Triumphant Tuesday - Breastfeeding a Baby With Prader Willi Syndrome

You are about to read the story of a mother who got a shock when her baby was born with Prader Willi Syndrome. Routine ultrasounds had not detected this non-hereditary genetic condition which results from a problem with chromosome 15. The syndrome typically causes the child to suffer from low muscle tone with motor and cognitive developmental delays, short stature if not treated with growth hormone, and later, incomplete sexual development. Most children with PWS are floppy at birth with initial difficulties in feeding, but then in early childhood begin to show an insatiable appetite which can be life-threatening. Read on to discover why it IS possible to breastfeed a baby with PWS, albeit not without hardship.


“Our third child was very much wanted and planned. After two boys, people would often ask if we were ‘trying for a girl’. We always replied that we just wanted a healthy baby, and when routine scans and tests showed that he (yes, another boy!) was, to their knowledge, healthy, we were happy and looked forward to meeting him.

Warning signs

At 40+12 I hadn’t notice him move for a bit, so went to my local hospital. I wasn’t overly concerned: this had happened a couple of times previously. This time, despite it being late November, I left the house with no coat and flat shoes (which is testament that I expected to be back very shortly. I always wear heels!). This time however, the CTG was pathological and I was blue lighted to the big, city hospital.

Within an hour I was having a caesarean section. Even at this point, I made it clear that I wanted my baby passed straight to me for skin-to-skin and that I wanted delayed cord clamping. The hospital staff were supportive of this plan and assured me we could go home after 24 hours….

Very low Apgar rating

Unfortunately Gideon was extremely poorly when he was born. His Apgar was 1. His breathing was very shallow, his heart rate was slow and he was extremely floppy and unresponsive.  I felt terrified. He was whisked away with just enough time for the briefest of kisses on his forehead. I thought he was going to die without me being able to hold him. I planned what he would wear to be buried in.

At 20 minutes of age, Gideon was transferred to the Neonatal Intensive Care Unit. I didn’t get my undisturbed skin-to-skin, I managed a fleeting kiss to his forehead and I was left wondering if his first kiss would also be his last. The obstetrician who delivered him said that had Giodeon been born a few hours later he would have been a still birth - a terrifying thought which makes me thankful I listened to my instincts and went to hospital when I did.

Gideon was several hours old by the time I first saw him. The doctors thought his floppiness was due to a ‘bad birth’; lack of oxygen and a possible brain injury. They were treating this by placing him on a cooling mat, which meant I couldn’t hold him or even touch him as his temperature had to be maintained.  It was horrid knowing that I couldn't hold him, but I could understand it was for the best. He was being given sugar water via a line through his umbilical cord at this point.

Pumping frustrations

My husband Peter went home to our two older boys and the hospital found me a private room and a double pump. I started pumping and hand expression immediately.  I had never been any good at hand expressing, so I found this frustrating as well as tiring. Despite being pretty knowledgeable and experienced in breastfeeding (I was still breastfeeding the older boys), this was very new territory for me.

That first night I pumped every two hours through the night. Drops of colostrum which were getting stuck on the pump. It was disheartening and I was scared that if I didn’t start producing something then Gideon would be given formula. As none of my babies have had formula, I really didn’t want that for Gideon – he had enough to deal with as it was.  Providing him with milk was the one thing I could do, so it gave me something to concentrate on.

The next day a wonderful midwife sat with me and helped me express tiny amounts of colostrum. It really was a two woman job – one of us to express, the other to catch the liquid gold. I will be forever grateful for her practical support: on a busy ward she spent hours with me helping me do what I could for my baby.  I continued to use the pump every two hours.

Tests

By day three my milk had come in, but Gideon was not improving. The doctors started further extensive tests to try and find out what was happening – brain scans, MRIs, chest/lung x-rays, blood tests, genetic tests. Gideon was now being fed my milk via an NG tube: he was still extremely weak and still on the cooling mat so I was unable to feed from the breast, or even hold him.

Tandem feeding to boost supply

Peter was traipsing back and forth between home and hospital, trying to look after two small (and very confused) boys, run a house, support me and deal with the fact we had a very poorly third son in hospital.  I was expressing milk every two-three hours and encouraging my older two boys (aged 2 and 4 at that time) to feed as much as possible to stimulate my supply.

After four days, Gideon was taken off the cooling mat and moved from NICI to SCBU. I was becoming increasingly keen to try and feed him, but the doctors were less keen: they were concerned about his gag reflex and the danger of aspiration. I thought that if I could put him to the breast, then he’d know what to do and everything would be OK. It was frustrating.

After a week I was discharged from the hospital. Going home without a baby was very tough. Nights were the worst. He should have been sleeping in my arms in the family bed - but he wasn't. Obviously, my two older boys were a great distraction. They had been very upset by my sudden and prolonged disappearance. We’d been preparing them for a new brother, but were expecting a home water birth, not a very ill baby in hospital. I was still having to express every two-three hours and Zachary, the youngest, was still wanting to be fed lots. I had to make sure that I fed him after I expressed to ensure I got the ‘easy’ milk off first and help him stimulate my supply. Zachary soon learned to hate the pump though – both because it put me out of action so was less attention for him and because he had to wait his turn. I was advised to double pump but couldn’t do this during the day as the children often needed my other hand!

I was still recovering from my caesarean, but really had to ignore the recovery advice and just get on with a very busy life: recuperation was a luxury I didn’t have time for. Gideon was still in hospital so I was visiting him every day – via public transport as I wasn’t allowed to drive at this point. The SCBU was well equipped to deal with breastfeeding mothers: they had plenty of pumps available and I was able to express either in a private room or with screens next to Gideon’s cot. The staff were very supportive of breastfeeding and I often heard them explaining to mums how important human milk is for ill or premature babies.

Peter would accompany me when he could but pre-schoolers and SCBU don’t mix! We did try to find people to help look after the older two, but after such a turbulent time they wouldn’t be left with anyone else. This meant that Peter and I took it in turns to visit Zachary – me with my cool bag of milk and an eye on the clock for the next time I had to express.

Test results

One by one, the tests were coming back with nothing to show – ‘normal’ as far as they could tell. Then, when Gideon was ten days old, we got back some of the genetic tests – which showed a positive diagnosis for Prader Willi Syndrome. My first reaction was one of relief: I had heard of PWS, I knew what it was and I felt better knowing what we were facing.

Immediately I started researching breastfeeding a baby with PWS. In babies, PWS is characterised by extreme hypotonia (floppiness) and failure to thrive. Most PWS babies never breastfeed and many never manage bottle feeds, relying instead on NG tubes. I was determined that my baby would breastfeed and started research straight away.

At this point Gideon was still being fed by an NG tube. He slept almost all day and never showed any hunger cues. He was fed on a timetable, every three hours. I was still expressing milk every three hours, waking at night to try and ensure I established my supply.

Home at last – let the mayhem begin

After three weeks, and just in time for Christmas, Gideon came home. He was being fed via an NG tube and was on oxygen 24 hours a day. How lovely it was to have him at home, in my arms and in the family bed where he belonged.

In the New Year, Peter went back to work and I was at home with three small children, school runs and housework…. I look back now and wonder how I managed. I was expressing every three hours, day and night. I was also breastfeeding the older two. I had to make sure I expressed the easy-to-get milk before I let them feed at the breast. I really struggled to find any information on long term expressing, but did get wonderful support and information from a friend who expressed for over two years for her son.

Expressing in public

I felt very restricted by the schedule of expressing so often. I am happy to breastfeed anywhere, but you can’t really sit in John Lewis café and express milk…. I have expressed milk in just about every car park and disabled loo in the North East of England though! We carried on as well as we could, oxygen and feeding tube notwithstanding. Having two older boys meant we had to just get on with life.

Latching on

I continued to try and put Gideon to the breast – he simply did not know what to do. He didn’t root, he didn’t latch. He would occasionally chomp down, but that was it. I found the experience frustrating and soul destroying. It was also very hard to find the time to keep trying whilst also looking after two other young children. Gideon’s hypotonia meant I had to be extremely careful with positioning as I had to make sure his airway was not compromised. All the tubes just made it harder still.

Bottle feeding

By 8 weeks, I’d met another mum with a daughter with PWS who helped teach me how to bottle feed Gideon. This meant his NG tube was removed. He took an awfully long time to take each bottle – up to an hour and would often fall asleep. He didn’t have the energy or stamina to eat any faster.

So I continued to express. I worried about everything – would my supply keep up, would I meet the needs of three breastfeeding children, would I ever breastfeed Gideon ‘properly’? I continued to have excellent support from friends, from LLL and from medical professionals. I spoke to PWS professionals across the world looking for an ‘answer’ – the magic technique that would enable Gideon to feed at the breast.

Eventually I did achieve a couple of occasions where Gideon managed a couple of mouthfuls of milk from the breast. But that’s as good as it got. Gideon just doesn’t have the strength to breastfeed effectively, nor does he have the correct suck/swallow/breathe coordination. When he was very young, any attempt to breastfeed compromised his airway, so he stopped trying.

Donor milk

I am now expressing milk four times a day and just about keeping up with the help of domperidone and donor milk. Giving donor milk was not an easy decision: my knee jerk reaction is that of course I’d rather have human milk than formula, but there are so many things to consider when accepting it, from the safety (does the donor smoke, drink, take drugs or medications) to the ethics (can I be sure the donor isn’t detrimentally affecting her own supply at the cost of her children’s wellbeing), but my informed decision was that I was happy to accept it.

That said, the first time I gave him donor milk I found it incredibly upsetting. I felt I had failed him in some way by not being able to produce enough myself. I’ve always had an abundant supply of breast milk – but I’ve never been much good at expressing!

Bottle feeding Gideon - even with expressed breast milk - has been a very different experience to breastfeeding my other two sons. I would rather have fed Gideon ‘from the source’ – giving Gideon ALL the benefits of breastfeeding that he misses by getting milk from a bottle, but I remind myself that every single drop of human milk he receives is giving him an advantage. I’m also very aware that quite simply, if it weren’t for modern medicine, he wouldn’t have survived birth and without tube feeding he would have starved.

Writing this, and looking back over the past 13 months, is bittersweet. Trying to pump enough whilst juggling the needs of three young children meant lots of guilt feelings for me – some of which remain! I felt I was not doing anything properly. I suspect most mothers of three feel like that at times, but I still feel terrible that my big boys have suffered, and I feel terrible that they will grow up having to be Gideon's carers.

I’m not sure when it sunk in that I would never feed Gideon at the breast. It was a slow dawning rather than a sudden realisation. It makes me very sad: I wonder if I’d persevered, or given more time, or spoken to more people…would we have managed?  Logically, I suspect the answer is that no, nothing I could have done would have helped him feed at the breast. There is no magic answer. Instead I try and focus on my successes: Gideon was exclusively breastfed for six month; he has never had formula, and at 13 months I am still giving him breast milk. Alongside that I am still breastfeeding his two older brothers who are now five and three. I am hoping to get to two years of expressing for Gideon, despite being back at work now.”

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