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Sharee's Story

BIRTH AND EARLY YEARS:

Due to increased amniotic fluid (polyhydramnios), Sharee was born at 32 weeks gestation rather than the usual 40 weeks.( Prior to birth, when the polyhydramnios became apparent, an ultrasound was performed to determine it's cause. It was noted that there appeared to be some thickness of tissue on one side of Sharee's facial cheek and two fingers were smaller. There may have been more notations made, but this is all we were told at the time)

At birth, Sharee had over seventy five percent of her face and body covered in mainly capillary stains and some port wine stain. She was quite colorful! Cosmetically, the other medically relevant things things we noted was her upper arms seemed to not have as much tissue as the lower -more so on one arm and she had two fingers that were smaller than her others making the third finger on that hand appear larger . Her feet were a little puffy -one foot more than the other and her second toe on that foot was a little longer than her big toe. The front of her foot was wider than a typical foot and there was a little extra space between her big toe and her second. Her one foot was sort of like a miniature bear claw donut. She had precious rosebud lips and beautiful eyes...but that is not medically relevant...so...we didn't realize upon looking her over that her one leg was wider in girth than the other- we found that out after the geneticist measured her extensively. There was no visible mass on her body at birth.

Fortunately, her lungs were fully developed , though she did have a quite high biliruben level causing her to have to be fed through a tube (gavage fed).We opted as a treatment choice to have Sharee placed under "bili"-lights to help bring her levels down. It worked wonderfully.

We met the next day with the geneticist at the University hospital who explained that he thought Sharee fit the Klippel-Trenaunay diagnosis . They did not think she had Sturge Weber syndrome, but due to the dark purple port wine on one side of her forehead, they said to watch her for seizures for the first two years. (Since this time, it has been noted that seizures can manifest themselves later than the first two years) They were ,also worried initially as to whether or not her liver was functioning due to the extensive capillary malformations covering her entire back. They weren't sure how deep into the organs the birthmarks went. Fortunately, that all was fine.

Sharee's father and I read all we could at the University Hospital library on Klippel-Trenaunay and, as others have stated it is usually the worse case scenarios. We were very worried for little Sharee' but after three and a half weeks we were delighted to take her home having learned how to gavage feed her pumped breast milk and infant CPR. (The usual guilt and worries as to why Sharee ended up with Klippel-Trenaunay were there. Yet, those had to be pushed to the background to best take care of her. And later, I would find out that I had been quite hypothyroid for years with Hashimotos thyroiditis and so I would wonder about that as a contributing factor as well.)

At home, Sharee's biliruben improved daily and slowly she worked her way to breast feeding. She did well. Her early days included visits to the local hospital (Sharee grew up 85 miles from the University hospital she was born in) to get her billiruben levels checked. Poor Sharee was poked in the foot everyday for her first several weeks home!

About a month after Sharee was at home, I had noticed that her port wine stain on her forehead as well as some of her other capillary stains appeared lighter. In a conversation with the geneticist about another matter, he mentioned that in lieu of that we could cease worrying about her having seizures. He stated that if her port wine was deep enough to be in the brain tissue that it would not lighten on it's own. I am not sure about the accuracy of that with today's current medical knowledge- but it was sure good news for Sharee at the time...and ,indeed, she has ,thus far, not had seizures.

ADOLESCENT YEARS:

Medically speaking, Sharee's concerns over her adolescent years were the usual concerns of most parents- the chicken pox etc.

Sharee was still mastering phonetics at the end of first grade and had to have some help with gross and fine motor skills as well as with math during her elementary years. I remember the nurse who worked with her on her gross and fine motor skills was concerned about Sharee's lack of stamina and that she would seem to be breathing really hard compared to the other kids after p.e. or recess. There was never anything found at the time to cause this.

We had not yet discovered the Klippel-Trenaunay support group as computers were not then (mid 1980's to early 90'0's) owned by almost every household as they are today.(Later a close and dear friend discovered the K-T support group and it is one of their members who introduced us to the Boston vascular group with her following CLOVES diagnosis) Hence, we were in ignorant bliss as to some of the possible medical concerns we should be watching for. So, in retrospect, while Sharee enjoyed a more "normal" early childhood, she was not followed as closely to rule out things like scoliosis or the possible need for growth plate stapling of the knee as she should have been. Sure, the school nurse did the annual checks, but in Sharee's case, she went, from what appeared to not be nothing noteworthy one year- to over a fifty five percent curvature within that next year duration! What did that mean for Sharee? Well, within that one year, she went from, as far as we knew, no scoliosis to being past the point she could be treated with a brace, to having no choice but to have surgery. So, after seeing several specialist, Sharee was scheduled for a knee stapling in November (an outpatient surgery) and major scoliosis surgery the following December. She was thirteen years old. (It turned out years and years later, when we gathered all Sharee's medical records for the doctors in Boston to review, that right there in her birth notes, scoliosis was listed. That we know of, we were never told. It definitely was not told to us to be something to watch for. Of course with the concerns about her just having Klippel-Trenaunay- it might have been overlooked)

Hence, advancements, such as the ones by Dr. Alomari in differentiating CLOVES syndrome and recognizing the scoliosis risk are immeasurable in value for patients and parents as they now know to keep a careful and medically supervised,( preferably by a specialist), watch on this, as it can worsen very quickly as shown by Sharee's case.

PUBERTY CHANGES,OVERGROWTH & SCOLIOSIS:

About this same time, I wish I could recall if it were before the surgeries or after, but I cannot, Sharee began to very slowly have overgrowth of her right shoulder blade and hip. This is of both the bones and tissue as well. I, later wondered if it was the curving of the spine causing it to appear that way- but while that may enhance the appearance of overgrowth- there really IS an overgrowth that was not there noticeably to the naked eye at birth or in her early childhood. Perhaps it was there...just so slight it wasn't then noticeable. Who knows? In any event, this is another medical thing to watch for- however, I am not sure whether or not all CLOVES patients have this, I suppose one would have to ask the specialists. As many with a chronic condition, Sharee does not have a self-indulging attitude but,rather, has a positive attitude with a smile for those she meets. However, puberty was a difficult time when others bodies are changing towards typical teenage pursuits,Sharee's was changing in a more painful way. Yet,she has always had hope due to her core beliefs and a wonderful people to support her. I must state, lest someone think their child is always exaggerating, that the discomfort and pain from Sharee's condition is in no way imagined, but is physically derived from hypertrophy of one side of the body. One only has to watch when she walks or see beneath what the clothes hide to know that she has to, at some times more than others, be in pain. Sharee wears a shoe lift to make up for the after surgery 1/2 " discrepancy remaining and has gone to men's tennis shoes for comfort.

MIGRAINES:

About four years prior to Sharee's scoliosis and knee stapling surgeries, Sharee began suffering from migraine headaches. I never associated them with the Klippel- Trenaunay as several in my family as well as myself, suffer from them as well.

However, over the next ten to fifteen years, the frequency of Sharee's migraines exceeded anything experienced by my family. They became almost daily. Not that they were all headaches she threw up with, but they were definitely there and she no longer felt like doing things other kids did a lot of the times. They are definitely problematic. In fact, due to the increasing frequency of migraines, when she was in eighth grade, she asked us if she couldn't home school in high school. We talked to her teacher and she explained how difficult it was becoming for Sharee due to her missing so many morning classes. She said that Sharee was an oral learner and that is when she did most of her oral instructing. So, after much deliberation, we all decided that it might be best for Sharee to participate in an accredited home school program in high school as she was missing so many mornings in school due to the headaches. In the state we are from, parents have to meet with the superintendent to receive his approval prior to making this change. Upon meeting with him, he told us that after reviewing Sharee's circumstances and how her grades had lowered after being on the honor roll for some time, that he felt that due to her headaches it would be in her best interest if she did make the change to work around her headaches. We knew Sharee to be a self-disciplined person and knew she would stay on task with her schoolwork.Sharee did well in the American Home school course and graduated with a nice GPA. Her being an oral learner became more obvious over time and while she succeeded in High School we knew she would need help in whatever she decided to go on to career wise. At that time Sharee opted to attend a tech school for medical assisting rather than a four year college. Her wonderful father, reviewed orally the information she would cover prior to exams and Sharee did well and received her national certification.

ADULTHOOD THUS FAR:

Recently, Sharee was found via CTA to have enlarged veins in her chest/neck area including the subclavian vein. For the time being, she cannot have surgery due to enlarged veins. She is going to have a veinography done to see the status of her left chest/neck veins as well. Sharee recently had a bad bout of what seemed to be persistent bronchitis. She had been on six different antibiotics this past winter.

As hinted at earlier, Sharee does not seem to have the same amount of energy as her peers. I am not sure if this is CLOVES syndrome related or due to the migraine struggles etc. In any event, on good days and if she wants to, Sharee can climb the tallest....MALL shopping! Mount Everest...may have to wait for now! However, before her leg length discrepancy and scoliosis became more prominent, she climbed fifteen hundred vertical feet starting at over 9,000 ft elevation to 11,000 ft of elevation- she was four years old! Mom and Dad were there as well beside her. As a child she would go camping and liked to fish . As a teenager she also liked to snowmobile. She has always liked to shop. Yet, on a typical day...she faithfully goes to work- she pushes herself even with the migraines and rarely ever has to take a day off. However, come the end of the day, she is usually done for. Her leg will ache or her head is hurting worse. So, she rests in the evenings. She is on facebook, likes movies, reads & tortures her grandmother and Aunt(just kidding). She and her Aunt and her grandmother all share a place and each pay for their own meals and a third of gas ,electricity etc. On the weekends, she may have a bad headache one day and need to rest and have one good day. She is active in her religious beliefs (sometimes she has to listen to the meetings on the phone due to a headache) and has people who care about her. Neurontin was recommended for her migraines recently and seems to be helping the intensity of her headaches. On good days...she is more active and her wit is boundless.

As others will say of themselves or their child with CLOVES syndrome, Sharee is not CLOVES and CLOVES is not Sharee, but they have met and battled and a truly beautiful and caring young woman is what has emerged! That is Sharee.

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