Sully's Bench

If there ever comes a day when we can't be together, hold me in your heart, I'll stay there forever - Winne the Pooh

I am a Dragon Mom

Notes from a Dragon Mom by Emily Rapp
October 15, 2011
NY Times Online

Emily Rapp is the author of “Poster Child: A Memoir,” and a professor of creative writing at the Santa Fe University of Art and Design.

My son, Ronan, looks at me and raises one eyebrow. His eyes are bright and focused. Ronan means “little seal” in Irish and it suits him.

I want to stop here, before the dreadful hitch: my son is 18 months old and will likely die before his third birthday. Ronan was born with Tay-Sachs, a rare genetic disorder. He is slowly regressing into a vegetative state. He’ll become paralyzed, experience seizures, lose all of his senses before he dies. There is no treatment and no cure.

How do you parent without a net, without a future, knowing that you will lose your child, bit by torturous bit?

Depressing? Sure. But not without wisdom, not without a profound understanding of the human experience or without hard-won lessons, forged through grief and helplessness and deeply committed love about how to be not just a mother or a father but how to be human.

Parenting advice is, by its nature, future-directed. I know. I read all the parenting magazines. During my pregnancy, I devoured every parenting guide I could find. My husband and I thought about a lot of questions they raised: will breast-feeding enhance his brain function? Will music class improve his cognitive skills? Will the right preschool help him get into the right college? I made lists. I planned and plotted and hoped. Future, future, future.

We never thought about how we might parent a child for whom there is no future. The prenatal test I took for Tay-Sachs was negative; our genetic counselor didn’t think I needed the test, since I’m not Jewish and Tay-Sachs is thought to be a greater risk among Ashkenazi Jews. Being somewhat obsessive about such matters, I had it done anyway, twice. Both times the results were negative.

Our parenting plans, our lists, the advice I read before Ronan’s birth make little sense now. No matter what we do for Ronan — choose organic or non-organic food; cloth diapers or disposable; attachment parenting or sleep training — he will die. All the decisions that once mattered so much, don’t.

All parents want their children to prosper, to matter. We enroll our children in music class or take them to Mommy and Me swim class because we hope they will manifest some fabulous talent that will set them — and therefore us, the proud parents — apart. Traditional parenting naturally presumes a future where the child outlives the parent and ideally becomes successful, perhaps even achieves something spectacular. Amy Chua’s “Battle Hymn of the Tiger Mother” is only the latest handbook for parents hoping to guide their children along this path. It’s animated by the idea that good, careful investments in your children will pay off in the form of happy endings, rich futures.

But I have abandoned the future, and with it any visions of Ronan’s scoring a perfect SAT or sprinting across a stage with a Harvard diploma in his hand. We’re not waiting for Ronan to make us proud. We don’t expect future returns on our investment. We’ve chucked the graphs of developmental milestones and we avoid parenting magazines at the pediatrician’s office. Ronan has given us a terrible freedom from expectations, a magical world where there are no goals, no prizes to win, no outcomes to monitor, discuss, compare.

But the day-to-day is often peaceful, even blissful. This was my day with my son: cuddling, feedings, naps. He can watch television if he wants to; he can have pudding and cheesecake for every meal. We are a very permissive household. We do our best for our kid, feed him fresh food, brush his teeth, make sure he’s clean and warm and well rested and ... healthy? Well, no. The only task here is to love, and we tell him we love him, not caring that he doesn’t understand the words. We encourage him to do what he can, though unlike us he is without ego or ambition.

Ronan won’t prosper or succeed in the way we have come to understand this term in our culture; he will never walk or say “Mama,” and I will never be a tiger mom. The mothers and fathers of terminally ill children are something else entirely. Our goals are simple and terrible: to help our children live with minimal discomfort and maximum dignity. We will not launch our children into a bright and promising future, but see them into early graves. We will prepare to lose them and then, impossibly, to live on after that gutting loss. This requires a new ferocity, a new way of thinking, a new animal. We are dragon parents: fierce and loyal and loving as hell. Our experiences have taught us how to parent for the here and now, for the sake of parenting, for the humanity implicit in the act itself, though this runs counter to traditional wisdom and advice.

NOBODY asks dragon parents for advice; we’re too scary. Our grief is primal and unwieldy and embarrassing. The certainties that most parents face are irrelevant to us, and frankly, kind of silly. Our narratives are grisly, the stakes impossibly high. Conversations about which seizure medication is most effective or how to feed children who have trouble swallowing are tantamount to breathing fire at a dinner party or on the playground. Like Dr. Spock suddenly possessed by Al Gore, we offer inconvenient truths and foretell disaster.

And there’s this: parents who, particularly in this country, are expected to be superhuman, to raise children who outpace all their peers, don’t want to see what we see. The long truth about their children, about themselves: that none of it is forever.

I would walk through a tunnel of fire if it would save my son. I would take my chances on a stripped battlefield with a sling and a rock à la David and Goliath if it would make a difference. But it won’t. I can roar all I want about the unfairness of this ridiculous disease, but the facts remain. What I can do is protect my son from as much pain as possible, and then finally do the hardest thing of all, a thing most parents will thankfully never have to do: I will love him to the end of his life, and then I will let him go.

But today Ronan is alive and his breath smells like sweet rice. I can see my reflection in his greenish-gold eyes. I am a reflection of him and not the other way around, and this is, I believe, as it should be. This is a love story, and like all great love stories, it is a story of loss. Parenting, I’ve come to understand, is about loving my child today. Now. In fact, for any parent, anywhere, that’s all there is.

The Healing Power of Baby Photos

Heartfelt is a voluntary organisation of professional photographers who photograph sick children, these children are either terminally ill, critically ill, extremely premature or stillborn. It is a privilege to photograph these children and their families providing precious memories for them to keep. Heartfelt is dedicated to providing this gift to families in a caring, compassionate and heartfelt manner.

It is an unfunded group and relies on the goodwill of photographers and suppliers who donate goods.

Helen Roberts took photographs of little Sully, she is a wonderful person and talented photographer. The photos we have of Sully and of us as family are very special to us. Thanks Helen.

Ross, of Tara

That is what the hospital writes on baby's name bands until they are named. I hold Sully's in my pocket. I can't write about the last afternoon of Sully's life today because I need more time to describe it as beautifully as we experienced it. We had professional photographs taken again so maybe just showing the pictures will be better. By Friday, May 6 Neil and I decided it was time to let Sully go. Someday I will write more about that too. Sincerest thanks to the all the staff caring for Sully but especially, his primary consultant Peter, surgeon Cathy, his primary nurses Billie, Olivia, Mary and Guy, and Honorah who was Sully's nurse on Friday. As we said goodbye to Honorah we told her how well she had done and she admitted it was the first time she had done it. I was really surprised, she seemed so comfortable with Sully dying and with our grief.

I have been so scared for months that Sully would die, and my biggest fear always was that he would die without me there. My second biggest fear was that I would be afraid. But I wasn't. I am sad, but not destroyed. I love being Sully's Mom, he will always be my 'of' and I will always be his place.

Belated update

Sorry I haven't updated my blog for a while Sully hasn't been feeling well for the last week and it has been extra stressful and exhausting. He is having some airway screening tests today and a CT scan of his lungs. He likely has a urinary tract infection which has been making him feel unwell but the major issue is that he is having trouble maintaining his oxygen saturations when awake and is having "life threatening episodes" several times a day. To help these episodes the doctors have adjusted his ventilator settings to CPAP; Sully is essentially breathing against continuous pressure and controls the rate and volume all by himself (this is actually a step forward). Also his feeding tube may not be in the right place so he is currently getting an x ray...he has mostly been sedated the last few days in order to keep him stable.

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Good day

Back on track today, had a 4hr nap this afternoon...and was still up at 10:30 pm when we left the hospital but content...he loves his monkey blanket it's really cute.

Sullivan Rallies

After sleeping the day away, slowing recovering from his hypertensive crisis, Sully woke up happy and alert. He played hard (lots of smiling, kicking and arm waving) then fell asleep again for the night. Neil and I slept in the parent room just in case. Yesterday took 10 years off my life. Who knows what today brings...

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Backstep

Unfortunately Sully has taken a turn for the worse and has been sedated the last couple of days. The sedation helps his blood vessels in his lungs relax so he can breath easier. He is being tested for infection and started on a course of steroids to bring down the inflammation in his lungs. Neil and I won't be venturing very far from his side this weekend.

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Cyclists raise money for CDH Australia

Neil has recently donated his time to coach some introductory cyclocross clinics. Participants donated a gold coin to CDH Australia. $300 was raised and donated in honour of Sullivan who is still very much fighting for his life. Thanks everyone and good luck in the first race today. For more information about the cyclocross series visit http://cx.pacc.org.au/

Monkey

Thanks for the monkey and clothes Auntie Hannie. I had my vaccines today and was very brave. My speech pathologist also visited me and gently played with my mouth with her finger. I liked it a lot. I now have a tube that puts food into my intestine instead of my stomach because I was swallowing heaps of air and not digesting my food very well. I feel much better and like dancing and singing with my Mum and Dad; they move my arms and legs for me and I laugh.

First Bath

Soothing Sullivan

He had some wind and was kicking his legs and crying so I asked his nurse if I could pick him up. After a few minutes he passed out. Sully is freakishly strong for a bed ridden sicky. I told him he could kick all he liked but I was still stronger. I loved being able to pick him up when he was upset; it is the first time I was able to do that. Sullivan has some trouble with swallowing too much air and vomiting or having painful wind pains. Poor baby, he gets so upset and turns white or dusky because he can't get enough oxygen.

The Hungry Caterpiller

Sully loves this book so much he silently squeals when I read it.

Growing brains and lungs

Sully is feeling well today and had a good echo - the best one so far. He needs to grow more blood vessels in his lungs...and more brain cells. His head circumference is quite small so less sleep and more play is required! Brain growth pushes out your skull...and if you don't use it you lose it. Fortunately I am sure he has inherited lots of brain from Dad and can afford a bit of loss...no one really needs to remember every single thing they read, hear or see.

Happy Birthday Daddy

I had a tube change this morning and have been sleeping and sleeping. I have been having some trouble maintaining my oxygen saturations but hopefully my new tube will help the air get into my lungs better. I am listening to nice music and Mummy is patting my belly. Love Sullivan

Mini Neil

Mini Neil is awake from surgery feeling out his new set up. Little monkey disconnects himself every 2 secs but seems much more comfortable already. I think he has my eyes??

Trachy in!

Sully was a star in surgery. He needs to be flat on his back with no cuddling for a week but then he will be like a new little man.

Day before surgery

waiting

as good as it gets

Kicking CDH ass

Despite having an infection, clotted central line, and potentially teething, Sully experiences moments of wellness and fun. His trachie is scheduled for this Friday.

Beautiful afternoon

Thanks

I just wanted to take a moment to say thank you for all your support. I haven't been able to respond to phone calls ect but your text messages, emails, cookies, dinners, dog walking, and gifts for us and Sully have really keep us going. There really is no good answer when people ask 'how are you coping' but I know that looking back on the past few months, each time someone sends some encouragement, the love just pushes us forward. Last night home to cookies a friend dropped by and in the morning I opened my mail on the way to the hospital and our friends from Canada had sent us a beautiful card and the most special keepsake necklace. The pendent is little footprints and Sully's initials sjr. I love it, best gift ever. Thanks Em and Andrew. To everyone has asked if there is 'anything you can do'-- yes, there is: please still be there when we resurface from our crisis because I miss you!! The photo of Sully is him showing off a new blanket from cousin Andrea. The nurses all knew the blanket must be from "overseas" because it was so trendy! xox Tara

Sully is

Sully is magnificent, special, beautiful, courageous. And either surviving or dying and I don't know which for sure.

We met with the doctor on Tuesday and he confirmed that Sully's symptoms late last week were a result of him not coping with the weaning of nitric oxide (gas delivered through ventilator). The doctor also told us that the PICU team consulted with colleagues worldwide and everyone is pessimistic about Sullivan's ability to survive his pulmonary hypertension. So, sorry not to update the blog this week, but it has just been very painful.

Neil and I have consented to a 2 month trial of oral medications (vasodilators) to try and get Sully off the nitric (and eventually off the ventilator). We also consented to a tracheotomy to allow more comfortable ventilation. It sounds hideous, but after healing from the trach surgery, it is much more comfortable and the kindest thing we can do for him. If the hypertension actually does start reversing, it will still be 4-6 months on a ventilator, and Sully would have to survive the cold and flu season. The doctor was very clear that Sully would not survive the common cold and unfortunately the PICU is full of kids struggling with colds in the winter.

Sully isn't gaining that much weight (he is still only 3.82kgs) but is managing to tolerate all his feeds of 25ml per hour, so the dieticians suggested adding polyjoule (sugar) to the breastmilk to add calories. We have tried formula twice before and Sully just threw it up, so we are hoping polyjoule is easier for him to digest. And hopefully he grows blood vessels with the extra calories, not just hair and nails!

Sully is a tough nut.

Wobble

Sully has had a rocky 24hrs with some hypertensive and bradycardic episodes but nurse Mary has him bundled and quiet today. He got re-intubated with a new tube yesterday to see if it made a difference. But he is also showing possible signs of inflammation or infection. Not really sure what is going on. He is on a bit more support again--bit more nitric, oxygen, higher pressures on the ventilator. An echo is scheduled for later today to check out his heart again.

My boys

Big boy bed

We moved Sully out of his bassinet onto a bed. Much easier to cuddle and play with him! All the nurses say that Sully looks like a mini Neil.

Nudist

Sully's nudist days are over and he got dressed for the first time. He really didn't mind getting dressed--it was the extended photo shoot that he objected too. He LOVES being wrapped really tightly in his muslin blanket.

Yum

Sully figured out how to put his fingers in his mouth. He has had a rough few days with a little fever one night and some vomits. He seems ok now though; Drs just monitoring for infection.

Well done!

Sully let the nurses re-tape his ventilator tube without drugs and everyone is very proud! He also impressed them by lifting and turning his head while prone and giving them lots of smiles!!

Peter Pan

When babies are in womb they receive oxygenated blood through the umbilical cord; they aren't really using their lungs so their circulatory system operates slightly differently. All babies are born with some level of pulmonary hypertension and there is a duct in the heart that acts like a blow off valve. This duct was open in the womb and allowed blood to skip the lungs by flowing backward through the heart out to the body. This duct closes as the hypertension naturally resolves and babies make the transition from fetal circulation to grown-up circulation.

Sullivan's hypertension was so bad that this duct needed to remain open or he would have gone into heart failure. Essentially the doctors were saving him by allowing him to use his fetal system until the pressure in his lungs reduced.

Two days ago (and based on an echocardiograph) Sully's doctors decided he was ready to grow up and took him off a drug that was helping to keep this duct open.

He had another echo yesterday and so far so good, the duct is still open a bit but his heart is working as it should and no signs of heart strain.

This is all very good! And I am reading him Peter Pan. We cuddle every afternoon for at least two hours until my extremities go numb and my bladder starts failing me.

Love therapy

Sully watching TV

Baby Einstein! For his brain, babies who have long hospitalizations tend to have delayed development. Brain development is the hardest to catch up on. Reading, singing, and giving Sul things to look at are really important.

Worth the wait

Sully and I love skin to skin!

Hope

Neil and I never know what we are going to get when we walk through these doors. We tend to call every few hours when we aren't here so we have a sense of how Sully is going. This reduces my anxiety, but I still find myself holding my breathe during the walk from the door to his bassinet. Before Sully was born we were clearly and repeated told that he was in a poor prognosis category for CDH. And while he has been very fragile since birth we have seen small improvements. However, when we met with the doctors on Thursday they informed us that Sully's pulmonary hypertension has not improved and that they were not optimistic about him being able to grow out of it, and were unsure if he would be able to be weaned off ventilation or his medication. I felt the hope drain right out of my body and I felt limp right through to the next morning when I just could not get out of bed. Neil pulled me out and we talked about all the improvements in Sul that WE saw. Neil reminded me that the doctors didn't tell us he was dying, they just weren't sure if he was going to live because they hadn't seen a baby so sick survive. If Sully did live they weren't sure if he would have additional health issues due to all his treatment. Since that conversation we have had cuddles with Sully everyday and he has been doing brilliantly. It is impossible to live without hope and I will hope and hope until it is clearly time to let go. I have dug right into the very deepest part of my stubborn nature to walk through these doors everyday and watch, love, and support our baby surprise and delight everyone.

Our first cuddle

Sully didn't even notice he wasn't in his bassinet! He was on a pillow on my lap as a first step to a full out cuddle. It has been a rough week so the nurse decided it was time for Sully and Mom to have a snuggle. He is over his second chest infection and is tolerating feeds again which were the main dramas of the week. We are having a meeting with the doctors and primary nurses this afternoon to discuss next steps in his treatment. Sul didn't make the transition from nitric oxide to Viagra but his ventilator pressures are down.

Wide Awake

Nana and Sully

hanging in

Sullivan has had a very good two days and so far no more infection, they grow his blood culture for another day just to be sure. He is almost off the nitric oxide and his withdrawal program is going much better. I, on the other hand, am exhausted.

Sullivan meets Auntie Jenn

Baby-ish

Sully's heat lamp has been turned off and he is being bundled. All in preparation for our eventual first cuddle! He has been struggling with his drug addiction this week in addition to his infections. Sul was having lots of withdrawal symptoms like tremors, vomiting, yawning, sneezing, crying and general agitation. All of this discomfort has a negative effect on his pulmonary hypertension. Sully's pain and sedation medication has been changed to methadone and Valium which are apparently easier to wean and are longer lasting. We are still waiting for the results of his latest blood test, hoping the infection has cleared.

Sullivan Slideshow

These photos were taken by Helen Roberts when Sully was 4 days old. We thought we were going to lose him that day...the hospital provides a photographer free of charge for families of critically ill babies. Click on the photo to view a flicker slideshow of a few more of these beautiful pics.

Visit from Captain(s) Starlight

I caught these two Captain Starlights admiring my baby this morning when I got to the PICU...so I asked for a photo and a balloon animal...they made Sully a green giraffe balloon. Sullivan seems to be kicking the infection and the doctors have started to address his underlying condition again -- the pulmonary hypertension. It will be try number 3 to get Sully off Nitric Oxide by increasing his dose of viagara (to dilate the blood vessels in his lungs)and S-L-O-W-L-Y reducing the nitric oxide.

EBM

EBM is the medical acronym for expressed breast milk. Sullivan is off yucky IV fake food and is being feed 100% of the good stuff. I am pumping every 3hrs during the day and 4 hourly overnight. Neil has been a huge support by getting up with me in the night when I have trouble. If he hadn't taken photos as evidence, I wouldn't have believed him that I could hold the bottles on while asleep at 2am!

Expressing is weird, especially at first, the midwives helped me "hand express" first to bring the milk in (literally, I was so tried I let them touch my boobs). They used a syringe to catch the first bit and eventually I was ready for the pump. I was really worried about whether I would be able to produce milk with all the stress, but it is going well so far. I think the most important thing I have done is stick with it, I almost gave up one day because Sully was having such a hard time. I was exhausted, stressed, and missed a whole day just because everything was so full on. It was Neil who really encouraged me and also one of the midwives on the ward. She simply said, you don't have to do it--it is something you should do only if you want to.

Keep fighting Sully!

Sullivan's blood test came back positive for an infection so his antibiotic was changed to best match the specific bug. Infections are a side effect of having IV lines in for long periods and of being on the ventilator.

Most adults wouldn't survive what Sul is going through right now. Our surgeon says that babies are more resilient than adults. Sully has a tube down one nostril going to his stomach, the ventilator down the other nostril, an IV in one hand, one foot and in both inner thighs...he has just a bit of lung but still tries to breath over the ventilator when he can and has had major surgery to rearrange his guts...now he is fighting a blood infection and pneumonia. I can feel how strong he is when I sit beside him and hold his hand.

Dig deep little one...

step forward step back

Nitric oxide is back on today and it seems Sullivan may have an infection so some blood was taken and he is on antibiotics just in case. Neil and I have been concerned that he has been showing withdrawal symptoms from the narcotics he has been on since birth. One of the consultants was around to see the symptoms we were concerned about and is addressing it more systematically. Poor little chipmunk!

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Look Mom no nitric oxide

One less machine, Sully is off the nitric oxide and on an oral medication delivered through his feeding tube instead. The nitric oxide is a gas running through the ventilator to treat his pulmonary hypertension. The new medicine does the same thing and we can give it to him at home eventually.

Wretching

Sully had a rough night last night he was throwing up and crying for hours. The volume of his feeds were doubled and his little tummy got upset. The feeds were halted then reintroduced more slowly. Today's challenge was to be weaned off Nitric Oxide and replace it with an oral drug. Sullivan wasn't keen and started to plummet so the NO was turned back on at a low dose. The doctor adjusted his ventilator settings while Sul was "cracking it" and found a sweet spot so he is having a lovey afternoon. He got a line removed out of his arm, so one less needle poking him! Mummy is going to go have a nap in the parent's overnight room.

Rocky days ahead

Sully is being challenged over the next few days, the doctors are trying to get a sense of where he is at on less sedation and less support in order to make some longer term treatment plans. The main concern is a life threatening condition called pulmonary hypertension which he has as a result of having little lungs (and not well developed) but a normal amount of blood volume to get pushed through to be oxygenated. So he has been weaned off some drugs and placed on others and the doctor is trying to slightly alter the ventilator settings. Sullivan needs to dig a bit deeper with each change. The process is commonly described as two steps forward one step back. He has made some progress with some things like he is now being fed 4mls of breast milk each hour, is pooping, got his catheter removed, and is tolerating been turned onto his belly without his lungs collapsing! In fact, I think he likes it. Sully also has the best hat collection in the ICU.

transition from sprint to marathon

The past couple of days have been rough for me and Sully. He has had some difficulties with the ventilator - bit complicated for me to try and explain but one issue was he was trying to breathe on his own and got upset and hyperventilated which caused a bunch of problems. At the same time, the ventilator was not calibrating properly and well, it was just a couple of days of up and downs. When Sullivan gets stressed his pulmonary hypertension (too much pressure in his lungs) gets worse and his blood desaturates (gets less oxygen) which is bad. And when Sully's is stressed, I am stressed. Today has been a much better day so far, he tolerated getting turned on his belly and the doctors are weaning him off some drugs including sedation so he will wake up a bit more. He needs to be more awake to cough, cry (without sound because of ventilator), and wiggle to move fluid out of his lungs (amongst other benefits). It is hard for me to see him cry and not be able cuddle him. During the last two days I have started to come to terms with what it really means to have a really sick bub and that as a parent there is not way ever of taking away a child's pain...no magic wands! Anyway, Sully's is a little soldier that's for sure. I bought him some new hats and decorated his room to lift my spirits.

Me

I finally had the courage to leave the hospital grounds Sunday morning. Neil and I had a chat with one of Sullivan's doctors, and to be perfectly honest I needed to ask if we had minutes, hours, days to get to the hospital if Sul's condition started to deteriorate. The doctor said days, and that everyone, including us, would be able to see what was happening. He was very kindly and told me I looked exhausted and needed to go home to rest. I was a bit teary when Neil took this pic but we had a plan to go back for an evening visit. I am feeling a better having slept in my own bed, and have had a couple of naps. We call the ICU nurse whenever we feel like it to get an update on how is is going so we call every few hours including in the middle of the night, so we sleep a bit easier.

First Feed

Sully had his first poop 3 days after surgery (Monday) so we had the green light to go ahead and try a feed - 2 mls of breastmilk every 4hrs. Sully tolerated this very well we are very excited that his guts are working in their new position! I have been expressing milk every 3hrs during the day and 4 hourly in the night. I owe a entire post to expressing milk so I will leave the details for now. If we are at the hospital for the feeds, Neil and I are given the task of pushing the syringe of liquid gold through Sully's feeding tube, which goes through his nose into his stomach. The nurse checked that it was absorbed by putting a syring back on the tube 2hrs after the feed and pulling up to see if any was left in his belly. Sounds gross but thought someone out there may be wondering.

Post Op

Sully's big day

I took this pic in the wee hours of this morning...Sully did really well in surgery today. All of his organs were up in his chest and now have been moved down to their proper place. He has a patch filling part of the hole.