Doubly Blessed by PWS (Prader-Willi Syndrome)

November 12, 2017 complex medical, developmental delays, Family Stories, growth hormone deficiency, hip dysplasia, Nutrition, Prader-Willi syndrome 6 Comments

Hi, I’m Kallie. I am married to David, and we have two precious sons through adoption, Jahleel and Camden. Jahleel is five and a half years old and Camden is three and a half. Camden just came home from China this past spring.

Both our boys have diagnoses of Prader-Willi syndrome (PWS), but they present very differently. I requested to submit a story because I wanted to give PWS a voice in the adoption world.

I cannot introduce Camden without mentioning our oldest child, our first child and first adoption, Jahleel. Jahleel was born in Hong Kong and came home to us in 2014. At the time, he was two years old and we had a vague list of medical diagnoses. We knew he was struggling with severe developmental delays, could not be fed by mouth (g-tube only), and that his doctors there suspected a genetic syndrome. We eventually got a comprehensive diagnosis of a paternal 15q11.2-14 deletion, also known as “expanded Prader-Willi syndrome.”

Jahleel is one of only a few known cases of this in the world. He remains severely delayed, g-tube dependent, is non-mobile, non-verbal, and has quite the medical team of half a dozen therapists and about 20 doctors. Jahleel also has a beautiful, charming smile, the best laugh, loves going places, and endures his medical appointments and procedures with a positive attitude beyond his years. It is because of Jahleel and his brave, fighting spirit that we had the courage to move forward with Camden’s adoption.

We first heard about Camden in the spring of 2015. A friend of mine casually mentioned a sweet baby boy in China who had Prader-Willi syndrome (PWS) and needed a family. At the time, we were so overwhelmed with Jahleel’s emerging needs, we didn’t even consider another adoption. But we prayed for him, and I bookmarked his sweet little face. I knew that with a diagnosis of PWS, placement was going to be hard for him.

Over the summer, Jahleel’s “expanded” version of PWS was confirmed. Rather than being news to grieve over, it was a tremendous relief. It was assurance that Jahleel’s persistent medical and developmental struggles weren’t my fault – he is simply missing a very large amount of genetic material, making him unusually delayed in the PWS world.

That fall, we decided to move forward with a second adoption. At some point, I remembered Camden and looked him up again. He was still waiting. This time, it was different. God laid him heavily on my heart. I was constantly in prayer for him, could not sleep because his story weighed on me so much. We knew this special need, we knew why he still waited, and we had a sad, rough idea of what his future held if no family chose him. Sadly, the internet information on Prader-Willi syndrome is mostly out-of-date, and creates a very negative picture of PWS.

One day in December 2015, the words, “What if he’s still waiting because he’s ours?” flew out of my mouth, and that was it. We knew this diagnosis, we knew what specialists and tests were needed, where to go for good medical care, where to find good pediatric therapists. Our boys could even attend back-to-back doctors’ appointments or receive therapy jointly.

It was a little overwhelming to think about, but after considering everything we had learned through Jahleel, it felt right. God had equipped us with many of the tools needed to move forward with adopting Camden. We had some unexpected issues along the way that delayed the process, but I boarded a plane on April 16th, 2017, to bring our second son home.

I met Camden on April 19th in our hotel lobby in Fuzhou, Fujian, China.

Camden has been a joy from the start! He spent most of his life in China with a loving foster family. He is a secure, confident, social little guy, and was obviously well cared-for and thoroughly prepared for his adoption. We have seen very few signs of grief thus far, and attachment and bonding are going smoothly.

Developmental delays are par for the course with Prader-Willi syndrome. Low muscle tone (hypotonia) is one of the first signs of PWS and often leads to gross motor delays. We were pleasantly surprised to see that Camden was walking quite well at the time of his adoption. However, walking is just one piece of gross motor development. We still use a stroller or wheelchair for him sometimes, because with hypotonia, he fatigues very easily.

If you are around Camden long enough, you will see that his gait is abnormal and his balance is concerning. He got heavy-duty arch supports last summer, and those have helped noticeably. He is also getting PT twice per week. It was obvious from our pre-adoption reports that speech was going to be a struggle. Camden now has about 20 English words that he can say, but he does not use his words to communicate, just to label situations. And of course, with hypotonia, articulation is a struggle – usually only David and I can understand what he says.

Additionally, he has a very difficult time “turning on his voice” and getting words flowing. He just started speech therapy, and his therapist strongly agrees with my suspicion that Camden has apraxia of speech (also very common with PWS). Evaluations with our local school district are forthcoming this fall.

Medically, Prader-Willi syndrome is quite complex and affects multiple body systems. So far, Camden’s vision, hearing, and sleep study have checked out just fine (by comparison, Jahleel needs glasses, had ear tubes for years due to fluid buildup that was impeding his hearing, and has severe obstructive sleep apnea that requires BiPAP usage at max settings while sleeping). Camden does have a growth hormone deficiency, as expected, and should be able to start treatment for that soon. He also has scoliosis that is right on the brink of needing to be braced, so we will continue to monitor his spine closely.

Camden recently had an upper endoscopy done that showed clear evidence of esophageal reflux (GERD). We are in the process of treating that with medications now. Finally, we had one big, major orthopedic issue to work through when a routine abdominal x-ray showed that Camden had significant bilateral hip dysplasia. His femoral osteotomies were done on July 12th , and he spent 44 days in a spica cast afterward.

The hallmark symptom of PWS is the hunger. Somewhere between preschool age and adolescence, individuals with PWS slowly lose their ability to feel satiated and are painfully hungry. It’s not surprising that a strict diet and food security are crucial pieces of raising a child with Prader-Willi syndrome. Every child with PWS is a bit different, and because the syndrome is multi-staged, what works in early childhood may not work a few years later.

We chose to lock our cabinets and refrigerator before Camden came home, so that is established as normal for him. For now, what works well for Camden is mindful portions, nutrient-dense food, balanced meals, satiating food choices, and routine and structure around food. He also gets supplements that help maximize his satiety.

In China, Camden weighed 50 pounds at his visa medical appointment. Today, he weighs 37 pounds. It was not our goal for him to come down that rapidly, but once he started getting the diet he needed, he stopped clearing his plate and asking for snacks.

Camden’s activity level also increased with the diet change and weight loss, and getting his orthotics also helped him become more active. It is obvious by his demeanor that he feels so much better!

A diagnosis of Prader-Willi syndrome can be scary, but at the end of the day, it’s just a diagnosis.

The words “Prader-Willi syndrome” on his file didn’t tell us a lot of things…

It didn’t tell us that Camden was so confident and secure, how much he loves books, about his adorable little voice, his precious smile, his delightful personality, or the great hugs he gives.

It didn’t tell us how much Camden would love to help me with housework, or about his special bond with his brother and our dog – he has such a sweet, serving heart.

It didn’t tell us how much he would enjoy his therapies, or how determined he would be to walk again after his hip surgery.

And it didn’t tell us how excited Camden would get about “singing” along with the hymns at church or babbling along to the responsive prayers.

Adopting a second child with PWS was scary beforehand, but now we look at our adorable, smiling little guy and wonder what we could possibly have been afraid of!

For more information on Prader-Willi syndrome, please see the Foundation for Prader-Willi Research, or the Prader-Will Syndrome Association.

– guest post by Kallie

6 responses to “Doubly Blessed by PWS (Prader-Willi Syndrome)”

  1. Judy Christian says:

    God bless you for adopting and especially adopting medically challenged children. The pro-abortion agenda says that imperfect babies should be aborted because they will have miserable lives and no one would want them. You show the lie for what it is. Bless you.

  2. Angela Latchney says:

    You are very brave! I only have a child with PWS and hate every moment of it. I am exhausted of therapies, medical appointments and every limitation that PWS brings to the table. I love my child but hate his PWS symptoms. His hunger has increased over time and he cries over food even after having a decent meal. Anyways, I don’t know how you can manage but I admire your determination, will power and capacity to love. God bless you abundantly.

  3. batya says:

    Hi. I work in adoption and have a newborn child with Prader-Willi that we are looking to place with an adoptive family. I saw your story and was quite moved. Any agencies you can point me to? I really want to find this little guy a loving home.

    • Kallie says:

      I know Special Angels Adoption has placed a few babies with PWS before. I believe they are both an agency and an advocacy organization.
      You can also contact Prader-Willi Syndrome Association (PWSA-USA); They are not an adoption organization, but they may be able to help get the word out.

  4. Maree says:

    Take my hat off to you both. But I always say for what I do for one I should have 5 but asked my son one day and he said no mum I don’t get enough of you now. Best of luck think you need some thanks for shareing

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