Mr. Squishy

Our life took an unexpected turn on Sunday, January 15 of this year when I discovered a squishy lump on Trey’s neck while applying aloe to his sun burn. Trey had gone fishing out on the ocean for the first time earlier that afternoon, and the Miami sun was brutal even for winter.



When we arrived back home, we quickly saw our pediatrician. It was a whirlwind of testing and uncertainty but thankfully, the road led to Vanderbilt Children’s and a comprehensive approach to treating our son. His diagnosis: Macrocystic Lymphatic Malformation.

Although we do not know for certain, it is most likely Trey was born with the malformation. As he grows, so does the malformation. We look back on older photos and now see Mr. Squishy (as Trey affectionately calls him) hanging out on the left side of his neck where his shirt typically sits off center as a result.

Lymphatic malformations are uncommon sponge-like collections of localized abnormal channels and spaces that contain clear fluid. The lymphatic system normally collects excess fluid from the tissues and transports it through a series of small vessels back into the venous system. With a lymphatic malformation, however, transfer of this fluid through these vessels is slowed resulting in malformations in the development of the lymphatic system. The excess fluid accumulates, resulting in a swelling of the affected area.

Surgical treatment has been the traditional form of therapy of this orphan disease. We considered surgery but discovered a high incidence of recurrence and complications including nerve damage, especially in consideration of the location of Trey’s tumor. This led us to explore other alternatives.

Sclerotherapy is a direct injection of an irritating agent, and there are a varieties of recipes for injecting agents. For macrocystic malformations, sclerotherapy using OK-432 as the injecting agent causes total or near-total shrinkage in 90% of the cases. For example, the use of sclerotherapy using OK-432 in a subgroup of Japanese patients with lymphatic malformations who have been followed for up to 10 years has been effective. The initial experience with OK-432 in the United States also has been promising.

Our surgeon, Dr. Steve Goudy, is directing efforts at Vanderbilt for a phase III clinical trial of OK-432 (Picibanil). Dr. Goudy has performed this same procedure using OK-432 on 25 other pediatric patients who have shown a 90% success rate. This treatment has limited effect on microcystic lesions which Trey has some microcysts, but we will focus on the big pockets first. If/when the smaller ones grow, we will know the treatment path.

We prefer sclerotherapy because of the higher success rates and less complications while also being a minimally invasive procedure. Our surgical team will use an ultrasound and needle injections to perform the procedure. Trey will be put to sleep for less than an hour.

We leave for Vanderbilt Children's tomorrow morning at 7:30 AM for the first of possibly two or more minimally invasive surgeries (We have the OR scheduled for July 5 should he need a second procedure).

Trey is scheduled to be in the OR from 12:30- 1:30 PM (CST). We anticipate spending one night at the hospital for observation then back to Chattanooga Friday night for recovery at home under the care of our pediatrician. The first week post-op will be when the medicine injected into the cysts during surgery does its magic. Mr. Squishy will double in size then shrivel up during the first week post op.

Trey has found comfort in knowing he is helping pave the way for other children with lymphatic malformations to have better access to OK-432. He is also very brave. On the way home from school yesterday he said: "Mom, I am scared and ready to get this over with." I told him he is the definition of courage.

We appreciate your prayers.