Ian hospitalized, but stable.

The poor Adams family tried for what feels like the 100th time to take a family trip out to see Ginger’s family in Albequerque, and Ian became very sick. Here is Ginger’s summary:

“Ian has been hospitalized since yesterday (Saturday). He had been sick with a cough and runny nose that we had been treating with albuterol breathing treatments and he was acting like he felt pretty well. Our family had plans to take a trip to visit family in Arizona and New Mexico.

Henry seemed to be feeling worse on Christmas day in the evening when he started vomiting but he seemed to be doing well enough that we decided to drive to Phoenix as planned on the 26th. At that point, Ian seemed to be getting over his cold and was acting fairly normal. We spent the night at Ginger’s Mom’s house and took our flight to Albuquerque early on the 27th.

Ian started vomiting before the flight and once after we landed. Ginger’s dad picked us up and we made the 3 hour drive to Ginger’s Dad’s house. Ian was acting a little more clingy and by the next morning, he was acting lethargic.

We decided to take him back to Albuquerque to UNM Children’s Hospital. They saw us and initially planned to observe for 6-8 hours given his medical history, despite him saturating well on room air and lungs sounding good. Theydid a chest x-ray and started oral steroids to treat “viral induced asthma” (because of his chronic lung disease, his lungs are fragile and sensitive to virus irritations, altitude changes, temperature change and all of these things happened with our trip).

He does not have pneumonia, his lungs can react to irritation by getting inflamed and twitchy. A few hours of being in the ED, he started to need oxygen support, and more when sleeping. Today he was able to go without any oxygen, but desaturated when he slept, so they will watch him tonight and if he is able to sleep without needing oxygen support, they will release us in the morning. Our flight back is scheduled for the 31st.”

Caid helping out little bro Ian with breathing treatment

The boys have had a few bugs over the past months and generally have been able to fight them off with some doctor visits and breathing treatments. So that’s been encouraging.

As frustrating as it is to have another attempt to visit family interrupted, I know Ginger & Joey are just happy that Ian appears to be stable.

Please be praying and thinking of them! I’ll update again as soon as i know more. ~cnp

a few recent pictures of the boys…

The family celebrating Caid’s 8th birthday, and the twins’ 3rd back in October

Ian hanging an ornament

Henry’s turn!

…and big brother.

happy day

SUPER happy update from Ginger:

“Ian came home this evening, which came as a surprise to us!

Ian has been continuing to improve in energy and has been eating and drinking well. Two nights ago, Ian (and me) got very little sleep because Ian is so afraid and anxious in the hospital that he wanted to be held all the time. I had been holding him in the bed, but they said PICU policy states I can’t be in his bed with him, so I had to be in a chair close to his IV pole, which was impossible to sleep in.

visiting with the brothers yesterday

Joey stayed the night with Ian last night so I could come home to get a good night’s sleep. Ian ended up crying almost the entire night asking to go home. This morning we asked about taking him home and if it would be possible to do IV antibiotics and antivirals ourselves and they had told us that it probably wasn’t going to work, so we anticipated staying until Monday. But then this afternoon they said we could switch to oral medications and come home.

Ian was so happy and so much more at ease since we have been home.  Thanks to everyone for the love and prayers!”

First bath in almost 2 weeks!

Also, we all remembered Gwennie yesterday, as it was her 5th birthday…~cnp

love you, darling girl

Tuesday morning

Ginger writes:

Yesterday Ian was very agitated for most of the day with lots of discomfort as his bowels and bladder started to work again. I think he was also agitated from having been on pain medications and sedation for some days and having them turned off.

We have been giving Tylenol to help with his likely sore throat (from the ventilator tube) and we just need him to start with clear fluids. He is refusing so far, but I’m sure as he continues to get better it will happen.

He is extremely traumatized by this hospitalization – this is a difficult age when he is old enough to be afraid and remember that doctors and nurses did things that hurt him, but not old enough to understand why or that a temperature or blood pressure is not going to hurt.

snuggle with dada

They removed his arterial line yesterday from his foot and may remove more of his IVs later. He still has a femoral central line and three peripheral IVs. He is done with steroids as of yesterday but is still receiving anti-viral and antibiotics. Another positive note: his platelets came up a little today – not to normal range yet, but today was t he first time they went up instead of dropping! He has been off oxygen since this morning and they plan to transfer us to the general peds floor today out of ICU.

We hope to work with physical therapy to help Ian once he is up for it, since he is very weak.

Waking up…

Ginger writes around 9:00 am:

“Just had rounds. The plan for today is to turn off sedation and pain medication so he can wake up a little more. They will also take an arterial blood gas to check that his oxygen and cO2 levels are good and then they will extubate.

In rounds this morning they said he weighs 8 kilos (17.5 lbs)! His weight before this hospitalization was 10.9 kilos (24 lbs). I could tell he lost weight, but this was a shocking amount to me. He has been getting IV nutrition, but we need to have everything go well so he can get back to eating normally as soon as possible!”

A little later, Ginger sent this picture…

thin & drugged up but successfully extubated

Ginger hopes to be able to hold him soon…

update & visit from Caid

Ginger writes:

“Ian continues to improve, although he is still on the ventilator. He is off dopamine (blood pressure support) and they removed his Foley catheter. He was fairly alert for about an hour when I came in. The nurse told us to ask him to move his foot, and he did it and also blinked when we asked that of him too!”

Caid came to spend some time with his little brother, along with Grammy Wren and Joey

I asked how Caid was doing with all of this, and Ginger responded simply:

“After he visited with Ian, he just wanted to hold Henry in a big hug, and I talked to him a little, but he didn’t say much. I thought it was pretty amazing that a seven year old’s reaction is pretty much the same as an adult’s – a new appreciation for things previously taken for granted.”

~cnp

GOOD news

Ginger writes:

Ian shows steady improvement. They successfully switched him to a regular ventilator last night and have been able to continually wean the settings.

They also weaned his sedation and pain medications a bit. This is so he would begin to initiate breaths, which he is now doing, so he is on a different setting on the ventilator which gives him extra pressure with each breath.

We are hoping that he may be able to come off the ventilator sometime tonight or tomorrow if he continues to improve.

Just so everyone knows, the doctors are a bit mystified as to how Ian improved as much as he did before they gave him enough antivirals to warrant it. Thank you, thank you for the battle you have all been fighting in prayer for this family! ~~cnp

the full scoop

Ginger wrote up the complete story of what’s been going on with Ian this week…

On Saturday Ian wanted to be held more than usual, so we thought he might be coming down with something. Sunday he started having a high fever and was very sleepy. I was worried that he had a urinary tract infection since the Monday prior he had had a urology procedure at CHLA and had no cough or runny nose.

The next day, Monday morning, it seemed his fever was broken and he was acting better, but we still got an appointment with our pediatrician. At the pediatrician’s office, he started acting tired again, and when the doctor listened to him, she heard crackling and wheezing in his lungs. She took his blood oxygen and it was 88%. They gave him an albuterol treatment and after that his blood oxygen went to 92%. Our pediatrician (who just last month sent Ian to CHLA in an ambulance from her office with pneumonia) said that we needed to go to the ER because with his chronic lung disease she wanted to make sure he didn’t get any worse.

Grammy Wren met us at the pediatrician’s office to take Caid and Henry, and Ian and I went to the CHLA ER. In the ER, doctors didn’t hear any wheezing or crackling, they took a chest x-ray, which didn’t look to have any pneumonia. We stayed in the ER for most of the day on a blood oxygen monitor which showed him to be saturating at 97-99% the whole time without any oxygen, so they sent us home on breathing treatments with a plan to follow up with our pediatrician. He had wanted to sleep most of the day that day in the ER, but the doctors thought it was likely due to fever and a virus.

Tuesday he continued to have a fever and seemed to progressively look like he was feeling worse and was still very sleepy and wanting to be held, so we brought him back to the ER at CHLA. While in the ER, he developed a rash on his trunk and back, and petichiae on the inside of his elbows, and he continued to act more lethargic, although he was saturating well without oxygen help. The doctors quickly decided they would admit him, started an IV and two kinds of broad spectrum antibiotics, did a repeat chest x-ray and blood work showed low platelet counts.

Once we were transferred to the oncology floor he started desaturating and needed oxygen.

They continued to do blood draws as hematologists ordered HLH specific labs, coagulation studies, and repeat CBCs to watch platelets (which continued to drop). By morning he was requiring higher oxygen support and as the day progressed, he was working harder to breathe, not opening his eyes as much and I was fearing septic shock (he looked and acted like Gwen did right before she went into septic shock). I shared my fears with the doctors and they were monitoring his blood pressures closely, which started dropping – and they started giving fluids to try to maintain his blood pressure.

I requested that they have PICU doctors consult right away instead of waiting and as those doctors arrived, his blood pressure continued to drop and he started coughing and it sounded very wet. They decided to transfer him and he went into shock. They sedated and intubated him, and Ian came through it okay. They continued to draw labs which showed his platelets continuing to drop, so they transfused him with platelets. They placed a central line which developed a bleed around it (from low platelets) and could not get an arterial line because of him being in shock and bleeding. They decided to hold off on the arterial line and he was stable for a few hours.

Late evening as they were suctioning him (something normally done when on a ventilator) he crashed – his heart rate, blood pressure and oxygen all went down and they called a code. They gave him rescue meds and hand bagged him (meaning they took him off the ventilator and had a respiratory therapist ventilate him by squeezing air through an apparatus to ventilate him) Once his numbers were better, they decided to place an arterial line with a cut-down (minor surgical procedure). After the arterial line was placed, they switched him to an oscillating ventilator and gave him nitric oxide to help with pulmonary hypertension. He responded well to those and they were able to stabilize him.

The rest of the night was fairly quiet and Joey and I were able to sleep for a stretch.

lamby

Today Ian has been improving, his blood work looks better, they have been able to wean the settings on the ventilator, and they have identified the virus they think caused him to get so sick. He tested positive for metapneumovirus, which is a virus that many people get and have viral cold symptoms with, but since Ian recently had a transplant and has chronic lung disease, he is more prone to developing serious complications.

They performed the bone marrow aspirate and it looked good, they feel confident that it is not a reactivation of HLH. Goal is to try to get him back on the regular ventilator tonight if he will tolerate it.

Keep on keeping on

Ginger here, checking in with an update on Ian’s progress. Things have been very good, not a day goes by when I am not grateful for being able to do normal things with the boys, like the park, the zoo, another park, more parks.

I am very germaphobic, so we are still sticking to mostly outdoor activities, since it is flu season. Despite that, Ian (and everyone else) picks up their share of germs and I am learning that Ian is still susceptible to developing pneumonia. We all got a little something at the very beginning of the month and Caid and Henry got through it with runny noses, a cough, but Ian’s turned into pneumonia in both lungs. We avoided the hospital, but had to spend several hours in the pediatrician’s office for several days getting antibiotic injections and breathing treatments.

Ian has permanently damaged lungs from the pneumonia and HLH flare that caused his respiratory failure and almost took his life. Our pediatrician encouraged me by saying that as he grows (and therefore as his lungs grow), the percentage of the damage will become smaller in proportion to his lungs, so he may be able to overcome some of this difficulty with developing pneumonias frequently. He said he may not be able to participate in sports that require a lot of exertion, we just have to wait and see how he does. And when I asked if this means that Ian should definitely not smoke, he was adamant about his agreement. 🙂

We had our monthly checkup at CHOC for blood work and Ian gets to take a break on vaccines for 9 months or so. One has to be completely revaccinated post BMT, because a BMT wipes out the immune system and replaces it with a new one. So, another thing poor Ian has to endure. When strangers comment on how he is so much “less friendly” than Henry, I don’t go into why, because they don’t really want to know; they just think they are making an observation about how “twins are always so different”. Which they are. But still…

Anyway, so because it is the height of flu season and we are going to the cancer clinic where every kid is immune compromised from chemotherapy, CHOC asks that only the patient and parent come to the appointment. Luckily, Papa Steve is close to CHOC and has been able to meet us there to watch Henry for the approximately 2 hour appointment.

So we received his latest chimerism number (the percentage of donor blood system) which was 57%. I know, that sounds bad, but his previous was 53%. When the doctors got the 53% number, they sent a T-cell chimerism count in addition to the regular chimerism. Basically, T-cells are the important thing with HLH. The donor T-cells are the thing you need to keep HLH from “re-flaring” (if that is even a word!) The T-cell chimerism count came back as 100% donor, which is excellent news. So we don’t worry about the 53% or 57% chimerism numbers, we just keep watching it.  Why don’t they just do a T-cell count every time? My guess is, because it is ridiculously expensive and specialized. So they just do it when they are worried about dropping chimerism numbers.

So yay for that news! I am daily reminded of how fortunate and blessed we are to have Ian, and to have him doing so well. As I have learned more about HLH, I really see how many obstacles there are, not just with the diagnosis and the disease, but with bone marrow transplant. And we have been incredibly blessed with so much support from so many!

sick (but happy!) little boys

Ginger wrote in a comment on facebook yesterday:

We went back to the doctor today and both boys have ear infections and Ian now has pneumonia.

The pediatrician gave him a “hospital grade” injection of antibiotics and we have oral antibiotics to take at home along with continuing breathing treatments. I am hoping to see improvement soon with the antibiotics!

just a quick update for everyone who is praying and asking! (: ~cnp

sick little boys need breathing treatment

these faces. ❤

 

steps forward, and steps back

Ginger writes:

I was riveted by this story on NPR today! This story is so amazing and exciting for what it could mean for many many people!

In less exciting news, Henry and Ian tested positive for RSV today. If anyone feels led, we would appreciate prayers that they would get better and avoid the scary complications.

On the bright side, they didn’t get it until now (as opposed to getting it as infants), but because they are ex-preemies and Ian is post BMT and post ECMO for respiratory failure, they are at more of a risk for complications.

making gingerbread cookies with the big brother

Ian eats his gingerbread man.

 

Henry thoughtfully nibbles his.

thank you as always for everything you all have done to encourage us…i know this is discouraging news for the Adams. we’re still hoping and praying for a peaceful Christmas! ~cnp