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$3,626 of $25,000
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38 donations

This fundraiser ended on 01/01/12

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Kiersten's Cause.. where family and friends are uniting to raise funds to offset medical costs during her battle with Sturge-Weber Syndrome

Kiersten's Story

Jake and Mary were blessed with the gift of Kiersten on September 5th, 2011. First time parents, they were both excited and nervous about the adventure and direction life with a newborn was going to take . Mary had a healthy pregnancy, carried full term and no signs indicated any concerns for Kiersten throughout her pregnancy. When Kiersten was born doctors realized that there were some visable complications with her health, but did not know to what extent these would affect Kierstens life and the lives of Jake and Mary. Kiersten was diagnosed with Sturge Weber Syndrome .



Sturge-Weber Syndrome (encephelotrigeminal angiomatosis) is a congenital, non-familial disorder of unknown incidence and cause. It is characterized by a congenital facial birthmark and neurological abnormalities. Other symptoms associated with Sturge-Weber can include eye and internal organ irregularities. Each case of Sturge-Weber Syndrome is unique and exhibits the characterizing findings to varying degrees.



About Sturge-Weber Syndrome



Sturge-Weber Syndrome (encephelotrigeminal angiomatosis) is a congenital, non-familial disorder of unknown incidence and cause. It is characterized by a congenital facial birthmark and neurological abnormalities. Other symptoms associated with Sturge-Weber can include eye and internal organ irregularities. Each case of Sturge-Weber Syndrome is unique and exhibits the characterizing findings to varying degrees.



Facial Birthmark



The most apparent indication of Sturge-Weber Syndrome is a facial birthmark or "Port Wine Stain" present at birth and typically involving at least one upper eyelid and the forehead. Much variation in the size of the stain has been reported and may be limited to one side of the face or may involve both sides. The stain, varying from light pink to deep purple, is due to an overabundance of capillaries just beneath the surface of the involved skin. In persons with dark pigmentation, the stain may be difficult to recognize. In rare instances, there is an absence of a Port Wine Stain.



Neurological Abnormalities



Neurological concerns relate to the development of excessive blood vessel growth on the surface of the brain (angiomas). These are located typically on the back (occipital) region of the brain on the same side as the port wine stain. These angiomas create abnormal conditions for brain function in the region. Seizure activity is the most common early problem, often starting by one year of age. The convulsions usually appear on the opposite side of the body from the Port Wine Stain and vary in severity. Vigorous attempts are made to control the seizures with medication. A weakening or loss of the use of one side of the body (hemiparesis), may develop opposite to the port wine stain. Developmental delay of motor and cognitive skills may also occur to varying degrees.



Other Manifestations



Increased pressure within the eye (glaucoma) is another condition which can be present at birth or develop later. The incidence of glaucoma in patients with Sturge-Weber is approximately 70% and 40% for choroidal lesions. The glaucoma is usually restricted to the eye which has the stain involvement. Enlarging of the eye (buphthalmos) can also occur in the eye which has been affected by the stain. Multiple other body organs are rarely affected in Sturge-Weber syndrome. Infants affected with Sturge-Weber Syndrome are often monitored by a pediatrician, neurologist, ophthalmologist and dermatologist.



Treatment



Laser treatment is available to lighten and or remove port wine stains in children as young as one month of age. Anti-convulsants are used to control the seizures. VNS implants and brain surgery can be also be utilized for seizure management. Eye drops or oral medications are used to control the glaucoma. Should topical or oral medications not prove effective, the next step would be surgery.



In 1992, E. Steve Roach, MD classified the SWS spectrum, delineating for the first time the varying degrees of involvement previously noted in this condition.



Type 1



he most common, this type involves both facial and leptomenigeal (brain) angiomas (vascular malformations) and may involve glaucoma. Seizures usually occur within the first year of life, as a result of the brain involvement. The ocular involvement is normally noted within the first year of life, as well. The white portion of the eye may appear "bloodshot" as a result of the over-proliferation of blood vessels on the eye. In rare cases, the facial and brain involvement are bilateral (involving both sides of the head). Mental and physical development can be impaired to varying degrees, depending on the amount of vascular birthmark throughout the brain and eye.



Type 2



This type involves a facial angioma and the possibility of glaucoma, but no evidence of intracranial disease. There is no specific time-frame for the exhibition of symptoms beyond the initial recognition of the facial PWS. Throughout the life of the individual, interrelated symptoms may manifest in glaucoma, cerebral blood flow abnormalities, headaches, and various other complications. Additional research needs to be conducted on this type of SWS to determine the course of the syndrome over its natural progression.



Type 3



This type of SWS is commonly noted to have a leptomeningeal angioma, with no facial involvement and usually no development of glaucoma. Commonly referred to as forme fruste, this type is identified through brain scans. It can also be confused with other diagnoses prior to a brain scan with contrasting agent. While social stigma is lessened by the absence of PWS, the unknown natural course of the syndrome is still frustrating for parents and professionals treating the condition.



Kiersten has Port Wine Staining on the entire half of her body, glaucoma and neurological concerns. Can you imagine, as parents walking into the hospital to be given the most wonderful gift, then being told that your beautiful daughter will have a very tough road ahead. The emotional spiral coupled with the financial outlook would be almost overwhelming. Jake and Mary have such wonderful hearts and have said we will do whatever it takes to help Kiersten have an amazing life. This is where we as family & friends can help ease these struggles. Together, we can all make a difference in this little girls life, not only through monetary support, but loving this family and giving them a wonderful support group to count on.



We are in the process of planning a fundraiser and exploring just the right ways to help Jake, Mary & Kiersten. Graciously, a Dr. in New York, has offered their services to begin lazer treatments on Kiersten to help diminish the port wine staining. The family must travel to New York every 2 weeks for 20 treatments. Although the medical costs are covered the transportation, hotel and time missed from work is not. This is an example of how our donations will help Kierstens parents. Jake owns his own Landscaping business and Mary is due to go back to work at the doctors office. They never planned on anything like this thappening and have very limited funds and income to assist with the future costs.



Again, thank you for reading Kierstens story, stay connected for updates on fundraising efforts, and please - any words of kindness will be appreciated by the family
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