Mallory Hope

Mallory and Marah

Mallory was born on November 15, 2001. One week later we took her for her one week check-up. Before leaving for the doctor's office while we were at home, she did not act as hungry as she had the day before and was very sleepy. At the Doctor's office, she began making some moaning type noises and seemed uncomfortable. Nichole decided to try to nurse her and see if it would calm her down. Shortly after she went to the back to nurse, the nurse came out and told me I could come on back?they had gone to an exam room.

When we went into the exam room, she still had not eaten and was moaning more than before, with what seemed like every breath. The nurse practitioner was concerned with her color and the way she was acting, so she called the doctor in to examine her. After the doctor came in, everything got real crazy. The staff at the doctors office handled the situation extremely well. They intubated her and got oxygen to her, however her heart was stressing out.

We were sent to the pediatric ICU in Macon where they started prostaglandin that re-opened Mallory's ductus. They classified it as if she went into cardiac arrest. The PICU at the Medical Center of Central Georgia in Macon, GA did an awesome job in diagnosing the problem and stabilizing Mallory's situation. Our stay there was limited to around 3.5 hours and we were quickly transported out.

Mallory was diagnosed with an interruption in her aorta and we were rushed to Children?s Hospital of Atlanta. We found out she also had a VSD, an ASD, a narrowing of the left ventricle and a smaller lower left chamber which, all together, camouflaged the problem before and after her birth. Her pulses were all the same on all extremities and the murmur was so large the turbulence could not be heard. With the prostaglandin holding the ductus open, Mallory?s condition was stable. The doctors indicated the longer they could keep her stable and allow her build up her strength, the better she would handle the surgery.

We were able to stay at the Ronald McDonald House, which wound up being more therapeutic than being at the hospital. We stayed at Mallory?s bedside almost constantly with our older daughter, Marah, who is five. Marah was our strength throughout the hospital stay.

It was the doctor's intent to perform surgery on the Monday after Thanksgiving barring any complications. Dr. Vincent Tam was to be her surgeon, and we couldn?t have asked for anyone better. He took time to explain her condition and what he was going to do when he got in. He really had two alternatives, a full repair and the Norwood Procedure. We were hoping that he would be able to repair the problem, but both of us knew that it was in Dr. Tam?s hands, with a lot of help from God, and that he would have to make the best decision for her when he got in. The surgery started on time and finished early. Dr. Tam came in and let us know that he had repaired the problems and her entire heart was functioning as it should. The only problem we know of is a very small VSD, which they feel will seal on its own, and some turbulence in the narrowed ventricle that will continue to be watched and could lead to surgery later.

We stayed in CICU for four or five days after the surgery and then moved to the floor. We were on the floor for two days and then went home.

October, 2002

We have been back to the cardiologist, Dr. McConnell, several times now. First, once a month, then every three months and ultimately every 6 months. It was scary to both my wife and myself to go so long with out a visit or checkup, but the doctor felt comfortable and reassured us we would know well before any problems arose.

?Mallory Hope? is 11 months now, crawling everywhere and quite the busy one. Our last visit to the cardiologist resembled the previous visits with no changes in the echocardiogram which we watch very closely! Her weight gain has been adequate, she?s small, but not too small and seems to be following the path of her sister in size. She has a definite attitude when in comes to her way, and has begun learning and understanding what we are saying when we talk to her.

She began talking at 9 ? months saying ?momma? as her first word and is yet to want to say ?daddy!? She loves her dogs and loves to make the sound a dog makes, which with her is more a grunt right now than woof! She?s a beautiful normal baby who loves to laugh and loves books. If you did not know there was a scar on her chest, you would never know there had been a problem. But we all know there was, and that there may be others. I say all those things because as a parent experiencing what we were experiencing, we couldn?t find anything that told us what to expect with her.

March, 2003

The cardiologist has discussed Mallory?s situation with the surgeons, and all are in agreement that there is no need to try to correct the narrowed ventricle at this time, their concern is with the Pulmonary Valve and whether the turbulence through the narrowing may damage it. Her prognosis for additional surgery is some time in the next 3 - 10 years depending on her growth and the expansion of the ventricle, with the intention of correcting the narrowed ventricle and possibly replacement of the pulmonary valve at that time.

Ultimately, we don?t know when or if we will need another surgery. At the post-op conference with the surgeon, he made the statement that at some time in the future we may have to have a valve replaced. As for the original surgery, the repair, he felt her aorta should not cause her any problems. As for the pictures we have seen on the echo, all is well with it and her ASD and VSD are both closing. Dr. McConnell has mentioned the valve as well. We have placed it in god?s hands, and will take it one day at a time.

From the beginning of our situation, we watched God?s hands take care of our little girl. From placing us at the pediatricians office when everything began, to the pediatric ICU and the friends and knowledge that were there to take control, to the room at the Ronald McDonald House being available, to the writing on the stone in the garden ?Hope? (her middle name), to my 5 year old daughter wrapping her arms around a weak momma and daddy to let us know that everything was going to be O.K., and her prayers for god to take care of her sister, we all felt god?s presence during the entire event.

February, 2003

March, 2003

We went back to the cardiologist this week, January 17th, braving the snow, and we saw a few flurries! Normal procedure with Mallory is to do her weight, length, EKG, and pulses and because of a loud murmur, the doctor really can't tell anything without doing an echocardiogram! She was perfectly content with the echo and calm until the end and then wanted to crawl around and was struggling to get down. We always want a calm reading and try to get her to sleep before so the readings will be resting instead of agitated which causes the reading to be skewed.

After reading the echo, Dr. McConnell came in to discuss it with us. One of the questions he asked was, "had Mallory been passing out?" We told him that she had not, but he said that the readings for the obstruction below at aortic valve were substantially higher than they had been at her previous visit. Elevated enough that he was concerned and would be contacting the surgeons at Egleston/Children's Hospital in Atlanta to see if they agreed with him.

We were scheduled for a heart catheterization of February 10th and afterwards have been scheduled for surgery on March 24th to relieve the pressure through her aorta. We have talked with both the surgeon who will be doing the surgery and the surgeon who performed her first operation. Both are in agreement in what needs to be done.

As for Mallory, her color, activity level, appetite and attitude are all good. With Mallory being in good physical condition, it is felt that now is the best time to go forward with the surgery. The cardiologist has always said that he would know long before us if there were concerns.

April, 2003

Mallory went for surgery on Monday, March 24, 2003. She was in surgery for around four hours and on the heart lung bypass for around two and a half hours. The procedure involved relieving the left ventricular outflow tract of the obstructions that were hindering blood flow. Some of the problem came from a nub protruding into the aorta that was disrupting flow, some disruption was from the repair of the VSD and some leaflets on the bottom of the valve that were removed (that?s my understanding). All of these were repaired during the surgery by reaching through the aortic valve and shaving off necessary tissue. Also in the process, a couple of stitches were placed at the opening (hole) in the VSD to completely close it.

Mallory did very well through surgery, the nurse (Gretchen) called every hour letting us know what was going on and how she was doing! Her last call came around 1:00 saying that Mallory was doing fine was off the Heart Lung Machine and the surgeon was going to close her. Dr. Forbess and the other surgeons had discussed if they could not get the gradients they were looking for, proceeding with a Ross Procedure and possibly a Kono Procedure after that. We prayed for that not to have to be done and thankfully it did not! Mallory was in and out throughout the day after surgery Monday and woke up in the night and was fully awake by 08:00 the next morning. She was uncomfortable and we did what we could to make her more comfortable. Around 2:00 P.M. we were moved to a room on the floor and were allowed to hold her, which made things a lot better for her. ?A Mother's Arms? are the best medicine in the world. We were unsure whether it would hurt when picking her up, but she let us know if she was uncomfortable.

Mallory?s big sister, Marah, was there with her again for this surgery. We have tried to include her in everything so she has an understanding of the seriousness of the situation. By the end of the week Marah had gotten tired, as we all were, so she went home on Wednesday with our friends and went back to school on Thursday.

Everything seemed to go well for Mallory throughout the rest of the stay. The worst thing for her was taking the tape off her skin. She really did not like having the bandages changed. We were released from the hospital on Thursday around 2:30 P.M.

We went back for her post-op appointment and to see the cardiologist in the next two weeks. Both thought she was doing exceptionally well, but warned of fluid collection around the heart and told us the symptoms to watch for. Dr. McConnell said that it was likely that Mallory would need to go back for a valve replacement some time in the future and that we would just take it day by day! I talked with Dr. Tam and he said, ?there is reasonable chance that the left ventricular outflow tract may become narrow again, over time,? and that he felt it would be very likely that she would need to go back for surgery in the future.

Dr. Forbess said that it was his hope that Mallory could grow and get much stronger before the surgery to replace the valves was needed! This surgery definitely bought us some time before the next will be necessary.

October, 2003

We returned to the cardiologist on August 15th, 2003. Mallory has grown accustomed to the test, the EKG and all the stickers, the echo and the messy gooey stuff, and Dr. McConnell because he?s so nice.

Mallory is doing very well! Looking at her, you really can?t tell she had a problem. However, what God has in store for the future, no one really knows. Dr. McConnell?s visit went well, he discussed the surgery with us and how Mallory?s echo and EKG looked. There was no significant change in either since March, which we were very glad to hear. Her valve, ?in his words,? was not leaking any more than it had been, and the stenosis was no greater than it was after surgery.

We were scared at surgery (the second one) I think that?s only normal. However, if you look at the pictures and her actions before and watch her now, there is a definite change in Mallory?s overall demeanor. She seems to have more strength and smiles more. She is a little clown, and has fun with life!

October, 2004

Another year has passed! Most recently during the summer, we went to Scottish Rite Hospital in Atlanta for surgery to assist Mallory with her speech. We were not directed by anyone in regard to pursuing her speech problems, but after much research we began making calls to see if there was anyone that could help with her speech. We were given a list of doctors who may be of assistance who specialized in treating kids that have DiGeorge and 22.q.11.

We contacted the craniofacial clinic and Dr. John Riski, who is a speech pathologist at Children?s in Atlanta. He was very helpful and explained that he normally begins seeing his patients a little earlier than the age Mallory was but it was certainly not too late. We met with him on Friday February 13, 2004. This was a very long day, we met with Dr. Riski, Dr. Burstein (the surgeon), the speech therapist, and Dr. Thompsen (the dentist).

We also were instructed to go to the ENT to determine if her ears were potentially a problem.

X-Rays were taken at this visit of the pharyngeal area to determine if she had a structural problem preventing her from speaking. It was determined there was a problem and surgery would be necessary to fix it. The procedure needing to be done was called a sphincter pharyngoplasty. The purpose of the procedure was to reduce the size of the pharynx so the pharyngeal flap would close properly and allow her to say her consonant sounds. The wait was the worst part?the surgery was scheduled for summer of 2003 just before our older daughter was supposed to start school again. Surgery was scheduled for July 29th, 2003. We were in the hospital for three days; we went in ?outpatient,? and stayed after that. Mallory did well with the surgery, however she was scared when she woke up and they sedated her until she got to the room. She had a tube in her nose to keep the airway from closing off due to swelling. It stayed in for a day and after it was removed she did much better. Mallory will be needing ?one on one? therapy to change the habits she learned with her deficiency. We saw Dr. Riski on Thursday October 28, 2004. His evaluation indicated that Mallory is right where she should be after the surgery. Her intellectual skills seemed to be where they need to be, and that we just need to keep working on her speech. Mallory has a glottal stop, that when she tries to make a sound as simple as ?daddy,? with the ?dah? sound she has a hard time with it. For now my name is ?ah-dah? and mama?s name is ?ah-mah.? And you know what, ?that is a blessed sound!?

Prior to the surgery, the Fourth International Conference for 22q.11.2 Deletions was held in Atlanta on July 23 ? 25th, 2004. The conference specifically had speakers talking to the sphincter pharyngoplasty that Mallory was scheduled for the following week. The conference was highly technical, but helped us in the understanding the procedure. This conference was tagged along with the 10th Annual International Meeting of the Velo-Cardio-Facial Syndrome Educational Foundation, Inc.

December, 2004

As of November 15th, 2004, Mallory is now three years old. We had a birthday party with all her cousins, aunts, uncles and grandparents. We all really had a great day. So close to Christmas, it was like Christmas had come early.

We are scheduled back to the cardiologist is January, Mallory is really doing well. She has started going to an organized church day care type program several days a week to give Mama some time to get out and do things. Mallory has taken to it really well and enjoys the crafts and play time. She seems to be developmentally on track with the rest of the kids with exception to her speech.

The therapist is working with Mallory in regard to her speech once a week. As for her heart, we are not on any meds and her appetite is excellent. She likes most any food you put in front of her. The flu shot was a concern this year, however, she was a candidate, but the rest of us were not. Mallory has been taking a tumble class since Aug./Sept. 2004 and really enjoys it. I don?t think she wants her sister to outdo her. She loves the acrobatics and tumbling.

When a child has been through all that Mallory has you often want to coddle them and restrain them from doing the normal things kids would do. Mallory loves the outdoors and her pets. She loves riding her push toys especially downhill at higher rates of speed. She is a child with a big heart, very caring and very creative. Although she cannot communicate verbally, she does her best at sign (not formal) and gestures to let us know what she needs. She is very conscious of her surroundings and what is going on, and very into Christmas this season this year.

Her absolute favorites so far are: Rudolf the Red Nose Reindeer, her Zebra riding toy, SpongeBob, her GameBoy XP, Play Dough, Cracker Barrel, Wal-Mart, Any Car Wash, Ms. Penny, Marah and anything on the Dinner Table.

July 2005

I have been asked why we have not updated Mallory?s website. And besides being busy, each time we start, we decide to wait until the next benchmark.

Mallory did go to the cardiologist this past January. In the test performed, Dr. McConnell saw some things he did not like in the pressures through the Aortic Valve. He reduced the length of time between appointments to 4 months. He stated it could simply be a quirk in the test, but to be totally cautious, he wanted her to come back in 4 months for a repeat test.

If it was an accurate test, the results should continue to worsen. If that is the case we would go back for a heart cath to determine the severity of the problem. Of course, if bad enough, she would go back for surgery, the one that would need to be performed would be both a Ross and Konno Procedure, hence the Ross-Konno Procedure.

Mallory has been taking speech regularly for over a year now. She has gained some muscle control in her throat but still struggles with many sounds. We stay in touch with Dr. Riski, Speech Pathologist at Children's Hospital (Scottish Rite) in Atlanta to try to help her in what ever way she needs to develop her vocal muscles.

We returned in May for the four month recheck and there had been no change since January. Mallory still looks good! We will have a recheck in September to see if there has been any change for the good or bad.

He did tell us that there was no good surgery for Mallory at this time. If surgery is necessary, which ever one we choose, additional surgeries would be necessary as she continues to grow!

January 2006

We returned for another visit to Dr. McConnell to check on Mallory?s progress. The checkup went well, there was no indication that Mallory?s gradients had changed in any direction, not better or worse. Dr. McConnell was pleased, as we were.

Mallory is still struggling with her speech. She has matured enough to know that she is saying things incorrectly and will try to restate what she is saying to correct it. She is in speech therapy three times a week, and seems to have clicked with the therapist. We encourage her with audio type toys saying the ABC's and by reading and talking to her. Things that record or repeat what she is saying work really well. Mallory visited her speech pathologist at Children's Hospital of Atlanta, Dr. John Riski, this past week and he was very pleased with her progress over the past four months.

We continue to pray and watch our baby grow into a little girl. She is now in school and enjoying it and leading a very active life.