A Chance to Smile
Nine years ago, my sister was born with a cleft lip and palate. Every three minutes, a similar birth occurs around the world; however, my sister did not simply become this statistic.1 She became an individual who changed my life. Our story might change yours.
Figure 1 at birth
A cleft lip “is an opening in the upper lip that can extend into the base of the nostril,” and this facial deformity can occur with or without a cleft palate, which “is an opening in the roof of the mouth.”2 A cleft lip with or without a cleft palate is the fourth most prevalent birth defect among infants born annually in the United States.3 According to the studies taken by the CDC between the years 2004-2006, 4,437 infants are born with or without a cleft palate and 2,651 children are born with a cleft palate in the United States every year.4 By the time you are finished reading this article, two children will be born with a cleft lip somewhere around the world.
Certain factors such as specific medicines, underlying syndromes, and genetics can increase the chances of a child being born with a cleft lip and palate. Some medicines are known to lead to a greater chance of a woman having a child with a cleft defect. Examples of these medicines include valproic acid, phenytoin, and topiramate, all of which are used to minimize symptoms of epilepsy specifically through treating seizures.5 Women who have diabetes, consume alcohol, or smoke will have a higher chance of having a child with this common birth defect.6 Genetics are also known to play a role in children having cleft features. Seattle Children’s Hospital notes, “More than 40% of children who have cleft palate but without cleft lip have conditions such as Van der Woude syndrome, 22q11.2-related disorders, Robin Sequence, Stickler syndrome, and Treacher Collins syndrome.”6 Syndromes that specifically impact the formation of the face and mouth are linked to children born with cleft lip and palates. For example, the “Van der Woude syndrome is a condition that affects the development of the face. Many people with this disorder are born with a cleft lip, a cleft palate… or both.”7 Cleft lips and palates are occasionally linked with genetic syndromes, while other times this birth defect happens at random.
In addition to the above factors contributing to cleft defects, many cases (60%) are random and do not correlate with a potential cause.6 My sister is one person of the 60% and falls under the category where genetics and medicines did not play a role in her being born with a cleft lip and palate. My parents were told that my sister would be born with a cleft lip and palate after an ultrasound. My sister’s lip simply did not fully form between my mother’s fourth and seventh weeks of the pregnancy, while her palate did not form between the sixth and ninth weeks.
Figure 1’s ultrasound showing her cleft lip and palate (as well as part of her lips and one nostril).
Development issues can affect several areas of childhood including a child’s feeding, gum, teeth, and speech. In most cases, feeding an infant with a cleft lip is difficult because the child is unable to grasp his or her mother’s nipple or a bottle and or effectively suction the milk because of the opening in the lip and palate. Various bottles or nipple covers are used to feed a child with this birth defect. Other developmental issues that may be experienced by infants with cleft lips and palates include having “teeth that are misshaped, crowded or missing…Children with clefts are at higher risk for cavities.”6 Speech impediments (such as velopharyngeal dysfunction) due to the lack of a soft palate and hearing problems due to increased fluid in the ear can occur as well. Thankfully, my sister had no serious feeding issues as an infant or speech impediments in her toddler years. Now at nine years old, she is already one year into her orthodontic work with an expander and will need braces soon, as well as another surgery when she is around ten years old for a permanent bone graph (called an alveolar bone graft) to hold her braces and keep her permanent teeth in place. Like others in our place, positive change becomes possible through science, passion, and dedication.
Surgeries in the United States can vary greatly from surgeries outside the country, but they are united in one common goal: to help give a child the chance to smile again. My sister was given the chance to smile again, so I can appreciate the work of craniofacial surgeons even more and I am able to share her experiences so others become more aware of this birth defect.
Figure 1 one week post-surgery
Operation Smile’s Dr. William Magee performed my sister’s cleft lip and palate surgery down in Norfolk, Virginia when she was only two months old. The surgery cost around $75,000. Fortunately, a majority of that amount was covered by insurance. The surgery cost will differ for each patient due to various factors such as their insurance or where the surgery is being performed. The time frame of her surgery was about four hours including the time it took for her to be put under anesthesia. Surgeries by Operation Smile that are performed in other countries will cost around $240 (the amount an individual can donate to cover the cost of a surgery to make it costless for a family in another country) and take at least 45 minutes.8
A cleft lip and palate is not only a medical issue, it is a social issue as well. A child born with a cleft lip and palate may feel excluded because “self perception plays a pivotal role in influencing an individual's self esteem and psychological adjustment affected by cleft lip and palate anomaly…The attitudes, expectations and degree of support shown by parents can influence a child's perception of their cleft impairment.”9 Beginning with a child’s family and then extending to the child’s friends and who he or she encounters on a daily basis, a child with a cleft defect can be influenced by the actions of others. Family and friends often provide a critical support system that decreases social anxiety or exclusion the child may feel. Whether a child is growing up in the United States or in a foreign country where finding the resources to fix a cleft lip and palate is more difficult, “[a] social stigma is created within an individual when he or she is negatively discriminated by labeling him/her different from normal…A negative response from outsiders, actual or perceived, may adversely affect self-image.”9 Most children with this facial deformity who are born in the United States are able to seek a treatment and surgery plan immediately after birth. With the greater availability of medical resources in the United States, children have an easier time growing up compared to children who are not able to fix the deformity by six months of age. When children and adults outside the country experience hardships while trying to find treatment and afford the surgery, those individuals are unable to fix this birth defect; they become targeted more for the social and psychological issues (such as bullying in schools) or simply have trouble performing daily tasks that many people are able to do easily (such as speaking or eating). Operation Smile gives these children and adults the chance to smile and experience a new life.
In countries, such as India, a child with cleft lip and palate will have a tough time finding surgical treatment. Researchers on cleft lip and palates in India, Peter Mossey and Julian Little state, “It is known, however, that in many parts of India the parents of a child born with a cleft have no access to counseling on the care and treatment of their children,” and this occurs because there is not sufficient documentation and records for researchers to study.10 Mossey and Little further analyze the situation in India by adding, “Cleft lip and palate may be perceived to be a life threatening abnormality and there may be little awareness of the fact that clefts can be surgically repaired with considerable success both aesthetically and functionally.”10
In order to prevent a cleft lip and palate from remaining a common birth defect in the United States and in other countries, researchers have recently successfully located the gene that causes the emergence of this facial deformity. Current research done on the cleft lip and palate causes is as follows: “An international team, led by Newcastle University, UK, and the University of Bonn in Germany, has found that variants near a gene called GREM1 (Gremlin1) significantly increase the risk for cleft lip and palate.”11 These studies have shown that “increased activity” of this gene causes a cleft lip, and by looking more into the gene examined in this study, researchers may be able to take steps to prevent this birth defect.11
The science surrounding the cleft lip and palate has advanced impressively over the years as research on this birth defect continues. Factors contributing to the problem have been identified; although there is not one single factor that causes cleft lips and palates, factors that have been targeted as a cause include specific medications, habits such as smoking or drinking alcohol, and genetics, or as a random occurrence that happens during the first few weeks of pregnancy. The ability for doctors and researchers to pinpoint these factors displays how we are beginning to learn more about a cleft lip and palate on a molecular and genetic basis. Also, a cleft lip and palate can cause various developmental issues and require the need for medical attention such as a surgery or a few surgeries. Many doctors are skilled in their abilities to fix this common birth defect, but there are differences between surgeries and patient development from one country to the next. Medical professionals are literally and figuratively changing the face of those with cleft features, and in so doing creating new smiles and enabling new paths in life. Doctors like Operation Smile’s Dr. Magee changed my sister’s life by giving her the chance to smile and with this experience, my life was changed as well. Hopefully, this good fortune will reach others around the world, so they too can have a chance to smile.
Figure 1at one-year of age
Endnotes:
“About Cleft Lip & Cleft Palate.” Operation Smile. N.p., n.d. Web. 12 Nov. 2016.
“Cleft Lip and Palate | Definition of Cleft Lip and Palate by Medical Dictionary.” The Free Dictionary. Farlex, n.d. Web. 24 Oct. 2016.
“Cleft Lip and Cleft Palate: Causes and Treatments.” WebMD. WebMD, n.d. Web. 24 Oct. 2016.
“Prevalence (Number of Cases) of Cleft Lip and Cleft Palate.” U.S National Library of Medicine. The Centers for Diseases Control and Prevention, 7 Mar. 2014. Web. 12 Nov. 2016.
“Facts about Cleft Lip and Cleft Palate.” Centers for Disease Control and Prevention. Centers for Disease Control and Prevention, 12 Nov. 2015. Web. 24 Oct. 2016.
“Cleft Lip and Cleft Palate.” Seattle Children's Hospital: World-Class Child Healthcare. N.p., n.d. Web. 24 Oct. 2016.
“Van Der Woude Syndrome.” Genetics Home Reference. N.p., 8 Nov. 2016. Web. 12 Nov. 2016.
“Donate Now to Support Operation Smile.” Operation Smile. N.p., n.d. Web. 22 Nov. 2016.
Sousa, Avinash De, Shibani Devare, and Jyoti Ghanshani. “Psychological Issues in Cleft Lip and Cleft Palate.” Journal of Indian Association of Pediatric Surgeons. Medknow Publications, 2009. Web. 24 Oct. 2016.
Mossey, Peter, and Julian Little. “Addressing the Challenges of Cleft Lip and Palate Research in India.” Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India. Medknow Publications, Oct. 2009. Web. 13 Nov. 2016.
Newcastle University. “Breakthrough Made in Cleft Lip and Palate Research.” ScienceDaily. ScienceDaily, 24 Mar. 2016. Web. 13 Nov. 2016.
Works Cited
“About Cleft Lip & Cleft Palate.” Operation Smile. N.p., n.d. Web. 12 Nov. 2016.
“Breakthrough Made in Cleft Lip and Palate Research.” ScienceDaily. Newcastle University, 24
Mar. 2016. Web. 12 Nov. 2016.
“Cleft Lip and Cleft Palate: Causes and Treatments.” WebMD. WebMD, n.d. Web. 24 Oct. 2016.
“Cleft Lip and Palate | Definition of Cleft Lip and Palate by Medical Dictionary.” The Free
Dictionary. Farlex, n.d. Web. 24 Oct. 2016.
“Cleft Lip and Cleft Palate.” Seattle Children's Hospital: World-Class Child Healthcare. N.p.,
n.d. Web. 24 Oct. 2016.
“Donate Now to Support Operation Smile.” Operation Smile. N.p., n.d. Web. 24 Oct. 2016.
“Dilantin: Uses, Dosage, Side Effects & Interactions - Drugs.com.” Dilantin: Uses, Dosage, Side
Effects & Interactions - Drugs.com. N.p., n.d. Web. 24 Oct. 2016.
“Facts about Cleft Lip and Cleft Palate.” Centers for Disease Control and Prevention. Centers
for Disease Control and Prevention, 12 Nov. 2015. Web. 24 Oct. 2016.
Mossey, Peter, and Julian Little. “Addressing the Challenges of Cleft Lip and Palate Research in
India.” Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India. Medknow Publications, Oct. 2009. Web. 13 Nov. 2016.
Newcastle University. “Breakthrough Made in Cleft Lip and Palate Research.” ScienceDaily.
ScienceDaily, 24 Mar. 2016. Web. 13 Nov. 2016.
"About Cleft Lip & Cleft Palate." Operation Smile. N.p., n.d. Web. 22 Nov. 2016.
"Prevalence (Number of Cases) of Cleft Lip and Cleft Palate." U.S National Library of Medicine.
The Centers for Diseases Control and Prevention, 7 Mar. 2014. Web. 12 Nov. 2016.
Sousa, Avinash De, Shibani Devare, and Jyoti Ghanshani. “Psychological Issues in Cleft Lip and
Cleft Palate.” Journal of Indian Association of Pediatric Surgeons. Medknow Publications, 2009. Web. 24 Oct. 2016.
“Van Der Woude Syndrome.” Genetics Home Reference. N.p., 8 Nov. 2016. Web. 12 Nov. 2016.
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