Diary January 2002
Jan 1 2002 News: A CT Scan revealed that Saffy's liver is considerably larger than when she was first diagnosed. She is scheduled for surgery tomorrow to install a Lifeport, so the chemotherapy may be administered directly to a major vein. She will then immediately start chemo.
Jan 3 2002 News: Saffy's surgery was not completed until late last night. Due to her small size and condition, she received a Broviac IV instead of a Lifeport. She began chemotherapy late this afternoon, and the first cycle will continue for three more days.
Jan 5 2002 News: Saffy received her third chemo dose today and will receive another tomorrow before resting for approximately ten days. Her immune system is compromised, so infections and colds are a serious concern. Exactly when the next chemo round starts will depend on the recovery of her white blood cells and the chemo's effect on her hemangiomas.
Saffy was also diagnosed with hypothyroidism, which is a byproduct of the hemangiomas and is probably the cause (or contributor) of her lack of growth. She has started receiving Synthroid.
Saffy was taken off the paralyzing drug two days ago. While she is still heavily sedated, she has begun to move a little and open her eyes.
Jan 7 2002 News: Saffy received the final dose of the first chemo round yesterday. She is doing well all things considered. She still requires a ventilator to breathe, and infection (or any illness) is a major concern. The next round will probably start in 10 days.
Saffy began receiving minute amounts of breastmilk via NG tube the day before yesterday. Today she is up to 4 cc per hour, which is about 3 ounces per day--not much!
Saffy's blood levels have also been a major concern. She received another blood transfusion yesterday, which is her fourth since being hospitalized ten days ago.
Jan 8 2002 News: While Saffy recovers for her next chemotherapy round, we continue to explore alternative treatment options in case the chemo fails. Unfortunately, her hypothyroidism has not improved. Her Synthroid dose has been doubled.
Jan 12 2002 News: Saffy's girth has increased, so we're not sure if the chemotherapy is working. A CT scan will be performed just before she starts her second chemotherapy round (currently planned for Jan 16), so we should have a better idea then. Saffy received Cyclophosphomide (Cytoxin) for her first round, but we are discussing changing the chemo to VAC (a combination of Vincristine, Actinomycin-D, and Cyclophosphomide).
Saffy's thyroid problem is coming under control, since she is now receiving mega doses of Synthroid. Her daily dose was increased from 25 micrograms to 50 to 200 to now 300 micrograms.
Saffy's heart is still carrying an increased volume load from the excess blood being circulated through the hemangiomas. Her left atrium is enlarged, but luckily she hasn't gone into congestive heart failure, which is a real danger and common with diffuse hemangiomas.
Saffy's breastmilk intake has increased, and she is now receiving 12 cc per hour via NG tube, which is 2 ounces every 5 hours.
Jeff Compagner, who's daughter Maria also had hepatic hemangioendothelioma, and other parents have been most helpful with information and support. Jeff listed Saffy on Maria's web page at www.MariasLiver.com.
Jan 15 2002 News: Unfortunately, Saffy's condition has once again taken a turn for the worse. Her abdominal girth has steadily increased, and we were hoping it was attributable to ascites (excess fluid in abdominal cavity). However, a CT scan performed yesterday revealed that the hemangiomas in her liver have continued to grow, and she is nearly literally bursting at the seams. The chemotherapy appears to have had no effect.
We are quickly running out of options. We've discussed another round of chemo (using VAC instead of Cytoxin), embolizations, and Interferons; however, a liver transplant may be the only option with a chance of success due to her severe condition and rapid decline. Saffy is being transferred to Mount Sinai Medical Center in New York City, where they can handle a living relative donor transplant (in which Daddy or Mommy or someone would donate a portion of their liver).
However, at 4 1/2 months, Saffy is still a very small child at approximately 10 pounds, so a transplant may not even be possible. Sorry to end this on a down note; but, unfortunately, this is the current status. Everyone has been very supportive, and your prayers and positive thoughts really mean a lot. Saffy is a tough little girl, and we continue to have high hope she will pull through.
Jan 16 2002 News: Saffy's transfer to Mount Sinai was delayed, but she is scheduled to be transported tomorrow. We're not sure if today's news reports [link updated] of the death of a living relative liver donor at Mount Sinai contributed to the delay, but it probably didn't help. (The hospital has temporarily suspended adult-to-adult living donor liver transplants pending an internal review, but living donor transplants to children aren't affected.)
Saffy is still heavily sedated and on a ventilator. Her girth continues to increase, and the massive size of her liver is compressing other organs. She can only take small breaths due to the pressure on her diaphragm and lungs, and the strain on her heart is increasing.
Jan 23 2002 News: Sorry for the silent treatment. Tonight is the first time home in a week.
Saffy was transferred to Mount Sinai via ambulance on Jan 17. She was evaluated for a liver transplant and the following day was listed as Status 1 (less than 7 days to live) on the UNOS national transplant waiting list. Due to the shortage of organs, Jill began tests to determine if she could be a live liver donor. Unfortunately, Alec was not a candidate due to his weight.
Saffy's condition worsened as the liver hemangiomas continued to grow. Her girth increased by approximately a centimeter each day, which put additional pressure on her other organs (especially her lungs) and increased the blood volume for her already overburdened heart. Luckily, further tests did not find hemangiomas in other organs (besides the superficial ones on her skin).
On Jan 19, our hopes were dashed when we learned that Jill was also not a suitable live liver donor. Nearly 20,000 people are currently waiting for liver transplants and over 1,500 die each year waiting.
To add to Saffy's problems, we learned that her Broviac IV was leaking, and she wasn't getting all her medications. A temporary IV was placed in Saffy's foot while her surgeons were consulted about a new line.
Luckily, later that day, we learned of the possibility of a cadaveric liver donor. Our emotions were certainly conflicted when we learned of some of the details of the donor, who lost her life as a young woman. Our heart goes out to this anonymous woman's family, who--during their grief--graciously agreed to donate their loved one's organs. The donor was brain dead, but her body was kept alive in preparation for organ donation. Amazingly, Mount Sinai planned a split liver transplant to save two lives! Saffy would receive the left liver lobe (30%), while an adult would receive the right lobe (70%).
After the suitability of the donor's liver was confirmed, Saffy was scheduled for surgery the following day. Because of complications related to Saffy's hypothyroidism, the transplant was delayed another day, and Saffy went into surgery on Jan 21. Due to Saffy's condition--especially her heart failure and hypothyroidism--her surgeons gave Saffy a 50% chance of survival during the operation, and the same chance of surviving the first few days with a new liver.
Saffy's transplant surgery took 11 hours. During that time, she received five units of blood plus plasma and platelets. Her diseased liver was about the size of a football. She lost nearly 3.5 pounds that day (much due to fluid loss).
All in all, Saffy's operation went well. The surgeons were even nice enough to repair Saffy's umbilical hernia. So far, her recovery shows good results, but she's not out of the woods yet. Her liver functions are improving, and her vascular structure seems to be working properly. Her hypothyroidism is getting better, so her Synthroid doses are being reduced. Unfortunately, Saffy's heart problems have not improved as quickly as hoped. She also required another blood transfusion yesterday.
Saffy is receiving a cornucopia of medications. She gets immunosuppressants to prevent the rejection of her new liver, which increases her susceptibility to infection. She receives antibiotics and other meds to fight infection. Saffy will need to take anti-rejection medication for the rest of her life.
Saffy is recovering in PICU. She is experiencing a lot of swelling and ascites, but her lung function has improved considerably. Her doctors began the process of weaning her from the ventilator today and also began feeding her small amounts of Pedialyte via NG tube. We expect Saffy to be hospitalized for another month.
We owe thanks to many people for helping us get through this difficult time. Our family and friends have been very supportive. Alec's employer has been especially understanding and accommodating.
The doctors, nurses, and other hospital personnel caring for Saffy have been wonderful. They have been especially good putting up with our endless questions and concerns.
We're saddened that a young woman lost her life, but we're grateful her family decided to save others by donating her organs. If you support organ donation, please discuss your wishes to donate with your family.
Hepatic hemangioendothelioma is an extremely rare condition. One pediatric textbook counts only 119 cases between 1970 and 1999. Saffy is now one of the very few patients to receive a transplant due to the condition. We are especially grateful for the help provided by Jeff Compagner, Julie Tovar, Sheila Zylstra, and Crystal Haydt, whose children also overcame liver hemangiomas.
Some pictures of before and after transplant have been added to Saffy's Liver Transplant Photo Album. (Not for the squeamish.)
Jan 29 2002 News: Saffy's recovery has been slow in the week since her transplant. We were expecting her to bounce back quicker; but, in hindsight, we were being much too optimistic.
So far, Saffy's new liver has been working relatively well. Her Liver Function Tests (LFTs) have been good in general, but some tests have shown results that could indicate rejection or infection. She was preemptively put on antibiotics, and her surgeons discussed performing a liver biopsy to detect rejection; but, luckily, her tests have improved and the biopsy has been delayed. This "fire drill" has been repeated a couple times.
To borrow a metaphor from her chief surgeon, Saffy's recovery will be a roller-coaster ride of ups and downs. She could reject her new liver tomorrow, in three months, or in three years. Infection is always a danger. A liver transplant is a life-long medical condition that will need to be managed non-stop. Of course, as time passes, the dangers will be reduced. Her immunosuppressant doses will be lowered, and her medical care will go from PICU to a regular hospital room to twice-weekly doctor appointments to weekly and then monthly appointments. Each of these steps could take months or years, but she has already passed the hardest part, which is the surgery and the first few days.
Unfortunately, Saffy's heart problems have not improved. Her heart is still enlarged, and her doctors now believe it could take 3-6 months before she fully recovers. We were hoping for a quick recovery now that the liver hemangiomas and the associated increased blood volume were removed; however, her heart muscle rapidly grew over a couple months, and it will take time for it to return to normal. Saffy was started on a new heart medication that will hopefully help.
Saffy's return to breathing for herself has been slow as well. Her doctors have tried to wean her from the ventilator a few times, but the results have been poor. Her left lung has collapsed a couple times, but she recovered each time the ventilator was reset to full. This problem could be due to edema (swelling), her heart problems, or damage to her diaphragm (or a combination of all three). The most worrisome is diaphragm damage, which could have resulted from the immense pressure in her abdomen prior to transplant or clamping during surgery. Saffy's doctors may need to look at her diaphragm using fluoroscopy to determine if it's damaged. If so, surgery may be necessary. However, her edema has since been reduced and recent attempts to wean the ventilator have netted better results, so hopefully swelling was the culprit.
Some good news is that Saffy's hypothyroidism has improved. She's now only receiving 15 cc of Synthroid per day (down from a high of 375 cc). Her doctors were concerned she might flip the other way and go into hyperthyroidism (which could add to her heart problems), but that hasn't happened. They expect to be able to stop the Synthroid altogether in a week or so. We're fairly certain that her large hemangiomas were the cause of the hypothyroidism--they actually metabolize the growth hormone--but a tissue sample was sent to a researcher at Harvard Medical School to hopefully confirm.
Saffy has also done well with her return to feeding. She went from receiving small amounts of Pedialyte via NG tube to larger amounts of breastmilk. She is now receiving 20 cc per hour, and the fluids and nutrition she receives via IV have been reduced considerably. Her constant blood draws take their toll though, and she received another transfusion today.
Saffy's weight has fluctuated considerably depending on her fluid levels. She floats between "wet" and "dry," but her true weight is probably less than 10 pounds. At five months, she's still the size of a large newborn.
Saffy's sedation has also been reduced as her doctors have tried to wean her from the ventilator. They still need to manage her pain and comfort though. She has actually built up a tolerance to the sedatives, and she has been switched between Fentnyl, Ativan, and morphine. She is often alert and awake.
Four new pictures have been added to Saffy's Liver Transplant Photo Album. (Still not for the squeamish.)
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