Biography
Maegan was born on June 20, 1996, five weeks early. She weighed 6 pounds, 3 ounces and was 17 ½ inches long. Maegan spent 18 hours in the NICU of Wesson Women and Infants (Baystate Medical Center).
When Maegan was six weeks old she was diagnosed with severe GERD (gastroesophageal reflux disease) . She was medicated with Prepulsid and Tagamet. Maegan was seen weekly from the time she was six weeks until she was six months to insure proper weight gain. Maegan had RSV (Respiratory Syncytial Virus) when she was five months old and was treated with Albuterol nebulizers and antibiotics. When she was six months old, the pediatric gastroenterologist felt the Maegan had outgrown the GERD and the medication was stopped.
Aside from colds, an occasional ear infection and chicken pox (seven months old), Maegan has been healthy. When Maegan was seven, she had pneumonia and mononucleosis. Recovery took about eight weeks. In the fall of 2006, Maegan’s health began to decline. In October she experienced stomach aches and vomiting in the middle of the night (she wouldn’t wake up, she would just throw up). Several trips to the pediatrician and Maegan was given a diagnosis of “abdominal migraines”. Maegan was constantly fatigued. In November, she began having headaches in addition to the stomach aches. These were treated with Excedrin and Inderal.
December began and the headaches continued. A ct-scan was ordered. On December 12th, she was diagnosed with a sinus infection. That is what the ct-scan showed. On the 20th of December, Maegan came home from school complaining that she didn’t feel good. I told her to go in a lay down. I called the pediatrician’s office and the nurse said there were a lot of “viruses” going around and we should give it a few days. I called Michael at work he said to call them back and get an appointment that this had gone on “long enough”.
Maegan was seen that evening and had an oxygen saturation rate of 94%. Maegan had two nebulizer treatments in the office. Antibiotics, an oral steroid and an albuterol inhaler were prescribed. Maegan had a follow-up appointment two days later and was then placed on a nebulizer every four hours. Maegan got better, but the cough lingered. We were told she could have a cough for several more weeks. Finally the cough subsided, but only for a couple of weeks. Then it again, came back and then subsided. This cycle continued for weeks. In April, 2007, Maegan had a chest x-ray. This was normal. However, the cough remained.
We continued to be in touch with the pediatrician’s office. On the 3rd of June, 2007 Maegan was prescribed Singular. It was now felt that this all could be allergy related. On the Saturday the 16th, Maegan complained that she didn’t feel good. We were at camp, so we had her go and lay down. She slept for a while. I took her temperature and it was 103. We gave her Tylenol and Motrin to bring the temp down and I gave her some Sudafed to help with the congestion. On Sunday, Maegan said she felt much better. She was more herself and was happy playing and actually went swimming.
I told Maegan that she didn’t need to go to school. It was “Move-Up” day at her school and Maegan wanted to go. I drove Maegan to school and told the secretary to call me if Maegan came in saying she didn’t feel well. She wanted us to see the school nurse. She listened to Maegan’s lungs. Her words “get her to your doctor’s office immediately”.
We arrived at the pediatrician’s office and Maegan vomited in the parking lot. Once in the office the nurse took her temperature, and got Maegan’s oxygen saturation level which was only 89%. They gave Maegan three albuterol treatments and with no improvement Maegan needed to go to the hospital. An ambulance was called and she was rushed to Milford Medical Center.
Upon arrival, the ER doctor sent Maegan for a chest x-ray. Maegan was diagnosed with bilateral pneumonia and was admitted to the hospital. Maegan spent her eleventh birthday in the hospital. Maegan was released on the 21st with a diagnosis of asthma. It was felt that because Maegan was not being treated for asthma that the pneumonia developed. Maegan came home on antibiotics, flovent, prednisone and round the clock nebulizer treatments. Dr. Lyons added Atrovent to the list of medications. Two weeks later the pneumonia still had not cleared. Dr. Lyons was able to get Maegan an appointment with Dr. Dorkin, the pediatric pulmonologist on the 11th of July.
We saw Dr. Dorkin and he felt that Maegan had GERD and added Prilosec. He felt that she was aspirating into her lungs which is common with mid and lower lobe pneumonia which Maegan had. Two weeks later, there was mild improvement, but Dr. Lyons felt that we should do another round of prednisone. Maegan began a 20-day cycle.
The end of August Maegan was diagnosed with bilateral pneumonia again.
In September, 2007 Maegan had a bronchoscopy. This was done at Massachusetts Eye & Ear Infirmary (MEEI) through the Airway/Swallowing clinic. It involved three doctors: an Otolaryngologist (ENT), Pulmonologist and Gastroenterologist. The cultures that were taken came back showing several different forms of bacteria. The biopsies came back showing no ciliary defects and the PH study for gastroesphageal reflux came back showing severe reflux. Dr. Dorkin treated with augmentin, however the pulmonologist from MEEI felt that the cultures had been contaminated. Dr. Dorkin, however, had Maegan finish the course of antibiotics. The pulmonologist from MEEI also had a genetic panel done. The preliminary results came back. There were no genetic defects. We breathed a sigh of relieve. Maegan, unfortunately was still not getting better!
On Tuesday, October 16th, my cell phone rang at 8:04 am. I had just arrived at school. It was Dr. Harnick from MEEI. There had been an addendum to the original testing. The addendum diagnosed atypical cystic fibrosis. Further testing was done and only one gene could be identified. It is felt that the other gene has not yet been discovered.
As November began, Maegan was getting worse. Finally on the 29th, she was admitted to Mass General. Maeagn was placed in isolation. She would spend nine days there. Maegan had a PICC line placed, which was a traumatizing experience. Maegan had IV antibiotics until the 24th of December. Maegan finally showed improvement.
In the midst of this, Dr. Dorkin left Mass General for Childrens Hospital Boston. Michael and I decided to go with Dr. Dorkin to Childrens. When Maegan’s health began to decline again, Dr. Dorkin felt that she might have Primary Ciliary Dyskinesia (PCD). There are six sites across the United States that do research on PCD and Dr. Dorkin contacted Dr. Margaret Leigh at the University of North Caroline at Chapel Hill. This is the lead site. Dr. Dorkin began the process of getting Maegan accepted into the research study. In the mean time, Maegan required another hospitalization and round of IV antibiotics. She also had another horrific PICC line experience. Michael tried to explain to the nurse, however he was not successful in getting them to understand. When the procedure was complete, the nurse turned to him and said, “I guess you were right”. Those words do not take away the fact that it was yet another traumatizing experience for Maegan.
In March we received the call that Maegan had been accepted into the PCD study. We flew to North Caroline on April 2nd. We were fortunate enough to be able to stay at the Ronald McDonald House in Chapel Hill. The study began with questions, blood tests, Pulmonary Function Tests, Nitric Oxide measurement and a nasal scrapping for a biopsy. It takes months to get the results of the biopsy. Maegan’s Nitric Oxide measurement was a 9. Normal is around 300. Individual’s with a NO measurement of less that 60 are usually individuals with PCD. Based on Maegan’s NO measurement she was diagnosed with PCD. Dr. Leigh invited Maegan to participate in the Longitudinal Study.
While in North Carolina, Maegan kept complaining that her neck hurt. She was not running a temperature and we thought it was just from the way Maegan tends to sleep. We flew home on Friday. Saturday morning Maegan woke up and her neck was extremely swollen. After a visit to the pediatrician’s office, Maegan was sent to the emergency room. A ct-scan revealed Maegan had a bronchial cleft cyst. Maegan had three doses of intravenous antibiotics and was also prescribed an oral one. We saw an Otolaryngologist. The plan was to do antibiotics for 14 days then surgically remove it a week after finishing the medication.
Maegan’s pulmonologist felt that she was to “respiratory fragile” to have the surgery done anywhere other the Childrens. He consulted with Dr. Volk (ENT from Childrens). An appointment was made for Maegan to see him on the 9th of April. He agreed that the cyst needed to be removed. He however did not feel the same urgency as Dr. Wilson. Dr. Volk was also concerned about the amount of fluid in both Maegan’s ears. He felt that Maegan would benefit from having tubes placed in both ears. It was a fight over the surgery date. We finally were given a date of May 12th. Also at this time Maegan would have a port-a-cath inserted to there would be no more PICC lines.
The day of surgery arrived and Maegan was scheduled for 2:30. We arrived at the hospital around noon as instructed and finally we were finally taken to pre-op waiting at 3:15. Dr. Volk came in to exam Maegan a short time later. He could not feel the cyst. He wanted to cancel the surgery and do an MRI in a couple of weeks. The surgery would then be rescheduled. Michael and I were livid. We asked why the MRI had not been scheduled prior to Maegan’s pre-op appointment. Dr. Volk’s response was “I forgot”. We requested that at least the port be inserted that day and since Maegan was already going to be under anesthesia Dr. Volk went ahead and did the tubes. Maegan did great with the anesthesia. Maegan was sent home later that night with tylonal w/ codine for pain and ciprodex drops for her ears.
The week before Memorial Day weekend, Maegan started complaining that her ears hurt and she had some nasty drainage coming out of both. I called Dr. Volk’s office and spoke to his nurse. She felt it was an ear infection and was going to talk to Dr. Volk. Dr. Volk prescribed the ciprodex drops again. We were told it would take a few days, but would clear up. It didn’t. We saw the physicians assistant, Bill, at our pediatrician’s office. He felt she needed to be seen as soon as possible by Dr. Volk. I called Dr. Volk’s office first thing the next morning. Dr. Volk called me back and said we needed to give the drops more time to work. This was not a big deal.
On Monday we saw Dr. Kahn (a partner of Dr. Lyons). He too felt that Maegan needed to see Dr. Volk. He cultured the drainage and prescribed 875 mg of augmentin to be taken twice a day. Maegan started the medication, but within 2 hours of taking it she had extreme diarrhea. In meantime, Dr. Kahn was able to get Maegan an appointment with Dr. Volk. We saw Dr. Volk on Wednesday and he again said that this was just an infection and was not a big deal. He did change the antibiotic to zithromax. He suctioned out as much the discharge as he could from both of Maegan’s ears. I told Dr. Volk that Dr. Kahn had cultured it on Monday. Dr. Volk said that was a waste of time and wasn’t going to show anything. Dr. Volk did arrange for Maegan to have an MRI the next afternoon. Maegan also had an appointment the next day with Dr. Dorkin. He felt that Maegan’s sounded better than before the surgery.
We proceeded to the MRI. The MRI took one look at Maegan and said that she would try to do the MRI, but didn’t think it would work because of Maegan’s braces. She recommended having the braces removed and then having the MRI. We left there very discouraged.
On Friday, Dr. Lyons called with the result of the ear culture. Maegan had a MRSA infection. Dr. Lyons had Maegan admitted to begin IV antibiotics. Dr. Lyons also asked Dr. Wilson (ENT) and a physician from Infectious Disease to consult. Maegan spent 24 hours inpatient. She had three doses of IV vancomycin and then was switched to oral zyvox. Maegan was seen twice a week, then weekly, and finally bi-weekly until the infection was completely gone which wasn’t until the end of July.
July and August were relatively “healthy”. Maegan was constantly doing something, whether riding her bike, scooter, swimming or just running around with the other kids at camp.
School began in September and Maegan moved onto middle school. She adjusted well and began playing soccer. Health wise, Maegan’s lung sounds began to change. She was diagnosed with bi-lateral pneumonia. She began taking zyvox. Maegan began to improve, but then on the 22nd her lung sounds were “different”. Maegan was taken by ambulance from school to Milford Hospital and then transferred to Mass General. IV unasyn was started and Maegan spent four days inpatient. A chest ct-scan was done and showed bronchiectasis in the both lower lobes and in the middle right lobe. Bronchiectasis is irreversible lung damage.
Maegan returned to school on the 29th of September. She finished her IV medication on the 7th of October and will complete a 14 day course of 500mg augmentin twice a day. She has returned to soccer and her regular activities.
The progression of Maegan’s illness is unknown. We are currently exploring fund raising opportunities so that we can travel back to North Caroline to begin the longitudinal study. Maegan’s doctors do the best they can to treat her, however, there are only two known cases of PCD in Massachusetts. Maegan is one of the two.
Michael and I will whatever is necessary for Maegan so that she may have as “normal” a childhood as possible.
Thank you for taking the time to read about Maegan and be part of her life. Your prayers are support are greatly appreciated.
Written by Maegan's mom in October 2008
