NS

NS
The Most Common Rare Syndrome You've Never Heard Of

Monday, April 1, 2013

Lynda Robinson -- Connor

       

 I was married to my husband for a few years and although our marriage was a stormy one, we were taking care of our first born son Lee together.  I had suffered an ectopic pregnancy before getting pregnant with Lee. Dr’s weren't sure if I would be able to have any children as I had lost one of my Fallopian tubes and they could not tell whether my other tube was OK or not. We were really lucky to have our first born miracle. Then when Lee was eight months old I had a miscarriage. Just more stress to add to the already worrying Mum whose biological clock was ticking as I was already 31 years old. I wanted so much to have a sibling for Lee. On April fool’s day 1993 I found out that I was pregnant, so I got my wish and I was over the moon. Pity my husband didn't feel the same way. This only put more strain on our marriage and my husband caused huge arguments about not being able to afford another baby. For the following few months our marriage became really strained and my health wasn't good. I worried about my unborn child and the thoughts that were going through my head were not good. I was scared that I wouldn't be able to bond with my baby, but for now I couldn't do anything but try and see if I could  salvage what was left of our marriage. I had to make a decision; after all I was responsible for my baby boys’ happiness, my unborn child’s health, and also my own well being. I was going to have the baby no matter what..

 When I was four and a half months pregnant my husband woke up one morning and told me that he just wanted to be a part time dad. I knew things were bad but I never knew that he felt that way, and of course I only wanted what was best for all concerned, so I agreed to the split and he began to pack his bags. It was devastating to think that he didn't want to be a family any more, but I wasn't going to beg him to stay. I needed to be sensible, so I told myself that things were going to be alright-- after all my kids needed me. Life had to go on. Life as a single parent was difficult and daunting but I was so lucky that I had the support from my parents, sisters and a few good friends.


The pregnancy had its ups and downs having recurring chest and kidney infections --I seemed to be on antibiotics forever. Then late into the pregnancy I was diagnosed with asthma and was given inhalers. At 7 and a half months into the pregnancy, on one of my routine clinics, bloods were drawn.  The results:I had protein in my urine, my blood pressure was high and I had the most horrible headaches. These were some signs of pre- eclampsia and my consultant wanted me to be admitted ASAP. After my appointment I went home to settle Lee with one of his aunts and collect my bag. I had no choice but to let my family share the responsibility of my little man for a while as I didn't know what lay ahead with the little one who was kicking inside me and causing so much disruption. As the next few days passed I was becoming sicker. My head was pounding and some tests were showing that my kidneys were not functioning properly so I was sent to another hospital that had more up to date scanning equipment. There detailed scans of my kidneys were carried out and it showed that my baby was lying on them but they could not tell if there was going to be any permanent damage. They also revealed that I was going to have a wee boy, but for now I had to keep the surprise of gender to myself.  As long as he was healthy that was the main thing, or so I thought.

On the morning rounds a Dr whom I had never met before told me that I would be staying until my due date, which was 6 weeks away. Oh! Boy I was not a happy bunny. No way, I couldn't believe what I was hearing. I called my Mum with tears running down my face and talking through the sobs. She tried so hard to calm me down but nope I was frustrated and angry, after all I could not eat as there just wasn't room anymore. I was crunching ice cubes and I was in a lot of pain. I couldn't even put a sheet over my legs as I was leaking fluid from my pores when pressure was applied. While on the phone my consultant popped his head round the corner and waved me over. He then asked me did I want to have my baby in my arms tomorrow.  Yes please I said, I was nervous but excited.


On the 26th October 1993 my labour was induced. My water broke . I was advised to have an epidural as my blood pressure was high and they would be able to have more control over it. My labour lasted 6hrs 13 minutes and at last my second miracle entered the world. My Mum was with me every step of the way and we both got such a shock when we looked at my baby boy. He was blue, filled with fluid and was struggling to breathe. In fact he looked just like his last scan photo that was taken only a week before. After a quick cuddle he was whisked away so that the staff could clear his airways. I didn't hear him cry so I knew that things weren't good and I began to worry. A few minutes later they asked me if he had a name yet before taking him to SCBU. I named him Connor John Robinson. He weighed 7lbs 3ozs which was remarkable for being six weeks early.  My Mum went with him to SCBU and on her return I must have scared the living daylights out of her as I had my very first asthma attack as she walked through the door. Staffs were good and managed to control my attack, but I had lost about four units of blood so I needed a blood transfusion and had to stay in intensive care. I couldn't believe that he had arrived and I was one happy and proud Mummy. During the night a Pediatric Dr came to tell me that Connor had been moved to Intensive care and was in an incubator and that he was being given fluids and antibiotics through an IV drip.  

Diagnosis
At 17mths Connor got a clinical diagnosis of NS. I wasn’t sure how I felt as I had watched my wee man go through some horrific tests, but now at least he had a name to his difficulties, maybe I could plan for our future. After all I had been blessed with two beautiful miracles and I knew that my life had been changed for ever. Raising my two special boys was difficult but I was up for the challenge. My emotions were flying high but every little achievement that my boys made let me see how blessed I was and that the hard work and sacrifice was well worth it. Our schedule was hectic as Connor seemed to have lots of different hospital and therapist appointments, not to mention visits from health visitors or nurses. I had never felt so isolated and I had thought the worst about whether my baby boy would be with us for long, I couldn’t stop worrying about what the future held for my boy but for now all I could do was take one day at a time and thank God for every precious moment that I spent with Connor.  Life with my boys was busy but I took things in my stride. There were times that I wanted to have a little bit of my life back and just as I thought it would happen Connor would be referred to another clinic. More appointments and time spent worrying about upcoming surgeries, not something that I was comfortable with but could not escape. For now I had to pray and hope that the surgery went well. His bowel was the first op, my poor baby was always constipated and it broke my heart to watch him squirming and screaming in pain when he needed to go. He was on Senna and Lactulose but they didn’t seem to work and Connor would only have a bm every three weeks so he was having an anal stretch done. 

Connors Journey
Mum and Dad invited the three of us to stay with them until I got on my feet and because it was just a few weeks until Christmas, they didn’t want me to be on my own.  My baby boy was losing weight fast and was projectile vomiting after every feed. He slept through feeds and I was getting really worried. Then one day as I looked at his legs, from the knee down to his toes were a very deep purple, by luck we had an appointment with his Paediatric consultant later that day. I was told that he would need a cauterization done to see what was going on with his heart, news that I wasn’t prepared for.  It was scheduled for the following week at Yorkhill hospital. The day of his surgery came and all sorts of thoughts were going through my head. As I carried him into the Operating Theatre and kissed him before putting his life in the surgeon’s hands, my emotions were all over the place and I thought the worst. Little did I know that our journey had only begun?

  I waited anxiously for my wee miracle to return to the ward. The relief that I felt when I saw him being wheeled towards me down the corridor. Again he looked so helpless attached to wires and monitors. My baby had pulmonary valve stenosis an ASD and a VSD. He would need heart surgery before the age of 4. For now he would be monitored closely by the cardiologist, but his valve had been stretched to allow his heart to function better.

Connor’s first year was a difficult one. He didn't meet any of his milestones on time and his immune system was so low that he seemed always to have an infection or virus. He was still being tube fed and wouldn't tolerate solid food at all. He would gag at the thought of solids. Vomiting was a huge problem and he was still losing weight. He was diagnosed as failure to thrive but still no one knew why. 

Going Home
I learned to replace his NG tube which I grew to hate and stayed in the family room to take over his care full time. While I was there an eye specialist came to see Connor. He did explain what he would be doing to his eyes and gave me the option to leave the room or stay, I chose to stay. He proceeded to place a clamp on my baby’s eye, it wasn’t nice to watch or hear as my wee lamb was screaming. Dr G was checking for bleeds as Connor had blown blood vessels in both eyes and they didn’t look like they were getting any better. The tests were over and he was given the all clear to go home. I was excited at the prospect of being home and being Mum to both miracles, but for now I had to stay another night and after all he had been there for seven weeks and they wanted to make sure I could cope.


Mystery 

The following day I couldn’t wait to see my baby boy but it wasn’t good news as I stood there watching the monitors, beeping of the alarms, and the needles and tubes that were in my wee man’s hand? The good news was that he was breathing his own air. I dreaded going to SCBU as there seemed to be something else that was going wrong with my precious baby boy. The consultant that was dealing with Connor had come to tell me that he had a heart murmur but that hopefully it was just because he was premature and that it would resolve its self. As the days passed he was not getting any better. He had a large liver, kidney and spleen. He was being fed by a NG tube, and he would no sooner be fed when he would vomit. Dr’s weren’t sure what was going on with him and many tests were carried out. He was low on potassium and needed a supplement. He wasn’t making any improvement and on many occasions he gave everyone a fright. As I walked into SCBU the nurse that had been looking after Connor came to give me the usual update. Looking at his chart there seemed to be a lot written. The nurse had told me that through the night Connor had stopped breathing many times and that he was having sleep apnoea. They couldn’t say for sure what was going on but had noticed that his liver was measuring 6cms when it shouldn’t have measured anything at all and that they wanted to do a liver biopsy. I was devastated and all sorts of thoughts were going through my mind, was he going to die? He certainly had all the Dr’s baffled and for three weeks after all negative tests results had returned. I said to his consultant that I wanted my baby boy to be moved to Children’s Yorkhill Hospital in Glasgow.

 It was arranged for us both to go to Yorkhill the next day. We were there for a week during which scans and x rays were carried out on his liver, heart and kidney. His heart had two holes in it and his pulmonary valve was thick. His liver and left kidney was still enlarged.  He had a visit from one of the leading geneticists Dr Stevenson. He and his colleagues examined Connor closely but he didn’t talk to me and I could feel myself becoming really anxious, I wanted so much to know what was going on with my baby. The next day as I was preparing to get ready for the move back to our local maternity hospital, a young Dr came to have a chat with me. She started to tell me about what they had found, my stomach was doing somersaults and then I heard Noonan Syndrome and that it was the next one to Down syndrome. I had never heard of it. She began by telling me that he has all the clinical signs which consist of low set ears, rotated ear lobes, eyes too far apart, wide spaced nipples, a concave breastbone, heart problem, eating problem, large liver and kidney. As she spoke I wanted so much to cry and I had so many questions but couldn’t find the words to say. I couldn’t take it in and oh! How I needed my Mum right now.

Being with Connor had been good after all he only had me to support him. His dad didn’t want to know and to be honest he wouldn’t have been any support to me. My emotions were all over the place and I was missing my 1st born miracle Lee. I couldn’t even be with him on his 2nd birthday and it was tearing me apart. There wasn’t any clear date of when I was getting my baby home for good so for now I would just have to be patient. I have learned to be more patient over time.


 We arrived back at our local hospital and Connor was settled back into SCBU. I had been discharged so I went to stay with my parents as they were looking after Lee. Oh! I had missed him so much and I needed to bridge the bond that I felt had been split. I visited Connor every day to take over his care for the next few weeks. He was still being fed by a NG tube and one day when I walked in I was asked did I want to take my baby boy home but in order to do so I had to learn to replace his tube. I was told to take him home, love him as they were not expecting him to see his first birthday. 


Connor has had 16 surgeries over the years. He had four eye ops because he had squints in both eyes and his surgeon didn’t know which muscles were responsible for controlling the movement of his eyes. He has had four Botox injections into the left eye as surgery was no longer an option. He has astigmatism so wears glasses. At age 4 he had a hernia and testis- repair done, he went on to have his tonsils out and a few sinus flushes because of the recurring throat and sinuses infections. At age 14 he had to have 7 teeth removed because of overcrowding and because he didn’t want to wear braces. His last surgery was when he was 18, though he hadn’t been well for a long time. He was constantly complaining that his tummy was hurting and he would vomit continually. Many times the GP would fob him off by telling him that he had a bug. He couldn’t stand it any longer and he got very sick, so bad that he had to be taken to hospital by ambulance. He was in hospital put on an IV as he couldn’t hold even a sip of water down. Scans were carried out and they showed that he had an inflamed gallbladder and some stones, but they could not operate until the inflammation and infection had gone. He stayed in hospital for a week and was sent home with strong a pain reliever until his surgery was scheduled. On the 23rd December 2011 we got a call to say that there was a cancellation for surgery and asked was he able to go in?    

There was a complication with his gallbladder surgery though we were never told exactly what it was but it was worrying to think that something was not right. Connor was allowed home on Christmas Eve but I wasn’t sure that he was ready. He still hadn’t passed any urine and was in an awful lot of pain. Early on Christmas morning he had to be readmitted to have a catheter inserted. His bladder had went into shock and it would take some time to get back to normal so until then he would have to stay in hospital. He wasn’t happy as he didn’t get to have Christmas dinner. It was never easy to watch my baby in so much pain for whatever the reason and Connor has had his fair share of struggles but he has always got there with help and support from all that is involved in his life. He has always got there in his own time.

Connor has checked out ptosis surgery but has decided no more surgery as it involves transplanting bone from his thigh to transplant onto his cheek bones as his are flat. He’s had enough and unless it’s life threatening then wants to be left alone. He still struggles every day but he is my brave soldier, I admire how strong and courageous he has been and I wouldn’t change him for the world.

Connor suffers with so many conditions that affect him on a daily basis. He has contractures of both elbows that seem to be getting worse over time. He has scoliosis in his spine and his muscles in his legs have become so tight that he is in constant pain. He is very sensitive to so many meds that he now has to use a pain patch.  



When I was first told that he wouldn’t see his 1st Birthday I told myself that I would love him, fight and support him every minute that God blessed me with him. That was 19 years ago. Connor struggled through mainstream school despite the support that I had fought so hard for already being in place. His High school didn’t honour his statement or IEP. Connor was bullied at school so he developed low self-esteem. He has spent years on and off seeing a clinical psychologist and doing CBT. He did have a lot of time off school because of all the hospital, Dr and therapist appointments. When he was 9yrs old things got so bad at his first Primary school that I had no other option but to move him to another primary to continue his education in mainstream school. He came on leaps and bounds, the classes were smaller and the classroom was a closed class which was a lot better, fewer distractions. I wasn’t sure how he would cope at High School but I was determined to make sure that he would get all the help and support that he needed. Despite Connor having dyslexia and Mearse Irlin syndrome, it didn’t stop him though; he passed all of his exams and went on to do Business at college. He has just completed his HNC in Business this year and will graduate at the end of this year.


         Just when we thought Connor was finished being diagnosed he was given a diagnosis of ADHD, Absence Seizures and his gene mutation was identified as NS SOS1 gene. Connor’s health hadn’t been good so we had an appointment again with Dr T the geneticist and he was given another diagnosis of Cardio facio cutaneous (CFC) syndrome. It was like fitting all the pieces of the puzzle together and we began to understand why Connor had all the unusual things wrong with him, but it didn’t matter for me as we had always lived one day at a time. He has taught me so much through the years and I am so proud to be his Mum.

     He has overcome so many obstacles in his life but has always been determined to do and succeed in whatever he puts his mind to. Who would ever have thought that he would defy all odds and achieve so much? I used to worry about his future and what it would hold for him but he is just like any other teenager trying to do what teenagers do and I do believe he has been given life for a reason.

Raising my boys has been a difficult journey and I have got to meet so many lovely people. It has made me appreciate not only our close family but also our extended family.



AUTHOR


Lynda lives in Scotland. Lynda is a full time caregiver to her son, Connor and her older son Lee who was diagnosed with Schizoaffective Disorder at the age of 15. Lynda says she keeps her sanity by studying at a college or adult community centre, performing voluntary work. She likes to spend her free time drawing, painting or writing poetry. She has had two of her poems published.  Lynda remarks, “I have survived I think because I have a very close knit family and they have always supported me through the difficult times. They are my rock.”  Because of this her boys have a close knit relationship with their grandparents.


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The article above was written by a guest blogger. The opinions and ideas written belong solely to the guest blogger. Noonan Syndrome Foundation DOES NOT endorse political candidates and religion or religious preferences.
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