SOPHIE PRUNTY PROFILE -Nutriiveda Original by Jody Prunty (with a little help from Lisa Geng)
“Getting healthy nutrition into Sophie’s body that she was able to digest and utilize and that wouldn’t cause the dangerous and painful GI tract pseudo-obstruction issues was so desperately needed. Nutrition including NV Original has taken my daughter to a whole new level of comfort- and happiness almost all the time for the first time in her 18 years of life. She is more clear headed than we’ve ever seen.” ~Jody Prunty
At this writing Sophie Prunty is 18 years old. My husband Rick and I were not aware when our special child Sophie was born in 1993 the journey that would lie ahead.
Sophie is a funny, happy, beautiful, delightful young lady. Despite her “floppy-baby” diagnosis, she has become a strong-willed, always moving, curious teen. What comes easily to other children, like picking up a small object, is a great challenge for her. She uses a specialized wheelchair and uses a talker to communicate. Her Father and I, along with her health professionals have marveled at her progress despite the challenges she faces. She adores animals, videos, music, playing on her computer, and kids. Due to health issues she is home tutored. She can dazzle you with her charms. Sophie loves life! Since an early age Sophie has been in a wheelchair. Trunk strength was always an issue, and with no fear of falling due to her lack of feeling peripheral pain, she never did learn to walk. We do believe it may be possible however for her to learn still as she has the patterning down and can do it with a specialized walker, but that is done only infrequently. Sophie has a huge stander at home; however she has pretty severe scoliosis and lordosis, and is not a candidate for surgery unfortunately due to her liver issues. She doesn’t do much floor time anymore, preferring to be in her wheelchair; probably due to the scoliosis and the gut surgery she had a few years ago.
Outside of being born with club feet, it was clear something was wrong early on with our precious daughter. She did learn to scoot on her bottom around the age of two. Finally after years of numerous tests, a one month stay in the hospital, and a trip from our home in Illinois to the Mayo Clinic in Rochester, Minnesota to see the experts Sophie was diagnosed with an extremely rare disorder called Hereditary Motor-Sensory Neuropathy Type II (now called HSAN type 2, non-CMT.) Sophie’s prognosis is unknown and there is little written about life expectancy.
The “orphan disease” that Sophie was diagnosed with has been approximated to occur in one in one and a half million cases in North America, though this statistic is not fully valid. Sophie displays global delays including large and fine motor skill delays, as well as delays in speech and language. One of the unique symptoms of this disorder is that Sophie lacks peripheral feelings. What that means is that she is oblivious to pain caused to her body from the outside. If Sophie cuts herself, or pokes her eye, she has no pain. This is both a blessing and a curse. Without peripheral pain she can do damage to herself easily, and pain can also be a great teacher. Prevention of injury and infection are high priorities. This is not to say that Sophie doesn’t feel any pain. Sophie spent much of her 18 years of life in life threatening pain from impairments to her GI tract.
For a sneak peek at our past we have a very old website www.oneinamillionkid.com which documents Sophie up till she is around 6 years old.
From Mom Jody “My Daughter Sophie”
“Sophie is very complicated medically, so there is a lot of information to share- and I don’t know how detailed I should go! But I hope to share enough information to help others who are in the same situation as us!
Sophie had many hospital stays. One special time we’ll always remember was in 1998 when the doctor’s saved my daughter’s life by placing a tube in her cecum (large intestine) and a Gastrointestinal tube. As of this writing April 26, 2012, we are just finally and hesitantly hopeful that her GI tract pseudo-obstruction issues, which have sent her back to the hospital far too many times, are behind us. This is due in part to getting the correct nutrition that her body can utilize which I’ll explain more about.
Since she was three years old, Sophie has received the bulk of her nutrition from a central line that goes directly to her heart. The problem is that over time, this nutrition can attack the organs of the body. Sadly, due to this line which saved my daughter’s life, Sophie is in the first stages of liver failure.
Sophie’s GI tract has never worked well. A number of years ago, we were constantly “flushing” her stomach due to the over production of mucus. At that time, her g-tube in her stomach was always attached to a drainage bag. She could drink electrolyte fluids, but much of it went into the drainage bag; her intestinal tract was not really working.
It’s said that the GI tract is a reflection of your health, and this was true for Sophie. Her blood work showed many vitamin and mineral deficiencies that we struggled to adjust from her IV food. When her spleen enlarged, her platelets and white blood counts went down to critical, and her liver numbers soared we knew that she was coming into liver failure.
My precious sweet daughter had a huge gut surgery in 2007 in which they cut all the way across her mid-section. Poor thing…all that muscle. The surgery had to be done however to save Sophie’s life once again. The surgeons re-routed the blood flow from the spleen to the liver to now be from the spleen to the kidneys.
The surgery was a success in that Sophie’s spleen shrank down, her liver numbers got a little better, however Sophie still wasn’t out of the woods, her platelets and white counts continued to stay in the critical category. She was diagnosed as having mitochondrial issues in 2010.
Just prior to this event we found MucoStop. Enzymedica® (an enzyme that “eats” mucus) and worked to get it into her system. With the decrease in mucus, her stomach was able to empty again and her GI tract began to work better; we were able to tie off her g-tube again. With her gut working better, we were more willing to try a few additional supplements to help her. The doctors prescribed medical-grade Ubiquinol (CoQ10) to help her mitochondrial issues. (Cyto-Q by Solace Nutrition) Sophie also has seizures that have been mostly controlled by medication, and for most of her life, Sophie has had sleep problems as well.
We’ve spent almost two decades now searching for solutions with Sophie’s doctors. Sophie is currently followed closely by a number of doctors including a Pediatric Neurologist, a Pediatric Gastroenterologist, and a specialized Pediatrician. Sophie has received Physical, Occupational, and Speech Therapies weekly pretty much her entire life. Nurses help us most nights and weekly in the morning hours with many of her medical needs. This is incredibly helpful as Sophie typically doesn’t sleep through the night, and her medical needs are extensive. Sophie suffered for too many years of her precious life- we cherished the few days/hours when she was not in pain in her gut area, and the strength she had to get through those times!
We knew it was important for us to find people/professionals to understand that she might not feel pain externally, but internally she was in excruciating pain. I just knew in my heart that if I kept searching I’d find things that would help her, and professionals that “got it”, as well as other families that lived a similar, crazy life.
I also knew because of her complete dependence upon her central line for her nutrition, that Sophie is lacking in vital nutrients that would help her on a number of levels.
But feeding Sophie wasn’t easy, and could even be dangerous for her. Every attempt to give her “regular food” ended in an episode of pseudo-bowel obstruction: a very painful, life-threatening, guaranteed hospital stay experience. It was a horror to watch my child go through this, and due to this it’s understandable that I am very slow to accept or try new foods. I research everything extensively and run by all Sophie’s medical doctors before even attempting anything new.
During this time, we finally were able to connect with some families who have children with HSANs, or some disorder that is similar. All these people display differently and are slightly different in their diagnosis. Through this group we met Lisa Geng and her lovely family, mostly through cyber-space. Lisa connected me with her Cherab FB group and introduced us to the effective use of fish oils (which we were already using) and spoke to me about a food product called Nutriiveda (NV) Original which I thought looked interesting, but really didn’t know if this was something that would help my Sophie.
A short time later Sophie’s night nurse sent a request to Make A Wish. Sophie’s doctor filled out a form for qualifying. You only can get wishes filled until the age of 18, which we didn’t know about. We were so happy to receive the phone call that Sophie was accepted to have a wish. They even provided us with contacts that helped us thru the process. (There were very few forms to fill out thankfully- I’m so sick of forms!) If it’s a Disney wish they have a direct line to getting it done quickly. Ours was done in about 4 months since we needed the cooler, winter months of Florida for Sophie. Give Kids the World is an amazing place that they suggested we stay at since Sophie has fragile health- and it was perfect for us!
Lisa Geng lives in Florida, and it was 6 months after I first learned about Nutriiveda Original and our trip to Florida with Make A Wish for Sophie to Give Kids The World, that I met up with Lisa and her family who brought me a canister of chocolate NV original to try.
January 23, 2012, after starting the NV, our lives changed…
This was the first day we officially started Sophie on Original Chocolate Nutriiveda, by giving Sophie about 1/8 of a scoop. As it was such a low dosage however since she was supposed to be on four scoops for her age, I wasn’t sure if we would note any progress, but at this point I just wanted to make sure Sophie’s body tolerated it. Sophie tolerated the small amount of Nutriiveda Original with no effects, which to me was HUGE. With great caution, and with supervision of Sophie’s medical team, over the month we very, very slowly upped the amount to 1/2, to 1 and then 2 scoops.
During this time we did see Sophie being more “verbal”, and her stomach emptying better. But during this time she was producing a lot of gas (which she already has issues with,) and that did make us and her caregivers a bit nervous, however she continued to have bowel sounds which was a relief. The reason bowel sounds were important and was because in the past, by now, Sophie would be in lots of discomfort and her intestines would be quiet. We noticed she was happier and at first we thought it was from the trip to Disney through Make a Wish, as she did have so much fun. But as time went on she continued to be happy all the time. Prior, Sophie would start showing signs of pseudo-bowel obstruction, we were thrilled that wasn’t happening and thrilled that Sophie seemed so happy! We decided to switch to the Vanilla flavor, as my lurking on the website seemed to show even though both were made up of similar food ingredients, it may be milder for some folks.
We continued to very slowly up the amounts of NV Original from 2 scoops a day to 2 1/2 scoops to 3 scoops which we split into three servings a day. We tried to spread it out because we didn’t want to tax her stomach too much, or do the boluses too close together. Sophie seemed much clearer-headed. It’s difficult to describe all the changes, but her home tutors were noticing that Sophie was no longer trying to fall asleep during her two hours of lessons a day (she is taught around 3-5pm daily.) They also noted that Sophie was communicating better with her talker. Instead of always just hitting one word on the talker over and over she was suddenly willing to give you more information.
Fortunately in March, Chicagoland was experiencing some unseasonably nice weather, so we met up with some other special needs kids and their moms at an Arboretum. I wish I had had a camera handy as my friends- the Moms who have lived this journey with me, watched in shock as they saw how much I was putting into Sophie’s stomach at one time. As we did it gradually it became normal for me. But, stepping back when others pointed it out, I too was astonished at how far she had come.
Sophie has a cecostomy, which is similar to a colostomy, only the hole is at a different point in her colon. She has always had “to drain” for her BMs, though her body can eliminate like “normal” people she never did produce anything that looked like a normal bowel movement.
The cecostomy was placed to help lessen the burden on her lower GI tract and to be a point of deflation if things got backed up/ too full of gas which would put her into the dreaded, life threatening and severely painful pseudo-bowel obstruction. But that was all before she was on NV Original.
Due to almost constant gut pain for almost her entire life, Sophie always had pretty hellish nights. After a few weeks on NV original we started getting some nights that weren’t so bad, at least for part of the night. Even though she was still waking late morning to midday, she was for the first time waking with a smile on her face most “mornings.” Her demeanor was much more tolerant, even in the evenings when she gets sort of nutty.
Also around this time Sophie appeared to be stronger when doing therapy. We are looking at transitioning to a new therapy place, so she is getting double the therapy she would have had before. In the past this would have and should have tired her out, but instead she is improving. Sophie now had her stamina to stand!
One of the many downsides of being dependent on central line food are the shortages that come up in the ingredients/additives of the medicinal foods and supplements she had to survive on most of her life. This isn’t a big deal if one could consume and absorb at least some foods , but it can be life-threatening for people completely dependent like my daughter Sophie. The timing has been almost perfect for us to be able to get some additional, more healthful nutrients into her system while there are shortages in multi-vitamins and minerals and selenium. With fingers crossed, we are hopeful that her gut is absorbing and utilizing the NV to keep her at optimal health.
In the past Sophie who has few verbal words and communicated mainly through her “talker” was what we called a “one word wonder” For example if she wanted to watch a particular video she would select on her talker the word “video” with some pointing. Then with me asking for more information will reluctantly give more and may say “Kidsongs” (of which she has both DVD and VHS and about 20 of them.) More questions, yes/no, asking for color of box…all time consuming and she has to work hard both motorically and mentally to find on her talker.
But after a few weeks on NV original shockingly she said this with her talker: “DVD Disney Ant Bug” We were shocked! We still however didn’t know what she wanted. Sophie is not a fan of It’s a Bug’s Life, so I couldn’t interpret that. I kept repeating the words as I looked at her DVDs, but I truly didn’t know what she was asking for even though I wanted to reward her for her great talking! While I was going thru the numerous Disney DVDs we have I hear Sophie say “Sing Along Songs”…Holy crap! She wanted the Sing Along Songs Flik’s Musical Adventure in Animal Kingdom! She hadn’t asked for this DVD in eons. It wasn’t just her speech I was proud of, I was also so proud of her persistence! Usually, if I can’t get it really quickly, she just gives up and selects something else. These are the times you wish you had a video camera on to document this good stuff! And we do plan on capturing more and more to share on this page!
I also want to update about bruising: Sophie gets shots in her thighs every night. In the past she’s always had lots of bruises due to her low platelets. We’ve noticed in the last 2 weeks (beginning/mid April 2012) that the bruises are fewer. The skin beneath is not as hardened. Hmm…blood work shows platelets at 26/27- still very, very low, but not as low as she has been in the past (in the teens.) Sophie’s labs have almost always lagged behind in her symptoms. I’m actually looking forward to her labs these days (as I’m writing this in May) to see if there are changes.
End of April 2012-
We are now on 4 scoops a day of NV Original (by g-tube), split up between the wee hours of the morning, early morning, midday and mid-afternoon, the late afternoon to night. We leave time for her GI tract to digest and empty, so it’s less taxing on her gut. We keep playing with timing and her hydration needs, as we did experience what looked like constipation- though she hasn’t struggled with it,
Her bowels are making sounds, and the waste is by-passing her cecostomy. She’s having what looks like normal bowel movements for the first time in her life! We’ve gone to flushing the cecostomy 3 times a day with saline water, just to be sure that things are moving along. Her gut is not in any distress- this is so new to us that we caregivers are kind of freaking out! (pinch us!)
Her OT and PT are noticing that she has increased strength and stamina, even more than a few weeks ago. All good things and it keeps improving!
Having read about others having successful, easy nights I was so hoping to see this happening. It is still early in evaluating this, I am hopefully optimistic and thrilled to share that we have had multiple nights in a row with Sophie having what we describe as “good” nights. Very exciting for us all! It makes us happy to see Sophie happy and not in pain, sleeping well, we hope that this will continue. One of the other “good things” that I’ve
observed is that her energy levels are better and she is able to stand with her therapists longer and do more repetitions! After seeing her sit for so much of her life this is incredible to see!
Getting healthy nutrition into Sophie’s body that she was able to digest and utilize and that wouldn’t cause the dangerous and painful GI tract pseudo-obstruction issues was so desperately needed. Nutrition including NV Original has taken my daughter to a whole new level of comfort- and happiness almost all the time for the first time in her 18 years of life. She is more clear headed than we’ve ever seen.
In addition to Sophie enjoying life for the first time, my husband and I, as well as her nurses and doctors and therapists are all enjoying this new part to her life! Her GI doc is simply amazed. I’m hopeful that we will see differences in her bloodwork for even more evidence of what is working for her.
And again the best part?
“She continues to be happy. As a mom, what more could you want for your child? So far, it’s been a wonderful journey!” ~Jody Prunty
Happy 18th Birthday Sophie Update!
5.22.2012 Just a quick update on Sophie: I was amazed on Saturday by her patience. I have helped put together a “theater day” with a local church here to show a movie in a theater-like presentation at the church. They were inspired by my renting a theater for Sophie and a bunch of her friends with special needs on her 18th birthday. They wanted to do the same thing, but for free for kids with special needs. Awesome group, they really make you feel like you are at the theater, and do extra things to make it special. Anyway, 2 months ago we showed the first film, and Sophie couldn’t stay to watch the whole thing- my helpers “walked” her around while the movie finished. She made it thru maybe 3/4 of the film. This past Saturday she made it thru the entire movie!! This is astounding as she doesn’t “sit” for anything really. Well, she’s in a wheelchair, but you know what I mean 😉
July 17, 2012 Update
I haven’t reported in a while, and wanted to be sure I was seeing what I thought I was seeing (Sophie being Sophie, we don’t always know if something is going to “stick” when we see changes…) She is sleeping SO much better these days. Granted she is still heavily drugged, we still have to have someone in the room with her, but her nights are so much easier on all of us!! This is so wonderful, we haven’t seen this for 12 years! She’s gained weight (which means she is absorbing at least some of the Nutriiveda!) so now we are going to back down on the calories of her central line food. We are planning on cutting down her calories in her central line food, NOT the Nutriiveda. Her gut hasn’t worked this well since…well ever. Awesome!