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Wesley’s Story

Site created on March 11, 2020

On January 27, 2020 mom and Wesley saw an orthopeadist for the curving and stiffening of his fingers. What began as an appointment after which we thought he would simply begin seeing PT and OT to help with his finger mobility has lead to a diagnosis that has turned our lives upside down. A few weeks later, after appointments with the geneticists at Children’s National Hospital in DC, blood tests, urine tests, an echocardiogram, and skeletal survey Wesley was officially diagnosed on February 19 with MPS-I, Hurler Disease.

This disease is extremely rare, it has no cure, and it is terminal. It is caused by a mutation that both Alex and Whitney unknowingly passed on to him that reduces or completely stops the production of an enzyme called a-l-iduronidase. Without this enzyme glycosaminoglycans (GAGs) build up in every cell throughout his body. These GAGs affect every single system in a human body - skin, eyes, tongue, internal organs, skeleton, heart, lungs, brain, and more.

Wesley has already seen several specialists and had two surgeries in his short life. Since diagnosis he has had blood drawn twice, a sleep study, and more. Moving forward he will have weekly enzyme replacement therapies to infuse synthetic a-l-iduronidase into his system. This will help improve many of the symptoms, but it cannot penetrate the blood-brain barrier.

In order to hopefully protect his brain from degrading over time, Wesley will need a stem cell transplant - either of cord blood or bone marrow. This will entail weeks or months of living in the hospital along with chemotherapy (just in time for coronavirus to hit the US in full force ::insert serious fear for our son’s life::) to knock out his immune system to prevent rejection of the donor cells. Before transplant Wesley will need to undergo surgeries to place a central line called a Broviac, possibly a G-tube for feeding after chemo, have sedated MRI and CT scans, correct the hernia that recurred after his initial surgery last summer, and correct the hydrocele that developed this January as well.

Wesley will need to be seen by between 10 and 20 individual specialist doctors to give him the best care possible. Moving forward after BMT he will likely need multiple surgeries on his hands, spine, eyes, and others as the enzyme cannot reach his cornea or his skeleton/cartilage.

All of these medical bills, plus parking at Childrens ($7/visit), travel (at minimum 1,25 hours to DC), hospital food, and more are extraordinarily daunting. Many of our friends and family have felt compelled to donate to help cover some of those needs. While our goal is high, we do anticipate that the level of need is real and imminent.

We will be updating GoFundMe and our CaringBridge webpages regularly and other donation opportunities may also come up (Meal Train/Amazon wish lists/Food order service giftcards/etc).

We cannot adequately thank our friends and family that have showered us with an absolute outpouring of love and support thus far. We are grateful to each and every one of you. We wish that healthcare was such that we did not have to ask for this help, but this is where this journey has brought us and we are humbled that so many have expressed interest in helping.

THANK YOU from all of us in The Huntington House.

As we navigate the treatment options available to us, we will attempt to update this page regularly.

Newest Update

Journal entry by Whitney Huntington — Oct 9, 2020

I can’t believe it’s been 161 days since Wesley received his second chance at life.

The road has not been easy, but we are making strides toward what will be our new normal.

From September 17-September 30 Wesley was inpatient at Children’s Hospital of Philadelphia (CHOP) for a second pericardial effusion. During this time Wes was on systemic steroids and several (four) medications to bring his blood pressure down.

Since leaving the hospital Wesley has struggled with energy and stamina partly because of all the weight he put on while on steroids. After two echocardiograms indicating that the small effusion leftover from the drain was. Not growing and three showing that it was fully resolved, Wesley was taken off steroids this Tuesday. He had his central line removed on Monday and is now able to take baths without being wrapped in plastic wrap!

Our next steps are continued weekly appointments at CHOP, our 6-month follow up appointments with Minnesota that will include another sleep study to determine is he still has sleep apnea, and other tests and scans and meetings with specialists.

This Tuesday Wes sees nephrology (who will hopefully be able to reduce the BP meds), neurosurgery to discuss the results of his spinal tap and CT/MRI, and pulmonology to determine if we can complete the sleep study at CHOP instead of doing it in Minnesota.

I know I haven’t been good about updating this site, and I will try my best to be better. We have been overwhelmed with Asher’s return to school, my return to work, and Wesley’s ongoing care needs.

Thank you to everyone who has reached out or sent prayers or positive thoughts and vibes. We appreciate all of your support.

Read More of the journal titled "October 09, 2020 +161"

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