Ben's Story (SMA)
On September 30, 2010 my husband, Mike, and I were blessed with the birth of our fourth child, a big (8 lb, 14 oz and 22" long) baby with red hair, our Benjamin Michael. What an amazing moment! We had waited to find out the gender as we had with our three older girls, loving that wonderful surprise at birth, and we sure were surprised to see a big beautiful boy!
Our early days with Baby Ben were busy of course with four little ones. At the time the girls were 6, almost 4 and 21 months. Ben joined along in rides to school, was an audience member at dance and school programs, and observed all the antics of his sisters. A little before 2 months, we noticed that Ben had low muscle tone. He was not lifting his head when lying on his tummy and in fact had done better with this in the first few weeks of his life. I consulted my friend and co-worker who is a physical therapist and she provided some activities which we incorporated many times per day. At 3 months, I realized that Ben's tone issues were not improving and seemed more concerning. I called to make two neurology appointments, to rule out any "scary stuff". I called our local early intervention team to schedule an evaluation for him…. I felt so strange making those phone calls, desperate for answers. I thought that he might have extremely low tone (hypotonia) or cerebral palsy. I tried to prepare Mike that I thought there would be lots of therapies ahead, that he may not walk until at least 18 months. I talked with co-workers, saying that I just hoped it was nothing regressive. The wait for the neurology appointments to come ticked on, and meanwhile Ben had a cold which never seemed to go away. He had noisy breathing. He had more leakage from the corners of his mouth when feeding. He also seemed to use his tummy a lot for breathing. All of these things we attributed to the low muscle tone. Our worry built as he approached 4 months.
A few days after he turned 4 months old, on Wednesday February 2nd, 2011, the day began as normal. In the afternoon Ben suddenly refused to take his bottle and was breathing with more effort then usual. We had been suspecting reflux and I heard gulps like he was refluxing. I thought I would call the doctor in the morning and talk about medicine. Mike tried to take him for a drive to calm him to sleep as we assumed the reflux was bothering him and that maybe he was getting the respiratory virus the girls had dealt with the week before. When they got back from the drive Ben had never fallen asleep and in that time he had developed a fever of 102. Mike took him to the emergency room at Children's St. Paul while I stayed home with the girls. His breathing got more out of control and they had to put him on a ventilator…. Mike called me and told me about the ventilator and that the doctors were thinking there was some underlying condition going on. That is when the terror struck. As Mike's dad stayed with the girls, Mike's mom drove me to the hospital in the wee hours of Thursday morning, February 3rd. I saw my baby lying sedated on the bed, the ventilator breathing for him, so helpless. In desperation and fear, I asked the doctor, "Is there any way this is just low muscle tone?" She slowly said that she worried about a disease called spinal muscular atrophy….
She asked me if I had ever heard of it…. I had never heard of it in my whole life, not in college studying to be an occupational therapist, not in years of working with kids with special needs…until that morning before at work. The other occupational therapist in birth-3 had mentioned a new child she was going to be working with who had SMA. I now know that there is a reason I heard the word "SMA" that morning. As I heard it again with my baby lying on that hospital bed, I knew this was a devastating diagnosis. Those hours through the night were the hardest maybe of this whole story. Not knowing for sure, dealing with the new realization that our baby may not live.
At 9 am that Thursday morning the neurologist came and confirmed that she believed him to have SMA, Type I. The geneticist came on Friday and came to the same conclusion. SMA is a neuromuscular disease and is the leading genetic killer of children under 2. It occurs in 1 out of 6,000 births. It typically occurs when both parents are carriers. 1 in 40 people are a carrier of SMA. When both parents are carriers, each baby they have has a 25% chance of having SMA. Generally the same type of SMA runs in a given family. SMA has 4 types; type I, which makes up 70% of SMA diagnoses, previously known as Werdnig-Hoffman disease, begins in infancy and typically infants with type 1 are not able to roll over, sit, or even hold up their heads. Ben had all the signs, and it was confirmed the next week when the blood test results came back. Ben was fighting pneumonia brought on by the parainfluenza (croup) virus. Babies with SMA are very susceptible to pneumonia. Despite our grief, we grasped onto the gift we hoped we were about to be given, more good time with our Baby Ben.
Most of the doctors in the pediatric ICU did think he had a good chance of coming off the ventilator, beating the pneumonia, and going home for some more good days. The nurses in the PICU were my confidantes. They listened for hours as we kept watch over sweet Ben. The chaplain found me almost every day it seemed, talked with me, prayed with me. I asked her what it would be like when Ben went to Heaven. She described it as being a holy time, special, sacred. I began to think of it as being like a birth, something bigger than myself, something I could never do alone but God would be with me. Those days by Ben's bedside were special to me because they were time I had with him, to hold his little hand, to sing to him, to pray and pray and pray for him. The child life specialist was such a comfort to me. She helped give us the words for how to explain the disease to the girls, and for how to later explain the shortness of the life Ben would have. A friend connected with the national support group, Families of SMA, and found a mom who herself had a daughter with SMA Type 1 who was born and went to Heaven in 2006. She visited me at the hospital and I was so grateful for being able to talk to someone who really knew what I was going through and who had somehow managed to go on. My sister and friend planned a benefit which relieved financial worries. So many people sprung into action to help us, with prayers, food, help with the girls, funds. We felt so blessed by everyone's generosity.
Ben did come home, after several weeks of getting better from the pneumonia and having surgery to place a feeding tube in his stomach. Ben had lost some skills in the 3 weeks he had been dealing with the pneumonia and hospitalized, and with the progression of the disease. He did not move his legs anymore, only his feet. He moved his arms from the elbows but could not use shoulder movement. Unfortunately SMA was one of those regressive diseases I had worried about. Ben came home with medical equipment (feeding pump, pulse oximeter to monitor his oxygen saturation and heart rate, nebulizer for respiratory treatments, vibrating percussor wand and cough assist machine to get mucus out of the lungs, suction machine as he had lost his ability to swallow saliva, and bipap mask which he tolerated during sleep to help with breathing). We had home nursing during the day and they, like the nurses in the PICU, were such blessings to us and to Ben, who loved them and shared many of his precious smiles with them. We had visits from the palliative care team from Children's, including a nurse, social worker, child life specialist, chaplain, and music therapist. This team ensured comfort for Ben and for our whole family. In May the palliative care team arranged for me, through the parent to parent program of Children's, to meet another mom who had a baby with SMA Type 1 in 2003. She visited us, bringing food and gifts, but most importantly understanding.
We were so overjoyed to have the gift of more time with Ben, and he had so many good times! He smiled all the time and would do a silent laugh/chuckle when tickled, during Peekaboo, or when his sister Ellie would throw his treasured balloons up in the air! He only cried when he was tired. He loved music therapy, especially the guitar. Ben loved to mouth light-weight toys that we placed in his hands, bat at Weebles toys, and knock down block towers with his little fist. He loved books and would pay attention to them for so long. He loved when Mike would take hold of his arm and pretend that Ben was punching him. He loved to play with my hair. He loved to watch the trees blow outside the living room window as he lay on his comfy pillow playing. We took many pictures and videos which are such treasures to us.
We decided that we would keep Ben at home and out of the hospital. We wanted his last days to be peaceful and at home. We chose not to do a tracheostomy tube with ventilator because we knew that given the progression of the disease for Ben, SMA would quickly take all of Ben's movement away and we did not want him to have to stay here, stuck in a body that could not move though his mind would be unaffected. As a parent, you will do anything for your child even if it is very, very hard for you. We were willing to be without Ben physically here so that he could be free.
Ben had several respiratory infections in the spring but made it through with just increasing his respiratory treatments and antibiotics. Due to some horribly scary episodes of him turning blue and unresponsive when semi-upright in the swing or being held, he had to use a sidelying position mostly and we carried him in a Moses basket to keep his position very stable. June 6th, 2011, Ben developed a fever and very high heart rate, with his little tummy working so very hard to breathe. We thought he might be going to Heaven and we lay next to him snuggling, praying, crying softly. That night we started giving him medicines for pain and they worked to make him comfortable. Ben did not pass that night, and slowly improved somewhat throughout that next week. After that night, Ben's work of breathing was more than before and every few days his heart rate would get high again and he would need a dose of medicine to make it go down. He returned to playing and smiling away, our sweet happy boy.
On June 22nd, Ben's heart rate became very high again and his work of breathing was so much. That night his pulse oximeter alarm went off and from then on he needed oxygen and constant medicines for pain with increases in doses. It seemed that Ben's body just could not go on working so hard anymore. In his last days on earth, Ben slept a lot and when awake he would mostly look upward, above our faces. We feel he was seeing angels and beginning to cross over to the next world. On June 28th at 8:45 pm with the sunset, our beautiful Benjamin went to Heaven. I was once again able to hold him in my arms as we told him we loved him, it was okay, and to go to Heaven. We felt such relief for him, to not have to work so hard to breathe and to be able to move effortlessly. It was so peaceful and we had such a distinct sense that he was no longer physically in his body. His long, fragile body which could not sustain him, which we so loved, was now just the shell from which he had moved on.
Since our Benjamin went to Heaven, we have found comfort in reflecting on his beautiful life. A lot of life can be lived in a short time. We may never on this earth know the power of this one precious life which God saw fit to bless us with. We find comfort in feeling him with us though not physically, in knowing that he sends us strength. After Ben was diagnosed, we read Walter Dudley Cavert's "Dragonfly Story", about the transformation to eternal life, which can be found online. We also read "Heaven is for Real" by Todd Burpo, and it was very comforting to us. We found healing in making photo albums with his pictures. We created a garden in his memory using blue flowers like his eyes and the sky, white like the clouds, red like his hair and with a red maple tree. We felt good decorating his spot at the cemetery with a red stone with a picture of his precious face on it, butterfly wind chimes, a little boy angel statue, and angel light. When visiting the cemetery, the girls blow bubbles up to Heaven, and run to collect petals which have fallen to the ground. On his birthday and on the anniversary of the day he went to Heaven, we had a picnic there and sent up balloons.
We have continued to connect with other families who have lost a child, me with the other "SMA moms" I met when Ben was alive and both Mike and I with other grieving parents recently at the national Families of SMA conference which was held in Bloomington this year. We have found healing in talking with a wonderful grief counselor together. We seek out things that bring us comfort, going out for coffee (me) or golf (Mike) with friends. We go on outings with the girls, knowing that Ben is with us too, and we so often see butterflies or dragonflies right by us, which we think of as signs from Ben of his transformation to eternal life. At Christmas-time, I bought butterfly hair accessories for the girls and had them stuffed in my sock drawer. One day I stepped on something in our bedroom, and somehow a blue butterfly hair clip had made its way out of the package and onto the floor! At work during my home visits, I so often see butterflies. One day at each house I went to I saw them, on the dishes of a little girl as we worked on feeding, on a light fixture at another house, on wall art at another.
I now look forward to that blessed day when I am born into eternal life, greeted by my precious Benjamin's face. Until then he will send me strength and I will make him proud as I continue the rest of my journey here.
He gives power to those who are tired and worn out; He offers strength to the weak. Even youths will become exhausted, and young men will give up. But those who wait on the Lord will find new strength. They will fly high on wings like eagles. They will run and not grow weary. They will walk and not faint.
For more information, please visit Ben's caring bridge site: caringbridge.org/visit/benjaminnewfield
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-The support, information and encouragement provided by the PPFL parents is not meant to take the place of medical advice by a medical professional. Any specific questions about care should be directed to a health care professional familiar with the situation.