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Judith, Paul, Rhianna and JoannaThis page is seventh of the set of Personal Stories of Type I babies. |
By now Joanna had had her six-week check and no problems were found. With two young children, life was hectic. I took Joanna to her fortnightly weight checks and all appeared well. She was breastfeeding and gaining weight well. Then suddenly in early February, our health visitor noticed Joanna's lack of movement and sent me to see our GP straight away. The doctor examined Joanna and said she would keep a close eye on her progress, but didn't seem too concerned. Over the next few weeks Joanna's lack of movement and lack of head control became more and more apparent.
Joanna was referred to a paediatrician. Not one of our health visitors or GPs could give us any idea what it could be. We saw the paediatrician on 31st March and he was very concerned about Joanna. He still couldn't, or wouldn't give us any indication of what it could be, but I didn't, for one minute, think it could be life threatening. However, I did ask and the consultant told us it wasn't at this stage, but he said he would like to refer Joanna to Bristol hospital. The following day, he joined us to say there was a new blood test available, and to bring Joanna down next week, when we were booked for physiotherapy.
We got to the paediatrician on 7th April. Joanna was now five and a half months. We were hopeful - at last it seemed that someone knew what might be wrong. People had been telling us not to worry; that there is so much that can be done these days for babies and children. Nothing could prepare us for what we were told next. The paediatrician suspected that Joanna had Spinal Muscular Atrophy (SMA) and if she did, she would not live to see her second birthday - realistically, she would not see her first birthday. When you are told something like this, your whole world falls apart. All your hopes and dreams for the future seem to be taken away, you can hope the doctors might be wrong, but you realise this is unlikely.
After being told what they thought was wrong with Joanna, she had to have a blood test to confirm whether she did have SMA. The result would take ten to fourteen days. In this time, we got a lot of information on SMA from the JTSMA web site. After reading this, we felt sure that this is what was wrong with Joanna. One sentence summed up Joanna: 'what they seem unable to do physically, the appear to compensate for with facial expressions, particularly their beautiful eyes and smiling faces.' Complete devastation is what you feel when you are told your baby will die and nothing short of a miracle will stop it. We got the final result on 22nd April 1999 - Joanna had Type I SMA. We had already started to tell friends and family about Joanna's condition. Family and friends alike were very shocked - nobody had heard of SMA. Even our health visitor and GPs had not heard of it, let alone seen it. By the time we saw the paediatrician, we knew that Joanna had a major problem, but not something, which was going to mean our baby daughter would die, most likely before she was a year old.
After being told all of this, we felt the only thing we could do was to take each day that we had with Joanna as it came along, and to make the most of the time that we had with her. Joanna was still very happy, very alert and had the most amazing blue eyes that would follow you everywhere. We took her for days out; we took her to the zoo, the Shire Horse Centre, to the shops. Joanna loved being outdoors and meeting people. Complete strangers who didn't know about Joanna's condition would comment on her beautiful and knowing eyes. We carried on with life as normally as we could. Joanna had a smile for everyone and was quite content.
The following text is a mixture of our experiences, good and bad, along with what we hope may be some good advice and ideas on how to help both yourselves and your special baby.
Joanna's best 'toy' was probably watching Rhianna, and when in the car they would squeal at one another. Jo would be facing backward in the front passenger seat and Rhianna facing forward behind the driver's seat.
Joanna loved to play with helium balloons, pulling them down by the string. She also enjoyed the Boots 'Octopull' toy, which was originally Rhianna's, because she could feel the different textures, and make it rattle if she hit it hard enough. We haven't had experience of other babies with SMA, but Joanna seemed to move her arms around quite a lot. Joanna always slept in our bed and she had a very good aim in the middle of the night! She loved to explore with her hands and feel the different textures, and she also had a habit of holding her ear. We bought some bells from the Early Learning Centre, which were on a strap and designed to go on a baby's wrist. Joanna also had a mobile, which hung from our living room ceiling, and a wedge, which she lay on.
Joanna also loved watching our cat, Tigger, and touching him. Sadly, Tigger died in a road accident, eleven days before Joanna died; so now they are together again. The best thing that Joanna liked was going out in her pram, and as we live in a village, lots of people would stop and say hello to her, and she always had a smile for them. While Joanna was well, she enjoyed coming with us when we visited Rhianna's friends. Now people say to me that they remember Joanna for her big blue eyes and lovely big smile.
Keep a diary; it will remind you of things that you can easily forget. Take lots of photographs, and if you have access to a video camera, this will capture their sounds, as well as their movements. This is useful in noting any changes in your baby, as well as providing memories later on.
There are organisations such as Homestart*, which can help ease the stress in some small ways. Also, ask about your local children's hospice - as this may not be offered by health professionals, but staying there will really ease the pressure during this time. Lastly, make every day as special and happy as you can for you and your special baby.
Early on, we were told by one of our GPs, while waiting for a referral to a paediatrician, that if it became more serious than they thought it was, they would hurry the referral along. In the end, we phoned the paediatrician's secretary, who got us an appointment within the week. Our regular GP was good, and in Joanna's last couple of weeks, he visited us almost daily to make sure we were all right; but having never seen SMA before, he didn't really know what to expect.
The consultant paediatrician was very good, but our problem was that our hospital was twenty miles away - a long way if an emergency arises, so we chose to care for Joanna at home, with the back up of our health visitor and GP, which they were happy to provide.
Joanna was in hospital for just two days for a chest infection, and although they were caring, when you are on a ward and people ask you what is wrong with your baby, it is not easy. We had an experience when Joanna was sent for a chest x-ray, and the man had a baby of the same age. He didn't know what was wrong with Joanna, and he said, "I wish my daughter would lie still like that", well what can you say! I chose to say nothing. Perhaps I should have said something, but I had more important things on my mind.
The main problem is a lack of knowledge of SMA by health professionals - even for those who have come across it. We stayed at our local children's hospice, Little Bridge House near Barnstaple, and met some wonderful people who helped us care for Joanna, and who cared for other babies with SMA. They were honest with us about what to expect. This is where Joanna chose to be when she died, thirty minutes after arriving for our third routine visit.
I continued to breast feed her and tried to encourage her to eat some solids, then on 12th June, while we were at the hospice, Joanna took a turn for the worst, and it was at this point that she had the feeding tube fitted. This gave both Joanna and us great relief. Looking back on it, we couldn't tell how much fluid Joanna was taking, and she was getting dehydrated. We think, now knowing what we do, that having a tube in earlier would have helped us all. Joanna took very well to tube feeding. She was very content and had a full tummy, it was the happiest she had been for a long time.
I continued to breast feed her for comfort and to get her to sleep at night, but she was not now as dependent on me for feeding her. Joanna had a pump for her feed at night, which we soon learnt to use. Joanna loved to watch us setting it up, and would lie there smiling at it, as she seemed to know that it was good for her. Her tube feeds were done by syringes during the day, something that sounds very daunting, but we quickly learnt to do it. Joanna liked to try to pull at her tube, but luckily, she never pulled it out. Tube feeding sounds very daunting, but when you see how happy it can make your special baby, it is worth it.
When Joanna became less good at taking solids, we added Maxijul powder to her solids - from about May time, and then when she could not take them at all, it was added to her formula milk. Later on, she was prescribed Infatrini, which is a high-energy infant milk feed. This went via the tube, which she tolerated well, and had five 60ml feeds during the day. Then she was fed by pump at night, from 9pm to 7am, with a continuous feed of 400ml in total. We were offered the option of putting her on the pump continuously, day and night, but we decided that this would make going out more difficult. As it was July by now, and very hot, we were worried about the milk keeping all day. We also received quite a bit of conflicting advice on whether this would be the best for Jo, but we felt that it left her with nowhere else to go, if and when she got worse. Joanna finished her final tube feed fifteen minutes before she died.
Joanna used to enjoy her chest physio, and would sometimes go to sleep while we were doing it. We had some splints made for her hands, which she was supposed to wear at night, but these were not successful. Joanna always slept well, better than her sister ever did at her age.
Joanna would dribble lots and lots, and we got through loads of bibs. I used to use flannels for bibs as well. It was summer when Joanna was at her weakest, and her little head would sweat lots due to the lack of movement. We found that moving her to different positions helped. We started to use Rhianna's vest tops on Jo, because they were very thin and were easy to put on. They looked like little dresses on Jo. Lots of Jo's clothes were Rhianna's old ones, but if we bought any, we made sure that buttons were on the front, as our special babies with SMA cannot be comfortable on their tummies, even when dressing them. Remember, you are the expert in caring for your baby, and you know what's best for him or her.
Having an older child around while caring for your SMA baby really helps. Rhianna was very good with her sister. She loved to help with her; she never showed any jealousy towards Joanna and loved to show her off. Rhianna helped to entertain Joanna and make her smile, and feed her chocolate at Easter, when Jo was taking solids. She would squeal and laugh at Jo, and Joanna would smile. Rhianna would also sit beside her and hold her hand and kiss her.
We were worried when Jo had her tube put in, worried that Rhianna might try and pull it out, but she never did. She is now convinced that she had a tube in her nose like Jo, when she was a baby. Rhianna would always wake up first in the morning and then wake up Joanna. Joanna always gave her a lovely smile.
Rhianna held her sister after she died; we felt it was important for Rhianna that she saw everything and we hid nothing from her. Others might not agree, but its how we wanted it to be. Rhianna talks about her sister a lot. She was two years and eight months old when Joanna died. She remembers lots of things that she did with Joanna, and she now squeals at photos of Jo, and shows them to anyone who visits our house. She picks lots of flowers from our garden to take for Jo when we visit the cemetery.
© JTSMA February 2000