions and treatments so that parents can speak intelligently about the issues with their doctor. Connecting with other families prior to your child’s birth is a very important way to get educated.

Click here to read an overview of Annie's story.

By Louise Kinross

At last, he sleeps. He floats. His chest fills and empties. His extravagant eyelashes flicker over a dream. He is still.

We've been at the hospital for six hours. Earlier, when nurses struggled to stick an IV into his thread-like veins, he screamed and swung his small, weak arms. D’Arcy and I held him down while they pricked at him, again and again, each poke a stab in my heart.

"It's okay Ben."

"I love you Ben."

"Ben. You need the IV for the operation!"

Now, he lies like deadweight in my lap. He’s four, but the size of a two-year-old. We breathe in tandem, in and out.

Through a glass window I can see the nurses and doctors in the operating room on the other side of the hall.

My son, Ben, has a rare genetic condition called Langer-Giedion syndrome, a random deletion of two genes that probably occurred during cell division. In addition to unusual facial features and protruding ears, he struggles to hear, walk, and speak. We’re in the hospital because he’s stopped growing. Doctors call it “failure to thrive.” Despite a high-fat diet and months of recording every morsel that passes his lips, he’s only 27 lbs.

He's here to have a hole punctured in his stomach so that an artificial feeding tube, known as a gastrostomy – or g-tube, can be placed. It's hoped that nighttime feeds will kickstart his growth. To try to make this palatable to Ben, we explained that he would be getting a new belly-button – he would have two!

Twice, the surgery was cancelled the day before scheduled because of a nursing shortage at the children’s hospital.

The second time I called the patient advocate. "Do you know how traumatizing it is to prepare our four-year-old son for this surgery? We’ve booked off work and arranged care for our two-year-old daughter. What am I supposed to tell my son?"

It's always been hard for me to speak out. I grew up in a British family that was concerned about manners and appearances. My Dad came from a long line of Scotsmen who never complained. I had a wobbly sense of self that relied heavily on the images others held of me. I wanted to be liked. As a result, I smoothed things over when there was a problem. I respected authority and didn’t rock the boat.

That didn’t bode well when I gave birth to a child who was medically compromised.

When Ben was only a day old, a nurse came to my hospital room and lifted him out of my arms. Speechless, I trotted down the hall behind her and into the clinic room, where the pediatrician waited.

“The doctor wants to look at him,” she finally explained – motioning me to the door.

“Alone.”

I walked back into the hall and the doctor slammed the door.

When Ben was close to a year, he developed torticollis – the muscles were too strong on one side of his neck, causing him to sit with his head in a perpetual tilt. “Ben has a rare genetic condition,” I said, as we introduced ourselves to the new therapist assessing him.

“I can SEE that,” she chortled.

Ten days later, as Ben lay on an examining table charming a handful of medical students, the surgeon who was to bring down his testicles pranced into the room and demanded angrily: “What is WRONG with his head?"

I explained that he was born with a head circumference in the 50th percentile, but height below the 3rd, making him look like a little Buddha. He’d already had an ultrasound to rule out hydrocephaly. “What about mental,” the surgeon continued, using his own shorthand for mental retardation. “What about mental?”

The implication that my son was imperfect, flawed, deformed – and lack of the most basic respect for him as a human being – could send me reeling with rage and grief for days. But I didn’t say anything.

Now, I have a four-year-old disabled son who can't speak. I am the only voice he has. So I call the patient advocate to complain about the cancelled surgery. She says the surgeon will call me, but he never does.

Instead, the surgery is rebooked a third time. Finally, the day arrives.

Ben is sprawled over my lap, hooked up to the IV pole and mercifully, after hours of misery, in a deep sleep. D’Arcy has gone to grab a coffee. We’ve graduated from the waiting room to the surgeon’s inner sanctum, an office directly across from the operating room where he’s giving me final instructions.

There's a tap at the door. A nurse sticks her head in, wide-eyed. "I need to talk to you," she says to the doctor.

The doctor leaves, closing the door. Seconds later, he’s back. "I'm sorry, but the surgery has been cancelled," he tells me.

The words are so unbelievable that at first they hang in the air like icicles.

"What?"

"There aren't enough nurses on the floor upstairs."

I look over at the OR and the expectant faces waiting around the table for my boy. I look down at my son.

I picture us taking him home, trying to explain when he wakes up that the surgery never happened. I imagine telling him that we have to do it all over again: nothing to eat or drink that day; the bungled IVs and pain in his bruised arm; the undignified barium up his bottom; the hours of waiting.

I feel the heaviness of his weight sink into me, the lack of resistance, the letting go.

I'm trapped. I can't do this to my son.

"I am not moving until my son has his surgery," I say, speaking with a force that surprises me. "I am not getting up, I am not going home. I am not going to put him through this again."

We sit in silence. Hot tears gather behind my eyelids. I look into his eyes and I can tell he is a good man.

"Let me see what I can do," he says. He picks up the phone and calls a senior executive.

"Thank you," I mouth, as the tears trickle down my face.

The surgeon overrides protocol. Ben goes to the operating room and I go to the waiting room.

I feel depleted and strangely giddy, powerful.

My son may never speak. But he's given me a voice.