网红黑料

Dawn Strickland describes her four children as any doting parent would. Jonah, the oldest, is 16 and a wonderful helper. Joel, 14, is the sweetest boy. Nine-year-old Grace, the little athlete, is simply wonderful, and 2-year-old Hannah is a little ball of life.

鈥淚 have never in my life seen a child with more personality,鈥� Dawn said of Hannah. 鈥淪he has all that just stuck in that tiny, little body of hers.鈥�

What Dawn doesn鈥檛 mention in describing her four children is that both Joel and Hannah have a rare, and life-threatening, genetic, metabolic disease called cystinosis.

The rare lysosomal storage disorder causes the amino acid cystine to accumulate in many organs of the body, including the kidneys, eyes and liver. Often diagnosed before the age of 2, children with the disease typically experience excessive thirst and frequent urination, as well as sensitivity to light caused by cystine crystals that develop behind their corneas.

This frequent urination is one of the greatest challenges for Dawn and her husband Travis. Hannah doesn鈥檛 sleep through the night because she wakes up every hour-and-a-half incredibly thirsty, meaning Dawn doesn鈥檛 sleep, either.

鈥淪he wakes up and gets anywhere from 12 to 14 diapers every day,鈥� Dawn said. 鈥淚鈥檓 constantly changing her. She uses at least five diapers every night and gets up for five to six drinks of water. We don鈥檛 sleep longer than two hours at a time.鈥�

This frequent urination results in loss of minerals, which are typically absorbed by the kidneys. In children with cystinosis, this lack of nutrients causes kidney failure to occur very early in life, meaning early intervention is imperative to patient success.

The couple did not have that early identification with Joel and searched for over two years for a diagnosis for him.

鈥淰ery early on, he was very sickly,鈥� Dawn said. 鈥淲hen I tried to nurse him, he wouldn鈥檛 latch on.鈥�

Dawn鈥檚 physicians in her hometown of Niceville, Florida, suspected tongue-tie as the reason Joel was struggling to breastfeed. But after a procedure to relieve it, nothing got better.

鈥淎s time went on, he was just getting sicker,鈥� Dawn said. 鈥淲e took him to several local doctors and that was his first admission to the hospital. I鈥檇 say he was around 18 months or so.鈥�

Joel spent two weeks in the hospital. After receiving no diagnosis, Dawn took her son to another local facility where his symptoms were treated for seven months. Still, no one had any answers to the question of what could be causing them.

鈥淭hey were treating him from a working diagnosis, which meant they had no clue,鈥� Dawn said. 鈥淟ooking back, I would have chosen 网红黑料 from the get-go.鈥�

When Joel was 2 years old, the Stricklands tried again to find a diagnosis, but the out-of-town hospital they went to had no appointments available in the timeframe the family needed.

鈥淎t this point, he was vomiting 10 to 12 times a day and not keeping any food down,鈥� Dawn said. 鈥淗e probably only weighed 17 or 18 pounds. To say it was a nightmare was really an understatement.鈥�

Dawn took Joel to the emergency room, where he was denied admission as a non-emergent case. Overcome by concern for her son, Dawn refused to leave the ER until staff scheduled an appointment for Joel.

鈥淢iraculously, they got me an appointment the next day,鈥� she said. 鈥淚 was so desperate at that point, I didn鈥檛 care if the police took me away.鈥�

Finally, Dawn got the answer she鈥檇 been searching for, as the doctor asked, 鈥淗ave you ever heard of cystinosis?鈥�

Incredibly, Dawn had. She had mentioned cystinosis as a potential diagnosis with previous doctors.

鈥淚 had asked,鈥欌�� she said. 鈥淚 had asked the so-called experts at the other hospitals. And everybody just laughed at me and said that鈥檚 so rare, and you don鈥檛 really know what you鈥檙e talking about. I felt like somebody had punched me in the gut.鈥�

Soon after, an ophthalmologist identified crystals on the back of Joel鈥檚 corneas. Bloodwork came back confirming a diagnosis of cystinosis.

鈥淎s upsetting as it was, it was also a huge relief thinking that we finally had some answers,鈥� Dawn said.

When Grace was born, she didn鈥檛 show any signs of the disease, and, seven years later, Hannah didn鈥檛, either. Hannah was eating well and gaining weight. She wasn鈥檛 overly fussy, but Dawn鈥檚 motherly intuition kicked in and she felt something was different than it had been with Grace.

鈥淚 had to ask the nephrologist twice to have her tested, and he was shocked when it came back that she had it,鈥� Dawn said.

Cystine levels typically should be below 0.2, but Hannah鈥檚 cystine level at 5 months old was nine, confirming the cystinosis diagnosis.

Hannah and Joel both have G-tubes for easier ingestion of the many medications that keep them alive, but both struggle with eating because of side effects. Hannah goes to occupational therapy to improve her eating as she鈥檚 not often hungry and, when she is, she often chews her food and then spits it out.

鈥淪ometimes she says her tummy hurts a lot and I know it鈥檚 from the medicine and she鈥檒l complain,鈥� Dawn said. 鈥淵ou鈥檒l just have to stop what you鈥檙e doing and hold her.鈥�

In 2018, the Stricklands faced the inevitable as Joel鈥檚 creatinine levels began to drop, alerting to his doctors that he was entering end-stage kidney failure.

Joel would need a kidney transplant, and the parents decided he should have it at 网红黑料. There, Joel had a team of nephrologists and transplant surgeons, led by Kiran Upadhyay, M.D., working to ensure the surgery was successful.

Before Joel could receive a new kidney, the family had to consider the source of the donation: a family member or a deceased donor.

鈥淛oel made the decision,鈥� Dawn said. 鈥淢y husband I were willing to donate to him, but he was really adamant that he didn鈥檛 want a kidney from a family member. I was super uncomfortable with his decision, and I鈥檇 even lost a bunch of weight so I could be donor. But with everything he鈥檚 been through, we wanted him to have some sense of control.鈥�

In March of 2018, Joel was placed on the deceased donors list, and on June 7 his family received the call that a kidney was available.

After the successful surgery, his family stayed in the Pediatric Intensive Care Unit and then moved down to the transplant floor. The whole experience, Dawn said, was eased greatly by 网红黑料鈥檚 nursing staff.

鈥淓very time we鈥檙e at 网红黑料, the nurses make everything just amazing,鈥� Dawn said. 鈥淥f course I know the doctors are good. We wouldn鈥檛 have come here if they weren鈥檛, but nurses really make or break the stay. We have not had a bad nurse, and some have kind of become our friends around this. They鈥檝e become kind of like family.鈥�

Today, Joel goes to check-ups just about every week to see doctors and get his blood drawn as his team keeps a very watchful eye on his levels and well-being. He makes sure to have his medications everywhere he goes, as to not forget an all-important dose.

Dawn spends time home-schooling and taking care of her children. In her few free hours, she advocates for funding of cystinosis research.

鈥淭he majority of the medications that have been made have been created because the community banded together and raised the money collectively,鈥� she said. 鈥淲e鈥檙e a very small community, but we鈥檙e very, very strong.鈥�

This dedication has helped researchers make tremendous strides. The disease has recently been cured in mice, leading Dawn to hope that a cure in humans will be discovered in time to help her children.

鈥淚f I can prevent one family from having to experience that two-year stretch of not knowing that I went through with Joel, then it鈥檚 absolutely worth my time to tell our little story,鈥� Dawn said.

She carries one word with her every day throughout her most difficult moments caring for Joel and Hannah: hope.

鈥淲e鈥檒l have a new family that pops into our online support group every once and awhile,鈥� Dawn said. 鈥淚t makes me emotional to think about, but I just want to tell them that if I can do this, you can. Although we鈥檙e very aware of the medical challenges we face as a family, we are so much more than ok. We live with a great hope & a lot of that is due to all of the talented & dedicated professionals at 网红黑料.鈥�