Stella’s Journey with Bronchopulmonary Sequestration (BPS)

By Sharlene Ramisingh, mom of CHOC Children’s patient Stella

My daughter Stella first came to CHOC Children’s when she was just a baby, but our journey really started before she was even born. At my 20-week ultrasound, my husband Havinder and I were so excited to find out the sex of our baby. We were having a girl!

That excitement quickly turned into a feeling of unease when the physician noticed an abnormal growth in the baby’s lung area. From that point on, we had to go back to the doctor’s office for monthly and eventually weekly ultrasounds to monitor the size of the mass. After additional imaging, doctors diagnosed her with bronchopulmonary sequestration (BPS). This means that abnormal tissue or a cyst formed outside her lung but wasn’t connected to her airway.

It was determined that the size of the mass wouldn’t affect delivery, and I was able to carry her to term and proceed with my birth plan. When Stella was born, there was a room full of doctors and nurses in case she wasn’t breathing. Time stood still when she came out and the room was so quiet. Everyone was waiting to hear her cry. And when she let out a tiny little sound, it felt like everyone in the room exhaled at once.

Despite the mass on her lung, she was healthy, so we got to take her home a few days after she was born. Stella’s first few months of life were filled with a series of appointments and tests. When we met Dr. Peter Yu, a pediatric general and thoracic surgeon at CHOC, he was able to give us confirmation that Stella needed surgery to remove the mass. At that point, Stella had grown bigger and stronger and was better prepared for the anesthesia required for surgery. We knew that our daughter was in good hands with Dr. Yu. He was patient, caring and we really felt like our daughter was the most important thing to him.

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Sharlene with her daughter Stella on the morning of surgery to remove her bronchopulmonary sequestration (BPS).

Stella was just five months old when she underwent surgery at CHOC Children’s Hospital. Thankfully, surgery went perfectly, and the whole team was great. The pediatric anesthesiologist was amazing and able to deal with my daughter’s small veins.

Before Stella’s diagnosis, I had never heard of BPS. During my pregnancy and up until the surgery I was part of a private Facebook group for parents of babies with BPS. Their success stories helped me manage the stress and anxiety that I experienced during this time. I was also able to ask questions to moms who went through the surgery and who had healthy kids. My husband and I were very private about her condition during my pregnancy, and only told our parents and siblings. We were dealing with our own emotions, and we just didn’t feel up to having to explain BPS over and over again. When it did come up, we would say she a birth defect that lead to a growth in her lungs, and that it would need to be removed.

These days, Stella is doing amazing. She sprints around the house with her five-year-old brother on a daily basis, and she has a voice that will make the windows shake. She goes to swim lessons every week.

stella-after-BPS-surgery-with-dog
Stella continues to thrive after surgery to remove her bronchopulmonary sequestration (BPS).

The worrisome portion of my pregnancy and Stella’s surgery seem like such a distant memory. You would never know by looking at my daughter that she ever had any complications.

My advice to new parents facing an unexpected diagnosis is to take things one step at a time. Don’t get caught up in the negative thoughts that may happen and trust your child is in good hands with Dr. Yu and at CHOC. Always remember that this too shall pass.

Meet our surgeons

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Meet Dr. Saeed Awan

CHOC Children’s wants its patients and families to get to know its specialists. Today, meet Dr. Saeed Awan, a pediatric general and thoracic surgeon. Dr. Awan attended medical school at Khyber Medical College, in Pakistan, and completed a surgery residency at Memorial University of Newfoundland & Labrador in Canada. He also completed two surgery fellowships, one at Royal College of Physicians and Surgeons of Edinburgh, in United Kingdom, and another at Royal College of Physicians and Surgeons in Canada. He has been on staff at CHOC for four and a half years.

Dr. Saweed Awan
Dr. Saeed Awan, pediatric general and thoracic surgeon at CHOC Children’s.

Q: What are your special clinical interests?

A: All aspects of pediatric and neonatal surgery, especially minimal invasive surgery. I also have expertise in liver and bile duct surgery and special interest in gastroschisis sutureless closure.

Q: What are your most common diagnoses?

A: Inguinal and umbilical hernias, hydroceles, undescended testes, appendicitis, lumps and bumps, as well as complex congenital pediatric and neonatal conditions.

Q: Are there any new programs or developments within your specialty (at CHOC or in the broader field)?

A: In the broader field, there has been remarkable advancement in minimally invasive surgical procedures with similar or better results than open surgical procedures.

Q: What would you most like community/referring providers to know about you/your division at CHOC?

A: We are a group of dedicated and highly trained pediatric surgeons who strive to provide excellent care to our patients. We make sure both parents and the patients are involved in the decision making. Being a father of four children, I treat my patients as I would like to have my own children treated.

In addition to our common surgical conditions, I treat complex neonatal and gastrointestinal conditions.

Q: What inspires you most about the care being delivered here at CHOC?

A: I’m inspired by our CHOC physicians, our excellent nursing and support staff, our wide range of subspecialists, as well as our state-of-the-art facility.

In our specialty, we meet a lot of children and the smiles on their and their parents’ faces after they have recovered from surgery, makes me very happy.

Q: Why did you decide to become a pediatric surgeon?  

A: I decided to become a pediatric surgeon when I was a third-year medical student. Pediatric general surgery is a field where you are able to take care of sick kids with a wide variety of surgical diseases. Although pediatric surgery is very challenging, it is very rewarding.

Q: If you weren’t a physician, what would you be and why?

A: I might have been a pilot as I like travel.

Q: What are your hobbies/interests outside of work?

A: Travel, soccer and spending time with my children.

Learn more about pediatric surgery at CHOC

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The Appendix: 5 Common Questions

More than 700 kids are treated for appendicitis at CHOC Children’s every year. The appendectomy is one of the most commonly performed surgeries in the world. But just what is the appendix, and why does it send so many people to the emergency room with stomach pain?

Dr. Peter Yu, CHOC Children’s pediatric general and thoracic surgeon, explains everything you’ve ever wondered about the appendix, and more.

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Dr. Peter Yu, pediatric general and thoracic surgeon at CHOC Children’s

 

  1. What does the appendix do?

The appendix is a small, finger-like projection that sticks out of the large intestine, located in the right side of the abdomen. It weighs about as much as an earthworm. In fact, its old-fashioned name is vermiform appendix, which means worm-like, Dr. Yu explains.

Everyone’s appendix is different. Some are long, some are short and stubby. But one thing they all have in common is that they are not necessary for a happy, healthy life.

“We are not sure if the appendix has a purpose. Some doctors don’t believe it does anything,” Dr. Yu says. “Some think it plays a role in the development of the immune system, and some believe it harbors ‘good bacteria’ that helps intestinal health. The bottom line, though, is that appendicitis is common, and patients do extremely well after removal of their appendix.”

  1. What causes appendicitis?

Bacteria naturally live in the large intestine and flow in and out of the appendix. Sometimes, the opening to the appendix gets blocked. Either constipation, a hard piece of stool called a “fecalith,” or enlarged lymph nodes cause these blockages.

The blockage traps the bacteria inside where it festers and multiplies. This leads to inflammation of the organ. If left untreated, the appendix can burst, releasing the infectious bacteria into the body.

Symptoms of a Burst or Inflamed Appendix

What are signs of appendicitis?

  • sudden severe pain
  • pain that starts near the belly button and moves to the lower abdomen on the right side
  • fever, nausea or vomiting

To diagnose appendicitis, the Julia and George Argyros Emergency Department at CHOC Children’s Hospital will check your child’s blood for signs of an infection and will do an ultrasound of the abdomen. While many hospitals use a CT scan to diagnose appendicitis, CHOC radiologists and sonographers have the training and experience to make a diagnosis using ultrasound, in order to minimize your child’s exposure to radiation. If the ultrasound is inconclusive, the radiologist may conduct a CT scan.

  1. How does the surgeon remove the appendix?

The surgeon will perform a procedure called a laparoscopic appendectomy to remove the appendix. A pediatric anesthesiologist will put your child to sleep using general anesthesia. The procedure takes about 30 minutes, though CHOC’s pediatric general surgeons can remove the organ in less than 10 minutes if needed.

During surgery, three tiny incisions are made on the abdomen. Carbon dioxide is blown into the belly to create a dome, giving the surgeon room to work. Small surgical tools are inserted in two of the incisions and a laparoscopic camera is inserted in the third. The appendix is identified, stapled or tied off, and removed.

The surgeon closes the incisions with surgical glue and dissolvable strips. In most cases, children will stay in the hospital for one day before the doctor discharges them. They should have no heavy activity or sports for two weeks after surgery and can usually return to school quickly, often even the next day.

  1. What do you do with the appendix after you take it out?

Pathologists then inspect the removed appendix in the pathology department under a microscope. This inspection is important because it will confirm the diagnosis of appendicitis and rule out other conditions such as ulcerative colitis, Crohn’s disease and carcinoid. Your surgeon will update you with the results during your follow-up appointment.

  1. What if my appendix bursts?

“Fortunately, perforated appendicitis is less common than non-perforated appendicitis, but it can happen,” Dr. Yu says. “For some, the appendix can burst quickly, and for others it does not burst at all. There are many factors that a surgeon will consider before deciding whether to operate immediately, or to wait.”

If your surgeon decides to wait, then treatment can include antibiotics, placement of a drain in the abdomen, and nutrition through an IV if needed. Most patients improve in several days, after which the doctor discharges them. Your surgeon will then schedule your child for an interval appendectomy, which is removal of the appendix 8-12 weeks later. This gives the body time to recover from the infection and inflammation, making surgery safer and less complicated.

The CHOC Emergency Department, equipped to treat appendicitis 24 hours a day, with pediatric surgeons ready for all situations is mainly for kids and teens.

Learn more about appendicitis in kids and teens

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An Unexpected Case of Appendicitis: Jordan’s Story

Six-year-old Jordan was enjoying a fun break with his family in Big Bear, Calif. when he started complaining of stomach pains one afternoon. He had no chronic conditions or other symptoms, so his family didn’t immediately think it was anything serious. They assumed it might be a stomach virus, and never imagined that he would need to undergo surgery at CHOC Children’s a mere 24 hours later.

stomach pain or appendicits
When Jordan complained of stomach pains while on vacation with his family, they never imagined he’d undergo surgery a mere 24 hours later.

Late that night Jordan was in tremendous pain, so his parents took him to an emergency room near their resort. Two local physicians ruled out anything serious. They told Jordan’s parents it was likely just constipation or gas, and sent Jordan home.

“We were wary of the diagnosis received and being vigilant, we cut our family vacation short and rushed home so we could be closer to CHOC if his condition worsened,” says Jordan’s mom, Emma.

His pain became unbearable overnight.

“We knew his pain was abnormal and didn’t want to take any more chances at any local hospital. I wanted to know my son was in a place that specializes in kids.”

An accurate and timely diagnosis at CHOC

Testing in the ED immediately revealed that Jordan had a unique case of appendicitis with ileo-colic intussusception (a condition where part of the intestine folds into itself, like a collapsing telescope), and would undergo surgery that same day.

“Appendicitis is the most common reason for abdominal surgery in children. Pediatric surgeons at CHOC saw over 700 cases of appendicitis last year,” says Dr. Peter Yu, a pediatric general and thoracic surgeon at CHOC. “Many of these patients first come through the emergency department, meaning our ED staff is well-versed in both classic and non-traditional symptoms of appendicitis, and very unlikely to miss a diagnosis. Also, when we discover unusual variations associated with common childhood illnesses, such as ileocolic intussusception with acute appendicitis, the pediatric surgeons here are prepared to deal with it.”

Surgery at a Place Designed Just for Kids

Receiving a correct diagnosis faster meant that Jordan was on his way to surgery before his symptoms worsened.

“Jordan’s parents made a good decision in bringing him straight to CHOC,” says Dr. Yu. “If they had gone to an ‘adult’ hospital first, they would likely have been transferred to CHOC for surgery anyway.  This can be costly, delays care, and can be a huge inconvenience for families.”

Learning that your child needs surgery can be scary for parents to hear. But thanks to Jordan’s parents’ decision to bring him to a hospital that only treats kids, Jordan was under the care of pediatric-trained specialists using equipment that’s made just for kids.

CHOC has pediatric surgeons on call 24/7, meaning there is always a pediatric-trained specialist ready to treat your child, no matter what time of day or night your child needs surgery.

“These events are extremely stressful as a parent because while we are still digesting the diagnosis, we need to ensure we make sound decisions in a short amount of time,” says Emma. “Dr. Yu was patient and very thorough, and I knew there was no one else I would rather have care for my son.  He was in excellent hands. We were probably his last surgery of the day but he made us feel like we were his first.

After making the decision to return to CHOC for Jordan’s correct diagnosis and surgery, their son was not only in the hands of a pediatric surgeon, but a pediatric anesthesiologist as well.

“Although, an appendectomy is one of the less invasive surgeries a child can undergo, as a parent you’re still very much concerned of the potential issues that could arise from going under anesthesia,” Emma recalls. “Knowing he was under the care of an anesthesiologist who specializes in children was a great comfort to my husband and me. After we met with his surgery team, we knew he was in the best hands possible.”

stomach pain or appendicitis
Jordan and his pediatric surgeon., Dr. Peter Yu.

Healing faster in an environment just for kids

Jordan sailed through surgery with flying colors. His appendectomy was done via a single incision hidden in his belly button, meaning he will have minimal to no scarring after surgery. His intussusception self-resolved and needed no additional surgical intervention.

After surgery, Jordan only needed to stay at CHOC for one night for observation, but he had so much fun that he would have stayed even longer if he could have, Emma recalls.

“Jordan loved being at the hospital because he felt like he was the boss! Everyone was so attentive to him. Every person that we interacted with, from the nurses that cared for him and spoke in a way he would understand, to the volunteer in the play room that let Jordan teach him how to play chess, helped make CHOC a kid-friendly place. I know any fear of doctors, hospitals and surgery that Jordan had were all lessened because of this environment.”

stomach pain or appendicitis
Jordan only had to spend one night in the hospital after his emergency surgery for appendicitis. In that time, he benefited from the generosity of a volunteer in one of the hospital’s play rooms, who let Jordan teach him how to play chess.

Jordan spent a few days resting at home and was back at school the following week.

“Dr. Yu helped get us through this high-stress situation with our son with the amount of professionalism and care you would ever want in a scary time. He is hands down one of the best. We’ll be forever grateful to Dr. Yu!” says Emma. “Now that I know how wonderful the emergency department and entire staff is at CHOC, I won’t take my kids anywhere else.”

Get answers to parents’ frequently asked questions about surgery

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Landon’s Life-Saving Jejunal Atresia Surgery

Lizette Lough, experiencing a seemingly normal pregnancy, was making final preparations to welcome her first baby, when her water unexpectedly broke at 33 weeks. She was rushed to San Antonio Regional Hospital in Upland, close to home, where her son Landon was born early on May 3, 2016.

After a few days in the hospital, Lizette and her husband Sean noticed the baby had not made a bowel movement. Tests revealed that Landon had an obstruction in his intestine. His physician recommended Landon be transferred immediately to CHOC for an emergency surgery with Dr. Peter Yu, a pediatric general and thoracic surgeon.

“My husband and I lost it,” Lizette says. “Our baby was only three days old and weighed about 4 pounds. The thought of surgery was beyond frightening.”

Upon arrival at CHOC, the Loughs were immediately made to feel at home by the staff, who helped them find a nearby hotel. Dr. Yu explained every scenario of the complex surgery in a compassionate and confident manner, the Loughs recall.

Jejunal atresia
Landon was born at 33 weeks and diagnosed with jejunal atresia, despite prenatal genetic testing and a healthy pregnancy.

“Landon was in stable condition when he arrived at CHOC, and I’m very pleased that our expert transport team was able to get him here quickly and safely. If there had been a delay in transfer, Landon could have become very sick and it’s very possible that more of his intestine could have died. If that would’ve happened, he may not have had enough bowel to adequately digest food, which can be incompatible with life,” Dr. Yu says.

Landon was diagnosed with jejunal atresia, a rare condition – approximately 1 in 5,000 births – in which the small intestine is incompletely developed, leading to one or more gaps, or blockages, in the intestinal tract.

Lizette had gone through the required genetic tests prior to Landon’s birth, and jejunal atresia – often diagnosed prenatally – was not detected.

Additionally, Landon had malrotation of his intestines, which failed to coil in the proper position in the abdomen. This led to twisting of his bowel. If surgery had been delayed for longer, Landon could have died.

Landon’s surgery involved making an incision on his abdomen, examining the entire length of his intestine and untwisting it, removing the dead bowel, stitching together his small intestine, and performing a Ladd’s procedure. A Ladd’s procedure places the intestines back into the abdomen in a safe configuration to prevent future twisting of the bowel.

jejunal atresia
Landon and his dad Sean in the surgical NICU at CHOC after Landon’s jejunal atresia surgery

Sean, who works as a law enforcement officer, recalls how traumatic this was for his family. “I’m used to working in stressful situations, but this was a different kind of stress,” he says. “We were so happy that our baby had a successful surgery and that he was better. However, we were still waiting for him to have his first bowel movement. We were trying to stay positive.”

After his first bowel movement indicated that his intestines were recovering well, and spending about a month in CHOC’s surgical NICU, Landon was finally able to go home with his family.

jejunal atresia
Landon and his mom Lizette during his first feeding, which took place in the surgical NICU at CHOC after his jejunal atresia surgery

“It takes a team to successfully care for sick babies and complex patients,” Dr. Yu explains. “Landon would not have had the excellent outcome that he had without our wonderful neonatologists, experienced and skilled pediatric anesthesiologists, Melissa Powell, our dedicated surgical neonatal nurse practitioner, and the outstanding NICU nurses who have dedicated their lives to taking care of newborn babies such as Landon and countless others. Together, we have the only dedicated surgical NICU in the area, with a special focus on taking care of newborns with surgical problems.”

Thanks to the expert multidisciplinary care provided at CHOC, today Landon is a happy baby, meeting all his milestones. The Loughs are enjoying their brave little boy, and look forward to his first birthday next month.

jejunal atresia
Landon is looking forward to celebrating his first birthday at home with his parents.

“Dr. Yu and the nurses in the NICU were so empathetic and amazing. They saw us through so much throughout our stay and we will forever be thankful,” Lizette says.

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Landon on his first day of preschool.
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