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Milandre is a total blessing! Her journey with PKU by Marian

Being able to fall pregnant naturally was such a big blessing for me, as our family has a history of #endometrioses. I had a very difficult pregnancy struggling with pre-eclampsia and extreme back pain due to all the swelling. The normal tests were done (e.g. Down syndrome etc.) and everything was normal. Our first baby was going to be perfect. From 32 weeks I was booked off from work and had to report to our house Doctor every second day for a check-up. Living in a small town with the nearest hospital services being 200 km away from us, the doctor was not taking any chances. I opted to have my baby under the supervision of my gynecologist in Bloemfontein and to give birth at Medic Clinic Bloemfontein, as it was the closest city to my parents and I wanted them to be able to be there for the birth.

I wanted my husband to have family close by as his parents live in Namibia and couldn’t come down for the birth. At 35 weeks I was injected to boost Milandré’s lungs as the doctor was suspecting that I will not be able to go the full term with my blood pressure being too high since it is a too great risk for me and Milandré. Every day I was at the doctor and when he said: “I think we must do a cesarean section earlier.” I was in tears, begging it is too early because she is too small. At 36 weeks I was admitted to hospital to monitor my blood pressure and baby. After about 3 days I could go home, but I was basically on bed rest. At 38 weeks the gynecologist said that it was time to get Milandré out as it was becoming too big of a risk. I was so scared that she would be too small and her lungs would not be developed properly and it would cause major future health problems.

On 11 August 2014 at 08:00 I was pushed into theatre. I was so scared as this was my first pregnancy, not knowing what to expect. Listening to the hospital staff making jokes like it was just another day in the office and praying all together: “Please God please just let Milandré be OK…” I suddenly heard her crying and I started crying with her. Hearing her cry, I knew she was OK. I saw her briefly, but she was the most beautiful baby girl I’ve ever seen. She was so perfect. Her Apgar score was 10/10. After a while which seemed like hours, she was pushed into my room. We started the feeding process and I was determined to breast feed. We struggled a bit and she was very tired.



They measured her sugar and it was a bit low so they gave her feed with a cup at first just to get her going. Eventually we got the hang of it. My baby girl was perfect. I was breast feeding. The horrible nine months was a distant memory and life was perfect. I was a Mommy. They did all the normal tests as per usual for all newborn babies. One sister came in and told me that the hospital started offering Newborn Screening this year which is performed at the North-West University Potchefstroom’s Newborn screening laboratory. It was some metabolic testing for which they only needed a heel prick sample of Milandré on special filter paper (DBS / Guthrie card). To be honest I was so tired, I did not listen and just wanted her to leave.

She asked permission to do this test on Milandré. I just said yes so she would leave and I can get some rest…thank God I said yes! The testing never crossed my mind again, my baby was perfect.After I was released from hospital we went to visit my parents for a few days before taking the long road trip home, 500 km from Bloemfontein. Milandré was such a good baby and only feeding once at night. I thought how lucky can I be… Then on the day we drove back to our house our lives would change forever. I remember it like it was yesterday. I got a phone call and it was a sister from Bloemfontein. All she said was they picked up something in the tests done on Milandré and more tests were needed to confirm.

When I asked her what they picked up she could not answer me. I was so devastated and angry I told her to let someone phone me that knows what is going on. Then our pediatrician phoned me, also not a 100% sure what they picked up, as he had never heard of it before. He requested we do a blood draw as soon as possible. We were half way home so we agreed to do it the following morning at our GP. The next day we were at the doctor for the blood test. The sister attempted to draw blood on Milandre’s hands without success. The Doctor attempted to draw blood from her feet…without success. Needless to say that I was crying harder than Milandré every time and was eventually told to go and wait outside.

I was so angry! How could they do this! For some stupid test! You can see she is perfect! Eventually we were sent to the next town where there was a pathology laboratory. There would be someone professional that can draw blood from a week old baby…so they said… After an hour’s struggle and more holes in my child hands and feet and neck they got the blood they needed. I was crying all the way home. I felt so helpless. I’m supposed to protect her. The blood was send to PLIEM laboratory in Potchefstroom (laboratory where the confirming analysis is done) as the first tests were done at the Newborn Screening laboratory also situated in Potchefstroom. Milandré was diagnosed with #Phenylketonuria (#PKU) two weeks after birth. We had just finished our two-week baby photo shoot when I got the phone call from the pediatrician.

We were to be in Bloemfontein the following day to discuss the results. I felt like I was caught in a nightmare. I wanted to do the tests again as there had to be some mistake. My daughter could not have some kind of disease.

The following day we were in the pediatrician’s office in Bloemfontein. Not knowing at all what PKU was, we sat there waiting in anticipation. Unfortunately, our pediatrician knew just as little about PKU as we did. Before Milandré’s diagnosis he had never heard of it before. He talked to Prof Chris Vorster from PLIEM and NBS laboratory that had done the tests to find out what this disease was and how it can be treated. Milandré was the youngest child diagnosed in South Africa.

We were stunned, we just sat there staring at him when he told me I need to stop breast feeding immediately and she can never have protein and she can become disabled if we don’t follow the guidelines for her special diet for PKU patients. She will have to take a special milk formula for babies with PKU. My whole world came crashing down that day. My perfect baby can become disabled…

After Milandré was diagnosed, it was an uphill battle. We struggled for almost a month to get her special milk formula into the country, since it had to be imported from overseas. Prof Chris Vorster gave our pediatrician the number of the metabolic specialist dietician, Dr Tuschka Reynders, who is familiar with PKU and the dietary needs and intervention.

I thank God every day we were fortunate enough to meet her. Without her our baby girl would definitely not have been so healthy today. She helped us get a formula we could use while waiting for the right one and she adjusted the amounts for Milandré’s age. My peaceful baby went from being an angel to being a complete nightmare in one day. She was not getting full from the milk, she was crying the whole time, and she was getting cramps from the new milk. She went from feeding only once a night to 4 times. We had to do blood testing every week to monitor phenylalanine levels. One day our pediatrician phoned us. Her levels were over 1300 µmol/L and for a PKU patient under treatment, the value should be below 400. This elevated level could therefore cause brain damage.

We had to get her to hospital as soon as possible. Immediately I was angry, because I knew this meant a whole lot of tests again. We had to drive all the way to Bloemfontein again getting there at night. To our surprise our pediatrician was not working the weekend. I felt like I was going to have a heart attack. They wanted to admit her and I wanted to know why and what the purpose would be for the admission to hospital. I had done my research in the meantime, so I knew only diet can manage the levels so why does she need to be admitted? Eventually we were told they want to take her milk completely away and put her on a drip only.

I thought these people were insane… I refused. How can you not give a baby milk? The doctor on call came and we had to explain to him what PKU was. Eventually we got him to realize that admitting her won’t make a difference. The test results they received were just after we were discharged from hospital so we needed to get the newest results to see the change in the levels. After four hours of fighting and getting a drip for no reason at all – which was also three holes again – we could go home. I was so angry I felt like suing someone…

With the help from Tuschka we got Milandré’s levels down to below 400 µmol/L in a month’s time. Today Milandré is a healthy girl, full of life and smiles.

She is reaching her milestones so far all in perfect time. She is at day care during the day and loves it. We are still struggling to get her levels to stabilize. Teething is playing a big role in the spikes, as well as tonsillitis. Having a child with PKU is a daily struggle: from having to plan everything perfectly to accommodate her and her meals to having to explain to other Mom’s at day care what she can eat at their kid’s party. Milandre is getting to the age where she now wants to eat what you eat so the struggle is getting tougher. Every time she starts teething or gets sick her levels spike, since illness has an influence on the metabolism, especially in a person with an inborn error of metabolism (Milandré’s case: PKU).

There are so few people who know what PKU is that even taking her to the doctor is a battle as they have prescribed medicine in the past that has protein in. If there is just more awareness of PKU in South Africa there would be less pain and suffering like our hospital episode, not even mentioning the financial implication of all the tests and specialized milk formula that she will have to take for the rest of her life. I don’t believe in luck, I believe in God. And till today I believe it was God who gave us guidance to do the birth at Medic Clinic Bloemfontein. Had it been in any other hospital, Newborn Screening would not have been done and I would be writing a very different story. We are so blessed to have picked it up so soon. I pray to God we can keep her healthy.

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