Emaleigh’s detailed timeline:
* 18 weeks pregnant the doctor noticed that there was a problem with Emaleigh’s feet, they looked like hands.
* 19 weeks pregnancy we were referred to a neonatologist 1.5 hours away to understand the potential issues and risk of carrying the baby to term
* 27 weeks I went into labor with my twins
* 28 weeks I delivered Michael 2lbs 4 ounces, Emaleigh 2lbs 13 ounces.
*
I first saw Emaleigh more than a day later in the NICU, her feet were very long and it looked like she had a hernia near her sternum, her right leg was a little fatter but we attributed it to prematurity as did he NICU team.
* 20 days old, she started putting on weight and her right leg and right buttock started growing more than double the size of the left. We realized the mass that looked like a hernia was a soft tissue tumor.
* Her right leg, right buttock, both feet, and left flank continued to overgrow as she gained weight.
* After being released from the NICU in 79 days we visited the genetic clinic and received the possible diagnosis of Proteus Syndrome. Emaleigh’s records were shipped off to Dr. Biesecker at NIH for a formal review. (Proteus is what they believe the elephant man had).
* January 2010 - My husband and I researched EVERY possible overgrowth syndrome. We found Dr. Fishman at Children’s Boston and sent Emaleigh’s records to be discussed at their multidisciplinary monthly meeting.
* January 2010 Seen by a gastroenterologist because her upper body is very small and her lower body (soft tissue tumors) is stealing her nutrition. Her bloodwork was normal
* March 15, 2010 – Received a letter from Dr. Biesecker at NIH stating they believed Emaleigh did not have Proteus but may have CLOVE or HHML (Hemihypertrophy)
* April 2010 we received a letter and call from the Boston team letting us know that they believed that they think she has CLOVES but we needed to be evaluated
* April 30 2010, Emaleigh was seen in Boston and officially diagnosed with CLOVES Syndrome. I believe the words used were “classic cloves” and they mentioned that she was the 34th or 35th person diagnosed in the world with CLOVES. Recommendations from Boston:
* surgery to remove her great and little toes from both feet and debulk some of the soft tissue mass on her left foot, to give her a chance to be shoed.
* Wilms’ tumor checks every 3 to 4 months to rule out Kidney cancer which is slightly more prevalent with patients of Overgrowth disorders.
* We would watch her leg length discrepancy and possibly use shoe lifts to minimize hip/knee problems and/or scoliosis.
* Watch and wait because as these patients age DVT’s (Deep Vein Thrombosis) and PE’s (Pulmonary Emboli) are very common because the overgrowth.
* Future Resection of significantly larger right buttock (our concerns were the affects of her posture and more importantly, her spine). She has to sit unevenly.
* May 2010 – Abnormal ultrasound – left kidney is significantly larger but kidney function tests are normal
* July 2010 – Dr. Rodgers Chief of Pediatric Surgery UVA offers to remove a small lipoma on Emaleigh’s left flank for a pathology consult. Pathology determined that the mass consisted of mature and immature adipose tissue that was not encapsulated. Dr. Rodgers worried that the tissue would grow back. As of 8/2011 no or minimal regrowth is seen.
* September 2010 – Abnormal ultrasound, left kidney shows dense speckled tissue and undefined nephrons but it appears to be the same shape and comparable to a kidney. All kidney tests are normal.
* October 2010 – Mass the size of a Nickel found on her spine in between her shoulder blades
* November 2010 – Surgery in Boston to remove both great and little toes and debulk left foot. Diagnosed with laryngomalacia but no problems with breathing. The mass seen on the back, could be a clot but after an ultrasound they determined it was a lymphatic cyst!
* January 2011– full body MRI using Boston’s Vascular Anomalies protocol. Kidney’s were similar as noted before but larger. Emaleigh showed large veins in her right leg but no vascular anomalies/malformations were seen in her chest or abdominal cavity. No scoliosis was noted.
* March 2011 – Review of MRI results with team in Boston. We need to think about resection of the right buttock in the near future but not a concern at this time, surgery on feet to remove toes and metatarsals. The original plan was at mid adolescence to drill out the growth plates to stop the growth of the toes but Dr. Spencer noticed that her feet have grown significantly. We discussed that she had very large deep veins in her right leg and we would watch those. Emaleigh started walking 3 weeks before her brother!
* May 2011 Kidney MRI - No change from previous MRI except minimal growth
* June 2011 – consulted with Dr. Adams and team about Emaleigh’s future. Ortho recommended the same foot surgery as Boston but mentioned that they would move the tendons to the next section of bone so she would still have similar movement, Dr. Dasgupta the general surgeon discussed a plan for Emaleigh’s future and what to expect (foot surgery this year, shoe lifts for her leg length discrepancy after her foot surgery, debulk the buttock at 7 or 8, early teen possibly drill out growth plate of femur/leg, early adult possible breast implant to restructure the breast tissue that will be lost because of a lipoma/fatty tumor) , a compression garment specialist came in to measure Emaleigh for garments to help with her circulation. Concerned about how puberty will affect the overgrown areas of her body, sometimes this makes symptoms “pop up” like Vascular Malformations.
* July 2011 - 501c3 approval for the CLOVES Syndrome Foundation with the mission of: The CLOVES Syndrome Foundation is focused on improving the lives of CLOVES patients by funding Overgrowth and Vascular Anomaly research. Our foundation is committed to raising public awareness and providing member support by offering educational literature and resources while also establishing support networks within the medical and overgrowth communities
* August 2011 – Amputation surgery of both feet scheduled for November 14. Will be removing all toes and metatarsals. Will also be moving the tendons.
* November 2011 – left foot toe amputation and right foot transmetatarsal amputation. Ultrasound for routine Wilms Tumor Screening
* December 2011 – MRI to review the masses in her left kidney. Revealed masses in both kidneys. Biopsy of large left kidney mass. After Christmas we received notification that Emaleigh is the first CLOVES person diagnosed with Wilms tumor (She has bilateral wilms)
* January 2012Started our 14 week Chemo therapy schedule with Actinomycin & Vincristine. The first 6 weeks is weekly doses of Vincristine and bimonthly doses of Actinomycin. January, her counts dropped (33 from 2500) and she was hospitalized for 5 days.
* February/March 2012 – 6 week MRI showed some reduction of the masses in the left kidney (top). The bottom mass didn't change. The right kidney’s masses are gone. Will continue with chemo for the rest of the schedule and determine the plan for moving forward.
* March/April 2012– The next 6 week MRI showed all masses are no longer visible in the right kidney but the left kidney did not respond. Scheduled a left kidney nephrectomy for 4/27
* May/June 2012 – continued final 2 doses of Chemo. Fit shoes for a .75cm shoe lift (left foot)
* July 2012 – Abdominal MRI gives us a clean bill of health
* June/July 2012 - We finished our last dose of Chemo and had our quarterly MRI. Her remaining kidney is clear and cancer free.
* Now that Emaleigh is in remission, we have the ability to focus on her Overgrowth Issues. We started a new drug in February 2013 called Sirolimus to either maintain her current overgrowth or reduce the lipomatous tissue. We will monitor her kidney with quarterly scans, discuss our orthopedic issues every 6 months, talk to our nephrology team, and discuss our next steps with our Overgrowth team.
* 4/2013, Both Emaleigh's kidney and abdomen look good but there appears to be a flat spot in the head of her femur, which turned out to be nothing but an "Emaleigh anomaly".
* 11/2013 - Emaleigh went through another surgery to remove some excessively large bones in her right foot.
* 12/2013 - Emaleigh developed a foot sore from her cast placement.
* 11/2014 - It's been a great year - just healing and clear scans!
* 7/2015 – We are talking about scheduling a debulking surgery of her right buttock. To learn more about CLOVES Syndrome. Go to www.clovesfoundation.org or Our community site is: www.clovessyndrome.org
