Prune Belly Syndrome

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    Lorinda Mamo

    The Worries of a Mom of a Child With Prune Belly Syndrome

    At my 20th week of pregnancy, the nurse conducting the ultrasound noticed an anomaly in the fetus. My gynecologist recommended in-depth 4D scans to try to assess if there were any other indications of more serious problems. The syndrome was not diagnosed in utero. Doctors didn’t want to take any chances, and a C-section was planned. I cannot begin to tell you how profoundly overwhelmed I felt at the extent of the medical staff that needed to be present at the time of my son’s birth — and how incredibly fortunate we were that the team was in place. My son Henry was diagnosed with prune belly syndrome (also known as Eagle-Barrett syndrome) the minute he was born, and soon after, chronic kidney disease. I become an expert in something I had no idea even existed. I remember lying there, post-C-section, aching to hold my son, with my husband at my bedside explaining that our son had prune belly syndrome. I lay there trying to comprehend it all, still doozy from the anesthetic and the tears rolled down my face as he proceeded to tell me that our son was in the neonatal pediatric intensive care unit (NPICU). I felt paralyzed with fear of the unknown, and little could I believe just how serious it was, because, let’s face it: no matter how many facts you have, you never think it could possibly be happening to you. Prune belly syndrome (PBS) affects approximately one in 30,000 to 40,000 births. It is characterized by a lack of, or no, abdominal muscle, undescended testicles and urinary tract problems — each child presents the syndrome in varying degrees and complications. The name of the syndrome arose because of the mass of wrinkled skin that is often (but not always) present on the abdomen. Henry spent the first six weeks of his life in the NPICU undergoing tests and scans, while the doctors worked tirelessly to find the right combination of treatments to help his body cope with the lack of function of his kidneys. Because of the deficiency of abdominal muscle, organs in the abdominal area are lax or undeveloped — his ureter was also long and distended, one kidney was not functioning and the other had little function. I visited him in the hospital three times a day, and during some of those visits doctors told us they were unsure of his survival. We were even referred to a bereavement therapist to prepare us for that which I can’t even write. At 7 months old, Henry was admitted to the hospital again. What started as a sore throat ended up as several other infections that were dangerous for him. I try not to worry about every sneeze, but it’s difficult not to be concerned and overly cautious of keeping him out of situations where he can potentially get sick. He has since had several surgeries, some with complications that were life-threatening. He has had one kidney removed and his ureter tied and placed to the outside of his tummy, making it difficult to keep him dry. Diapers that absorb fluid from the abdominal area do not exist, and urine bags are not used on small children. Currently, Henry is stable with medications, treatment and regular visits to the hospital for blood tests. The biggest, most immediate problem that needs attention is his kidney disease. Medications and diet play a huge role in his well-being, but as he grows and develops, his body will no longer be able to cope with the little function that his one kidney has. His diet is a struggle — kidney disease patients may have a poor appetite, but it is just as important for them to eat a proper diet — and with a picky toddler, that’s a feat. Since his disease is silent, or invisible, his health is monitored mostly through blood tests. And when values started to increase last November, his doctor informed us that we needed to start taking action towards his eventual transplant. We will be visiting Great Ormond Street Hospital for the planning and preparation. We have appointments with specialized teams of surgeons, doctors, nurses and therapists who will be preparing us for his eventual transplant. Prior to his transplant, he must also undergo surgery for a bladder reconstruction, rectifying the urinary system before getting a new kidney. During this upcoming trip to London, my husband will also be undergoing tests as a potential donor. Until I was diagnosed with breast cancer I wanted nothing more than to be a potential kidney donor myself for my son, but cancer made that choice for me, and donating is not an option anymore. We are now starting the next part of this journey. Very difficult situations are coming our way, but we have been through a lot and he has thrived and fought with his fierce will. When you meet Henry, his infectious smile and bubbly character will give you no indication of him being any different from any other little boy — but he is one little and mighty kid suffering from a rare disease no one had any control over. Although I am terrified and sometimes wonder if being prepared is a good thing, I will take things one day at a time. My advice to other parents of children with rare diseases is to listen to doctors but also trust your instincts. If you think something is wrong, ask and investigate continuously. Keep an organized track of documents and record as much as you can. Be in the best spirits you can be around your child — they need to feel as safe and protected as possible, especially when they have so much to deal with. If the child is curious and wants to know, educate them, even from a young age. It’s something they need to live with, accept and not fear. Henry Follow this journey on A Bird With a French Fry. The Mighty is asking the following: Tell us one thing your loved ones might not know about your experience with disability, disease or mental illness. What would you say to teach them? If you’d like to participate, please send a blog post to community@themighty.com. Please include a photo for the piece, a photo of yourself and 1-2 sentence bio. Check out our Submit a Story page for more about our submission guidelines.

    Sarah Savickas

    Detroit Lions, You’re More Than Football Players to My Son With a Chronic Illness

    Dear Detroit Lions, I know your bodies are sore and your confidence is running on empty. I am writing this to hopefully give you some inspiration. I am just the mom to a little boy who loves you. I am the mom to a 5-year-old little boy who believes in you. I am the mom to an amazing little boy who can’t play contact sports, like football, because of his medical condition. Sarah’s son holding Detroit Lions tickets Each hit Matthew Stafford takes, my son cringes. “Oh, no, Stafford!” “Look, he’s bleeding, is he OK?” “Mom, I think Calvin hurt his ankle.” He gears himself up each week to watch you play and his little heart breaks with every loss but he never gives up on you. He looks at me with his big hazel eyes. “Next week, right Mom?” He has laid in a hospital bed many times, and if he is in the hospital when you are playing, you better believe he makes sure his game is on that TV. So, no matter how many jerseys people spit on or burn, no matter how many fans leave that stadium early, you are still somebody’s hero. While you are waiting to run out on that field, you might be worried about what the fans are thinking or what the media is going to say. You might think you are just a football player, but you are so much more, and I want to thank you for taking the time out of your busy schedules to make my son smile. Sarah and her children with Detroit Lions player Stephen Tulloch Oakley had taken a liking to #35, Joique Bell, when he started playing for the Lions. He thought he could run fast and jump high (his words). Come to find out, Joique’s sister received a kidney transplant after being diagnosed with renal disease. This hit close to home as Oakley was born with kidney disease and will eventually require a kidney transplant once his are unable to keep up with his body’s growth. Mr. Bell had held a contest on his Facebook page for an autographed jersey. It was a contest where you had to post a photo and whoever received the most likes won the jersey. Team Oakley came together, and Oakley had received the most likes! Next thing you knew, Mr. Bell’s manager, who is also his mother, contacted me and asked me to give her a call. She said she wanted to surprise Oakley and have Joique give it to him personally after the football game we were attending. After the game, we went and met Joique’s mom, Candy. She brought us back into the family reception area where Oakley and his siblings were able to meet him and several other players, such as Golden Tate and Coach Caldwell. They were so nice and even allowed us time to take some photos with them. Not long after, kicker Sam Martin hosted a Christmas party for the Project Scissor Gait Foundation, which is a foundation a good friend of mind started to help children with Prune-Belly syndrome and arthrogryposis. Several families with Prune-Belly syndrome attended, and a few players also came out to meet the children as well. It was held at Dave & Busters in Livonia, Michigan. Sarah’s son (bottom right) and his siblings with Detroit Lions players To the Detroit Lions team members: you are his inspiration, and maybe he is yours. Sarah’s son wearing his Detroit Lions jersey Follow this journey on medicine & smiles. The Mighty is asking its readers the following: Share a powerful moment you or a loved one has had with a public figure. Or, write a letter to a public figure who you feel has helped you or a loved one through his or her work. If you’d like to participate, please send a blog post to community@themighty.com. Please include a photo for the piece, a photo of yourself and 1-2 sentence bio. Check out our Submit a Story page for more about our submission guidelines.

    Sarah Savickas

    When People Respond to My Child’s Diagnosis With ‘Everything Happens for a Reason’

    I’ve been asked many times what condition my son has. When I tell them, they look at me with a confused on their face. Prune belly syndrome is a rare syndrome, and most people have never heard of it. Honestly, I had never heard of it either – until my child was diagnosed. I start on how his bladder doesn’t work right, which leads to more questions and me telling them how his belly button has a hole in it that we catheterize him through. By the looks I’m getting, I start to wonder if I have something on my face. I fumble over words like ureter, hydronephrosis and creatinine. I need to remind myself to speak in layman’s terms because to them, I’m speaking a foreign language. I eventually just settle on the fact that he has kidney disease. Then, they say it. “Everything happens for a reason.” What do you do at that point? What do you say? My son’s condition has not been proven to be genetic. It is not environmental, and it isn’t due to something I did during my pregnancy. It just happened. No explanation available, no reason. I used to lie awake at night pondering that statement. I would think, what is the reason for this happening? Why us? Why this diagnosis? It’s a normal phase to go through when your child is diagnosed with something you’ve never heard of. You want answers, and you want them now. It’s been five years, and I’ve finally come to terms with the fact that there isn’t always a reason for everything. I understand that a lot of the time, people just don’t know what to say or how to act, and sometimes they can say the wrong things. I get it, I’ve been there. We all have. If I can offer any insight to help others avoid this phrase, it would be to say, “So what does this all mean?” But sometimes you don’t need to say anything. Just listen, and if you have the time to listen, I have the time to talk. Follow this journey on Medicine & Smiles. The Mighty is asking the following: What’s one thing people might not know about your experience with disability and/or disease, and what would you say to teach them? If you’d like to participate, please send a blog post to community@themighty.com. Please include a photo for the piece, a photo of yourself and 1-2 sentence bio. Check out our Share Your Story page for more about our submission guidelines.

    Sarah Savickas

    12 Things to Know About a Mom Raising a Child With a Chronic Illness

    While I was pregnant, we knew our son would would have a multitude of health issues. But it wasn’t until he was born that we learned he had prune belly syndrome. Prune belly is a rare and life-threatening syndrome affecting just 1 in 40,000 births. There is no cure at this time, but there are treatments available such as catheterization, medication and kidney transplantation. Here are 12 things I would like you to know about raising a child with a chronic illness: 1. It isn’t easy. It definitely isn’t easy. 2. Everyone tells us how strong I am, but I’m really not that strong. I’m a mom, not a superhero. I don’t wear a cape. I just do what any other mom would do, which is taking care of my child. Trust me, you would do the same thing. You don’t really have a choice; you just dive in and get it done. 3. I’m tired, restless and sometimes lonely. I put my child to bed at night and stay up late. I stay up late scouring the Internet for new studies and information about treatments for my child. I hop onto my online support groups and answer questions of others and ask for help ourselves. I stay up late doing those endless loads of laundry and dishes I couldn’t get to earlier in the day because I was busy. I wasn’t busy doing fun activities with my child, I was busy on the phone with insurance companies, doctors’ offices and medical supply companies. Busy going to and from appointments, procedures and lab draws. 4. Real friends are hard to come by. Coffee at Starbucks? Dinner at 7? Play date at 11? Not so much anymore. I try. I really do try to get out and socialize, but sometimes I just can’t swing it. It sounds so good when I say we will be there. But by the time I juggle homework with my other kids, tackle a load of laundry and administer medicines, it’s already 9 p.m. To those friends who have stuck by my side after I have broken plans (again and again), I thank you! 5. Stop feeling sorry for us. I don’t need anyone to feel sorry for us. Yes, it’s difficult my child has to have surgery. Yes, it’s heartbreaking he’s sick and in the hospital. But please, don’t feel sorry for us. Don’t pity us. When I talk to you, I just want you to act normal and talk about normal things. It’s my escape from the challenges my son and I face. A little bit of normalcy does wonders. 6. When I say I don’t need anything, I’m lying. I don’t want to burden anyone with my own issues. So when I say we don’t need anything, take it with a grain of salt. I’m not going to speak up and say, “I could really use some company” or “I could really use some help.” If I’m in the hospital, please bring coffee or lunch. Force me to get out of the house and don’t let me make any more excuses. Sometimes I need an extra nudge to get up and get out even if it’s just for a walk or to sit outside and chat. Just being there allows me to breathe a sigh of relief, knowing somebody really does care. 7. Ask me before posting to social media. A lot of moms have blogs or Facebook pages. I encourage you to hit the share button or copy and paste the links. But please, and I am begging you, do not log onto Facebook or your other social media accounts and start posting pictures of my child with your own version of what’s going on in our lives. Sometimes I tell you things in confidence and I’m not ready to make this information public. I’ll always appreciate the prayers and well wishes, but please use a generic statement and don’t name names until you check with me first that it’s OK. I might be waiting for an official diagnosis from a doctor or might not be ready to announce the news with everyone yet. 8. I’m smiling, but my heart is aching. Some days it takes all I have to put on a smile. My child’s illness is emotionally, physically, spiritually and financially draining. My child deserves the best, and I don’t want him to know my heart is really aching. So I make sure to smile for him even when I feel like crying because some days are so hard. 9. I just need you to be there. I can go through a ton of emotions in one day. Please don’t take offense if I don’t return your phone call or text right away. I saw it and love that you’re thinking about me. I promise I’ll get back with you once I have a minute. It might not seem like it to you, but just being there is a huge deal. Knowing I have somebody in my corner makes me feel 110 percent more confident. 10. It feels like I’m a nurse without the degree. I change dressings on my child’s raw and bleeding skin. I administer medicines multiple times a day. I wipe away tears and soothe the soul. I cleanup vomit and stand vigilant through the night monitoring a fever. I know how to take blood pressure, insert a catheter and administer a feeding through a tube. I may not be a nurse, but I have learned so much from hands-on experience, I feel like I could pass for one. 11. My child can go 0 to 100 in a matter of hours. I can wake up and go from having a great morning to spending the evening in the emergency room. Oh, and I’m ready for that. I’m always on my toes and ready to expect the unexpected. 12. I wouldn’t give it up for the world. To the outsider looking in, this can all seem overwhelming. Honestly, it can be overwhelming, but the love I have for my child is not measurable, and the smile I see on his face is indescribable. This is the life of a mother with a chronically ill child. This is my normal. I get paid in smiles. Does it get any better than that? Follow this journey on medicine & smiles.

    Mom Outraged After GoFundMe Removes Photos of Her Son Deemed 'Graphic'

    One mom is on a mission to have her son’s story told after a popular crowdfunding site removed photos of her him. Oakley, 4, has Prune-Belly syndrome, which is a rare disorder characterized by partial or complete absence of abdominal  muscles, failure of both testes to descend into the scrotum, and/or urinary tract malformations, according to the National Organization for Rare Disorders. Courtesy of the Team Oakley Facebook page. Because of the high cost of his medical care, his mother, Sarah Savickas, agreed to let her friend, Amber Maynard, start a GoFundMe page for them. The page had been up for nearly a month when Savickas received an email from GoFundMe telling her they had removed two photos of her son from the page for containing “blood, graphic or inappropriate content.” The photos were of Oakley after his appendicovesicostomy surgery, a procedure which greatly improved his quality of life, his mother says. She told The Mighty that she shared the images because it’s important to her to share every step of her son’s journey with the world. “This is my son’s journey. This is the reality of what we live with and what he goes through. Who do you think you are to go and tell people what they can or can’t share in their story?” Savickas wrote in an open letter to GoFundMe that she posted on Oakley’s Facebook page. “This is what my son went through. Are you trying to tell me that his disability is too much for your eyes, GoFundMe?” Outraged, Savickas wrote an email to GoFundMe. who responded by saying the removal of the photos was an accident, apologizing to Savickas and telling her to post whatever photos she wanted on the account. Then, just a few hours later, Savickas received another email from the company about her photos not being “appropriate.” The Mighty spoke to GoFundMe’s Media Director, Kelsea Little, over email, where she explained that the second email was accidentally sent by a customer service agent who was unaware that action had already been taken on Savickas’s account. Little said that Savickas is now able to use whatever images she wants on the account, and that GoFundMe is working to improve their system in the future. “Our sensitive image policy is always evolving based on feedback from the community,” Little told The Mighty in an email. This incident comes on the heels of an issue earlier this month where a mother, Christina Hicks, had her GoFundMe page “hidden” (meaning only accessible to those who had the link) because the site deemed images of her premature child too graphic, NBC reported. Despite GoFundMe’s second apology, Savickas still wants her story to be heard in the hopes that it will prevent other parents in an already stressful situation from having to go through this. “I am a huge advocate for my son and other children who are fighting illnesses, disease, syndromes, cancer, and even un-diagnosed conditions,” Savickas wrote in a post on the Team Oakley Facebook page. “I will not stop, I will not give up. I am fighting for all of us to make sure that our kids voices and journeys are allowed to be told in real life. It is important to show the good, the bad, and the ugly.”