Born with a Cleft Palate

I have two children, William, aged three and a half and Freya, who is 19 months. After the initial problems latching him on in the early days, William breastfed happily and uneventfully until he was 18 months. I joined La Leche League when he was eight months old as I wanted to meet other mothers who saw breastfeeding as a wonderful and valuable way of mothering their babies. William weaned when I was halfway through my pregnancy with Freya. I remember his last breastfeed very clearly as it was the first time in a long while that I hadn’t felt irritated by nursing him. He looked at me and I looked at him and we both knew it was the last time. By the time he weaned my supply had all but disappeared and once he stopped, he never asked again.

I was born with Pierre Robin sequence (PRS), which involves a cleft palate, small lower jaw (which grows to a normal size by adulthood), and a tendency of the tongue to fall backward producing chronic airway obstruction and difficulty feeding. PRS can occur spontaneously but can also be inherited, or it can occur alongside other syndromes. In my case it occurred as a result of Stickler syndrome, which affects the collagen genes and has a 50 percent chance of being passed on to my children. Only when I was pregnant with William did I realize the
significance of this.

My mother often talks about my early days and how she had to cope with me having PRS. I was in a hospital 60 miles away from where my parents lived until I was 18 weeks old and I had a tracheaostomy until I was two and a half. If this were what having a baby with PRS would be like for me, I couldn’t worry about it. The worry would have overwhelmed me!

I had a calm and positive labor and found I didn’t need any pain relief. Freya was delivered onto my chest and I had a precious ten minutes with her. I loved her instantly and passionately. She was managing to breathe on her own and I was desperately hoping that this meant she didn’t have PRS. But we started to notice that she was blue around the lips and so she was taken over to the resuscitation trolley and from there to the Special Care Baby Unit (the SCBU). When we saw her next she had a naso-pharyngeal airway in, an oro-gastric tube, and a big splint on her hand. But she was beautiful: her pink skin, her golden hair, her lovely shaped head, and her gorgeous smell.

The next few days were a bit of a blur. The shock of having a baby with ‘issues’ only struck me when she was three days old and I felt terribly guilty for even having her. Then there was the shock of having to learn to deal with a baby who needed quite specific nursing care. I felt there needed to be five of me: one to look after Freya, one to look after William (who was still only 22 months old), one to express my milk, one to sort everything out, and one to lie down in a darkened room to recover. I was able to stay with Freya in the SCBU overnight but had my meals on the prenatal ward, where there were lots of lovely posters promoting breastfeeding and women breastfeeding their newborn babies. I found I couldn’t even look at them without crying. I had a baby who, in her first days at least, could not even be held without stopping breathing and whom I knew I would never breastfeed.

But I could express my milk for her. She had received formula after the donor milk ran out and before my supply picked up. The Infant Feeding Advisor understood how disappointed I was and told me to think of the formula as medicine. This was a good suggestion for me and I chose not to dwell on it and got on with expressing. I knew I had to express regularly and I soon fell into a pattern that was just about workable, combined with looking after William and Freya and fitting in sleeping! Fortunately my husband was able to get a significant amount of time off work and he was able to look after William so I could be with Freya as much as possible. We learned how to hold Freya so she could breathe easily; we learned how to feed her carefully with her naso-gastric tube; we learned to watch her for signs of breathing distress. We had to learn how to insert a naso-pharyngeal tube into her nose to support her breathing overnight, which was one of the hardest things I’ve ever had to do. We had to feel confident in doing this before we could take her home and there were times when I despaired that I’d ever be able to put that tube in.

At five weeks old Freya finally came home. I often felt overwhelmed with the demands of looking after William and Freya, expressing regularly, tube feeding and all the rigmarole that went with it, and looking after the house. We soon found that inserting the naso-pharyngeal tube at night made Freya’s breathing far more labored and so we took the decision, against medical advice, to stop putting it in, instead relying on the saturation monitor to tell us if she was breathing. This, along with everything else, was very stressful and we were glad of the support we got from the community pediatric nursing team and the cleft nurse, who came to visit us regularly. In her early weeks, Freya was not able to coordinate sucking, breathing, and swallowing and would become very distressed if any amount of liquid was put in her mouth. But at 13 weeks we found that Freya was able to coordinate these actions to the extent that she could take all her milk from a bottle and her naso-gastric feeding tube was permanently removed. It was wonderful to be able to feed her in a way that enabled her to have control over what she was eating, to be able to feed her in a ‘normal’ way.

I carried on expressing and was making so much milk that I was able to freeze huge amounts. I was very pleased about this. We were told that we should start Freya on solids at 16 weeks, which I was very reluctant to do. Babies with a cleft palate are more susceptible to ear infections and developing ‘glue ear’ than babies without and I knew that exclusive breastmilk feeding would help to lessen these risks. Instead, I decided to let Freya take things at her own pace. She started to nibble on soft carrots when she turned five months. I wanted to let her have as much freedom with food as possible, especially after three months of tube feeding but it was hard to know what to feed her. She struggled to chew up harder foods because of her wide cleft palate and very soft food, such as yoghurt, just ended up coming out of her nose and she’d get frustrated. So she continued mainly on my milk. By the time Freya was five and a half months, my milk supply was starting to diminish and, to my intense disappointment, I found that all the milk I’d frozen had taken on a distinct smell and taste caused by the action of the lipase enzyme.* One day, I found that I didn’t have any milk to express. And she was refusing to drink any of the milk I had frozen for her. She still wasn’t eating much solid food so I had to give
her formula. To say I felt disappointed about this is something of an understatement. Ultimately she needed feeding and, at the very least, formula provides some form of nutrition. I had heard Freya cry from hunger and I never wanted to hear her cry like that again.

Herbal supplements hadn’t helped to increase my supply and I was finding it impossible to express more frequently as well as look after William and Freya. It was very stressful and the anxiety I was feeling about my milk supply did not help me to express. I phoned the LLLGB Helpline one day and spoke to a Leader, who suggested I might request a prescription for domperidone** (a drug generally used for disorders of the gut, which as a side effect increases milk production). Fortunately my doctor was happy to prescribe it to me on a repeat
prescription. This helped maintain my supply enough to provide approximately half the milk Freya needed until she was a year old. I didn’t enjoy expressing. I found it time consuming, boring, and stressful. When Freya was a small baby, I’d express sitting on the edge of my bed with her lying beside me along my thigh. As she got older this became harder to do; and I’d often have to combine expressing with keeping William from hurting Freya (as two-year-old siblings may often do), keeping him occupied, or keeping Freya from crying. I never really managed to hold her while I expressed. I expressed in the car, in public restrooms, in rooms full of relatives. I expressed at a friend’s house and at LLL meetings. I didn’t really care. What was difficult was that only I knew I had to do it; there was no baby needing to be latched on and so I had to be very disciplined.

I was often in turmoil about expressing. On the one hand, I hated it — the amount of time it took and that it took me away from being with William and Freya, the relentlessness of it, the fact that I could clearly see my milk supply declining despite my best efforts but, on the other hand, I couldn’t face stopping and being unable to give her my milk. So I just carried on.
My goal was to keep expressing until Freya had her cleft repair. Not only would I be able to feed her my milk when she was recovering from the surgery but also I might be able to teach her to breastfeed. Although the initial repair was a success, about a week afterwards Freya started to cry loudly and we found that a hole had formed in her hard palate. I felt terrible, but there was nothing to be done but wait to see if the hole closed up by itself.

In order to encourage Freya to breastfeed after the repair, I was advised to keep her with me all the time and at feeding times rest her against my breast — just to allow her to get used to it. However, she much preferred her bottle. And even if she had attempted to breastfeed, I very much doubt she would’ve been able to extract milk from my breast — she was unable to suck because of the hole in her hard palate. Freya’s cleft repair occurred when she was ten months old and it was then that I realized I might be able to express for the rest of her first year. We went on vacation over Freya’s first birthday and I stopped taking domperidone. I only expressed once in 48 hours and then stopped completely. I didn’t experience any fullness or leaking after I stopped. Even though I knew that it was the right time for me to stop, and I felt that I literally couldn’t do it any more, I felt very sad that Freya would no longer get my milk. Since being involved with LLL I’ve come to realize just how important and normal it is for a child to breastfeed throughout the toddler years. A week later, I had a long conversation with my local LLL Leader, about whether I should start up again. I came to the conclusion that I would not.

Freya is still of an age at which it is perfectly normal for a child to be breastfeeding and there are times when I long to be able to breastfeed her. When I first realized that I wouldn’t be able to breastfeed Freya, I felt almost as if I’d lost the use of a limb. Like many women who for whatever reason find they can’t breastfeed their baby, I’ve gone through a process of grieving for what we’ve lost. When feeding William I really discovered the ‘magic’ of breastfeeding and just how good a mothering tool it is –so much more than just providing nutrition — a complete way of loving and communicating with your baby. How would I cope with a baby without being able to do this? Well, we have coped; in fact, we’ve coped very well. I learned how to mother a baby through breastfeeding William and I hope that I have used what I learned to mother Freya as responsively and as kindly as possible. Freya is now two and a half years old. The hole in her hard palate closed up by itself to our immense relief. Shehas a big personality and even bigger hair! We may have had a difficult start but she is now a happy, healthy little girl — and I am so glad I was able to give her my milk for a year.

* A few mothers find that their refrigerated or frozen breastmilk begins to smell or taste soapy, sour, or even rancid soon after it’s stored, even though all storage guidelines have been followed closely. The speculation is that these mothers have an excess of the enzyme lipase in their milk, which begins to break down the fat soon after the milk is expressed. Lipase is an enzyme that is normally present in human milk and has several known beneficial functions. By scalding the milk as soon after expression as possible it is possible to inactivate the
lipase and stop the process of fat digestion. See our post on Milk Issues for more information.

** Information about domperidone. It is not approved for use or sale in every country.

Originally published in New Beginnings,Vol. 26 No. 2, 2009. At the request of the author some names have been changed.